Surgical intervention for esophageal atresia in patients with trisomy 18

Eriko Nishi, Shigeru Takamizawa, Kenji Iio, Yasumasa Yamada, Katsumi Yoshizawa, Tomoko Hatata, Takehiko Hiroma, Seiji Mizuno, Hiroshi Kawame, Yoshimitsu Fukushima, Tomohiko Nakamura, Tomoki Kosho

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16 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)324-330
Number of pages7
JournalAmerican Journal of Medical Genetics, Part A
Volume164
Issue number2
DOIs
Publication statusPublished - 2014 Feb

Keywords

  • Causes of death
  • Esophageal atresia
  • Neonatal intensive care
  • Surgical intervention
  • Survival
  • Trisomy 18

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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    Nishi, E., Takamizawa, S., Iio, K., Yamada, Y., Yoshizawa, K., Hatata, T., Hiroma, T., Mizuno, S., Kawame, H., Fukushima, Y., Nakamura, T., & Kosho, T. (2014). Surgical intervention for esophageal atresia in patients with trisomy 18. American Journal of Medical Genetics, Part A, 164(2), 324-330. https://doi.org/10.1002/ajmg.a.36294