[Anesthetic management in a patient with Simpson-Golabi-Behmel syndrome].

Abstract

We report the anesthetic management of a 4-yr-old boy with Simpson-Golabi-Behmel syndrome. This syndrome is an X-linked condition characterized by pre- and postnatal overgrowth with visceral and skeletal anomalies. Affected males have a distinctive facial appearance with wide nasal bridge, anteverted nostrils, wide-open mouth, enlarged tongue, and large protruding maxilla and jaw. Although it had been speculated preoperatively that airway management would be complicated by the macroglossia, there was no difficulty in endotracheal intubation in the present patient. Preparations for difficult airway management should be made by the anesthesiologist before induction of anesthesia.

• Authors: K Tsuchiya, O Takahata, K Sengoku, I Hamada, A Suzuki, H Iwasaki
• Journal: Masui. The Japanese journal of anesthesiology (Masui) Vol. 50 Issue 10 Pg. 1106-8 (Oct 2001) ISSN: 0021-4892 [Print] Japan
• PMID: 11712343 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Abnormalities, Multiple
• Anesthesia, Inhalation
• Child, Preschool
• Face (abnormalities)
• Genetic Linkage
• Gigantism
• Humans
• Intraoperative Care
• Macroglossia
• Male
• Syndrome
• Tongue (surgery)
• X Chromosome