Indian Journal of Clinical and Experimental Ophthalmology
Official Publication of Innovative Education and Scientific Research Foundation
Official Publication of Innovative Education and Scientific Research Foundation
Print ISSN: 2395-1443
Online ISSN: 2395-1451
CODEN : IJCEKF
Indian Journal of Clinical and Experimental Ophthalmology (IJCEO) is open access, a peer-reviewed medical journal, published quarterly, online, and in print, by the Innovative Education and Scientific Research Foundation (IESRF) since 2015. To fulfil our aim of rapid dissemination of knowledge, we publish articles ‘Ahead of Print’ on acceptance. In addition, the journal allows free access (Open Access) to its content, which is likely to attract more readers and citations of articles published in IJCEO. Manuscripts must be prepared in more...Case Report
Author Details :
Volume : 9, Issue : 4, Year : 2023
Article Page : 653-656
https://doi.org/10.18231/j.ijceo.2023.123
Abstract
Background: Plexiform neurofibroma with neurofibromatosis 1 is a autosomal dominant condition and a relatively rare condition. It is also termed as Von Recklinghausen disease. PN involving the structures like eyelid, orbit, periorbital and facial structures are labelled as orbital-periorbital PN(OPPN). These are slowly progressing lesions and are present since birth but increase in size during childhood and are locally infiltrating in nature.
Aim: To describe a lid incision to simultaneously debulk both upper and lower eyelid, preserving the vascularity in a case of Orbital Periorbital Plexiform Neurofibroma (OPPN).
Case Presentation: We report a case of 22 year male who presented with a disfiguring swelling of right side of face since childhood. After thorough clinical and radiological evaluation it was diagnosed to be a OPPN. Staged debulking was planned and after stage 1 debulking, tissue was sent for HPE which revealed overgrowth of peripheral nerve components and connective tissue dermis showing infiltrating tumour composed of oval to spindle cells with pleomorphic nuclei and moderate cytoplasm. Staged debulking with lid reduction surgery was performed.
Discussion : Neurofibroma is a rare entity with plexiform neurofibroma occurring in 5-15% of patients. OPPN infiltrates locally without respecting the anatomical planes but follows the trigeminal nerve distribution usually. Indications for debulking procedure are usually the disfigurement and optimal timing is also not certain because the extent and rate of growth cannot be predicted.
Conclusion: The facial appearance and outcome in patients with OPPN can be significantly improved through lid reduction surgery.
Keywords: Periorbital, Neurofibroma, Debulking, Incision.
How to cite : Kumar U, Bera S, Jaiswal S, Navya K C N, Kumar V V, Maurya R P, Novel incision to debulk eyelids in a case of Orbital Periorbital Plexiform Neurofibroma (OPPN): A case report. Indian J Clin Exp Ophthalmol 2023;9(4):653-656
This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
