TNOA Journal of Ophthalmic Science and Research

PHOTO QUIZ
Year
: 2022  |  Volume : 60  |  Issue : 2  |  Page : 214--216

Photo Quiz


Deepthi Mudduluru, Anitha Venugopal, Aditya Ghorpade 
 Cornea and Refractive Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Tirunelveli, Tamil Nadu, India

Correspondence Address:
Deepthi Mudduluru
Cornea and Refractive Services, Aravind Eye Hospital and Post Graduate Institution, Tirunelveli, Tamil Nadu - 627 001
India




How to cite this article:
Mudduluru D, Venugopal A, Ghorpade A. Photo Quiz.TNOA J Ophthalmic Sci Res 2022;60:214-216


How to cite this URL:
Mudduluru D, Venugopal A, Ghorpade A. Photo Quiz. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Oct 7 ];60:214-216
Available from: https://www.tnoajosr.com/text.asp?2022/60/2/214/349504


Full Text



 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]{Figure 1}{Figure 2}{Figure 3}{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.{Figure 5}{Figure 6}{Figure 7}{Figure 8}

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.