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PMID: 33016662 Published · epublish English

Esthetic subunit approach in massive facial plexiform neurofibroma: a case report.

Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica ·Vol. 33 ·No. 4 ·2020-10-01

de la Torre Macías M, Berenguer Frohner B, Lorca García C, de Tomás Palacios E

Abstract

Plexiform neurofibroma is a presentation of neurofibromatosis type 1 (NF1) which can cause great facial deformities. Treatment rarely has a healing effect, so the surgical approach is aimed at improving esthetics and function. It requires a cross-disciplinary approach and typically needs multi-stage surgery. This is the case of a 16-year-old male patient with NF1 presenting with left periorbital and malar facial plexiform neurofibroma with slow-growth intraconal and extraconal invasion. He presented at the plastic surgery consultation for facial soft tissue deformity correction. Removal was performed using an esthetic subunit approach, with canthopexy and orbital cavity reconstruction, resulting in facial region symmetrization. This allowed for remarkable esthetic and functional improvement, facilitating ocular prosthesis adaptation. The subsequent use of selumetinib allowed the lesion to be stabilized.

Keywords
Esthetic subunit approach Neurofibromatosis type 1 Ocular prosthesis Plexiform neurofibroma
MeSH 主题词
Adolescent Facial Neoplasms/etiology,surgery Humans Male Neurofibroma, Plexiform/diagnosis,etiology,surgery Neurofibromatosis 1/complications,surgery Reconstructive Surgical Procedures/methods
Article Info
Journal
Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
Abbr.
Cir Pediatr
ISSN
2445-2807
Published
2020-10-01
Language
English
Country/Region
Spain
NLM ID
8900492
External Links
PubMed source
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