Objective: To study the clinical manifestations and surgical treatment of Type II orbital neurofibromatosis.
Methods: From Jan, 2001, to Oct, 2006, clinical data of 16 cases with type II orbital neurofibromatosis were retrospectively analyzed in the Department of Ophthalmology, Ninth People's Hospital, Medical School of Shanghai Jiao Tong University.
Results: All patients had orbito-temporal or intra-orbital neurofibromatosis, combined with periorbital deformities. After partial tumor resection, the orbital reconstruction and blepharoplasty were performed in the same stage. The patients were followed up for 6-12 months with no relapse. The vision was improved in 6 cases and kept the same in 10 cases. The cosmetic results were satisfactory in all cases. 5 cases were re-operated because of relapse of blepharoptosis.
Conclusions: The function and appearance can be markedly improved after one-stage partial tumor resection, orbital reconstruction and blepharoplasty in patients with type II orbital neurofibromatosis.