Kabuki̇ sendromlu hastada anestezi̇ deneyi̇mi̇mi̇z

Translated title of the contribution: An anesthesia experience of a pediatric patient with Kabuki syndrome

Türkay Yücel, Emine Aysu Şalviz, Deniz Sariarslan, Mehmet Ter, Rauf Tuǧrul Aksoy

Research output: Contribution to journalArticlepeer-review

Abstract

Kabuki syndrome (Kabuki make-up syndrome, Niikawa-Kuroki syndrome) is a multiple malformation and mental retardation condition. It is characterized by distinctive facial features, musculosceletal anomalies, dermatoglyphic abnormalities, growth retardation, congenital heart defects, renal/genitourinary anomalies, anorectal anomalies, biliary atresia, epilepsia, adenoid hypertrophy, recurrent otitis media, diaphragmatic hernia, joint laxity, dental anomalies, high-arched palate, ophthalmologic abnormalities and mental retardation. Although this syndrome is rare, the surgical requirements of these patients increase because of their abnormalities. Therefore, anesthetists should improve their knowledge and skills, and also evaluate the patients with detailed anamnesis and physical examination. Possible difficult airway, cardiovascular, genitourinary, musculosceletal anomalies and epilepsia may raise the risk of anesthesia. The objective of this report was to describe a case of successful anesthesia experience in a 5-years-old girl patient with Kabuki syndrome, who had under gone inguinal hernia and ankyloglossia operations, and we also aimed to remind this rare syndrome with this case.

Translated title of the contributionAn anesthesia experience of a pediatric patient with Kabuki syndrome
Original languageTurkish
Pages (from-to)182-186
Number of pages5
JournalAnestezi Dergisi
Volume19
Issue number3
StatePublished - 2011

Keywords

  • Anesthesia, general
  • Ankyloglossia
  • Inguinal hernia
  • Kabuki syndrome

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