Remodelling the upper eyelid in the management of orbitopalpebral neurofibromatosis

Abstract

Neurofibromatosis of the orbitopalpebral complex is a debilitating disease. The development of safe craniofacial surgical technique has greatly improved outcome in the surgical palliation of this progressive condition, and various centres have reported their experience. Extensive upper eyelid neurofibroma creates a functional and aesthetic problem, which is inadequately addressed in the literature. The experience of the senior author (DM) representing the treatment of 27 such patients is currently reported. The stigmata of eyelid neurofibromatosis were scored retrospectively by a novel scale applied to standardised photographs at patient presentation. A numeric severity score was given to each of: upper eyelid ptosis, canthal malposition, and oculo-palpebral diastasis. Disease progression and post-operative result were similarly scored at various stages follow-up photography. All patients underwent a full thickness, transverse or transverse-oblique resection of upper eyelid disease, with immediate levator reconstruction and canthopexies as necessary. A mean improvement of 3.33 points was achieved in the majority of patients (median one operation per patient; mean 1.5, range 1-3). Secondary eyelid procedures (n=9 pts) represented tumour debulking, canthopexy, or procedures to deepen the fornix in patients with prostheses. This series demonstrates that a tailored, often radical palpebral remodelling in orbitopalpebral neurofibromatosis may provide functional and aesthetic benefit.