The response of a patient with von Recklinghausen's disease to succinylcholine and atracurium.

The anesthetic management of a 17-year-old female with von Recklinghausen's disease (multiple neurofibromatosis) is described. Multiple neurofibromatosis is a rare syndrome characterized by abnormal cutaneous pigmentation and numerous skin tumors. In addition to the usual stigmata of the syndrome, patients with neurofibromatosis have been reported to be either sensitive or resistant to succinylcholine. Further, all reports indicate that response to nondepolarizing muscle relaxants is exaggerated. In this patient, the nerve conduction studies were within limit of normal values. During surgery, neuromuscular function was monitored throughout using the train-of-four mechanical twitch response. The response of this patient to succinylcholine was normal and the response to atracurium was slightly prolonged. It is concluded that atracurium offers advantages over the other nondepolarizing muscle relaxants in patients with von Recklinghausen's disease.