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Novel incision to debulk eyelids in a case of Orbital Periorbital Plexiform Neurofibroma (OPPN): A case report

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Case Report

Author Details : Umesh Kumar*, Sudipta Bera, Shikha Jaiswal, K Cheruvu Natraj Navya, Vivek Vijay Kumar, Rajendra P Maurya

Volume : 9, Issue : 4, Year : 2023

Article Page : 653-656

https://doi.org/10.18231/j.ijceo.2023.123

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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

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