Abstract
Purpose: To report a pediatric case of Coats plus syndrome that initially presented resembling familial exudative vitreoretinopathy (FEVR). Methods: A single case was analyzed. Results: A pediatric patient was referred at 2 years of age to the retina clinic for exotropia and decreased visual acuity in the right eye and was found to have a dense vitreous hemorrhage. She later developed persistent gastrointestinal bleeding requiring regular blood transfusions and intravenous bevacizumab. Treatment with systemic bevacizumab resolved the patient's cystoid macular edema (CME). Although her presentation and examination were initially suggestive of FEVR, genetic analysis revealed heterozygous biallelic mutations in the STN1 gene, mutations that are known to be associated with Coats plus syndrome. Conclusions: Coats plus syndrome is a rare and life-threatening microangiopathy that affects the retina, central nervous system, and gastrointestinal system. The patient's resulting CME significantly improved with intravenous bevacizumab.