Male Patient Born with Abdominal Distention, Requiring Reoperation

Case Summary

• 3-yr-old presents for evaluation. He was born and was noted to have abdominal distention, inability to pass stool in first 48 h of life and feeding intolerance.
• On day of life 12, he underwent a transanal pull-through at an outside hospital. He had not had a rectal biopsy prior to that procedure.
• Postoperative course was complicated by pneumonia sepsis with K. pneumonia, X. oxycata, and E. coli.
• At 2 mo of age, he underwent laparotomy with ileostomy and appendectomy.
• He was diagnosed with rectal stenosis at the site of the previous anastomosis.
• He was referred for evaluation due to severe and persistent rectal bleeding.
• Distal ileostogram showed evidence of colitis.
• Stenosis was observed in the pelvis.
• Patient was evaluated by a pediatric gastroenterologist in his home country to rule out inflammatory bowel disease. The only marker that was concerning for inflammatory bowel disease was a positive calprotectin.
• Rectal irrigations without metronidazole plus iron (IM and PO) plus folic acid PO and rectal mesalamine were initiated. Rectal bleeding resolved.
• Proctectomy with resection of the stricture with transanal full thickness and abdominal approach, biopsy above the rectal stenosis (normo-ganglionic), pull-through of the descending colon, colorectal anastomosis (Swenson) and ileostomy closure were performed.
• Final report of the histopathology showed that the distal margin had transition zone (nerve hypertrophy with some submucosal plexus without ganglion cells but all myenteric plexuses with ganglion cells).
• Proximal margin was normoganglionic in both plexus and the stomas were both normoganglionic.
• Patient is currently 4 yr old. He has two bowel movements per day and takes Senna 8.6 mg every day. He received dilation with a size 15 Hegar daily.

Takeaways

• This patient had a severe stricture.
• Historically, it has been difficult based on pathology to differentiate diversion colitis vs enterocolitis. Diversion colitis is a rare finding.
• There is not typically a correlation between inflammatory bowel disease and Hirschsprung disease. It would be rare to have both disease processes.