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Published in: Pediatric Surgery International 10/2020

01-10-2020 | Gastrostomy | Original Article

Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality

Authors: K. M. O’Shea, M. L. Griffiths, K. L. King, P. Losty, M. Jones, J. Minford, F. Murphy

Published in: Pediatric Surgery International | Issue 10/2020

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Abstract

Introduction

Oesophageal atresia ± tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a worse prognosis than EA/TEF alone. Though the Spitz classification takes major CHD into account, there are no data regarding survival with the specific combination of EA/TEF and Tetralogy of Fallot (TOF). With advances in postnatal care, we hypothesised that, survival is improving in these complex patients. This study reports morbidity and mortality outcomes of newborns with oesophageal atresia and TOF cardiac malformations

Methods

All patients with EA/TEF and TOF treated at Alder Hey Children’s Hospital between the years 2000–2020, were identified. Data sets regarding gestation, birth weight, associated anomalies, operative intervention, morbidity, and mortality were analysed.

Results

Of a total of 350, EA/TEF patients 9 (2.6%) cases had EA/TEF associated with TOF (M:F 4:5). The median gestational age was 35/40 (range 28–41 weeks) with a median birth weight of 1790 g (range 1060–3350 g). Overall survival was 56% (5/9 cases) and all survivors remain under follow up (range 37–4458 days). Surgical strategies for managing EA/TEF with Fallot’s tetralogy included 6/9 primary repairs and 3/9 cases with TEF ligation only (+ gastrostomy ± oesophagostomy).

Conclusions

This study reports outcome data from one of the largest series of EA TEF patients with Fallot’s tetralogy. Whilst outcomes may be challenging for this unique patient cohort, survival metrics provide important prognostic information that can be widely shared with health care teams and parents.
Literature
1.
go back to reference Leonard H et al (2001) The influence of congenital heart disease on survival of infants with oesophageal atresia. Arch Dis Child Fetal Neonatal Ed 85(3):F204–F206CrossRef Leonard H et al (2001) The influence of congenital heart disease on survival of infants with oesophageal atresia. Arch Dis Child Fetal Neonatal Ed 85(3):F204–F206CrossRef
2.
go back to reference Spitz L et al (1990s) Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg 29(6):723–725CrossRef Spitz L et al (1990s) Oesophageal atresia: at-risk groups for the 1990s. J Pediatr Surg 29(6):723–725CrossRef
4.
go back to reference Tennant PW et al (2010) 20-year survival of children born with congenital anomalies: a population-based study. Lancet 375(9715):649–656CrossRef Tennant PW et al (2010) 20-year survival of children born with congenital anomalies: a population-based study. Lancet 375(9715):649–656CrossRef
5.
go back to reference Gross RE (1953) The surgery of infancy and childhood. Its principles and techniques. W.D Saunders Co, Philadelphia Gross RE (1953) The surgery of infancy and childhood. Its principles and techniques. W.D Saunders Co, Philadelphia
6.
go back to reference Sulkowski JP et al (2014) Morbidity and mortality in patients with esophageal atresia. Surgery 156(2):483–491CrossRef Sulkowski JP et al (2014) Morbidity and mortality in patients with esophageal atresia. Surgery 156(2):483–491CrossRef
7.
go back to reference Stoll C et al (2017) Associated anomalies in cases with esophageal atresia. Am J Med Genet Part A 173(8):2139–2157CrossRef Stoll C et al (2017) Associated anomalies in cases with esophageal atresia. Am J Med Genet Part A 173(8):2139–2157CrossRef
8.
go back to reference David TJ, O'Callaghan SE (1974) Cardiovascular malformations and oesophageal atresia. Br Heart J 36(6):559–565CrossRef David TJ, O'Callaghan SE (1974) Cardiovascular malformations and oesophageal atresia. Br Heart J 36(6):559–565CrossRef
9.
go back to reference Webber EM, Gillis DA, Ross DB (1996) Tetralogy of Fallot with total anomalous pulmonary venous drainage and esophageal atresia: complete correction in infancy. Ann Thorac Surg 62(2):571–573CrossRef Webber EM, Gillis DA, Ross DB (1996) Tetralogy of Fallot with total anomalous pulmonary venous drainage and esophageal atresia: complete correction in infancy. Ann Thorac Surg 62(2):571–573CrossRef
10.
go back to reference Puri K et al (2018) Characteristics and outcomes of children with ductal-dependent congenital heart disease and esophageal atresia/tracheoesophageal fistula: a multi-institutional analysis. Surgery 163(4):847–853CrossRef Puri K et al (2018) Characteristics and outcomes of children with ductal-dependent congenital heart disease and esophageal atresia/tracheoesophageal fistula: a multi-institutional analysis. Surgery 163(4):847–853CrossRef
11.
go back to reference Diaz LK et al (2005) Tracheoesophageal fistula and associated congenital heart disease: implications for anesthetic management and survival. Paediatr Anaesth 15(10):862–869CrossRef Diaz LK et al (2005) Tracheoesophageal fistula and associated congenital heart disease: implications for anesthetic management and survival. Paediatr Anaesth 15(10):862–869CrossRef
12.
go back to reference Waterston DJ, Carter RB, Aberdeen E (1962) Oesophageal atresia: tracheo-oesophageal fistula: a study of survival in 218 infants. Lancet 1(7234):819–822CrossRef Waterston DJ, Carter RB, Aberdeen E (1962) Oesophageal atresia: tracheo-oesophageal fistula: a study of survival in 218 infants. Lancet 1(7234):819–822CrossRef
13.
go back to reference Poenaru D et al (1993) A new prognostic classification for esophageal atresia. Surgery 113(4):426–432PubMed Poenaru D et al (1993) A new prognostic classification for esophageal atresia. Surgery 113(4):426–432PubMed
Metadata
Title
Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality
Authors
K. M. O’Shea
M. L. Griffiths
K. L. King
P. Losty
M. Jones
J. Minford
F. Murphy
Publication date
01-10-2020
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Surgery International / Issue 10/2020
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI
https://doi.org/10.1007/s00383-020-04732-x

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