Unilateral angle-closure glaucoma in combination with neurofibromatosis: a case report

Background: Neurofibromatosis I(NFI) is an autosomal dominant genetic disease that causes multi-system damage. It is rare have both angle-closure glaucoma and NF, especially in pediatric patients. We report a case of unilateral angle -closure glaucoma with NFI. Case presentation: A 5-year-old girl accompanied by a large subcutaneous soft mass and multiple coffee-milk spots scattered presented with low vision ,increased IOP and angle-closure in her right eye. Slit-lamp examination demonstrated multiple Lisch nodules in both eye. In her right eye, Ectropion uveae were observed at the top and bottom margins of the pupi. There were no abnormalities on MRI of skull and orbit. Finally ,trabeculectomy was performed on the right eye, after which the right eye showed a stable IOP. Conclusions: : The incidence of NFI with angle-closure glaucoma is low and clinically rare. Early diagnosis and treatment can generally achieve good results, but the long-term efficacy still needs further follow-up, especially the contralateral eye.