Anesthetic considerations in Smith-Lemli-Opitz syndrome
- PMID: 10875719
- DOI: 10.1007/BF03018947
Anesthetic considerations in Smith-Lemli-Opitz syndrome
Abstract
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.
Similar articles
-
[Anesthetic management of a patient with Smith-Lemli-Opitz syndrome complicated with thrombocytopenia].Masui. 2003 Nov;52(11):1240-2. Masui. 2003. PMID: 14661577 Japanese.
-
Airway management of patient with Smith-Lemli-Opitz syndrome for gastric surgery: case report.Paediatr Anaesth. 2006 Mar;16(3):322-4. doi: 10.1111/j.1460-9592.2005.01685.x. Paediatr Anaesth. 2006. PMID: 16490099
-
Smith-Lemli-Opitz syndrome: case report and literature review.J Pediatr Surg. 2000 Dec;35(12):1840-2. doi: 10.1053/jpsu.2000.19291. J Pediatr Surg. 2000. PMID: 11101752 Review.
-
Mild Smith-Lemli-Opitz syndrome: further delineation of 5 Polish cases and review of the literature.Eur J Med Genet. 2008 Mar-Apr;51(2):124-40. doi: 10.1016/j.ejmg.2007.11.004. Epub 2007 Dec 8. Eur J Med Genet. 2008. PMID: 18249054
-
Behavioral phenotype of RSH/Smith-Lemli-Opitz syndrome.Ment Retard Dev Disabil Res Rev. 2000;6(2):131-4. doi: 10.1002/1098-2779(2000)6:2<131::AID-MRDD7>3.0.CO;2-R. Ment Retard Dev Disabil Res Rev. 2000. PMID: 10899806 Review.
Cited by
-
Treatment of Smith-Lemli-Opitz syndrome and other sterol disorders.Am J Med Genet C Semin Med Genet. 2012 Nov 15;160C(4):285-94. doi: 10.1002/ajmg.c.31347. Epub 2012 Oct 5. Am J Med Genet C Semin Med Genet. 2012. PMID: 23042642 Free PMC article. Review.
-
Smith-Lemli-Opitz-syndrome: How different is the anesthetic technique?Saudi J Anaesth. 2014 Jul;8(3):440-2. doi: 10.4103/1658-354X.136653. Saudi J Anaesth. 2014. PMID: 25191210 Free PMC article. No abstract available.
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources
Medical