[Retinal detachment in Ehlers-Danlos syndrome. Treatment by pars plana vitrectomy]

Abstract

Ehlers-Danlos syndrome (EDS) is an hereditary connective tissue disorder caused by defective collagen synthesis, the main features being hyperelasticity and vulnerability of the skin, recurrent bleeding from fragile blood vessels, and secondary deformities of the joints. Ocular involvement is a rare occurrence, e.g., corneal and scleral rupture from minor blunt injury, lens displacement, rhegmatogenous retinal detachment. To date, few reports exist concerning the treatment of retinal detachment in Ehlers-Danlos syndrome, all of them dealing exclusively with conventional scleral buckling surgery.

Patient and methods: We report on a 47-year-old male patient suffering from EDS type VI (so-called ocular type, lysine-hydroxylase deficiency). He presented with rhegmatogenous retinal detachment in his only eye. A scleral buckling procedure was not feasible because of marked scleral atrophy. A three-port vitrectomy was therefore carried out.

Results: During the operation, pronounced choroidal detachment and bleeding developed, subsiding within weeks 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 was performed 18 months ago, and the retina has been completely reattached under 5000 cs silicone oil since then. Visual acuity is 0.1.

Conclusion: Primary vitrectomy permits successful treatment of retinal detachment in EDS patients if a buckling procedure cannot be performed because of scleral atrophy. However, serious complications may occur. Surgical procedures other than primary vitrectomy should therefore always be carefully considered, e.g., pneumatic retinopexy, temporary balloon, dura patch with episcleral pocket.