Hirschsprung’s Disease is a rare but serious congenital condition affecting the large intestine (colon), characterized by the absence of nerve cells (ganglion cells) in parts of the bowel. These nerve cells are essential for coordinating the muscular contractions that move stool through the intestines. Without them, affected sections of the colon cannot properly contract and push contents forward, leading to bowel obstruction and severe constipation.
There are two main types of Hirschsprung’s Disease, distinguished by the extent of the colon affected:
Short-segment Hirschsprung’s Disease: This is the most common form. The missing nerve cells are limited to the rectum and a small part of the colon.
Long-segment Hirschsprung’s Disease: In this type, a longer portion of the colon is affected, sometimes extending into the small intestine. It is more severe and less common.
In rare cases, a total colonic aganglionosis can occur, where the entire colon lacks nerve cells.
Hirschsprung’s Disease is primarily a congenital condition, meaning it develops before birth. It arises during fetal development when nerve cells fail to migrate to certain parts of the colon. The exact cause is not fully understood, but genetic mutations are believed to play a role. It is sometimes associated with other inherited conditions like Down syndrome and congenital heart defects. It can run in families, suggesting a hereditary link.
Symptoms vary based on the extent of the affected bowel and the age at diagnosis. Some babies show signs soon after birth, while in others, symptoms appear later in infancy or childhood.
In newborns:
Failure to pass meconium (baby’s first stool) within 48 hours
Swollen abdomen
Vomiting, especially green or brown material
Reluctance to eat or poor feeding
Constipation and gas
In older infants and children:
Chronic constipation
Abdominal swelling
Malnutrition or failure to thrive
Ribbon-like or foul-smelling stools
Anemia due to poor absorption and chronic illness
Complications such as enterocolitis (inflammation of the intestines) may develop, leading to fever, diarrhea, and a very ill-appearing child. This can be life-threatening and requires urgent care.
While Hirschsprung’s Disease is a structural condition that requires surgical correction, certain precautions can help manage symptoms and prevent complications before and after surgery:
Monitor stool patterns: Parents should observe for changes in bowel habits and signs of constipation.
Hygiene: Proper hygiene is crucial to prevent infections, especially if a colostomy (temporary surgical opening of the colon) is in place.
Hydration and diet: Ensure the child remains hydrated and follows a fiber-rich diet as advised post-surgery to ease bowel movements.
Regular follow-ups: Adhere strictly to follow-up schedules for monitoring recovery and bowel function.
Surgery is the definitive treatment for Hirschsprung’s Disease. The most common procedure is a pull-through surgery, where the affected segment of the colon is removed, and the healthy part is attached to the anus. In severe cases or when the child is very ill, a temporary colostomy may be done first to allow recovery before the pull-through operation.
Post-operative care is vital. Some children may experience issues like constipation, incontinence, or infections even after surgery, requiring ongoing medical care and sometimes therapy.
Prompt medical attention is essential if a newborn:
Does not pass meconium within the first 48 hours
Shows persistent vomiting and abdominal swelling
Has constipation that does not respond to typical treatments
In older children:
Chronic constipation unrelieved by dietary changes or laxatives
Delayed growth or weight gain
Frequent abdominal pain or bloating
If enterocolitis is suspected—fever, explosive diarrhea, and distended abdomen—seek emergency care.
Hirschsprung’s Disease, though rare, can have serious consequences if left untreated. With timely diagnosis, appropriate surgical treatment, and careful post-operative care, most children go on to lead healthy lives. Awareness of its symptoms and early medical intervention are key to effective management and improved outcomes.
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