TITLE

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

AUTHOR(S)
Miyazu, Mitsunori; Sobue, Kazuya; Ito, Hiroaki; Azami, Takafumi; Ito, Shoji; Takeuchi, Akinori; Sasano, Hiroshi; Tsuda, Takako; Katsuya, Hirotada
PUB. DATE
November 2005
SOURCE
Journal of Anesthesia;2005, Vol. 19 Issue 4, p309
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
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.
ACCESSION #
18711928

 

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