MANAGEMENT OF OPHTHALMIC COMPLICATIONS OF HOMOCYSTINURIA

Objective To determine the safety and efficacy of surgical versus medi cal management in the treatment of ophthalmic complications of homocys tinuria, and also to document ocular complications of homocystinuria o ther than lens dislocation. Design: Retrospective case series. Partici pants: Forty-five patients with ophthalmic complications of homocystin uria participated. Intervention: Eighty-Four surgical procedures were performed on 40 patients. There were 82 procedures done with the patie nts under general anesthesia and 2 with the patients under local anest hesia. Medical therapy was attempted initially in all patients with le ns dislocation and was the sole therapy used for five patients. Main O utcome Measures: Complications resulting from medical or surgical trea tment and final visual acuity were studied. Results: All patients had a history of lens subluxation or dislocation, Fourteen (31%) were rece iving dietary treatment at the time of presentation and 29 (64%) were mentally retarded. Eighty-two procedures were performed with the patie nts under general anesthesia with 2 surgical complications and 1 posts urgical complication. Lens dislocation into the anterior chamber was t he most frequent indication for surgery (50%) followed by pupillary bl ock glaucoma (12%), Prophylactic peripheral iridectomy was not success ful in preventing lens dislocation into the anterior chamber in five p atients. Anesthetic precautions such as stockings to prevent deep veno us thrombosis, preoperative hydration, or aspirin were given in 85% of cases. Other common ophthalmic complications found include optic atro phy (23%), iris atrophy (21%), anterior staphylomas (13%), lenticular opacities (9%), and corneal opacities (9%). Conclusion: Laser iridecto my was unsuccessful in preventing lens dislocation into the anterior c hamber. With appropriate anesthetic precautions and modern microsurgic al techniques, the risks associated with the surgical management of oc ular complications of homocystinuria are reduced. Surgical treatment s hould be considered, especially for cases of repeated lens dislocation into the anterior chamber or pupillary block glaucoma. If a conservat ive, nonsurgical approach is undertaken, these patients must be observ ed carefully for repeat episodes of lens dislocation.