What is the procedure for performing a pullthrough operation in Hirschsprung's disease?

Pullthrough Procedure for Hirschsprung Disease

The optimal surgical approach for Hirschsprung disease is a transanal pullthrough (TAPT) procedure with laparoscopic assistance, using the anorectal line as the landmark for dissection to ensure optimal postoperative bowel function. 1

Preoperative Considerations

• Accurate diagnosis must be confirmed with rectal biopsy showing absence of ganglion cells before proceeding with surgical intervention 2
• The extent of aganglionosis should be determined preoperatively through contrast studies and biopsies to plan the appropriate extent of resection 3
• Patients should be optimized nutritionally before surgery, especially those presenting with enterocolitis or intestinal obstruction 2

Surgical Technique

One-Stage Neonatal Pullthrough

• One-stage neonatal pullthrough is preferred over traditional three-stage procedures as it avoids stoma complications, reduces hospitalizations, and provides comparable outcomes 4
• The procedure can be safely performed in neonates as young as 10-15 days of age with excellent results 4, 5

Laparoscopic-Assisted Approach

• Begin with placement of a 5-mm umbilical trocar and two additional 5-mm trocars on either side of the umbilicus for a single-incision laparoscopic surgery (SILS) technique 3
• Mobilize the affected colon laparoscopically using high-frequency cutting and sealing devices 3
• Take multiple seromuscular biopsies to determine the transition zone between aganglionic and ganglionic bowel 2
• Ensure complete mobilization of the colon beyond the peritoneal reflection 3

Transanal Component

• Use the anorectal line (ARL) rather than the dentate line as the landmark for beginning the transanal dissection to preserve the delicate sensory innervation in the anal transition zone 1
• Create a circumferential mucosal incision at the anorectal line 1
• Develop a submucosal plane and continue proximal dissection for 3-4 cm 4
• Perform a full-thickness rectal wall transection and continue proximal dissection in the perirectal plane 4
• Pull the ganglionic bowel through the muscular cuff and perform a coloanal anastomosis 3, 1
• Complete total excision of the posterior rectal cuff to ensure normal defecation 1

Special Considerations for Long-Segment Disease

• For long-segment Hirschsprung disease (LSHD), a subtotal colectomy may be required 3
• The affected colon should be mobilized successively beyond the peritoneum before performing the pullthrough procedure 3
• In cases of total colonic aganglionosis, more extensive resection with ileoanal pullthrough may be necessary 3

Postoperative Care

• Monitor for early complications including anastomotic leak, wound infection, and enterocolitis 4
• Patients with Down syndrome may have higher complication rates and require closer monitoring 4
• Begin enteral feeding once bowel function returns, typically within several days postoperatively 4

Long-term Follow-up

• Objective assessment of postoperative bowel function is essential for thorough follow-up 1
• Monitor for late complications such as enterocolitis, stool retention, intestinal obstruction, or incontinence 2
• Most patients achieve normal bowel control with 3-4 stools per day by follow-up at 2 years 4, 5
• For patients with persistent complications after primary pullthrough, redo procedures may be necessary and should be performed at specialized pediatric referral centers 2

Outcomes

• One-stage pullthrough procedures have shown excellent results with low complication rates 4
• Expected hospital stay is approximately 20 days for the initial procedure 4
• Long-term continence rates are excellent, with most patients achieving normal bowel control 4, 5
• Enterocolitis may occur in approximately 18% of patients postoperatively 3