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Monday, July 11, 2011

Histopathology of Hirschsprung disease.Enzyme histochemistry showing aberrant acetylcholine esterase (ACHE)-positive fibres (brown) in the lamina propria mucosae.

I had Hirschsprung disease. I don't remember it, but I had it. My parents tell me that when I was only a few years old, I had to undergo a big surgery operation. I have scars from it around my stomach, but I don't remember a single thing about it.

Apparently, only 14 out of 100,000 people receive it. I was one of those lucky winners. As Wikipedia does a general introduction of the subject: 
Hirschsprung's disease (HD), or congenital aganglionic megacolon, involves an aganglionic section of bowel[1] (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.[14] 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.[15][16]
    • 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
  • fever
Children who do not have early symptoms may also present the following:
  • constipation that becomes worse with time
  • loss of appetite
  • delayed growth
  • passing small, watery stools
Symptoms of Hirschsprung's disease may resemble other conditions or medical problems. Please consult your child's physician for a diagnosis.

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!


  1. Good thing it all ended well

  2. What a horrible thing to happen to children! Glad I've been made aware of it, thanks! +Followed, for life,

  3. Wow that's some pretty scary stuff... I'll be sure to keep this in mind in the.... far future.

  4. very intelligent post! great info thank you

  5. It was pretty interesting, a bit rough though. The more i know the more paranoid i get. This kind of posts scare me and illustrate me in the same measure.

  6. Great post. Horribile diesis.

  7. What happens when one of these got you in a trip or long travel or anywhere else? Can be mortal?

  8. Wow, after reading all that, I am glad you made it out ok, and that you aren't mentally scarred from dealing with that. Really intelligent and informative post! Followed!

  9. great post, well done, thanks 4 sharing

  10. Good lord, from the info you certainly don't sound like the lucky winner =D

    Very interesting post with lots of information.

    Followed, keep up the great articles!

  11. wow interesting, and really thanks for your awesome comment!

  12. kinda scary, but still great article!

  13. oof, how unfortunate... at least you don't have to remember the event.

  14. Ouch dude, I wish I could relate, but pooping blood doesn't really compare to this. It's only every so often...should I be worried? o.O

  15. thanks for this important infos +followed

  16. very interesting.
    This disease is pretty rare, sharing infos about it could really help somebody for a diagnosis!

  17. luckily you made it ,thank you for sharing your knowledge

  18. Interesting post, I like your blog!

  19. This is strangely interesting. And I really like the way you write. Following!

  20. That sounds crazy.... but wow you don't remember it? I suppose that makes it better.

  21. great article, found it really interesting ad learned a thing or too to make quick talk about

  22. that's crazy...thanks for sharing

  23. Wow this looks really bad, good thing it's a thing in the past now.

  24. good to know, though I hope this will never come into use

  25. Very informative post you got here! Very interested in coming back! +1