Glyn A. S.

Lloyd

Diagnostic Imaging
of the Nose and
Paranasal Sinuses

With 297 Figures

Springer-Verlag
London Berlin Heidelberg New York
Paris Tokyo
Glyn A. S. Lloyd. MA. DM. FRCR
Consultant Radiologist. Royal National Throat. Nose and Ear Hospital. Gray's Inn Road.
London WCIX SDA. UK.

ISBN-13:978-1-4471-1631-8 e-ISBN-13: 978-1-4471-1629-5

001: 10.1 007/978-1-4471-1629-5

Lloyd. Glyn A. S.
Diagnostic imaging of the nose and paranasal sinuses.
1. Man. Nose & paranasal sinuses. Changes in radiography
I. Title
616.2'12075

Library of Congress Cataloging-in-Publication Data

Lloyd. Glyn A. S.
Diagnostic imaging of the nose and paranasal sinuses.
Includes bibliographies and index.
1. Nose - Imaging. 2. Paranasal sinuses - Imaging. 3. Diagnostic imaging. I. Title. [DNLM: 1. Diagnostic Imaging.
2. Nose Diseases - Diagnosis. 3. Paranasal Sinus Diseases - diagnosis. WV 300 L793s]
RF345.L56 1988 617'.5230757 88-4620

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Preface

In the past two decades the radiological investigation and imaging of paranasal sinus disease
has been revolutionised by the introduction of two new techniques: magnetic resonance and
computerised tomography. These have made the diagnosis and localisation of neoplastic and
non-neoplastic disease a far more exact process and they have completely replaced con-
ventional pluridirectional tomography as a means of augmenting the initial plain radiographic
examination of the paranasal sinuses. The recent introduction into clinical practice of the
paramagnetic contrast agent gadolinium DTPA has extended further the range of magnetic
resonance in the demonstration of naso-sinus disease. The account which follows is an attempt
to summarise present knowledge in this division of otorhinolaryngology imaging.
The format of the chapters has been arranged in two halves: the initial nine chapters are
concerned with investigative techniques and non-neoplastic disease. while the second half of
the book is entirely devoted to benign and malignant tumours. The latter chapters are arranged
according to the nomenclature proposed by my two ex-colleagues. Professor I. Friedmann and
the late Dr D. A. Osborn. in their book The Pathology of Granulomas and Neoplasms of the Nose
and Paranasal Sinuses (Churchill Livingstone. 1982).

Acknowledgements

The author is deeply grateful to his surgical and radiological colleagues for their cooperation
and help in the preparation of the text. In this respect I am particularly indebted to Dr Peter
Phelps. Professor D. F. N. Harrison. Miss V. J. -Lund and Mr John Wright; many of the patients
described presented initially at his Orbital Clinic at Moorfields Eye Hospital. I would also like
to thank Mrs Joanne Evans and her staff of radiographers at the Royal National Throat. Nose
and Ear Hospital and Mr Andrew Gardner of the photographic department for their assistance
with the illustrations.
I am indebted to the Editors of the following journals for permission to reproduce previously
published illustrations: British Journal of Radiology (Figs. 2.1. 2.2. 6.18. 7.5. 7.6. 7.10. 7.11.
7.12. 7.13. 7.14. 7.15. 8.5. 8.6. 8.7. 10.1. 10.2. 10.3 10.4. 10.5. 10.6. 10.7. 10.8. 10.9.
10.10. 10.22. 10.26. 13.10. 13.16. 14.4. 14.6. 15.2. 15.3. 15.14. 15.15. 18.11. 18.12);
Clinical Radiology (Figs. 6.38. 6.40. 6.41, 6.42. 6.43. 6.44. 6.45. 15.12); Clinical Otolaryngology
(Figs. 11.1, 11.2. 11.3. 11.4. 11.5. 11.6. 11.7. 11.8. 11.9. 11.10. 11.11. 11.12. 11.14.
11.15); Journal of Laryngology and Otology (Figs. 5.19.6.26).
I am also grateful to W.B. Saunders and Co. for permission to reproduce Figs. 1.15.4.12.
4.15.5.6.5.12.5.17.6.17.6.37.7.1.10.11.11.19 and 18.3; to H. K. Lewis and Co. for Figs.
3.5 and 5.23; and to Messrs Churchill Livingstone for Figs. 4.5. 4.6. 4.7. 4.8. 4.10. 4.11.
4.18.10.13.10.27 and 10.28.
Schering Health Care Ltd. kindly supplied the gadolinium DTPA for use with the magnetic
resonance studies.

London. March 1988 G.A.S.L.

Contents

1 Basic Radiographic Technique and Normal Anatomy. 1

Basic Radiographic Technique 1
Nasal Bones 6
Normal Anatomy 7
External Nose 7
Nasal Cavity 7
Paranasal Sinuses 7
Developmental Variations 10

2 Special Procedures l3
Conventional Tomography l3
Computerised Tomography l3
Technique . 14
Contrast Medium . 14
Magnetic Resonance Tomography 15
Principle and Method of Examination 15
Pulse Sequences 15
Paramagnetic Contrast Agents and Proton Relaxation 16
Technique and Application 16
Tissue Characteristics . 16
Effects of Gadolinium DTP A 18

3 Congenital Disease 21
Choanal Atresia 21
Congenital Nasal Masses 21
Nasal Dermoids 22
Nasal Glioma 23
Encephalo-meningocoeles 23
Progressive Hemifacial Atrophy (Parry-Romberg Disease) 26

4 Trauma 29
Fractures of the Nasal Bones . 29
Fractures of the Maxillary Antrum 29
Malar Fractures 29
LeFort Injuries . 31
viii Contents

Blow-Out Fractures 32
Fractures of the Frontal Sinus 33
Fractures of the Sphenoid Sinus 34
Fractures of the Ethmoid Cells 36
Cerebrospinal Fluid Leaks 37
Radiological Investigation 38

5 Inflammatory and AUergic Sinus Disease 41

Acute Sinusitis 41
Complications 42
Chronic Sinusitis 44
Allergic Sinusitis 44
Nasal Polyposis 45
CT and Magnetic Resonance . 48
Antro-choanal Polyps 49

6 Cysts and Mucocoeles 51

Cysts of the Paranasal Sinuses 51
Intrinsic Cysts . 51
Cholesteatoma 52
Cysts of Dental Origin .. 52
Miscellaneous Cysts 54
Mucocoeles 55
Frontal Sinus Mucocoeles 56
Ethmoid Mucocoeles . 57
Sphenoid Mucocoeles . 60
Mucocoeles of the Maxillary Antrum 63
Magnetic Resonance and CT . 63
Pneumosinus Dilatans 67

7 Granulomata ofthe Nose and Paranasal Sinuses 73

Mid-facial Granuloma Syndrome 73
Wegener's Granuloma 73
Mid-line Lethal Granuloma 73
Radiological Features . 73
Magnetic Resonance and CT . 74
Sarcoidosis of the Nose and Sinuses. 77
Radiological Features 77
Tuberculosis 77
Syphilis 78
Leprosy 78
Cholesterol Granuloma 78

8 Mycotic Disease 83
Phycomycosis . 83
Mucormycosis . 83
Entomophthorosis 83
Aspergillosis 85
Imaging Features 85
Contents ix

9 Giant Cell Lesions of the Nose and Paranasal Sinuses. 89

Radiological Features . 91
Magnetic Resonance and CT . 91

10 Epithelial Tumours . 95
Inverted Papilloma 95
Malignant Epithelial Tumours 99
Squamous Cell Carcinoma 99
Adenocarcinoma 101
Adenoid Cystic Carcinoma 103
Ameloblastoma 105
Malignant Melanoma . 106
Metastatic Carcinoma 108

11 Tumours of Vascular Origin 111

Juvenile Angiofibroma 111
Haemangiopericytoma 118
Radiological Features 119
Angiosarcoma (Haemangio-endothelioma) 120
Cavernous Haemangioma 121
Venous Malformations 121
Angiolymphoid Hyperplasia with Eosinophilia (Kimura's Disease) 122

12 Lymphoreticular Tumours 125

Lymphoma 125
Radiology and Imaging 125
Plasmacytoma 127
Radiology and Imaging 128

13 Tumours of Neurogenic Origin . 131

Peripheral Nerve Tumours 131
Radiology and Imaging 131
Olfactory Neuroblastoma . 133
Radiology and Imaging 134
Meningioma 136
Primary Meningioma . 136
Secondary Meningioma in the Sinuses 140

14 Tumours of Muscle Origin 143

Skeletal Muscle Tumours 143
Smooth Muscle Tumours 144
Radiology and Imaging 144

15 Fibro-osseous Disease 149

Fibrous Dysplasia . 149
OSSifying Fibroma . 150
Radiology and Imaging 151
x Contents

16 Fibrous Tissue Tumours 159

Fibromatosis 159
Fibrosarcoma 159
Malignant Fibrous Histiocytoma . 160
Radiology and Imaging of Fibrosarcoma and Malignant Fibrous Histiocytoma. 160

17 Cartilaginous Tumours 163

Radiology and Imaging 163

18 Osteogenic Tumours 167

Osteoma 167
Benign Osteoblastoma 168
Osteosarcoma . 168
Radiology and Imaging 170
Ewing's Sarcoma . 172

Subject Index . . 175

1 Basic Radiographic Technique and Normal
Anatomy

Basic Radiographic Technique centred through the antrum (Fig. 1.5). The super-
imposition of the frontal sinuses and also of both
maxillary antra detracts somewhat from the value
Several technical factors are essential for good radi- of this projection. An alternative version of the
ography of the sinuses. These include accurate lateral sinus projection, which better demonstrates
coning of the incident beam, a fine focal spot X-ray the sinus and nasopharyngeal air spaces, is a high
tube, and a Potter Bucky or fine grid to obtain kilovolt lateral film using 150 kV or above and
maximum contrast. The standard projections which 3 mm of brass filtration (Fig. 1.6).
may be employed are: 4. Submentovertical projection. The head is
extended so that the vertex rests against the table
1. Occipito-mental projection. The subject sits top and the incident beam is centred between the
facing the film and the radiographic base line is angles of the jaw so that it is at right angles to
tilted to 45°. The incident beam is horizontal and is the base line (Fig. 1.7). In middle-aged and elderly
centred on the occipital bone 3 cm above the exter- patients the tube has to be tilted upwards con-
nal occipital protuberance (Fig. 1.1). With most siderably as few patients in this age group can attain
subjects a tube tilt is unnecessary since they can the degree of extension necessary to bring the skull
readily extend the head to the required position, but base parallel to the film. This projection dem-
in older persons it may be necessary to tilt the tube onstrates the sphenoid sinuses and also the maxil-
slightly to compensate for any restricted extension lary antra and orbital walls (Fig. 1.8).
of the head. This view shows the maxillary antra
free of any overlap of the petrous bones, and if the 5. Oblique projection. Rotation of the sagittal
mouth is kept open during the examination the plane of the skull through an angle of 39° will
sphenoid sinuses and nasopharynx can be seen enable the posterior ethmoid cells to be projected
through the open mouth (Fig. 1.2). through the orbit and will show these cells largely
clear of overlap shadows. The optic foramen is seen
2. Occipito-frontal projection. The subject sits end-on in this projection.
facing the film with the orbito-meatal line raised
20°, the incident beam horizontal and the tube Examination of the sinuses should always be
centred to the nasion (Fig. 1.3). This projection made in the erect position with a horizontal X-ray
demonstrates the fine detail of the frontal sinuses; beam to allow the demonstration of fluid levels
the lateral walls of the antra are also seen, although within the sinus. Fluid in the sinus may be pus or
the overlapping petrous temporal bones largely mucopus occurring as a sequel of infection or
obscure the antra (Fig. 1.4). allergy, but on occasion may be frank blood as a
3. Lateral projection. The subject sits with the result of trauma. These different types of fluid cast
radiographic base line horizontal and the sagittal shadows of identical density on the radiograph and
plane parallel to the film. The incident beam is the nature of the fluid cannot be recognised.
2 Basic Radiographic Technique and Nonnal Anatomy

Fig. 1.1. Position for occipito-mental projection.

Fig. 1.2. Nonnal occipito-mental projection. The maxillary antra are shown free of any overlap of the petrous bones. The sphenoid
sinuses are visible through the open mouth.
Basic Radiographic Technique 3

Fig. 1.3. Position for oCcipito-frontal projection.

Fig. 1.4. Normal occipito-frontal view ofthe sinuses. This view shows both vertical and horizontal parts (arrows) of the frontal sinus.
the ethmoid cells and orbits.
4 Basic Radiographic Technique and Nonnal Anatomy

Fig. 1.5. Position for the lateral projection ofthe sinuses.

Fig. 1.6. Lateral high kilovolt view of the paranasal sinuses. The air spaces in the nose, sinuses and nasopharynx are better
demonstrated by partial elimination of the bone structures.
Basic Radiographic Technique 5

Fig. 1.7. Position for submentovertical projection.

Fig. 1.8. Submentovertical view of the paranasal sinuses.

6 Basic Radiographic Technique and Nonnal Anatomy

Nasal Bones
The nasal bones are shown in the occipito-mental
view but bone detail is poor, and although fractures
and displacements may be visible two further views
are needed for their proper demonstration:

Lateral Projection
The examination is performed using non-screen film
and with the patient seated. With the tube hori-
zontal. the central ray is directed through the base
of the nose to the centre of the film (Fig. 1.9).

Supero-inferior or Occlusal View

With the patient sitting, an occlusal film is placed
lengthways between the teeth so that two thirds of
the film projects forward. The central ray is directed
along the vertical plane of the forehead and the tube
centred in the mid-line through the nose (Fig. 1.10).

Fig. 1.10. Position for axial or supero-inferior occlusal view of

the nasal bones.

Fig. 1.9. Lateral projection for the nasal bones.

Normal Anatomy 7

Normal Anatomy it the ostium of the maxillary antrum. The inferior

meatus is bounded above and medially by the
External Nose inferior turbinate and laterally by the lateral wall
of the nasal cavity. The naso-Iacrimal duct drains
The external nose is a pyramidal-shaped structure into it.
with its base directed inferiorly and supported by
the facial bones. It consists of cartilage covered
by skin and subcutaneous tissue and is lined by Paranasal Sinuses
mucoperiosteum. The nasal cartilages which con-
tribute to the structure of the nose consist of: (1) The paranasal sinuses consist of frontal sinuses.
paired lateral cartilages superiorly; (2) two greater sphenoid sinuses. maxillary antra and the ethmoid
alar cartilages forming the tip of the nose and its complex of air cells. The sinuses lie within the facial
lateral aspect; and (3) numerous lesser alar car- bones and are grouped around the nasal cavities.
tilages forming the lateral wall. These are supported which contain the superior. middle and inferior
by a mid-line triangular plate of thin cartilage - the turbinate bones on their lateral wall.
mobile nasal septum - which is attached anteriorly
along its inferior surface to the nasal anterior maxil-
lary spine and to the perpendicular process of the Development
ethmoid and vomer posteriorly. This cartilaginous
plate is continuous posteriorly with the bony nasal The sinuses are rudimentary at birth and the fron-
septum. The nasal cartilages are attached superiorly tals not formed. They develop from buds of res-
to the paired nasal bones and supported on either piratory epithelium and grow rapidly from about
side by the facial process of the maxilla. the age of 7 until after puberty. The maxillary
The cartilaginous structure of the external nose is antrum is the first to develop and by the age of 2
readily examined clinically. and radiology or other years the outer wall of the antrum has extended
imaging methods are generally not required except laterally as far as the infraorbital foramen; by 8-10
to demonstrate changes in the nasal bones and years aeration is complete.
anterior nasal spine. The frontal sinuses can seldom be demonstrated
radiographically until they have extended into the
vertical portion of the frontal bone. a process which
Nasal Cavity varies conSiderably in its timing but which seldom
occurs to any degree before the age of 2 years. By
Superiorly the nasal cavity has a narrow roof this time they generally measure less than 1 cm in
formed by the cribriform plate of the ethmoid bone. height. reaching adult size by puberty. Pneu-
Its lateral walls. which slope outwards and down- matisation of the sinus within the frontal bone pro-
wards. consist of the orbital plates of the ethmoid ceeds in a postero-anterior direction; unilateral
and palatine bones and the lacrimal bone. whilst its failure. or partial failure. of this process may lead
anterior margin is formed by the nasal process of to a thickening of the anterior wall of the adult
the maxilla. The floor of the nasal cavity is formed sinus producing a relative loss of translucence on
by the palatal process of the maxilla. Posteriorly the the affected side on plain sinus radiographs.
nasal cavity opens into the nasopharynx by two The sphenoid sinuses are absent at birth and do
oval openings - the posterior nares. Subdividing the not extend below the planum sphenoidale until the
nasal cavity is the bony nasal septum; this is formed age of 2-3 years. Pneumatisation inferior to the
by the vertical plate of the ethmoid in its upper two pituitary fossa can be seen by 4 years and is present
thirds. whilst the lower third is formed by the vomer. in the majority at 8-10 years.
Projecting from the lateral walls of the nose are
the three paired turbinate bones running in a
roughly horizontal direction. and dividing the nasal Frontal Sinuses
cavity into three sulci on each side: the superior.
middle and inferior meatus. The superior meatus Thefully developed frontal sinuses are two air-filled
lies above the middle turbinate bone. and the pos- irregular cavities lying between the inner and outer
terior ethmoid and sphenoid cells drain into it. The tables of the frontal bone. They consist of a central
middle meatus lies between the middle and inferior cavity forming the main body of the sinus and two
turbinates. On its lateral wall there is a curved extensions: an extension upwards into the vertical
depression - the hiatus semilunaris - into which part of the frontal bone; and a horizontal compon-
drains the fronto-nasal duct anteriorly and behind ent. which extends backwards into the orbital plate
8 Basic Radiographic Technique and Normal Anatomy

of the frontal bone and pneumatises the orbital roof. and pre-molar teeth. Beneath the cheek the antero-
The two frontal sinuses are separated by a bony or lateral wall is formed from the facial aspect of the
fibrous septum. which is seldom exactly mid-line in maxilla. and behind. the postero-lateral wall sep-
position; they communicate with the middle meatus arates the antrum from the pterygo-palatine fossa
of the nose by the fronto-nasal duct. This varies and the infratemporal fossa. The lateral wall is
greatly in diameter. length and direction and is grooved by branches of the superior dental vessels
often very tortuous. being encroached upon by the and this may show as a small dehiscence on the
fronto-ethmoidal air cells. Sometimes the ducts radiograph.
open directly into the ethmoid cells instead of into Although they are the most symmetrical of the
the nose. paranasal sinuses some variation in the size of the
The frontal sinuses are almost never symmetrical: antra may occur in the normal subject. The com-
one or both components of the sinus cavity may be monest defect is a failure of pneumatisation of the
congenitally absent or underdeveloped. Incomplete alveolar recess. but not infrequently the whole air
pneumatisation of the orbital roof is the commonest cavity is under-pneumatised (Fig. 1.11). The result-
variant. Sometimes the frontal sinuses are totally ant increase in the thickness of the antral walls
absent - a feature usually associated with a per- will produce a relative loss of translucence on the
sistent metopic suture. radiograph on the side affected. and may lead to
misinterpretation of the change as sinus infection
or mucosal thickening.
Sphenoid Sinuses Sub-division of the antral cavities can occur by
the formation of septa (Fig. 1.12). which may be
The sphenoid sinuses are paired cavities occupying bony or membranous. partial or complete. Not
the body of the sphenoid bone. They are separated infrequently a posterior ethmoid cell grows down-
by a thin bony septum and each drains into the wards into the body of the maxilla and may
superior meatus of the same side with the posterior encroach on the sinus. usually occupying the
ethmoid cells. In the average subject pneum- supero-medial part of the antral cavity. These
atisation extends posteriorly for approximately aberrant cells may become infected without
two thirds of the sphenoid bone. but the degree of involvement of the antrum. or they may expand
pneumatisation varies widely. On occasion com- into the antral cavity as part of a spheno-ethmoidal
plete failure of pneumatisation may occur. or con- mucocoele.
versely the whole bone is completely pneumatised.
the posterior clinoids being involved in the process.
Sometimes the pneumatisation may involve the Ethmoid Labyrinth
pterygoid processes. the greater wings of the sphe-
noid and exceptionally the basilar process of the The ethmoids are a complex of small air cells lying
sphenoid bone. The sphenoid sinuses may also in the lateral wall of the nasal cavity between its
encroach upon the antral cavity and extend into upper part and the medial wall of the orbit and are
the ethmoidal field. separated from the anterior cranial fossa by the
orbital plate or roof of the ethmoid bone. The medial
wall is formed by a thin plate of bone to which are
Maxillary Antra attached the superior and middle turbinates. and
into which the air cells may sometimes extend (Fig.
The adult maxillary antra are a pair of air-filled 1.13). The lateral wall of the ethmoid labyrinth
cavities lying on either side in the body of the consists of a thin lamina papyracea which separates
maxilla. Viewed in the postero-anterior plane they the air cells from the orbit. Inferiorly the ethmoid
are pyramidal in shape with their apices directed cells articulate with the maxilla. The cells forming
downwards. The medial boundary of the antrum is the ethmoid labyrinth are divided into anterior.
formed from the lateral wall ofthe nasal cavity. and middle and posterior groups. The posterior group of
through it the antral ostium opens into the middle cells drain into the superior meatus in company
meatus. Anteriorly in the medial wall the naso- with the sphenoid sinuses. The middle and anterior
lacrimal duct runs in a bony canal into the inferior groups drain into the middle meatus; the anterior
meatus. The roof of the antrum is the bony floor of group into the infundibulum or the hiatus semi-
the orbit. across which is a bony ridge enclosing the lunaris; and the middle group posteriorly in the
infraorbital nerve and vessels. Inferiorly the floor of region of the bulla ethmoidalis.
the antrum is formed from that portion of the The ethmoid cells may migrate during their
alveolar process of the maxilla containing the molar development beyond the confines of the normal
Normal Anatomy 9

Fig. 1.11. Occipito-mental view of the maxillary antra showing underdevelopment of the sinus on the right side. On the left there is
a bony groove on the lateral wall of the antrum caused by the superior dental vessels and nerves (arrow).

Fig. 1.12. Septate antrum. Occipito-mental view showing a bony septum traversing the antral cavity.
10 Basic Radiographic Technique and Nonna! Anatomy

a b
Fig. 1.13a, b. Pneumatisation of the middle turbinate shown on a hypOcycloidal tomography and b computed tomographic scan in
coronal sections.

ethmoid labyrinth. These are known as agger cells described a triad of abnormalities consisting of
and may occur in the sphenoid bone, the frontal chronic rhinosinusitis, bronchiectasis and situs
bone and the nasal bones, and may pneumatise the inversus viscerum. This has recently been dem-
ethmoid conchae. onstrated to be the result of a genetic defect manifest
The normal anatomy of the sinuses as dem- in structural and functional abnormalities of the
onstrated by computerised axial tomography is cilia (Imbrie 1981).
shown by the series of scans in Fig. 1.14. The ethmoid cells show increased width and over-
pneumatisation in hypertelorism from whatever
cause, congenital or acquired: for example in the
Developmental Variations naso-encephalocoeles, or in untreated nasal
polyposis (Lund and Lloyd 1983). Hypoplasia of
An increased pneumatisation of the frontal sinuses the ethmoids is seen in hypotelorism, which is a
associated with overdevelopment of the supraor- narrowed space between the orbits and occurs in
bital ridge is usually a prominent feature of acro- several congenital syndromes affecting the facial
megaly, and cerebral atrophy or agenesis of a btilnes, notably the holoprosencephalies (Becker and
cerebral hemisphere may also result in over- McCarthy 1986).
pneumatisation of the frontal sinus. Congenital
absence of the frontal sinus is said to occur in 5%
of the normal population and is often associated
with persistence of the metopic suture. Under- Fig. 1.14. 1, Frontal sinuses: anterior' and posterior walls; 2,
development may also be seen in premature fusion crista galli; 3, anterior ethmoid cells; 4. lamina papyracea of the ~
of the cranial sutures (craniostenosis) and absence ethmoids; S, sphenoid sinuses; 6, posterior ethmoid cells; 7,
of the frontal sinuses occurs in the majority of sphenoid intersinus septum; 8. nasal septum; 9, summit of the
antrum; 10, pterygo-palatine fossa; 11, pneumatised pterygoid
patients with Down's syndrome. Agenesis or under- plates; 12, naso-lacrimal duct; 13, maxillary antrum; 14, infra-
development of the frontal sinuses is also a feature temporal fossa and fat pad; IS, pterygoid laminae; 16. lateral
of Kartagener's syndrome. Kartagener (1933) pterygoid muscle.
Normal Anatomy 11
12 Basic Radiographic Technique and Normal Anatomy

Fig. 1.15. Bilateral anophthalmos. The sinuses have overgrown to occupy the space left by the absent eyeballs.

The maxillary antra seldom show any appreci- References

able over-pneumatisation. but relative over-
development may occur in microcephaly (Samuel Becker MH, McCarthy JG (1986) Congenital abnormalities. Diag-
and Lloyd 1978) and may also be seen in congenital nostic imaging in ophthalmology. Springer, Berlin Heidelberg
anophthalmos in which the ethmoid cells and to a New York
lesser extent the maxillary antra overgrow. to Imbrie JO (1981) Kartagener's syndrome: a genetic defect affect-
ing the function of cilia. Am J Otolaryngol 2:215-222
occupy the space left by the absent eyeball (Fig. Kartagener M (1933) Zur Pathogenese der Bronkiektasien, Bron-
1.15). Total absence of pneumatis ation of the antra Idektasien bei situs viscerum inversus. Beitr Klin Tuberk
may be seen in sickle cell anaemia and in thal- 83:489-495
assaemia due to bone marrow hyperplasia. Lloyd GAS (1985) Orbital pneumosinus dilatans. Clin Radiol
36:381-386
Local over-pneumatisation of the paranasal Lund VJ, Lloyd GAS (1983) Radiological changes associated with
sinuses (pneumosinus dilatans) may occur in the benign nasal polyps. J Laryngol Otol 97:503-510
presence of fibro-osseous disease or as the response Samuel E, Lloyd GAS (1978) Clinical radiology of the ear, nose
to a meningioma (Lloyd 1985) (see Chap. 6). and throat. UK Lewis, London
2 Special Procedures

The initial radiological investigation of the para- Ideally conventional tomography should be per-
nasal sinuses is by the techniques described in formed using a machine capable of complex motion
Chap. 1. If the appearances on plain radiography tomography rather than a simple linear movement.
are consistent with allergic or inflammatory disease A full series of paranasal sinus tomograms consists
and there is good correlation of the clinical findings, of:
no further investigation is usually necessary. If,
however, the evidence from plain radiography indi- 1. Coronal projections using an undertilted occi-
cates an expanding or destructive lesion of the sinus pito-mental position of the skull angled 3 0'-35'
walls or a tumour mass, and the clinical findings cranially, or a projection corresponding to the
suggest a less benign process (for example pain and occipito-frontal view described in Chap. I, with
paraesthesia, epistaxis, facial swelling, nasal mass the forehead placed in contact with the table top
or orbital involvement), tomographic investigation and the orbito-meatalline at right angles to it.
is indicated. There are now three tomographic tech-
2. Lateral tomograms.
niques available.
3. Axial tomography. For these films a specially
designed wooden platform is placed on the table
top so that the head may be hyperextended into
the submentovertical position (Lloyd 1975).
Conventional Tomography

Conventional tomography has been almost com-

pletely superseded by high-resolution computerised
tomography (CT) and now plays only a minor role Computerised Tomography
in the investigation of paranasal sinus disease. It
has been reduced to functioning as a simple adjunct
to plain radiography at initial examination. For CT scanning has provided an important addition
example it is useful in showing whether there is a to the radiographic investigation of the paranasal
nasal mass present in association with an opaque sinuses, and has virtually replaced conventional
sinus, particularly if the maxillary antrum is tomography as a means of assessing tumours,
involved. Small nasal masses often go undetected mucocoeles and other expanding lesions in the
on plain radiographs, and patients who are found sinuses. CT has the advantage of shOWing both bone
to have unilateral total opacity ofthe antrum should destruction and the soft tissue extent of disease. In
have immediate tomography as a screening pro- malignant disease it provides an accurate method
cedure. of staging a tumour prior to radiotherapy or surgery
14 Special Procedures

and is important post-operatively to show recur- generally the window settings should be within the
rence of tumour. In addition it has extended the range of 200-3000 Hounsfield units.
possibilities of differential diagnosis in the sinuses.
not only by showing the soft tissue pattern of sinus
disease. but by a more sensitive demonstration of Contrast Medium
calcification within a tumour. which in some
instances may be characteristic. Occasionally CT The CT attenuation values of both normal and
may allow the radiologist to make the primary diag- abnormal tissues generally show an increase after
nosis of sinus malignancy prior to the clinician. and the administration of intravenous contrast medium.
to indicate the best area for confirmatory biopsy. In sinus neoplasia the degree of enhancement varies
with tumours of different histology and there is also
a considerable variation within the same his-
Technique tological type. Enhancement usually correlates
closely with the vascularity of the tissue concerned.
Routine axial and coronal sections are obtained on so that strong enhancement is to be expected for
all patients. Direct coronal scanning is necessary inflammatory tissue while retained secretion and
for adequate demonstration of sinus disease. Refor- uninfected mucocoeles should not enhance. By.
matted views should be reserved for sagittal utilising any differential contrast enhancement a
sections. which are not directly obtainable with distinction can sometimes be made between tumour
most scanner designs. They should only be used to and adjacent normal or inflammatory tissue.
provide coronal scans when direct scanning is for In practice these differences are often unclear.
any reason impossible - for example if a patient largely because of the wide range of tumour
cannot extend the head or cervical spine. enhancement encountered. and this method of

Axial Scans
Axial scans should be orientated in the same plane
as those used for CT of the orbit (Lloyd 1979). The
position of the patient's head is adjusted so that the
scanning plane forms an angle of 16° caudally from
the orbito-meatal line: in this way the plane of
section will conform to the length of the optic nerve.
and will also provide axial views of the optic canals
and the adjacent posterior ethmoid cells and sphe-
noid sinuses (Fig. 2.1).

Coronal Scans
Coronal scans are performed by hyperextension of
the patient's head and angulation ofthe gantry with
the patient either prone or in the supine position. In
some patients it is impossible to obtain true coronal
scans. either because the patient cannot achieve
sufficient extension of the neck. or because the
angulation may need to be adjusted out of the
coronal plane to avoid the effect of metallic dental
fillings. These will degrade the image unless suitable
computer software modification is available to over-
come the problem.
For imaging of the sinuses 5-mm sections in both
planes are generally adequate. with contiguous
slices through the lesion. Imaging should include Fig. 2.1. Axial CT scan showing the optic canals in plan view
both wide window settings for bone detail and nar- (arrows) and their close relationship to the posterior ethmoid cells
rower window widths for good soft tissue contrast: and sphenoid sinus.
Computerised Tomography 15

assessing tumour extent and recurrence has now the static magnetic field is exponential and is
largely been replaced by the use of magnetic res- described by the time constant Tl ' The return to
onance tomography using paramagnetic contrast equilibrium for the transverse component is also
medium (see below). In these circumstances little or exponential and is described by the time constant
no added information is provided by giving contrast T2 •
medium prior to CT of the nose and sinuses when The intensity of the signal is related to the proton
magnetic resonance tomography is available, Intra- density, which refers to the distribution of reson-
venous contrast should be reserved for the following ating hydrogen nuclei within the patient. It must
categories of patients: be emphasised, however, that not all protons give
1. Patients with vascular tumours such as angio- a magnetic resonance signal. The protons in large
fibroma. In these a bolus injection or drip infusion molecules such as proteins do not as a rule con-
should be employed, scanning taking place during tribute to the signal, nor is there Signal from solid
the actual administration of the contrast to catch structures such as bone. The distribution of the
the vascular phase of tumour enhancement. resonating protons is fairly uniform in the soft
tissues and differences in density therefore slight.
2. Patients with suspected tumour spread into Contrast between areas of differing proton density
the anterior or middle cranial fossae, i.e. when the can be enhanced if the scan is biased towards T1 or
blood-brain barrier is involved. The tumour is then T2 relaxation characteristics. In practice, therefore,
outlined against the non-enhancing brain tissue. the resultant image is affected by the proton density
3. Patients with sinus infection in whom abscess and by one or other, or both, of these components.
formation is suspected either in the anterior fossa
or in the orbit, when there is an associated orbital
cellulitis. This also applies to pyocoeles, which may Pulse Sequences
show a typical ring enhancement after contrast.
Three pulse sequences are commonly used in mag-
netic resonance tomography:

Magnetic Resonance Tomography Saturation Recovery. This is the simplest pulse

sequence. Ninety-degree radio-frequency pulses are
Principle and Method of Examination repeatedly applied to the patient and the nuclear
magnetic resonance signal is measured after each
Atomic nuclei with an odd number of protons or pulse. Provided the repetition time is short in
neutrons possess a magnetic moment and behave relation to the T1 relaxation time of the tissue con-
like spinning magnets. Hydrogen has such a cerned, a proton density image with some T1
nucleus and because of its wide distribution in the weighting will be produced.
body is the element most commonly used for mag-
netic resonance tomography. When placed in a Inversion Recovery. This sequence produces images
static magnetic field the hydrogen nuclei align their which contain a greater amount of T1 information
magnetic axes parallel to the field, the sum of these than is provided by saturation recovery, although
nuclear magnets giving rise to a weak mag- the principle is very similar. The difference in inver-
netisation through the patient in the direction of sion recovery is that the nuclei are caused to res-
the field, because more of these nuclear magnets onate by a 180° pulse prior to the 90· pulse, so that
align with the field than against it. the nuclei are not at equilibrium when the 90° pulse
Application of an oscillating radio-frequency is applied. The resulting image contains approxi-
pulse of a specific frequency introduces energy into mately twice the amount of T1 information com-
the patient and can cause the magnetisation to pared with the saturation recovery mode and is
rotate into the transverse plane when a 90° pulse therefore to be preferred when T1 spin charac-
is used. Alternatively the magnetisation vector can teristics are to be evaluated.
be completely reversed in direction by a 180° pulse.
When the pulse is discontinued the nuclei tend to Spin Echo. The spin echo pulse sequence produces
return to their original orientation in the static a signal to which both T1 and T2 contribute.
magnetic field, while emitting the absorbed energy. Although it contains information about T1 and T2
When at 90° to the field, the transverse nuclear it is principally to obtain the latter that it is used in
magnetisation will cut the receiver coils and induce clinical practice. The pulse sequence is the reverse
a resonant signal voltage in the coil. The return to of inversion recovery in that a 90· pulse is followed
equilibrium for the component in the direction of by a 180· pulse. The time between the initial 90°
16 Special Procedures

pulse and the signal is known as the time to echo; Coupled with the use of a head coil and three-plane
the larger this value the greater the T2 contribution imaging it provides total coverage of the head and
to the signal. neck and allows identification of associated disease
away from the primary site in the paranasal sinuses:
for example neck malignancy (Fig. 14.6. p. 146).
Paramagnetic Contrast Agents and Proton This is of great importance to the oncologist in
Relaxation treatment planning and represents a major advance
over CT scanning.
It is the water content of the tissues which provides
the signal for magnetic resonance tomography and Slice Thickness. Thin slices are advantageous in
it is the behaviour of this water which affects the magnetic resonance tomography when trying to
relaxation times TI and T2 • A proportion of the visualise small areas. Definition is improved because
water in the tissues is bound to the surface of the amount of overlap between structures lying
proteins. which has the effect of lowering the T1 obliquely through the slice is reduced and their
value. Unbound (free) water has a much higher TI edges become more distinct. However. thin sections
value than the bound water and the T1 value of any suffer from an important disadvantage: the thinner
given tissue will depend upon the ratio of bound to the slice the greater the amount of "noise" on the
free water: the higher the proportion of free water. scan. increasing the signal to "noise" ratio and
the higher the T1 values and vice versa. It is thought degrading the image. The effect on the image is less
that the increase in T1 in tumour tissue compared obvious at high static field strengths because the
with normal tissue is dependent upon the release of machines with the more powerful magnets possess
free water resulting in a change in the ratio of free a higher inherent spatial resolution than low field
to bound water. strength systems.
The T1 and T2 relaxation times can also be affected
by the presence of paramagnetic substances. Para- Choice of Pulse Sequences. For optimum tissue
magnetic ions have magnetic moments that are of characterisation TI- and Trweighted sequences are
the order of 1000 times as large as that of protons required. Spin echo sequences using a long time to
(Carr and Gadian 1985). These produce large local echo will give maximum T2 weighting and signal
fields and can enhance the relaxation rates of water differentiation between tissues. depending upon the
protons in the immediate vicinity of the ions. It is relaxation times of the tissues concerned. T1 weight-
found that the increase in the relaxation rate is ing can be achieved using the saturation recovery
directly proportional to the concentration of the technique (see above) or a spin echo sequence with
paramagnetic agent and to the square of its mag- a short repetition time. producing images of good
netic moment. anatomical detail and containing some T2 infor-
The first paramagnetic agent to be introduced mation. Greater TI weighting is achieved. however.
into clinical practice as a magnetic resonance con- by using inversion recovery sequences. and these
trast medium is the substance gadolinium DTPA. are to be preferred when available. Multi-slice inver-
Gadolinium is a very effective paramagnetic agent sion recovery sequences are not always obtainable
but as a free ion it is toxic to liver. spleen and bone on 1. 5-tesla scanners. which makes these machines
marrow. However. when chelated to diethy- somewhat less versatile than models with medium
lenetriamine penta-acetic acid (DTP A) its toxicity is strength static fields. Inversion recovery sequences
reduced. permitting it to be used as a safe relaxation are especially important when a paramagnetic con-
enhancing agent. In the soft tissues it is distributed trast agent is employed (see below).
mainly in the extracellular space. and to date no
short-term toxicity has been detected (Carr and
Gadian 1985). Tissue Characteristics
In general malignant tumours of sinus ongm.
Technique and Application whether epithelial or mesenchymal. produce signal
of medium intensity on TI-weighted spin echo
Multi-slice Facility. An advantage of magnetic res- sequences and a medium to strong signal on T2
onance tomography over other methods is the images. In contrast. retained secretion produces
multi-slice facility which is standard on most high signal on spin echo sequences. Differentiation
current machines. This allows multiple sections to between tumour and retained secretion is par-
be obtained simultaneously using either 1-cm or ticularly striking on Trweighted images. the
O.5-cm contiguous slices to a depth of up to 12 cm. retained secretion always giving a higher signal
Magnetic Resonance Tomography 17

than tumour. An additional feature is the hetero- allowing discrimination between tumour and
geneous signal of tumour in comparison with normal mucosa. On the other hand, when TI-
that shown by retained secretion, which is weighted sequences are used the signal intensities
invariably homogeneous. The vascularity of the are very similar and tumour differentiation is
tumour is a major contributor to the lack of homo- difficult. Inflamed or oedematous sinus mucosa also
geneous signal seen in juvenile angiofibroma. In produces a signal of high intensity on Tz-weighted
these benign tumours large vessels ca,n be identified, spin echo sequences, and may be distinguished from
both in the tumour itself and in the adjacent muscu- tumour by this means. This distinction is especially
lature. They are shown as areas of negative signal or apparent on heavily weighted Tz-weighted
signal void and when present are totally diagnostic sequences, using a long time to echo and a long
(Lloyd and Phelps 1986; see Chap. 11). repetition time (Fig. 18.12, p. 172). This is a valu-
Magnetic resonance scanning can show simple able feature of the technique, both for post-operative
inflammatory or allergic changes in the sinuses. assessment of possible tumour recurrence and for
It is possible to show single or multiple polyps, monitoring the effects of radiotherapy or chemo-
thickened mucosa, or fluid levels in the presence therapy.
of infection. These conditions are generally well Nasal polyps are oedematous, prolapsed mucosa
demonstrated by conventional radiography and the of the ethmoidal cells, with a histological picture of
need to recognise them is simply to be able to dis- respiratory epithelium covering a grossly oedema-
tinguish them from more serious disease. One of the tous stroma. The differentiation of retained
advantages of magnetic resonance scanning is the secretion in the sinuses and nasal polyps is less
strong signal which is received on Tz-weighted spin easily made by magnetic resonance than the dis-
echo images from retained mucus or mucopus in tinction between retained secretion and a true neo-
the sinuses. This enables an important distinction plasm, either benign or malignant. The close signal
to be made between tumour in the sinuses and characteristics of an oedematous polyp and its
secondary mucocoele formation, even within the associated mucosal secretion make the differ-
same sinus cavity (Fig. 2.2). In the same way, entiation difficult, particularly on TI-weighted
primary mucocoeles or pyocoeles are optimally images, but differentiation may be possible on Tz-
demonstrated by this technique (see Fig. 6.20, weighted pulse sequences.
p. 59). Because of its highly vascular nature, nasal The spin characteristics of mucocoeles vary with
mucosa gives a signal similar to inflamed or their surgical history: that is, whether or not the
oedematous sinus epithelium: that is, a high-inten- patient has received any previous surgical treat-
sity signal on T2-weighted spin echo sequences, ment for the condition. This is important because
the presence of haematoma or altered blood affects
the spin characteristics. This is thought to be due
to the breakdown of red cells, with the release of
methaemoglobin - a paramagnetic agent (Gomori
et al. 1985). An untouched mucocoele will present
a high signal on Tz-weighted spin echo sequences
and a very low signal on inversion recovery due to
a long TI relaxation time (Figs. 6.20 and 6.21,
pp.59 and 60). In contradistinction, a mucocoele
in which there has been previous surgical drainage
may produce high Signal on inversion recovery (Fig.
6.21). This effect is presumed to be due to the release
of methaemoglobin from haematoma and short-
ening of the TI relaxation time.
In summary, it can be said that mucocoeles,
pyocoeles, retained secretion and inflamed mucosa
give a stronger signal on Tz-weighted spin echo
sequences than do tumours. In addition, examples
of dense fibrous tissue in association with para-
nasal sinus disease have shown entirely different
Fig. 2.2. Coronal magnetic resonance scan (T,-weighted magnetic resonance features, characterised by a
sequence) showing a slightly expanded sphenoid sinus con-
taining tumour in the lower part and retained secretion in the low Signal on T2-weighted spin echo sequences.
upper. The tumour was an angiofibroma. Areas of signal void All these tissue characteristics are represented
are visible (arrows) due to large vessels in the tumour mass. graphically in Fig. 2.3.
18 Special Procedures

_ - - - - Retained Secretion
Inflamed Mucosa

Malignant Tumours

Dense Fibrous Tissue

IR T1-----S.E.------T2
Fig. 2.3. Average signal intensities plotted against inversion recovery (IR) and T)- and T2-weighted spin echo (SE) magnetic resonance
sequences. Tissue differentiation is possible between retained secretion in the sinuses, inflamed mucosa, malignant tumours and
fibrous tissue.

