Hirschsprung’s disease care in Denver

Hirschsprung’s disease is a rare condition affecting the large intestine and colon in infants and young children. Rocky Mountain Hospital for Children’s (RMHC) specialists in maternal-fetal health, neonatology and pediatric surgery in Denver are specially trained to diagnose and treat this condition, and we are uniquely equipped to promptly treat Hirschsprung’s disease after birth.

To find a specialist experienced with Hirschsprung’s disease, please call our Center for Maternal/Fetal Health at (720) 754-7642.
Rocky Mountain Hospital for Children - Center for Maternal/Infant Health

Hirschsprung’s disease is a congenital abnormality and typically diagnosed in the first few days after birth. Most cases are identified before the child is two years old. 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 this 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 only the area close to the rectum (short-segment disease), which is more common.

Hirschsprung’s disease generally affects more males than females and runs in families. It is often seen in patients with other congenital abnormalities, especially Down syndrome.

Diagnosing Hirschsprung’s disease

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 gastrointestinal exam) to confirm the diagnosis. This special X-ray procedure uses dye to provide a clear view of the intestine. If Hirschsprung’s disease is present, the intestine will appear to be narrowed where cells are missing.

Your RMHC doctor may also want to perform a rectal suction biopsy to remove and test cells from the colon mucous lining. Diagnostic 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, and it is typically performed within the first few days or the first month after birth. Rocky Mountain Pediatric Surgery is uniquely equipped with knowledgeable, experienced staff and specialized equipment to care for the unique needs of infants and children undergoing surgery.

Our pediatric surgeons are skilled in using laparoscopic surgery techniques to perform Hirschsprung’s disease surgery. This minimally invasive technique requires very small incisions, which leads to shorter recovery times and less pain. 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 in 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 is performed a few months to a year later to close the stoma and reattach the healed intestine.

Long-term outlook for Hirschsprung’s disease

After proper treatment, babies with Hirschsprung’s disease are typically 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.