Published online: 14.02.

2019
IJRSMS

Rohit Juneja et al 10.5005/jp-journals-10053-0068

CASE REPORT

Once in a Blue Moon, Congenital Anterior Urethral

Diverticulum
1
Rohit Juneja, 2L Manjunath, 3HS Veerendra, 4Ananth S Mathad

ABSTRACT
Anterior urethral valves (AUVs) are rare congenital anomalies
causing lower urinary tract obstruction in children. Although
they are referred to as valves, these obstructive structures often
occur in the form of a diverticulum. Congenital anterior urethral
diverticulum (CAUD) is an uncommon/rare entity in children
which can lead to obstructive lower urinary tract symptoms
and urosepsis. Over the past 20 years, more than 260 cases
have been reported. However, most cases are diagnosed on
prenatal ultrasonography (USG) in early life if patients present
with bilateral hydronephrosis and/or azotemia. Diagnosis is by
ureteroscopy and radiological imaging. Surgical treatment can
be open or endoscopic. Here, we report a new case of CAUD Fig. 1: Anterior urethral valve
and discuss its clinical presentation, diagnosis, and manage-
ment. A brief review of the literature is also presented.
Keywords: Anterior urethral diverticulum, Anterior urethral
valves, Congenital anterior urethral diverticulum, Micturating
cystourethrogram, Transurethral resection, Ultrasonography.
How to cite this article: Juneja R, Manjunath L, Veerendra HS,
Mathad AS. Once in a Blue Moon, Congenital Anterior Urethral
Diverticulum. Int J Recent Surg Med Sci 2018;4(1):32-36.
Source of support: Nil
Conflict of interest: None

Fig. 2: Anterior urethral diverticulum

INTRODUCTION
Anterior urethral valves (Fig. 1) are rare congenital anom-
The CAUD (Fig. 2) is classified into saccular variety
alies causing lower urinary tract obstruction in children.
and globular variety, the former being more common
Although they are referred to as valves, these obstructive
than the latter.2 Several possible causes of the congenital
structures often occur in the form of a diverticulum.
diverticulum have been suggested, including a develop-
Anterior urethral diverticulum (AUD) may be classi-
mental defect of the corpus spongiosum, cystic dilatation
fied as congenital or acquired. Acquired diverticulum is
of the urethral glands, and sequestration of an epithelial
more common than the congenital variety and urethral
nest after closure of the urethral folds.3 The cause and
trauma, catheterization or instrumentation, infection,
effect relationship between AUD and AUV has been
and obstruction can play a role in its pathophysiology.1
extensively debated. Some authors do not distinguish
between AUD and AUV, while others believe that these
1,4
Resident, 2,3Associate Professor are different entity. The diverticulum that is associated
1,3
Department of Urology, SS Institute of Medical Sciences and with AUV is not a true diverticulum because in AUD,
Research Centre, Davangere, Karnataka, India an acute angle is formed between the proximal part of
2
Department of Paediatric Urology, SS Institute of Medical dilated portion and the ventral floor, whereas this acute
Sciences and Research Centre, Davangere, Karnataka, India angle is not present in AUV.4 Over the past 20 years, more
4
Department of General Surgery, SS Institute of Medical than 260 cases have been reported.5
Sciences and Research Centre, Davangere, Karnataka, India The clinical presentation of CAUD is highly vari-
Corresponding Author: Rohit Juneja, Resident, Department able, depending on the child’s age and the degree of
of Urology, SS Institute of Medical Sciences and Research obstruction.6-9 It causes obstruction of male urethra
Centre, Davangere, Karnataka, India, Phone: +919108927347 and complaints of dysuria, dribbling of urine, recurrent
e-mail: [email protected]
urinary tract infection (UTI), or a fluctuant ventral penile

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Once in a Blue Moon, Congenital Anterior Urethral Diverticulum