Effects of Gadolinium DTPA sinuses in which there is both inflammatory

mucosal thickening and fluid. The high-intensity
Intravenous injection of the paramagnetic contrast enhancement of the nasal and sinus mucosa is
agent gadolinium DTPA has effects on the spin clearly outlined against the non-enhancing fluid
characteristics of both naso-sinus tumours and (Fig. 13.12, p. 137).
normal and inflammatory tissue in the nose and Gadolinium DTPA pools in the capillary bed of the
sinuses. As described above the majority of tumours highly vascular nasal mucosa, and this produces a
show high or moderately high signal on Tr high-intensity signal on magnetic resonance scans.
weighted spin echo sequences. Without the use of Initially this led to an overestimate of the size of
contrast, tumours are best shown by this technique tumour in a patient with an olfactory neuro-
and best differentiated from retained secretion or blastoma, and might be considered a drawback of
inflammatory mucosa (Lloyd et al. 1987). After using paramagnetic enhancement. In practice this
intravenous gadolinium DTPA tumour enhance- is not so: non-enhancing tumour may be outlined
ment on the T1-weighted spin echo and inversion against high signal from the nasal mucosa and from
recovery sequences varies from high intensity to inflammatory mucosal thickening in the sinuses
zero, the degree of enhancement probably depend- (Fig. 2.4); and a totally opaque sinus may be
ing upon the vascularity of the individual tumour. revealed as containing thickened mucosa and fluid
Post-contrast inversion recovery series have been rather than tumour. Alternatively all three may be
shown to be the best method of tumour dem- demonstrated in the same sinus cavity (Fig. 2.5);
onstration in over 80% of cases and were superior this is clearly important in the selection of the best
to pre-contrast Trweighted spin echo sequences in site for tumour biopsy.
differentiating tumour both from retained secretion In the nose and sinuses tumour tissue can be
and from inflammatory mucosal thickening in the recognised by its heterogeneous signal; thickened
sinuses. Retained secretion does not enhance after mucosa, polyps and retained secretion always
contrast: this helps to make the differentiation present a homogeneous signal with or without con
between tumour and fluid in the sinuses more trast enhancement. Tumours usually have some
obvious than it is on the unenhanced Trweighted degree of signal heterogeneity and this is increased
spin echo scans (Fig. 13.ll, p. 137). This applies after intravenous gadolinium, especially on '
equally to secondary mucocoele formation and to inversion recovery sequences, which are mf
 Magnetic Resonance Tomography 19

~
Fig. 2.4. a T,-weighted spin echo sequence showing a lymphoma
invading the floor of the orbit from the maxillary antrum.
b Inversion recovery sequence after intravenous gadolinium
DTP A. The non-enhancing lymphoma is outlined against the
enhanced mucosa in the antrum and the orbital fat (arrows).

Fig. 2.5a.b. Same patient as Fig. 2.4. T,-weighted coronal mag-

netic resonance scans before and after intravenous gadolinium
DTP A. a The pre-contrast scan shows no discrimination between
tumour, thickened mucosa, or fluid in the antrum. b The post-
contrast scan shows the non-enhanced tumour (arrow) outlined
against the enhanced mucosa in the left antrum. which contains
b fluid. The right antrum also contains fluid and thickened mucosa.
20 Special Procedures

affected by the contrast agent (Curati et al. 1986). References

Differential signal enhancement from tumour is a
feature of the post-contrast scans and probably
results from areas of varying vascularity in the Carr OM, Gadian DG (1985) Contrast agents in magnetic res-
tumour. This was particularly marked in a chon- onance. Clin Radiol 36:561-568
drosarcoma. where the central chondromatous part Curati WL, GraifM. Kingsley OPE. NiendorfHP, Young IR (1986)
Acoustic neuromas: Gd-DTPA enhancement in MR imaging.
of the tumour failed to enhance after intravenous Radiology 158:447-451
gadolinium while the more peripheral cellular Gomori 1M. Grossman RI. Goldberg m. Zimmerman RA, Bilaniuk
element of the tumour showed fairly intense LT (1985) Intracranial haematomas: imaging by high field
enhancement (Fig. 17.7. p.166). MR. Radiology 157:87-93
Lloyd GAS (1975) Radiology of the orbit. WB Saunders. London
Lloyd GAS (1979) CT scanning in the diagnosis of orbital disease.
Comput Tomogr 3:227-239
Lloyd GAS, Phelps PO (1986) Juvenile angiofibroma: imaging by
magnetic resonance. CT and conventional techniques. Clin.
Otolaryngol 11:247-259
Lloyd GAS, Lund VJ, Phelps PO, Howard OJ (1987) Magnetic
resonance imaging in the evaluation of nose and paranasal
sinus disease. Sr J RadioI60:957-968
3 Congenital Disease

Choanal Atresia posterior nares, and may be confirmed radio-

logically. The bony septum can be demonstrated
by conventional or computerised tomography (CT).
Posterior choanal atresia is thought to result from Positive confirmation is made by instilling contrast
a failure of rupture, between the 35th and 38th day medium into the nasal cavities and taking radio-
of foetal life, of the partition which separates the graphs with a horizontal beam so that the contrast
bucco-nasal and bucco-pharyngeal membranes can be seen to pool posteriorly (Fig. 3.1). Alter-
(Williams 1971). The choanal atresia which results natively, a submentovertical projection can be used.
may be unilateral or bilateral, bony or membra- The above techniques clearly demonstrate the
nous, complete or incomplete. The bony atresia is presence of choanal atresia, but do not provide
commonly located 1-2 mm anterior to the posterior information concerning the nature of the stenosis:
margin of the hard palate; the membranous form whether it is bony or membranous. This, however,
usually occurs more posteriorly. In approximately can be established by CT scan. Hasegawa et al.
one third of patients the atresia is bilateral, and (1983) investigated a 6-year-old female with bilat-
some patients have associated deformities including eral choanal atresia and were able to demonstrate
a high arched palate and increased thickness of the an osseous stenosis on the right side and a mem-
vomer and nasal septum. A familial incidence has branous stenosis of the left choana. These authors
been described by some authors (Wilkerson and point out that once an infant is suspected, by rhi-
Coyce 1948). noscopic examination or nasal catheterisation, of
Ronaldson (1881) gave the first clinical descrip- having congenital choanal atresia, CT is the pre-
tion of this condition, which may present as an ferred method of examination because it is a non-
acute respiratory emergency in the neonate. In the invasive technique. It should therefore be used prior
newborn oropharyngeal breathing is an acquired to any contrast radiography.
function (Williams 1971) and takes several days or
weeks to learn. Initially the baby is obliged to breath
through the nose, so that bilateral choanal atresia
usually presents with respiratory distress soon after Congenital Nasal Masses
birth. A problem may also arise during attempts at
feeding, when the infant is unable to breathe and
suck simultaneously. Unilateral choanal atresia is The nasal encephalocoele, the glioma and the
seen in later childhood or in adult life, the chief dermoid are the most common of the congenital
symptom being nasal obstruction and nasal dis- mid-line nasal masses. All three may be associated
charge. with leakage of cerebrospinal fluid (CSF) and the
The diagnosis of choanal atresia is established by possibility of fatal meningitis. A variety of theories
the inability to pass a probe or catheter through the have been advanced to explain the development of
22 Congenital Disease

squamous epithelium in a congenital dermal sinus.

At any point along a congenital dermal sinus there
may be expansion into a cyst. Such a cyst is termed
an epidermoid if it contains only epithelial debris
and is lined by stratified squamous epithelium; it is
called a dermoid if in addition it contains sebaceous
material and hair, and the lining includes elements
of the deeper layer of the skin.
The clinical and imaging features of nasal
dermoids depend upon the extent and location of
the sinus and its cystic component. The sinus pre-
sents as a dimple or pit over the mid-dorsum of the
nose or at the junction ofthe bony and cartilaginous
tip, and characteristically contains a hair Oohnson
and Weisman 1964). These changes are often
associated with hypertelorism and a soft tissue
swelling over the bridge of the nose. Dermoids may
have a communication with the dura through a
patent foramen caecum (Hughes et al. 1980) and
in some the cystic component of the sinus is intra-
cranial, carrying the threat of meningitis and CSF
leakage.
On plain radiograph, conventional tomography
or CT it may be possible to show a well-demarcated
defect in the mid-line of the nasal bones or fronto-

Fig. 3.1. Choanal atresia. Lateral view with horizontal beam and
chin elevated showing obstruction of the contrast posteriorly.

the nasal glioma and encephalocoele. Embry-

ologically, the latter is thought to result from faulty
closure of the neuropore leading to herniation of
the intracranial contents into the nasal area. A
nasal glioma is regarded as a nasal encephalocoele
which has lost its connection with the sub-arach-
noid space (Bradley and Singh 1982).
Nasal dermoids are either simple or complex.
Complex dermoids are thought to be due to faulty
closure of the bones in the nasal area with entrap-
ment of dermal elements. Other theories have been
advanced to explain the simple dermoid, including
entrapment of facial cleft, simple inclusion cyst, and
aberrant development of skin appendages (Hughes
et al. 1980).

Nasal Dermoids
A classical description of a dermoid was given by Fig. 3.2. Nasal dermoid. Well-demarcated defect in the mid-line
Matson and Ingraham (1951). They described the of the nasal bones shown on coronal tomography. Note the
lesion as a cutaneous defect lined by stratified fusiform enlargement of the septum (arrow).
Congenital Nasal Masses 23

nasal suture (Fig. 3.2). The cyst may involve the bridge of the nose. The lesion tends to be located to
nasal septum producing a fusiform soft tissue mass one side of the nasal bridge; this distingUishes them
within the septum or the latter may be bifid. When from dermoids. which are mid-line. A small number
large the cyst may erode the glabellar region or of cases are encountered in an older age group
extend into the ethmoid cells or frontal sinus; in occasionally extending into middle life (Smith et al.
those which have an intracranial communication 1963; Blumenfeld and Skolnik 1965).
it may be possible to show a defect in the cribriform The extranasal glioma is the commonest type and
plate area. may show radiologically as a bone defect in the
locality of the soft tissue mass. In the intranasal
variety the mass tends to occur high in the nasal
Nasal Glioma fossa. to one side of the nasal septum (Fig. 3.3).
the latter being displaced to one side. When both
The first comprehensive account of nasal glioma intranasal and extranasal components are present
was given by Schmidt (1900). since when several there is a communication between the two. usually
extensive reviews have appeared (Black and Smith through a defect in the nasal bone or at its lateral
1950; Walker and Resler 1963; Enfers and margin (Dodd and Bao-Shan Jing 1977). A cranial
Herngren 1975). defect may be present in the intranasal variety but
The term nasal glioma is really a misnomer since difficult to identify. Routine computerised tom-
the lesion is not a true tumour but results from ography (Hughes et al. 1980) in the management
sequestration of primitive brain tissue and consists of patients with suspected nasal glioma is advised.
histologically of glial cells (astrocytes) interlaced in addition to magnetic resonance studies.
with vascular fibrous tissue septa. Gliomata may be
entirely extranasal. intranasal. or both components
may be present. and they mayor may not have
intracranial connections. When intracranial com- Encephalo-meningocoeles
munications do exist the connection passes through
a defect in the cribriform plate or in the region of Encephalo-meningocoeles may be grouped. accord-
the nasal attachment of the frontal bone (Walker ing to their anatomical site. as occipital. sincipital
and Resler 1963). Gliomata most frequently present and basal; the latter two are the only varieties of
at birth or shortly afterwards as a mass on the interest to the otolaryngologist.

Fig. 3.3. Nasal glioma shown on axial cr scan (arrow).

24 Congenital Disease

Sincipital Encephalo-meningocoeles
The sincipital (pertaining to the anterior and upper
part of the head) encephalo-meningocoeles are sub-
classified as follows:
1. Naso-frontaI. in which the herniated cerebral
or meningeal tissue passes between the nasal and
frontal bone. giving rise to a mid-line protuberance
at the nasal root.
2. Naso-ethmoid. in which the defect lies between
the nasal, ethmoid and frontal bones. the swelling
presenting at the junction of the bony and carti-
laginous part of the nose.
3. Naso-orbitaI. in which there is a defect in the
suture line between the frontal, lacrimal and
ethmoid bones with herniation into the orbit.
causing a protuberance at the inner canthus of the
eye.
Haverson et al. (1974) have recorded the radio-
logical changes associated with naso-frontal and
naso-ethmoid encephalocoeles. Naso-frontallesions
present with a V-shaped defect in the frontal bone.
lateral displacement of the medial orbital wall.
depression of the nasal bones. which are attached
to the cribriform plate below the hernia. a low
cribriform plate and a mid-line soft tissue mass.
The naso-ethmoidal variety present with a circular
defect between the orbits. increased inter-orbital
distance. elevation of the nasal bones. which are Fig. 3.5. Same patient as Fig. 3.4. The submentovertical view
attached to the frontal bones above the hernia. a shows the defect in the sphenoid and posterior ethmoids.
normal position of the cribriform plate. and a soft
tissue mass to one side of the mid-line.

Fig. 3.4. Spheno-ethmoidal encephalocoele showing widening of the ethmoid labyrinth and hypertelorism.
Congenital Nasal Masses 25

Basal Encephalo-meningocoeles
The basal cerebral hernias are those which occur
through the cribriform plate and through the sphe-
noid bone, the herniation appearing in the nasal
cavity, nasopharynx, sphenoid sinus, posterior
orbit, or pterygo-palatine fossa. The different var-
ieties of basal encephalocoeles may be described as:
1. Transsphenoidal
1. Transethmoidal
3. Spheno-ethmoidal
4. Spheno-orbital
Pollock et al. (1968) have described the clinical
and radiological features of eight patients with basal
encephalocoeles (five transsphenoidal, three trans-
ethmoidal). Two clinical findings suggest a basal
encephalocoele: a facial abnormality with hyper-
telorism (Fig. 3.4), and a mid-line soft tissue mass
in the nose or epipharyngeal space. In the trans-
sphenoidal variety a defect in the base of the skull
can be shown in plain axial views (Fig. 3.5), but in
the transethmoidal variety some form of tom- Fig. 3.6. Coronal CT scan showing a bone defect at the cribrifonn
ography is needed for their proper demonstration. plate and a transethmoidal encephalocoele.

Fig. 3.7. Same patient as Fig. 3.6. The encephalocoele (arrow) is demonstrated by a sagittal magnetic resonance scan.
26 Congenital Disease

Progressive Hemifacial Atrophy

(Parry-Romberg Disease)

Hemifacial atrophy is a condition of unknown aeti-

ology originally described by Parry (1825) and by
Romberg (1846). Eulenberg (1871) gave the con-
dition the name progressive facial hemiatrophy. but
the disease now goes under the more appropriate
title of progressive hemifacial atrophy (PHA)
(Goldhammer et al. 1981).
The disease is usually first noticed in the first or
second decade of life and is more common in
females. Over several years there is a slow. pro-
Fig. 3.8. Posterior encephalocoele (spheno-orbital) of the left orbit
gressive unilateral atrophy of the face with involve-
in neurofibromatosis. ment of skin and subcutaneous fat. and more rarely
of muscles and bone (Bramley and Forbes 1960;
Gorlin and Pindborg 1964). The atrophy begins in
Lusk and Dunn (1986) have recorded the findings the skin or subcutaneous tissues and spreads to
of magnetic resonance studies in a 19-month-old involve the whole of one side of the face. with
child who was shown at surgery to have an anterior atrophy of orbital fat and enophthalmos. Neuro-
cranial fossa encephalocoele. In this case magnetic logical complications including migraine. tri-
resonance proved superior to CT in its ability to geminal neuralgia. epilepsy and hemiplegia may
distinguish brain from normal tissues in the nose. ensue (Wolf and Verity 1974; Asher and Berg
Examples of an encephalocoele demonstrated by CT 1982).
and magnetic resonance are shown in Figs. 3.6 and In rare cases hemifacial atrophy affects the bone
3.7. more than the soft tissues (Goldhammer et al.
The spheno-orbital type of encephalocoele 1981). resulting in a general atrophic collapse of
involves the posterior orbit and superior orbital the walls of the maxillary antrum (Figs. 3.9. 3.10).
fissure and is commonly associated with neuro- In this respect hemifacial atrophy needs to be dis-
fibromatosis. It produces the characteristic empty tinguished from other causes of inward collapse of
orbit sign of this condition (Fig. 3.8). the antral boundaries - most notably the orbital

Fig. 3.9. Progressive hemifacial atrophy. There is collapse of the orbital floor (arrow) resembling a severe blow-out fracture. but
without a history of trauma.
Progressive Hemifacial Atrophy 27

Fig. 3.10. Same patient as Fig. 3.9. The axial CT scan shows that there is also an incurvation of the posterior wall ofthe maxillary
antrum (arrow).

floor - such as blow-out fractures and chronic osteo- Gorlin RJ, Pindborg JJ (1964) Syndromes of the head and neck.
myelitis of the maxilla (see Chap. 5). The diagnosis McGraw-Hill Book Company, New York, pp341-344
Hasegawa M, Oku T, Tanaka H et al. (1983) Evaluation of CT
is made on the clinical findings and the radiological in the diagnosis of congenital choanal atresia. J Laryngol Otol
features shown on plain radiographs and CT scan; 97:1013-1015
the striking change is the collapse and incurvation Haverson G, Bailey IC, Kiryabwire JWM (1974) The radiological
of all the sinus walls of the maxillary antrum on diagnosis of anterior encephalocoeles. Clin Radiol 25: 317-
the side involved (Figs. 3.9, 3.10). 322
Hughes GH, Sharpino G, Hunt W, Tucker HN (1980) Man-
agement of the congenital mid-line mass: a review. Head
Neck Surg 2:222-233
Johnson GF, Weisman PA (1964) Radiological features of
dermoid cysts of the nose. Radiology 82:1016-1023
Lusk RP, Dunn VD (1986) Magnetic resonance imaging in
References encephalocoeles. Ann Otol Rhinol Laryngol 95:432-433
Matson DO, Ingraham FD (1951) Intracranial complications of
congenital dermal sinuses. Pediatrics 8:463-474
Asher SW, Berg BO (1982) Progressive hemifacial atrophy. Arch Parry CH (1825) Collections from the unpublished medical
Neurol 39:44-46 writings of the late Caleb Hillier Parry. Underwood, London
Black BK, Smith DE (1950) Nasal glioma. Arch Neurol Psychiatr Pollock JA, Newton TH, Hoyt WF (1968) Transsphenoidal and
64:614-630 transethmoidal encephalocoeles. Radiology 90:442-453
Blumenfeld R, Skolnik EM (1965) Intranasal encephalocoeles. Romberg HM (1846) Klinische Ergebnisse. A Forstrer, Berlin
Arch OtolaryngoI82:527-531 Ronaldson TR (1881) Note on a case of congenital closure of the
Bradley PJ. Singh SD (1982) Congenital nasal masses: diagnosis posterior nares. Edinburgh Med J 26:103 5-103 7
and management. Clin Otolaryngol 7:89-97 Schmidt MB (1900) Ober seltene Spaltbildungen in Beireiche des
Bramley p, Forbes A (1960) A case of progressive hemiatrophy mittleren Stimfortsatzes. Virchows Arch [A] 162:340-370
presenting with spontaneous fractures of the lower jaw. Br Smith KR, Schwartz HG, Luse SA, Ogura JH (1963) Nasal
Med J i: 1476-1478 gliomas; a report of 5 cases, with electron microscopy of one.
Dodd GD, Bao-Shan Jing (1977) Radiology of the nose, paranasal J Neurosurg 20:968-982
sinuses and nasopharynx. Williams and Wilkins, Baltimore Walker EA, Resler DR (1963) Nasal glioma. Laryngoscope
Enfers B, Hemgren L (1975) Nasal glioma. J Laryngol Otol 73:93-107
89:863-868 Wilkerson WW, Coyce LE (1948) Congenital choanal occlusion.
Eulenberg A (1871) Lehrbuch des functionellen Ner- Trans Am Acad Ophthalmol 52:234-236
venkrankheiten. Berlin Williams HJ (1971) Posterior choanal atresia. Am J Roentgenol
Goldhammer Y, Kronenberg J, Tadmor R, Braham J, Leventon 112:1-11
G (1981) Progressive hemifacial atrophy (Parry-Romberg's Wolf SM, Verity MA (1974) Neurological complications of pro-
disease), principally involving bone. J Laryngol OtoI95:643- gressive facial hemiatrophy. J Neurol Neurosurg Psychiatry
647 37:997-1004
4 Trauma

Fractures of the Nasal Bones minution (T.R. Bull. 1987. personal com-
munication).

Fractures of the nasal bones are common and are

seen either in isolation or. not infrequently. associ-
ated with other injuries to the facial bones. They Fractures of the Maxillary Antrum
have been classified into linear fractures. lateral
fractures. frontal fractures (partial or complete) and
fronto-Iateral fractures. There is also the very severe Isolated fractures of the maxillary antrum occur
"smash fracture". with flattening and comminution most commonly in the antero-Iateral wall. Usually
of the nasal bones. This involves the frontal pro- they are due to direct impact and are often com-
cesses of the maxilla. the lacrimal bones and nasal minuted with depressed fragments. They are best
septum. and may extend to the anterior ethmoid demonstrated by CT (Fig. 4.1). A less common type
cells and cribriform plate. of isolated fracture involves the orbital rim at the
Lateral fractures are the commonest variety and infraorbital margin and mayor may not be associ-
all may be demonstrated using the techniques ated with an antral blow-out fracture. In addition
described in Chap. 1. However. radiology has little fracture of the alveolus and floor of the maxillary
part to play in the management of nasal bone frac- antrum may occur during dental extraction or oral
tures unless there is extension to other facial bones. surgery.
De Lacey et al. (1977) concluded from a study of
100 patients with nasal bone trauma that radio-
graphy of the nasal bones is an unnecessary pro-
cedure and should only be carried out at the specific Malar Fractures
request of an ENT surgeon. Reduction of a nasal
bone fracture is indicated because of the clinical
deformity rather than the radiological appearance. Malar fractures are common fractures of the middle
When the blow to the nose may have involved other third of the facial skeleton and occur as the result
facial bones clinically appropriate views of facial of laterally disposed violence to the facial bones.
bone should be obtained; but views of the nasal The force of the blow causes the malar bone to
bone itself are often unnecessary. However. most be displaced downwards. inwards and posteriorly.
ENT surgeons would justify nasal radiographs after with a hinge movement and slight separation or
trauma on medico-legal grounds as a permanent fracture at the fronto-malar suture. This is
record of the trauma. and to give some idea of the accompanied by a fracture of the zygomatic arch
severity of the injury. particularly with regard to with little or no displacement. At the same time
displacement of the fragments and degree of com- separation occurs at the junction of the medial third
30 Trauma

The patient may present with facial anaesthesia

or paraesthesia due to damage to the infraorbital
nerve, and if the zygomatic arch is depressed it may
interfere with the action of the mandible by pressure
on the coronoid process. A fracture through the
floor of the orbit may cause trapping of the inferior
rectus muscle or the inferior oblique in a similar
manner to an antral blow-out fracture.
The best plain radiographic projection for the
demonstration of these fractures is an overtilted
occipito-mental view or 30· occipito-mental pro-
jection. In this the radiographic base line is adjusted
Fig. 4.1. Depressed fracture (arrow) of the anterior wall of the
maxillary antrum shown on axial CT. as in the standard oCcipito-mental view but with
the tube centred through the vertex of the skull and
angled 30· towards the feet. In this view the lower
and lateral two thirds of the inferior orbital rim, orbital margin and the zygoma form a continuous
usually with depression of the outer fragment. arch; any break in this arch will indicate the site
Sometimes this is also associated with disruption of of fracture and can also show displacement and
the orbital floor. If the force is moderate the body of impaction of the main fragment (Fig. 4.2). The
the malar bone is left intact, but in a more severe injury may be accompanied by a generalised opacity
injury this bone is comminuted and may become of the antrum due to haemorrhage into the sinus
impacted into the maxillary antrum. With severe cavity, or more commonly there is a localised thick-
degrees of impaction there is nearly always a com- ening of the mucosa of the sinus due to mucosal
minution of the orbital floor, with prolapse of the
orbital contents into the maxillary antrum.

Fig. 4.2. Overtilted occipito-mental projection showing a malar fracture with fractures of the zygoma and infraorbital margin (arrows).
Note the thickening of the mucosal shadow along the lateral wall of the sinus due to submucosal haemorrhage or oedema.
Malar Fractures 31

oedema or submucosal haemorrhage (Fig. 4.2).

Occasionally a blood fluid level may be present.
Of all the imaging methods available. axial CT is
the best for showing displacements of the malar
eminence in the antero-posterior plane and may
also show the lateral wall fractures. In addition
coronal CT will show inferior displacement of the
outer fragment with a characteristic step deformity
of the inferior orbital rim (Figs. 4.3. 4.4).

LeFort Injuries

The sinuses may be involved in severe fractures of a

the facial bones. usually sustained during a road
accident. Le Fort (1901) described anatomical lines
of weakness in the facial skeleton. and his name
has been given to the fractures which follow these
lines. LeFort I fractures are usually sustained by
force from in front and in an upward direction.
Minor LeFort I injuries may simply consist of a
fracture of the upper alveolar margin. but in more
severe injuries to the lower third of the facial bones
the fracture may extend through the dense bone of
the maxilla to the pterygoid plates. and separate the
hard palate and alveolus (Fig. 4.5).
In a LeFort II injury the line of fracture runs
across the nasal bones. extending on both sides
across the frontal process of the maxilla. and across
the naso-Iacrimal canal. From this point the line of
weakness (or fracture) traverses the floor of the
b

Fig. 4.4. a Coronal cr scan of a malar fracture with characteristic

step deformity of the outer orbital rim. b The parasagittal refor-
matted views also show a step deformity of the orbital floor
anteriorly.

orbit. crossing the lower orbital margin in the region

of the maxillo-zygomatic suture. and eventually
involving the lateral wall of the maxillary antrum
and pterygoid laminae (Fig. 4.5). This type of frac-
ture is produced by force applied to the middle level
of the facial skeleton from an antero-posterior direc-
tion. or sometimes from a lateral direction.
Force applied to the facial bones from a superior
direction will result in a local fracture of the nasal
and ethmoid bones if the momentum is small. or a
high-level LeFort III fracture when the impact is
severe. The line of fracture starts at the upper part
of the nasal bones close to the fronto-nasal suture.
In some cases the dislocation of the nasal bones at
Fig. 4.3. Coronal cr scan showing a comminuted malar fracture this site results in disruption of the cribriform plate
(arrow) with depression of the outer orbital floor and fracture of of the ethmoid. and fracture of the floor of the
the latera! wall of the antrum. anterior fossa. From its mid-line point of origin the
 32 Trauma

ball, hurling ball or baseball, but the vast majority

are the result of a blow in the eye from a closed fist.
According to Smith and Regan (1957) the mech-
anism of injury is as follows: Part of the impact
of the blow is absorbed by the orbital rim, which
remains intact, but the force of the blow causes a
backward displacement of the eye and an increase
in intraorbital pressure, with a resultant fracture of
the orbital floor or medial wall, decompressing the
orbit and causing a herniation of orbital soft tissues
into the maxillary antrum or ethmoid cells. The
a adjacent extraocular muscles may be impeded as a
result of this herniation, producing an impairment
of ocular mobility and diplopia. The inferior rectus,
inferior oblique, or more rarely the medial rectus
muscle may be involved.
It is the impairment of muscle function which
constitutes the particular gravity of a blow-out frac-
ture. Early surgical intervention may be required to
release the trapped muscle and restore free move-
ment of the globe. Unless this is done the diplopia
will persist. Clinically, vertical diplopia is the most
important feature and may be accompanied by par-
aesthesia or anaesthesia of the cheek and upper lip,
in the distribution of the infraorbital nerve, which
b may be involved in the fracture.
The characteristic radiological appearance is that
Fig. 4.5. The lines of fracture in LeFort injuries to the mid-face: of a small, soft tissue shadow in the roof of the
a LeFort I, b LeFort II and c LeFort III injuries. maxillary antrum, due to the herniated soft tissues
from the orbit; this is associated with a depressed
bony fragment, which is nearly always visible on
fracture runs through the medial wall ofthe orbit in plain radiography at the fracture site. Sometimes
the neighbourhood of the fronto-maxillary suture, oblique views are necessary to show a depressed
crossing the lacrimal bone and medial orbital wall fragment convincingly or it may be best shown on
and extending backwards to the inferior orbital a lateral film. But the projection which consistently
fissure. At this point the line of fracture bifurcates, shows these fractures, even when only minimal
one component running upwards and forwards to changes are present, is the nose-chin or occipito-
terminate in the lateral wall of the orbit immediately oral view of the orbits (Fig. 4.6). Tomography in
below the fronto-malar suture, while the lower the coronal plane may confirm the fracture but is
component of the fracture runs downwards and not usually needed to make the diagnosis.
backwards from the pterygo-maxillary fissure to CT can be used as a substitute for conventional
involve the roots of the pterygoid laminae (Fig. 4.5). tomography in the demonstration of blow-out frac-
In this injury, therefore, there is a virtual separation tures. CT has the advantage of showing not only
or partial separation of the facial bones from the the site of fracture, but also the state of the rectus
cranium above the zygomatic arch, the force of the muscles; and it is possible to show herniation of
trauma causing the entire middle third of the facial the extraocular muscle. However, herniation of the
skeleton to hinge backwards through the ethmoid medial or inferior rectus muscle does not imply
labyrinth. that the muscle action is impeded; conversely the
absence of herniation does not necessarily mean
that there is no entrapment. The explanation for
this has been given by Koorneef (1982) (Fig. 4.7).
Blow-Out Fractures Anatomical studies have shown that in the normal
orbit there is a series of fibrous connective tissue
septa which join the periorbita to the rectus muscle
Blow-out fractures are classically caused by violent sheaths and the tendinous ring connecting them.
contact with a round hard object such as a cricket Frequently it is the entrapment of these connective
Blow-Out Fractures 33

Fig. 4.6. Undertilted oCcipito-mental projection showing a typical antral blow-out fracture (arrow) .

tissue elements which causes the muscle dys-

function without the muscle being displaced.
Entrapment of the connective tissue septa in an
orbital blow-out fracture may cause a downward
drag on the medial or lateral rectus muscle via the
tendinous ring which joins them to the inferior
rectus muscle (Fig. 4.7). This change can be
detected on CT scan as a downward displacement
of the medial rectus muscle (Figs. 4.8. 4.9) and.

Fig. 4.8. Blow-out fracture of the floor of the orbit (white arrow).
Medial rectus drag is shown on coronal CT scan (black arrow).
indicating entrapment of the inferior rectus muscle.

when present. may indicate the need for surgical

intervention to release the trapped muscle. Another
feature which can be shown on CT is the presence
of fibrous adhesions within the antrum (Fig. 4.10).
which clearly need to be dealt with if restoration of
a normal orbital floor is to be achieved surgically.

Fractures of the Frontal Sinus

Fig. 4.7. Line drawing showing entrapment ofthe fibrous septa

at the site of an antral blow-out fracture. with downward drag Fractures of the frontal sinus are usually caused by
on the medial rectus muscle. (Reproduced with permission from direct violence. but the sinuses may be involved
Koomeef 1982.) indirectly by a fracture of the skull vault and frontal
34 Trauma

Fig. 4.11. Fragments of windscreen glass in the frontal sinus

(arrow).
Fig. 4.9. Reformatted coronal and sagittal CT sections of the
orbital floor. The coronal section (top) shows a downward dis-
placement of the medial rectus muscle. or medial rectus drag
(small arrow). The sagittal section (bottom) shows the herniation
of soft tissues (large arrow) and confirms muscle entrapment.

Fig. 4.10. In orbital floor fractures adhesions (arrow) may form

between the herniated soft tissues or bony fragments and the
opposite walls of the maxillary antrum. These can only be
appreciated if the CT section is imaged with the window level set Fig. 4.12. Severe comminuted fracture of the frontal bone and
in the negative range of the Hounsfield scale (Le. - 400 to - 500 frontal sinuses. The patient received a direct blow to the forehead
Hounsfield units). by the blunt end of a wood-chopper.

bones. Fractures may involve one or both walls. Fractures of the Sphenoid Sinus
Fracture of the posterior wall of the frontal sinus is
rare and is usually associated with fractures of the
floor of the anterior fossa. These fractures may be
complicated by traumatic rhinorrhoea, pneumo- Fractures of the sphenoid sinus usually occur as
cephalus or meningitis. Fractures involving the part of a severe fracture of the skull base, frequently
anterior wall are far more common. They are associated with a longitudinal fracture of the
usually due to direct trauma in road accidents and petrous bones. The sphenoid may also be involved
not infrequently fragments of windscreen glass are in a LeFort III fracture of the facial bones or a very
present within the sinus cavity (Fig. 4.11). the severe direct injury to the frontal bones (Fig. 4.15).
radiological demonstration of these injuries is by On plain radiographs direct evidence of a fracture
plain radiograph and CT scan (Figs. 4.12, 4.13 and line may be difficult to see unless the floor of the
4.14). pituitary fossa is involved (Fig. 4.15), but indirect
Fractures of the Sphenoid Sinus 35

Fig. 4.13. Comminuted fracture ofthe frontal sinus shown on coronal CT scan, with drainage tube in situ.

evidence may be given by the presence of an effusion

into the sinus cavity with a fluid level (Fig. 4.16).
The only way to show the full extent of these bony
injuries is by CT scan. and the axial projections
are particularly helpful in this respect (Fig. 4.17).
Pneumocephalus or CSF rhinorrhoea is a not
infrequent complication of these fractures.

