Iridocorneal endothelial syndrome: a mysterious question

¹Université Mohammed V de Rabat, Centre Hospitalier Universitaire Ibn Sina, Hôpital des Spécialités, Ophtalmologie A, Rabat, Morocco

^&Corresponding author
Rida El Hadiri, Université Mohammed V de Rabat, Centre Hospitalier Universitaire Ibn Sina, Hôpital des Spécialités, Ophtalmologie A, Rabat, Morocco

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A 43-year-old healthy woman complained for progressive visual loss over 5 years OD with greyish reflect of his eye. Her ophtalmological and medical history was unremarkable. Visual acuity was light perception OD and 6/6 OS. Adnexal examination and pupillary reflex were normal. She had ocular hypertension estimated at 52 mmHg by Goldmann applanation tonometry. Gonioscopy showed angle closure with multiple peripheral synechiae. On slit-lamp examination we noticed a tear break up time test at 04 seconds with diffuse punctuate keratitis, corneal hypoesthesia, diffuse corneal edema, dyscoria, polycoria with iris atrophy and holes. Endothelial evaluation with high magnification showed "hammered silver" appearance that was difficult to document at slit lamp photography. The crystalline lens was clear and fundus ophtalmoscopy was inaccessible. This constellation of signs was consistent with Iridocorneal Endothelial (ICE) syndrome. The fellow eye was normal. The present case reports clinical findings of an end stage neglected unilateral glaucoma secondary to ICE syndrome. Like our case, this rare pathology presents unilaterally and affects typically young women with negative family history. Our patient was managed medically for dry eye disease and glaucoma.

Figure 1: (A, B) slit-lamp photography showing diffuse corneal edema, dyscoria, polycoria with iris atrophy and holes in direct illumination; (C) dyscoria and polycoria highlightd by retro-illumination; (D) fluorescein eye staining showing diffuse punctuate keratitis