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Hirschsprung's Disease in Children

Hirschsprung’s Disease Treatment, Denver

Hirschsprung’s Disease is a condition affecting the large intestine/colon. It is typically diagnosed in the first few days after birth and most cases are identified before the age of two. The pediatric surgeons of Rocky Mountain Pediatric Surgery and neonatologists of the Center for Maternal Fetal Health at Rocky Mountain Hospital for Children, Denver are specially trained to diagnose and treat this rare condition. In addition, our staff and facility are uniquely equipped to handle prompt treatment of this condition after birth.

About Hirschsprung’s Disease

Babies born with Hirschsprung’s Disease are missing nerves in the muscle lining of the colon (large intestine). These nerves control muscle contractions (peristalsis) in the colon, allowing stool to move through the intestines and waste to be eliminated. In babies with Hirschsprung’s Disease, the nerve cells are not fully formed and the muscles do not contract properly. As a result, stool backs up behind the underdeveloped area, which can lead to a potentially life-threatening infection called enterocolitis. Hirschsprung’s Disease can affect the entire colon (long-segment disease) or, more commonly, affect only the area close to the rectum (short-segment disease).  

Hirschsprung’s Disease is a congenital, or inborn, condition. Typically it affects more males than females, and runs in families. It also is often seen in patients with other congenital defects, especially Down’s syndrome.

Hirschsprung’s Disease Diagnosis

Most infants are diagnosed with Hirschsprung’s disease after failing to have a bowel movement in the first 48 hours after birth. Your pediatrician also may look for additional symptoms such as:

  • Swollen belly
  • Vomiting (especially green or brown)
  • Gas
  • Diarrhea
  • Failure to gain weight (older children)
  • Fatigue (older children)
  • Chronic constipation (older children)

If Hirschsprung’s Disease is suspected, physicians typically use a barium enema/ lower GI exam to confirm the diagnosis. This special X-ray procedure uses dye to provide a clear view of the intestine. In Hirschsprung’s Disease patients, the intestine will show as narrowed where cells are missing. Doctors also may want to perform a rectal suction biopsy to remove and test cells from colon mucous lining. Tests for older children may include manometry (inflating a small balloon inside the rectum to test the muscles of the anus) or a surgical biopsy (removing a sample of tissue from the colon).

Hirschsprung’s Disease Treatment

Surgery is the recommended treatment for Hirschsprung’s Disease. The team at Rocky Mountain Pediatric Surgery is experienced in treating this condition and attending to the needs of the smallest patients. Additionally, our facility is uniquely equipped with knowledgeable, experienced staff and specialized equipment to care for the unique needs of a newborn, baby or child undergoing surgery.

Our pediatric surgeons are skilled in using laparoscopic surgery techniques to perform Hirschsprung’s Disease surgery, these minimally invasive techniques utilize to smaller incisions, which leads to shorter recovery times and less pain. Typically, surgery is performed within the first few days or the first month after birth. During the surgery, the affected section of the colon is removed and the healthy section of the intestine is attached to the rectum. In some cases (usually older children), an ostomy (either an ileostomy or a colostomy) may need to be performed first. During this procedure, the healthy portion of the intestine is attached temporarily to a small hole (stoma) in the abdomen through which stool will leave the body and be collected in a bag. This creates a bypass, giving the intestine a chance to heal. Another surgery then is performed a few months to a year later to close up the stoma and reattach the healed intestine.

Long-Term Outlook for Hirschsprung’s Disease

After proper treatment, babies with Hirschsprung’s Disease typically are able to pass stool normally without complication. If a complication arises, it is most often lingering constipation or bowel control issues, which our team will continue to assess and work to correct.

To find pediatric surgeon experienced in Hirschsprung’s Disease treatment, contact our program care coordinator at 720-754-4902.