Hirschsprung’s disease(HSD)

When i was in my preclinical years…. I thought the diseased part is the dilated part.it turns out that i am actually wrong all these years!!

That usually happens if you plan to study and NOT UNDERSTAND.boohoo.

It is the narrown part that is the diseased….why? The parasympathetic ganglions are missing so…the sympathetic takes the upper hand… Leading to spasm.

Basically….. The complaint will be delayed passage of MECONIUM….normally it would take one to two days… If it goes beyond that…. List a handful of DDs.one of them being HSD.the abdomen is so freakin distended that you can literally see the colon through the abdomen!!!

The problem with hsd is actually enterocolitis…. In babies…this is fatal! Due to the stagnation of stools…..this invites EC so easily….. And what we fear from is SEPSIS…..

The Dr said…PLEASE DONT DELAY SURGERY FOR HSD!!!

There is no rule for conservative treatment of course….

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So…what to search for??? I dont think just by plain xray would 100% conclude that its hsd. Anyway, you will find a distended colon…filled with gases…. And in one of the pics… The rectum is devoid of any gases….

By barium enema…on the lateral view, clearly shows…. The proximal dilated segment….and the distal narrower segment denoting the defective part….

Some important notes to remember…
An excerpt form emedicine:

Barium enema
Avoid washing out the distal colon with enemas before obtaining the contrast enema because this may distort a low transition zone.
The catheter is placed just inside the anus, without inflation of the balloon, to avoid distortion of a low transition zone and the risk of perforation.
Radiographs are taken immediately after hand injection of contrast and again 24 hours later.
A narrowed distal colon with proximal dilation is the classic finding of Hirschsprung disease after a barium enema. However, findings in neonates (ie, babies aged < 1 mo) are difficult to interpret and will fail to demonstrate this transition zone approximately 25% of the time.[21]
Another radiographic finding suggestive of Hirschsprung disease is the retention of contrast for longer than 24 hours after the barium enema has been performed.

If you suspect perforation or enterocolitis..so dont perform barium enema as it may pass to the peritoneum if this happens and leading to chemical peritonitis.

From what i have studied…. Sometimes… There is no delayed passage of meconium….so when this happens… You need to DD it with other diseases..

If the results is negative for hsd after rctal biopsy, do the SWEAT TEST to detect whether it is CYSTIC FIBROSIS.

this is a nice read. 🙂

Renew your intention

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