Hirschsprung's disease (HD), or congenital aganglionic megacolon, involves an aganglionic section of bowel (the normal enteric nerves are absent) that starts at the anus and progresses proximally. The length of bowel that is affected varies but seldom stretches for more than about 30 cm. It arises when certain nerve cells in the gut (called ganglion cells) fail to develop and mature correctly. The result is a section of bowel that is essentially paralyzed.There is also a limited amount of ways to treat this:
Treatment of Hirschsprung's disease consists of surgical removal (resection) of the abnormal section of the colon, followed by reanastomosis. There used to be two steps typically used to achieve this goal.
- The first stage used to be a colostomy. When a colostomy is performed, the large intestine is cut and an opening is made through the abdomen. This allows bowel contents to be discharged into a bag.
- Later, when the child’s weight, age, and condition is right, a pull-through procedure is performed.
Orvar Swenson, the same man who discovered the cause of Hirschsprung’s, first performed it in 1948. The pull-through procedure repairs the colon by connecting the functioning portion of the bowel to the anus. The pull through procedure is the typical method for treating Hirschsprung’s in younger patients. Swenson devised the original procedure, but the pull-through surgery has been modified many times.The Swenson, Soave, Duhamel, and Boley procedures all vary slightly from each other:
- The Swenson procedure leaves a small portion of the diseased bowel.
- The Soave procedure leaves the outer wall of the colon unaltered. The Boley procedure is just a small modification of the Soave procedure. The term "Soave-Boley" procedure is sometimes used.
- The Duhamel procedure uses a surgical stapler to connect the good and bad bowel.
Of those 15% of children who do not obtain full control, various other treatments are available. If constipation is the problem then usually laxatives or a high fiber diet will overcome the problem. If lack of control is the problem then a stoma may be necessary. The ACE or Malone is also an answer. This is where a tube goes through the abdominal wall to the appendix, or if available, to the colon. Then once a day the bowel is flushed. Children as young as 6 do fine with administering this on their own.If the affected portion of the lower intestine is restricted to the lower portion of the rectum, other surgical procedures, such as the posterior rectal myectomy, can be performed.
As you can see, the Duhamel producer uses a surgical stapler to connect the good and bad bowel. But, in contrast, the Soave procedure leaves the outer wall of the colon unaltered. The Boley procedure is just a small modification and thus the "Soave-Boley" procedure is sometimes used. If the procedures and such do not come out to become successful, careful measures must be taken. A Stoma becomes necessary.
What are the symptoms of Hirschsprung's disease?Eighty percent of children with Hirschsprung's disease show symptoms in the first 6 weeks of life. Children who only have a short segment of intestine that lacks normal nerve cells may not show symptoms for several months or years. The following are the most common symptoms of Hirschsprung's disease. However, each individual may experience symptoms differently. Symptoms may include:
- not having a bowel movement in the first 48 hours of life
- gradual bloating of the abdomen
- gradual onset of vomiting
- constipation that becomes worse with time
- loss of appetite
- delayed growth
- passing small, watery stools
As a conclusion, it is fatal for parents and nurses to take the full measures of diagnosing a newborn child. Due to unknown reasons, the symptoms Hirschpung disease may appear. Medical assessments are absolutely necessary! Please, if you know the parents of a newborn, be sure to tell them!