PEDIATRICS Vol. 42 No. 3 September 1968, pp. 525-528
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Choanal Atresia and/or Cardiac Disease

Sylvan E. Stool M.D.1 and Bennett I. Kemper M.D.2

1 Departments of Pediatrics and, Otolaryngology, University of Pennsylvania
2 Department of Otolaryngology, Children's Hospital of Philadelphia, University of Pennsylvania, 18th and Bainbridge Streets, Philadelphia, Pennsylvania 19146

There are few catastrophies in the neonatal period which present so dramatically and uniquely, yet are remedied so quickly and simply, as congenital atresia of the posterior choanae. The lesion, once thought to be rare, is being detected more frequently and probably accounts for 0.5% per thousand major congenital anomalies. Unfortunately, even with early accurate diagnosis, many of these infants do not survive associated organic defets.1-4

Over 50% of the patients with bilateral choanal atresia studied at the Children's Hospital of Philadelphia between January 1960 and January 1967 had associated life-threatening congenital anomalies, especially cardiac disorders. One might speculate that maldevelopment in both organ systems occurs during the sixth to eighth week of fetal development, although no chromosomal aberration, chemical agent, or physical agent has been correlated.