Quizwise 70

Q.1 The iridocorneal endothelial (ICE) syndrome is a disease spectrum that includes-

A) Chandler syndrome (CS),

B) Progressive iris atrophy (PIA)

C) Cogan-Reese syndrome (CRS)

D) All of the above

Ans. D

The iridocorneal endothelial (ICE) syndrome is a disease spectrum that includes Chandler syndrome (CS), Progressive iris atrophy (PIA), and Cogan-Reese syndrome (CRS). The clinical variants are distinguished primarily based on the changes in the iris. Progressive or essential iris atrophy is used when there is predominant iris involvement in the form of extensive iris stromal atrophy and hole formation. Predominant corneal involvement with normal or mild iris stromal atrophy are classified as Chandler syndrome, and those with characteristic iris nodules with any degree of iris stromal atrophy are classified as Cogan-Reese syndrome.

Chandran P, Rao HL, Mandal AK, Choudhari NS, Garudadri CS, Senthil S (2017) Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. PLoS ONE 12(3): e0171884. doi: 10.1371/journal.pone.0171884

Q.2 The pathology of ICE syndrome presents in which layer of the cornea?

A) Corneal epithelium

B) Corneal endothelium

C) Corneal stroma

D) Descemet’s membrane

Ans. B

The ICE syndrome is a progressive disease of the corneal endothelium and is associated with variable features like corneal edema, the hammered silver appearance of the posterior corneal surface, iris abnormalities ranging from corectopia to polycoria or iris nodules, broad-based peripheral anterior synechiae (PAS) and secondary glaucoma.

Chandran P, Rao HL, Mandal AK, Choudhari NS, Garudadri CS, Senthil S (2017) Glaucoma associated with iridocorneal endothelial syndrome in 203 Indian subjects. PLoS ONE 12(3): e0171884. doi: 10.1371/journal.pone.0171884

Q.3 Corneal endothelium in ICE syndrome can be assessed by-
A) Slit lamp biomicroscopy
B) Specular microscopy
C) In vivo confocal microscopy
D) B and C

Ans D

Ultrastructural assessment of the endothelium by in vivo techniques like specular microscopy and in vivo confocal microscopy (IVCM) can thus play a pivotal in confirming the clinical diagnosis. Vis a vis specular microscopy, IVCM has the advantage of superior resolution and less deterioration of image quality in the presence of corneal edema or mild scarring. Abnormal endothelial cells, initially labeled “ICE cells” by Sherrard et al., have subsequently been identified by various groups on both specular and confocal microscopy.

Malhotra, Chintan; Seth, Natasha G; Pandav, Surinder S; Jain, Arun K; Kaushik, Sushmita; Gupta, Amit; Raj, Srishti; Dhingra, Deepika. Iridocorneal endothelial syndrome: Evaluation of patient demographics and endothelial morphology by in vivo confocal microscopy in an Indian cohort. Indian Journal of Ophthalmology 67(5):p 604-610, May 2019. | DOI: 10.4103/ijo.IJO_1237_18

Q.4 Differential diagnoses of ICE syndrome are –

A) Posterior Polymorphous Corneal Dystrophy (PPMD)

B) Axenfeld- Rieger’s syndrome and

C) Aniridia (Iris hypoplasia)

D) All of the above

Ans D

Posterior polymorphous corneal dystrophy (PPMD)

This endothelial corneal dystrophy is an autosomal dominant disease similar to ICE syndrome on microscopic examination, with multilayered endothelial cells that look and behave like epithelial cells or fibroblasts.

Axenfeld-Rieger Syndrome (ARS)

Axenfeld-Rieger syndrome is a bilateral, heterogeneous congenital condition that is autosomal dominant in most cases but can also occur sporadically and may include developmental abnormalities in the anterior chamber angle, iris, and trabecular meshwork. Corectopia, polycoria, ectropion uveae, posterior embryotoxon, and increased intraocular pressure are common ophthalmologic findings with ARS.

Aniridia (Iris Hypoplasia)

Aniridia is a bilateral congenital disease that presents with the absence of the iris (commonly, a rudimentary iris stump exists), cataracts, glaucoma, and corneal pannus.

1. External Disease and Cornea. Basic and Clinical Science Course (BCSC). American Academy of Ophthalmology, 2010-2011; pp344-345.
2. Axenfeld Rieger Syndrome. Eye Wiki. Available from: https://eyewiki.aao.org/Axenfeld_Rieger_Syndrome
3.  Pediatric Ophthalmology and Strabismus. Basic and Clinical Science Course (BCSC). American Academy of Ophthalmology, 2010-2011; pp223-224.