- 10-yr-old female patient presents for evaluation.
- Her history included that he presented with a right colon perforation at 7 wk of age.
- She underwent partial colon resection and ileostomy.
- Incorrectly diagnosed with Hirschsprung disease.
- She underwent Duhamel procedure at 9 mo of age.
- Her operative note included mention of removal of colonic “spur” but it did not clearly state how much of her colon was removed.
- She has been having loose/watery stools, bowel movements 3-4 per day, and sleeps in a pull up due to urinary incontinence.
- She does not have incontinence of stool.
- Recently started to have vague abdominal pain/ nausea. This was crampy and sometimes associated with eating but there was not a clear pattern.
- Contrast enema showed that the contrast fills the small bowel that is dilated for 30 cm.
- Difficult to correlate the contrast enema findings with the available operative notes.
- CT abdomen with rectal contrast showed a widely patent ileo-rectal anastomosis, long segment of residual colon, distal small bowel dilated to 5 cm and there was no mechanical obstruction.
- There was external compression on the ileum by the dilated colon.
- Patient did not have Hirschsprung disease based on review of the pathology.
- Unfortunately, there are many cases of patients who are operated on for Hirschsprung disease but were misdiagnosed.
- Reoperations are technically difficult procedures so risk of potential complications should be considered.
- The colon on the contrast study does not look dilated which means it likely has peristalsis.
- A protective stoma should always be placed when there is an incision in the posterior rectal wall to prevent leak and allow healing.
- Enemas could be administered into the residual colon to see if the abdominal discomfort improves.
- This patient does not have Hirschsprung disease.
- A re-do Duhamel could be performed.
Patient Case Discussion