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 Table of Contents  
Year : 2022  |  Volume : 60  |  Issue : 2  |  Page : 214-216

Photo Quiz

Cornea and Refractive Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India

Date of Submission15-Jan-2022
Date of Decision17-May-2022
Date of Acceptance20-May-2022
Date of Web Publication30-Jun-2022

Correspondence Address:
Deepthi Mudduluru
Cornea and Refractive Services, Aravind Eye Hospital and Post Graduate Institution, Tirunelveli, Tamil Nadu - 627 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_11_22

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How to cite this article:
Mudduluru D, Venugopal A, Ghorpade A. Photo Quiz. TNOA J Ophthalmic Sci Res 2022;60:214-6

How to cite this URL:
Mudduluru D, Venugopal A, Ghorpade A. Photo Quiz. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Aug 12];60:214-6. Available from: https://www.tnoajosr.com/text.asp?2022/60/2/214/349504

  Questions Top

  1. What is the pathophysiology of this condition? What other systemic features we should look for? [Figure 1]
  2. Name the dystrophy and its classic appearance. What else can mimic this picture? [Figure 2]
  3. What are the systemic associations with this condition? What are the differential diagnosis? [Figure 3]
  4. What could be the probable diagnosis? How to differentiate this condition from pellucid marginal degeneration? [Figure 4]
Figure 1:

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Figure 2:

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Figure 3:

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Figure 4:

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  Photo Quiz Answers Top

  Answers Top

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.
Figure 1: Posterior embryotoxon

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Figure 2: Granular stromal dystrophy

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Figure 3: Keratoglobus

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Figure 4: Pellucid-like keratoconus

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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]


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