A Case of Pupillary Block Glaucoma with Familial Exudative Vitreoretinopathy

Purpose: To report a case of angle-closure glaucoma caused by pupillary block in a patient with familial exudative vitreoretinopathy (FEVR).Case summary: A 20-year-old man with FEVR presented with sudden-onset pain in his left eye. The best-corrected visual acuity (BCVA) was 0.04, and the intraocular pressure (IOP) was 76 mmHg. The central anterior chamber depth was reduced to twice the corneal thickness. Gonioscopy revealed 360° angle closure. Anterior segment optical coherence tomography and ultrasound biomicroscopy demonstrated a closed angle and anteriorly convex peripheral iris due to forward displacement of the lens–iris diaphragm and contact between the iris and lens. Trabeculectomy and cataract surgery were performed because the IOP was not controlled despite maximal medical therapy. At 9 months postoperatively, the IOP was 13 mmHg without the use of glaucoma medications, and the BCVA was 0.1.Conclusions: In patients with FEVR, angle-closure glaucoma due to pupillary block can occur even at a young age. FEVR should be considered in young patients with acute angle-closure glaucoma.