Fig. 4.14. Same patient as Fig. 4.13. Axial CT section showing Fig. 4.15. Same patient as Fig. 4.12. Force transmitted from a
fractures of both anterior and posterior walls of the sinus cavity blow on the forehead has fractured the sphenoid and disrupted
(arrow). the floor of the pituitary fossa (arrows).
36 Trauma

Fractures of the Ethmoid Cells

Fractures of the ethmoid cells occur as part of the

more extensive LeFort III injuries (see above), but
they may also be found in isolation. They may occur
in the roof of the ethmoid labyrinth in the region of
the cribriform plate or they may involve the medial
orbital wall. In the latter fractures the evidence from
a series collected by the author (Lloyd 1966) would
suggest that the mechanism of injury can be of two
types:
1. A direct injury to the medial wall of the orbit
and base of the nose. Sometimes the injury is
associated with a fracture of the nasal bones.
Fig. 4.16. Fluid level in the sphenoid sinus (arrow) due to a 2. A medial wall blow-out fracture through the
fracture with CSF leak. lamina papyracea of the ethmoids. This may
be associated with a blow-out fracture into the
antrum; many patients with blow-out injuries
have a combined antro-ethmoidal fracture.
Logically the lamina papyracea would seem the
part of the orbit most likely to fracture by the blow-
out mechanism, since it is the most fragile part of
the orbital wall. This type of injury presents radi-
ologically in many patients as an orbital emphy-
sema, associated with clouding of the ethmoid cells
on the injured side (Fig. 4.18). The actual site of the
fracture is seldom directly visible on plain radio-
graphs, but the injury is not usually of any sig-
nificance and only rarely is there any interference
with muscle function, so that more detailed radio-
logical investigation is seldom required. If it does

Fig. 4.17. Axial CT sections of a fracture of the skull base involv- Fig. 4.18. Ethmoid blow-out fracture. Clouding of the ethmoid
ing both petro-mastoids and the sphenoid sinus (arrows). cells and orbital emphysema can be seen.
Fractures of the Ethmoid Cells 37

become necessary to show the exact site of the Cerebrospinal Fluid Leaks
fracture and its extent then CT scan in the axial
plane is the procedure of choice. These fractures can
also be demonstrated by coronal CT and magnetic CSF rhinorrhoea is caused by an abnormal con-
resonance scans (Figs. 4.19, 4.20). nection between the sub-arachnoid space and the
nasal cavity. This may be directly from the anterior
cranial fossa via the cribriform plate or ethmoid
cells; from the middle fossa via the sphenoid sinus;
or indirectly from the posterior fossa via the eusta-
chian tube. The majority of leaks are either trau-
matic or spontaneous in origin, some occurring
after surgery (Fig. 4.21). Intracranial and extra-
cranial tumours, encephalocoeles and infection are
all rare causes.
Fractures with CSF leaks are most common in
the ethmoid cells and cribriform plate area. CSF
leaks also occur in fractures of the frontal sinuses,
sphenoid sinuses and temporal bones. CSF fistulae
arising spontaneously without a history of trauma
occur at several sites: (1) from the cribriform plate
area in which the CSF can reach the nasal cavity
via a prolongation ofthe sub-arachnoid space along
the olfactory nerve filaments; (2) via a dehiscence
in the basi-sphenoid (Fig. 4.22); (3) via the petro-
mastoid in certain congenital anomalies ofthe inner
ear; and (4) into the sphenoid sinus from the middle
cranial fossa. Fistulae of the last type may occur in
cases of non-tumorous enlargement of the pituitary
fossa - the so-called empty sella (Kaufman et al.
1977). These authors also reported five patients in
whom the aetiology was thought to be herniation
of the meninges through natural pits or holes in the
Fig. 4.19. Coronal cr scan of a small blow-out fracture of the
medial wall (arrow) caused by an elbow in the face during floor of the middle fossa into a well-pneumatised
basketball. At surgery the medial rectus muscle was shown to be sphenoid sinus. With fixation ofthese prolongations
tethered. the dura becomes thinned and eventually allows
herniation of the arachnoid with the formation of
an arachnoid diverticulum; rupture then results in
a CSF fistula.

Fig. 4.20. Coronal magnetic resonance scan showing an antro- Fig. 4.21. Coronal CT scan showing a CSF leak resulting from
ethmoidal blow-out fracture. The medial component of the frac- fracture of the roof of the ethmoid labyrinth (arrow) following
ture is arrowed. nasal polypectomy.
38 Trauma

Fig. 4.22. Congenital dehiscence (arrow) shown in the basi-sphenoid on coronal CT scan. At surgery CSF was seen to be leaking into
the nasopharynx from this site.

Recognition of spontaneous rhinorrhoea is abnormality of the inner ear, or fracture if there is

dependent upon a good clinical history to differen- a history of trauma. The common deformity associ-
tiate it from chronic rhinitis. The discharge is ated with recurrent meningitis and a spontaneous
usually unilateral and the flow changes with alter- CSF leak has been described by Phelps and Lloyd
ations in posture. Confirmation of the presence of (1978, 1983) and consists of a severely dysplastic
CSF is obtained by demonstrating a high con- labyrinth with a wide communication between the
centration of glucose in the fluid. vestibule and cochlea and a tapering internal audi-
tory meatus. This deformity allows CSF to pass into
the vestibule and thence through the oval window
Radiological Investigation into the middle ear.

Initially enquiry should be made into the state ofthe

patient's hearing: if there is unilateral sensorineural
hearing loss then the petrous bones need to be
examined tomographically to exclude a congenital

Fig. 4.23. High-resolution coronal CT scan showing a depressed Fig. 4.24. CT with water-soluble contrast medium in the sub-
fracture of the floor of the anterior fossa into the horiwntal part arachnoid space. Coronal sections show seepage of contrast into
of the frontal sinus - the site of CSF leak. the frontal sinus and ethmoids through the fracture site.
Cerebrospinal Fluid Leaks 39

radiograph. This is best achieved by a combination

of CT (Fig. 4.23) and conventional tomography. the
latter being used to provide sagittal sections of the
anterior fossa.
If these methods fail to show the site of a fistula
then CT with an intrathecal contrast ' agent
(Iohexol) may identify the leak (Figs. 4.24. 4.25).
The contrast medium is injected into the sub-arach-
noid space by the translumbar or transcervical
route. The contrast is run into the cranial cavity
with the patient prone and made to gravitate over
the suspected area (Le. the cribriform plate and
planum sphenoidale); the scans are made with the
patient in this position. Delayed scans after several
minutes may be helpful to allow time for the con-
trast to seep into the appropriate air cell.

References

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ology of nasal injuries: problems of interpretation and clinical
relevance. Br J Radiol 50:412-414
Kaufman B. Neilson FE. Weiss MH. Brodkey JS. White RJ. Sykora
Fig. 4.25. Spontaneous CSF leak shown by positive contrast GF (1977) Acute spontaneous. non-traumatic normal-pres-
cisternography. A small quantity of the contrast' medium has sure cerebro-spinal fluid fistulas Originating from the middle
leaked into the upper part of the nasal cavity via the cribriform fossa. Radiology 122:379-387
plate (small arrow). Note the contrast medium absorbed by the Koorneef L (1982) Current concepts in the management of orbi-
nasal plug (large arrow). tal blow-out fractures. Ann Plast Surg 9(3):185-20b
LeFort R (1901) Etude experimentale sur les fractures de la
mouchoire superieuse. Rev Chir 23:208-220
On plain radiographs and CT the presence of a lloyd GAS (1966) Orbital emphysema. Br J Radiol 39:933-938
fluid level will give a clue to the probable site of a Phelps PD. lloyd GAS (1978) Congenital deformity of the internal
CSF fistula into the sinuses (Fig. 4.16); or there auditory meatus and labyrinth associated with CSF fistula.
may be simple opacification of an air cell in the Adv OtorhinolaryngoI24:51-55
Phelps PD. Lloyd GAS (1983) Radiology of the ear. Blackwell
neighbourhood of the leak. In post-traumatic fist- Scientific Publications. Oxford
ulae direct evidence of bone interruption should be Smith B. Regan WF (1957) Blow-out fracture of the orbit. Am J
sought if a fracture is not obvious on the plain OphthalmoI44:733-740
5 Inflammatory and Allergic Sinus Disease

Acute Sinusitis is by no means clear-cut. however. and does not

always co-relate well with the findings at antro-
scopy and mucosal biopsy. Acute sinusitis is
Acute sinusitis is most often due to secondary bac- accompanied by an outpouring of fluid into. the
terial infection following an upper respiratory tract sinus cavity causing a total loss of translucence in
infection of viral origin. Infection can also occur in the affected sinus on the radiograph. This is a non-
the maxillary antrum by secondary extension from specific sign and. although in the vast majority of
an infected tooth in the upper jaw. The primary site patients it denotes infection. a sinus filled with blood
of infection is the lining mucosa of the sinus. The or new growth could give a similar appearance.
accompanying oedematous swelling of the mucosa More certain evidence of infection is provided radi-
will show on the radiograph as an opaque rim ologically when there is a fluid level (always
around the periphery of the sinus. It is said that in assuming that there has been no trauma or recent
infective sinusitis the rim of mucous membrane antral washout). It is important for the dem-
follows the contour of and is parallel to the walls of onstration of fluid levels that all sinus radiographs
the sinus - in contradistinction to allergic sinus are taken with the patient in an upright position
disease in which the mucosa assumes a polypoid with a horizontal X-ray beam; if there is any doubt
aspect with a convex inner border. This distinction about the presence of fluid a tilted view should

Fig. 5.1. Bilateral fluid levels in the antra shown on a tilted Fig. 5.2. Magnetic resonance scan. T,-weighted spin echo
occipito-mental view. sequence showing high signal and fluid level in the antrum.
42 Inflammatory and Allergic Sinus Disease

be obtained (Fig. 5.1), which will lead to a new

horizontal level. It should be remembered that thick
viscid pus or mucus may require a few moments to
assume a new level when the head is tilted. In
many patients the upright lateral view ofthe sinuses
shows a fluid level most obviously. Although fluid
levels can be adequately demonstrated in all the
sinuses on plain radiographs. they are often more
obvious on CT scan. They may also be shown by
magnetic resonance studies, when the fluid in the
sinus cavity and the thickened mucosa give a
characteristically strong signal on Trweighted spin
echo sequences (Figs. 5.2, 5.3 and 5.4).
Fig. 5.3. Same patient as Fig. 5.2. Fluid levels are also present in
the ethmoids and sphenoid sinus.
Complications
Osteomyelitis
An empyema is found most commonly in the maxil-
lary antrum and is a complication of acute sinusitis.

Fig. 5.4. Inflammatory mucosal thickening shown on a T,-

weighted spin echo sequence using a long time to echo.

Fig. 5.5. Infection in the maxillary antrum with early osteolysis

and fragmentation of the sinus walls. There is a fluid level cen- Fig. 5.6. Osteomyelitis in the left frontal bone following frontal
trally in the antrum. (The CT scan was made in the prone sinus infection. An osteolytic area in the wall of the sinus with
position.) a sequestrum (arrow) can be seen.
Acute Sinusitis 43

a b
Fig. 5.7a,b. Two axial CT sections showing sequestration of the orbital floor in a patient with maxillary antritis (arrow). Note the
associated loss of muscle outline in the infratemporal fossa.

It may rarely lead to bone involvement and osteo- points of differentiation from tumour malignancy
myelitis in the surrounding bony walls. The radio- may be observed. For example, bone destruction in
logical changes of osteomyelitis lag behind the a malignant tumour is almost always accompanied
clinical signs. Loss of outline of the sinus wall fol- by a large tumour mass in the sinus concerned
lowed by frank osteolysis (Fig. 5.5) and seques- and in the adjacent nasal cavity, with outward
tration is the usual sequence. The latter change is expansion and destruction of the sinus walls. Con-
most often seen in the frontal bone in association versely infections accompanied by osteomyelitis
with frontal sinus infection (Fig. 5.6), but may also may produce an inward collapse of the sinus (Fig.
occur in the maxilla. In this situation the bone 5.8), frequently with a central air shadow in the
destruction may be difficult to differentiate on a sinus cavity and sometimes with a fluid level.
plain radiograph from that caused by malignant Inflammatory disease in the antrum may also be
sinus disease, and the identification of a sequestrum associated on CT with loss of muscle definition in
is important in this respect (Fig. 5.7a,b). Other the infratemporal fossa due to oedematous changes
(Fig.5.7b).

Frontal Lobe Abscess

Spread of infection posteriorly through the wall of
the frontal sinus may result in an intracranial
abscess either within the brain substance, sub-
durally or extradurally. Plain radiographs give no
indication of the development of this complication
unless gas is present in the cavity of the abscess,
but CT scan after intravenous contrast will show
the characteristic ring enhancement of an abscess
(Fig. 5.9). An abscess may also be demonstrated by
magnetic resonance (Fig. 5.10).

Orbital Cellulitis
Fig. 5.8. Chronic osteomyelitis. Axial CT section showing inward
collapse of the antral walls (right). This serves to distinguish Orbital cellulitis may result from acute bacterial
osteomyelitis from neoplastic bone destruction. where there is infection in the ethmoids (Fig. 5.11), frontal sinus or
inevitably some bone expansion present and a soft tissue mass. maxillary antrum. In the author's series of patients
44 Inflammatory and Allergic Sinus Disease

with this condition. two thirds showed obvious

sinus infection on plain radiographs. and very
occasionally gas or air with a fluid level was visible
within the soft tissues at the site of an abscess (Fig.
5.12). The abscess is better demonstrated by CT as
a soft tissue mass frequently extraconal and outside
the periorbita adjacent to the infect~d ethriJ.Oid cells
(Fig. s.l3a.b). In this situation the abscess may
assume a crescentic shape around the periphery of
the supero-medial orbit. It is important to localise
abscess formation when it is present. Unequivocal
evidence is provided when there is soft tissue gas
with or without a fluid level. Ring enhancement
on CT after intravenous contrast will also indicate
abscess formation.

Chronic Sinusitis

Chronic sinusitis usually follows an acute infection.

but infections such as tuberculosis or actinomycosis
are chronic from inception. A chronically infected
sinus shows mucosal thickening. which as a rule
alters little with time or treatment unless there is
an acute exacerbation. In some patients a reactive
change may occur in the sinus walls. Skillern
(1936) described the conversion of granulation Fig. 5.10. Sagittal magnetic resonance scan of a large squamous
tissue in the sinus to osteoid by osteoblast invasion. cell carcinoma associated with frontal sinus infection. osteo-
followed by calcium deposition and the conversion myelitis and an abscess in the scalp (Pott's "puffy tumour":
of the osteoid into true bone. The result is a pro- arrow).
liferative osteitis causing an increased bone density
in the sinus walls and leading to partial or com-
plete obliteration of the sinus cavity. The frontal sinuses are most commonly affected.
but in some patients there may be multiple sinus
involvement. and the condition may be associated
with granulomatous changes in the orbit or recur-
rent orbital cellulitis (Fig. 5.14).
A similar bony thickening of the sinus walls may
be seen in the maxillary antrum after a Caldwell-
Luc operation. producing an overall opacity of the
sinus on the plain radiograph (Cable et al. 1981).

Allergic Sinusitis

Allergic sinusitis is caused by an allergic reaction

in the mucosa of the upper respiratory tract giving
rise to oedema of the lining membranes of the
sinuses and nose accompanied by hypersecretion.
The radiological changes may be difficult to dis-
Fig. 5.9. Cerebral abscess. Typical ring enhancement on CT after tinguish from those of infective sinusitis. especially
intravenous contrast. as the two conditions may coexist: for example total
 Allergic Sinusitis 45

Fig. 5.12. Orbital cellulitis. The plain radiograph shows an

abscess in the medial orbit and right ethmoid cells with fluid level
(arrow).

b
Fig. 5.11a,b. Orbital cellulitis. a The coronal CT section shows
an abscess in the supero-medial quadrant of the left orbit. b The
underlying cause is shown in the axial section: a pyocoele in the
posterior ethmoid cells (arrow).

opacity of a sinus may occur in both conditions. a

Thickening of the nasal turbinates is characteristic
of allergic sinusitis and is usually accompanied by
the polypoid type of mucosal thickening in the
adjacent sinuses, the thickened mucosa producing
a convex indentation into the cavity of the sinus.
Bilateral changes in the sinuses are also typical of
allergic sinus disease and the condition is frequently
complicated by nasal polyposis. Sinus fluid levels are
uncommon in allergic sinusitis and when present
usually denote superadded infection.

Nasal Polyposis b
Fig. 5.13a,b. Axial and coronal CT scans shOWing infection in
the ethmoid cells with orbital cellulitis and abscess formation.
Simple nasal polyps are pedunculated sections of Note the bone destruction in the medial orbital wall (arrow) at
oedematous upper respiratory mucosa. They can the site of the abscess.
46 Inflammatory and Allergic Sinus Disease

8 b
Fig. S.14a,b. Chronic infection in the sinuses with sclerosing osteitis. An increase in the thickness and density of the walls of the
sphenoid sinus is shown on axial (a) and coronal (b) CT scans. Note that the maxillary antrum is also affected. These sinuses were
chronically infected. and the patient had had six attacks of orbital cellulitis in the preceding year.

arise from any part of the nasal and sinus mucosa may undergo squamous metaplasia (Fig. 5.26).
and are often multiple and bilateral. Histologically Their pathogenesis is poorly understood, but it is
they consist of a grossly oedemato us stroma covered likely that the majority of polyps are related to
by respiratory epithelium, which in some places hypersensitivity mediated through mast cells
(Michaels 1987). Non-allergic asthma is a common
accompanying condition and polyps are also associ-
ated with aspirin hypersensitivity. Their com-
monest site of origin is the mucosa of the ethmoid
cells; much less commonly they arise in one or
other maxillary antrum, entering the nasal cavity
through its ostium and passing backwards through
the posterior nares to form an antro-choanal polyp.
In either case the mechanism of formation is the
same.
Multiple nasal polyps may completely occlude the
nasal cavity and prolapse through the posterior
choana into the nasopharynx, where they may be

Fig. 5.15. Nasal polyposis. Plain radiograph showing loss of

translucence in the ethmoid cells, frontal sinuses and the upper Fig. 5.16. Nasal polyposis. Widening of the nasal bones shown
part of the nasal cavity. on an occipito-mental projection.
Nasal Polyposis 47

visible on lateral radiographs. However they are

seldom recognised individually on plain radio-
graphs; more often the nasal cavity presents a
homogeneous loss of translucence, especially in its
upper part (Fig. 5.15). Long-standing polyps can
enlarge to such a degree that they produce expan-
sion of the walls of the nasal cavity (Fig. 5.16) and
ethmoid labyrinth (Lloyd 1971, 1975). This affects
the whole labyrinth, but its general form is largely
retained (Fig. 5.17). The expansion may produce
an increase in the interpupillary distance of the
eyes and hypertelorism. Typically the changes are
accompanied by a generalised loss of translucence
in the sinuses on plain radiographs, decalcification
of their walls and erosion of the ethmoid septa (Fig.
5.18). The essential feature of nasal polyposis with
or without ethmoid expansion is the bilateral nature
of the changes. This distinguishes it from antro-
ethmoidal malignancy, where the changes often
remain unilateral even when the disease is far
advanced.
The changes described above are, in a small min-
ority of patients, associated with a local expansion
of a sinus or bone erosion (Wilson 1976; Winestock
et al. 1978). In some patients there may be muco- Fig. 5.18. Nasal polyposis. Axial CT scan showing total loss of
coele formation - either single or multiple. When translucence in the ethmoid cells with erosion of the ethmoid
these changes are found in children under 10 they septa. Note the slight expansion of the lamina papyracea on the
are usually associated with mucoviscidosis (Fig. right side.
5.19), and nasal polyps are sometimes the pre-
senting symptom of this disease (Schwachman et
al. 1962). They are estimated to occur in 6.7% of
children with cystic fibrosis. Microscopically they
resemble the adult polyp, produce similar radio-
graphic features and exhibit the same tendency to
recur (Toma and Stein 1968; Fonsman 1970).

Fig. 5.19. Plain radiograph showing changes due to benign nasal

polyps associated with mucoviscidosis.

Bone destruction in the pa.ranasal sinus associ-

ated with benign nasal polyps was described in
Fig. 5.17. Nasal polyposis. Lateral expansion of the ethmoids 1885 by Edward Woakes, although it may have
shown on a postero-anterior radiograph. been recognised as early as 1628 (Wentges 1972).
48 Inflammatory and Allergic Sinus Disease

In a series of 100 patients with nasal polyposis

reviewed by Lund and Lloyd (1983) there was evi-
dence of expansion of the ethmoids on plain radio-
graphs in 20 cases (19 bilateral and 1 unilateral).
The changes were seen most often in the younger
patients in whom the onset of symptoms occurred
below the age of 30 years. In addition in the same
group, 11 patients were observed in whom there
was mucocoele or pyocoele formation in association
with polyps. The majority of these patients had
developed the mucocoele following surgery to the
nose and sinuses, and this would appear to be the
predominant cause of mucocoele formation in these
patients rather than direct obstruction of sinus
drainage by the polyps.

CT and Magnetic Resonance

On CT nasal polyposis shows as a mass of homo-
geneous density in the ethmoid cells with erosion
Fig. 5.20. Coronal CT scan showing nasal polyposis and muco-
of the ethmoid septa (Fig. 5.18). In some patients
coele formation in the right frontal sinus (arrow). this is associated with expansion of the ethmoid
cells and mucocoele formation (Figs. 5.20, 5.21).
The bone erosion is usually completely asympto- Polyps produce a strong signal on Trweighted spin
matic without evidence of infection and is therefore echo magnetic resonance sequences, the signal
unlikely to be due to an associated osteomyelitis. intensity approaching that of retained secretion in
The mechanism is probably one of passive bone the sinuses. This makes differentiation of fluid and
erosion in a similar fashion to that produced by polyp difficult, which is not surprising because the
mucocoeles. Once the nose or sinus cavity has filled fluid secretions are almost identical in composition
with polypoid tissue any further increase in tissue to the oedema fluid in the polyp. The polyps can be
bulk is likely to lead to bone expansion, with thin- identified by both CT and magnetic resonance as a
ning of bony septa, widening of the bridge of the soft tissue mass occupying the upper part of the
nose and hypertelorism. nasal cavity and ethmoids (Fig. 5.22).

Fig. S.21a,b. Coronal CT sections showing nasal polyposis with upward expansion of the ethmoids (a) due to mucocoele formation . .
Nasal Polyposis 49

Antro-choanal Polyps
Antro-choanal polyps are readily seen on plain
radiographs when they project into the air-filled
nasopharynx. They then appear as a soft tissue
shadow with a well-defined smooth outline. They
may be seen on lateral views of the nasopharynx
or through the open mouth on an occipito-mental
projection (Fig. 5.23). Usually the maxillary antrum
and the nasal cavity on the side of the polyp are
opaque.
The diagnosis of antro-choanal polyp is important
in adolescent boys. when the condition needs to be
differentiated from juvenile angiofibroma. Surgeons

Fig. 5.22. Coronal magnetic resonance scan showing nasal

polyps in the ethmoids and upper part of the nasal cavity. Note
the typical appearance of a polyp in the cavity of the left antrum
(arrow).

Fig. 5.24. Axial magnetic resonance section showing an antro-

choanal polyp within the antrum and emerging from the ostium.

Fig. 5.23. Occipito-mental projection of a child with an antro-

choanal polyp. Note the opaque antrum with the outline of a Fig. 5.25. Same patient as Fig. 5.24. Trweighted spin echo
polyp in the nasopharynx (arrow). sequences showing the nasopharyngeal component of the polyp.
50 Inflammatory and Allergic Sinus Disease

onstrate an antro-choanal polyp directly, showing

the site of origin of the polyp in the antrum and the
characteristically high homogeneous signal on T2-
weighted spin-echo sequences (Figs. 5.24, 5.25).
Figure 5.26 shows the thickened capsule of the
nasopharyngeal component of the polyp - the result
of squamous metaplasia.

References

Cable HR. Jeans WD. Cullen FJ. Bull PD. Maw AR (1981) Com-
puterized tomography of the Caldwell-Luc cavity. J Laryngol
OtoI95:775-783
Fonsman J (1970) Mucoviscidosis and nasal polyps. Acta Oto-
laryngoI69:152-154
Lloyd GAS (1971) Axial tomography of the orbits and paranasal
sinuses. Br J RadioI44:373-381
Lloyd GAS (1975) Radiology of the orbit. WB Saunders. Phil-
adelphia
Lloyd GAS. Phelps PD (1986) Juvenile angiofibroma: imaging by
magnetic resonance. CT and conventional techniques. Clin
Otolaryngol 11 :247-259
Lund VJ. Lloyd GAS (1983) Radiological changes associated with
benign nasal polyps. J Laryngol Otol 97:503-510
Michaels L (1987) Ear. nose and throat histopathology. Springer.
Berlin Heidelberg New York
Fig. 5.26. Same patient as Fig. 5.24. The diminished signal from Schwachman H. Kulczycki LL. Mueller HL. Flake CG (1962)
the peripheral part of the mass is shown. This is due to squamous Nasal polyposis in patients with cystic fibrosis. Paediatrics
metaplasia of the epithelium. forming a hard outer capsule. 30:389-401
Skillern SR (1936) Obliterative frontal sinusitis. Arch Otolaryngol
23:267-276
are naturally reluctant to biopsy a nasopharyngeal Toma GA. Stein GE (1968) Nasal polyposis in cystic fibrosis. J
mass in this age group because of the danger of Laryngol Otol 82:265-268
Wentges RTR (1972) Edward Woakes: the history of an eponym.
profuse haemorrhage from the highly vascular J Laryngol Otol 86:501-512
angiofibroma. Diagnosis can be achieved in the first Wilson M (1976) Chronic hypertrophic polypoid rhinosinusitis.
instance by demonstrating the typical bone changes Radiology 120:609-616
associated with angiofibroma on conventional tom- Winestock DP. Bartlett PC. Sondheimer FK (1978) Benign nasal
polyps causing bone destruction in the nasal cavity and para-
ography or CT scan (Chap. 11). Magnetic resonance nasal sinuses. Laryngoscope 88:675-679
can also show diagnostic features of this tumour Woakes E (1885) Necrosing ethmoiditis and mucous polypi.
(Lloyd and Phelps 1986); alternatively it can dem- Lancet 1:619-620
6 Cysts and Mucocoeles

Cysts of the Paranasal Sinuses opacity. resembling a mucous cyst. They are often
associated with apparently normal mucosa in the
rest of the sinus and do not attain sufficient size to
Cysts of the paranasal sinuses may be classified into expand the bony sinus cavity.
two groups: intrinsic cysts arising within the sinuses
and extrinsic cysts which take origin in adjacent
structures such as the orbit or dental tissues.

Intrinsic Cysts
Mucous Retention Cysts
Mucous retention cysts are lined by columnar epi-
thelium and are the result of obstruction of the
ducts of a mucous gland. usually as an aftermath
of infection. They commonly occur in the maxillary
antrum and appear on the radiograph as a dome-
shaped. clearly demarcated opacity (Fig. 6.1) which
may sometimes grow to fill the sinus cavity. These
cysts are opaque to both transillumination and
X-rays; in contrast non-secreting cysts trans-
illuminate normally and are opaque only to X-rays.

Non-secreting Cysts
Non-secreting cysts are formed from oedematous
dehiscence of a connective tissue plane underlying
the sinus mucosa. They therefore have no epithelial
lining and are regarded as post-inflammatory in
origin; they contain fluid with a high cholesterol
content. which allows normal light trans-
illumination (Samuel and Lloyd 1978). Radi- Fig 6.1. Mucous retention cyst shown as a dome-shaped opacity
ologically they present as a smooth. dome-shaped in the floor of the maxillary antrum on conventional tomography.
52 Cysts and Mucocoeles

Cholesteatoma
In the past there has been some confusion between
cholesteatoma and cholesterol granuloma. and a
proportion of cases reported under the former diag-
nosis were clearly cholesterol granulomata (Osborn
and Wallace 1967). True cholesteatoma is a rarity
in the sinuses. and several theories have been
advanced to explain its presence. The most likely is
a metaplasia of the normal sinus mucosa to kera-
tinising stratified squamous epithelium as a result
of chronic sinus infection. Another theory is that
cholesteatoma occurs by invasion of buccal epi-
thelium via an oro-antral fistula. This would
account for the majority of these lesions being
reported in the maxillary antrum. Clinically the
patient may have pain in the cheek. deformity of
the face and displacement of the eye. Radiologically
they present with expansion of the sinus very like
that caused by a mucocoele (Fig. 6.2).

Cysts of Dental Origin

There are three common types of dental cyst which
may affect the maxillary antrum:
1. Odontogenic keratocysts. These are formed by Fig. 6.2. Expansion of the sphenoid sinus and erosion of the floor
cystic degeneration of the enamel organ before of the pituitary fossa by a cholesteatoma.
the formation of a tooth.
2. Dentigerous cysts. These are due to cystic
degeneration of the enamel organ after it has ment. The recurrence rate varies from 12% to
been formed but before it has erupted. 62.5% depending on the series (Lund 1985). The
high recurrence rate and aggressive behaviour are
3. Radicular cysts. These are formed after eruption thought to derive from the highly proliferative ker-
of a tooth and are associated with apical infec- atocyst epithelium. An example of late recurrence
tion. of this lesion has been recorded by Lund (1985).
The patient was a 64-year-old female who gave a
44-year history of problems associated with the left
Odontogenic Keratocysts upper jaw. Plain radiography and CT showed a
large multiloculated area occupying the lateral wall
Odontogenic keratocysts arise from cystic degener- of the left maxilla and adjacent orbit (Fig. 6.3).
ation of the enamel organ as described above. or This was associated with osteolysis and new bone
they may sometimes take origin from ectopic odon- formation in the areas affected. Tissue removed at
togenic epithelium. The term .odontogenic ker- surgery from the antral floor and lateral wall of the
atocyst was first used by Philipsen (1956); orbit showed numerous cysts in both soft tissue and
previously these lesions were generally called pri- bone. containing large amounts of keratin.
mordial cysts. The majority occur in the mandible
but a minority arise in the maxilla and in this
situation they may expand into the maxillary Dentigerous Cysts
antrum and buccal soft tissues (Mcivor 1972). Radi-
ologically most of them present as a well-defined Dentigerous cysts are associated with mal-
monolocular translucence in the bone with a well- development of a tooth bud. resulting in a uni~
corticated margin. but others may be multilocular. locular cyst related to the crown of an unerupted
The most striking feature of the odontogenic ker- tooth. The cyst may extend upwards to invade the
atocyst is its tendency to recur after surgical treat- maxillary antrum and nasal cavity (Fig. 6.4).
Cysts of the Paranasal Sinuses 53

b
Fig. 6.3a.b. Coronal (a) and axial (b) CT scans showing erosion of the antrum and lateral orbital wall by a recurrent odontogenic
kerotocyst. There is much reactive new bone formation on the lateral wall of the orbit (arrow).

Radicular Cysts Calcifying Odontogenic Cysts

Radicular cysts are the commonest variety of dental A calcifying odontogenic cyst is not a true cyst but
cyst. They lie directly on the apex of a tooth and is in fact a tumour with cystic change. Like other
follow apical infection. The cyst has a well-defined odontogenic tumours it may invade the maxilla.
margin and the adjacent tooth root shows loss of producing the appearance of a multiloculated cyst
lamina dura. These cysts may discharge into the on CT (Fig. 6.5).
maxillary sinus. and an antro-oral fistula may
develop when the tooth is extracted.
54 Cysts and Mucocoeles

Fig. 6.4. Dentigerous cyst shown on conventional tomography.

The cyst has expanded upwards into the maxillary antrum (black
arrow). An unerupted tooth (white arrow) is seen associated with Fig. 6.5a,b. Coronal (a) and axial (b) CT scans of a calcifying
the cyst and lying within the nasal cavity. odontogenic cyst. The cyst appears as a huge multilocular expan-
sion of the maxillary antrum.

Miscellaneous Cysts
radiographs they are best demonstrated on occlusal
Globulo-Maxillary Cysts films, but may be better shown by conventional
tomography or coronal CT.
Globulo-maxillary cysts are the most common type
of fissural cyst. They are of developmental origin
and represent cystic inclusions from a failure of Dermoid Cysts
complete fusion between the globular and maxillary
processes of the maxilla. They appear in the canine Dermoid cysts are rare in the paranasal sinuses and
fossa and as they grow they erode the hard palate generally occur as a secondary extension from a
and invade the maxillary antrum. Their charac- cyst of the nose or orbit (Figs. 6.6, 6.7 and 6.8).
teristic feature on the radiograph is a separation of The radiological features are variable depending
the roots of the canine and incisor teeth. upon the location of the cyst, but generally they
show an expanding cystic lesion which, in the bones
forming the orbit, is characterised by a well-defined,
Incisor Canal Cysts slightly sclerotic edge. From the orbit the cyst may
displace and erode the bony wall of the adjacent
Incisor canal cysts are also designated as fissural sinus. Dermoids have a diagnostic feature on CT:
cysts and result from failure of the two halves of the approximately two thirds of them will show areas
premaxilla to fuse. They lie in the mid-line and of negative attenuation due to the presence of a
cause enlargement of the incisive canal. On plain loculus of fat or oil within the cyst.
Miscellaneous Cysts ss

Fig. 6.6. Dermoid cyst. Axial cr scan showing a huge indentation

into the ethmoid labyrinth.

Fig. 6.7. Same patient as Fig. 6.7. On magnetic resonance scan

there is strong signal from the tumour on T,-weighted spin echo
sequences.

Hydatid Cysts
One example of a hydatid cyst has been seen in the
nasal cavity (Fig. 6.9).

Mucocoeles
Cyst-like expansions of the paranasal sinuses have
been recognised for over 160 years (Evans 1981), Fig. 6.8a,b. Dermoid cyst. Tt-weighted spin echo sequence (a)
the term mucocoele first being used to describe them and sagittal inversion recovery sequence (b) both show moderate
by Rollet (1896). Mucocoeles are defined as expan- to low signal. In this case the dermoid did not show evidence of
fat on magnetic resonance scan.
sHe lesions occurring within the cavity of a sinus,
containing mucoid secretions and limited by the
56 Cysts and Mucocoeles

included osteomata and maxillary sinus surgery for

cysts; and those in which no aetiological factor
could be determined. This last group was the largest.
representing 29% of the total.

Frontal Sinus Mucocoeles

The classical radiographic appearance of a frontal
sinus mucocoele is that of an expanded sinus
showing loss of the scalloped margin and an overall
loss of translucence on the affected side (Fig. 6.10).
Other features include depression or erosion of the
supraorbital ridge and extension across the mid-line
through the septum to the opposite frontal sinus
(Fig. 6.11). Loss of translucence is not. however. an
invariable feature. While it is true that in most
patients the affected sinus is more opaque than
normal. due to displacement of the air content and

Fig. 6.9. Conventional tomogram showing a hydatid cyst in the

nasal cavity.

lining membrane (Canalis et al. 1978). They are

locally destructive and cause pressure erosion and
expansion of the bony walls. encroaching upon and
displacing adjacent structures. They occur most fre- Fig. 6.10. Frontal sinus mucocoele. Expansion with loss of scal-
quently in the frontal sinus but may arise in the loped edge. loss of translucence and erosion of the supraorbital
ethmoids and rarely in the sphenoid sinus or maxil- ridge. can be seen on the radiograph.
lary antrum. The relative frequency of mucocoele
formation is illustrated by the author's series seen
over a 20-year period: there were 91 frontal sinus
mucocoeles. 27 ethmoid. 11 sphenoid. and 5 in the
maxillary antrum.
Mucocoeles may result from infection; trauma.
including bullet wounds and previous surgery;
polyps (Lund and Lloyd 1983); or tumours. benign
and malignant. All of these may affect the normal
drainage ofthe sinus concerned. In some cases there
is histological evidence of an increase in the number
of secretory cells in the lining epithelium. and
hypersecretion of mucus may then be a con-
tributory causative factor. Lund (1987). in review-
ing 80 cases of mucocoeles treated surgically.
identified seven groups according to their aetiology.
These included direct sinus trauma involving frac- Fig. 6.11. Frontal sinus mucocoele. There is depression of the
ture of the facial bone; nasal polyps untreated or supraorbital ridge and extension of the mucocoele across the
removed surgically; a miscellaneous group which septum to the opposite frontal sinus.
Mucocoeles 57

Fig. 6.12. Frontal sinus mucocoele. Mucocoele formation (arrow)

has produced hypertranslucence.

fluid accumulation, in others it may appear more

radiolucent in some projections iflocal bone erosion
is sufficient to produce an image that is less dense
overall (Fig. 6.12).
Another inconstant feature is loss of the scalloped
margin of the sinus walls. This occurs when the
vertical section of the sinus is involved in the expan-
sion, but will not be seen when only the horizontal
part is expanded - as it was in nearly 25% of the
patients in the author's series (Fig. 6.13). This group
of patients present with proptosis and downward
displacement of the eye, and are usually seen
initially by the ophthalmologist. The mucocoele can
be missed on plain radiographs when the full classi-
cal picture of a frontal sinus mucocoele is not
present. An important observation is the downward Fig. 6.14a,h. Large fronto-ethmoidal mucocoele demonstrated
on coronal (a) and axial (b) CT scans.
displacement ofthe supraorbital ridge. CT scan may
be necessary to make the diagnosis (Price and Dan-
ziger 1980), as this allows both the bone changes 6.14,6.15); but these expansions affecting the roof
and the soft tissue expansion to be appreciated (Figs. of the orbit are now optimally demonstrated by
magnetic resonance studies (Fig. 6.16). The import-
ance of accurate pre-operative assessment of this
type of mucocoele cannot be overemphasised; it is
essential that all loculi of the expansion are recog-
nised by the surgeon and drained adequately, since
this variety of mucocoele is very prone to recur.

Ethmoid Mucocoeles
Ethmoid mucocoeles are predominantly anterior in
location: in the author's series 25 out of 27 were
anterior and only 2 posterior. Most posterior muco-
coeles are in fact part of a generalised expansion of
both the sphenoid sinus and posterior ethmoid
Fig. 6.13. Frontal sinus mucocoele. There is expansion of the cells - a result of their common drainage into the
horizontal section of the sinus cavity (arrow) but the scalloped superior meatus of the nose, and presumably a
outline of the vertical part of the sinus is preserved. common obstructing agent.
58 Cysts and Mucocoeles

Fig. 6.15. Frontal sinus mucocoele. Coronal CT scan showing

expansion of the horizontal part of the frontal sinus with down-
ward displacement of the globe.

Mucocoeles arising from the frontal sinus are not

as a rule difficult to recognise radiologically, but
those derived from the anterior ethmoid cells may
be surprisingly difficult to detect on standard sinus.
views. They are usually more obvious clinically
than radiologically, over 90% presenting with a
mass at the medial canthus in addition to proptosis Fig. 6.16. Sagittal magnetic resonance scan showing a muco-
and lateral displacement of the eyeball. Some coele in the roof of the orbit derived from the horizontal part of
patients may present to the ophthalmologist with the frontal sinus.

