Anesthesia and perioperative medical management of children with spinal muscular atrophy.

OBJECTIVE: To describe the perioperative medical care, anesthetic considerations, and the risk of postanesthetic respiratory failure in patients with pediatric spinal muscular atrophy (SMA).

METHODS: There is a retrospective chart review carried out at an urban, tertiary care pediatric hospital. All patients with ICD-9 codes corresponding to SMA diagnoses were identified, and records screened for anesthetic and perioperative medical management. Medical records were reviewed for demographic, clinical, and outcome data.

MAIN RESULTS: Twenty-five children with SMA (10 type I, 8 type II, 7 type III) accounted for 56 general and regional anesthetic cases. Twenty-one (38%) cases had preexisting ventilator dependence. Total intravenous anesthesia with nitrous oxide was provided in 14 (25%) cases, balanced anesthesia with inhalational agents and epidural or systemic opioids were used in 41 (84%) cases, and one infant received a spinal anesthesia. Intraoperative and postoperative complications occurred in 2 (4%) and 2 (4%) cases respectively. One case required an unplanned postanesthetic intensive care unit admission, and there were two late deaths with redirection of care.

CONCLUSIONS: Perioperative care can be provided for children with SMA safely and effectively with total intravenous or inhaled anesthetics along with the judicious use of opioids to improve patient comfort without increased morbidity. Further prospective studies of standardized anesthetic and perioperative respiratory medical management in this population are required to minimize the decompensation of tenuous preoperative respiratory function.