PARS-PLANA VITRECTOMY FOR RETINAL-DETACHM ENT IN A PATIENT WITH EHLERS-DANLOS-SYNDROME

Ehlers-Danlos syndrome (EDS) is an hereditary connective tissue disord er caused by defective collagen synthesis, the main features being hyp erelasticity and vulnerability of the skin, recurrent bleeding from fr agile blood vessels,and secondary deformities of the joints. Ocular in volvement is a rare occurrence, e.g., corneal and scleral rupture from minor blunt injury, lens displacement, rhegmatogenous retinal detachm ent. To date, few reports exist concerning the treatment of retinal de tachment in Ehlers-Danlos syndrome,all of them dealing exclusively wit h conventional scleral buckling surgery. Patient and methods: We repor t on a 47-year-old male patient suffering from EDS type VI(so-called o cular type, lysine-hydroxylase deficiency). He presented with rhegmato genous retinal detachment in his only eye. A scleral buckling procedur e was not feasible because of marked scleral atrophy. A three-port vit rectomy was therefore carried out. Results: During the operation, pron ounced choroidal detachment and bleeding developed, subsiding within w eeks postoperatively. Closure of the sclerotomies was difficult due to scleral thinning. Two revitrectomies were necessary because anterior PVR with traction retinal detachment occurred. The last revitrectomy w as performed 18 months ago, and the retina has been completely reattac hed under 5000 cs silicone oil since then. Visual acuity is 0.1. Concl usion: Primary vitrectomy permits successful treatment of retinal deta chment in EDS patients ifa buckling procedure cannot be performed beca use of scleral atrophy. However,serious complications may occur. Surgi cal procedures other than primary vitrectomy should therefore always b e carefully considered, e.g., pneumatic retinopexy, temporary balloon, dura patch with episcleral pocket.