|Year : 2022 | Volume
| Issue : 2 | Page : 214-216
Deepthi Mudduluru, Anitha Venugopal, Aditya Ghorpade
Cornea and Refractive Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India
|Date of Submission||15-Jan-2022|
|Date of Decision||17-May-2022|
|Date of Acceptance||20-May-2022|
|Date of Web Publication||30-Jun-2022|
Cornea and Refractive Services, Aravind Eye Hospital and Post Graduate Institution, Tirunelveli, Tamil Nadu - 627 001
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mudduluru D, Venugopal A, Ghorpade A. Photo Quiz. TNOA J Ophthalmic Sci Res 2022;60:214-6
| Questions|| |
- What is the pathophysiology of this condition? What other systemic features we should look for? [Figure 1]
- Name the dystrophy and its classic appearance. What else can mimic this picture? [Figure 2]
- What are the systemic associations with this condition? What are the differential diagnosis? [Figure 3]
- What could be the probable diagnosis? How to differentiate this condition from pellucid marginal degeneration? [Figure 4]
| Photo Quiz Answers|| |
| Answers|| |
1. Abnormal migration of neural crest cells of anterior chamber.
Posterior embryotoxon isolated - 8–15% patients-no glaucoma risk
Axenfeld anamoly - along with angle abnormalities + glaucoma risk
Rieger anamoly - along with angle + iris stroma abnormalities + glaucoma risk
Rieger syndrome-associated systemic features
Defects in differentiation, migration or arrest of neural crest cells in facial bones, teeth, cardiovascular system and periumbilical skin can result in systemic features like facial dysmorphism, deafness, congenital heart defects, redundant periumbilical skin.
2. Granular stromal dystrophy. Small, discrete, ray or disc shaped, sharply demarcated greyish-white anterior stromal opacities with intervening clear stroma shows characteristic bread-crumb appearance.
In pearl-type posterior capsule opacification, clusters of residual lens epithelial cells appears as Elschnig's pearls that shine on retro illumination.
3. Keratoglobus associated with other connective tissue disorders like Ehlers–Danlos, Marfan syndrome.
Buphthalomos - >12 mm corneal diameter, corneal edema, Haab's striae, optic disc cupping, elevated intraocular pressure and increased axial length of eyeball
Megalocornea - >12 mm horizontal corneal diameter, no thinning or ectasia
4. A case of pellucid-like keratoconus (PLK).
In elevation-based topography, anterior sagittal curvature map takes a crab-claw appearance in both pellucid marginal degeneration (PMD) and PLK, whereas in thickness map, “bell shape” sign is hallmark of PMD, it is absent in PLK.
On slit lamp examination, in PMD, the maximal corneal protrusion occurs superior to area of thinning than within area of thinning in keratoconus giving the appearance of beer belly.
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Conflicts of interest
There are no conflicts of interest.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]