Sub total and Total Resection of Orbitopalpebral Plexiform Neurofibromatosis

Dr. Golam Haider,FCPS, MS

Associate Professor (Oculoplasty)
National Institute of Ophthalmology and HospitalAgargaon, Dhaka-1207, Bangladesh

1. Associate Professor (Oculoplasty), NIO,H
2. Registrar, NIO,H
3. Assistant Professor(Oculoplasty), NIO,H
4. Associate Professor, NIO,H
5. Assistant Professor, Lions' Eye Hospital
6. HMO, NIO,H

Purpose: To observe the post operative results of surgical intervention of plexiform neurofibromatosis of upper lid.

Materials and methods: This prospective study was done in oculoplasty clinic of National Institute of Ophthalmology and Hospital and Harun Eye Hospital from 2007 to May 2010. Eleven patients were operated among which one was female and rests were male. Age range was 2 years to 25 years. Extensive involvement of upper eyelid, temple and lateral part of lower lid was found in five patients. In another five patients S- shapped deformity of upper eyelid was found. Neovascular glaucoma was present in one male child of 11 years for which evisceration was done. Debulking, horizontal and vertical lid shortening followed by ptosis correction was done in seven cases. Frontalis brow suspension with fascia lata was in two cases.

Result: Five patients obtained almost satisfactory result. Two patients needed revision surgery. Four patients with Orbitopalpebral Neurofibromatosis had incomplete resection.

Conclusion: Surgery of neurofibromatosis is often difficult. Satisfactory outcome is unpredictable. Recurrence is inevitable in younger age group. So revision is frequently required. Therefore preoperative counseling along with patients' expectation is a vital issue for both the surgeons and patients.