Korean J Anesthesiol.  2011 Feb;60(2):119-123. 10.4097/kjae.2011.60.2.119.

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

• Choi JH
• Kim JH
• Park YC
• Kim WY
• Lee YS

• KMID: 1766530
• DOI: http://doi.org/10.4097/kjae.2011.60.2.119

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.

Keyword

Difficult intubation; Malignant hyperthermia; Total intra-venous anesthesia; Wolf-hirschhorn syndrome

MeSH Terms

Anesthesia
Anesthesia, Intravenous
Arm
Chromosome Aberrations
Humans
Inhalation
Intubation
Male
Malignant Hyperthermia
Microcephaly
Muscle Weakness
Neuromuscular Blocking Agents
Preschool Child
Tympanoplasty
Wolf-Hirschhorn Syndrome
Neuromuscular Blocking Agents