Progressive ectropion uveae and secondary angle-closure glaucoma in type 1 neurofibromatosis

PURPOSE: To present a case of progressive ectropion uveae and secondary angle-closure glaucoma in association with type 1 neurofibromatosis (NF-1). OBSERVATION: An 11-year-old-Hispanic-male with a known history of NF-1 who was followed for the ocular manifestations of NF-1 developed an irregular pupil and ectropion uveae in the right eye at the age of 3 years that gradually increased in severity. The area of ectropion uveae increased in size and extended superiorly with concurrent superior synechial angle closure and intraocular pressure (IOP) elevation. The patient subsequently developed chronic angle-closure glaucoma that could not be controlled with medical therapy. He underwent successful implantation of an aqueous drainage device which resulted in excellent intraocular pressure reduction. CONCLUSIONS AND IMPORTANCE: Ectropion uveae can be progressive and lead to the development of extensive angle closure in patients with NF-1. Despite the low incidence of glaucoma in patients with NF-1, the presence of ectropion uveae in this condition necessitates careful observation of the anterior segment, including the anterior chamber angle and close monitoring of the IOP.