Anesthetic considerations in Smith-Lemli-Opitz syndrome

Purpose: Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive syndro me characterized by congenital anomalies affecting the airway, cardiorespir atory, gastrointestinal, genitourinary, and central nervous systems. The pr esence of these anomalies as well as the potential for muscle rigidity with or without hyperthermia present challenges to anesthesia. This report desc ribes our institution's experience with SLOS patients undergoing anesthesia in Canada. Clinical features: Three patients diagnosed with SLOS at McMaster Universit y 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 admin istered. One patient. was noted to have a diminished laryngoscopic view but no difficult intubations were encountered. One patient required re-intubat ion due to upper airway obstruction related to the surgical procedure in th e postoperative period. No cardiovascular, respiratory, or temperature comp lications were experienced. Conclusion: This report describes the anesthetic management in three patien ts 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.