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Congenital aganglionosis of the entire colon in neonates
Insights
Total colonic aganglionosis (TCA), a severe form of Hirschsprung's disease, is often missed in neonates. Early recognition through specific barium enema findings is crucial to prevent high mortality from enterocolitis.
Area of Science:
- Pediatric Radiology
- Gastroenterology
- Neonatology
Background:
- Total colonic aganglionosis (TCA) is an underdiagnosed variant of long segment Hirschsprung's disease.
- TCA presents a significant diagnostic challenge in neonates, often leading to delayed treatment.
- The condition is associated with high mortality rates, frequently complicated by enterocolitis.
Observation:
- Radiological diagnosis in six neonatal cases is reported.
- Obstructive plain film changes in neonates warrant suspicion for colonic aganglionosis.
- Barium enema findings in TCA can be subtle and easily overlooked.
Findings:
- Diagnostic barium enema findings include free ileal reflux.
- A transition point identified in the ileum is a key diagnostic indicator.
- Persistent barium retention throughout the entire colon post-examination is diagnostic for TCA.
Implications:
- Improved recognition of TCA can lead to earlier intervention.
- Timely diagnosis and management can reduce the high mortality associated with TCA.
- Radiologists and clinicians should be aware of specific barium enema signs suggestive of TCA in neonates.
Abstract:
A report of six cases and their radiological diagnosis in neonates. Total colonic aganglionosis (TCA) is a variety of what is commonly known as long segment Hirschsprung's disease. This is not a rare condition, but is often unrecognized. It has a high mortality, frequently with a complicating enterocolitis. Aganglionosis of some part of the colon should be suspected in all babies who show obstructive plain film changes. In TCA the barium enema changes may easily be passed as unremarkable. However, free ileal reflux during the examination, with a transition point in the ileum, and retention of barium in the entire colon after the examination, are diagnostic.