Fig. 6.17. Ethmoid mucocoele. The edge of the ethmoid expansion is shown as a fine bony rim (arrow).
Mucocoeles 59

Fig. 6.18. Dacryocystogram of a right ethmoid mucocoele causing extrinsic obstruction and displacement of the lacrimal sac.

epiphora if the expansion impinges on the lacrimal the orbit partly covering the expansion (Fig. 6.17).
drainage apparatus at an early stage. Loss of trans- Obstruction with forward and lateral displacement
lucence in the affected ethmoid cells is the change of the lacrimal sac and canaliculi may be dem-
most often observed on plain radiographs but is onstrated on dacryocystography (Fig. 6.18), but
entirely non-specific. Other patients may show loss axial CT (Fig. 6.19) and axial magnetic resonance
of the vertical line forming the anterior component scans show the lesion optimally (Figs. 6.20, 6,21).
of the medial orbital wall (Lloyd et al. 1974), and
in some a bony rim may be demonstrated within

Fig. 6.20. Same patient as Fig. 6.19. On magnetic resonance

scan the mucocoele gives a strong signal on a T2 -weighted spin
Fig. 6.19. Anterior ethmoid mucocoele shown on axial CT scan. echo sequence.
60 Cysts and Mucocoeles

Sphenoid Mucocoeles
Under the heading of sphenoid mucocoeles are
included expansions of the posterior ethmoid cells in
addition to sphenoid expansions; they are probably
better designated as spheno-ethmoidal mucocoeles.
Imaging techniques playa key role in the diagnosis
of this condition and it is important that it be recog-
nised by the radiologist at an early stage and dealt
with surgically before vision is seriously compro-
mised. Because of the proximity of the sphenoid
sinus to the optic nerve, cavernous sinus and ocular
motor nerves, mucocoele expansion of the sinus
results in symptoms due to involvement of these
structures. The patient commonly presents with
headache combined with eye symptoms such as
blurred vision or diplopia. On plain radiographs
sphenoid mucocoeles are liable to be misdiagnosed
as pituitary tumours, or as nasopharyngeal car-
cinoma invading the sphenoid; in either case this
may result in inappropriate treatment.
In general the radiological features become more
emphatic as the lesion expands the sphenoid sinus.
Early in the course of the disease the changes may
be limited to the sinus itself (Minagi et al. 1972)
and may consist only of opacification of one or both
Fig. 6.21. Same patient as Figs. 6.19 and 6.20. On sagittal
sphenoid sinuses. At this stage certain radiological
magnetic resonance scan there is a characteristic low signal on diagnosis is not always possible, but as pressure
the T,-weighted inversion recovery sequence. within the sinus continues, an expansion of the

Fig. 6.22. Sphenoid mucocoele. Expansion of the sphenoid sinus with elevation of the floor of the pituitary fossa.
Mucocoeles 61

Fig. 6.23. Erosion of the floor of the pituitary fossa caused by a

sphenoid mucocoele.

Fig. 6.24. Re-formation of the sella floor following drainage of

the sphenoid mucocoele.

cavity ensues with elevation (Fig. 6.22) or bone

erosion of the floor of the pituitary fossa on the
lateral skull film. This change may be reversible
after drainage of the mucocoele (Figs. 6.23, 6.24).
Progressive expansion of the sinus will also cause
erosion of the medial wall of the optic canal. and
elevation of the planum sphenoidale (Fig. 6.25).
Another sign may be present if the posterior
ethmoid cells are involved in the expansion: the
oblique line which forms the lateral boundary of
the posterior ethmoid cells on the occipito-frontal
projection may be lost as the posterior ethmoid cells
expand with the sphenoid sinus.
Once the diagnosis is suspected then CT or mag-
netic resonance studies should be undertaken (Fig.
6.26). Takahashi et al. (1973) described a diag-
nostic feature on conventional tomography which
can also be shown on CT (Fig. 6.27a-c). They
reported the presence of multiple cyst-like expan-
sions, which appeared to be intercommunicating
and connected to the expanded sinus. These result
from expansion of compartments of the sinus or
aberrant pneumatisations participating in the
general expansion of the main sinus cavity. Mag-
netic resonance has now replaced CT as the method
of choice for identifying sphenoid mucocoeles. The
recognition of retained secretions in the sinus is
more easily demonstrated and discrimination
between mucocoele and any associated tumour or
Fig. 6.25. Sphenoid mucocoele showing elevation ofthe planum polyps is more obvious (see below) (Figs. 6.28,
sphenoidale (arrow). 6.29).
62 Cysts and Mucocoeles

Fig. 6.26a.b. Examples of sphenoid mucocoeles shown on axial

CT scan.
Fig. 6.27a-<:. Sphenoid mucocoele showing expansion of a
loculus of the sinus within the cavity of the maxillary antrum.
Mucocoeles 63

Mucocoeles of the Maxillary Antrum

The maxillary antrum is the least common site
in the sinuses for mucocoele formation. and the
majority of expansions of the antrum are due to
other causes such as odontogenic cysts or chol-
esterol granuloma. Clinically the symptoms are due
to sinus expansion into the nose, mouth and orbit.
resulting in upward displacement of the eye. prop-
tosis and swelling of the cheek over the affected side.
On plain radiographs the sinus is invariably opaque
and expanded (Fig. 6.30). encroaching upon the
ethmoid cells. As in the other sinuses enlargement
with good preservation of the bulging walls is the
clue to the diagnosis. but in some projections the
changes may look very like early malignancy. CT
or conventional tomography is needed to confirm
the bone expansion and magnetic resonance studies
will indicate the fluid content of the expanded sinus
(Figs. 6.31. 6.32).

Magnetic Resonance and CT

Magnetic resonance is now the imaging method of
Fig. 6.28. Coronal magnetic resonance scan of a mucocoele ofthe choice for assessing mucocoele formation. both for
sphenoid sinus secondary to nasal polyposis. Note the elevation of primary mucocoeles and secondary mucocoeles
the planum sphenoidale. associated with sinus tumours. benign or malig-

Fig. 6.29. Sagittal magnetic resonance scan of a sphenoid mucocoele (arrows) secondary to an angiofibroma.
64 Cysts and Mucocoeles

Fig. 6.30. Mucocoele of the maxillary antrum demonstrated on plain radiograph.

nant. Secondary mucocoele has been shown to be

far more common at pre-operative assessment since
the use of this technique (Figs. 2.28, 6.29). The
strong signal shown by retained secretion in the
sinuses on Trweighted spin echo sequences makes
differentiation between tumour and secondary
mucocoele much easier than by CT scan.

Fig. 6.32. Same patient as Fig. 6.31. On magnetic resonance

Fig. 6.31. Coronal CT scan showing an infected mucocoele or scan the high homogeneous signal on Trweighted spin echo
pyocoele. sequence confirms the presence of fluid in the sinus.
Mucocoeles 65

Fig. 6.33. Frontal sinus pyocoele showing ring enhancement on cr scan after intravenous contrast (arrows).

Fig. 6.34. Magnetic resonance scan showing high signal on axial inversion recovery sequence from loculi of a frontal sinus mucocoele
(arrows) which contained altered blood.

Theoretically with the latter technique muco- diagnostic on CT is the ring enhancement seen after
coeles should show low attenuation on the Houns- intravenous contrast, which indicates an infected
field scale, without change after contrast admin- mucocoele or pyocoele (Fig. 6.33).
istration. However some mucocoeles show initial As outlined in Chap. 2 the signal characteristics
attenuation values well into the tumour range, so of mucocoeles on magnetic resonance differ if there
that accurate discrimination between cystic and has been previous surgical intervention and blood
solid lesions in the sinuses has not always been contamination of the mucus or mucopus. The
possible from the attenuation values alone. untouched mucocoele produces very low signal on
Very often on CT the diagnosis is better made by inversion recovery sequences and high signal on
recognising the classical features of a mucocoele, T2-weighted spin echo sequences (Figs. 6.20, 6.21).
namely an overall rounded expansion with some In contrast a mucocoele containing blood break-
preservation of the sinus boundaries, as opposed to down products with released methaemoglobin will
destruction of bone in situ - the hallmark of produce strong signal on both sequences (Figs.
malignancy in the sinuses. One feature which is 6.34,6.35).
66 Cysts and Mucocoeles

Fig. 6.35. Same patient as Fig. 6.34. Frontal sinus mucocoele

giving a high signal on T2-weighted spin echo sequence.

Fig. 6.36. "Blistering" . or pneumosinus dilatans. affecting the ~

sphenoid sinus shown on coronal hypocycloidal tomography.
The sinus cavity is elevated and drawn towards the side of a
foraminal optic nerve meningioma (arrow).

Fig. 6.37. Pneumosinus dilatans due to an extradural meningioma. The macroradiograph shows local dilatation of the right fronto-
ethmoidal air cells.
Pneumosinus Dilatans 67

Pneumosinus Dilatans

Pneumosinus dilatans refers to an abnormal dila-

tation of a local part of the paranasal sinuses which
contains air only and is lined by normal epithelium.
The change is most commonly observed when it
affects the sphenoid sinus as a response to a local
intracranial meningioma of the tuberculum sellae
or planum sphenoidale (Fig. 6.36). The condition
was first described by Benj amins (1918) and was
extensively reviewed by Lombardi (1967). More
recently the change known as "blistering" of the
planum sphenoidale has been described by Wiggli
and Oberson (1975) and the same condition
(referred to as sphenoid pneumosinus dilatans)
described in three patients by Hirst et al. (1982).
Pneumosinus dilatans affecting the sinus walls
forming the boundaries of the orbit is less common.
Recently six such cases have been described by
Lloyd (1985). All six patients presented with a
slowly progressive proptosis over a period of months
or years. Female patients predominated in a 5: 1 Fig. 6.39. Coronal tomogram shOWing fronto-ethmoidal dila-
ratio with an age range of 15-28 years and an tation due to an adjacent meningioma.
average age of 20.7 years. In four patients the
ethmoid cells showed unilateral expansion and in
three, part of the frontal sinus was also involved
(Figs. 6.37, 6.38). In two patients the expansion
involved both maxillary antra. Biopsy showed the
pneumosinus dilatans to be caused by a men-
ingioma adjacent to the affected sinuses in three
patients (Figs. 6.39-6.42); in the remaining three

Fig. 6.40. Dilatation ofthe ethmoid cells (arrow) shown on plain

radiograph.

it was associated with fibro-osseous disease of the

sinus walls (Figs. 6.43, 6.44, 6.45).
Before making the diagnosis ofpneumosinus dila-
tans it is important that the radiologist should keep
in mind the variations in the pneumatisation of
Fig. 6.38. Coronal CT scan showing dilatation of the right fronto- the sinuses which can occur normally or in some
ethmoidal air cells (arrow) due to libro-osseous disease. congenital and acquired conditions (see Chap. 1).
68 Cysts and Mucocoeles

Fig. 6.41. Same patient as Fig. 6.40. Axial CT scan shows the soft tissue mass of a mengioma (arrow) adjacent to the dilated ethmoid
cells.

a b

Fig. 6.42. a Axial CT scan showing recurrent sheath meningioma in the orbit. The presence of the tumour has expanded the orbit
and caused an inward bowing of the medial orbital wall (arrow). b Further scan 4 years after exenteration of the orbit. Residual
meningioma is still present in the orbit and has now provoked a hyperostosis of the medial orbital wall with dilatation of the ethmoid
cells locally (arrow). Note the stretching of the ethmoid septa accompanying the sinus dilatation.
Pneumosinus Dilatans 69

Fig. 6.43. a Axial CT scan showing bony thickening due to fibrous dysplasia of the anterior walls of the maxillary antra. b Coronal
CT scan showing bilateral fibrous dysplasia with encroachment into both orbits by thickened bone and dilated maxillary antra.

Confirmation of this change can only be made at evidence has been found in the literature. Various
surgical decompression, when the criteria for diag- possible explanations have been advanced. Sugita
nosis laid down by Lombardi (1967) need to be et al. (1977) suggest a valve-like obstructive mech-
fulfilled: namely a dilated sinus lined with macro- anism of the sinus. Hirst et al. (1982) suggest either
scopically normal epithelium and containing air a congenital abnormality or the stimulating effect
only. of a local meningioma, causing the bone to bulge.
The possible reasons for the sinus dilatation The young age of the patients would support a
remain speculative and no relevant experimental developmental anomaly, probably the result of an
 70 Cysts and Mucocoeles

a b
Fig. 6.44. a Coronal CT scan showing upward dilatation (arrows) of both maxillary antra. b Enlarged view of the antrum showing
bony thickening due to fibrous dysplasia (arrow).

Fig. 6.45. Same patient as Fig. 6.44. Axial CT scan showing

irregular dilatation of both maxillary antra posteriorly.
associated with bilateral bony thickening (arrows).

aberration of bone growth and remodelling pro- obvious. but the hyperostosis produced by a men-
voked by the fibro-osseous disease or meningioma. ingioma may be minimal and the presence of pneu-
leading to a weakening of the wall and expansion mosinus dilatans should alert the radiologist to the
of the sinus. The practical consideration for the possibility of an occult meningioma. It is important.
radiologist is to recognise this condition and to therefore. that these patients should be examined
appreciate the underlying causes. The changes of by soft tissue imaging techniques - either CT or
fibro-osseous disease in the sinuses are usually magnetic resonance.
Pneumosinus Dilatans 71

McIvor J (1972) The radiological features of odontogenic ker-

References atocysts. Br J Oral Surg 10:116-125
Minagi H. Margolis MT. Newton TH (1972) Tomography in
diagnosis of sphenoid sinus mucocoele. Am J Roentgenol
115:587-595
Benjamin CE (1918) Pneumosinus frontalis dilatans. Acta Osborn DA. Wallace M (1967) Carcinoma of the frontal sinus
OtolaryngoI1:412-422 associated with epidermoid cholesteatoma J Laryngol Otol
Canalis RF. Zajtchuk JT. Jenkins HA (1978) Ethmoidal muco- 81:1021-1032
coeIes. Arch Otolaryngol 104:286-291 Philipsen HP (1956) Om Keratocyster (Kolesteatomer) i
Evans C (1981) The aetiology and treatment of fronto-ethmoidal Koeberne. Tanglaegebladet 60:963-970
mucocoeles. J Laryngol Otol 95:361-375 Price m. Danziger A (1980) Computerised tomographic findings
Hirst LW. Miller MR. Hodges FJ. Corbett J. Thomson S (1982) in mucocoeles of the frontal and ethmoid sinuses. Clin Radiol
Sphenoid pneumosinus dilatans. A sign of meningioma orig- 31:169-174
inating in the optic canal. Neuroradiology 22:207-210 Rollet M (1896) Mucocoele de I'angle superointerne des orbites.
lloyd GAS (1985) Orbital pneumosinus dilatans. Clin Radiol Lyon Med 81:573-575
36:381-386 Samuel E. lloyd GAS (1978) Clinical radiology of the ear. nose
lloyd GAS. Bartrum CI. Stanley P (1974) Ethmoid mucocoeles. and throat. HK Lewis. London
Br J Radiol 47:646-651 Sugita K. Hirota T. Iguchi I (1977) Transient amaurosis under
Lombardi G (1967) Radiology in neuro-ophthalmology. Williams decreased atmospheric pressure with sphenoid sinus dysplasia.
and Wilkins. Baltimore J Neurosurg 46:111-114
Lund VJ (1985) Odontogenic keratocyst of the maxilla: a case Takahashi M. Jingu K. Makayama T (1973) Roentgenologic
report. Br J Oral Maxillofac Surg 23:210-215 appearances of spheno-ethmoidal mucocoele. Neuroradiology
Lund VJ (1987) Anatomical considerations in the aetiology of 6:45-53
fronto-ethmoidal mucocoeles. Rhinology 23:83-88 Wiggli Q. Oberson R (1975) Pneumosinus dilatans and hyper-
Lund VJ. lloyd GAS (1983) Radiological changes associated with ostosis: early signs of meningiomas on the anterior chiasmatic
benign nasal polyps. J Laryngol Otol 97:503-510 angle. Neuroradiology 8:217-221
7 Granulomata of the Nose and Paranasal
Sinuses

Mid-facial Granuloma Syndrome found in the lungs and spleen and in the viscera. In
general the bone destruction in the nose and sinuses
is never as marked in Wegener's granuloma as it is
Friedmann (1964) has classified the non-healing in mid-line (Stewart's) granuloma.
granulomata of the nose into two basic types: lethal
mid-line granuloma (Stewart 1933) and Wegener's
granuloma (Wegener 1936, 1939). Mid-line Lethal Granuloma
Mid-line lethal granuloma is less common than
Wegener's Granuloma Wegener's granuloma and has a broadly based age
distribution ranging from 15 to 80 years. The con-
Wegener's granulomatosis is a multi-system disease dition starts as an indurated swelling of some part
characterised by necrotising granulomata of the of the nose such as the vestibule, septum or more
upper and lower respiratory tract together with a rarely the turbinates. Ulcerations spread inexor-
glomerulonephritis and systemic vasculitis (Wolff et ably, destroying the soft tissues, cartilage and bone,
al. 1974). It is encountered most commonly in the and involving the hard palate and eventually the
fourth and fifth decades but may also be found in pharynx. Death may. ensue from cachexia, haem-
young subjects. The patient presents with con- orrhage or intercurrent infection. Histologically the
stitutional symptoms: pyrexia, loss of weight and lesions are characterised by non-specific granu-
raised sedimentation rate. These symptoms often lation tissue with a dense accumulation of ple-
overshadow a nasal discharge and sinusitis, which omorphic cells in the affected tissues and as a rule
is often of long duration and may be indicative of no vasculitis. The condition differs from Wegener's
the prodromal stage of the disease. Examination granuloma in that the disease is localised and the
may reveal a crusted granularity of the mucosa of mid-facial destruction is the most prominent
the nasal septum and turbinates, with ulceration feature, with severe mutilation and bone necrosis.
and bone destruction. Ulcers may also develop in The intense upper airway destruction seen in
the mouth and pharynx (McKinnon 1970), and patients with this condition is rarely if ever found in
atypical cases may present as rheumatoid arthritis Wegener's granuloma (Kornblut and Fauci 1982).
(Pritchard and Gow 1 976).
Histologically a necrotising arteritis is an essen-
tial component of the microscopy. The vessel walls Radiological Features
are infiltrated by acute inflammatory cells and show
partial fibrinoid necrosis and giant cell formation. Milford et al. (1986) have reported the radiological
The kidneys show a focal glomerulitis and vasculitis findings in 27 patients with mid-facial granuloma:
whilst vasculitis and granuloma formation may be there were 20 patients with Wegener's granuloma
74 Granulomata of the Nose and Paranasal Sinuses

Fig. 7.1. Mid-line lethal granuloma. Massive destruction ofthe hard palate. nasal bones and ethmoid labyrinth can be seen.

and 7 with mid-line lethal granuloma. The majority Magnetic Resonance and CT
of patients with Wegener's granuloma showed
either no abnormality on the plain radiograph or A combination of magnetic resonance and CT pro-
non-specific changes; evidence of even minimal vides the most comprehensive assessment of the
bone destruction could only be seen in 25%. In mid-facial granuloma syndrome. CT, using wide
Wegener's granuloma some form of tomography is window widths, provides optimum demonstration
needed to show the bone changes. In contrast 6 of the bone destruction in the nose and sinuses
of the 7 patients with lethal mid-line granuloma (Figs. 7.2 and 7.3), besides showing the state of
showed obvious bone erosion on initial plain radio- the soft tissues and orbital invasion, when present
graphic examination (Fig. 7.1). (Figs. 7.2, 7.3 and 7.4).
These authors concluded that the difference in Three patients with mid-facial granuloma syn-
the radiological pattern in Wegener's and lethal drome have been examined by magnetic resonance
mid-line granuloma would seem to be one of degree. imaging. In a single example of a patient with lethal
In Wegener's granuloma the systemic nature of the mid-line granuloma the technique made little con-
disease allows little time for bone destruction of the tribution to the diagnosis: the changes were almost
nose and bony walls of the sinuses. The condition entirely confined to bone and were better dem-
is either arrested by treatment or, if this is ineffect- onstrated by CT (Fig. 7.2).
ive, the patient dies of its systemic effects. On the Two patients with Wegener's granuloma have
other hand the localised nature of lethal mid-line also been examined by magnetic resonance. The
granuloma allows progressive bone erosion over a first was a long-term survivor of the condition, the
period of years. diagnosis haVing been established for 16 years. The
The essential feature of both diseases is the pres- magnetic resonance scan was dominated by a
ence of bone destruction in the nose and sinuses massive central bone necrosis in the nose and
without a large soft tissue mass as in malignant sinuses, interpretation being complicated by pre-
neoplasia: and if bone destruction is easily recog- vious orbital decompression surgery. The resultant
nised on plain radiographs the condition is likely to cavity was lined by granulations giving a strong
be due to the lethal mid-line granuloma syndrome Signal on Trweighted sequences (Fig. 7.5). This
rather than Wegener's granuloma. patient also had massive infiltration of both orbits.
Mid-Facial Granuloma Syndrome 75

Fig. 7.2. Mid-line lethal granuloma. Axial CT scan showing total

destruction of the ethmoid cells on the right side (the patient
presented with right-sided proptosis). There was also infiltration
of the medial orbit by the granuloma (arrow) .

~
Fig. 7.3a, b. Same patient as Fig. 7.2. Massive bone destruction
in the right side of the nose. ethmoids and maxillary antrum can
be seen.

Fig. 7.4. Wegener's granuloma. There is massive bilateral orbital

infiltration. At decompression surgery the tissue was shown to
be densely fibrotic. Note the destruction of the posterior ethmoid
septa.
76 Granulomata of the Nose and Paranasal Sinuses

Fig. 7.5. Wegener's granuloma. On magnetic resonance scan T2-weighted spin echo sequences on axial section showed a massive
central cavity in the nose and sinuses lined by irregular inflammatory tissue giving high signal.

At decompression this was shown to be due to

dense fibrosis. The signal characteristics were quite
different from those of the nose and sinus granu-
lation tissue, giving a low signal on both T1- and Tz-
weighted spin echo sequences (Fig. 7.6). The second
patient with Wegener's granuloma produced
exactly similar magnetic resonance changes: there
was a high-intensity signal on Tz-weighted spin
echo sequences from the granulations in the nose
and sinuses (Fig. 7.7) and a hypo intense signal from
the tissue infiltrating the left orbit on both spin echo

Fig. 7.7. Wegener's granuloma. On axial magnetic resonance

Fig. 7.6. Same patient as Fig. 7.5. On sagittal magnetic resonance scan the T2-weighted spin echo sequence showed high signal
scan the tissue infiltrating the orbit (arrow) showed low signal from the granulomatous tissue lining the maxillary antra. The
on both T,- and T,-weighted spin echo sequences. This is charac- irregularity of the inflammatory tissue is characteristic of the
teristic of dense fibrous tissue from whatever cause. necrotising granulomata.
Mid-Facial Granuloma Syndrome 77

and inversion recovery sequences. This was again

due to the presence of dense fibrous tissue as
opposed to the more active granulomatous process
in the mucosa of the nose and sinuses.

Sarcoidosis of the Nose and Sinuses

Sarcoidosis is a generalised disease which affects

many organs and the generalised condition may be
associated with lesions of the nose or paranasal
sinuses. Geraint James et al. (1982). in a series of
818 patients with histologically confirmed multi-
system sarcoidosis. found that 6% had upper res-
piratory tract sarcoid involving the nose and/or the
paranasal sinuses. In this situation the disease is
commonly associated with lupus pernio and other
manifestations of chronic fibrotic sarcoidosis. The
nasal mucosa of the septum and inferior turbinates
is most commonly involved and the microscopical
picture is that of non-caseating epithelioid
tubercles. In some patients there may be polypoid
hypertrophy of the mucosa causing nasal obstruc-
tion and in a minority the paranasal sinuses are
also involved.
Fig. 7.9. Nasal sarcoid. There is soft tissue swelling with osteolysis
of the tip of the nasal bones (arrow).

Radiological Features
In the nasal bones sarcoid produces alterations in
the bone texture (Fig. 7.8). Scattered areas ofpunc-
tate osteolysis or zones of frank destruction may
be seen (Curtis 1964) (Fig. 7.9). In longstanding
involvement a wide-meshed trabecular pattern may
form due to the presence of granulomata within the
bone. and in such lesions the suture lines may
disappear (GeraintJames et al. 1982).
The changes recorded in the paranasal sinuses
are less definite and are usually non-specific. such
as mucosal thickening or clouding of one or more
of the sinuses. sometimes with a soft tissue mass in
the sinus or nasal cavity. Bone destruction has also
been observed. however (Bordley and Proctor 1942;
Dodd and Bao-Shan Jing 1977).

Tuberculosis
Fig. 7.8. Typical features of nasal sarcoid affecting the nasal
bones: a soft tissue mass associated with typical "lacework" Tuberculosis of the nose may be cutaneous or
rarefaction. mucosal. The former represents lupus vulgaris. a
78 Granulomata of the Nose and Paranasal Sinuses

tuberculous infection of the skin which may extend Cholesterol Granuloma

into the nasal vestibule. The mucosal form appears
in the nose as a small lobulated mass or granulation
on the mucosa of the cartilaginous part ofthe nasal In the head and neck cholesterol granuloma is most
septum; or it may affect the mucosal lining of the frequently found in the mastoid antrum and middle
adjacent maxillary antra. The condition is usually ear following haemorrhage, but it has also been
associated with pulmonary tuberculosis. The lining described in other parts of the skull, including the
membrane is affected first, with pofypoid thickening maxillary antrum (Graham and Michaels 1978;
followed by bone involvement. The radiographic
features consist of an opacity of the affected sinuses
with erosion and destruction of the bony walls, and
active tuberculosis in the lung fields.
The sinuses may also be involved in orbital
tuberculosis when the bony walls of the orbit are
eroded (Evans et al. 1963); and the sphenoid sinuses
may also be affected in skull base tuberculosis. Wit-
combe and Cremin (1978) have described lytic bone
changes in the sphenoid occurring in four black
children - emphasising the susceptibility of blacks
to this form of skull tuberculosis.

Syphilis

Primary syphilis is very rare in the nose and is

usually the result of inoculation resulting from
scratching or picking the nose with an infected
finger after attending an infected patient. Tertiary
lesions have been more commonly observed and
may represent either acquired or congenital disease.
The pathological lesion i~' a syphilitic gumma which
invades mucosa, periosteum or bone and may
spread from the nose to the paranasal sinuses when
the lateral nasal wall is involved. Radiologically
there is an irregular mucosal thickening in the sinus
cavity involved, accompanied by bone destruction
and some sclerotic change in the sinus walls.

Leprosy

Leprosy is an infective disease of the skin, the

mucosa of the upper respiratory tract and the per-
ipheral nervous system. The disease may infiltrate
the mucosa of the nose with nodular thickening
and may result in perforation of the cartilaginous
septum, although the nasal bone is seldom involved. Fig. 7.10a, b. CT scans showing a well-demarcated defect in the
Destruction of the nasal septum results in a saddle anterior ethmoid cells with a dearly defined bony margin. This
nose similar to that seen in congenital syphilis. was initially diagnosed as a meningo-encephalocoele.
Cholesterol Granuloma 79

Hellquist et al. 1984). In this situation it may

produce cystic expansions within the sinus cavity.
Although the majority of sinus cholesterol gran-
ulomata occur in the maxillary antrum, other
sinuses may also be involved (Figs. 7.10 and 7.11)
and a histologically identical lesion may be seen in
the frontal bone in the supero-temporal quadrant
of the orbit (Lloyd 1 986).
The condition is totally unrelated to choles-
teatoma; the latter is a post-inflammatory response
with the formation of squamous epithelium and
keratin. while cholesterol granuloma is essentially
a granulomatous change provoked by the formation
of cholesterol crystals (Fig. 7.12). Graham and
Michaels (1978). in reviewing a series of 5 antral
cholesterol granulomata. postulated that when
blood stagnates in a closed space. cholesterol comes
out of solution as crystals, which then provoke
the granulomatous process; the key factor in the
aetiology is the presence of a closed cavity con-
taining exudate and blood. This would imply that
poor drainage from the site of haemorrhage and
stagnation is an important aetiological factor.
because cholesterol is relatively insoluhle and needs
time to become dissociated from the lipoprotein
complex and be precipitated as crystals (Hellquist
Fig. 7.11a, b. Same patient as Fig. 7.10. Axial and sagittal mag- et al. 1984). This hypothesis has been supported by
netic resonance scans showing a large cystic lesion occupying the work of Hiraide et al. (1982), who were able to
the anterior ethmoid and cribriform plate area. At surgery this
was shown to be due to a cystic cholesterol granuloma. The
induce middle ear cholesterol granuloma exper-
cystic cavity was packed with cholesterol crystals, and typical imentally in monkeys by prolonged obstruction of
cholesterol clefts were shown in the lining membrane of the cyst. the eustachian tube.

Fig. 7.12. Histological appearance of cholesterol granuloma showing typical cholesterol clefts.
80 Granulomata of the Nose and Paranasal Sinuses

Nine examples of cholesterol granuloma in the

maxillary antrum have recently been reported
(Lloyd 1986). The commonest presenting symp-
toms were nasal obstruction. rhinorrhoea and facial
pain. The duration of symptoms ranged from 3
months to 10 years with an average duration of
2.75 years. In addition there was a past history of
sinus disease in 5 patients. 4 of whom had under-
gone previous surgery.
The radiological features in those antra with
proven cholesterol granuloma were of two types:
one variety in which the changes were entirely
non-specific and could not be differentiated from
inflammatory or allergic sinus disease (Fig. 7.13).
and another in which there was a more charac-
teristic appearance of a cyst-like expansion within
the sinus cavity accompanied by expansion of the
bony walls of the antrum involved (Figs. 7.14.
7.15).
Cholesterol granuloma is now readily diagnosed
by magnetic resonance scan; the presence of chol-
esterol has the effect of shortening the T 1 and T2
relaxation times. producing a strong signal on both
T 1- and Trweighted pulse sequences. The diag-
nostic feature is the very strong signal on inversion
recovery sequences (Fig. 7.16).
Fig. 7.13. Cholesterol granuloma of the maxillary antrum
showing a polypoid soft tissue mass in the antral cavity.

a b
Fig. 7.14a. b. Antral cholesterol granuloma. a Coronal CT section showing gross expansion of the maxillary antrum. b Axial CT
section showing expanded maxillary antrum and cyst-like expansion within the antral cavity.
 Cholesterol Granuloma 81

Fig. 7.15a, b. Coronal (a) and axial (b) CT sections showing an expanded loculus of the right maxillary antrum due to a cholesterol
granuloma. The patient had undergone a Caldwell-Luc procedure on the affected antrum many years previously.

a b
Fig 7.16a. b. Inversion recovery magnetic resonance sequence showing a cholesterol granuloma of the frontal bone. The two sections
show high signal due to the presence of cholesterol shortening the T 1 relaxation time - in this situation diagnostic of cholesterol
granuloma.

Curtis GT (1964) Sarcoidosis of the nasal bones. Br J Radiol

References 37:68-70
Evans RA. Schwartz JF, Chutorian AM (1963) Radiologic diag-
nosis in pediatric ophthalmology. Radiol Clin North Am
Bordley JE, Proctor DF (1942) Destructive lesion in the paranasal 1:459-495
sinuses associated with Boeck's sarcoid. Arch Otolaryngol Friedmann I (1964) Mid-line granuloma. Proc R Soc Med
36:740-742 57:289-297
Dodd GD, Bao-Shan Jing (1977) Radiology of the nose, paranasal Geraint James D. Barter S, Jash D. McKinnen DM, Carstairs LS
sinuses and nasopharynx. Williams and Wilkins, Baltimore (1982) Sarcoidosis of the upper respiratory tract (SURT). J
82 Granulomata of the Nose and Paranasal Sinuses

Laryngol Otol 96:711-718 nasal sinuses in non-healing granulomas of the nose. Clin
Graham J. Michaels L (19 78) Cholesterol granuloma of the maxil- Otolaryngol11:199-204
lary antrum. Clin Otolaryngol 3: 15 5-160 Pritchard MH. Gow PJ (1976) Wegener's granulomatosis pre-
HeIIquist H. Lundgren J. Olofsson J (1984) Cholesterol granuloma senting as rheumatoid arthritis. Proc R Soc Med 69: 501-504
of the maxillary and frontal sinuses. Otorhinolaryngology Stewart JP (1933) Progressive lethal granulomatous ulceration
46:153-158 of the nose. J Laryngol OtoI48:657-701
Hiraide F. Inouye T. Miyakogawa H (1982) Experimental chol- Wegener F (1936) Ober generalisierte. septische Gefas-
esterol granuloma. J Laryngol Otol 96:491-501 serkrankungen. Verh Dtsch Pathol Ges 29:202-212
Kornblut AD. Fauci AS (1982) Idiopathic mid-line granuloma. Wegener F (1939) Ober eine eigenartige rhinogene Gran-
Otolaryngol Clin North Am 15:685-692 ulomatos mit besonderer Beteiligung des Arteriensystems und
Lloyd GAS (1986) Cholesterol granuloma of the facial skeleton. der Nieren. Beitr pathol Anat allgem Pathol 102:36-38
Br J Radiol 59:481-485 Witcombe JB. Cremin BJ (1978) Tuberculous erosion of the
McKinnon DM (1970) Lethal mid-line granuloma of the face and sphenoid bone. Br J Radiol 51:347-350
larynx. J Laryngol Otol 84:1195-1203 Wolff SM. Fauci AS. HornRG. Dale DC (1974) Wegener's granu-
Milford CA. Drake-Lee AB. Lloyd GAS (1986) Radiology of para- lomatosis. Ann Intern Med 81:513-525
8 Mycotic Disease

Phycomycosis lation tissue. The fungus has a predilection for

invading vessels, resulting in thrombosis, infarction
and areas of necrosis (Martin et al. 1954; Carpenter
By definition phycomycosis is caused by fungi et al. 1968).
belonging to the class Phycomycetes. There are two The radiological features consist initially of an
important orders in this class: Mucorales, which opacity of the sinuses involved (Fig. 8.1) (Becker et
cause mucormycosis, and entomophthorales, al. 1968), followed by bone destruction mimicking
which give rise to entomophthorosis. There are sig- a malignant neoplasm. CT demonstrates, in
nificant clinical and histopathological differences addition to the bone changes, the exact degree of
between these two conditions. soft tissue invasion, particularly in the orbit, and
abscess formation can be shown in the frontal lobes
(Berthier et al. 1982). Vessel invasion can be demon-
Mucormycosis strated angiographically (Courey et al. 1972), with
filling defects in the arteries concerned and total
Mucormycosis appears principally in the immune- occlusion leading to embolism and brain infarction.
suppressed patient and has a particular predilection
for those with acidosis secondary to diabetes mel-
litus (Berthier et al. 1982). The disease presents Entomophthorosis
with an acute onset, the duration of symptoms
being numbered in days. The clinical patterns are Entomophthorosis differs from mucormYCOSIS In
variable but the naso-orbital-cerebral form is the that it is more likely to occur in otherwise healthy
most common. When the nasal infection has been subjects and develops more slowly, the clinical pres-
established it spreads through the sinuses to the entation being less dramatic than that of mucor-
vasculature of the conjunctiva and invades orbital mycosis. The general condition of the patient is
structures giving rise to orbital cellulitis and good and the common form of presentation is nasal
ophthalmoplegia. Subsequent spread from the obstruction together with swelling of the nose and
orbits may cause thrombosis of the cavernous sinus. cheek often of many months' duration - which
Extension to the cranial cavity may occur either contrasts sharply with the rapid progress of mucor-
via the orbital roof (Parmentier et al. 1965) or mycosis. Spread from the nose to the paranasal
cavernous sinus, or the fungus may invade the sinuses often occurs but the infection is usually
anterior fossa directly through the cribriform plate. confined to these regions. Invasion of the anterior
This gives rise to a meningo-encephalitis with fossa can occur, however. CT may show a soft tissue
necrosis of brain tissue predominantly in the inferior mass in the sinuses involved and in the nose, with
part of the frontal lobes. Histologically aseptate massive invasion of the orbit and erosion of bone
fungal hyphae are seen within inflammatory granu- and sinus walls (Fig. 8.2).
84 Mycotic Disease

a b
Fig. 8.1a. b. Mucormycosis. Axial CT sections through the antra and ethmoids showing an irregular soft tissue mass with destruction
of the medial antral wall. invasion of the pterygo-palatine fossa and early encroachment on the orbital apex.

Fig. 8.20-<. Entomophthoro ·j. . 0 /\xlal C1' section showing

fungus disease in the phenoid inus with massi\'e invasion of
the left orbit. b Coronal er section howlng the soft tissue ma s
in the phenoid inus and erosion of the plunum sphcnoidule
(arroll'). c Coronul C'I'l>CCtion through the ' phenold sinus using
wide window widths for bone detail. Generalised erosion of the
Inu walls by the fungu can be secn (arrtlws).
Aspergillosis 85

Aspergillosis an antro-choanal polyp or a tumour such as an

inverted papilloma. With further extension the
fungus may destroy the lateral wall of the antrum
The fungus Aspergillus is a common saprophyte and invade the orbital floor. In some patients the
found in soil, dust, and decaying organic matter fungus may reach the orbit via the ethmoid cells
such as fruits and grains. It may be pathogenic in or the naso-Iacrimal duct (Bahadur et al. 1983).
man, animals and birds. The usual point of entry is Intracranial extension is not uncommon from the
the nose, from where it may spread to the sinuses orbit and may result in areas of curvilinear cal-
and bronchi or disseminate into lung tissue. The cification on skull radiographs (Rudwan and Sheik
condition may occur in the immune-compromised 1976).
host or in those debilitated by other primary con- Recent publications (Glass et al. 1984; van Haake
ditions; it may also affect otherwise healthy subjects 1984) have emphasised the use of CT to dem-
with no underlying disease. Aspergillus fumigatus is onstrate aspergillosis in the paranasal sinuses. In
the species which most often causes paranasal sinus
disease; Aspergillus flavus is less common, but in
the Sudan, where the disease is endemic, it is the
predominant organism.
There are two forms of the disease: an invasive
form which behaves similarly to a malignant neo-
plasm, with bone destruction, orbital involvement
and occasional intracranial extension; and a non-
invasive form which may present much like a bac-
terial sinusitis, with rhinorrhoea, nasal congestion
and an opaque antrum on plain radiography. The
distinction between these two varieties of the
disease is not entirely clear-cut and it may well be
that the invasive form follows the non-invasive type
in some patients.
More recently McGill et al. (1980) have described
fulminant aspergillosis, a new variety of the disease
that occurs in individuals with a severely depressed
immune system and is characterised by a rapidly
progressive gangrenous mucoperiostitis with
destruction of the nasal cavity and paranasal
sinuses. Fig. 8.3. Aspergillosis. Coronal CT section shOWing a soft tissue
mass in the right antrum. right nasal cavity and ethmoids with
erosion of the medial antral wall.
Imaging Features
The maxillary antrum is the commonest site of
Aspergillus infection aahrsdorfer et al. 1979), pro-
ducing a simple opacity in the sinus on plain radi-
ography. This of course is a non-specific sign, but
when there is involvement of two adjacent sinuses
in a patient from a particular locality such as the
Sudan, or in a patient on immunosuppressive drugs,
the possibility of aspergillosis needs to be excluded.
According to Rudwan and Sheik (1976) fluid levels
in the sinuses are not seen in this condition, so this
must be a point of differentiation from bacterial
infection. Bone destruction is a common feature in
the invasive form of the disease and the antrum is
most often involved. This may result in a mass
which is continuous from the antrum into the nasal
cavity, with a clearly defined erosion of the medial Fig. 8.4. Same patient as Fig. 8.3. Axial CT section showing
wall (Fig. 8.3). At this stage the lesion may mimic diffuse calcification in the fungal mass within the antrum (arrow).
86 Mycotic Disease

Fig. 8.5. Coronal magnetic resonance scan showing aspergillosis

of the sinuses with massive invasion of both orbits.

