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Am J Med Genet. 1990 Jan;35(1):46-54.

Aberrant bronchi and cardiovascular anomalies.

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1
Department of Human Genetics, University of Manitoba, Winnipeg, Canada.

Abstract

In an investigation of malformation associations in consecutive perinatal autopsies, 4 infants were identified as having a displaced or supernumerary bronchus. Each had a different type of bronchial abnormality and 3 had congenital heart defects and other malformations. Review of the literature found 38 other cases of aberrant bronchi with additional defects, especially cardiovascular or costovertebral anomalies. Structural cardiac defects were more common in patients with super-numerary tracheal bronchi (67%) than in those with displaced tracheal bronchi (27%) or bronchoesophageal connections (23%). There was also a strong negative association (P = 0.01) of cardiovascular and costovertebral defects unless multiple anomalies were present. The combinations of anomalies seen appear to reflect relatively specific developmental field defects affected both by the spatial relationships of organs near the developing foregut and by temporal sequence. Recognition of these patterns has clinical and embryological importance. Aberrant bronchi should be considered when children with cardiac defects or multiple anomalies such as the VACTERL association have unexplained respiratory symptoms and surgeons planning to treat such bronchial abnormalities should be aware of the high frequency of abnormal vessels in these cases.

PMID:
2405669
DOI:
10.1002/ajmg.1320350109
[Indexed for MEDLINE]

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