Characterization of Unique Lens Morphology in a Cohort of Children with Familial Exudative Vitreoretinopathy.

: To characterize the lens morphology and to measure the clinical features of familial exudative vitreoretinopathy (FEVR) in children. : Unique lens changes were observed in a cohort of children with FEVR from March 2015 to November 2017 using slit lamp examination and all the patients underwent cycloplegic refraction, ultrasound A and B, keratometry and fundus fluorescein angiography. : Twelve eyes of eight children with FEVR had unique lens changes. The contraction of the posterior capsule caused unique lens changes resulting in myopia in nine eyes of six children and astigmatism in eight eyes of five children. Retinal lesions in the affected eyes were all stage 1 to 2. Six eyes of three patients underwent lensectomy and intraocular lens implantation due to high anisometropia which could not be corrected by conventional optical correction. During lensectomy, the opacification in the posterior capsule was found to be due to the fibrous membrane that protruded into the anterior vitreous and not due to lens opacification. Three patients had bilateral lensectomy, in two of whom significant macular involvement was observed in one eye and in one of whom significant macular involvement was observed in both eyes. After surgery visual acuity (VA) improved obviously in two eyes without significant macular involvement and did not improve in the four eyes which had significant macular involvement. Among the five patients who did not have lensectomy, one patient was lost to follow-up and one patient had VA improved in both eyes without significant macular involvement. The other three patients did not have much change in VA. : Clinicians should be aware that when a high myopia or astigmatism does not match the corneal curvature and the length of the eye, one should check carefully the changes of lens and fundus after dilating the pupil, to avoid misdiagnosis and missed diagnosis.