Facial plexiform neurofibromatosis: A surgical challenge
A 29-year-old female patient presented to us with a history of overhanging folds of loose skin affecting the right side of her face and right forearm []. The swelling appeared during childhood and progressed slowly. She complained of occasional pain and itching on the affected part. The patient initially sought treatment elsewhere, about 10 years back, when excision of forearm swelling was carried out, but the swelling recurred. She came to us now with complaints of inability to open her right eye and sought improvement in her facial appearance.\nOn physical examination, the face was disfigured due to overhanging folds of skin affecting the temporal, orbital and cheek areas. She was unable to open her right eye due to the overhanging folds affecting both eyelids, and the eye was pulled inferiorly. Vision acuity of the right eye was however not affected. Café au lait macules (some measuring over 15 mm) as well as several freckles were observed in the axillary, back and chest regions. She had multiple neurofibromas over her forehead, neck and trunk regions. She also had a similar swelling and an old healed scar over the right forearm.\nReconstructive surgical procedure in the form of subtotal excision of tumor and re-draping of the facial skin was performed. Operative findings revealed grossly thickened subcutaneous nerves and neo-vessels in the tumor mass, which was infiltrating deeply, and no clear tissue planes were identifiable. The neo-vessels were very friable and bled profusely during dissection. Complete excision was not possible; hence, subtotal excision and redraping of skin flap was carried out. Anchoring sutures were applied over the lateral canthal region between the skin flap and the lateral orbital margin to prevent pulling down of eyelids []. The post-operative period was uneventful. However, on review after 6 months, there was partial regrowth of the swelling.