High risk of mortality in sickle cell patients requiring pars plana vitrectomy

Abstract

Purpose : Sickle cell disease (SCD) causes vascular occlusion associated with ischemia and increased mortality. SS genotype has been shown to have worse mortality than SC. Major complications of SCD that contribute to premature death include organ failure, acute sickle crisis, and stroke. Since these major complications of SCD are associated with increased vascular occlusion, we wanted to investigate if increased vascular occlusion in the retina could be a sign of worsening disease and therefore, a risk factor for increased mortality. There have not been studies looking at ophthalmic characteristics with SCD mortality. The goal of this study was to evaluate high risk ophthalmic characteristics that could be correlated more with deceased SCD patients.

Methods : This retrospective study included adult SCD patients seen routinely at Montefiore Medical Center (Bronx, NY) from January 2014 to June 2020. Patients were categorized as Deceased Patients (DP, n=57) and Living Patients (LP, n=985). Data on demographics, lab values, comorbidities, and ophthalmic care were obtained from Montefiore EMR (Epic, Verona, WI). Deceased patients were identified either from notification of the primary care provider or hospital records. Statistics were analyzed using R. Wilcoxon ranked sum test was used for continuous variables and Chi Square test was used for categorical variables unless the frequency counts were small (<10) in which case, a Fisher’s exact test was used.

Results : The study included 1036 patients. 26 DP and 484 LP had at least one documented eye exam. Of these, DP were significantly older than LP (p=0.003). This difference was significant for SS patients (p=0.004) but not for SC (p=0.81). There were no significant differences between DP and LP in race or sex. DP were significantly more likely than LP to have had pars plana vitrectomy (PPV) (p=0.007). This significant difference was seen even when stratified for SS (p=0.003). There were no significant differences for diagnosis of proliferative sickle retinopathy (PSR) or laser retinopexy between DP and LP.

Conclusions : Higher rates of PPV in DP suggests that SCD patients requiring PPV may be at a higher risk of death. We recommend that SCD patients requiring PPV should be more carefully monitored regarding their general health.

This is a 2021 ARVO Annual Meeting abstract.

 

Table 1. – Demographics and retinopathy of sickle cell patients SS/SC and DP/LP with documented eye exams.