Anesthetic experience using total intra-venous anesthesia for a patient with Wolf-Hirschhorn syndrome -A case report-

다국어 초록 (Multilingual Abstract)

We present here the case of a 33-month-old male patient with Wolf-Hirschhorn syndrome (WHS) and who underwent tympanoplasty and myringotomy. WHS is caused by a rare chromosomal abnormality, which is the deletion of the short arm of chromosome number 4. The typical craniofacial features of WHS patients such as micrognathia,microcephaly and the muscular weakness can make using neuromuscular blocking agents and performing intubation difficult. Moreover, there are a few previous case reports showing that malignant hyperthermia occurred during and after an operation in which the anesthesia was done with inhalation agents, so special anesthetic care is needed when operating on a WHS patient. By carefully intubating the patient and using total intravenous anesthesia,we performed successful anesthesia without any complications. We describe here the anesthetic management of a WHS patient and we review the relevant literature.

참고문헌 (Reference)

1 Battaglia A, "Wolf-Hirschhorn Syndrome. In: GeneReviews [PMID: 20301362 ]" University of Washington, Seattle

2 Battaglia A, "Update on the clinical features and natural history of Wolf-Hirschhorn (4p-) syndrome: experience with 87 patients and recommendations for routine health supervision" 148C : 246-251, 2008

3 Frova G, "Recommendations for airway control and difficult airway management in paediatric patients" Gruppo di Studio SIAARTI "Vie Aeree Difficili" 72 : 723-748, 2006

4 "Practice guidelines for management of the difficult airway: an updated report by the American Society of Anesthesiologists Task Force on Management of the Difficult Airway" American Society of Anesthesiologists Task Force on Management of the Difficult Airway 98 : 1269-1277, 2003

5 Souliere CR Jr, "Markedly delayed postoperative malignant hyperthermia" 112 : 564-566, 1986

6 Muldoon S, "Malignant hyperthermia, thermoregulation, and perioperative hypothermia. In: Anesthesiology" The United States, The McGraw-Hill companies 1964-1998, 2008

7 Ginsburg R, "Malignant hyperthermia in the Wolf- Hirschhorn syndrome" 43 : 386-388, 1988

8 Rosenberg H, "Malignant hyperthermia" 2 : 21-, 2007

9 Gerald AG, "Malignant Hyperthermia. In: Miller’s Anesthesia. 6th ed" Philadelphia, Elsevier Churchill Livingstone 1169-1190, 2005

10 Shannon NL, "An epidemiological study of Wolf-Hirschhorn syndrome: life expectancy and cause of mortality" 38 : 674-679, 2001

11 Chen JC, "4Psyndrome(Wolf-Hirschhorn syndrome) complicated with delay onset of malignant hyperthermia: a case report" 32 : 275-278, 1994