CASE

PRESENTATION

DR MUHAMMAD AHMED
PGR EYE-I JHL
HISTORY

- A 13-year-old boy presented in OPD presented with:

- Sudden decrease in vision of Right eye 10 days
HISTORY OF PRESENTING ILLNESS

- The patient was in the usual state of health 10 days back he had a decrease in vision in his right eye
which was:
- Sudden
- Painless
- Watering
- Photophobia Right Eye
- Hazy Cornea
PAST OCULAR HISTORY

- The patient had a history of:

- Frequently changing in the number of glasses.
- Eye rubbing
- Seasonal allergy for the last 6 years

- No history of:
- Ocular trauma
- Surgery
- Contact Lens Wear
PAST MEDICAL HISTORY

- No history of:
- Asthma
- Down Syndrome
- Marfan Syndrome
- Ehlers danlos
- Turner’s
- Osteogenesis imperfacta
- Night blindness
DRUG HISTORY

- The patient has been using:

- Histazolin eye drops 3 times a day
- Softeal eye drops 3 times a day Last 2 years

- No history of steroid usage and PST kenacort.

- No significant personal and family history.

DDx

- Keratoconus

- Acute hydrops

- VKC

- Keratoglobus

- High myopic astigmatism

- Acute corneal edema secondary to increased IOP

OCULAR EXAMINATION
OD OS

Visual Acuity CF 6/60

BCVA CF 6/12

K1 55.45
K-Readings error
K2 62.80

Pupils Hazy view RRR

EOM Full Full

SLIT LAMP EXAMINATION
OD OS

AT(IOP) 08

LIDS B/L Clogging of orifices in lower lids

CONJUNCTIVA B/L palpebral conjunctival hyperemia with micro papillae

CORNEA • Diffuse dull corneal haze • Vogt striae at the level of

• Microcystic epithelial & stroma
stromal edema with Descemet • Prominent nerves superiorly
folds

A/C D/Q D/Q

LENS No view Clear

FUNDUS Hazy View NAD

DIAGNOSIS

- B/L Keratoconus leading to right Acute hydrops.

CORNEA
- The cornea is a transparent, avascular tissue that consists of 5 layers:
- Epithelium (It is composed of 4–6 layers & 40–50 μm thick)
- Bowman layer (I, III, V, VI) (This layer is 15 μm thick and helps maintain the shape of the cornea. If disrupted, it
will not regenerate.)
- Stroma ( I MC, III, V, VI)
- 90% of total corneal thickness
- composed of stromal cells (keratocytes), fibers, and an extracellular matrix.
- The anterior stroma is denser than the posterior stroma due to an increased number of keratocytes
- Descemet membrane (III, VIII, V, VI)
- Its thickness increases with age; at birth, it is 3 μm, increasing to 10–12 μm by adulthood.
- Endothelium
- the concentration is typically highest in the periphery.
- Central endothelial cell density decreases with age at an average rate of approximately 0.6% per year,
- diminishing from a count of about 3400 cells/mm2 at age 15 years
- 2300 cells/mm2 at age 85 years.
- The normal central endothelial cell count in young adults is between 2000 and 3000 cells/mm2.
In adults :

- Cornea measures approximately 11–12 mm horizontally and 10–11 mm vertically

- It is 500–600 μm thick at its center

- Thickness increases gradually towards the periphery.

- Long ciliary nerves

 Corneal Zones
- Clinically, the cornea can be divided into 5 zones
1. central zone: 1–3 mm in diameter; closely resembles a
spherical surface
2. paracentral zone: a 3–4 mm “doughnut” surrounding the
central zone; has an outer diameter of 7–8 mm
3. apical zone: comprises the paracentral and central zones,
used in contact lens fitting; is primarily responsible for the
refractive power of the cornea
4. peripheral or transitional zone: adjacent to the paracentral
zone, has an outer diameter of approximately 11 mm; is the
area of greatest flattening and asphericity in the normal
cornea
5. limbal zone (limbus): where the cornea steepens prior to
joining the sclera at the limbal sulcus; outer diameter
averages 12 mm
Keratoconus
- Keratoconus (KC) is a progressive disorder in which central or paracentral corneal stromal thinning
occurs, accompanied by apical protrusion and irregular astigmatism.

