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Central Surgical Association| Volume 82, ISSUE 3, P342-348, September 1977

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The operative management of von Recklinghausen's neurofibromatosis in children, with special reference to lesions of the head and neck

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      Abstract

      Eighty-five patients with von Recklinghausen's neurofibromatosis were seen at the Children's Hospital of Pittsburgh from 1952 through 1976. Nineteen of these patients had significant head or neck extracranial lesions. Based upon these and a personal series of seven other head and neck patients, we emphasize several critical points in the care of these patients: (1) large, painful, conspicuous, or strategically placed tumors should be removed; (2) early removal is easier and improves cosmetic results; (3) neck and chest tumors carry a high risk of subsequent rapid vertebral angulation with possible paralysis; (4) close observation allows timely application of appropriate surgical procedures.
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        Malignant degeneration of thoracic neurofibromata.
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