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- congenital malformations
- tracheo-oesophageal fistula
- cystic malformations
- congenital diaphragmatic hernia
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 …
Footnotes
Reproduced in full with permission from Thorax 2001;56:65–72
Series editors: P Sly, S Stick
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