Fig. 8.6a, b. Invasive aspergillosis. Axial (a) and sagittal (b) mag-
netic resonance scans showing both orbits massively infiltrated
by dense fibrous tissue containing microscopic calcium deposits
and Aspergillus. These T]-weighted spin echo sequences showed
low signal from the intraorbital fibrous tissue.
y

b
a

one patient examined by the author using high-

resolution CT a mass was present in the right side
of the nasal cavity continuous with a mass in the
antrum (Fig. 8.3). Within the antral mass a diffuse
calcification was shown (Fig. 8.4) which was not
appreciated on conventional tomography but was
clearly demonstrated on CT because of the inher-
ently high contrast of the latter technique. This type
of calcification is unlike that seen in any neoplastic
process and is suggestive of aspergillosis when
present.
Calcification can also be nodular and very dense
on radiography. Stammberger et al. (1984)
reported the presence of calcification in 27 patients
out of 59 with sinus aspergillosis. The calcification
Fig. 8.7. Post-operative magnetic resonance scan of the same was seen on conventional X-ray studies and was
patient as Fig. 8.6 after cranio-facial resection. The axial T2 -
weighted spin echo sequence shows very low signal from the
often of metal radiopacity. By means of light and
fibrous tissue within the orbits and high signal from the tissue electron microscopy and X-ray fluorescence analy-
lining the cranio-facial cavity. sis, these authors showed that the areas of cal-
Aspergillosis 87

Fig. 8.8. Aspergillosis. High density in the sphenoid and ethmoids

is seen on axial CT scan prior to intravenous contrast.

cification were due to the deposition of calcium

phosphate in the centre of the fungal mass.

Magnetic Resonance
Both the invasive and the non-invasive forms of
aspergillosis have been studied by magnetic res-
onance scan. The invasive form showed a mass
in the nose and paranasal sinuses with massive
invasion of both orbits (Fig. 8.5). A distinctive
feature of this case was the different spin charac-
teristics ofthe intranasal tissue and the tissue invad-
ing the orbits. The latter consisted of very hard
fibrous tissue on biopsy with microscopic evidence
of small calcium deposits. It gave a low signal on Tr
weighted spin echo sequences similar to the dense
fibrous reaction in the orbits provoked by other
conditions such as Wegener's granuloma. In com-
parison the intranasal tissue gave a fairly strong
signal (Figs. 8.6, 8.7).
A single example of the non-invasive form of
aspergillosis has also been investigated by magnetic
resonance. This presented initially as a sphenoid
sinus mucocoele and showed a generalised high Fig. 8.9a-<:. Same patient as Fig. 8.8. Axial T,-weighted spin echo
density on CT prior to intravenous contrast magnetic resonance scans showing total absence of signal from
(Fig. 8.8). The material evacuated from the sphe- the substance occupying the expanded sphenoid sinus. This was
noid and ethmoid sinuses and from the maxillary non-invasive aspergillosis with secondary mucocoele fonnation.
antrum at surgery was of a soft consistency and
brownish-black in colour and gave no signal either
on TI-weighted or Trweighted spin echo sequences
(Fig. 8.9, 8.10 and 8.11). The explanation for this cadmium and mercury) in mycotic concrements of
absence of signal is speculative. From the evidence aspergillosis, some of which may have been respon-
of the CT scan the material almost certainly had a sible for the signal void. Information derived from
high calcium content, which would contribute to nuclear magnetic resonance spectroscopy studies
the lack of signal. Stammberger et al. (1984) found suggests that tight molecular binding of hydrogen
traces of several heavy metals (silver, lead, copper, might also be a causative factor.
88 Mycotic Disease

Fig. 8.10. Same patient as Fig. 8.8. The sagittal T2-weighted spin Fig. 8.11. Same patient as Fig. 8.8. Sagittal T,-weighted spin echo
echo sequence again shows a total absence of signal from the sequence through the maxillary antrum showing fungus (arrow)
fungus within the expanded sphenoid sinus. within the antrum giving no signal.

References Jahrsdorfer RA. Ejercito VS. Johns MME. Cantrell RW. Sydnor JB
(1979) Aspergillosis of the nose and paranasal sinuses. Am J
Otolaryngoll:6-14
Martin FR. Lukeman JM. Ranson RF et al. (1954) Mucormycosis
Bahadur S, Kacker JK, D'Souza B, Chopra P (1983) Paranasal of the nervous system associated with thrombosis of the inter-
sinus aspergillosis. J Laryngol Otol 97:863-867 nal carotid artery. J Paediatr 44:437-444
Becker MH. Ngo N. Berambaum SL (1968) Mycotic infection of McGill TJ. Simpson G. Healy GB (1980) Fulminant aspergillosis
the paranasal sinuses. Radiology 90:49-51 of the nose and paranasal sinuses. A new clinical entity.
Berthier M. Palmieri O. Lylyk P. Leiguarda R (1982) Rhino- Laryngoscope 90:748-754
orbital phycomycosis complicated by cerebral abscess. Neuro- Parmentier M. Belasse E. Pirart J. Van der Haegen II (1965)
radiology 22:221-224 Mucormycose orbitaire. Arch Ophthalmol (Paris) 25:689-704
Carpenter OF. Brubaher LH. Powell RA. Valsamis MP (1968) Rudwan MA. Sheik HA (1976) Aspergilloma of the paranasal
Phycomycotic thrombosis of basilar artery. Neurology sinuses - a common cause of unilateral proptosis in Sudan.
18:807-812 Clin Radiol 27:497-502
Courey WR. New PFJ. Price DL (1972) Angiographic mani- Stammberger H. Jakse R. Beaufort F (1984) Aspergillosis of the
festations of craniofacial phycomycosis. Radiology 103:329- paranasal sinuses. X-ray diagnosis. histopathology and clinical
334 aspects. Ann Otol Rhinol Laryngol 93:251-256
Glass RBJ. Hertzanu Y. Mendelsohn DB. Posen J (1984) Para- van Haake N (1984) Aspergillosis of the paranasal sinuses. J
nasal sinus aspergillosis. J Laryngol OtoI98:199-205 Laryngol Otol 98:193-197
9 Giant Cell Lesions of the Nose and Paranasal
Sinuses

The existence of tumours or tumour-like lesions associated with hyperparathyroidism and sub-
showing multinucleate giant cells has been known periosteal bone resorption and referred to as a
for over 100 years and the giant cell tumour of brown tumour (Figs. 9.1,9.2)
bone is a well-recognised entity occurring at the 3. An active neoplasm with locally aggressive
ends of long bones and characteristically presenting activity similar to the giant cell tumour of long
in the third and fourth decades. The histological bones
similarity of this lesion to the so-called brown
tumour of hyperparathyroidism is also well known. These conditions are very similar histologically.
Giant cell tumours have also been reported in the Friedberg et al. (1969) have suggested histological
jaw and cranium since the mid-nineteenth century, differentiation based on the appearance of the
and in this situation they have generally been
regarded as behaving in a more benign manner
than those in the long bones.
In 1953 Jafl'e introduced the term giant cell
reparative granuloma to designate a rare condition
involving the jaw bones. He described it as non-
neoplastic and a separate entity from the giant cell
tumour, which he regarded as extremely rare in the
skull. Undoubtedly most of the early and even later
reports of giant cell tumour of the cranio-facial
bones were il\ fact examples of reparative granu-
loma, thus accounting for their relatively benign
behaviour (Friedmann and Osborn 1982). Some
authorities question whether true giant cell
tumours occur at all in the skull, but there would
appear to be a few authentic cases, particuiarly
those which have developed frankly malignant
characteristics (Potter and McLennan 1970; Fu and
Perzin 1974).
There are thus three entities in the nose and
sinuses presenting histologically as a dispersion of
multinucleate giant cells in a stroma of spindle cells:

1. Giant cell granuloma Fig. 9.1. Expansion of the left maxillary antrum and bone destruc-
2. A giant cell lesion identical to the above but tion due to a brown tumour.
 90 Giant Cell Lesions of the Nose and ParanasaJ Sinuses

Fig.9.2a-c. Brown tumour. a CT scan showing expansion and bone

destruction in the maxillary antrum. b CT scan showing second lesion
present in the occiput and posterior fossa. c Sub-periosteal bone resorp-
tion in the phalanges due to hyperparathyroidism. (Courtesy of Dr. P.
c Woodhead.)
Radiological Features 91

spindle cells and the distribution of giant cells: giant

cell granuloma shows cytoplasmic preponderance
in the spindle cells and the giant cells are variable
in number and show irregular dispersion; in giant
cell tumour the stroma shows nuclear pre-
ponderance and the giant cells are numerous and
evenly distributed.
In spite of their histological similarity these two
lesions behave in a different manner. Giant cell
granuloma is a benign. slow-growing lesion most
common in the second decade of life that is usually
controlled by simple curettage. Giant cell tumour
presents in the third and fourth decades and is
characterised by extremely aggressive behaviour
with invasion of neighbouring structures and a high
recurrence rate.

Radiologica] Features

The lesions may present. on plain radiographs. as

a simple opacity of the affected sinus resembling.
a localised thickening of the lining membrane.
Hlavacek and Jolma (1974) described a giant cell
tumour arising in the upper part of the frontal sinus
and presenting as a mid-line circumscribed opacity.
More often bone expansion or destruction is present.
In the maxillary sinus erosion and destruction of
the alveolar ridge may be seen (Dodd and Bao-Shan
Jing 1977) and nine out of ten patients reviewed by
Potter and McLennan (1970) in whom the sphenoid
was involved showed destruction of the sellar floor.
Seven patients with an initial histological diag-
nosis of giant cell lesion in the nose or paranasal
sinuses have been investigated radiologically. Two
of these cases were brown tumours of hyper-
parathyroidism and were diagnosed on the evidence
of the blood chemistry and bone survey. Both
occurred in the maxillary antrum (Figs. 9.1. 9.2).
Of the remaining five lesions three were localised in
the sphenoid sinus and posterior ethmoid cells. one
was situated in the nose and anterior ethmoid cells
(Fig. 9.3) and one in the maxillary antrum. All five
presented on plain radiographs as a soft tissue mass
in the sinuses - some with simple expansion of the
bony walls. as in a benign process. others with frank
bone destruction as in a malignant tumour.

Magnetic Resonance and CT

Fig. 9.3a. b. Giant cell reparative granuloma in the anterior nasal
cavity (arrow) shown on axial (a) and coronal (b) CT scans.
Five patients were investigated by CT and one by
92 Giant Cell Lesions of the Nose and Paranasal Sinuses

Fig. 9.4. Giant cell reparative granuloma in a 14-year-old male. Three axial CT sections and one coronal section show a soft tissue
mass in the sphenoid sinus with destruction of the posterior orbit and optic canal and massive upward extension into the anterior
and middle fossa.

magnetic resonance. As on plain radiography, CT expanded into the middle fossa (Fig. 9.5). The type
demonstrated either expansion or destruction of the of bony change seen on initial examination gave no
sinus walls. In one instance an enhancing mass was clue to the eventual clinical outcome; nor did it
shown on CT arising in the sphenoid sinus with allow any differentiation between giant cell
massive invasion of the anterior fossa and total tumours and granulomata. The lesion most destruc-
destruction of the adjacent optic canal (Fig. 9.4). tive of bone (Fig. 9.4) has not recurred 5 years after
The single case examined by magnetic resonance cranio-facial resection, while the lesion shown in
showed a generalised expansion of the sphenoid Fig. 9.5 has recurred twice in 2 years following
sinus by a soft tissue mass which gave high signal initial surgery and its histological appearance has
on Trweighted spin echo sequences and which become unequivocally malignant.
Magnetic Resonance and CT 93

Fig. 9.5a, b. Axial (a) and coronal (b) magnetic resonance scans showing a giant cell reparative granuloma of the sphenoid sinus.
There is a generalised expansion of the sphenoid with encroachment on the middle fossa and high signal on T,-weighted spin echo
sequences.

References Fu y, Perzin KH (1974) Non-epithelial tumours of the nasal

cavity. paranasal sinuses and nasopharynx. Cancer 33:1289-
1305
Dodd GD. Bao-Shan Jing (1977) Radiology of the nose. paranasal Hlavacek V. Jolma VH (1974) Giant cell tumours of bone in ENT
sinuses and nasopharynx. Williams and Wilkins. Baltimore organs. Acta Otolaryngol (Stockh) 77:374-380
Friedberg SA. Eisenstein R. Wallner LJ (1969) Giant cell lesions Jaffe HL (1953) Giant cell reparative granuloma. traumatic bone
involving the nasal accessory sinuses. Laryngoscope 79:763- cyst and fibrous (fibro-osseous) dysplasia ofthe jaw bones. Oral
776 Surg Oral Med Oral Pathol 6:159-175
Friedmann I. Osborn DA (1982) Pathology of granulomas and Potter GD. McLennan BL (1970) Malignant giant cell tumour of
neoplasms of the nose and paranasal sinuses. Churchill Liv- ' the sphenoid bone and its differential diagnosis. Cancer 25:
ingstone. Edinburgh 167-170
10 Epithelial Tumours

Inverted Papilloma Radiology and Imaging

The radiological features of inverted papilloma
Inverted papilloma is an uncommon epithelial derive from its usual poi~t of origin in the nasal
tumour of the nose and paranasal sinuses which has cavity - the mucosa of the middle meatus or tur-
been the subject of much interest and considerable binate. Initially, when small and confined to the
debate since it was first described by Ringertz nose, the inverted papilloma is difficult to detect
(1938). Although the earliest references to nasal on plain radiographs, often producing non-specific
papilloma were by Ward (1854) and Billroth features due to concomitant infection or allergic
(1855), Ringertz first drew attention to the "in- change in the sinuses. Alternatively the sinuses may
verted" nature of the tumour and since then show no abnormality. The nasal mass needs to be
considerable controversy has surrounded identified by pluri-directional tomography, CT scan
the nomenclature, natural history and treatment or magnetic resonance. All three techniques will
of these lesions. demonstrate the characteristic appearance of a
Inverted papillomata arise from the lateral wall mass continuous from the middle meatus into the
of the nose in the region of the middle turbinate. antrum through a well-defined bone defect at the
They are almost always unilateral and produce a level of the antral ostium (Lund and Lloyd 1984)
nasal mass with involvement of the maxillary (Figs. 10.1, 10.2 and 10.3).
antrum in the majority of patients at first pres- CT has advanced the possibilities of diagnosis in
entation. The ethmoids are also frequently affected sinus pathology by its ability to detect minor degrees
but involvement of the frontal and sphenoid sinuses of increased density not readily demonstrable by
is uncommon. On histological examination the conventional radiographic techniques. In addition
lesions present a characteristic infolded appearance to showing the extent of the soft tissue mass, areas
and are covered by alternating layers of columnar of calcification may be demonstrated within the
and squamous epithelium which has given rise to tumour (Fig. 10.4), accompanied in some patients
the alternative name of transitional papilloma. In by sclerotic bone shown either in the turbinates or
200 cases described by Friedmann and Osborn in the sinus walls adjacent to the papilloma (Figs.
(1982) the patients' ages ranged from the second 10.5, 10.6).
to the ninth decades, peaking in the sixth decade, Inverted papillomata present two aspects of clini-
with a 5: 1 male predominance. Patients present cal importance: firstly, a strong tendency to recur
clinically with nasal obstruction and discharge; the after local removal, and secondly, occasional malig-
lesions are polypoid in appearance and may be mis- nant change. Recurrence is common and may occur
taken both clinically and radiologically for an antro- in more than 30% of cases (Friedmann and Osborn
choanal polyp. 1982), with some patients having multiple recur-
96 Epithelial Tumours

Fig. 10.1. Typical appearance of inverted papilloma shown on

hypocycloidal tomography. There is a nasal mass occupying the
middle meatus and extending into the adjacent maxillary antrum Fig. 10.3. Recurrent inverted papilloma demonstrated by coronal
through a well-demarcated bone defect. magnetic resonance scan.

Fig. 10.2. Coronal CT scan showing an inverted papilloma occu- Fig. 10.4. Axial CT scan showing plaques of calcification in an
pying the right side of the nasal cavity and filling the maxillary inverted papilloma of the ethmoids.
antrum.

rences over a period of years~ Radiological assess- The problem of malignant change is somewhat
ment in cases of recurrent disease is especially confused by the simultaneous finding of inverted
difficult when there has been repeated resection papilloma and carcinoma at initial and subsequent
of tissue, and a combination of CT and magnetic histological examination. Such an observation is
resonance studies is needed in these patients. strong circumstantial evidence of malignant change
Inverted Papilloma 97

but it is not proof. However. unequivocal evidence

of malignant change provided by long-term obser-
vation and histological control has been recorded
in the literature (Kramer and Som 1935; Ringertz
1938; Mabery et al. 1965; Worgan and Hooper
1970) and there is no doubt that malignancy does
occur in a minority of patients (from 2% to 13%
depending on the series). Proof of malignant change
depends upon biopsy and at an early stage radio-
logical differentiation is not usually possible. At a
later stage. however. when the tumour becomes
frankly invasive. it will show features which are
indistinguishable from those of a squamous cell
carcinoma or adenocarcinoma in which there is
widespread bone erosion and invasion of adjacent
areas such as the infratemporal fossa. orbit or
anterior cranial fossa.
Hyams (1971). in a clinico-pathological study of
315 cases of papillomata. described thinning or
erosion of the bony sinus wall by the papillomatous
mass. but no definite invasion of bone unless there
Fig. 10.5. Axial CT scan of an inverted papilloma involving the
was associated malignancy. Other authors (Brown
ethmoids and showing sclerosis of the sinus walls and septa. 1964) have stressed the distinction between pres-
sure erosion by the benign papillomata and the
development of bone destruction with the presence
of malignant change.

a b
Fig. 10.6a. b. Axial (a) and coronal (b) CT scans of a recurrent inverted papilloma. There are changes in the anterior ethmoid cells
similar to those seen in Fig. 10.5.
98 Epithelial Tumours

Fig. 10.7. Axial high-resolution CT scan of the frontal sinuses showing linear calcification (arrow) within the soft tissue mass of an
inverted papilloma occupying the sinus cavity.

Differential Diagnosis
When a nasal mass is shown with complete opacity
of the ipsilateral maxillary antrum and erosion of
bone around the sinus opening, an inverted pap-
illoma or antro-choanal polyp are the two most
likely diagnoses. At a stage in the development
of squamous cell carcinoma, adenocarcinoma or
lymphoma, similar appearances may arise,
although there is usually more widespread bone
destruction on initial radiological examination of
patients with primary sinus malignancy. Fungus
infection must also be considered in the differential
diagnosis and may present as an antro-nasal mass
(see Chap. 8). Calcification is a common feature of
aspergillosis but is most often diffuse and unlike the
Fig. 10.8. Axial CT scan demonstrating ring-like calcification linear (Fig. 10.7), curvilinear or circular (Fig. 10.8)
(arrow) in a recurrent inverted papilloma.

a b

Fig. 10.9a,b. Axial (a) and coronal (b) CT scans shOWing an extensive inverted papilloma associated with sclerosis and deformation
of the posterior maxillary antrum (arrows).
Inverted Papilloma 99

When the patient presents the tumour is usually

widespread in the nose and paranasal sinuses, but
occaSionally a more localised neoplasm may be
seen, and if largely confined to the nasal cavity
nasal obstruction may draw early attention to it.
However, in carcinoma confined to the maxillary
antrum early symptoms of pain and swelling of the
cheek may be first diagnosed as sinusitis, and it is
not until dental symptoms or those related to orbital
spread become apparent that the true nature of the
disease is realised. Proptosis is.in fact the commonest
sign of sinus malignancy after nasal obstruction
and epistaxis (Shaw 1964).
Diagnostic imaging has a threefold part to play
in the management of these patients: at an early
stage in the development of sinus malignancy the
tumour may be inaccessible to clinical methods of
Fig. 10.10. Axial CT scan showing the soft tissue mass of a
examination and imaging studies may demonstrate
recurrent inverted papilloma with a deformed and sclerotic pos- the lesion comparatively early when surgery or
terior wall of the maxillary antrum (arrows). radiotherapy have a reasonable chance of success;
it may help to form a prognosis by establishing the
site of the tumour; and above all it is the optimum
means of determining the extent of disease prior to
calcification seen in inverted papilloma. Sclerosis treatment. In this respect the role of CT and mag-
of the sinus walls is a non-specific change most netic resonance is especially important: a com-
frequently seen in chronic sinus infection. However, bination of these techniques will show the best area
in these patients the new bone formation is dis- for biopsy of the tumour, will demonstrate the pres-
tributed evenly along the sinus walls without defor- ence of bone destruction and will delineate the
mation. The combination of bone deformity and precise outlines of the mass and its relationship to
sclerosis suggests a slow-growing tumour such as vital structures.
an inverted papilloma (Figs. 10.9, 10.10).

Plain Radiographic Changes

Malignant Epithelial Tumours In most patients both the antrum and ethmoids are
already involved at the time of first examination
Squamous Cell Carcinoma and will show complete loss oftranslucence on plain
radiographs. Bone destruction in the sinus walls
Squamous cell carcinoma is the most common may be demonstrable either by direct radiography
malignant epithelial tumour of the nose and para- or by tomography, and these changes are frequently
nasal sinuses, comprising 63% of nasal and 44% of associated with a soft tissue mass in the nasal cavity
paranasal carcinomata. Most arise in the nose or with bowing of the nasal.septum in a convexity
maxillary antrum, but when first seen the tumours away from the side of the tumour (Fig. 10.11).
are mostly naso-antro-ethmoidal in distribution Destruction of bone in situ is characteristic ofmalig-
(see Fig. 10.17). The majority of patients are male, nant tumours as opposed to the simple expansion
the male to female ratio being approximately 2: 1; of the sinus cavity seen w~th benign tumours and
and most patients are over 65 years of age at the Qlucocoeles. In the antrum the medial wall is most
time of onset of the disease. An occupational basis frequently involved by squamous cell carcinoma,
for the origin of this disease has been established in but is difficult to show on plain radiographs due to
the nickel industry, but this would appear to be the overlap of other structures. Destruction of bone in
only known aetiological agent. Histologically, as in the roof of the antrum, in the lateral recess and in
squamous carcinoma in other locations, there is a the alveolar recess is usually identifiable on plain
wide range of cellular differentiation with varying radiographs.
degrees of keratin production, but in 80%-85% of
cases the tumour is well to moderately well differ-
entiated (Michaels 1987).
100 Epithelial Tumours

Fig. 10.12. Coronal CT scan following intravenous contrast

medium, showing a carcinoma invading the anterior fossa.

Fig. 10.11. Typical appearance on plain radiography of an

advanced squamous cell carcinoma. There is a mass in the left
nasal cavity, ethmoids and antrum and displacement of the nasal
septum by the tumour (arrow).

Magnetic Resonance and CT

Magnetic resonance and CT are of particular value
in assessing several anatomical sites:
1. Extension of disease through the cribriform
plate area into the anterior cranial fossa is now best
demonstrated using sagittal magnetic resonance
scans following intravenous gadolinium DTP A. It is
also well demonstrated by direct coronal CT sections
following intravenous contrast medium. When a Fig. 10.13. Axial CT scan showing invasion of the orbit from
tumour of the paranasal sinuses has invaded the an ethmoid carcinoma. The tumour is simply displacing the
structures rather than infiltrating them. This is by far the most
anterior or middle cranial fossa it will normally common type of orbital invasion seen in both benign and malig-
enhance against the non-enhancing brain sub- nant sinus tumours.
stance (Fig. 10.12).
2. Extension of disease into the soft tissues of
the orbit is also optimally demonstrated by these ning is mandatory to show the presence and degree
techniques. There are two varieties of orbital of orbital extension.
involvement which may be demonstrated. The first 3. Evidence of invasion of the pterygo-palatine
is more common and is seen in both benign and fossa and infratemporal fossa can also be dem-
malignant sinus tumours: in this the orbital con- onstrated by these techniques. Invasion of the pos-
tents are displaced laterally without direct invasion, terior wall of the maxillary antrum is particularly
the periorbita acting as a barrier and resisting well shown on high-resolution CT. It is possible
tumour infiltration for a long time (Fig. 10.13). The to show simple expansion of the posterior wall;
second less common type of orbital invasion is a expansion with early erosion; or direct invasion
direct infiltration ofthe orbital structures by tumour of the soft tissues and musculature (Figs. 10.15,
so that the normal anatomical landmarks are oblit- 10.16). Occasionally a squamous cell carcinoma
erated (Fig. 10.14). In both varieties coronal scan- may show areas of punctate calcification on CT.
Malignant Epithelial Tumours 101

Fig. 10.14. Axial CT scan of a recurrent squamous cell carcinoma Fig. 10.16. Axial CT scan showing massive invasion of the
after maxillectomy. The tumour has infiltrated the whole orbit. infratemporal fossa by squamous cell carcinoma.
totally obliterated the normal retrobulbar structures. and
destroyed the bone of the lateral orbital wall (arrows).

Fig. 10.15. Post-contrast axial CT scan showing an undifferentiated carcinoma invading the pterygo-palatine fossa and nasopharynx
(arrows).

Adenocarcinoma pseudopapillary form resembling adenocarcinoma

ofthe colon in structure and cell type. Other authors
Adenocarcinoma is predominantly adenomatous in have noted the resemblance of some high-grade
structure and arises from the glands in the nasal adenocarcinomata of the nose and sinuses to aden-
and paranasal mucous membrane. It is not common ocarcinoma of the colon (Michaels 1987). In some
in this situation. Ringertz (1938) found 10 aden- cases there is abundant mucus production and the
ocarcinomata out of 31 glandular tumours involv- tumour may then resemble colloid carcinoma of the
ing the nose and paranasal sinuses, representing colon. The age distribution shows a peak occurrence
less than 4% of all carcinomata in this region. This in the sixth and seventh decades with a marked
author described two histological types: an alveolar predominance of males.
variety, which he regarded as the malignant Patients suffering from this condition may
counterpart of adenoma, showing large cystic present with nasal obstruction and epistaxis, or pain
spaces with abundant mucus secretion; and a and local swelling when the tumour becomes large.
102 Epithelial Tumours

Ultimate spread to the anterior fossa is well recorded naso-ethmoidal. the tumour occupying the upper
in the literature and reflects the site of origin of part of the nasal cavity and the adjacent ethmoid
these tumours - which. the majority of authors cells. This is the common location reported by other
agree. is high up in the nasal cavity and adjacent authors (see above). The distribution of this tumour
ethmoid cells (Ringertz 1938; Batsakis 1970). is illustrated graphically in Fig. 10.17. From this
Much ofthe interest in this type of carcinoma has point of origin the adenocarcinoma will cause
centred on its relation to occupation. Hadfield and erosion of the anterior ethmoid cells with extension
her colleagues (Macbeth 1965; Acheson et al. to the orbit and cribriform plate and early invasion
1967; Hadfield 1970; Hadfield and Macbeth 1971) of the anterior fossa. For this reason these patients
have demonstrated the prevalence of this disease in need some form of cranio-facial surgery for the
woodworkers employed in the furniture industry disease to be extirpated.
and it has been established that hardwood dusts On CT scan the location of the tumour is the
are the causative agent. Hadfield has expressed the most suggestive feature. The majority of this type of
belief that woodworker's carcinoma probably arises carcinoma enhance well with intravenous contrast.
primarily in the middle turbinate. on the anterior Four showed calcification. a feature well recognised
end of which the wood dust is found to be deposited. in colloid adenocarcinoma of the large bowel with
This site of origin would correlate well with the similar histology (Figs. 10.18. 1O.19). In some
known distribution of this tumour in the nose and patients a sclerotic reaction was observed in the
sinuses (see Fig. 1O.17). walls of the affected sinus cavity. but this was
usually of low intensity and not distinctive. In three
patients the tumour clearly took origin in the frontal
Radiology and Imaging sinus - a rare location for any malignant tumour
in the sinuses.
Thirty-two patients with sinus adenocarcinomata The changes shown on magnetic resonance scans
have been investigated: 29 by CT scan. 3 of whom were also non-specific. Generally the tumours gave
also had magnetic resonance. and the remainder moderately high signal on Trweighted spin echo
by conventional radiographic studies. sequences. with inhomogeneous features within the
Approximately 37% ofthese tumours were antro- tumour but clearly demarcated contours (Fig.
ethmoidal in location and did not show changes in 1:0.20). the tumour being easy to distinguish from
any way different from those described for squam- the higher-intensity signal of retained secretion.
ous cell carcinoma. However a greater number Failure to demonstrate calcification was a drawback
(63%) showed a different location. being principally of magnetic resonance: to show this and any associ-

Adenocarcinoma

Squamous Carcinoma

Adenoid Cystic Carcinoma

nose ethmoid antrum sphenoid
Fig. 10.17. The distribution of three common types of carcinoma seen in the paranasal sinuses. derived from 73 patients examined
by cr and magnetic resonance. Note that the adenocarcinomata are predOminantly naso-ethmoidal in locatio!).. while the distribution
graph of adenoid cystic carcinoma is virtually the converse of that for adenocarcinoma.
Malignant Epithelial Tumours 103

Fig. 10.18. Woodworker's adenocarcinoma. Axial CT scan

showing tumour mass with calcification.

b
Fig. 10.20a,b. MagnetiC resonance scans (Trweighted spin echo
sequence) showing a well-demarcated adenocarcinoma of the
ethmoids invading the orbit.

the nose and paranasal sinuses. It was first described

by Billroth (1859), who gave it the name of "cylin-
droma"; since that time it has become apparent
that this neoplasm has a relentless tendency to
recurrence over many years, leading eventually to
Fig. 10.19. Same patient as Fig. 10.18. Coronal CT scan showing
calcification in the adenocarcinoma. Note the early invasion of
the death of the patient. The term adenoid cystic
the anterior fossa. carcinoma has been generally adopted and better
fits the histological appearance and behaviour of
the tumour.
ated bone destruction, the magnetic resonance The age incidence is broadly based with a peak
studies need to be augmented by CT (Figs. 10.21, distribution in the fourth decade; there is a slight
10.22). However, it should be emphasised that the preponderance of female patients. As in other malig-
essential pre-operative requirement is to show the nant tumours nasal obstruction, epistaxis with
extent of intracranial invasion above the cribriform facial pain and swelling are the common forms of
plate, and this is best achieved by sagittal magnetic presentation. To these may be added paraesthesia
resonance with or without an intravenous para- and anaesthesia in the distribution of branches of
magnetic contrast agent (Fig. 10.23). the fifth nerve, particularly the infraorbital branch
of the second division, due to perineural infiltration
by tumour: this is the most constant form of spread
Adenoid Cystic Carcinoma of this neoplasm. The tumour may also spread to
lymph nodes, and blood stream metastases occur in
Adenoid cystic carcinoma is a malignant tumour nearly 40% of cases (Spiro et al. 1973). The tumour
derived from salivary type sero-mucinous glands in has a variable histology but the essential feature is
104 Epithelial Tumours

Fig. 10.21. Coronal CT scan showing calcification in an aden-

ocarcinoma.

Fig. 10.23. Adenocarcinoma shown on sagittal magnetic res-

onance scan. Extension of the tumour into the anterior fossa is
dearly demonstrated. The high signal seen in the area immedi-
ately above the tumour was shown at surgery to be due to
necrotic brain tissue.

and the remainder by conventional radiography.

Adenoid cystic carcinoma in the sinuses may
present as a bulk tumour (Fig. 10.24) occupying an
antro-ethmoidal location and may show the usual
features of malignancy in the sinuses with bone
Fig. 10.22. Coronal magnetic resonance scan (T,-weighted spin destruction on plain radiography and CT. However
echo sequence) ofthe same patient as Fig. 10.21. showing orbitai
invasion but no evidence of the calcification seen on CT. a high proportion do not present a large mass but
exhibit a more infiltrative process: they tend to infilt-
rate tissue planes (Fig. 10.25) and advance along
the cribriform or "sieve-like" pattern of the cells nerve sheaths. Perineural infiltration may show on
without which the diagnosis cannot be established plain radiographs as an enlargement of the infra-
(Friedmann and Osborn 1982). The cribriform orbital canal and local destruction of the floor of
pattern is produced by lumina or holes within the the orbit. from an adenoid cystic tumour in the
cell structure which contain connective tissue maxillary antrum. In more advanced lesions se-
mucin or epithelial mucin. quential enlargement of the foramen rotundum and
foramen ovale may occur. With the new soft tissue
imaging techniques it may be possible to see the
Radiology and Imaging nerve enlargement as such rather than its sec~.
ondary effect on the adjacent bone. The perineural
Twenty-two patients have been investigated with infiltration may be remarkably well demonstrated
this condition: 17 by CT. 1 by magnetic resonance. (Figs. 10.26. 10.27).
Malignant Epithelial Tumours 105

Fig. 10.24. Coronal CT scan of an adenoid cystic carcinoma

shown as a rounded tumour mass in the ethmoids.

Fig. 10.26. Magnetic resonance scan (Tl-weighted spin echo

sequence) of a recurrent adenoid cystic carcinoma of the lacrimal
gland. The tumour has invaded the floor of the orbit and the
upper part of the maxillary antrum, and there is perineural
infiltration and enlargement of the infraorbital nerve (arrow).

Fig. 10.25. Axial CT scan of an adenoid cystic carcinoma of the

right nasal cavity and maxillary antrum. There is infiltration of
the mucosa and bone destruction without a large soft tissue
mass.

The distribution of these tumours is shown in

Fig. 10.17. It can be seen that the shape of the
distribution graph for adenoid cystic carcinoma is
almost the converse of that for adenocarcinoma and
indicates that the majority of these tumours take
origin in the lower part of the nasal cavity and
maxillary antrum. In the patients investigated by
CT the hard palate was shown to be involved in
over 40% at first examination. providing supporting
evidence for this site of origin.

Ameloblastoma
Fig. 10.27. Same patient as Fig. 10.26. Branches ofthe second
Ameloblastoma is an uncommon epithelial tumour division of the fifth cranial nerve show perineural infiltration.
which may occur in the maxilla and invade the The upper arrow indicates the infraorbital nerve, the lower arrow
sinuses by direct extension. Malassez (1885) recog- the posterior superior dental nerve.
106 Epithelial Tumours

nised the origin of this tumour from dental epi-

thelium and was responsible for the introduction of
the name adamantinoma. This was, however, a
misnomer since enamel is not produced by the
tumour and Churchill (1934) gave it the more
appropriate title of ameloblastoma. The neoplasm
may arise from the remnants of the dental lamina
and the enamel organ, the basal layer of the oral
mucous membrane, or the epithelial lining of a
dentigerous cyst. The tumours are slow-growing
and are generally considered to be benign, although
they are locally invasive and metastasise on rare
occasions.
The radiological features of 16 cases of ame-
loblastoma have been described by Mcivor (1974).
In five of his patients a non-specific unilocular radi-
olucent area was present, in the bone of the alveolar
margin or mandible. The remainder of the lesions
also showed as a radiolucent area but in com-
bination with at least three of the following
additional features: expansion of the overlying cort-
ical plate; a corticated scalloped margin; a multi-
locular appearance; and resorption of adjacent
tooth roots.
When it invades the maxillary antrum the
tumour presents either as a dentigerous cyst or
more commonly as a solid tumour filling the sinus
cavity, expanding and eroding bone (Fig. 10.28). Fig. 10.29. T2 -weighted sagittal magnetic resonance scan
Allor part of a tooth may be embodied in the showing extensive recurrence of an ameloblastoma in the nose
tumour. and sinuses.
In three patients with sinus involvement seen by
the author, two had extensive recurrence of the
tumour, which spread to other paranasal sinuses
from the maxillary antrum (Fig. 10.29).

Malignant Melanoma
Malignant melanoma is relatively rare compared
with other malignant neoplasms of the nose and
sinuses. Holdcraft and Gallagher (1969) in a review
of 1029 neoplasms of the mucosa of the nose and
paranasal sinuses found 39 melanomata, repre-
senting 3.8% of all malignant tumours in this situ-
ation. Melanomata can arise de novo in mucosal
tissues which normally are non-pigmented. The
patient, after a period in which there may be nasal
obstruction and epistaxis, eventually presents with
a darkly pigmented or fleshy nasal mass. The
majority of tumours arise in the nasal cavity; a
minority arise in the sinuses, predominantly the
maxillary antrum. Within the nasal cavity the
common sites of origin are the septum, the lateral
wall and the inferior turbinate. The tumours are
Fig. 10.28. Axial cr scan of an ameloblastoma of the nose and probably derived from melanocytes in the nasal
maxillary antrum. epithelium and microscopically the common
Malignant Epithelial Tumours 107

pattern is the presence of sheets of spheroidal cells

and spindle cells with variable degrees of pig-
mentation.
There is evidence of a constant risk of death from
melanoma. no matter how long after treatment
the patient lives (Lund 1982). This is unlike the
situation with most other malig~ant tUmours
where survival for 1 or 2 years reduces the risk of
death substantially. This record of survival would
imply that in almost all patients the disease has
disseminated by the time of initial diagnosis (Har-
rison 1976). and that the secondaries are controlled
immunologically throughout the body. Thus the
host-tumour balance is crucial to the final outcome
(Lund 1982).

Fig. 10.31. Axial CT scan of a malignant melanoma showing

Radiology extensive bone destruction in the posterior ethmoid cells. This
was a metastasis from a primary skin melanoma which had been
In the author's series 25% of cases showed no evi- removed previously from the scapular region.
dence to suggest the presence of a neoplasm in the
sinuses on the initial plain radiographic examin-
ation. while another 25% showed a mass in the
nasal cavity without other significant abnormality.
In 50% of patients bone destruction was recognised
at initial examination. usually at the antro-
ethmoidal junction and associated with a large
antro-nasal mass. These tumours may spread
quickly. with a rapid transformation of the radio-
graphic appearance over a short period of obser-
vation. Recurrence after surgery is also a common
feature.

