9-Year Old-Male Patient Born with Trisomy 21, Hyperthyroidism, and Hirschsprung Disease

• 9-yr-old male patient born with trisomy 21, hyperthyroidism, and Hirschsprung disease presents for evaluation.
• He underwent Soave pull-through at 9 mo.
• He was initially evaluated by Gastroenterology for “constipation and overflow pseudo-incontinence” with a plan to administer rectal Botox.
• Colorectal Surgery was invited to examine the patient while in the operating room.
• Anal canal was evaluated and noted to have rectal mucosa sutured to the skin. The anal canal was damaged.
• Contrast enema was subsequently obtained, and daily enemas were initiated with an enema of 250 mL normal saline and 10 mL glycerin.
• After 2 mo, a Malone procedure was performed.

Takeaways

• If a patient has been operated on for Hirschsprung disease by another surgeon, an examination under anesthesia should be performed to evaluate the integrity of the anal canal.
• Botox should not be administered to a patient with a damaged anal canal because it will cause the patient to have fecal incontinence.
• Patients with Hirschsprung disease are born with a normal anus. If the anal canal is damaged during the pull-through procedure, the patient will not have bowel control. Laxatives are contraindicated in patients without bowel control.