Refractive Status and Biometric Characteristics of Children With Familial Exudative Vitreoretinopathy

PURPOSE: To compare biometric characteristics between patients with early-stage familial exudative vitreoretinopathy (FEVR) and healthy controls. METHODS: This case-control study included 50 FEVR eyes in stage 1-2 and 50 control eyes matched by age, gender and spherical equivalent (SE). Biometric parameters including axial length (AL), white-to-white diameter (WTW), central corneal thickness (CCT), anterior chamber depth (ACD), lens thickness (LT), pupil diameter, vitreous chamber depth, anterior and posterior corneal surface curvature radius (ACR and PCR), anterior lens surface curvature radius (ALR) and posterior lens surface curvature radius were measured using IOLMaster 700 and compared between cases and controls using paired t-test. Correlations between SE and biometric measures were assessed using Pearson correlation coefficient (r) in cases and controls. RESULTS: Both FEVR cases and matched controls had a mean age of 7.6 years, 48% female and mean SE of −5.3 D (80% myopia). Compared to controls, FEVR eyes had smaller AL (P = 0.009), WTW (P = 0.001), ACD (P < 0.001), and ALR (P = 0.03), but larger CCT (P = 0.02) and LT (P = 0.01). In FEVR eyes, SE was negatively correlated with AL (r = −0.79, P < 0.001), positively correlated with ACR (r = 0.29, P = 0.04) and PCR (r = 0.33, P = 0.02), whereas in controls, SE was negatively correlated with AL (r = −0.82, P < 0.001) and LT (r = −0.34, P = 0.02), positively correlated with ALR (r = 0.29, P = 0.04). CONCLUSIONS: Patients at early stage of FEVR exhibited a unique eye morphology resembling ocular development arrest, which may help to develop screening and early detection tools for FEVR. In FEVR patients, myopia is very prevalent and significantly associated with corneal curvature increase.