- It can be graded by the highest axis of corneal power on keratometry:

- Mild (<48 D)
- Moderate (48–54 D)
- Severe (>54 D)

- Autosomal dominant transmission with incomplete penetrance has also been proposed.
Risk Factors
- Factors that may play a role in the onset and progression of KC include
1. Genetic predisposition
2. Eye rubbing
3. Atopic disease and allergic keratoconjunctivitis
4. Contact lens wear
5. Abnormalities in collagen that result in hyperelastic joints
6. Ablative keratorefractive surgery in patients with preoperative high myopia or thin corneas
ASSOCIATIONS
 Pathophysiology
- Stromal collagen is primarily affected structurally and biochemically.

- A hallmark of keratoconus is stromal thinning, which may be related to alterations in enzyme levels in
the cornea, causing stromal degradation. This is supported by multiple studies suggesting increased
levels of degradative lysosomal enzymes(matrix metalloproteinase (MMP)-1 and MMP-9) and
decreased levels of inhibitors of proteolytic enzymes (superoxide dismutase, catalase, and
glutathione peroxidase) which leads to oxidative stress in corneal epithelium. These findings are
consistent with the observation of increased collagenolytic and gelatinolytic activity in keratoconic
cells.

- Structural and biochemical changes affect all layers of the cornea from epithelium to endothelium.

- Increased apoptosis of stromal keratocytes has been reported in keratoconus, as suggested by confocal
microscopy. It is postulated that this loss of keratocytes results in a decrease in collagen and
extracellular matrix production, leading to reduced stromal mass.
- Redistribution, degradation, and lysis of collagen fibrils occur, which changes the compactness and
resilience of the tissue.

- The mechanism by which eye rubbing contributes to keratoconus is not completely understood, but it
may be related to mechanical epithelial trauma, triggering a wound-healing response that leads to
keratocyte apoptosis.

- Other factors, such as slippage of collagen fibrils and a decrease in ground substance viscosity, may
play a role. The cytokine interleukin-6 has been suggested as a mediator of eye rubbing and stromal
degradation.

- Overall, KC may be caused by an aberrant tissue response to one or more unidentified stimuli that
subsequently leads to keratocyte depletion or dysfunction, loss of collagen, and ultimately a
biomechanically weak cornea with a degenerated Extracellular Matrix (ECM).
SIGN & SYMPTOMS
- SYMPTOMS
- Progressive changes in vision not easily corrected with eyeglasses.

- SIGNS
- Scissoring of the light reflex on retinoscopy: an early but nonspecific sign of KC; commonly
associated with irregular astigmatism (Video 9-1)
- Munson sign: a late-stage nonspecific sign involving inferior deviation of the lower eyelid contour
on downgaze
- Rizzuti sign: focusing of the light within the nasal limbus when a penlight is shone from the
temporal side; an early but nonspecific sign.
- Oil Droplet (Charleaux’s sign) : dark reflex in the area of the cone on observation
- of the cornea with the pupil dilated using a direct ophthalmoscope.
- Fleischer ring, partial or complete: a brown-colored ring found at the base of the cone formed by the deposition of iron within the basal
epithelium, which becomes narrower and increasingly well-defined with disease progression. It is best seen with the slit lamp using a broad,
oblique beam or diffuse illumination with the cobalt blue filter.

- Vogt striae: fine, parallel lines observed in the posterior stroma at the apex of the cone, which may disappear with the application of
external pressure

- Apical scarring: types commonly seen include

- reticular scarring related to breaks in the Bowman layer
- nummular scarring at cone apex related to contact lens wear
- deep stromal scarring related to prior corneal hydrops
ACUTE HYDROPS
- Acute hydrops is a sudden onset of corneal edema that results from a tear in the Descemet membrane and is usually
observed late in the disease course(caused by a rupture in the stretched Descemet membrane that allows a sudden
influx of aqueous into the cornea with accompanying pain, photophobia, and decreased vision.

