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15 - Esophageal atresia

from Part III - Thorax

Published online by Cambridge University Press:  08 January 2010

By
Spencer W. Beasley ,
Philip A. J. Chetcuti  and
John W. L. Puntis
Edited by
Mark D. Stringer ,
Keith T. Oldham  and
Pierre D. E. Mouriquand
Spencer W. Beasley
Affiliation:
Department of Paediatric Surgery, Christchurch Hospital, New Zealand
Philip A. J. Chetcuti
Affiliation:
Clarendon Wing, Leeds General Infirmary, UK
John W. L. Puntis
Affiliation:
Clarendon Wing, Leeds General Infirmary, UK
Mark D. Stringer
Affiliation:
University of Otago, New Zealand
Keith T. Oldham
Affiliation:
Children's Hospital of Wisconsin
Pierre D. E. Mouriquand
Affiliation:
Debrousse Hospital, Lyon
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Summary

Introduction and historical aspects

William Durston is credited with providing the earliest description of esophageal atresia (EA) when he observed its occurrence in one of conjoined twins in 1670. In 1888, Charles Steele was the first to attempt surgical correction of EA. Although the child died, it gave an indication that the abnormality was potentially treatable. It was not until 1939 that Ladd and Leven independently obtained the first long-term survivors with EA. Two years later Cameron Haight successfully achieved a primary esophageal anastomosis.

Before 1939, esophageal atresia was considered a uniformly fatal condition. Nowadays, all patients with EA are expected to survive irrespective of their gestation, provided there are no major concomitant congenital abnormalities. There has been a steady decline in the overall mortality from EA in most institutions, but there remains a significant group of patients who will die as a result of their associated abnormalities: the prognosis for many of these infants is considered so poor that no active treatment to correct the atresia is justified.

The eventual quality of life is good in the majority of survivors. Despite this, many have ongoing minor but troublesome symptoms, many of which relate to esophageal function (Table 15a.1). In particular, there are concerns about the ultimate risk of malignancy in the esophagus exposed to persisting gastroesophageal reflux. This chapters outlines how the early management of esophageal atresia influences long term outcome, and identifies some of the persistent difficulties that adults with repaired EA may suffer.

Type
Chapter
Information
Pediatric Surgery and Urology
Long-Term Outcomes
 , pp. 192 - 216
Publisher: Cambridge University Press
Print publication year: 2006

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