Abstract
Abstract: :
Purpose: To report the first case in the literature of postoperative pre–retinal hemorrhages in a patient with homocystinuria. Methods: Case report. Results: A 13 year old African American girl with known homozygous homocystinuria presented with bilateral anteriorly subluxated lenses and medically uncontrollable pupillary block glaucoma. The left eye underwent cataract extraction using phacoemulsification with anterior vitrectomy. Postoperative fundus examination revealed diffuse small sub–hyaloid hemorrhages that resolved over 10 weeks. Two months later the right eye underwent pars plana lensectomy and vitrectomy. Tiny pre–retinal hemorrhages were noted to form within seconds of initiating the central core vitrectomy and stabilized as soon as the vitrectomy was stopped. Vitrectomy was re–initiated, and, despite a higher cutting rate and lower suction, new hemorrhages formed. Only a limited core vitrectomy was performed with no attempt to induce posterior vitreous detachment. The hemorrhages resolved over 8 weeks. The patient was not on anti–coagulants in the pre–operative period in either surgery. Conclusions: Patients with homocystinuria have a high risk of arterial and venous thromboembolism. Numerous studies have shown generalized increased stiffness and fragility of large caliber, high resistance vessels due to endothelial damage, smooth muscle proliferation and fibrotic changes in the sub–endothelial space. It is possible that the same pathologic phenomenon also affects small arterioles and capillaries. The minimal trauma induced by surgery on the superficial neurosensory layers of the retina may be enough to cause rupture of these pathologically altered capillaries.
Keywords: retina • vitreoretinal surgery • vascular occlusion/vascular occlusive disease