CT Findings

Eight patients have been examined by CT. seven of

whom had a primary melanoma in the nose and
sinuses. and one who had a naso-ethmoidal meta-
stasis from a skin melanoma excised previously. The Fig. 10.32. Coronal CT scan of a malignant melanoma invading
the floor of the anterior cranial fossa (arrow).

average age of these patients was 56 years and the

age range 34-70 years. The tumours were pre-
dominantly naso-antro-ethmoidal in location and
showed no characteristic attenuation features on
CT. The nasal mass was the most prominent
element supporting the view that these tumours
take origin in the nasal cavity. Associated bone
destruction was present in all. involving the maxil-
lary antrum (Fig. lO.30). the ethmoids (Fig. 10.31)
or the cribriform plate area (Fig. lO.32). The orbit
was invaded in 50% of patients at initial exam-
ination (Fig. 10.33). and the overall appearance
Fig. 10.30. Axial CT scan of a malignant melanoma of the nose that of a highly malignant neoplasm showing
and antrum with extension into the infratemporal fossa. relentless spread and recurrence.
108 Epithelial Tumours

a
Fig. 10.33a,b. Axial (a) and coronal (b) CT scans showing an extensive antro-nasal melanoma invading the orbit.

Metastatic Carcinoma severe epistaxis (Eneroth et a1. 1961), and in some

patients the primary tumour in the kidney may
Metastases in the nose and paranasal sinuses are remain occult until the patient presents with a
very rare and for this reason may be overlooked by metastasis (Harrison et a1. 1964). Like primary
the clinician and also by the histopathologist, since tumours in the sinuses, secondaries may also
microscopically they may closely resemble some present to the ophthalmologist with proptosis (Fig.
forms of primary carcinoma. Almost all are derived 10.34). Other examples of sinus metastases
from primary tumours ofthe genito-urinary system, encountered include examples of secondary car-
with a strong predominance of renal carcinoma. In cinoma of the pancreas (Figs. 10.35, 10.36) and
a review by Friedmann and Osborn (1965) it was secondary malignant melanoma (Fig. 10.31). Sinus
noted that nearly 50% of secondary tumours in the metastases should be suspected when the his-
ENT region involved the nose and sinuses and that tological appearance of a biopsy of a neoplasm does
of these 80% were derived from renal aden- not resemble the usual pattern of primary malig-
ocarcinoma. Other primary tumours include semi- nant tumours of the sino-nasal tract (Michaels
noma of the testis (Garrett 19 59) and uterine 1987). In these circumstances an intravenous
chorio-carcinoma (Salimi 1977; Mukherjee 1978). .urogram becomes essential to exclude a primary
Examples of metastases from carcinoma of the renal neoplasm.
breast, stomach and lung have also been recorded.
An explanation for the predominance of meta-
stases from renal carcinoma in the nose and sinuses
has been given by Nahum and Bailey (1963). It is
well known that this tumour is prone to grow into
renal veins, and these authors quote the work of
Batson (1940), who demonstrated that, during
periods when the intrathoracic pressure is greatly
increased, a retrograde venous flow can occur
through the prevertebral and vertebral venous
plexus. This retrograde flow may at times progress
to the base of the skull and may also be observed in
the jugular venous system. With the rich venous
anastomoses near the paranasal sinuses - notably
the pterygoid plexus - metastases might find their
way from the kidney by this route.
The marked vascularity of metastases from renal Fig. 10.34. Metastasis from renal carcinoma presenting as a
carcinoma may result in a clinical presentation with simple opacity of the left frontal sinus on plain radiography.
Malignant Epithelial Tumours 109

Fig. 10.35. Axial CT scan showing a metastasis in the ethmoid Fig. lO.36. Same patient as Fig. 10.35. Coronal CT scan of the
cells from carcinoma of the pancreas. metastasis in the ethmoids.

Hadfield EH. Macbeth RG (197l) Adenocarcinoma of ethmoids

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Harrison DFN (1976) Malignant melanoma ariSing in the nasal
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nasal cavity and accessory sinuses in woodworkers. Lancet from renal carcinoma. J Laryngol Otol 78:103-107
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Batson OV (1940) Formation ofthe vertebral veins and their role Hyams VJ (1971) Papillomas of the nasal cavity and paranasal
in the spread of metastases. Ann Surg 112: 13 8-14 7 sinuses. Ann Otol Rhinol Laryngol 80:192-206
Biliroth T (1855) Ueber dem Bau des Schleimpolyp. Reimer. Kramer R. Som ML (1935) True papilloma of the nasal cavity.
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cheldriisen. Virchows Arch Pathol Anat Histol 17:357-375 paranasal sinuses. J Laryngol Otol 96:347-355
Brown B (1964) The papillomatous tumours of the nose. J Lund VJ. Lloyd GAS (1984) Radiological changes associated with
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Eneroth CM. Martensson G. Thulin A (1961) Profuse epistaxis laryngol 82:296-300
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Friedmann I. Osborn DA (1965) Metastatic tumours in the ear. J Laryngol Otol 79:592-612
nose and throat region. J Laryngol Otol 79:576-591 Melvor J (1974) The radiological features of ameloblastoma. Clin
Friedmann I. Osborn DA (1982) Pathology of granulomas and RadioI25:237-242
neoplasms of the nose and paranasal sinuses. Churchill Liv- Malassez L (1885) Sur Ie role des debris epitheliaux paradentaires.
ingstone. Edinburgh Arch Physiol Normal Pathol (3rd series) 6:379---449
Garrett MJ (1959) Metastatic tumours ofthe paranasal sinuses Michaels L (1987) Ear. nose and throat histopathology. Springer.
simulating primary growths. J Fac Radiol (Lond) 10:151-155 Berlin Heidelberg New York
Hadfield EH (1970) A study of adenocarcinoma of the paranasal Mukherjee DK (1978) Choriocarcinoma of the nose. Ann Otol
sinuses in woodworkers in the furniture industry. Ann R ColI Rhinol Laryngol 87:257-259
Surg Engl 46:301-319 Nahum AM. Bailey BJ (1963) Malignant tumours metastatic to
110 Epithelial Tumours

the paranasal sinuses: case report and review of literature. the paranasal sinuses. Proc R Soc Med 57:742-747
Laryngoscope 73:942-953 Spiro RH. Koss LG. Hajdu SI. Strong EW (1973) Tumors of minor
Ringertz N (1938) Pathology of malignant tumours arising in salivary origin. Cancer 31:117-129
the nasal and paranasal cavities and maxilla. Acta Otolaryngol Ward N (1854) Follicular tumour involving nasal bones. nasal
[Suppl] 27:1-390 processes of superior maxillary bone and septum of nose.
Salimi R (1977) Metastatic choriocarcinoma ofthe nasal mucosa. Removal, death from pneumonia. autopsy. Lancet 11:480
J Surg OncoI9:301-305 Worgan D. Hooper R (1970) Malignancy in nasal papillomata.
Shaw H (1964) Clinical importance of orbital signs in cancer of J Laryngol OtoI84:309-316
11 Tumours of Vascular Origin

Hereditary haemorrhagic telangiectasia (Osler- neoplasms or as hamartomata. Most radiologists

Weber-Rendu syndrome) is a disease in which are aware of this tumour as a lesion that can be
groups of dilated vessels are present in the skin and well demonstrated by angiography, because of its
mucosae. The nose is commonly affected and the highly vascular nature, and which may require pre-
lesions are important because they frequently give operative embolisation, but the radiologist may also
rise to epistaxis. This condition does not require any be called upon to make the initial diagnosis. With
sophisticated imaging method, and the same is true such a vascular tumour, severe bleeding may
for another condition which may give rise to epis- accompany biopsy and for this reason most sur-
taxis: capillary haemangioma. Here the lesion is geons are reluctant to undertake biopsy of a naso-
usually localised to the mucosa of the nasal septum pharyngeal mass in an adolescent male patient and
or inferior turbinate. prefer to rely upon clinical and radiological features
There are six lesions of vascular origin that to decide whether the mass is likely to be an angi-
concern the imaging diagnostician: ofibroma or a non-vascular lesion such as an antro-
choanal polyp. Once the diagnosis is established
1. Juvenile angiofibroma then the role of the radiologist is to define the limits
2. Haemangiopericytoma of.the tumour prior to surgery, since this may in-
3. Angiosarcoma (haemangio-endothelioma) fluence the surgical approach.
4. Cavernous haemangioma According to Tapia Acuna (1956) this tumour
5. Venous malformations was known to Hippocrates, but the first recorded
6. Angiolymphoid hyperplasia with eosinophilia description of juvenile angiofibroma involving the
post-nasal region was given by Chelius in 1847.
(Kimura's disease)
He noted the fibrous nature of the lesion and its
occurrence at about the time of puberty. Gosselin
(18 73) emphasised the occurrence of naso-
Juvenile Angiofibroma pharyngeal fibrous polyps almost exclusively in
young males and noted that while some lesions
tend to regress as the patient becomes adult others
Juvenile angiofibroma is a rare benign but highly require surgical removal. The term juvenile naso-
vascular tumour occurring in adolescent males. The pharyngeal fibroma was introduced by Chaveau
juvenile nasopharyngeal angiofibroma was so (1906) and Friedberg (1940) suggested the name
called because it was formerly thought to arise in angiofibroma. Since these descriptions a large
the nasopharynx. It is not known why these volume of literature has accumulated about this
tumours occur almost exclusively in a restricted age relatively rare condition. There is general agree-
group and restricted site at the base of the skull, ment that in most cases the age of onset is in the
or indeed whether they should be considered as second decade of life and that there is an over-
112 Tumours of Vascular Origin

whelming predominance of male patients. In the

author's series all patients were male and no
example of this condition was seen in a female.
The diagnosis is almost always made clinically
by the history of a young or adolescent male with
nasal obstruction or epistaxis or both; expansion of
the lesion may lead to facial deformity with swelling
of the cheek and proptosis. Physical examination
shows a nasopharyngeal mass. The angiofibroma
has a reddish-purple nodular appearance and is
composed of vascular and fibrous tissue, the latter
varying in texture from a compact hyaline mass
to oedematous granulation tissue. The vessels also
vary considerably, particularly in the degree of
development of the muscular component of the
walls.
The site of origin is important. It was first thought
that the lesions arise in the nasopharynx, but
examples can be found in the literature in which
there was no nasopharyngeal mass. Hora and
Weller (1961) described an extranasopharyngeal
angiofibroma with apparent origin from the pter-
ygo-maxiUary space and attachment to the medial
Fig. 11.2. Five-millimetre coronal CT scan taken immediately
pterygoid plate. The tumour extended into the infra- posterior to Fig. 11.1. showing early erosion of the base of the
temporal fossa and presented as a swelling of the medial pterygoid plate (arrow).
cheek, but there was no associated nasopharyngeal
mass. Other authors (Friedmann and Osborn 1983)
have described attachments around the margins of
the choanae (that is the medial pterygoid plate, the
posterior border of the hard palate and the posterior

Fig. 11.3. Same patient as Fig. 11.1 and 11.2. Axial CT scan
Fig. 11.1. Coronal CT scan showing enlargement of the spheno- showing early extension into the posterior maxillary antrum and
palatine foramen (short arrow) by early angiofibroma. Early sphenoid sinus (arrows) and widening of the pterygo-maxillary
invasion of the sphenoid sinus is also present (long arrow). fissure (arrowheads).
Juvenile Angiofibroma 113

margin of the vomer), but the evidence is that the Extension into the infratemporal fossa may
tumour originates from the region of the spheno- eventually result in the mass emerging between
palatine foramen within the pterygo-palatine fossa. the upper molar teeth and ascending ramus of the
This is important not only to the surgeon but also to mandible, the tumour coming to lie beneath the
the radiologist, both for making the initial diagnosis skin of the cheek (Fig. 11.10). Thus the tumour
and also for understanding the expected spread of comes to present a bilobed shape with a medial
the tumour. . component in the' nose and nasopharynx and a
The earliest bone changes seen are illustrated in lateral component in the infratemporal fossa, the
Figs. 11.1-11.4. These appearances suggest that two lobes being joined by an isthmus of tumour
the site of origin is likely to be within the spheno- lying between the maxillary antrum and the ptery-
palatine foramen (Figs. 11.5, 11.6) at the supero- goid plates. All these features have been clearly
medial extremity of the pterygo-palatine fossa. This demonstrated by the imaging techniques used.
point of origin fits with the known extension of the Essentially, then, the tumour causes an expan-
tumour and the early bone changes visible. An sion of the spheno-palatine fossa, and the early bone
expanding tumour in this situation would enlarge changes to be looked for by the radiologist will
the spheno-palatine foramen, grow medially into consist of pressure erosion and invasion of struc-
the area of least resistance - the nose and naso- tures in its immediate vicinity. Polyps or tumours
pharynx (Figs. 11.4, 11. 7) - erode the root of the not arising in this situation are unlikely to present
medial pterygoid plate (Figs. 11.2, 11. 7), invade the this pattern of change, which serves to distinguish
sphenoid sinus (Fig. 11.7) and indent the postero- an angiofibroma from other conditions, for example
superior border of the maxillary antrum giving rise . an antro-choanal polyp, which may present with a
to the so-called antral sign described by Holman nasopharyngeal mass.
and Miller (1965). This consists of anterior bowing The diagnostic role of plain radiography is to
of the posterior wall of the maxillary antrum, best identify the antral sign when present. In Holman
seen on a lateral projection or on lateral tom- and Miller's (1965) series this sign was present in
ography (Fig. 11.8). 87% of patients. In the series described by Lloyd
The tumour can also gain access to the infra- and Phelps (1986) it was positive in just over 81%
temporal fossa by lateral extension via the pterygo- (Fig. 11.8). Long-term pressure on the posterior
maxillary fissure (Fig. 11.9) and invade the orbit antral wall will cause bowing of the wall anteriorly.
through the inferior orbital fissure. Further exten- Thus any slow-growing tumour, usually but not
sion from these areas may result in middle fossa always of a benign nature, will cause this deformity.
invasion, either by way of the orbit and superior The antral sign cannot be considered, therefore, as
orbital fissure or directly through the lateral wall of pathognomonic for angiofibroma. Bowing of the
the sphenoid sinus. posterior antral wall has been seen in a proven

Fig. 11.4. Axial CT scan showing early angiofibroma extending into the nasal cavity and nasopharynx (arrows). and widening of
the spheno-palatine foramen (arrowheads).
114 Tumours of Vascular Origin

Fig. 11.5. Dried skull showing the site of origin of angiofibroma within the spheno-palatine foramen (arrow).

Fig. 11.6. Line drawing showing the close relationship of the sphenoid sinus (1). the spheno-palatine foramen (2) and the base of
the medial pterygoid plate (3).
Juvenile Angiofibroma 115

neurofibroma (Fig. 11.11) and also in a single

example of haemangiopericytoma. Schaffer et al.
(1978) have also described two cases of tumours
other than angiofibromata which gave a positive
antral sign. A more important and constant diag-
nostic feature is the tomographic demonstration of
deformity ofthe base ofthe medial pterygoid lamina.
This was present in 100% of 28 patients reviewed
by Lloyd and Phelps (1986) and was demonstrated
either by conventional tomography or coronal CT
scan. This change does not occur in simple naso-
pharyngeal polyps; when there is no such bone
deformity the radiologist can reassure the surgeon
that it is safe to take a biopsy without the danger
of severe haemorrhage from an angiofibroma.
The next duty of the radiologist is to map the
extent of the tumour prior to surgery. Most import-
ant in this respect is to demonstrate whether there
is lateral spread into the infratemporal fossa, which
Fig. 11. 7. Coronal cr scan showing an angiofibroma projecting
into the air space at the posterior nares (thick arrow). invading may determine the surgical approach used. With
the sphenoid sinus (thin arrows) and eroding the base of the no spread into the infratemporal fossa a transpalatal
medial pterygoid plate. surgical approach may be feasible, but with any
degree of involvement of the infratemporal fossa
a lateral rhinotomy or transmaxillary approach is
required. The extent ofthe tumour and in particular
the degree of involvement of the infratemporal fossa
may be demonstrated by enhanced CT scan in the
immediate post-injection phase (Fig. 11.12), but is
now better achieved by magnetic resonance (Fig.
11.13).

a ...-.__ b
Fig. 11.8. a Lateral plain radiograph of the sinuses showing an early antral sign (arrow): forward bowing of the posterior antral wall.
b Lateral hypocycIoidal tomogram showing enlargement of the spheno-palatine fossa with an early antral sign (arrow).
116 Tumours of Vascular Origin

Fig. 11.11. Axial CT scan showing forward bowing of the pos-

terior wall of the maxillary antrum (arrow) by a proven neuro-
Fig. 11.9. Axial CT scan showing early extension of an angio- fibroma.
fibroma into the infratemporal fossa (arrows). Note the widening
of the pterygo-palatine fossa and the pterygo-maxillary fossa by
the tumour.

b
Fig. 11.10. a Coronal magnetic resonance scan showing an angiofibroma. b Enlargement scan of the angiofibroma showing large
vessels within the tumour (small arrows) and the tumour bulge beneath the skin of the cheek (large arrow).

Magnetic resonance has several advantages over The vascular nature of angiofibroma as dem-
CT. It better demonstrates the exact extent of the onstrated by negative signal from the vessels within
tumour and its superior density resolution shows the tumour is also clearly revealed by magnetic
the edge of the tumour more clearly in relation resonance (Figs. 11.10, 11.15). A nasopharyngeal
to other soft tissue structures in the infratemporal mass in an adolescent male shoWing this appear-
fossa. Three-plane imaging and good sagittal sec- ance is clearly diagnostic of angiofibroma. Finally,
tions are an added advantage, and the distinction all this is achieved without the use of ionising radi-
between tumour invasion of a sinus and secondary ation or intravenous contrast injection.
mucocoele formation is clearly shown (Fig. 11.14).
Juvenile Angiofibroma 117

Fig. 11.12. Axial CT scan after contrast injection showing exten- Fig. 11.13. Axial magnetic resonance scan showing extension
sion of an angiofibroma into the infratemporal fossa. of an angiofibroma into the infratemporal fossa.

Fig. 11.14. Sagittal magnetic resonance scan of a patient with a large angiofibroma. The white areas (arrows) giving a strong signal
indicate retained secretion and mucocoele formation in the posterior ethmoids and sphenoid sinus.
118 Tumours of Vascular Origin

Fig. 11.15. Axial magnetic resonance scan showing the vascularity in an angiofibroma. The vessels are shown as dark areas of
negative signal (arrows).

Conclusions In fact most of these tumours do not require

embolisation; and in the series reported by Lloyd
1. Angiofibroma originates at the spheno-pala- and Phelps (1986) there was some evidence to
tine foramen. It enlarges the foramen and erodes suggest that the recurrence rate was increased and
bone locally at the base of the medial pterygoid the rate of recurrence in individual patients accel-
plate, the floor of the sphenoid sinus and the pos- erated by embolisation. The reasons for this are
terior wall of the maxillary antrum. Further exten- speculative, but it is possible that excessive shrink-
sion leads to invasion of the infratemporal fossa, age of the tumour after embolisation may lead to
orbit and middle cranial fossa. minute fragments being overlooked at surgery;
these may then quickly re-vascularise in the post-
2. The antral sign on plain radiography is not
operative period.
completely reliable. When in doubt coronal tom-
ography, or preferably CT scan, should be under-
taken to show signs of early bone erosion of the
medial pterygoid lamina, enlargement of the Haemangiopericytoma
spheno-palatine foramen and invasion of the sphe-
noid sinus.
3. In the presence of a positive antral sign, three- Haemangiopericytoma is an uncommon vascular
plane magnetic resonance is the investigative tumour believed to derive from the pericyte, a cell
method of choice. It best demonstrates the extent of with processes which encircle the endothelial cells
the tumour; it uses non-ionising radiation; and it of capillaries. The tumour was first described by
will show the vascular nature of the angiofibroma Stout and Murray (1942). These authors believed
and confirm the diagnosis. that the blood vessels were an integral part of the
4. Angiography should only be performed if neoplasm and not part of its framework, and
embolisation is deemed necessary prior to surgery. because each vessel in the tumour was lined by
Haemangiopericytoma 119

normal endothelial cells they suggested that the fossa. From there it had extended upwards through
tumour consisted essentially of pericytes. Since that the inferior orbital fissure to invade the orbital apex
time many cases of vascular neoplasms with these (Fig. 11.17). The second tumour expanded one side
features have been published. and the concept of of the nasal fossa and encroached upon the antral
haemangiopericytoma is now generally accepted. cavity (Fig. 11.18). while the third was essentially
Twenty-three examples of this tumour occurring in naso-ethmoidal in location.
the nose and paranasal sinuses have been reported
by Compagno (1978). His patients presented with
epistaxis and nasal obstruction. most commonly in
the sixth and seventh decades of life; the lesions
mimicked allergic polyps clinically.
Eleven patients with haemangiopericytoma
affecting the facial skeleton have been investigated:
in eight the tumour was located in the orbit and in
three it was in the nose and sinuses. It is said that
haemangiopericytoma of the nose and sinuses has
a reduced recurrence rate when compared with
those found at other sites in the body (Michaels
1987). An equally benign course has been found in
the eight tumours arising in the orbit. In only one
patient was there a history of recurrence of the
tumour (Fig. 11.16). This followed several previous
attempts at surgical removal elsewhere. The evi-
dence is that no recurrence is to be expected if the Fig. 11.17. Postero-anterior radiograph showing bone erosion in
tumour is removed in toto with intact capsule at the orbital apex due to a haemangiopericytoma. See also Fig.
first surgery. 11.19.

Radiological Features
Of the three patients who presented with the tumour
in the nose or sinuses. all showed a mass on one
side of the nasal cavity. In one the tumour had
extended massively into the infratemporal fossa via
the pterygo-palatine fossa and pterygo-maxillary

Fig. 11.16. Coronal CT scan of the left orbit showing a recurrent Fig. 11.18. Coronal hypocycloidal tomogram showing a large
haemangiopericytoma invading the lateral wall of the orbit and haemangiopericytoma in the nasal cavity. encroaching upon the
maxillary antrum. left maxillary antrum.
 120 Tumours of Vascular Origin

a b

Fig. 1l.I9a.b. Same patient as Fig. 11.17. a Hypervascularity in a haemangiopericytoma which arose in the infratemporal fossa
and invaded the posterior wall of the maxillary antrum and the orbital apex. b Typical strong tumour blush in the late arterial phase
of the arteriogram.

The diagnostic feature of haemangiopericytoma The clinical features show a broadly based age
is its high vascularity. All tumours examined by distribution and no sex predilection. Nasal bleeding
carotid angiography. whether in the nose. sinuses and obstruction is the common form of presen-
or orbit. have invariably shown a strong tumour tation. with swelling of the cheek when the antrum
blush (Fig. 11.19). This can now be demonstrated is involved and proptosis when the orbit is invaded.
adequately by digital subtraction angiography To the naked eye the tumour is seen as highly
using intravenous contrast. and in this way arterial vascular masses of extremely friable consistency.
puncture may be avoided. Microscopically the changes vary according to the
degree of differentiation of cell pattern. but the
essential feature is the presence of vascular chan-
nels lined by malignant endothelial cells.
Two patients with this tumour have been inves-
tigated. The first was not investigated ab initio but
Angiosarcoma (Haemangio- was referred for CT scan prior to cranio-facial resec-
endothelioma) tion for a recurrence of the neoplasm. The second
patient was a 50-year-old male who presented with
a swelling of the dental alveolus. nasal obstruction
Angiosarcoma is the malignant counterpart of haem- and epistaxis. CT showed a massive tumour arising
angioma and is a very rare tumour in the nasal from the floor ofthe maxillary antrum. The changes
region. Fu and Perzin (1974) recorded two cases were non-specific but were those of a highly malig-
comprising 2% of malignant non-epithelial tumours nant neoplasm which had destroyed the alveolar
of the nose and paranasal sinuses; Friedmann and margin. extended into the nasal cavity. and had
Osborn (1982) encountered only one case repre- eroded the posterior wall of the antrum with
senting 0.1 % of all tumours of this region. invasion of the infratemporal fossa (Fig. 11.20).
Cavernous Haemangioma 121

Fig. 11.20. Axial (above) and coronal (be/ow) CT scans showing an angiosarcoma of the right maxilla. The large tumour has caused
massive bone destruction in the alveolus and antral walls.

Cavernous Haemangioma Haemangiomata may also be confined to the bone

of the sinus wall or to the adjacent skull. Here they
produce a well-demarcated osteolytic defect with
Cavernous haemangioma is a very rare tumour in a fine honeycomb appearance resulting from the
the nose and sinuses. but in the adjacent orbit it is formation of numerous thin trabeculae of new bone
the commonest benign neoplasm. It is made up of between the vascular sinuses (Fig. 11.21). In a
thin-walled blood-filled channels lined by a single tangential view or in profile it may be possible to
layer of endothelial cells and is found either in the see fine spicules radiating out from the bony surface.
marrow spaces of the bone or as a vascular swelling producing the so-called sunray or sunburst appear-
beneath the mucosa (Michaels 1987). It arises most ance.
often from the lateral wall of the nose but may also
involve the sinus cavities. Clinically when a tumour
arises in these situations it presents with nasal
bleeding or obstruction. and a tender swelling of Venous Malformations
the cheek if the antrum is involved (Fordham 1978).
Some tumours may bleed following dental extrac-
tion (Broderick and Round 1933). Radiologically Under the title venous haem angioma Fu and Perzin
most reports have shown an opaque sinus. often (1974) described three patients who presented with
with adjacent bone erosion and a nasal mass. Tsut- small mass lesions in the nasal vestibule. Micro-
sumiuchi et al. (1982) recorded a solitary phlebolith scopically these lesions were composed of small.
shown on CT in a cavernous haem angioma located thick-walled venous channels. The vessels often
within the maxillary antrum and extending to the extended into the adjacent striated muscle and
nose and ethmoid cells. normal cartilage. Although rare in the nose and
122 Tumours of Vascular Origin

Fig. 11.21. Haemangioma in the frontal bone and frontal sinus. There is a clearly demarcated area of osteolysis in the bone within
which is a fine trabecular pattern.

sinuses venous malformations are relatively

common in the adjacent orbit. They present radi-
ologically with a classical triad of signs: an enlarged
orbit. the presence of phleboliths. and associated
venous "lakes" in the frontal bone over the affected
orbit.
Congenital venous malformations are best dem-
onstrated by orbital venography (Fig. 11.22). They
may replace the normal venous system with a
network of dilated venous channels. and in the most
extensive malformations the adjacent sinuses may
be involved (Fig. 11.23). In the orbit phleboliths
invariably indicate the presence of abnormal veins
(Lloyd 1982). They may be encountered in a sinus
cavity (Fig. 11.24) and presumably have the same
diagnostic implications.

Angiolymphoid Hyperplasia with

Eosinophilia (Kimura's Disease)

In 1948 Kimura et al. described a peculiar disease in

which there was an unusual granulomatous lesion Fig. 11.22. Orbital venogram. Subtraction study showing a
combined with hyperplastic changes of lymphatic venous malformation in the orbital apex.
 Angiolymphoid Hyperplasia with Eosinophilia (Kimura's Disease) 123

composed of hyperplastic lymphoid tissue which

contained well-developed lymph follicles and infilt-
rates of eosinophils. In 1969 Wells and Whimster
reported nine similar cases under the heading of
angiolymphoid hyperplasia with eosinophilia. The
subcutaneous nodules were composed of unen-
capsulated masses of lymphoid tissue admixed with
newly formed vessels and infiltrates of eosinophils.
Reed and Terazakis (1972) recorded six further
examples of this peculiar angioblastic lesion. The
lesions in their patients were either solitary or mul-
tiple and presented as tumour-like swellings in the
subcutaneous tissue of the head and neck.
a The true nature of this lesion has been unclear
for a long time, but it is now generally thought to
be a neoplasm consisting of proliferating endothelial
cells (Welch et al. 1987). It may occur in the orbit
and extend from there into the sinuses. Three
patients with orbital involvement have been inves-

b
Fig. 11.23a,b. Axial (a) and coronal (b) CT scans showing a huge
venous malformation in the orbit with multiple phleboliths. The
venous malformation also involved the adjacent ethmoid cells.

tissue. In 1965 Kawada et al. reported four similar

cases which were characterised by flat or dome-
shaped swellings and by a blood eosinophilia. The
lesions occurred almost exclusively on the cheek,
axillary, cubital or inguinal regions. They were

Fig. 1l.25a,b. Angiolymphoid hyperplasia with eosinophilia.

Coronal (a) and axial (b) CT scans showing massive invasion of
Fig. 11.24. Phleboliths (arrows) shown in the right maxillary the orbit with extension to the antrum. ethmoids and middle
antrum on routine sinus radiography. cranial fossa.
124 Tumours of Vascular Origin

tigated during the past 8 years. In two the orbital suffocant et rebelle. Clinique Chirugicale de L'hopital de la
soft tissue structures were totally replaced by the Charite (Paris) 1:92-116
Holman CB, Miller WE (1965) Juvenile nasopharyngeal fibroma.
angiolymphomatous mass, which expanded and AJR 94:292-298
eroded the bony walls and invaded the sinuses (Fig. Hora IF, Weller WA (1961) Extranasopharyngeal juvenile an-
11.25). The disease has proved difficult to control giofibroma. Ann Otol Rhinol Laryngol 70: 164-170
and in one patient has necessitated cranio-facial Kawada AK, Takahashi H, Anzai T (1965) Eosinophilic
folliculosis of the skin (Kimura's disease). Jpn J Dermatol
resection. 76:61-72
Kimura T, Yoshima S, Ishikawa E (1948) Unusual granulation
combined with hyperplastic change of lymphoid tissue. Trans
Jpn Pathol Soc 37:179
lloyd GAS (1982) Vascular anomalies in the orbit. Orbit 1:45-
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Broderick RA, Round H (1933) Cavernous angioma of the Otolaryngol11:247-259
maxilla: a fatal haemorrhage after teeth extraction with notes Michaels L (198 7) Ear, nose and throat histopathology. Springer,
of a similar, non-fatal case. Lancet II: 13-15 Berlin Heidelberg New York
Chaveau C (1906) Histoire des maladies du pharynx. Bailliere, Reed RJ, Terazakis M (1972) Subcutaneous angioblastic lymph-
Paris oid hyperplasia with eosinophilia (Kimuras's disease). Cancer
Chelius MJ (1847) A system of surgery, vol 2. Renshaw, London 29:489-497
Compagno J (1978) Haemangiopericytoma-Iike tumours of the Schaffer K, Victor M, Farley H, Friedman J (1978) Pitfalls in the
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tissues. Laryngoscope 88:460-469 428
Fordham SD (1978) Haemangioma of the maxillary sinus. Ear Stout AP, Murray MR (1942) Haemangiopericytoma. Ann Surg
Nose ThroatJ 57:333-335 116:26-33
Friedberg SA (1940) Nas.opharyngealfibroma. Arch Otolaryngol Tapia Acuna R (1956) The nasopharyngeal fibroma and its
31:313-326 treatment. Arch Otolaryngol 64:451-455
Friedmann I, Osborn DA (1982) Pathology of granulomas and Tsutsumiuchi K. Hasegawa M, Okuno H, Watanabe I, Okayasu
neoplasms of the nose and paranasal sinuses. Churchill Liv- I, Suzuki S (1982) Haemangioma of the maxillary sinus. ORL
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cavity, paranasal sinuses and nasopharynx. I. General features plasia with eosinophilia. J R Soc Med 80:384-385
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Gosselin L (1873) Fibrome ou polype fibreux nasopharyngien hyperplasia with eosinophilia. Br J Dermatol 81: 1-15
12 Lymphoreticular Tumours

Lymphoma by pluri-direction tomography and plain radio-

graphs. All but one of these tumours arose in the
nasal cavity or in the anterior ethmoid cells and at
Approximately one quarter of malignant lym- the time of examination both were usually involved
phomata arise in an extranodal site, of which the to a greater or lesser extent. The anterior location
nose and paranasal sinus region is one of the less produces the facial or nasal swelling common to
common. The term lymphosarcoma was first these tumours and it should be noted that a similar
applied to tumours in this situation by Schmidt anterior site is found in lymphomata in the orbit.
(1897), and Greifenstein (1937) was the first to In a series of 46 orbital lymphomata (Lloyd 1987),
report reticulum cell sarcoma occurring in the two thirds were anteriorly located and the majority
ethmoid region. The tumours are all of the non- of patients presented with a soft tissue swelling
Hodgkin's variety and no convincing example of around the eye.
isolated primary Hodgkin's disease in the nasal Lymphoma may expand the nasal cavity (Figs.
region has been presented (Friedmann and Osborn 12.1, 12.2) or cause local bone destruction, which
1982). is detectable in most patients at initial plain radio-
The elderly are the most often affected, presenting graphic examination, and usually seen in the
with nasal obstruction and often a facial swelling. ethmoid cells at the antero-medial border of the
Of 37 patients reported by Wang (1971), two thirds orbit. The latter was involved ab initio in 46% of
were older than 50 years of age and 50% com- the patients seen (Fig. 12.3). These tumours were
plained of an asymmetric swelling of the face and of soft tissue density and none showed calcification.
mouth. Although originally localised, malignant They present no distinctive attenuation features on
lymphoma may extend to adjacent sinuses or the CT. Two patients were examined by magnetic res-
orbit. In the antrum it may extend forwards to onance, and both produced a signal of only mod-
involve the cheek and the palate may be invaded erate intensity on T2 -weighted spin echo sequences
through the antral floor. Regional lymph node on pre-contrast scans. One female patient presented
involvement occurs in 20% of cases and generalised with a non-Hodgkin's lymphoma in the maxillary
lymphadenopathy may sometimes follow. antrum and orbit (Fig. 12.4), and was examined
using gadolinium DTP A. There was no enhance-
ment of the intensity of the signal from the tumour
Radiology and Imaging on the post-contrast scans, either on T j-weighted
spin echo sequences or inversion recovery
Thirteen examples of lymphomata taking origin in sequences, but the tumour was outlined against the
the nose and paranasal sinuses have been inves- enhanced signal from sinus and nasal mucosa (see
tigated: seven were demonstrated by CT and two by Figs. 2.4 and 2.5, p.19), and against the fat in the
magnetic resonance; the remainder were examined orbit and cheek (Fig. 12.5).
126 Lymphoreticular Tumours

Fig. 12.1. Axial CT scan of a soft tissue mass in the anterior nasal cavity due to lymphoma.

Fig. 12.2. Same patient as Fig. 12.1. Coronal CT scan showing Fig. 12.3a.b. Axial (a) and coronal (b) CT scans showing a
nasal lymphoma. lymphoma of the ethmoid cells with massive invasion of the orbit.
Lymphoma 127

Fig. 12.4. Coronal CT scan showing the orbital extension of a

lymphoma of the maxillary antrum.

Fig. 12.6. Coronal hypocycloidal tomogram of an elderly female

with lymphoma. There is a soft tissue mass over the bridge of the
nose, and bone destruction in the nasal bones and adjacent
frontal sinus.

In summary. the diagnosis of lymphoma should

be considered when an elderly patient presents with
a facial swelling in or around the nose that is
accompanied by a mass in the anterior ethmoid cells
and nasal cavity and associated with demonstrable
bone destruction (Fig. 12.6).

Plasmacytoma

Multiple myeloma is a disease of unknown aetiology

which almost always starts in the bone marrow.
Occasionally it may present as a solitary lesion in
bone or as an extramedullary soft tissue tumour.
Extramedullary plasmacytoma may arise in any
structure containing reticulo-endothelial tissue.
and such tumours may either be isolated or form the
initial lesion of a generalised disseminated condition
(Booth et al. 1973). Extramedullary tumours of
Fig. 12.5. Same patient as Fig. 12.4. Sagittal magnetic resonance either form are infrequent. but the head and neck
scan, using the inversion recovery mode, with intravenous gado-
linium DTPA. There is no signal enhancement of the tumour, area - especially the upper respiratory tract. with
which has been outlined against the fat of the orbit and cheek its abundant lymphatic tissue - is by far the most
(arrows). common site. In the series of 192 plasmacytomata
 128 Lymphoreticular Tumours

Fig. 12.7a.b. Axial (a) and coronal (b) CT scans of a plasma-

cytoma. showing a slightly lobulated mass (arrows) in the
alveolar recess of the right maxillary antrum associated with
b local bone destruction.

of the head and neck reported by Castro et al. Radiology and Imaging
(1973). the nose and paranasal sinuses were the
commonest locations and accounted for 37.5% of There are two main tasks for the radiologist in
cases. The nose is the usual site. followed by the evaluating these patients prior to surgery: the first
maxillary antrum (Heatly 1953). Clinically the is to show the extent of the local disease by con-
symptoms are non-specific: epistaxis and rhi- ventional radiographic and imaging studies; the
norrhoea followed by nasal obstruction are the second is to determine the presence or absence of
common presenting features. generalised bone disease by skeletal survey. In some
Plasmacytoma 129

Fig. 12.9. Osteolytic defect in the olecranon. which was associ-

ated with an extramedullary plasmacytoma in the nasal cavity.

when localised to an unusual site (Fig. 12.7). The

diagnosis is also suggested when characteristic skel-
etal changes occur in combination with a destruc-
tive lesion in the nose and paranasal sinuses (Figs.
12.8. 12.9).

References

Booth JB. Cheesman AD. Vincenti NH (1973) Extramedullary

plasmacytoma of the upper respiratory tract. Ann Otol Rhinol
Laryngol 82:709-715
Castro EB. Lewis JS. Strong EW (1973) Plasmacytoma of para-
Fig. 12.8. Plasmacytoma. Axial (a) and coronal (b) CT scans nasal sinuses and nasal cavity. Arch Otolaryngol 97:326-
showing a mass in the nasal cavity. with bone destruction in the 329
Friedmann I. Osborn DA (1982) Pathology of granulomas and
ethmoids and maxillary antrum.
neoplasms of the nose and paranasal sinuses. Churchill Liv-
ingstone. Edinburgh
Greifenstein A (1937) Die Klinik der Retothelsarkome. dergestellt
auf Grund von 31 eigenen Beobachtungen. Arch Nasen Ohren
Kehlkopfheilkunde 143:189-215
reported series less than 50% of tumours in the Heatly CA (1953) Primary plasma cell tumors of the upper
nose and sinuses have shown plain radiographic air passages with particular reference to involvement of the
changes. probably because of the usual location maxillary sinus. Ann Otol Rhinol Laryngol 62:289-306
Lloyd GAS (1987) The orbit and eye. In: Sutton D (ed) Textbook
of this tumour in the nasal cavity. Some form of of radiology and imaging. vol 2. Churchill Livingstone. Edin-
tomography is therefore essential for demonstrating burgh. chap. 51
the soft tissue mass. Generally its appearance is non- Michaels L (1987) Ear. nose and throat histopathology. Springer.
specific. but in some cases plasmacytoma produces a Berlin Heidelberg New York
polypoidal thickening of the mucosa or lobulated Schmidt M (1978) Die Neubildungen in den oberen Luftwegen
in die Krankheiten der oberen Luftwege. Zweite Auflage Kap
appearance with underlying invasion of the soft 18:620-654
tissues or bone (Michaels 1987). This can be shown Wang CC (1971) Primary malignant lymphoma of the oral cavity
on CT and is a distinctive combination. especially and paranasal sinuses. Radiology 100: 151-15 3
13 Tumours of Neurogenic Origin

Peripheral Nerve Tumours part of more generalised neurofibromatosis (Fig.

