Article Text

Download PDFPDF
Adult outcome of congenital lower respiratory tract malformations
  1. M S Zach,
  2. E Eber
  1. Respiratory and Allergic Diseases Division, Paediatric Department, University of Graz, Austria
  1. Correspondence to:
    Prof Dr M Zach, Klinische Abteilung für Paed. Pulmonologie/Allergologie, Univ Klinik für Kinder- und Jugendheilkunde, Auenbruggerplatz 30, A-8036 Graz, Austria;
    maximilian.zach{at}kfunigraz.ac.at

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Congenital malformations of the lower respiratory tract are usually diagnosed and managed in the newborn period, in infancy, or in childhood. To what extent should the adult pulmonologist be experienced in this predominantly paediatric field?

There are three ways in which an adult physician may be confronted with this spectrum of disorders. The most frequent type of encounter will be a former paediatric patient, now reaching adulthood, with the history of a surgically treated respiratory malformation; in some of these patients the early loss of lung tissue raises questions of residual damage and compensatory growth. Secondly, there is an increasing number of children in whom paediatric pulmonologists treat respiratory malformations expectantly; these patients eventually become adults with their malformation still in place. Thirdly, there is a small group of patients in whom the malformation goes unrecognised throughout childhood; in these a late complication or the coincidental discovery of a radiographic anomaly may demand a late diagnosis and management in adulthood.

All three of these adult presentations will require some expertise on the part of the managing physician. The subsequent text reviews the most common “classical” congenital malformations of the lower respiratory tract from the above three perspectives.

TRACHEO-OESOPHAGEAL FISTULA (TOF)

Paediatric presentation

TOF usually occurs in association with oesophageal atresia; thus, the outcome is invariably fatal unless the anomaly is surgically repaired in the first days of life. The rare “H type” TOF without oesophageal atresia is an exception to this rule and may persist into later childhood or even adulthood before the diagnosis is established.1 The malformation is explained by the defective progression of a complex system of folds that separate the foregut into trachea and oesophagus.2 Approximately 50% of infants with TOF have associated anomalies, most often involving the urinary, gastrointestinal, and cardiac systems.3,4

The first successful repair of …

View Full Text

Footnotes

  • Reproduced in full with permission from Thorax 2001;56:65–72

  • Series editors: P Sly, S Stick

Linked Articles

  • Atoms
    Harvey Marcovitch