Spontaneous ciliary body detachment in a Marfan patient: A case of personalized management

We present a case of a patient with Marfan Syndrome who underwent a personalized approach to manage a spontaneous ciliary body detachment and degeneration of the ciliary processes, causing refractive ocular hypotony.

A 20-year-old MFS man, with a history of bilateral juvenile cataract surgery with failed IOL positioning due to subluxation and consequent explantation, was referred to our clinic after the discovery of persistent ocular hypotonia in his left eye for the past two months, non-responder to corticosteroids. Slit-lamp examination showed shallow anterior chamber and aphakia, with chorioretinal folds, optic disc swelling and mild peripheral retinal lifting. Intraocular pressure (IOP) was 4 mmHg. Ultrasound biomicroscopy (UBM) revealed the presence of a flat, annular ciliochoroidal detachment, posterior pole congestion and total ciliary body separation. One-month systemic therapy with corticosteroids revealed ineffective, and a newly performed UBM showed a significant reduction in the number and thickness of the ciliary processes. At this point, he underwent 23-gauge pars plana vitrectomy with silicone oil endotamponade, combined with a focal trans-scleral cryopexy of the ciliary body, 2 mm behind the limbus and one spot per quadrant, in order to favor ciliary body reattachment. Post-operative IOP was 28 mmHg and the choroidal detachment was resolved, with UBM highlighting ciliary body reattachment. Six months after, with good IOP control using topical therapy, silicone oil was removed. One year after, visual acuity had inccreased to 6/10 and a good IOP control was still maintained with eye drops.

A rare case of spontaneous ciliary body detachment in a long standing aphakic MFS patient was successfully managed with focal trans-scleral cryopexy of the ciliary body combined with pars plana vitrectomy and silicone oil endotamponade.