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Ophthalmology · Glaucoma: Sturge-Weber in adults
Sample itemA 35-year-old man with a facial port-wine stain has raised pressure in one eye. Gonioscopy shows a wide-open angle with blood visible in Schlemm canal. Which mechanism best explains the glaucoma?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Raised episcleral venous pressure from the vascular malformation
In adult-onset glaucoma with Sturge-Weber syndrome, the episcleral vessels shunt blood at higher pressure, so the pressure that aqueous must overcome rises. Blood reflux into Schlemm canal on gonioscopy is the clue. Infantile onset in the same syndrome is instead due to an angle anomaly.
Option by option
A. Trabeculodysgenesis from arrested neural crest migration
Trabeculodysgenesis explains the infantile form, but it would not typically present first in a 35-year-old with blood in the canal.
B. Raised episcleral venous pressure from the vascular malformation (correct)
Correct. Raised episcleral venous pressure and blood in Schlemm canal explain adult-onset glaucoma in this syndrome.
C. Reduced trabecular outflow from steroid exposure
Steroid exposure is not described, and it would not produce blood in Schlemm canal.
D. Pupillary block caused by seclusio pupillae
Seclusio pupillae causes pupillary block with a closed angle, but the angle here is wide open.
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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