Anesthetic considerations in Smith-Lemli-Opitz syndrome

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive syndrome characterized by congenital anomalies affecting the airway, cardiorespiratory, gastrointestinal, genitourinary, and central nervous systems. The presence of these anomalies as well as the potential for muscle rigidity with or without hyperthermia present challenges to anesthesia. This report describes our institution's experience with SLOS patients undergoing anesthesia in Canada.

Three patients diagnosed with SLOS at McMaster University underwent a total of five anesthetics for diagnostic imaging and surgical procedures. The age of the patients ranged from two months to four years. Four intravenous anesthetics and one balanced general anesthetic were administered. One patient was noted to have a diminished laryngoscopic view but no difficult intubations were encountered. One patient required re-intubation due to upper airway obstruction related to the surgical procedure in the postoperative period. No cardiovascular, respiratory, or temperature complications were experienced.

This report describes the anesthetic management in three patients with SLOS. Although the syndrome raises a number of potential anesthetic concerns, few complications were seen. This is consistent with other cases reported in the literature, which is reviewed in this report.