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Anesthetic considerations in Smith-Lemli-Opitz syndrome

Peter T.-L. Choi, MD FRCPC^*, and Malgorzata J.M. Nowaczyk, MD FRCPC FCCMG^,,

^* From the Department of Anaesthesia, St. Joseph's Hospital, the
Departments of Pathology and Molecular Medicine and
Paediatrics, McMaster University,
Hamilton, Ontario, Canada.

Address correspondence to: Dr. Peter T.-L. Choi, Department of Anaesthesia, McMaster University, HSC-2U7, 1200 Main Street West, Hamilton, Ontario, L8N 3Z5 Canada. Phone: 905-521-2100; Fax: 905-523-1224; E-mail: choip{at}fhs.csu.mcmaster.ca

Purpose: 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.

Clinical features: 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.

Conclusion: 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.