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Surgical intervention for esophageal atresia in patients with trisomy 18
Author(s) -
Nishi Eriko,
Takamizawa Shigeru,
Iio Kenji,
Yamada Yasumasa,
Yoshizawa Katsumi,
Hatata Tomoko,
Hiroma Takehiko,
Mizuno Seiji,
Kawame Hiroshi,
Fukushima Yoshimitsu,
Nakamura Tomohiko,
Kosho Tomoki
Publication year - 2014
Publication title -
american journal of medical genetics part a
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.064
H-Index - 112
eISSN - 1552-4833
pISSN - 1552-4825
DOI - 10.1002/ajmg.a.36294
Subject(s) - medicine , trisomy , gastrostomy , surgery , tracheoesophageal fistula , atresia , radical surgery , esophagectomy , esophageal cancer , cancer , genetics , biology
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. © 2013 Wiley Periodicals, Inc.