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Indian Journal of Surgery, Vol. 66, No. 2, Mar-Apr, 2004, pp. 121 Images in Surgery Plexiform neurofibromatosis A. Rekha Sri Ramachandra Medical College, Research Institute and Deemed University,
Porur, Chennai - 600116, India.
Paper Received: April 2003. Paper Accepted: July 2003. Source of Support: Nil. Code Number: is04033 How to cite this article: Rekha A. Plexiform neurofibromatosis. Indian J Surg 2004;66:121. A 21-year-old came to the hospital with complaints of a swelling present over the side of the face since birth. Examination revealed unsightly folds of skin interspersed with nodular firm swellings. These were found to extend over the maxillary and mandibular divisions of the trigeminal nerve. There was hypertrophy of the lip and the mid-face of the affected side as compared to the opposite side. Vision was normal though there appeared to be an anti-mongoloid slant due to the weight of the soft tissue below. A diagnosis of Plexiform Neurofibromatosis was made and the patient was referred to the plastic surgeon. Neurofibromas (NF) are soft tissue tumours that contain both neural and fibrous elements. NF can be solitary or a part of a generalized neurofibromatosis. The plexiform variant of neurofibromatosis is a rare condition that occurs in connection with the fifth cranial nerve. This is due to myxofibromatous degeneration of the endoneurium. The upper lip is commonly affected and sarcomatous change rarely occurs. The other variant of NF is the pachydermatocoele (elephantiasis neuromatosis) where the neck is commonly affected. The skin is coarse, dry, thickened and the subcutaneous tissue becomes grossly thickened. © 2004 Indian Journal of Surgery. The following images related to this document are available:Photo images[is04033f1.jpg] |
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