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Radiological findings in total aganglionosis coli
Insights
Total colonic aganglionosis, a form of Hirschsprung's disease, presents with bowel obstruction in infants. Radiological findings vary, emphasizing the need for biopsy for definitive diagnosis.
Area of Science:
- Pediatric Radiology
- Gastrointestinal Surgery
- Medical Imaging
Background:
- Total colonic aganglionosis is a severe form of Hirschsprung's disease.
- Early diagnosis is crucial for effective management of congenital intestinal disorders.
Purpose of the Study:
- To review radiological findings in patients diagnosed with total colonic aganglionosis.
- To evaluate the utility of barium enema in diagnosing this condition.
Main Methods:
- Retrospective review of radiological findings in 13 patients with total colonic aganglionosis.
- Analysis of plain films and barium enema studies.
Main Results:
- All patients presented with plain film evidence of bowel obstruction.
- Barium enema findings were non-specific, showing a spectrum of abnormalities including normal calibre colon, microcolon, shortened colon, colonic wall irregularity, and ileal reflux.
- Delayed barium evacuation was noted in two cases.
Conclusions:
- Total colonic aganglionosis should be suspected in infants with plain film evidence of bowel obstruction, irrespective of barium enema findings.
- Definitive diagnosis of Hirschsprung's disease and transition zone requires biopsy.
Abstract:
The radiological findings in 13 patients with total aganglionosis coli were reviewed. There was a male to female ratio of 7:6. Fifty-four percent of patients presented in the first week of life, but a significant number (31%) did not present until after 1 month of age. All patients had plain film evidence of bowel obstruction when referred for a barium enema. There were no pathognomonic barium enema findings, and barium enema results covered the entire spectrum of findings which can be encountered in the neonate and young infant with bowel obstruction. Seventy-seven percent had normal calibre colon, 23% had micro colon, 23% had a shortened colon, 46% had colonic wall irregularity, 33% had significant ileal reflux. Delayed evacuation of barium from colon occurred in the two patients who had delayed films. Total colonic aganglionosis should be considered in any infant or young child with plain film evidence of bowel obstruction, whatever the barium enema findings. Hirschsprung's disease and the level of transition can only be definitively diagnosed by biopsy.