Long Colon "Spur" After Duhamel Procedure

Case Summary

• 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.

Takeaways

• 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.