title: Management of an expansile orbital mass: Plexiform neurofibroma decompression by orbitozygomatic approach.
abstract: Neurofibromatosis types 1 (NF-1) and 2 are significant entities to otolaryngologists because they frequently involve head and neck skin, brain, skull base, orbits, and surrounding neural and vascular structures. We present the case of a 52-year-old male with progressive, marked unilateral proptosis due to a multilobulated orbital mass, secondary to biopsy-proven plexiform neurofibroma (PN). Acute worsening of proptosis leading to corneal abrasion, diplopia, and pain required debulking surgery, for which an orbitozygomatic approach was utilized. Genetic testing for NF-1 revealed no mutation. This rare case of NF-negative orbital PN and multidisciplinary treatment considerations for expansile orbital tumors are discussed.
authors.affiliation: Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Kovatch
authors.affiliation: Department of Otorhinolaryngology.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Purkey
authors.affiliation: Department of Pathology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, U.S.A.
authors.forename: Maria
authors.initials: M
authors.lastname: Martinez-Lage
authors.affiliation: Department of Otorhinolaryngology.
authors.forename: Roberta E
authors.initials: RE
authors.lastname: Gausas
authors.affiliation: Department of Neuroradiology.
authors.forename: Laurie A
authors.initials: LA
authors.lastname: Loevner
authors.affiliation: Department of Neurosurgery.
authors.forename: M Sean
authors.initials: MS
authors.lastname: Grady
authors.affiliation: Department of Otorhinolaryngology.
authors.forename: Bert W
authors.initials: BW
authors.lastname: O'Malley
authors.affiliation: Department of Otorhinolaryngology.
authors.forename: Christopher H
authors.initials: CH
authors.lastname: Rassekh
mesh_terms: D003664:Decompression / Q000379:methods; D005094:Exophthalmos / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000150:complications / Q000473:pathology / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000473:pathology / Q000601:surgery*; D009918:Orbital Neoplasms / Q000473:pathology / Q000601:surgery*
keywords: Neurofibromatosis; expansile mass; neurofibromatosis type 1; orbit; orbitozygomatic approach; plexiform neurofibroma; von Recklinghausen disease