Colorectal Quiz Episode 19: Hirschsprung Disease - The Obstructed Patient Part 1

The anatomy of the original pull-through can explain the patient's obstructive symptoms, and it is important to know what pull-through type was performed to determine if there is a fixable problem.

When performing initial rectal exam on a distended child with suspected enterocolitis, step to the side because there may be an explosive release of stool and gas.

Post-pull-through obstruction can be caused by stricture, twist (any pull-through type), Soave cuff (Soave procedure), Duhamel spur or non-functional distended segment (Duhamel procedure), or non-functional segment (Rehbein procedure).

If the original operative note is unavailable, a contrast study can help infer the original surgery type based on imaging findings; expertise in reading post-pull-through contrast studies is essential.

In a very ill child, a contrast study would not be the best initial option; resuscitation and stabilization should come first.

In obstructed Hirschsprung patients, the colon fills with liquid stool with severe bacterial overgrowth, causing fluid loss into the bowel lumen, hypovolemia, and bacterial translocation/bacteremia, all occurring without passage of stool.

In Hirschsprung patients with distention, irritability, and fever, enterocolitis should be assumed until proven otherwise.

Irrigation is the best way to break the cycle of enterocolitis because patients are not passing stool due to distal obstruction.

Enterocolitis can occur before surgery, after surgery, and even after successful surgery in babies who don't relax their sphincters and hold stool so efficiently they develop enterocolitis.

For a patient with prior Hirschsprung diagnosis presenting sick, assume Hirschsprung-associated enterocolitis; treatment with rectal irrigations, IV fluids, and antibiotics will not cause harm even if the diagnosis is different (e.g., Crohn's disease, E. coli enterocolitis).

When reading literature on Hirschsprung enterocolitis rates, be aware that definitions vary (admission, need for irrigations, need for antibiotics); the PCPLC consortium is working on validating the Langer score for uniform application.

Post-pull-through enterocolitis within the first 3 months occurs in about 20% of patients, based on a study from Cincinnati and Columbus.

After pull-through surgery, wait one to two weeks before performing the first irrigation, and the first irrigation should be performed by someone who is confident and knows where the anastomosis is.

Families should not undergo Hirschsprung pull-through surgery until they can demonstrate ability to perform rectal irrigations, and they should be discharged with supplies to perform irrigations at home.

In rare circumstances where a patient is too ill and sedation in the ER doesn't work, go to the OR under general anesthesia for irrigation until the patient improves; occasionally an ileostomy is needed to get the child out of trouble and work up the pull-through problem later.

In a logical workup, obtain the contrast study before going to the OR for rectal exam under anesthesia, as it may reveal findings that guide the surgical approach.

On contrast enema, it is important to look at the presacral space (space between the hollow of the sacrum and the pull-through); a widened presacral space is an abnormal finding.

A lateral view on contrast enema is important because it provides significant information about the presacral space and distal rectum anatomy.

On contrast enema, if the catheter is inserted too high or the balloon is under too much pressure, distal pathology cannot be easily ascertained.