- Acute episodes are initially treated with:

- cycloplegia, hypertonic (5%) saline ointment, and patching or a soft bandage contact lens.
- Accelerated resolution has been reported with intracameral gas injection (SF6 or C3F8) in the acute stage. Pupil
dilation and/or inferior peripheral iridectomy may reduce the risk of pupillary block after intracameral gas
injection.

- Risk factors include allergy and eye rubbing.

- Acute hydrops is commonly observed in patients with Down syndrome.

- Spontaneous perforation in KC is rare.

• It is important to caution these patients against eye rubbing because it contributes to disease progression and
increases the risk of hydrops in advanced cases.

Corneal edema typically clears within 3 months(heals within 6–10 weeks) but can lead to posterior stromal scarring.
Diagnostic procedures
- Keratometry readings are steep.

- Videokeratography (VKG)/ corneal topography or tomography(Gold standard)

- Types
- ORB scan (Scanning slit technique)
- Pentacam (Rotating Scheimpflug camera)
- Galilei scan (Double Scheimpflug camera)

- Replaced old techniques

- Keratometry (K readings)
- Placido disc (To see mires)

- ▪ Used to diagnose, and monitor the disease progression, treatment response, and grading of Keratoconus

- (Note: Prior to the scan, Soft CL should be discontinued for 3 days and hard CL should be discontinued for 3
weeks. B-scan should be done if there is no fundal view)
MEASURING IOP IN KERATOCONUS
- In keratoconus or other ectasia, the cornea is thin, so the
tonometer might measure 14 mmHg when the correct measure
might be 19 or 23 mmHg
- In patients with irregular corneal shape, as in keratoconus, it
can be difficult to determine the endpoint using Goldmann but
with GAT, two GAT readings should be obtained: one with the
prism oriented horizontally and the other with the prism
oriented vertically. “The mean of those two measurements is a
better estimate of true IOP
- Pascal Dynamic Contour Tonometer provides more accurate
IOP measurements in these eyes. It is the tonometer that
appears to be least impacted by corneal thickness, corneal
curvature, and irregular astigmatism. “The problem is the
device is difficult and time-consuming to use.”
MANAGEMENT
- Avoid eye rubbing

- Treat VKC (First acute phase with topical/ supra tarsal steroid inj./ systemic med)

- Mild cases (<48 D)

- Spectacles (for mild cases)
- Soft contact lens (Soft toric, custom soft toric)(for mild cases)

- Moderate cases (48 - 54D)

- Rigid contact lens
- RGP
- Hybrid (Rigid center and soft skirt)
- Piggyback (Soft CL + RGP over it)
- Scleral lens (for highly irregular cornea)

- Safety spectacles over contact lens (if thinning is marked)

- ICRS (intracorneal ring segments) implantation
- Corneal collagen cross-linking (CXL)
- Can be combined with ICRS
- Stabilize or even reverse Keratoconus
- Severe cases (> 54 D)
- DALK
- PKP
- BMT (Bowman’s membrane transplant)
- New modality
- In corneal scars
- Indicated in patients in which CXL cannot be done and to avoid DALK/PKP.

- LASIK is Contraindicated

- Keratoplasty becomes an important option under the following circumstances:

- poor vision even with a comfortable stable fitting contact lens (usually due to scarring)
- contact lens intolerance despite the good vision achieved with the lens
- unstable contact lens fit (even with good vision and lens tolerance)
- progressive thinning toward the corneal periphery approaching the limbus, which requires a very
large graft (associated with increased risk of rejection)
- corneal hydrops that fails to clear after several months
 HOW TO STOP PROGRESSION
- Early stages can be treated with glasses, but with progression of the disease into late childhood and
early adulthood, corneal transplantation may be needed to restore sight.

- Corneal collagen cross-linking is a procedure designed to stop the progression of keratoconus or slow it
down.