title: Surgical intervention for esophageal atresia in patients with trisomy 18.
abstract: Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single-stage esophago-esophagostomy with TEF division (Group 3) and five who underwent two-stage operation (gastrostomy followed by esophago-esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1-year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management.
authors: Division of Medical Genetics, Nagano Children's Hospital, Azumino, Japan; Department of Medical Genetics, Shinshu University Graduate School of Medicine, Matsumoto, Japan.
authors: Eriko
authors: E
authors: Nishi
authors: Shigeru
authors: S
authors: Takamizawa
authors: Kenji
authors: K
authors: Iio
authors: Yasumasa
authors: Y
authors: Yamada
authors: Katsumi
authors: K
authors: Yoshizawa
authors: Tomoko
authors: T
authors: Hatata
authors: Takehiko
authors: T
authors: Hiroma
authors: Seiji
authors: S
authors: Mizuno
authors: Hiroshi
authors: H
authors: Kawame
authors: Yoshimitsu
authors: Y
authors: Fukushima
authors: Tomohiko
authors: T
authors: Nakamura
authors: Tomoki
authors: T
authors: Kosho
mesh_terms: D002423:Cause of Death; D002675:Child, Preschool; D002887:Chromosomes, Human, Pair 18; D004933:Esophageal Atresia / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009026:Mortality; D011183:Postoperative Complications; D011296:Prenatal Diagnosis; D011379:Prognosis; D016896:Treatment Outcome; D014314:Trisomy / Q000175:diagnosis; D000073842:Trisomy 18 Syndrome
keywords: causes of death; esophageal atresia; neonatal intensive care; surgical intervention; survival; trisomy 18