- 16-yr-old presents for evaluation because of fecal impaction.
- History of constipation from 6 mo of age.
- He has been to the Emergency Department multiple times for disimpaction.
- He did not have appropriate follow up.
- He had multiple visits to the gastroenterology clinic, where polyethylene glycol and enemas were recommended.
- Mother was a teenager when the patient was born and was illiterate with developmental delay.
- He was admitted to the hospital due to fecal impaction.
- Diagnosed with generalized anxiety disorder and attention deficit hyperactivity disorder.
- Manometry study showed inhibitory rectoanal reflex present, preserved rectal sensitivity, hypotension, and decreased rectal contractility with findings of anal tone not recovered after the inhibitory rectoanal reflux.
- Normal MRI.
- Fecal disimpaction was performed and a rectal biopsy that was aganglionic.
- Protectomy with transanal and abdominal approach, colectomy of the proximal dilated colon above the aganglionosis, pull-through of the descending colon, and colorectal anastomosis were performed.
- Interoperative biopsy was obtained 12 cm from the pectinate line.
- Jackson Pratt drain was left in place.
- 4 d after pull-through, patient began to have feculent drainage from the Jackson Pratt drain.
- X-ray showed intra-abdominal free air so an ileostomy was performed.
- Laparotomy showed a clean abdominal cavity without abscesses.
- During the postoperative period, rectal dilations were performed with a size 24 hegar dilator.
- Distal ileostogram was performed 4 mo after the pull-through, which showed patency of the distal bowel.
- Ileostomy closure was subsequently performed.
- This patient likely had an anastomotic leak which caused feculent drainage from the JP drain which healed on its own with diversion.
- Hirschsprung disease is a spectrum of diagnoses from benign to severe disease. For this patient to have lived to age 16 prior to diagnosis he must have been on the more benign end of the spectrum.
- With a massive dilation of the colon, a protective diversion with a stoma during surgery could be considered.
Patient Case Discussion