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Ophthalmology · Retina: Stickler syndrome
Sample itemA child has high myopia, a flat midface, micrognathia with cleft palate, early joint problems and hearing loss. The vitreous appears optically empty with membranous strands. Which sight-threatening complication is most likely?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Retinal detachment from giant retinal tears
Stickler syndrome is an autosomal dominant collagen disorder, usually of type II collagen, with abnormal vitreous, high myopia and a high lifetime risk of giant retinal tears and detachment. Prophylactic laser or cryotherapy of the retina may be considered.
Option by option
A. Retinal detachment from giant retinal tears (correct)
Correct. Abnormal vitreous and thin peripheral retina lead to giant tears and detachment.
B. Acute angle-closure glaucoma attacks
Angle closure is not a characteristic complication of Stickler syndrome.
C. Early central retinal vein occlusion
Retinal vein occlusion is a vascular disease of older adults.
D. Corneal ectasia with scarring
Corneal ectasia is typical of keratoconus, not of this collagen disorder.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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