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Late-presenting congenital diaphragmatic hernia.
Yoshihiro Kitano1, Kevin P Lally, Pamela A Lally
1National Center for Child Health and Development, Tokyo Japan.
Journal of Pediatric Surgery
|December 13, 2005
Summary
Late-presenting congenital diaphragmatic hernia (CDH) can manifest with respiratory or gastrointestinal symptoms. Early diagnosis and primary repair lead to excellent outcomes for this rare condition.
Area of Science:
- Pediatric Surgery
- Congenital Abnormalities
- Gastrointestinal Surgery
Background:
- Late-presenting congenital diaphragmatic hernia (CDH) is a rare condition with limited data.
- Existing information often comes from small case series.
Purpose of the Study:
- To document clinical manifestations of late-presenting CDH.
- Utilize a large, multicenter database for comprehensive data.
Main Methods:
- Retrospective review of 3098 cases from the CDH Study Group (1995-2004).
- Inclusion criteria: CDH diagnosed after 30 days of age.
- Analysis of clinical presentation, hernia location, associated anomalies, and outcomes.
Main Results:
- 79 cases (2.6%) met criteria, diagnosed between 32 days and 15 years.
- Common symptoms: respiratory (43%), gastrointestinal (33%).
- Left-sided hernias predominantly caused GI symptoms; right-sided hernias more often caused respiratory symptoms.
- 100% survival with primary repair, no patch needed.
Conclusions:
- Late-onset CDH presents with diverse symptoms, influenced by hernia laterality.
- Gastrointestinal symptoms common with left-sided hernias; respiratory symptoms with right-sided lesions.
- Prognosis is excellent with timely diagnosis and surgical intervention.