swelling.10 However, most cases are diagnosed on pre- antenatal history. On examination (Fig. 3), swelling on
natal USG in early life if patients present with bilateral the ventral aspect of the penile urethra was present.
hydronephrosis and/or azotemia.6-9 Swelling was soft, cystic, fluctuant, and compressible,
The treatment options in AUD include endoscopic collapsed completely on manual compression with urine
division of distal lip of diverticulum,11 excision of diver- coming per urethral. Lab studies showed normal urine
ticulum with primary repair,12 marsupialization with analysis, routine blood counts, blood urea, and creati-
staged urethroplasty, or even suprapubic diversion fol- nine. Ultrasound kidney, ureter, and bladder was normal
lowed by definitive repair.13 with no hydronephrosis or hydroureter; kidneys and
Here, we report a new case of CAUD and discuss its bladder were normal. Retrograde urethrography was
clinical presentation, diagnosis, and management. A brief done, suggestive of the presence of diverticulum at the
review of the literature is also presented. level of penile urethral (Fig. 4). Findings were again con-
firmed on cystopanendoscopy. Patient underwent open
CASE REPORT diverticulectomy and urethroplasty for the same (Fig. 5).
Foley removal was done at 10th postoperative day.
We report the case of a 1-year-old child presented to
Postoperative period was uneventful (Fig. 6). Follow-up
pediatric urology outpatient department with com-
of patient was normal after 3 months with good urinary
plaints of dribbling urine and swelling on the surface of
stream and no urinary complaints.
penis since birth. His parents gave history that he never
had a good urinary stream, with dribbling and used to
DISCUSSION
have intermittent spikes of fever with recurrent UTIs.
They denied any history of trauma, burning micturi- Congenital anterior urethral diverticulum may be found
tion, or urethral instrumentation. There was no unusual all along the anterior urethra, but is usually located

A B
Figs 3A and B: Cystic swelling at the penoscrotal junction

A B

Figs 4A and B: Retrograde urethrography s/o diverticulum at penile urethral

International Journal of Recent Surgical and Medical Sciences, January-June 2018;4(1):32-36 33

Rohit Juneja et al

A B

C D
Figs 5A to D: Intraoperative findings and procedure

peri-urethral glands, ruptured syringocele, incomplete

hypospadias, incomplete fusion of a segment of the ure-
thral plate, and sequestration of an epithelial nest after
closure of the urethral folds.5,7-9,18-20 Suter21 proposed
a theory that a diverticulum of the urethra develops
because of epidermal pockets communicating with the
ventral urethral wall.
A urethral dilatation in this region, in the absence of
a corpus spongiosum, may develop into a diverticulum.3
Diverticula can be described as saccular or diffuse.
The saccular type is a localized protrusion from the ure-
thral lumen into the ventral wall of the anterior urethra,
and the diffuse type is a generalized dilatation of the
Fig. 6: Postoperative view entire anterior urethra. A congenital saccular diver-
ticulum may produce anterior urethral obstruction by
between the bulbous and the mid-penile urethra, mostly a valve-like mechanism of its distal lip, which obscures
on the ventral surface and in the anterior urethra with the urethral lumen during filling.22,23 In this case, they
diameter typically between 3 and 5 cm.14-17 would not be true diverticula. In a true CAUD, an acute
The embryology remains unclear. No family pattern angle is formed between the proximal part of the dilated
of inheritance has been detected yet.5 The etiology of portion and the ventral floor.4
this rare condition has provoked controversy for years. The primary differential diagnostic considerations
Various hypotheses include a developmental defect include AUV, dilated Cowper’s gland ducts, and post-
of the corpus spongiosum, cystic dilatation of the traumatic diverticulum.24
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Once in a Blue Moon, Congenital Anterior Urethral Diverticulum

The presence of a penile or penoscrotal mass clinically Quoraishi et al32 adopted an endoscopic approach
and radiologically, which is seen as an accurate filling with incision of the lip of the diverticulum. However,
defect, distinguishes the diverticulum from the valve. these patients can again develop a flap, requiring repeat
Also, the proximal lip of the diverticulum forms an acute procedures.
angle with the rest of the urethra, while the anterior valve In our case, we used the technique of plication of the
forms the obtuse angle.24,25 In dilated Cowper’s gland redundant diverticular wall with good result.33 Some
ducts, a tubular channel is seen in the ventral surface of authors have also advocated making a triangular flap
the bulbous urethra which it parallels. Its termination is which is fitted into the distal lip and double breasting
in the urogenital diaphragm.24,25 of the urethral suture line, as described in literature.12
Congenital anterior urethral diverticulum may present In situations where there are back pressure changes of
itself at any age, from infant to adult. Generally, one-third upper tracts with deranged renal function, urinary diver-
of cases are diagnosed in the neonatal period and one- sion, either by marsupialization of the diverticulum or even
half by 2 years of age in patients who have never been suprapubic cystostomy/vesicostomy is a safer option.11,13
catheterized urethrally.5 In summary, a history of poor urinary stream and
Newborns and infants present with symptoms, such dribbling, recurrent UTI, and a palpable penile or peno-
as fever, diarrhea, and vomiting.10,14,26 Most children with scrotal mass on physical examination strongly suggest the
this condition present with difficulty in initiating micturi- diagnosis of CAUD. A MCUG will confirm the diagnosis.
tion, dribbling of urine, poor urinary stream, or UTI. A Early surgical intervention prevents the onset of uremia.
careful history will reveal that these children never had
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