Pd. Losty et al., CONGENITAL DIAPHRAGMATIC-HERNIA - DOES THE SIDE OF THE DEFECT INFLUENCE THE INCIDENCE OF ASSOCIATED MALFORMATIONS, Journal of pediatric surgery, 33(3), 1998, pp. 507-510
Background/Purpose: Patients with congenital diaphragmatic hernia (CDH
) frequently have associated anomalies, Experiments in the nitrofen CD
H model have shown differential embryonic cell death patterns in roden
ts suggesting unique mechanisms in the formation of right-sided (RCDH)
or left-sided (LCDH) diaphragmatic hernia. These findings provide ins
ight into the pathogenesis of CDH and may aid our understanding on the
spectrum of associated anomalies commonly observed in humans, This st
udy therefore set out to test the hypothesis that the side of the diap
hragmatic defect in humans is related to the incidence and severity of
coexistent organ malformations. Methods: The medical and autopsy reco
rds of 301 CDH patients presenting to two institutions over a 23-year
period were examined to analyze these factors. Results: One hundred pa
tients (33%) were found to have one or more associated anomalies. The
incidence of multiple-RCDH (10%) versus LCDH (7.3%) and cardiac anomal
ies-RCDH (10%) versus LCDH (8.5%) was similar in both groups of patien
ts, However, the hypoplastic heart syndrome was a unique feature in 5
of 22 patients (23%) with LCDH who had cardiac abnormalities. This car
diac anomaly may be related developmentally to LCDH. Conclusion: The c
ellular mechanisms underlying the genesis of th is spectrum of abnorma
lities in humans and the nitrofen CDH model warrant further study to e
lucidate factors governing embryonic cell fate and phenotype expressio
n. Copyright (C) 1998 by W.B. Saunders Company.