Proliferative Sickle Cell Retinopathy: Outcomes of Vitreoretinal Surgery

Abstract

Purpose: To report the clinical presentation and outcomes in patients who underwent surgery for proliferative sickle cell retinopathy (PSCR).

Design: Retrospective, consecutive case series.

Subjects: All patients who underwent vitreoretinal surgery for complications secondary to PSCR between January 1, 2014, and December 31, 2021, at a university referral center.

Methods: Retrospective consecutive case series.

Main outcome measures: Best-corrected visual acuity (BCVA), single operation anatomic success rate.

Results: The study included 65 eyes of 61 patients. Disease distribution included 24 (44.4%) eyes with hemoglobin SC disease, 14 (25.9%) with hemoglobin SS disease, 13 (24.1%) with sickle cell trait, and 3 (5.6%) with sickle cell-β thalassemia. Preoperative transfusion was not performed in any study patients. Regional anesthesia with monitored anesthesia care (RA-MAC) was utilized in 58 (89.2%) eyes and general anesthesia in 7 (10.8%). In eyes that underwent surgery for retinal detachment (RD; N = 52) the rate of single operation anatomic success was 72.4% with combined scleral buckling/pars plana vitrectomy (SB/PPV; N = 29) compared with 47.8% with PPV alone (N = 23; P = 0.07). Mean BCVA at the last follow-up examination was 1.27 (20/372) in the SB/PPV group and 1.05 (20/226) in the PPV group (P = 0.48). In all SB cases, an encircling band was utilized and there were no known cases of anterior segment ischemia. All eyes that had surgery for vitreous hemorrhage (N = 13) underwent PPV with endolaser and mean BCVA improved from 1.67 (20/944) preoperatively to 0.45 (20/56) at last follow-up examination (P < 0.001). Mean preoperative BCVA, indication for surgery, single operation success rate, and mean BCVA at last follow-up examination did not differ based on sickle cell disease type (P > 0.05).

Conclusions: In patients with RD, SB/PPV achieved slightly higher rates of single operation anatomic success compared with PPV alone. Visual acuity outcomes were similar in the 2 groups. The majority of patients received RA-MAC anesthesia and preoperative transfusions were not performed. There were no cases of postoperative anterior segment ischemia. Hemoglobin SC disease was the most common disease type in the current study and surgical outcomes did not differ between sickle cell disease types.

Financial disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Keywords: Proliferative sickle cell retinopathy; Retinal detachment; Vitreous hemorrhage.