Surgical and Medical Perioperative Management of Sickle Cell Retinopathy: A Literature Review

Abstract

The most common ophthalmic manifestation of sickle cell disease (SCD) is sickle cell retinopathy (SCR), which can lead to loss of vision due to complications of proliferative sickle retinopathy (PSR). Eventually, vitreoretinal surgery may be indicated in these patients to help preserve or improve vision. Unfortunately, SCD can cause systemic and ophthalmic vaso-occlusive and ischemic complications, which necessitates adequate perioperative planning in these patients undergoing surgery. The purpose of this review was to appraise studies of patients with PSR undergoing vitreoretinal surgery to identify the common medical and surgical perioperative measures employed in these cases. The full-texts of 11 original studies published between 1973 and 2018 were reviewed. Over the last 16 years, 7 studies of 108 eyes undergoing posterior segment surgery for vitreoretinal complications of PSR did not report any ischemic complications related to SCD. It is likely that modern surgical techniques dramatically reduced the risk of these complications. Perioperatively, most studies avoided retrobulbar anesthesia and the use of epinephrine, and the use of exchange transfusions is seemingly not required prophylactically in these patients undergoing vitreoretinal surgery. Customized perioperative planning may be required in complex cases, and these patients should be identified and treated through collaboration between ophthalmic surgeons and hematology specialists.