13.4). In either case it shows as a soft tissue mass
in the roof of the maxillary antrum. causing expan-
There are four varieties of peripheral nerve sion of the infraorbital canal.
tumours. all of which are derived from the pro- Neurofibromatosis affects the orbit and skull gen-
liferation of Schwann cells: erally and the changes which may occur are strik-
1. Neurilemmoma ing and characteristic: enlargement of the orbit is
2. Neurofibroma
3. Plexiform neurofibroma associated with neuro-
fibromatosis
4. Malignant schwannoma
Each of these tumours has been identified in the
nose and paranasal sinuses.
The typical neurilemmoma is encapsulated and
consists histologically of spindle cells arranged in
compact bundles with nuclear palisading and the
characteristic Antoni type A and B areas. The plexi-
form neurofibroma and solitary neurofibroma are
distinguished from neurilemmoma by the presence
ofaxons: they are essentially fibrous lesions with
nerve fibres traversing the tumour. Malignant
schwannomata resemble the pattern of the neur-
ilemmoma histologically. but the cells are obViously
malignant.

Radiology and Imaging

Solitary tumours. usually neurilemmomata. may
occur in the nose and sinuses (Fig. 13.1). One par-
ticular form can be readily diagnosed on plain radi-
ography and by soft tissue imaging techniques. This
is the infraorbital neurofibroma. which is often seen Fig. 13.1. Coronal CT scan showing a solitary neurilemmoma in
as a solitary lesion (Figs. 13.2. 13.3) but may be the right side ofthe nasal cavity.
 132 Tumours of Neurogenic Origin

a .. ~ ____________ ~~~ ___________ b

~ Fig. 13.2a-<:. Neurilemmoma of the infraorbital nerve shown on plain

radiography (a) and on coronal hypocycloidal tomography (b). The sagittal
tomogram (c) demonstrates enlargement of the infraorbital canal.

Fig. 13.3. Soft tissue mass associated with enlargement of the infraorbital
c canal (arrow). At surgery this was shown to be a malignant schwannoma.
T
Peripheral Nerve Tumours 133

Fig. 13.4. Coronal CT scan showing orbital neurofibromatosis

with involvement of the infraorbital nerve (arrow).
a

accompanied by eievation of the sphenoid ridge to

the level of the orbital roof; there is often a large
defect in the greater wing of the sphenoid forming
the posterior boundary of the orbit. producing an
encephalocoele (Fig. l3.5a). The result of these
changes is the so-called empty orbit of neuro-
fibromatosis seen on the postero-anterior radio-
graph (Lloyd 1975). The paranasal sinuses are
usually only secondarily affected. the enlargement
of the orbit associated with plexiform neuro-
fibromatosis causing a deformity and under-
development of the ipsilateral maxillary antrum
(Fig. l3.5b).

b
Fig. 13.5. a Axial CT scan showing orbital neurofibromatosis
Olfactory Neuroblastoma with typical posterior encephalocoele. b Same patient. The
coronal CT scan shows that the orbital neurofibromatosis is
associated with a deformity of the antrum and ethmoid cells.
Olfactory neuroblastoma is a malignant neoplasm
of the olfactory apparatus and is composed of
undifferentiated neuroectodermal tissue. The assimilating the reports of 97 cases. found a bi-
tumour was first described in the literature by modal incidence with the majority of cases in the
Berger et al. in 1924: they called it esthesio- 50-60 year age group but another peak (16%)
neuroepithelioma. Berger and Coutard in 1926 between 11 and 20 years. The age distribution
described another intranasal neurogenic tumour of the tumour is unlike that of adrenal neuro-
differing in histological pattern and named it blastoma. which is almost always a disease of
esthesioneurocytoma. These are now considered to childhood and adolescence.
be subgroups of the same tumour type under the Histological similarities with sympathetic neuro-
generic name of olfactory neuroblastoma; one con- blastoma do exist however. Well-demarcated
tains both nervous and epithelial elements. the lobules of uniform tumour cells with congeries of
other is derived from nervous elements only. The blood vessels are characteristic features. The
tumour arises from neuroectodermal tissue in the tumour cells are small with little cytoplasm. Pseudo-
cribriform plate. upper third of the nasal septum rosettes occur in 50% of cases. Electron microscopy
and along the superior turbinate. It is said to be shows neurofibrils in the lobules and this technique
slightly commoner in women (54%) than in men may be helpful in confirming the diagnosiS
(46%) (Elkan et al. 1979). These authors. when (Michaels 1987). It is recognised that the his-
134 Tumours of Neurogenic Origin

b
Fig. 13.6. a Axial CT scan showing an olfactory neuroblastoma with early invasion of the orbit. b Same patient. The coronal CT
scan shows that the olfactory neuroblastoma has also eroded through the frontal sinus and cribriform plate.

tological features do not necessarily indicate the eight of these also by magnetic resonance. four
clinical aggressiveness of the tumour (Elkan et al. using the paramagnetic contrast agent gadolinium
1979). DTP A. The patients ranged in age from 9 to 67
The presenting symptoms are non-specific. Olsen years; ten were female and fourteen male. The
et al. (1983) and Kadish et al. (1976) noted nasal results of plain film radiography were non-specific.
obstruction in 70% of patients. epistaxis in 50%- the typical changes being those of a soft tissue mass
70%. anosmia. diplopia. epiphora. proptosis and in the nose with clouding of the frontal sinus and
metastatic cervical lymphadenopathy. The tumour ethmoid cells on the affected side. Bone erosion in
usually grows slowly. eroding surrounding bony the region of the cribriform plate is not easy to
structures. The site of origin ensures that the cribri- assess on plain radiographs. Before the introduction
form plate is involved early in the disease. Preformed of CT it was usually adequately shown by coronal
holes in this bone allow rapid penetration intra- hypocycloidal tomography. but this technique has
cranially (Harrison 1984). The latter author been largely superseded by high-resolution CT.
reviewed the surgical pathology in eight patients The olfactory epithelium covers both superior
and described dural involvement and extension into turbinates and the upper part of the nasal septum.
the anterior cranial fossa in the absence of radio- and it is probable that olfactory neuroblastoma
logical demonstration of bone erosion.
The diagnosis of olfactory neuroblastoma is estab-
lished by biopsy. but some characteristic features
may be demonstrated radiologically. It presents as
a mass in the nose and ethmoid air cells. Initially
there is unilateral involvement but later the tumour
extends to both sides of the ethmoid labyrinth. Bone
erosion is evident in most patients at initial exam-
ination and the orbit is often invaded. The feature
of the orbital extension is a displacement of the
orbital contents rather than infiltration by tumour
(Fig. l3.6). Characteristically the anterior cranial
fossa is invaded through the cribriform plate on the
affected side. This may be demonstrated by CT or
magnetic resonance.

Radiology and Imaging

Fig. 13.7. Olfactory neuroblastoma. In this patient the tumour
Twenty-four patients with this tumour have been is shown on coronal CT scan to be invading the horizontal section
investigated. Eighteen were examined by CT and of the frontal sinus.
Olfactory Neuroblastoma 135

takes origin in these areas. Tumour growing in the

superior nasal meatus readily gains access to the
frontal sinus and ethmoids by upward extension
(Figs. 13.6b, 13.7). The thin bone of the fronto-
ethmoidal cells in this area presents little barrier to
invasion of the anterior cranial fossa. The other
route of invasion is directly via the cribriform plate
as described by Harrison (1984) (Fig. 13.8).
The principal task of the radiologist is to map
the extent of the tumour pre-operatively. Although
olfactory neuroblastoma is radiosensitive, it is not
curable by radiotherapy and the correct treatment
is primary cranio-facial resection. In these patients
the most important area of involvement is the
anterior cranial fossa. The surgical exposure
required is to a large degree determined by tumour
extension above the cribriform plate. The other
important area is the orbit. The presence and degree
of orbital involvement will influence the decision to
preserve or exenterate the orbital contents.
In olfactory neuroblastoma the level of enhance-
ment on CT following intravenous contrast is vari-
Fig. 13.8. Coronal CT scan of an early olfactory neuroblastoma. able. Rosengran et al. (1979) reported no
In this patient the tumour has already eroded the cribriform plate enhancement, while Manelfe et al. (1978) noted
from the upper part of the nasal cavity. an increase in attenuation from 30-40 Hounsfield
units to 66-80 after intravenous contrast. Contrast
enhancement is of little value in establishing the
diagnosis but is important for showing the extent

Fig. 13.9. Coronal CT scans of an olfactory neuroblastoma taken after a bolus injection of intravenous contrast. showing the
extension of enhanced tumour above the cribriform plate.
136 Tumours of Neurogenic Origin

siderably in the eight patients examined and in

some the tumour produced very high signal. The
effectiveness of this modality has been increased by
the use of gadolinium DTP A. The most emphatic
demonstration of tumour above the cribriform plate
was shown on inversion recovery sequences fol-
lowing intravenous gadolinium. The nasal mucosa
also enhances with this paramagnetic contrast
agent (Carr and Gadian 1985), so that poor differ-
entiation in the nose between enhanced tumour
and normal mucosa may lead to an overestimate
of tumour size. However in the sinus cavities this
disadvantage is offset by superior discrimination
between tumour and fluid or mucosal thickening.
Tumours producing high signal on Tz-weighted
spin echo sequences may be difficult to distinguish
from retained secretion in the sinus cavities. It is,
however, possible to make this distinction following
intravenous gadolinium. The degree of enhance-
ment of tumour by gadolinium is most marked on
TI-weighted images and is particularly well shown
on inversion recovery sequences (Fig. 13.11). After
intravenous gadolinium discrimination between
tumour and retained secretion or concomitant
inflammatory changes becomes more obvious
because retained secretion in the sinuses is not
affected by the gadolinium. This is clearly dem-
onstrated on the pre- and post-contrast T I-weighted
spin echo sequences shown in Fig. 13 .12.
The magnetic resonance signal characteristics of
olfactory neuroblastoma are almost certainly
related to the highly vascular nature ofthis tumour.
In the eight patients examined by this technique
the typical features were those of an intense or
moderately intense signal on pre-contrast T2-
weighted spin echo sequences, and strong signal
enhancement after intravenous gadolinium on TI-
weighted sequences. A characteristic feature of the
response to gadolinium was an enhancement of
Fig. 13.1 Oa,b. Massive extension of olfactory neuroblastoma into
tumour higher than that of turbinate mucosa on
the anterior cranial fossa shown on sagittal magnetic resonance inversion recovery and less than that of the mucosa
scans. when TI-weighted spin echo sequences were
employed.

of tumour invasion of the anterior cranial fossa.

The normal procedure has been to give a bolus
of contrast immediately prior to direct coronal CT Meningioma
scanning (Lund et al. 1983), so that the enhanced
tumour is outlined against the non-enhanced brain A meningioma in the nose or paranasal sinuses
tissue (Fig. 13.9). may occur as a primary tumour or as the secondary
An alternative and superior way of dem- extension of an intracranial growth.
onstrating the extent of these tumours is by mag-
netic resonance (Fig. 13.10). Like most tumours Primary Meningioma
of the paranasal sinuses olfactory neuroblastoma
produces the strongest signal on T2 -weighted spin Meningiomata are thought to arise from "cap" cells
echo sequences. The signal intensity varied con- or meningocytes located in clusters at the tips of
 Meningioma 137

a b
Fig. 13.11 a.b. Olfactory neuroblastoma demonstrated by magnetic resonance. a Sagittal scan using a T,-weighted spin echo sequence
made prior to intravenous gadolinium DTPA. There is poor discrimination between tumour and retained secretion in the frontal and
sphenoid sinuses. b Inversion recovery sequence made after intravenous gadolinium. Enhancement of the tumour now allows
discrimination between tumour and retained secretion. and the tumour extent is optimally demonstrated.

the arachnoid villi (Kernohan and Sayre 1952). In and Panyathanya (1973). who believed that men-
primary extracranial tumours the meningocyte is ingiomata can arise directly from multipotential
believed to arise from arachnoid cells along cranial mesenchymal cells wherever situated. Men-
nerve sheaths or from embryonal ectopic arachnoid ingiomata arising primarily in the sinuses are rare.
cells. Another theory of origin is that of Shuangshoti Willen et al. (1979) could find only nine examples

a b
Fig. 13.12a.b. Tj-weighted spin echo sequences before (a) and after (b) intravenous gadolinium. Coronal sections through the
sphenoid sinus. The thickened mucosa enhances. the retained fluid centrally does not. This was correctly interpreted as inflamed
mucosa with no tumour present. Note enhancement of tumour tissue below the sphenoid in the nasopharynx.
138 Tumours of Neurogenic Origin

Histologically meningiomata in this situation

show a meningothelial or fibroblastic pattern. in
which tumour cells of epithelial or fibroblastic
appearance are arranged concentrically around
small blood vessels. sometimes with psammoma
bodies (Michaels 1987). Most of them are benign in
that metastases are very rare; but they spread
locally invading foramina (Fig. l3.l3). whilst pres-
sure erosion may result in spread from one cavity
to another. Recurrence after surgery is common.

Radiology and Imaging

Six patients with histologically proven men-
ingiomata arising primarily in the paranasal sinuses
have been investigated. In addition to conventional
X-ray studies all six patients had CT scans and
two were examined by magnetic resonance. The
average age of the patients was 45 years and the
age range 11-75. with two patients in the second
Fig. 13.13. Recurrent meningioma in the sphenoid sinus invad- decade and three in the seventh. Five of these
ing the pterygoid canal and foramen rotundum.
patients had known tumour recurrence. some mul-
tiple; one patient was lost to follow-up.
in the literature. and seven meningiomata were The tumours showed initially as a soft tissue mass
seen in a 20-year period at the Institute of Lar- in the sinus involved. demonstrated either on a
yngology in London (Papavasiliou et al. 1982). plain radiograph or by CT scan (Fig. l3.14). On CT
They have been reported in the nose (Lindstrom and the tumour usually enhanced strongly after
Lindstrom 1969; Kjeldsberg and Minckler 1972; contrast. Calcification was shown in the tumour
Willen et al. 1979). where they may present as a mass in four patients. Hyperostosis was present in
nasal polyp. Tumours have also been reported in two patients ab initio and was shown eventually in
the maxillary antrum (Hill 1962). in the frontal varying degree in all patients following repeat scans
sinus (New and Devine 1947; Majoros 1970) and for tumour recurrence. In four patients this involved
in the sphenoid sinus (Sardar et al. 1979). the cribriform plate and anterior fossa (Fig. l3.15).

b
Fig. 13.14a.b. Axial (a) and coronal (b) CT scans showing meningioma of the ethmoids invading the orbit. Note the hyperostosis in
the cribriform plate area and in the roof of the orbit.
 Meningioma 139

a b

Fig. 13.15a.b. Recurrent meningioma in the ethmoid cells. a Axial cr scan showing calcification and hyperostosis (arrow) . b Coronal
section showing the calcification and hyperostosis.

Two of the recurrent extradural meningiomata may occur in the sinuses: this is the condition
were examined by magnetic resonance. Failure to known as pneumosinus dilatans (see Chap. 6). It
demonstrate hyperostosis and calcification is a may be provoked in the frontal sinuses by an intra-
drawback of this technique and generally it was of cranial subfrontal meningioma. or the sphenoid
less diagnostic value than CT. However. in one sinuses may be affected by a meningioma of the
patient the tumour showed a low-intensity rim (Fig. tuberculum sellae or planum sphenoidale. It may
13.16). a sign which has been described for intra- also occur as the secondary effe.ct of an extradural
cranial meningiomata (Zimmerman et al. 1985; meningioma in the orbit (Lloyd 1985) (see Fig.
Mawhinney et al. 1986). 6.42. p. 68).
Another change characteristic of meningioma

Fig. 13.16. Sagittal magnetic resonance scan of a recurrent meningioma in the nose and sphenoid sinus. The tumour shows a
hypointense border (arrows) .
140 Tumours of Neurogenic Origin

Fig. 13.17. Occipito-frontal view showing hyperostosis on the greater and lesser wings of the sphenoid (right) due to a sphenoidal
ridge meningioma. Note that there is loss of translucence in the right ethmoids and sphenoid sinuses (arrow).

Secondary Meningioma in the Sinuses described five patients with post-operative involve-
ment of the sinuses. In contrast a pre-operative
Secondary involvement of the sinuses by a primary survey of 80 patients with sphenoidal ridge men-
intracranial meningioma is well recognised and ingioma revealed only one patient showing
usually occurs as a recurrence, following surgery invasion of the sinuses. He concluded that surgery
to the intracranial growth (Kendall 1973). Kendall rather than chronicity of the lesion favours the
extracranial spread of meningioma.
Two examples of sphenoid ridge meningioma
invading the sphenoid sinus have been seen prior
to any surgery. One patient presented with proptosis
and a mass in the nasopharynx; biopsy of the latter
confirmed the diagnosis of meningioma (Figs.
13.17,13.18).

References

Berger L, Coutard H (1926) L' esthesioneurocytome olfactif. Bull-

etin de I'Association de France d'etude. Cancer 15:404-421
Berger L, Luc, Richard (1924) L'esthesioneuroepitheliome olfac-
tif. Bulletin de I'Association de France d'etude. Cancer 13:
410-421
Carr DM, Gadian DG (1985). Contrast agents in magnetic res-
onance imaging. Clin Radiol136:561-568
Elkan D, Hightower S, Lim Meng, Cantrell RW, Constable WC
(1979) Esthesioneuroblastoma. Cancer 44:1087-1094
Harrison D (1984) Surgical pathology of olfactory neuro-
blastoma. Head Neck Surg 7:60-64
Hill CL (1962) Meningioma of the maxillary sinus. Arch Oto-
Fig. 13 .18. Same patient as Fig. 13 .17. Lateral skull radiograph laryngoI76:547-549
showing an opacity in the sphenoid sinus and nasopharynx. The Kadish S, Goodman M, Wang CC (1976) Olfactory neuro-
meningioma presented as a mass in the nasopharyflx (arrow) blastoma: a clinical analysis of 17 cases. Cancer 13 7: 15 71-
and the diagnosis was confirmed at biopsy. 1576
Meningioma 141

Kendall B (1973) Invasion of the facial bones by basal men- New GB. Devine MD (1947) Neurogenic tumour of nose and
ingiomas. Br J RadioI46:239-244 throat. Arch OtolaryngoI46:163-179
Kernohan JW. Sayre GP (1952) Tumours of the central nervous Michaels L (1987) Ear. nose and throat histopathology. Springer.
system. Armed Forces Institute of Pathology. Washington DC Berlin Heidelberg New York
Kjeldsberg CR. Minckler I (1972) Meningioma presenting as Olsen K. De Santo L (1983) Olfactory neuroblastoma. Arch Oto-
nasal polyps. Cancer 29:153-156 laryngol 109:797-802
Lindstrom CG. Lindstrom DW (1969) On extracranial meningi- Papavasiliou A. Sawyer R. Lund V (1982) Effects of meningiomas
oma. Acta Otolaryngol (Stockh) 68:451-456 on the facial skeleton. Arch OtolaryngoI108:255-257
Lloyd GAS (1975) Radiology ofthe orbit. SaU)1ders. London and Rosengran J. Bao Shan J. Wallace S. Danzinger J (1979) Radio-
Philadelphia graphic features of olfactory neuroblastoma. AJR 132:945-
Lloyd GAS (1985) Orbital pneumosinus dilatans. Clin Radiol36: 948
381-386 Sadar ES. Conomy JP. Benjamin SP. Levine HL (1979) Men-
Lund VJ. Howard DJ. Lloyd GAS (1983) CT evaluation of para- ingiomas of the paranasal sinuses. benign and malignant.
nasal sinus tumours for craniofacial resection. Br J Radiol 56: Neurosurgery 4:227-231
439-446 Shuangshoti S. Panyathanya R (1973) Ectopic meningiomas.
Majoros M (1970) Meningioma of the paranasal sinuses. Lar- Arch Otolaryngol 98:102-105
yngoscope 80:640-645 Willen R. Gad A. Willen M. Qvarnstrom O. Stahle J (1979)
Manelfe C. Bonafe A. Fabre P. Pessey n (1978) CT in olfactory Extracranial meningioma presenting as a nasal polyp. ORL
neuroblastoma. J Comput Assist Tomogr 2:412-420 41:234-239
Mawhinney RR. Buckley JH. Worthington BS (1986) Magnetic Zimmerman RD. Fleming CA. Saint-Louis LA. Lee BCP. Manning
resonance imaging of the cerebello-pontine angle. Br J Radiol n. Deck MDF (1985) MagnetiC resonance imaging of men-
59:961-969 ingiomas. Am J NeuroradioI6:149-157
14 Tumours of Muscle Origin

Tumours of muscle origin are divided into skeletal muscular tissue or undifferentiated mesenchymal
muscle tumours (rhabdomyoma, rhabdomyo- tissue with a potency for aberrant differentiation
sarcoma) and smooth muscle tumours (leiomyoma, into muscle fibres.
leiomyoblastoma and leiomyosarcoma). Rhabdomyosarcoma in the sinuses is a disease of
the adolescent and young adult. Of 10 patients
investigated 60% were in the second or third
decade; the average age was 26.7 years and the age
range 10-56 years. The common form of pres-
Skeletal Muscle Tumours entation is facial swelling with or without pain.
Other clinical features include epistaxis, nasal
obstruction, epiphora, and proptosis when the orbit
Although benign rhabdomyoma has been found in is involved (90% of patients). Spread to the sphenoid
the nasopharynx, no example of this tumour has sinus and skull base may give rise to cranial nerve
been reported as occurring in the nose and sinuses. palsies and the tumour may invade the neck directly
In contrast rhabdomyosarcoma (which represents via the nasopharynx or by lymph node metastases.
8% of all malignant disease in children) shows a Fu and Perzin (1976) reported 50% cervical node
predilection for the head and neck region especially involvement in their series of 16 rhab-
the orbit, but the nose and paranasal sinuses may domyosarcomata. This is in marked contrast to
also be primarily involved. Histologically the malignant epithelial sinus tumours, where neck
tumour is derived from the malignant rhab- metastases are unusual. The overall clinical picture
domyoblast, and has been classified by Horn and is that of widespread tumour involvement at initial
Enterline (1958) into pleomorphic, embryonal, examination, subsequently confirmed by CT and
alveolar and botryoid sarcoma. The presence of magnetic resonance. This widespread extent of the
cross-striation within the cell structure is the most tumour at presentation would account for the poor
characteristic histological feature. prognosis of rhabdomyosarcoma in the nose and
The fact that the nose and paranasal sinuses sinuses compared with similar tumours of orbital
do not normally contain skeletal muscle poses a origin. For example Sutow et al. (1970) recorded a
problem of derivation of these tumours. Some 75% 5-year survival for orbital rhabdomyosarcoma
authors (Cooper 1934) have thought that their as opposed to 21 % survival for patients with rhab-
origin might be from the medial pterygoid muscle domyosarcoma elsewhere in the head and neck.
with secondary invasion ofthe sinuses, but the most Orbital rhabdomyosarcoma characteristically
commonly accepted view is that the rhab- involves the anterior orbit, produces early clinical
domyosarcomata of children and adolescents are signs and does not as a rule invade bone until a late
derived from embryonic tissue, either immature stage of the disease.
144 Tumours of Muscle Origin

Smooth Muscle Tumours muscle origin one was a leiomyoblastoma and two
leiomyosarcomata. The age range of the patients
with skeletal muscle tumours was 11-56 years and
In the nose and sinuses smooth muscle tumours. the average age 28.5 with a peak distribution in
either benign or malignant. are extremely rare. Lei- the second decade. The two patients with a lei-
omyoma has so far only been reported in the nasal omyosarcoma were aged 47 and 56 respectively
cavity. Leiomyosarcoma is also found in the nose and the single patient with a leiomyoblastoma was
but may in addition involve the paranasal sinuses. 5 years old (Papavasiliou and Michaels 1981). All
The only smooth muscle normally present here is patients were examined by conventional radi-
in the vasculature. and some believe that this tissue ography and eleven by CT. In addition three patients
constitutes the origin of leiomyomatous tumours. had magnetic resonance scanning.
although their derivation from multipotent mes- The results of these investigations can be sum-
enchymal cells is at least an equally valid possibility. marised as follows:
Histologically the tumours form interlacing bundles 1. All patients were shown to have a soft tissue
of spindle cells in which "myofibrils" may be dem- mass in the nose or paranasal sinuses by plain
onstrated by appropriate staining. This feature is radiography. This was confirmed by tomography-
constantly found in leiomyoma and in the greater whether conventional. computerised or magnetic
proportion of leiomyosarcomata (Stout and Hill resonance tomography.
1958). The sarcomatous smooth muscle tumours
have a very poor prognosis. spreading to involve
more than one cavity in the sinuses. often with
secondary spread to the orbit. and producing sys-
temic metastases typically in the lung fields. Spread
to cervical lymph nodes may also occur.

Radiology and Imaging

In a period of 7 years 12 patients with histologically

proven muscle-derived tumours of the nose and
paranasal sinuses have been investigated. Nine had
skeletal muscle tumours. all of which were rhab-
domyosarcomata. Of the three tumours of smooth

b
Fig. 14.1. Axial CT scan of a'leiomyosarcoma of the maxillary Fig. 14.2a. b. Axial (a) and coronal (b) CT scans in a 16-year-old
antrum. expanding the posterior wall of the antrum into the female showing extensive rhabdomyosarcoma of the nose and
infratemporal fossa (arrow). sinuses with bone destruction and invasion of the orbit.
Radiology and Imaging 145

2. In all patients the tumour involved the nasal extension (Figs. 14.1. 14.2. 14.3 and 14.4). Only
cavity and adjacent maxillary antrum. the nasal cavity was involved in the child with the
3. Of the nine patients with rhabdomyosarcoma. leiomyoblastoma (Fig. 14.5).
in eight (89%) the ethmoids were also involved and
in five (55%) the sphenoids or frontal sinuses were
involved by tumour.
4. Tumour calcification was not observed. In
some patients displaced fragments of sinus wall
were incorporated within the tumour mass but no
new bone formation or ectopic calcification was
demonstrable.
5. At initial exarpination the orbit was found to
be involved in eight (89%) of the patients with
rhabdomyosarcoma and in both patients with lei-
omyosarcoma. in addition to infratemporal fossa

Fig. 14.4. Axial magnetic resonance scan showing a very large

rhabdomyosarcoma of the maxillary antrum in a 41-year-old
male. There is massive invasion of the infratemporal fossa.

Fig. 14.3a. b. Axial (a) and coronal (b) CT scans of a 21-year-old Fig. 14.5. Coronal hypocydoidal tomogram showing bone
male with a rhabdomyosarcoma of the maxillary antrum. There erosion and expansion of the nasal cavity in a 5-year-old. This
is invasion of the orbit and pterygo-palatine fossa. was histologically a leiomyoblastoma or epithelioid leiomyoma.
146 Tumours of Muscle Origin

a b

Fig. 14.6a-c. Same patient a Fig. 14.4. agittal

(a). coronal (b) and axial (c) magnetic resonanc
sections showing cervical Involvement In ainu
rhabdomyo arcoma. A parapharyngeal cxten-
Ion of the tumour I hown (arrow). which, a
un u peeted clinically or on cr.

6. Magnetic resonance studies were carried out fossa to the parapharyngeal region. This was unsus-
in three of the cases of rhabdomyosarcoma. In all pected clinically or by CT scan and only recognised
there was a high-intensity signal from the tumour on the magnetic resonance scans (Fig. 14.6). The
on Trweighted spin echo sequences. At pres- demonstration of the full extent of the tumour was
entation these tumours were all well advanced. The a result of the wide coverage achieved by three-
high incidence of neck involvement by this tumur plane multislice imaging using a head coil, which
is well recognised (Fu and Perzin 1976), and two allowed total scanning of the head and neck. This
of these patients had cervical involvement when is a major advantage of the method and. in this
scanned. One had direct extension of the tumour respect it has revolutionised the imaging of head
from the maxillary antrum and pterygo-palatine and neck tumours.
Radiology and Imaging 147

References Horn RL. Enterline HT (1958) Rhabdomyosarcoma: a clinico-

pathological study and classification of 39 cases. Cancer
11:181-199
Cooper KG (1934) Plasmacytoma and rhabdomyoma of the Papavasiliou A. Michaels L (1981) Unusual leiomyoma of the
paranasal sinuses. Arch Otolaryngol 20:329-339 nose (leiomyoblastoma): report of a case. J Laryngol Otol
Enzinger FM. Weiss SW (1983) Soft tissue tumours. Mosby. St 95:1281-1286
Louis Stout AP. Hill WT (1958) Leiomyosarcoma of the superficial soft
Fu Y. Perzin KH (1976) Non-epithelial tumours of the nasal tissues. Cancer 11:844-854
cavity. paranasal sinuses and nasopharynx., V. Skeletal muscle SutowWW. SullivanMP. RiedHL. TaylorHG. Griffith KM (1970)
tumours (rhabdomyoma and rhabdomyosarcoma). Cancer Prognosis in childhood rhabdomyosarcoma. Cancer 25: 13 84-
37:364-376 1390
15 Fihro-osseous Disease

The principal conditions included under the bodies because of their similarity to the psam-
heading of fibro-osseous disease are fibrous dyspla- momata of meningioma.
sia, ossifying fibroma and benign osteoblastoma. In Osteoblastoma is a benign neoplasm which con-
the nose and sinuses accurate differentiation of sists microscopically of a vascular fibrous stroma
these lesions on histological grounds can be difficult. containing irregular trabeculae of bone and osteoid
The criteria used for the different entities show some surrounded by proliferating osteoblasts (see Chap.
overlap and the problem is further compounded 18).
by the variable histology which is often found in
different parts of the same lesion (Michaels 1987).
In particular the dividing line between fibrous
dysplasia and ossifying fibroma is unclear. Some Fibrous Dysplasia
authors regard the latter as a variety of monostotic
fibrous dysplasia, while others maintain that it is a
separate entity. The aetiology of this disorder of bone formation is
Fibrous dysplasia is a condition in which normal unknown. In the past some authors have attributed
bone is replaced by fibrous tissue of spindle cells and the initiation of fibrous dysplasia to trauma, but the
poorly formed trabeculae of woven bone with an most widely held view is that it is a developmental
irregular shape and distribution. Ossifying fibroma defect derived from embryological faults, since the
may be considered as a localised form of this con- lesions arise in childhood, enlarge during the period
dition but differs in that there is the presence of of body growth, and cease to grow after puberty.
osteoid, which is not often found in fibrous dyspla- There are monostotic and polyostotic forms of the
sia. Unlike fibrous dysplasia, in ossifying fibroma disease. The monostotic type may involve any ofthe
the lesion is well delimited from the surrounding facial bones but the maxilla is the most commonly
bone and at the periphery the abnormal bone may affected. The polyostotic variety of the condition can
show a lamellar structure frequently rimmed by occur with or without systemic changes. In the
osteoblasts. A more distinctive histological pattern female the association of polyostotic fibrous dyspla-
is often seen in these lesions. This was referred to sia with sexual precocity and cafe au lait pig-
as calcific spherulation by Sherman and Sternberg mentation of the skin is known as Albright's
(1948) and more recently Margo et al. (1985) have syndrome.
used the term "psammomatoid ossifying fibroma". Irrespective of the site of origin of the disease it is
Juvenile ossifying fibroma and cementifying fibroma predominantly one of young subjects and since its
are names that have also been given to this change. common location is in the maxilla it usually pre-
The histological pattern is that of small spherular sents with swelling and deformity of the cheek,
masses composed of calcified material, osteoid or sometimes associated with nasal obstruction, prop-
bone and sometimes referred to as psammoma-like tosis and disturbances of vision. Some cases are
 ISO Fibro-osseous Disease

a b
Fig. 1S.la, b. Axial (al and coronal (b) CT scans of a 21-year-old female with an ossifying fibroma of the ethmoids. There is typical
"ground glass" opacity and very clear demarcation of the mass from normal structures.

asymptomatic. the lesion being identified as an inci- appearances of ossifying fibroma and fibrous dyspla-
dental finding. sia is that the former is a unilocular lesion while
fibrous dysplasia may be multilocular, not confined
to one part of the bone and often shows diffuse
hyperostosis. Later Sherman and Glauser (1958)
Ossifying Fibroma described the radiological changes in 1 7 histo-
logically proven cases of fibrous dysplasia of the
jaws, and described three types radiologkally: one
The name ossifying fibroma was originally used showing diffuse homogeneous sclerosis tending
by Montgomery (1927) to describe fibro-osseous generally to follow the contour ofthe bone; a second
lesions of the jaws, and since the introduction of this type having a multiloculated osteolytic appearance,
term there has been much argument concerning the oval in shape, with septa and frequently calcific
histological classification of the condition: whether strands or flecks; and a third type presenting a
it constitutes a separate entity - a benign tumour unilocular pattern, which could not always be dis-
composed of cellular fibrous tissue containing bone tinguished from ossifying fibroma.
or osteoid - or simply a variant of fibrous dysplasia.
The radiological evidence for ossifying fibroma
was first presented by Sherman and Sternberg
(1948). These authors reported the radiographic
changes in 12 patients with the condition in the
maxilla and mandible, and they described what
they believed to be characteristic appearances. The
picture was that of: (1) a unilocular osteolytic
lesion, oval or spherical in shape; (2) a distinct
boundary to the lesion described as "egg shell' in
character; (3) progressive enlargement with the for-
mation of spherical densities in the mass; and (4)
a unique growth change in the maxilla: the
dissolution of adjacent bone without pressure
displacement.
Some of these tumours were said to be out-
standing microscopically in that they showed exten-
sive spherulation, a change synonymous with that
described subsequently as "psammomatoid" by
Margo et al. (1985). Sherman and Sternberg (1948) Fig.1S.2. Axial CT scan showing expansion of the left antrum
suggested that the dissimilarity in the radiographic due to an ossifying fibroma .
Radiology and Imaging 151

Radiology and Imaging

Since the papers described above there has been a

revolution in imaging techniques which has
resulted in much new information concerning fibro-
osseous disease affecting the nose and paranasal
sinuses. Thirty-one patients with this disease have
been investigated: 16 with fibrous dysplasia and 15
with ossifying fibroma.
The diagnosis was based on clinical and radio-
logical evidence initially, but histological evidence
was obtained in a third of the cases classified as
fibrous dysplasia and all those of ossifying fibroma.
Fig. 15.3. Same patient as Fig. 15.2. Axial magnetic resonance All patients were examined by plain radiography
scan. The diagonal bar of calcification is shown as an area of and the majority had conventional tomography.
signal void. Fourteen had CT scans (7 in each category) and 3
had magnetic resonance studies. The histology was
that of fibro-osseous disease, and the majority of
the cases diagnosed as ossifying fibroma showed
spherulation and psammomatoid appearances. In a
few patients the latter change was present in some
sections whilst others showed the trabecular
pattern usually associated with fibrous dysplasia.
There was therefore no clear-cut histological dis-
tinction between the two conditions.

Fig. 15.4. Same patient as Fig. 15.3. Axial CT scan showing

nodule of calcification in the posterior part of the mass (arrow).

Fig.lS.6. Lateral plain radiograph showing typical "ground

Fig. 15.5. Axial CT scan of an ossifying fibroma in a 38-year-old glass" density of fibrous dysplasia affecting the maxilla and sphe-
female. noid bone.
IS2 Fibro-osseous Disease

Fig.lS.7. Four axial CT scans showing the dense bone changes of fibrous dysplasia of the maxilla and sphenoid bone. The sphenoid
is extensively affected and the enlarged bone is encroaching on the orbit. and on the middle and temporal fossae of the skull.

The imaging changes in this series were of two 15.2-15.5). In some patients a mixture of these
sorts: either a dense, "ground glass" appearance two processes was apparent, but the changes were
similar to that seen in the long bones in fibro- essentially the same whether the lesion was classi-
osseous disease (Fig. 15.1) or a more osteolytic fied as fibrous dyspiasia or ossifying fibroma.
process with expansion of the bone and strands or However in fibrous dysplasia the lesion was more
nodules of dense calcification within the lesion (Figs. diffuse with widespread involvement of one or

a b

Fig. 15.8a-<:. Axial (a) and coronal (b) CT scans showing fibrous dysplasia ofthe maxillary antrum. c See opposite.
Radiology and Imaging 153

occasionally two of the bones forming the facial

skeleton (Figs. 15.6, 15.7 and 15.8), while in oss-
ifying fibroma the lesion was discrete, usually round
(Fig. 15.9) or oval in shape, confined to a part of
one bone and frequently appeared encapsulated
(Fig. 15.1). One particular feature of fibrous dyspla-
sia affecting the maxilla is its typical situation in
the lateral wall of the antrum and zygoma, with
encroachment of the dense bone on the antral
cavity (Figs. 15.10, 15.11).

Fig. 15.8. (continued). c A more posterior coronal scan shows that

the sphenoid is also affected.

Fig. 15.10. Coronal hypocycloidal tomogram showing fibrous

dysplasia of the maxilla. There is typical encroachment by the
dense bone on the lumen of the maxillary antrum.

Fig. 15.9. Lateral hypocycloidal tomogram of an ossifying Fig. 15.11. Axial CT scan of a child with fibrous dysplasia of the
fibroma in the sphenoid sinus of a 14-year-old male. It appears maxilla. showing bone expansion with encroachment on the
as a discrete rounded mass (arrow) arising from the pituitary antral cavity.
floor.
154 Fibro-osseous Disease

Fig. 15.12. Ossifying fibroma shown on axial cr scan. This was associated with pneumosinus dilatans (see Chap. 6).

a b

Fig.15.13a,b. Axial (a) and coronal (b) cr scans using wide window settings showing a ballooned expansion of the orbital roof. The
lesion was partially cystic at surgery. Note the solid tissue within the lumen ofthe frontal sinus. This showed typical "psammomatoid"
ossifying fibroma on microscopy.
Radiology and Imaging 155

Fig. 15.14. Same patient as Fig. 15.13. Axial magnetic resonance

scan. The T,-weighted image shows high signal from the area of a
expansion in the orbital roof.

