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

Abstract

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.

Unilateral chronic angle-closure glaucoma in a pediatric patient with neurofibromatosis: a case report

“…Moreover, this ectropion uveae alteration in NF may be progressive. Esfandiari et al 18 described a 3-year-old patient with NF who had unilocular ectropion uveae and developed glaucoma in this eye at the age of 10 years. IOP changes should therefore be closely followed up in patients presenting with ectropion uveae.…”