Hirschsprung’s Disease Treatment
The only treatment for Hirschsprung’s is surgery. All children will have to undergo a procedure at some stage to connect the healthy portion of bowel to their anus in order to allow them to pass bowel motions normally. Initially, the condition may be managed with regular enemas as a temporary measure until the surgery is required.
The majority of children will go on to live normal lives without any further issues, however occasionally some children may experience further issues with constipation or have difficulty with controlling their bowel motions. This may be managed with an ACE or require a permanent stoma.
CONSERVATIVE TREATMENTS
Bowel Washouts/ Anal Irrigation
This involves inserting a thin catheter tube or cone into your child’s anus and ‘washing the bowel with warm water. This treatment will soften the faeces and will pass out the anus along with the water solution. This is usually a temporary measure until surgery is performed.
SURGICAL
Pull-Through/ Duhamel Pull-Through
Your child will first need to undergo a series of tests and biopsies to identify the total length of bowel that the ganglion cells are missing from. The surgery will then be performed either laparoscopically (via key-hole) or open, the defective piece of bowel removed and the remaining healthy bowel attached to the anus to allow the child to pass bowel motions the normal way. Further information on the pull-through procedure for Hirschsprung’s can be found here.
Stoma Formation and Pull-Through
Some children will undergo a staged repair to allow the bowel to heal before it is joined up to the anus. In this case, the first operation will consist of a stoma being formed on the abdomen (either a colostomy or ileostomy), followed by a second operation a few month later to perform the pull-through operation described above. Read below for descriptions on colostomies and ileostomies.
Colostomy
A colostomy is when a part of your large bowel or colon is pulled through from an incision made on your abdomen to form a stoma. There are two types of colostomy that can be formed.
End Colostomy – where one end of the colon is pulled through and sewn to your abdomen. This can be permanent or temporary. The other part of the diseased bowel is usually either removed or allowed to heal before being joined back up together.
Loop Colostomy – this is when a looped portion of your colon is pulled through to your abdomen. An incision is made in the loop and then sewn to your stomach with a rod to keep it above surface level. A loop colostomy is usually a temporary measure performed in emergency operation and will be reversed a few weeks/months down the line.
Ileostomy
An ileostomy is when part of your small bowel (the ileum) is pulled through an incision made on your abdomen to form a stoma. There are two types of ileostomy that can be formed.
End Ileostomy – where one end of the ileum is pulled through and sewn to your abdomen. This can be permanent or temporary. The other part of the diseased bowel is usually either removed or allowed to heal before being joined back up together.
Loop Ileostomy – this is when a looped portion of your ileum is pulled through to your abdomen. An incision is made in the loop and then sewn to your stomach with a rod to keep it above surface level. A loop ileostomy is usually a temporary measure performed in emergency operation and will be reversed a few weeks/months down the line.
ACE Procedure
Occasionally some children may still experience bowel issues after pull-through surgery with either constipation or being unable to control their bowel movements. In instances of severe constipation, children can undergo a procedure called ACE (Antegrade Colonic Enema).
This procedure involves creating a continent stoma from usually the appendix but can also be from the small bowel. An small artificial opening in created low down in the abdomen or through the belly button. A catheter can be inserted into the stoma and a washout solution injected to carry out a controlled bowel movement.
Further information on the ACE operation can be found here.