Two further changes may be seen when the facial

bones and sinuses are affected by fibro-osseous
disease:
1. Three patients in this series showed abnormal
dilatation of the sinus cavities when their bony
walls were affected by fibro-osseous disease; this
was the condition known as pneumosinus dilatans.
Two of these patients were females with bilateral
fibrous dysplasia of the maxillae. In both the antra
were enlarged and the orbits encroached upon (Figs.
6.43 and 6.44. pp.69 and 70). The third patient
was a 24-year-old male who was shown to have an
ossifying fibroma growing into the orbit from the
ethmoid labyrinth. This was accompanied by dila-
tation of the adjacent fronto-ethmoidal air cells
b
(Fig.lS.l2) (see Chapter 6).
2. Three patients presented radiologically with a Fig. 15 .15a.b. Same patient as Fig. 15.14. Coronal (a) and sagittal
ballooned expansion of the orbital roof. and were (b) magnetic resonance scans of the ossifying fibroma expanding
shown histologically to have ossifying fibroma. the orbital roof.
Margo et al. (1985), in reviewing a large series of
patients presenting in this manner. regarded this
change as unique to the orbit and diagnostic of
psammomatoid ossifying fibroma (POF). There was.
however. evidence in two of the three patients we In conclusion it would seem from a practical point
investigated that the horizontal part of the frontal of view that there is justification in distinguishing
sinus was involved and it may well be that this between fibrous dysplasia and ossifying fibroma
expansion of the diploe of the orbital roof stems even though there may be no clear demarcation of
from frontal sinus pathology (Figs.lS.l3. 15.14 pathogenesis (Friedmann and Osborn 1982). The
and 15.15). Other authors have described frontal fact that ossifying fibroma may enlarge after the
sinus involvement by ossifying fibroma (Corbet cessation of skeletal growth (Williams and Faccini
1951; Thomas and Kaspar 1966); and Lehrer 1973). and that very large examples of this encap-
(1969) reported that these lesions in the orbital roof sulated lesion can be removed without recurrence
could be mistaken for a frontal sinus mucocoele. A (Lund 1982). makes the distinction important in
cystic form of ossifying fibroma has also been seen tems of clinical management and assessment for
in the other paranasal sinuses (Figs. 15.16. 15.17). surgery.
156 Fibro-osseous Disease

Fig. 15.16. Cystic ossifying fibroma in a 9-year-old male. Axial CT scans showing expansion of the sphenoid. ethmoid and maxillary
sinuses with low attenuation.

Fig. 15.17. Same patient as Fig. 15.16. T,-weighted axial magnetic resonance scans showing high signal from the expanded areas.
The lesion gave no signal on the inversion recovery sequences. and the spin characteristics were typical of a cystic mass or mucocoele
in the sinuses.
Radiology and Imaging 157

Margo CE. Ragsdale BD. Pernan KI. Zimmerman LE. Sweet DE

References (1985) Psammomatoid Ouvenile) ossifying fibroma of the orbit.
Ophthalmology 92:150-159
Michaels L (1987) Ear. nose and throat histopathology. Springer.
Berlin Heidelberg New York
Corbet CC (1951) Calcifying or ossifying fibroma of frontal sinus. Montgomery AH (192 7) Ossifying fibromas ofthe jaw. Arch Surg
J Laryngol Otol 65:607-608 15:30-44
Friedmann I. Osborn EA (1982) Pathology of granulomas and Sherman RS. Glauser OJ (1958) Radiological identification of
neoplasms of the nose and paranasal sinuses. Churchill Liv- fibrous dysplasia of the jaws. Radiology 71:553-558
ingstone. Edinburgh Sherman RS. Sternberg WCA (1948) The roentgen appearances
Lehrer HZ (1969) Ossifying fibroma of the orbital roof: its dis- of ossifying fibroma of bone. Radiology 50:595-609
tinction from "blistering" or "intraosseous meningioma". Thomas GK. Kaspar KA (1966) Ossifying fibroma of the frontal
Arch NeuroI20:536-541 bone. Arch Otolaryngol 83:43-46
Lund VJ (1982) Ossifying fibroma. J Laryngol Otol 96:1141- Williams JL. Faccini JM (1973) Fibrous dysplastic lesions of the
1147 jaws in Nigerians. Br J Oral Surg 11: 118-12 5
16 Fibrous Tissue Tumours

Fibromatosis

Fibromatosis is the name given to a locally aggress-

ive fibrous tissue tumour which grows slowly and
infiltrates locally but does not metastasise. It may
cause morbidity and even death due to local infilt-
ration which is difficult to control surgically. The
condition is best categorised as a less aggressive
form of fibrosarcoma.
The lesion consists of fibrocytes with a benign
appearance and associated with abundant collagen.
Fu and Perzin (1976) described six patients with
this disease with ages ranging from 2 to 61 years.
Presenting symptoms were nasal obstruction or a
mass in the maxillary area associated with pain
and epistaxis. The radiographic changes in these
a
patients consisted of clouding of one or more of the
paranasal sinuses, with focal bone destruction in
two patients; in one there was increased bone
density suggesting fibrous dysplasia.
Two patients with this condition have been seen,
both of whom were 2-year-old children who were
examined by CT scan. There was expansion of the
maxilla involving the antrum (Fig. 16.1) and in one
case this was associated with areas of increased
density and was indistinguishable from the changes
seen in fibrous dysplasia (Fig. 16.2). Overall the
appearances were those of a benign expansile mass
rather than a radiologically malignant lesion.

Fibrosarcoma
b
Fig. 16.1a, b. Fibromatosis in a 2-year-old child. Coronal (a) and
In the past a variety of other lesions have been axial (b) CT scans showing a cyst-like expansion of the maxillary
misdiagnosed as fibrosarcoma in the nose and antrum.
160 Fibrous Tissue Tumours

affecting the deep structures of the head and neck

(six in the sinuses), listed 21 synonyms including
such names as sclerOSing haemangioma, derm-
atofibroma, xanthofibroma and fibroxanth-
osarcoma. Some tumours previously diagnosed
as fibrosarcoma were in fact examples of this neo-
plasm (Friedmann and Osborn 1982). Perzin and
Fu (1980) have described nine examples of this
tumour in the nose, paranasal sinuses and naso-
pharynx. These authors believe that fibrous histi-
ocytoma is most probably derived from
undifferentiated mesenchymal stem cells that have
the ability to differentiate into two different path-
ways - one fibroblastic, the other histiocytic - the
proportion of these two elements varying in dif-
Fig. 16.2. Fibromatosis. Axial cr scan showing expansion of the
bone with a soft tissue mass in the lower part of the maxillary
ferent lesions.
antrum and areas of nodular calcification. These appearances The age distribution of fibrous histiocytoma is
could not be distinguished from fibrous dysplasia. broadly based, ranging from early childhood to the
ninth decade; the sexes are affected equally. The
clinical picture has no specific features, the common
form of presentation being nasal obstruction, epis-
paranasal sinuses and in fact the tumour is rare. Fu taxis and proptosis when the orbit is affected. The
and Perzin (1976) reported 13 examples out of 2 56 maxillary sinus is most often involved but the nasal
non-epithelial tumours, and Jackson et al. (1977) cavity and the ethmoids may also be affected. Histo-
only 3 cases out of 115 malignant neoplasms. logically the tumour consists of spindle cells
The tissue of origin is uncertain but fibrosarcoma arranged in a storiform pattern (meaning "like a
is thought to arise from the periosteum of bone rush mat") with histiocytic cells, foam cells and
(Hoggins and Brady 1962), and is generally con- multinucleate giant cells. Mitoses both normal and
sidered to be a tumour of fibroblasts, consisting of atypical may be seen. The recurrence rate is high
fusiform cells separated by collagen. Mitotic figures and long-term survival exceptional.
are always present in variable numbers and are
most numerous in the poorly differentiated
tumours. The neoplasm does not produce bone or
cartilage but new bone formation may be found at
the edge of the lesion due to reactive change in the Radiology and Imaging of
adjacent bone. The tumour has a strong tendency Fibrosarcoma and Malignant
to recur, but metastases are unusual except in the
poorly differentiated neoplasms. Fibrous Histiocytoma
Fibrosarcoma has a broadly based age dis-
tribution and usually presents clinically as a facial
mass with pain and sometimes nasal obstruction. Three examples of malignant fibrous histiocytoma
The only known aetiological agent is irradiation, and four patients with fibrosarcoma have been
and the literature includes a number of reports of investigated. The imaging features were similar in
this tumour occurring after radiotherapy (Pettit et both varieties of tumour and were non-specific.
al. 1954; Cade 1957). They showed bone expansion and destruction in
the sinuses (Figs. 16.3, 16.4) without new bone
formation, calcification or reactive change in the
bone. In both tumour types there was one example
of a tumour arising in the infratemporal fossa and
Malignant Fibrous Histiocytoma involving the antrum secondarily (Figs. 16.5, 16.6).
One undifferentiated fibrosarcoma took origin in the
frontal sinus and had invaded the anterior cranial
In 1973 Townsend et al. described the first case fossa at initial examination. A feature of the three
of fibrous histiocytoma in the nose and paranasal malignant fibrous histiocytomata was the relentless
sinuses. Before that date the condition had been advance ofthe tumour, with many recurrences (Fig.
described under a variety of other names. Blitzer et 16.7) necessitating multiple CT investigations and
al. (1977), in a review of29 examples ofthis tumour repeated surgery.
Radiology and Imaging of Fibrosarcoma and Malignant Fibrous Histiocytoma 161

Fig. 16.3. OCcipito-mental view showing massive bone destruc-

tion in the maxilla by a fibrosarcoma.

Fig. 16.6. Axial CT scan showing a malignant fibrous histi-

ocytoma arising in the infratemporal fossa (arrow) and indenting
the posterior wall of the maxillary antrum.

Fig. 16.4. Fibrosarcoma. Coronal CT scan showing expansion of

the maxillary antrum and bone destruction.

Fig. 16.7. Recurrent malignant fibrous histiocytoma in the infero-

medial orbit shown on coronal CT. A maxillectomy had been
performed 2 years previously.

References

Blitzer A. Lawson W. Biller HF (1977) Malignant fibrous histi-

ocytoma of the head and neck. Laryngoscope 87:1479-1499
Cade S (1957) Radiation induced cancer in man. Br J Radiol 30:
3393-3402
Fig. 16.5. OCcipito-mental view showing expansion of the infra- Friedmann I. Osborn DA (1982) Pathology of granulomas and
temporal fossa and erosion of the maxillary antrum by a large neoplasms of the nose and paranasal sinuses. Churchill
fibrosarcoma. Livingstone. Edinburgh
162 Fibrous Tissue Tumours

Fu Y. Perzin KH (1976) Non-epithelial tumours of the nasal cavity. paranasal sinuses. and nasopharynx: a clinico-patho-
cavity. paranasal sinuses and nasopharynx. VI. Fibrous tissue logical study. XI. Fibrous histiocytomas. Cancer 45:2250-
tumours. Cancer 37:2912-2928 2266
Hoggins GS. Brady CL (1962) Fibrosarcoma of the maxilla. Oral Pettit YD. Chamness JT. Ackennan LV (1954) Fibromatosis and
Surg Oral Med Oral PathoI15:34-38 fibrosarcoma irradiation therapy. Cancer 7:149-158
Jackson RT. Fitz-Hugh GS. Constable WC (1977) Malignant Townsend GL. Neel HB. Weiland LH. Devine KD. McBean JB
neoplasms of the nasal cavities and paranasal sinuses. (1973) Fibrous histiocytoma of the paranasal sinuses. Arch
Laryngoscope 87:726-736 OtolaryngoI98:51-52
Perzin KH. Fu Y (1980) Non-epithelial tumours of the nasal
17 Cartilaginous Tumours

Chondromata are very rare in the nose and para- netic resonance scans, one using the paramagnetic
nasal sinuses. Some arise from the nasal septum contrast agent gadolinium DTP A. The average age
and are found incidentally during examination of of the patients in the series was 48.2 years with
the nose; others occur in the ethmoid cells. Kilby an age range of 12-71 and a bimodal distribution
and Ambegoakar (1977) found on review of the peaking in the third and seventh decades. The dis-
literature that 50% of reported tumours arose from tinctive feature of these tumours is the presence of
the ethmoids and 17% from the septum. It is gen- calcification within the soft tissue mass shown on
erally considered likely that many of the cases that plain radiographs (Fig. 17.1) and conventional or
have been described in the literature as chon-
dromata were in fact chondrosarcomata; and malig-
nant features of the cartilage cells may be seen
in the illustrations accompanying some of these
publications (Michaels 1987).
Chondrosarcomata are less uncommon. They
occur in the nasal cavity and have a wide age
distribution. According to Lichtenstein and Jaffe
(1943) chondrosarcomata arise from mature car-
tilage. An origin from the nasal septum would
explain their presence in the nasal cavity but not
in the sinuses, where they are probably derived from
cartilaginous cell rests. The prognosis for patients
with chondrosarcoma depends upon the resect-
ability of the tumour and the degree of histological
differentiation. They may be cured by total surgical
excision, but posteriorly located tumours involving
the sphenoid and skull base are not totally resect-
able. Overall 5-year survival rates are recorded as
60% (Fu and Perzin 1974) and 77% (Evans et al.
1977). Clinically they present with nasal obstruc-
tion, facial swelling, proptosis and visual dis-
turbances when the orbit is involved.

Radiology and Imaging

Seventeen patients with histologically proven chon-

drosarcomata have been investigated. Sixteen of Fig. 17.1. Plain lateral radiograph showing calcification in a
these were studied by CT and three also had mag- large chondrosarcoma ofthe posterior maxilla and nasopharynx.
164 Cartilaginous Tumours

a b
Fig. 17.2a,b. Axial (a) and coronal (b) CT scans of a chondrosarcoma showing multiple confluent calcifications.

computerised tomography. Calcification was

present in 73% of the tumours: in some it consisted
of one or two punctate areas, extending in some
instances to multiple confluent calcifications (Fig.
17.2). However the most typical changes seen were
dense irregular plaques of calcification scattered
throughout the mass. When present they are diag-
nostic of chondrosarcoma (Figs. 17.3, 17.4).
The location of these tumours is predominantly
naso-ethmoidal (93%), the antra and sphenoids
being less frequently involved. Extension to the orbit
was present in 60% of cases and to the anterior and
middle fossae of the skull in 66%. Nearly 50% of
the tumours were centrally located in the nose and
sinuses, some entirely confined to the nasal cavity
a

Fig. 17.4a,b. Coronal (a) and axial (b) CT scans showing irregular
Fig. 17.3. Axial CT scan showing dense punctate calcification in plaques of calcification in a chondrosarcoma of the ethmoids
a chondrosarcoma. invading the orbit.
 Radiology and Imaging 165

a b

between the peripheral and central areas of the

mass. The outer, more cellular layers of tumour
showed strong enhancement, while the chon-
dromatous core was unaffected and gave low signal
(Figs. 17.7, 17.8).

c
Fig 17.5a-c. Three examples of chondrosarcoma of the nasal
septum shown on coronal CT scans, a Shows central necrosis in
the mass. c Shows a typical dense plaque of calcification within
the tumour.

and clearly taking ongm from the septum (Fig.

17.5). The septal chondrosarcomata were more fre-
quently seen in the younger age group but not
exclusively so. These tumours gave relatively low
signal on all magnetic resonance sequences in the
three patients examined. This allows good dif-
ferentiation of tumour from retained secretion on
the Trweighted spin echo sequences. however, and
the extent of the tumour is still optimally dem- Fig. 17.6. Sagittal magnetic resonance scan of an extensive
chondrosarcoma of the nose and sinuses. The tumour'shows
onstrated, especially in sagittal section (Fig. 17.6). relatively low signal on a Trweighted spin echo sequence. but
The tumour in the patient receiving intravenous there is good differentiation of tumour from retained secretion in
gadolinium DTP A showed differential enhancement the frontal and sphenoid sinuses.
166 Cartilaginous Tumours

b
Fig. 17.7a,b. Coronal magnetic resonance scans of a chondrosarcoma. a TJ-weighted spin echo sequence before contrast. b Same
sequence using intravenous gadolinium DTP A as contrast agent. There has been differential enhancement of the tumour, the more
peripheral and microscopically cellular part showing enhanced signal. The central. chondromatous part of the tumour does not
enhance.

Fig. 17.8. Same patient as Fig. 17.7. Sagittal inversion recovery

magnetic resonance scan after 12 cc of intravenous gadolinium
DTPA. The more cellular peripheral part of the tumour shows
enhanced signal. The central chondromatous part is unaffected
by the paramagnetic agent.

References

Evans HL, Ayola AG, Romsdahl MM (1977) Prognostic factors

in chondrosarcoma of bone. Cancer 40:818-831
Fu y, Perzin KH (1974) Non-epithelial tumours of the nasal
cavity, paranasal sinuses and nasopharynx. III. Cartilaginous
tumours. Cancer 34:453-463
Kilby D, Ambegoakar AG (1977) The nasal chondroma. Two
case reports and a survey of the literature. J Laryngol Otol 91:
415-426
Lichtenstein L, Jaffe HL (1943) Chondrosarcoma of bone. Am J
Pathol 19:553-589
Michaels L (1987) Ear, nose and throat histopathology. Springer,
Berlin, Heidelberg New York
18 Osteogenic Tumours

Osteoma occurring with or without associated sinus infec-

tion. Large osteomata in the frontal or ethmoid
sinuses frequently cause proptosis either by direct
Osteoma is the commonest tumour to arise in the invasion of the orbit or by the formation of a muco-
paranasal sinuses, and is said to occur in 1% of coele from a blocked fronto-nasal duct (Fig. 18.2).
individuals (Mehta and Grewal 1963). It is seen OccaSionally upward extension into the anterior
more frequently in the frontal sinus, and less so in cranial fossa (Fig. 18.3) may lead to erosion of the
the ethmoids; it is rare in the sphenoid sinus and dura and a fistula into the cranial cavity or brain,
antrum. with an ensuing CSF leak, meningitis, aerocoele or
There are two varieties: the ivory type showing brain abscess. This is especially likely to occur after
predominantly lamellar bone with irregularly dis- surgery of the larger osteomata~
persed vascular spaces and some woven bone; and Multiple osteomata in the paranasal sinuses are
cancellous osteoma showing a predominantly found in Gardner's syndrome (Gardner and Plenk
lamellar structure. It has been stated that ivory 1952) (Fig. 18.4). They are associated with benign
or compact osteoma arises in bone preformed in tumours and cysts ofthe skin such as dermoids, and
membrane, and cancellous osteoma in bone derived multiple intestinal polyps in the colon and rectum
from cartilage. However Atallah and Jay (1981), in
their review of 23 sinus osteomata removed surg-
ically, showed that all were composed of cancellous
bone surrounded by peripheral dense compact bone.
The proportions of each varied considerably, some
tumours being largely composed of compact and
others of cancellous bone. These authors suggested
that the histological pattern is a reflection of the
rate of bone growth rather than origin from mem-
brane or cartilage. The osteomata also showed an
age discrepancy, occurring at an earlier age in
patients from the Middle East than in the European
group, which showed maximum occurrence in the
third and fourth decades.
The majority of sinus osteomata are asympto-
matic and are found during routine sinus and skull
radiography (Fig. 18.1). In those patients who do Fig. 18.1. Typical asymptomatic osteoma in the right frontal
develop symptoms the commonest is facial pain, sinus shown on routine sinus radiography.
168 Osteogenic Tumours

which show a marked tendency to malignant Radiologically osteoma is the easiest tumour to
degeneration. One family reported by Gardner demonstrate in the sinuses. It is satisfactorily shown
showed a history of nine deaths from large bowel by plain radiography (Figs. 18.1, 18.4) and rarely
carcinoma in five generations. are any more sophisticated techniques required.
However, osteomata of the more cancellous variety
may show relatively low density and their full extent
is then better shown by CT.

Benign Osteoblastoma

This condition was first given the title osteogenic

fibroma by Lichtenstein (1951) and described as
giant osteoid osteoma by Dahlin and Johnson
(1954). The more appropriate name of benign
osteoblastoma was used by Jaffe (1956) and Lich-
tenstein (19 56). The lesion is very rare in the
sinuses, but involvement of the maxilla has been
reported by Borello and Sedano (1967) and Kent et
Fig. 18.2. Secondary mucocoele formation in the frontal sinus al. (1969); and Fu and Perzin (1974) described one
due to a large osteoma. tumour in the ethmoid region.
Microscopically the lesion consists of numerous
trabeculae of osteoid or immature bone surrounded
by osteoblasts, the intervening spaces being occu-
pied by a highly vascular stroma containing multi-
nucleate giant cells. A single example of this
condition has been investigated in a 9-year-old
female. The tumour was massive but well cir-
cumscribed (Fig. 18.5) and showed homogeneous
density on CT that was marginally above soft tissue
attenuation but consistent with the pattern of his-
tology described for osteoblastoma.

Osteosarcoma

Although it is the commonest primary tumour of

the bony skeleton, osteosarcoma is much less
common in the nose and sinuses. Garrington et al.
(1967) estimated that approximately 6.5% of all
osteosarcomata arise in the jaws, the maxilla and
sinuses being less frequently involved than the man-
dible. The disease may follow irradiation of the same
bone and, as in the long bones, osteosarcoma may
be a complication of Paget's disease (Fig. 18.6). The
tumour affects an older age group than skeletal
osteosarcoma, which predominates mainly in the
second decade of life. In the jaw the tumour has a
wider age distribution, with ages ranging from 15 to
Fig. 18.3. Huge osteoma expanding upwards into the anterior 50 (Garrington et al. 1967) and 30 to 69 (Windle-
cranial fossa from the ethmoid cells. Taylor 1977).
Osteosarcoma 169

Fig. 18.4a,b. Gardner's syndrome. Plain radiograph (a) and axial hypocyc\oidal tomogram (b) showing one large ethmoid osteoma
invading the orbit and several smaller osteomata in the ethmoid labyrinth (arrows). The patient had had a colectomy for large bowel
polyps.
170 Osteogenic Tumours

Fig. IS.6. Paget's disease of the skull. with osteosarcoma of the

maxilla (arrow).

but one involved the sphenoid and another was

located in the frontal sinus. Three patients were
male and four female. The average age was 38 years
and the age range 11-66 years.

Fig. IS.Sa,b. Benign osteoblastoma. Massive example of the

tumour shown by axial (a) and coronal (b) CT scans. Despite its
size. the tumour remained well demarcated and was removed
successfully.

The main clinical features are those of a visible

swelling and pain in the upper jaw, sometimes with
loosening ofthe teeth or dentures. Nasal obstruction
and epistaxis follow when the tumour invades the
nose, and proptosis when the orbit is affected.
Macroscopically the tumour presents the features
of an irregularly calcified destructive tumour, which
histologically is composed of spindle cell sarcoma
with osteoid and immature neoplastic bone for-
mation.

Radiology and Imaging

Seven patients with this condition have been inves-
tigated: five by CT, two by magnetic resonance and
two by conventional techniques only. The majority Fig. IS.7. Massive new bone formation in a recurrent osteo-
of tumours (five patients) took origin in the maxilla, sarcoma of the maxilla and zygoma.
Osteosarcoma 171

Fig. 18.9. Osteosarcoma of the maxilla. Axial CT scan shOWing

massive tumour with bone destruction and areas of ill-defined
calcification or new bone formation.

patients examined. This is frequently ill defined and

may be diffuse or nodular and of variable density.
Fig. 18.8. Osteosarcoma of the sphenoids. Lateral tomogram It gives the appearances both on' plain radiography
showing new bone formation around the sphenoid sinus. and on CT of being freshly formed (Figs. 18.8, 18.9).
These areas may coexist with relatively well-defined
nodular calcification which is radiologically indis-
Basically these tumours present an osteolytic tinguishable from chondrosarcoma (Fig. 18.10).
process as in other malignant sinus tumours, but It might be anticipated that in the presence of
they are characterised by new bone formation (Fig. osteoid and new bone formation osteosarcoma
18.7), which was present in all but one of the would give low signal on magnetic resonance. This

Fig. 18.10. Osteosarcoma. (Same patient as Fig. 18.9.) Coronal CT sections showing nodular calcification or new bone formation
indistinguishable from a chondrosarcoma.
172 Osteogenic Tumours

Fig. 18.11. Coronal magnetic resonance section of a recurrent Fig. 18.12. Same patient as Fig. 18.11. Axial magnetic resonance
osteosarcoma in the paranasal sinuses. The TJ-weighted scan section. On T2-weighted spin echo sequences using a long time
shows no discrimination between tumour in the left antrum and to echo and a long repetition time there is strong signal from
inflammatory mucosa in the right. inflamed mucosa in the right antrum and a very low-intensity
signal from the left antrum containing recurrent osteosarcoma.

was so in one patient but the other showed mod- Ewing's Sarcoma
erately high signal on Tz-weighted spin echo
sequences with areas of signal void corresponding
to the nodular calcification shown on CT. One Ewing's sarcoma is an uncommon round cell
patient was scanned for a recurrent tumour. By tumour of bone which was first identified as a sep-
using spin echo sequences with a long time to echo arate entity by Ewing in 1921. It is a locally aggress-
and a long repetition time it was possible to dis- ive neoplasm occurring predominantly in male
criminate with total accuracy between tumour and patients in the first three decades of life and usually
inflammatory changes in the sinuses (Figs. 18.11, affecting the long bones. In the head and neck the
18.12). tumour is rare. It may occur in the mandible and

a b
Fig. 18.13a,b. Coronal (a) and axial (b) CT sections showing Ewing's sarcoma in the anterior ethmoid cells invading the orbit and
anterior cranial fossa.
Ewing's Sarcoma 173

sometimes in the maxilla, involving the maxillary References

antrum.
Brownson and Cook (1969) reported the first case
to appear in an otolaryngological journal and found Atallah N. Jay MM (1981) Osteomas of the paranasal sinuses. J
10 cases in the general literature. Four additional LaryngoI0t0195:291-309
patients with Ewing's sarcoma in the maxilla were Borello ED, Sedano HO (1967) Giant osteoid osteoma of the
maxilla. Oral Surg Oral Med Oral Pathol 23:563-566
reported by Ferlito (1978). This author described Brownson RJ. Cook RP (1969) Ewing's sarcoma of the maxilla.
the tumour as a solitary lesion occUrring between Ann Otol Rhinol Laryngol 78:1299-1304
the ages of 5 and 20 years which could be identified Dahlin DC. Johnson EW (1954) Giant osteoid osteoma. J Bone Jt
by the presence of abundant glycogen in the cyto- Surg [Am] 36:559-572
plasm of the neoplastic cells. All authors stressed Ewing J (1921) DIffuse endothelioma of bone. Proc NY Pathol
Soc 21:17-24
the non-specific nature of the radiological changes, Perlito A (1978) Primary Ewing's sarcoma of the maxilla. J
emphasising the lack of the "onion peel" character LaryngoIOtoI92:1007-1024
peculiar to Ewing's tumour in the long bones. Fu y, Perzin KH (1974) Non-epithelial tumours of the nasal
A more recent publication is that of Howard and cavity. paranasal sinuses and naso-pharynx. Cancer 33:
1289-1305
Lund (1985), who described a case of primary Gardner RJ, Plenk HP (1952) Hereditary pattern for multiple
Ewing's sarcoma arising from the ethmoid cells and osteomas in a family group. Am J Genet 4:31-36
extending via the cribriform plate to the anterior Garrington GE, Scofield HH. Comyn J. Hooker SP (1967) Osteo-
cranial fossa. The patient was a 14-year-old male sarcoma of.the jaw. Analysis of 56 cases. Cancer 20:377-391
Howard D, Lund VJ (1985) Primary Ewing's sarcoma of the
who presented with recurrent swelling over the ethmoid bone. J Laryngol 0t0199:1019-1023
bridge of the nose associated with a firm mass at Jaffe HL (1956) Benign osteoblastoma. Bull Hosp Jt Dis 17:141-
the medial canthus and swelling of the middle tur- 151
binate. On CT the anterior ethmoid cells and right Kent JL. Castro HF. Girotti WR (1969) Benign osteoblastoma of
upper nasal cavity were occupied by a well-defined the maxilla. Oral Surg Oral Med Oral PathoI27:209-219
Lichtenstein L (1951) Classification of primary tumours of bone.
soft tissue mass, which was beginning to expand Cancer 4:335-341
into the orbit and anterior cranial fossa (Fig. 18.13). Lichtenstein L (1956) Benign osteoblastoma. Cancer 9:1044-
Mter surgical removal the tumour was shown to 1052
have the typical histological features of Ewing's Mehta BS. Grewal GS (1963) Osteomas of the paranasal sinuses
along with a case report of an orbito-ethmoidal osteoma. J
sarcoma. LaryngoIOtol77:601-610
Windle-Taylor PC (1977) Osteosarcoma of the upper jaw. J Max-
illofac Surg 5:62-68
Subject Index

Acute sinusitus 41--4 Basi-sphenoid dehiscence 37 dental 52

complications 42--4 Benign osteoblastoma 168 dentigerous 52
Adenocarcinoma 101-3 Bilateral anophthalmos 12 dermoid 54
clinical features of 101 Blow-out fractures 32-3, 36 globulo-maxillary 54
CT scan 102-3 Brown tumour 89,90 hydatid 55
imaging 102 incisor canal 54
magnetic resonance 102-3 intrinsic 51
radiology 102 Capillary haemangioma III mucous retention 51
Adenoid cystic carcinoma ] 03-5 Cartilaginous tumours 163-6 non-secreting 51
clinical features 10 3 Cavernous haemangioma odontogenic keratocysts 52
CT scan 104-5 clinical features 121 of paranasal sinuses 51-3
imaging 104-5 CTscan 121 radicular 52, 53
magnetic resonance 104-5 Cerebral abscess 44
radiology 104-5 Cerebrospinal fluid, see CSF
Albright's syndrome 149 Choanal atresia 21 Encephalocoele 133
Allergic sinusitis 44-5 Cholesteatoma 52 Encephalo-meningocoeles 23-6
Ameloblastoma 105-6 Cholesterol granuloma 52, 78-80 Entomophthorosis 83
clinical features 105-6 examples of 80 Epithelial tumours 95-110
radiological features 106 histological appearance 79 malignant 99-108
Angiofibroma magnetic resonance 80 Ethmoid carcinoma 100
CTscan 50 radiological features 80 Ethmoid cells 10
magnetic resonance 50 Chondroma 163-6 fractures of 36-7
Angiolymphoid hyperplasia with CT scan 163-6 Ethmoid labyrinth 8
eosinophilia 122--4 radiology and imaging 163-6 Ethmoid mucocoeles 57-9
Angiosarcoma Chondrosarcoma 20, 163-6 Ewing's sarcoma 172-3
(haemangioendothelioma) 120 CT scan 163-6 CTscan 173
clinical features 120 magnetic resonance 165, 166 External nose, normal anatomy 7
CTscan 120 radiology and imaging 163-6
Antral cavity 79 Chronic fibrotic sarcoidosis 77
Antro-choanal polyp 49-50, 98 Chronic sinusitis 44 Fibro-osseous disease 67-70, 149-57
diagnosis of 49-50 Computerised tomography (CT) 13- CT scan 151-5
Antro-nasal melanoma 108 15 magnetic resonance 151-5
Aspergillosis 85-7 axial scans 14 radiology and imaging 151-5
calcification 98 blow-out fractures 32-3 Fibromatosis 159
CTscan 85-7 contrast medium 14-15 Fibrosarcoma 159-60
fulminant 85 coronal scans 14 CT scan 160
imaging features 85-7 intrathecal contrast agent 39 radiology and imaging 160
invasive 85-7 technique . 14 Fibrous dysplasia 69, 70, 149
magnetic resonance 87 see also under specific applications Fibrous tissue tumours 159-62
non-invasive 85,87 Congenital dehiscence 38 Fractures
Aspergillus 85 Congenital disease 21-7 blow-out 32-3, 36
Aspergillus flavus 85 Congenital nasal masses 21-6 CSF leaks 37
Aspergillus fumigatus 85 Congenital venous malformations 122 LeFort injuries 31-2
Conventional tomography 13 malar 29-31
Basal encephalo-meningocoeles CSF fistula 37, 39 of ethmoid cells 36-7
spheno-ethmoidal 25 CSF leakage 21 of frontal sinus 33--4
spheno-orbital 25 CSF rhinorrhoea 35,37 of maxillary antrum 29
transethmoidal 25 Cysts of nasal bones 29
transsphenoidal 25 calcifying odontogenic 53 of sphenoid sinus 34-5
176 Subject Index

Frontal lobe abscess 43 Leiomyoma 143-5 secondary 168

Frontal sinus 7-8 Leiomyosarcoma 143-5 sphenoid 60-1
developmental variations 10 Leprosy 78 Mucormycosis 83
fractures of 33-4 Lupus pernio 77 Multiple myeloma 12 7
Frontal sinus mucocoeles 56-7 Lupus vulgaris 77 Muscle-derived tumours 143-7
Lymphoma 19,125-7 radiology and imaging 144-6
CTscan 125 Mycotic disease 83-8
Gadolinium DTPA 16, 18-20, 100, diagnosis of 12 7
125 imaging 125 Nasal bones 6
Gardner's syndrome 167,169 radiology 125 fractures of 29
Genito-urinary tumour 108 Lymphoreticular tumours 125-9 Nasal cavity, normal anatomy 7
Giant cell lesions 89-93 Lymphosarcoma 12 5 Nasal dermoids 21-3
CTscan 91-2 Nasal encephalocoele 21
magnetic resonance 92 Nasal glioma 21, 23
radiological features 91 Magnetic resonance tomography Nasal polyposis 45-50
Giant cell reparative granuloma 89, (MRT) 15-20 CTscan 48
92,93 choice of pulse sequences 16 magnetic resonance 48
Giant cell tumour 89, 92 inversion recovery pulse Nasal polyps 17
Glomerulonephritis 73 sequence 15 Nasal sarcoid 77
Granuloma 73-82 multi-slice facility 16 Necrotising arteritis 73
paramagnetic contrast agents 16 Neurilemmoma 131
principle and method of CT scan 131
Haemangioendothelioma, see examination 15 radiology and imaging 131-3
Angiosarcoma proton relaxation 16 Neurofibroma 115,131
Haemangiopericytoma 115,118-20 pulse sequences 15 Neurofibromatosis HI, 133
clinical features 118-19 saturation recovery pulse Neurogenic tumours 131-41
CTscan 119 sequence 15
diagnostic feature of 120 slice thickness 16 Oblique projection 1
radiological features 119-20 spin echo pulse sequence 15 OCcipito-frontal projection 1
vascularity of 120 technique and application 16-17 Occipito-mental projection 1
Hearing loss 38 tissue characteristics 16-1 7 Odontogenic keratocysts 52
Hereditary haemorrhagic see also under specific applications Olfactory neuroblastoma 133-6
telangiectasia III Malar fractures 29-31 CT scan 134-6
Hodgkin's disease 125 Malignant fibrous histiocytoma 160 magnetic resonance 134-6
CTscan 160 radiology and imaging 134-6
radiology and imaging 160 Orbital cellulitis 43-4
Infraorbital neurofibroma 131 Malignant melanoma 106-7 Orbital venography 122
Infratemporalfossa 113,115,120 clinical features 106 Osler-Weber-Rendu syndrome III
Inverted papilloma 95-9 CT scan 107 Ossifying fibroma 149, 150
calcification 98 radiology 107 Osteogenic tumours 167-73
clinicalfeatures 9 5 Malignant schwannoma 131 Osteoma 167-8
CT scan 95 Mastoid antrum 78 Osteomyelitis 42-3
differential diagnosis 98-9 Maxillary antrum 8, 12, 78, 79, 80 Osteosarcoma 168-72
imaging 95-7 fractures of 29 CT scan 170-2
malignant change 96-7 mucocoeles of 63 magnetic resonance 172
recurrent 95,99 Maxillectomy 10 1 radiology and imaging 170-2
Medial pterygoid plate 114
Juvenile angiofibroma 49,111-18 Meningioma 67-70,136-40 Paget's disease 168, 170
age of onset III CT scan 138-9 Pancreatic carcinoma 109
bone changes 113 diagnosis of 140 Papilloma, clinico-pathological
clinical features III magnetic resonance 13 9 study 97
CT scan 115 primary 136-9 Paranasal sinuses
diagnosis 112 radiology and imaging 138-9 cysts of 51-3
history III secondary 140 development 7
magnetic resonance 115-16 Meningitis 21, 38 normal anatomy 7
plain radiography 113 Metastatic carcinoma 108 tomograms 13
site of origin 112 Middle ear 78 Parry-Romberg disease 26-7
vascular nature of 116 Mid-facial granuloma, radiological Peripheral nerve tumours 131-3
features 73-4 Petrous bones 38
Mid-line lethal granuloma 73 Phleboliths 123
Kimura's disease 122-4 CTscan 74 Phycomycosis 83
magnetic resonance 74 Planum sphenoidale, blistering of 67
Mucocoeles 17,55-65 Plasmacytoma 127-9
Lateral projection I, 6 CT scan 63-5 CT scan 128-9
LeFort I fracture 31 ethmoid 57-9 imaging 128-9
LeFort II fracture 31 frontal sinus 56-7 radiology 128-9
LeFort ill fracture 31,34,36 magnetic resonance 63-5 Plexiform neurofibroma 131
Leiomyoblastoma 143-5 of maxillary antrum 63 Pneumocephalus 35
Subject Index 177

Pneumosinus dilatans 67-70, 154 Septate antrum 9 Syphilis 78

Positive contrast cistemography 39 Sincipital encephalo-meningococoeles Systemic vasculitis 73
Posterior maxillary antrum 112 naso-ethmoid 24
Progressive hemifacial atrophy naso-frontal 24
(PHA) 26 naso-orbital 24 Tomographic techniques 13-20
Psammomatoid ossifying fibroma 149, Skeletal muscle tumours 143-7 Tuberculosis 77-8
154 radiology and imaging 144-6 Tumour demonstration 18
Pterygo-maxillary fossa 112, 113, 119 Skull base tuberculosis 78 Tumour enhancement 18, 20
Pterygo-palatine fossa 113, 119 Smooth muscle tumours 144-6 Tumour of muscle origin 143-7
Pyocoeles 17 radiology and imaging 144-6
Sphenoid mucocoeles 60-1
Radiography Sphenoid ridge meningioma 140 Vascular tumours 111-24
Sphenoid sinus 8,112-14 Venous haemangioma 121
blow-out fractures 32
fractures of 34-5 Venous malformations 121-2
technique 1-6
Spheno-palatine foramen 112-14
Renal carcinoma 108
Rhabdomyoma 143 Squamous cell carcinoma 99-100
CTscan 100 Wegener's granuloma 73
Rhabdomyosarcoma 143-6
plain radiographic changes 99 CTscan 74
recurrent 101 magnetic resonance 74
Sarcoidosis of the nose and sinuses Sub-arachnoid space 38 radiological features 73-4
clinical features 77 Submentovertical projection 1
radiological features 77 Supero-inferior occlusal view 6