Orbitotemporal neurofibromatosis. Clinical features and surgical management.

Abstract

To classify the periorbital deformities of adult orbitotemporal neurofibromatosis (NF) and describe new clinical findings, and to recommend guidelines for surgical treatment and management of surgical complications. Retrospective noncomparative case series. Thirty-three patients over age 16 with orbitotemporal NF. Retrospective surgical case record and serial photographic review recording the laterality and the severity of periorbital involvement, the presence of complications from previous surgery, the surgical techniques undertaken, and the surgical outcome and complications. Comparison of preoperative and postoperative level of deformities. New classification of periorbital deformities: (1) brow ptosis, (2) upper lid infiltration with ptosis, (3) lower lid infiltration, (4) lateral canthal disinsertion, and (5) conjunctival and lacrimal gland infiltration. Two patients had bilateral and 31 patients (94%) had unilateral orbitotemporal NF. All patients had upper and 19 patients (58%) had lower lid involvement. Six (18%) patients had significant brow infiltration. Fourteen (42%) patients had a dropped lateral canthus requiring surgical reattachment, 28 (85%) required anterior levator resection for ptosis, and 28 (85%) had lid-debulking surgery. New findings included severe brow infiltration, lacrimal gland involvement, and functional nasolacrimal duct obstruction. Complications from previous surgery included residual ptosis, ptosis overcorrection, poor lid contour, dry eye, corneal exposure, and upper and lower lid entropion/ectropion. The periorbital appearance and comfort of patients with NF type 1 who have orbitotemporal NF can be significantly improved through oculoplastic surgery.