Case Report
Year: 2019 | Month: September | Volume: 6 | Issue: 9 | Pages: 220-224
Surgical Management of Oculotemporal Neurofibromatosis - A Case Report
Dr Neebha Anand1, Dr Jaya Gupta2, Dr Preetam Kurrey3, Dr Reena Gupta4
1Professor, RIO, PGIMS, Rohtak, Haryana, India
2Resident, RIO, PGIMS, Rohtak, India
3Resident, PGIMS, Rohtak
4Assoc Prof, RIO, PGIMS, Rohtak
Corresponding Author: Dr Neebha Anand
ABSTRACT
Introduction- Neurofibromatosis is an autosomal dominant, multisystem disorder affecting cell growth of neural tissue, leading to tumour growths that impact skin, nervous system, eye and other organs. It is of two types- neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF 2). Patients with NF1 manifest variety of ophthalmic conditions including optic glioma, glaucoma, choroidal nodules, Lisch nodules and plexiform neurofibromas (causing mechanical ptosis), involving the eyelid, orbit, periorbital and facial structures. Plexiform neurofibromas in these areas are most appropriately labelled as OPPN (orbital periorbital plexiform neurofibroma) to encompass all locations where they occur. A frequent cause of visual loss is deprivational or anisometropic amblyopia. Equally important is the alteration in physical appearance, secondary to ptosis, dystopia and facial disfigurement leading to social embarrassment. A regular and long term monitoring is needed in all these cases Proper and timely management of these cases is a must to prevent complications.
Case - An 18 year old male presented with drooping of right upper lid associated with non progressive periorbital swelling and decreased vision since childhood. BCVA was finger count half metre OD and 6/9 OS. On examination right side mild dystopia was seen along with right upper lid severe ptosis which had a characteristic ‘S’ shaped, with a good LPS action (11mm). This was associated with periorbital swelling in the temporal fossa. The soft multilobulated swelling on the lateral half of the right upper eyelid on palpation felt like a “bag of worms”. The patient was considered for removal of neurofibroma and cosmetic correction of right upper lid. The contralateral normal left upper eyelid and palpebral aperture was taken as the desired end result for full cosmetic correction. SURGERY- An en block full thickness resection of the excess of the right upper lid tissue (debulking ) with lid shortening , lateral canthopexy and the required LPS resection was performed.
Conclusion- Our patient underwent debulking with shortening of right upper lid along with lateral canthopexy and LPS resection, all done in one surgical procedure. At the end of 2 weeks post op follow up, a good cosmetic outcome with the desired lid height was obtained.
Key words: Neurofibromatosis, oculotemporal, autosomal dominant disorder.
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