Hirschsprung's Disease (Adult) Treatment | Pristyn Care

Risk Factors

Risk factors include:

• Family history of Hirschsprung’s disease
• Male sex (4:1 male to female ratio)
• Down syndrome (trisomy 21)
• Other congenital anomalies
• RET gene mutations in family members

Who Is at Risk

Males with a family history of HD, those with Down syndrome, and individuals with long-standing refractory constipation diagnosed since childhood are at highest risk for adult-presenting Hirschsprung’s disease.

Diagnosis

Diagnosis involves:

• Contrast enema (barium) showing transition zone
• Anorectal manometry (absence of recto-anal inhibitory reflex is diagnostic)
• Rectal suction biopsy (gold standard) showing absence of ganglion cells
• Full-thickness rectal biopsy if suction biopsy inconclusive
• Genetic testing for RET and other mutations

Treatment for Hirschsprung's Disease (Adult)

Pristyn Care performs laparoscopic Swenson, Soave, or Duhamel pull-through procedures to remove the aganglionic segment and restore normal bowel function in adult HD patients.

Procedure

Surgical procedure involves:

• Pre-operative bowel preparation and nutritional optimisation
• Laparoscopic mobilisation of the colon to identify the transition zone
• Intraoperative frozen section biopsy to confirm normal ganglion cells
• Resection of the aganglionic segment
• Pull-through procedure (Swenson/Soave/Duhamel technique)
• Colorectal anastomosis with preservation of sphincter function

After the Surgery

Post-operative care includes:

• Nasogastric tube decompression initially
• Gradual reintroduction of oral diet
• Bowel habit monitoring and diary
• Pelvic floor physiotherapy if continence is affected
• Regular follow-up colonoscopy
• Dietary fibre and fluid intake optimisation

Possible Complications of Hirschsprung's Surgery

Potential complications:

• Hirschsprung-associated enterocolitis (HAEC) post-operatively
• Anastomotic leak or stricture
• Bowel frequency and urgency issues
• Temporary or permanent faecal incontinence
• Residual aganglionic segment requiring re-operation