Orbital/Periorbital Plexiform Neurofibromas: Classification and Surgical Strategies for a Better Outcome.

Plast Reconstr Surg Glob Open

From the Department of Plastic and Reconstructive Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Published: July 2023

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10325737PMC
http://dx.doi.org/10.1097/GOX.0000000000005108DOI Listing

Publication Analysis

Top Keywords

orbital/periorbital plexiform
4
plexiform neurofibromas
4
neurofibromas classification
4
classification surgical
4
surgical strategies
4
strategies better
4
better outcome
4
orbital/periorbital
1
neurofibromas
1
classification
1

Similar Publications

Neurofibromatosis Type 1-Associated Plexiform Neurofibromas of the Face and Adjacent Head Regions: Topography of Lesions and Surgical Treatment Data of 179 Patients.

J Maxillofac Oral Surg

September 2023

Department of Oral and Craniomaxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Martinistraße 52, 20246 Hamburg, Germany.

Introduction: Facial plexiform neurofibromas (FPNF) are rare tumors frequently diagnosed in patients with neurofibromatosis type 1 (NF1), a tumor predisposition syndrome. FPNF often grows invasively and destructively, which may complicate surgical treatment. Data on the frequency, location, and surgical procedures of patients with NF1-associated FPNF are scarce.

View Article and Find Full Text PDF

To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected.

View Article and Find Full Text PDF

Introduction: Plexiform neurofibromas (PNF) in neurofibromatosis type 1 (NF1) are usually diagnosed in childhood and can grow rapidly during this period. In 10% of patients, PNF involve the orbital-periorbital area and may cause visual problems including glaucoma, visual loss from amblyopia (deprivational, strabismic, or refractive), optic nerve compression, or keratopathy. Ptosis, proptosis, and facial disfigurement lead to social problems and decreased self-esteem.

View Article and Find Full Text PDF

Background: Orbital/periorbital plexiform neurofibroma (OPPN) can compromise physical appearance and visual function. However, the clinical characteristics and NF1 mutation landscape in patients with heritable OPPN have not been reported.

Methods: The medical charts of 26 Chinese patients with OPPN from 12 families were reviewed.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!