A case of bilateral probable Posner–Schlossman syndrome in a child with familial exudative vitreoretinopathy

Most cases of Posner–Schlossman syndrome (PSS) present as unilateral disease and primarily affect individuals between 20 and 50 years of age. [1] Familial exudative vitreoretinopathy (FEVR) is a hereditary retinal disorder often linked to high myopia. [2] To date, there have been no reported cases of probable bilateral PSS occurring in a pediatric patient with FEVR. A 10-year-old female was diagnosed with bilateral FEVR following fundus fluorescein angiography (FFA). Subsequently, rhegmatogenous retinal detachment developed in the left eye. Five months after scleral encircling and buckling surgery, episodes of alternating elevated intraocular pressure and anterior chamber inflammation were observed in both eyes, presenting a phenotype consistent with, though not entirely typical of, PSS. The patient’s father also has a diagnosis of FEVR. Although no definitive pathogenic gene for FEVR was identified, whole-exome sequencing (WES) of the patient revealed mutations in four potentially deleterious genes. FEVR-related variants may predispose adolescents to bilateral recurrent hypertensive uveitis exhibiting PSS-like features.