1-Month-Old Male Patient with Abdominal Distension

• 1-month-old male patient presents for evaluation.
• Mother was 17 yr old when patient was born.
• On second day of life, he passed meconium, but had recurrent abdominal distension. He was breastfed.
• He was referred for evaluation because of abdominal distension.
• Rectal irrigation was performed, which improved the abdominal distension.
• Rectal biopsy showed lack of ganglion cells in the submucosal plexus, immature cells, and no significant hypertrophic nerves.
• Contrast enema showed a suspected transition zone in the sigmoid.
• Transanal Swenson procedure was performed at 2 mo of age.
• Biopsies at 15 cm, 20 cm, and 24 cm were aganglionic, so the procedure was converted to an abdominal approach.
• Biopsy obtained at 15 cm from the ileocecal valve showed ganglion cells.
• A full thickness pull-through was performed and protective ileostomy placed. The surgeon planned to leave the ileostomy in place until the patient had urinary control.
• The mother learned how to irrigate the distal bowel and the patient received rectal dilations for 6 mo. He received sodium chloride supplementation.
• Patient was lost to follow up for a period of time. He presented again when 3-yr-old with abdominal distension.
• Catheter was placed into the proximal stoma to perform irrigations. This improved the distension.
• Distal ileogram was performed in preparation for ileostomy closure, which identified a stricture proximal to the stoma.
• Rectal examination under anesthesia identified a preserved pectinate line.
• There was a large size discrepancy between proximal and distal stomas.
• A proximal chimney with a Santulli type anastomosis was performed.

Takeaways

• It is important to carefully dissect the rectum using caution near the prostate and the urethra to avoid injury.
• The diverting ileostomy should remain in place until the patient is continent of urine and accepts rectal irrigations. It is important to monitor the ileostomy for stricture. Dilation of the bowel proximal to the stoma on abdominal radiograph might suggest the presence of stricture.
• Metronidazole dosing is 10 mg/kg Q 8 h for treatment if enterocolitis.
• The final pathology confirmed total colonic aganglionosis.
• An alternative anastomosis would be end to side anastomosis.