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. © 2013 Wiley Periodicals, Inc.

Fetal outcome of trisomy 18 diagnosed after 22 weeks of gestation: Experience of 123 cases at a single perinatal center

“…Although palliative care had been adopted for trisomy 18 patients, there have recently been reports on intensive treatment, including invasive procedures, for infants with trisomy 18 (Graham et al 2004;Kosho et al 2006Kosho et al , 2013Kaneko et al 2008Kaneko et al , 2009Iwami et al 2011;Maeda et al 2011;Nishi et al 2013). Iwami et al (2011) reported that the survival rate and the infants who were able to be discharged home increased significantly after intensive treatments, such as surgical operations for heart disease.…”

“…Because of the mortality rate, withholding or withdrawal of intensive treatment (cesarean-section (C-section) for fetal indication, resuscitation, respiratory support and surgery) had been considered and palliative care had been recommended to the parents (Carter et al 1985;Bos et al 1992;Embleton et al 1996). Recently, some reports have been published about the intensive care and the surgical procedures for infants with trisomy 18, concluding that intensive care and surgical procedures could improve the prognosis of infants with trisomy 18 (Graham et al 2004;Kosho 2008;Kosho et al 2006Kosho et al , 2013Kaneko et al 2008Kaneko et al , 2009Iwami et al 2011;Maeda et al 2011;Nishi et al 2013).…”

Clinical courses of children with trisomy 13 receiving intensive neonatal and pediatric treatment

“…We evaluated 24 children with full T13 (15 boys, 9 girls), who were admitted to Nagano Children's Hospital from 1994 to 2016. Children with T18 or T13 were managed under the principle of providing standard intensive neonatal and pediatric treatment through careful discussion with the parents based on evidence from previous literature (Kosho et al, ; Nishi et al, ). Management comprised resuscitation (e.g., intratracheal intubation), appropriate respiratory support, establishment of enteral nutrition (e.g., corrective and palliative surgery for gastrointestinal malformations), and pharmacological treatment for infection, seizures, and congenital heart defects.…”

“…Although cardiac surgery has been suggested to be efficient (Graham et al, ; Kaneko et al, ; Maeda et al, ), the efficacy of intensive neonatal and pediatric management without cardiac surgery has not been described. Previously, we demonstrated efficacy of intensive neonatal treatment without cardiac surgery (Kosho et al, ) and surgery for esophageal atresia (Nishi et al, ) on survival of children with T18 from experiences in Nagano Children's Hospital. This hospital is a tertiary prefectural hospital for sick children in Japan, where intensive neonatal and pediatric management without cardiac surgery is routinely provided to children with T18 or 13.…”

Difficult esophageal atresia: Trick and treat

“…In such syndromes, with poor long-term survival, surgical treatment of associated EA may be seen as cause of unjustified, prolonged suffering to the infant and a waste of the scarce medical resources. A recent report 37 describes the results of surgical treatment of EA in 24 patients with Edwards syndrome. The authors performed palliative surgery in 9 patients (gastrostomy or jejunostomy with/without TEF ligation) and complete repair (primary or delayed) in 15 patients.…”