The complications of Hirschsprung’s disease range from mild bowel issues to life-threatening infections. While the condition is treatable with surgery, many children continue to face long-term challenges. Ongoing care is often needed. Understanding these complications helps families and healthcare providers act early, reduce risks, and improve a child’s quality of life.
Hirschsprung-Associated Enterocolitis (HAEC)
One of the most serious complications is Hirschsprung-associated enterocolitis (HAEC). This life-threatening infection can occur before or after surgery. It causes symptoms like fever, vomiting, abdominal pain, explosive diarrhoea, and a swollen belly. In severe cases, HAEC leads to dehydration, sepsis, and even death if not treated quickly.
Doctors are not exactly sure what causes HAEC. However, it likely results from stool build-up, bacterial overgrowth, and weak gut immunity. The highest risk is during the first year of life and soon after pull-through surgery. Treatment includes IV antibiotics, fluid replacement, bowel rest, and rectal irrigations. If HAEC returns often, doctors may prescribe long-term antibiotics or consider another surgery.
Constipation After Surgery
Chronic constipation is another common problem after surgery. Even when the aganglionic bowel has been removed, some children still struggle to pass stool. This might happen due to poor motility in the remaining bowel or incomplete resection. These children may need stool softeners, enemas, or a bowel management programme. In more complex cases, tests such as manometry or biopsies can help find the cause.
Faecal Incontinence and Its Effects
Some children develop faecal incontinence after surgery. They may have trouble holding in stool due to weak sphincters, reduced sensation, or behavioural issues. This complication can impact mental well-being—especially for older children. It often leads to embarrassment, low confidence, and social withdrawal.
Multidisciplinary support makes a big difference. A care team that includes paediatric gastroenterologists, continence nurses, and psychologists can help children manage incontinence and build self-esteem.
Rare but Serious Complications
In rare cases, children develop intestinal failure. This happens when the remaining bowel cannot absorb enough nutrients or fluids. It is more likely in children with total colonic aganglionosis or those who’ve had many surgeries. These children may need long-term nutrition support, including tube feeding or total parenteral nutrition (TPN).
TPN carries risks of liver problems, blood infections, and growth delays. Children with intestinal failure often work with intestinal rehabilitation teams who manage these complex treatments.
Bowel perforation is another serious risk. It can happen when enterocolitis is not diagnosed or treated in time. A perforation allows stool and bacteria to enter the abdomen, causing peritonitis—a life-threatening emergency. This condition requires immediate surgery and intensive care.
Other Medical and Surgical Complications
Some children with Hirschsprung’s disease also have urinary tract problems. These may include urinary retention or bladder dysfunction. This is more likely when the disease affects long segments of the bowel or involves the nervous system more broadly. Children may need a urologist’s help to manage these issues and prevent infections.
Surgical complications can also arise. Common problems include:
- Strictures (narrowing at the surgical site)
- Anastomotic leaks (leaking at the bowel connection)
- Adhesions (scar tissue that blocks the bowel)
These issues may require additional surgery, especially if symptoms like pain or blockage occur.
Emotional and Developmental Challenges
Complications aren’t only physical. Many children face emotional and social difficulties, especially if they’ve had long hospital stays or repeated surgeries. Bowel problems can cause anxiety, depression, and issues with body image or friendships. These challenges often grow more serious during the teenage years.
Support from mental health professionals, social workers, or peer groups can help children cope. Emotional care is just as important as physical treatment in ensuring a healthy recovery.
Complications in Children with Genetic Syndromes
About 12% of children with Hirschsprung’s disease also have a genetic syndrome, such as Down syndrome, Waardenburg syndrome, or MEN2. These conditions bring their own challenges and may affect treatment plans. A multidisciplinary team—including endocrinologists, geneticists, and developmental specialists—is usually needed to coordinate care and ensure good outcomes.
Importance of Long-Term Follow-Up
Many of these complications can be managed well—especially when caught early. Regular follow-up is key. Children should be monitored for growth, nutrition, bowel function, and emotional well-being. A strong care plan can prevent long-term problems and help children thrive.
Advances in surgery, better awareness, and improved aftercare have all contributed to better outcomes. With the right support, most children with Hirschsprung’s disease go on to live full and healthy lives.
