Diagnosis, Treatment, and Recovery

Hirschsprung's Disease

When a child is born with Hirschsprung's disease, part of their colon does not have nerve cells. These nerve cells are important because they help push stool through the intestines. Without them, the colon cannot move stool properly, which can cause constipation, swelling and stomach pain.

Diagnosis of Hirschsprung’s Disease

Most babies with Hirschsprung's disease are diagnosed within the first few days of life, especially if they have difficulty passing their first bowel movement, called meconium, or experience severe bloating and vomiting.

Diagnosing the condition involves a combination of medical history evaluation, physical examination, and specialized tests:

  • Medical and Family History: Your health care provider will ask about your baby’s symptoms and any family history of Hirschsprung's disease.
  • Physical Examination: This may include checking for abdominal swelling and performing a digital rectal exam to evaluate for stool presence and muscle tone.
  • Diagnostic Tests:
    • Imaging Studies: A contrast enema X-ray can highlight areas of the intestine lacking nerve cells.
    • Rectal Biopsy: The definitive test where a small tissue sample is taken from the rectum to check for the absence of nerve cells.
    • Manometry test: Measures the muscle reflexes in the rectum

Symptoms of Hirschsprung's Disease

Symptoms typically appear within the first 48 hours of life or later in childhood. Common symptoms include:

  • Failure to pass the first stool, called meconium, within 24-48 hours after birth
  • Swollen bell, called abdominal distension
  • Vomiting, sometimes green or brown
  • Poor feeding or lack of appetite
  • Chronic constipation in older children
  • Slow growth and weight gain
  • Sever diaper rash due to frequent stool after surgery

A serious complication called Hirschsprung-associated enterocolitis can occur, causing fever, explosive diarrhea, abdominal swelling, and lethargy. This condition requires immediate medical attention.

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Pediatric General Surgery Clinic

CHRISTUS Children's General Surgery Clinic in San Antonio provides exceptional surgical care for infants, children, and teens. 

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Treatment of Hirschsprung’s Disease

Surgical intervention is the primary treatment for Hirschsprung's Disease, removing the affected portion of the colon. 

For corrective treatment, the Swenson pull-through surgery is a common surgical treatment for Hirschprung's Disease. During this procedure, the surgeon removes the part of the intestine that doesn’t work and reconnects the healthy intestine so your child can pass stool normally.

Swenson Pull-through Surgery

Anesthesia and preparation

Your child will undergo anesthesia so they will be safely asleep during the procedure.

Removing the Diseased Colon

  • The surgeon uses a tiny camera and instruments to guide the surgery (laparoscopy) from the abdominal side.
  • The last part of the diseased colon near the sphincter muscles is resected through the anus.
  • The part of the colon that doesn’t have nerve cells is carefully removed.

Pulling Down the Healthy Colon

  • The healthy intestine is brought down and connected to the anus (the opening where stool comes out).
  • Frozen section histology is used to determine what piece of bowel is healthy and safe to pull through
  • You will not see any incisions on the bottom, as all the joining up is done internally.
  • Connecting the healthy parts of the colon together creates a normal pathway for stool to leave the body.
  • After the procedure is complete, the incision is closed, and the body is given time to heal.

Recovery from Swenson Pull-through Surgery

After Swenson Pull-Through Surgery, children may receive IV fluids for hydration and nutrition in the first few days until their intestines begin to function normally. In some cases, a temporary stoma may be required if additional healing time is needed. This stoma is typically removed after a couple of months when healing is complete.

In the recovery process, some children may experience frequent loose stools, constipation, or issues with bowel control. Stool softeners, dietary adjustments, or medications may be necessary to help manage bowel movements. Regular follow-ups with the doctor will be scheduled to monitor healing, address concerns, and ensure the child’s progress.

While every child’s recovery is unique, many go on to experience normal bowel function and live healthy lives. However, enterocolitis can still occur even after surgery. Parents should watch for signs such as fever, diarrhea, or abdominal swelling and seek medical care promptly if these symptoms arise. In some cases, a long-term bowel management program may be needed. 

Temporary Ostomy

If your child is unable to undergo surgery immediately, your surgeon may recommend a temporary stoma (a small opening in the stomach that allows stool to pass from the intestine into a bag) until surgery is possible. This allows the bowel to decompress and heal before the main corrective procedure.

Long-Term Outlook for Children with Hirschsprung's Disease

With proper treatment and ongoing care, most children with Hirschsprung’s disease grow up healthy and lead normal lives. Our team of specialists, including pediatric colorectal surgeons, gastroenterologists, nutritionists and psychologists helps ensure the best possible outcomes for children with Hirschsprung disease.