CHILD SURGEON
HIRSCHSPRUNG DISEASE (CONGENITAL MEGACOLON)
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Hirschsprung disease is a rare congenital disorder (disorder present since birth) that affects the large intestine (colon) and causes problems with bowel movements.
Hirschsprung disease is caused by a problem with the nerves in the colon. During development, the nerves that control the colon's muscle contractions (called peristalsis) don't form correctly. Normally, these nerves would help to move stool through the colon and out of the body, but in Hirschsprung disease, the affected section of the colon lacks these nerves, making it difficult or impossible to pass stool.
The absence of these nerves in the affected section of the colon causes a blockage, or obstruction, of stool. This can lead to symptoms such as delayed passage of meconium (first stool passed by a newborn) constipation, abdominal swelling, and vomiting. The obstruction can also cause the colon to become enlarged (called megacolon), which can further exacerbate the problem.
Over time, the obstruction and buildup of stool can cause inflammation and damage to the affected section of the colon. In severe cases, the colon may rupture, leading to a life-threatening infection in the abdominal cavity (called peritonitis).
Surgery related
Surgery is the primary treatment for Hirschsprung disease. The goal of surgery is to remove the section of the colon that is affected by the disease, allowing for normal bowel movements.
Timing: Surgery for Hirschsprung disease is typically performed in infancy, usually within the first few months of life. However, in some cases, surgery may be delayed until later childhood or adulthood.
Type of surgery: There are several different surgical approaches to treating Hirschsprung disease, including the Duhamel procedure, the Soave procedure, and the Swenson procedure. All the procedures have comparable results so the procedure choice will depend more upon the comfort and experience of the surgeon.
Colostomy: In some cases, a temporary colostomy may be necessary before the final surgery. A colostomy is a surgical procedure that creates an opening in the abdomen, allowing stool to bypass the affected section of the colon and exit the body through a stoma (an opening in the skin).
Recovery: After surgery, the patient will likely need to stay in the hospital for several days to a week for monitoring and recovery. The patient may also need to take medications, such as antibiotics and pain relievers, during the recovery period.
It is important to note that the specific details of surgery for Hirschsprung disease may vary depending on the individual case. It is always best to consult with a qualified healthcare professional for personalized medical advice and treatment recommendations.
Follow up
While the long-term outlook for individuals with Hirschsprung disease can vary depending on the severity of the condition and the effectiveness of treatment, many individuals are able to lead healthy, normal lives with appropriate medical management.
Some of the potential long-term effects of Hirschsprung disease may include:
Constipation: Even after surgical treatment, some individuals with Hirschsprung disease may experience ongoing issues with constipation and bowel movements.
Fecal incontinence: In some cases, individuals with Hirschsprung disease may experience problems with fecal incontinence (involuntary bowel movements) due to damage or dysfunction of the rectal muscles.
Enterocolitis: Hirschsprung disease increases the risk of a serious bowel infection called enterocolitis, which can cause inflammation, fever, and other symptoms. These infection can occur in few cases even after surgical correction
Treatment of these conditions may include dietary changes, medications, surgical interventions, and other approaches to manage symptoms and improve bowel function.Therefore, it is important for individuals with Hirschsprung disease and their families to work closely with their healthcare team to develop a comprehensive care plan that meets their individual needs and supports their ongoing health and well-being