Perioperative management of a neonate with aglossia-adactylia syndrome

Aglossia-adactylia syndrome is a rare disorder characterized by aglossia and deformity of the limbs of variable degree. We managed a 2-day-old neonate with ileojejunal atresia with aglossia-adactylia syndrome. He was scheduled for the repair of the intestinal atresia, gastrostomy and palatal impress...

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Bibliographic Details
Published inMasui. The Japanese journal of anesthesiology Vol. 50; no. 7; p. 773
Main Authors Mandai, H, Kinouchi, K
Format Journal Article
LanguageJapanese
Published Japan 01.07.2001
Subjects
Abnormalities, Multiple
Anesthesia, General
Humans
Infant, Newborn
Intestinal Atresia - surgery
Intubation, Intratracheal
Limb Deformities, Congenital
Male
Perioperative Care
Syndrome
Tongue - abnormalities
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Summary:Aglossia-adactylia syndrome is a rare disorder characterized by aglossia and deformity of the limbs of variable degree. We managed a 2-day-old neonate with ileojejunal atresia with aglossia-adactylia syndrome. He was scheduled for the repair of the intestinal atresia, gastrostomy and palatal impression taking, under general anesthesia with sevoflurane supplemented with fentanyl. In this case we could achieve tracheal intubation by direct laryngoscopy with cricoid pressure applied, and could manage without any complications. But many of the patients with this syndrome are complicated with cleft palate and micrognathia, and we should be careful of the difficult airway at the induction of general anesthesia and aspiration during perioperative period.
ISSN:0021-4892