Late onset sclerotomy dehiscence in a patient with Marfan syndrome presenting as recurrent episodes of raised intraocular pressure.

A man in his 50s presented to the eye emergency department on three separate occasions complaining of blurred vision and discomfort in the left eye. He had a history of Marfan syndrome and had undergone bilateral 20-gauge (G) pars plana vitrectomy and lensectomy 20 years prior for ectopia lentis. On examination he had epithelial corneal oedema, raised intraocular pressure >40 mm Hg and conjunctival chemosis, which later appeared as a bleb-like conjunctival elevation. Acute treatment with oral acetazolamide and topical ocular hypotensive agents produced a marked reduction in intraocular pressure to 2-4 mm Hg. A presumed diagnosis of a leaking scleral wound was made. He underwent scleral exploration under general anaesthesia and a leaking sclerotomy was uncovered. The defect was repaired successfully using a scleral patch graft. Late dehiscence of a sclerotomy has been reported rarely in patients with Marfan syndrome. This is the first reported case to present atypically with intermittent episodes of raised intraocular pressure rather than with hypotony.
Competing Interests: Competing interests: None declared.
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