Responses to nondepolarizing neuromuscular blockers and succinylcholine in von Recklinghausen neurofibromatosis.

Patients with type 1 neurofibromatosis (NF-1) have been reported to have prolonged responses to nondepolarizing (ND) neuromuscular blockers (NMBs). Responses to succinylcholine (SCh) have been described as increased, decreased, or normal. The purpose of this study was to assess responses to NMBs in NF-1 patients in order to determine the clinical significance of abnormal responses. We retrospectively identified all NF-1 patients who received anesthetics at Strong Memorial Hospital between January 1, 1984 and December 31, 1994. We then reviewed all anesthetic records to classify responses to NMBs as normal, abnormal, or indeterminate. Records of 114 anesthetics provided to 44 NF-1 patients were reviewed. Nondepolarizing NMBs were used during 73 anesthetic cases in 38 patients. Responses were normal in 69 cases and indeterminate in 4 (3 in patients with normal responses during other anesthetics). SCh was used during 42 anesthetic cases in 23 patients. Responses were normal in all but one case (indeterminate) in a patient who had had other documented normal responses. Standard milligram per kilogram doses of NMBs were used in all cases, and in none was there evidence of abnormal response. The risk of abnormal response to NMBs in individuals with NF-1 appears to be minimal. We recommend no alteration in dosing of either SCh or ND NMBs in patients with NF-1.