Unilateral pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula: Report of two cases

Mark A. Hoffman*, Riccardo Superina, David E. Wesson

*Corresponding author for this work

Research output: Contribution to journalArticle

18 Scopus citations


The combined anomalies of pulmonary agenesis with esophageal atresia and distal tracheoesophageal fistula are an exceedingly rare and highly lethal association. The longest survivor in the literature is 10 months. We describe two neonates with this association who underwent primary repair shortly after birth and who are alive and well at 24 and 18 months. One of these neonates also demonstrated duodenal atreasia. The prime goal in the management of these patients is early protection and preservation of respiratory units. This goal is best accomplished by primary repair of the tracheoesophageal lesion, when feasible, rather than by more complex procedures.

Original languageEnglish (US)
Pages (from-to)1084-1085
Number of pages2
JournalJournal of pediatric surgery
Issue number10
StatePublished - Oct 1989



  • Pulmonary agenesis
  • duodenal atresia
  • esophageal atresia
  • tracheoesophageal fistula

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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