Abstract
Purpose
The aim of our study was to elucidate if the defect size reflects the magnitude of
the developmental defect in patients with CDH.
Methods
All patients recorded in the CDH Study Group registry between January 1, 2007, and
December 31, 2010, and with defect classification were included in the study. They
were divided according to defect size (A–D) and compared for: gestational age (GA),
birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies,
cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and
hernia sac.
Results
A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and
182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively,
(p < 0.0001; p for trend < 0.0001). GA, BW, prevalence of associated anomalies, particularly CVM, number of abnormal
organ systems, and prevalence of sac were significantly different between the groups,
with a significant reduction of GA, BW, and prevalence of sac. There was an increase
in prevalence of associated anomalies, liver in the chest, and number of abnormal
systems as the defect size increased.
Conclusion
Defect size is directly correlated with mortality rate, prevalence of other anomalies
(particularly CVM), and number of abnormal systems, and inversely with GA, BW, and
prevalence of hernia sac. The defect size may be a marker for the magnitude of developmental
abnormality, thereby explaining its relationship with the outcome.
Key words
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Article info
Publication history
Accepted:
March 8,
2013
Received:
February 17,
2013
Identification
Copyright
© 2013 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
