Anesthesia management in a patient with kabuki syndrome

Med Arch. 2014 Oct;68(5):359-60. doi: 10.5455/medarh.2014.68.359-360. Epub 2014 Oct 15.

Abstract

Background and objectives: The aim of this case was to describe the anesthetic approach to a patient with Kabuki syndrome.

Case report: A patient with Kabuki syndrome had revision surgery for scoliosis. On physical examination, shown were long palpebral fissures, large, prominent fissures with an eversion of the lateral third of the lower eyelids, large, prominent malformed ears with low implantation, a short nasal septum, micrognathia, thoracolumbar scoliosis, a depressed left shoulder, a low-set occipital hairline and a short neck. The skin was elastic, and joints were lax. The laryngoscopy showed a Grade II Cormack and Lehane view of the larynx. The trachea was intubated easily. The patient was positioned carefully. Vital signs remained stable during surgery. The patient was extubated and transported to the post-anesthetic care unit.

Conclusion: Anesthesiologists should be aware of possibly difficult tracheal intubation cardiac lesions, respiratory problems, neurological and musculoskeletal disorders, and a latex allergy when managing anesthesia for a patient with Kabuki syndrome.

Keywords: Kabuki syndrome; anesthesia; general.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Anesthesia, General / methods*
  • Anesthetics / administration & dosage*
  • Child
  • Face / abnormalities*
  • Female
  • Hematologic Diseases / complications*
  • Humans
  • Scoliosis / surgery*
  • Treatment Outcome
  • Vestibular Diseases / complications*

Substances

  • Anesthetics

Supplementary concepts

  • Kabuki syndrome