ABSTRACT

Surgery for esophageal atresia (EA) is widely regarded as one

of the most notable success stories in newborn surgery.

Advances in neonatal intensive care have now led to greater

than 95% survival for many babies treated in the modern era

with increasing attention now focusing on morbidity, long-

term clinical outcomes, and quality of life (QoL) in adult

survivors. Recent noteworthy developments in EA manage-

ment include minimally invasive thoracoscopic surgery and

the deployment of the axillary skin crease thoracotomy

pioneered by Bianchi. Expert opinion controversially dom-

inates best practice with regard to pure long-gap esophageal

atresia without fistula, medical versus surgical treatment of

gastro-esophageal reflux (GER) disease, therapies for anasto-

motic stricture, and tracheomalacia. Applied embryology and

molecular genetic studies continue to yield fascinating back-

ground into the etiology of EA and tracheo-esophageal fistula

(TEF), with insightful contributions emerging from animal

models sharing striking similarity to the human phenotype.2,3

The history of EA and TEF is well described in the literature.2,3

The first survivors were not recorded until 1939 with Leven

and Ladd achieving success with staged esophageal repair.

Cameron Haight (an American surgeon working at Ann

Arbor, Michigan) is fully credited with the first successful

primary repair and survival of a 12-day-old female neonate.

Reports from the UK soon followed with Franklin (1947) at

the Hammersmith Hospital, London, Sir Denis Browne (1948)

at Great Ormond Street Hospital, London, and Peter Paul

Rickham (1949) at Alder Hey Children’s Hospital in Liverpool