Hirschsprung's disease surgery
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Surgery
Treatment of Hirschsprung's disease consists of surgical removal of the abnormal section of the colon. 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 (Swenson 839). 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. Sawin, an expert in pull-through surgery, notes that, "Although there are about five different pull-through procedures, they are all more or less equally effective in treating the disorder."
The Swenson, Soave, Duhamel, and Boley procedures all vary slightly from each other with equivalent results (Sawin).
- 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 Duhamel procedure uses a surgical stapler to connect the good and bad bowel. The front of the bowel will end up with no cells, but the back will be healthy (Sawin).
Sawin notes that “Pull-through procedures used to require a colostomy, but with increased awareness among doctors and parents about the symptoms of Hirschsprung’s and with early diagnosis, doctors can keep the colon clean and perform the pull-through procedure without a colostomy”. In general, “85 percent of patients that have the pull-through surgery live normal lives afterwards. The other 15 percent have to take a laxative for the rest of their lives”.
In rare cases, if the mother has Hirschsprung’s and passes it on to her child, the child is said to have “total Hirschsprung’s”, where there are no ganglion cells at all in the colon (Sawin). In this case, the Martin pull-through operation is conducted. The Martin operation is the most invasive of the operations, for it requires a colostomy no matter how early you diagnose total Hirschsprung’s.
Even rarer, notes Dr. Sawin, "there are times where the disease begins in the small intestine, making treatment even harder". When a child has total Hirschsprung's or small intestine Hirschsprung's, an ileostomy or jejunostomy is conducted along with the Martin operation, and the child is then treated for short bowel syndrome. On very rare occasions, pull-through surgery is not the preferred method when it comes to treating Hirschsprung’s disease.
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.