Anesthetic and airway management of general anesthesia in a patient with Meckel-Gruber syndrome.

Abstract

Meckel-Gruber syndrome, characterized by occipital encephalocele, microcephaly, polydactyly, cleft lip or palate, mandibular micrognathism, and anatomical abnormality of the larynx and tongue, along with other associated malformations, is in the list of diseases associated with difficult airway. However, there has been no report on the management of general anesthesia and airway management for such patients. A 2-year-old girl with Meckel-Gruber syndrome was scheduled for cardioplasty and gastrostomy for gastroesophageal reflux under general anesthesia. Preoperative examination revealed obesity, microgenia, dysspondylism, proteinuria, hypoplastic kidneys, and stenosis of the anal canal. Although we anticipated some difficulty with the intubation and prepared several alternative methods for intubation, such as a bronchofiberscope and a laryngeal mask airway, tracheal intubation was completed without difficulty using conventional laryngoscopy after inhalational induction with sevoflurane. Because most patients with this syndrome die before and shortly after delivery, those who survive to some age might have less severe deformities.

• Authors: Mitsunori Miyazu, Kazuya Sobue, Hiroaki Ito, Takafumi Azami, Shoji Ito, Akinori Takeuchi, Hiroshi Sasano, Takako Tsuda, Hirotada Katsuya
• Journal: Journal of anesthesia (J Anesth) Vol. 19 Issue 4 Pg. 309-10 ( 2005) ISSN: 0913-8668 [Print] Japan
• PMID: 16261468 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anesthetics, Inhalation
• Methyl Ethers
• Sevoflurane

Topics

• Abnormalities, Multiple (surgery)
• Anesthesia, General (methods)
• Anesthetics, Inhalation
• Child, Preschool
• Female
• Gastroesophageal Reflux (surgery)
• Humans
• Intubation, Intratracheal
• Laryngoscopy
• Methyl Ethers
• Sevoflurane
• Syndrome
• Trachea