The surgical management of childhood orbito-temporal neurofibromatosis

Purpose. (1) To describe the natural history, changing manifestations and new signs of orbito-temporal neurofibromatosis (NF) during childhood and puberty. (2) To describe the surgical treatment and recommend guidelines for management of this condition in children compared to adults. Methods. Retrospective case note and serial photographic review Results. There were 9 patients (5M, 4F) with orbito-temporal NF who were children (<16 years) at the time of first presentation with a minimum of 5 years follow up. All the patients had unilateral periorbital neurofibromatosis with blepharoptosis, orbital enlargement and hypoglobus. All nine patients underwent blepharoptosis surgery. Eight patients underwent neurofibroma debulking (and lid shortening) and lateral canthal reattachment. Three had enucleation of their blind eyes. Seven patients had two or more operations for their orbito-palpebral deformities. New signs included conjunctival and lacrimal gland infiltration with a secondary dry eye. Conclusion. Blepharoptosis surgery is indicated if the visual axis is compromised and there is a chance of limiting amblyopia. Definitive surgery for appearance is best delayed until the age of 18 years, or when the disease progression has stabilised, unless there are compelling social reasons for earlier surgery.