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

Jae Ho Choi, Jae Hwan Kim, Young Cheol Park, Woon Young Kim, Yoon Sook Lee

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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.

Original languageEnglish
Pages (from-to)119-123
Number of pages5
JournalKorean Journal of Anesthesiology
Issue number2
Publication statusPublished - 2011 Feb 1



  • Difficult intubation
  • Malignant hyperthermia
  • Total intra-venous anesthesia
  • Wolf-Hirschhorn syndrome

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine

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