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.