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Welcome to another episode of the Colorectal Quiz. I am Filipe Jalos, Colorectal Research

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Fellow at Chidwun's National Hospital and today we'll be discussing Hirschsprung disease

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constipation. Make sure you download the StakeHair and App to follow along with images and other

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related cases. Welcome back everyone!

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Today we have a fascinating case from Dr. Chris Geyer coming to us from Children's Hospital Los Angeles.

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As always, we are joined today by Dr. Jason Fischer and Mark Levitt. Dr. Geyer,

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let's start with your case. Sure, so this patient was 20 years old at the time that I met him,

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the Down syndrome patient, but a pretty high functioning Down syndrome patient. He had a

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pull-through done around six months of age and has been dealing with constipation and his mom had a

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myriad of different management schemes she had tried over this time with some intermittent success

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and currently she was managing with daily intermittent enemas which was becoming problematic

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with him going to school. So this patient had surgery as a baby and continued to have problems

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for 20 years. I thought Hirschsprung was considered curable with surgery.

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So the ongoing medical management for this patient and his mother was not adequate. What are some

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reasons for the decrease in the number of patients that are suffering under the condition of Down

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syndrome? You will find a lot of great information in the description below.

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So the ongoing medical management for this patient and his mother was not adequate. What are some

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reasons for the compensation in these patients.

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Thank you. So, Dr. Geier, how did you evaluate this patient?

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We took this patient to the OR for an exam under anesthesia,

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but they didn't have an X-ray.

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So we took this patient to the OR for an X-ray.

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And we got the results that the patient's

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X-ray results were not good enough.

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So we took this patient to the OR for an X-ray.

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We took this patient to the OR for an exam under anesthesia.

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We did not identify any strictures at the anoplasty.

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There was no evidence of a suave cuff.

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We were pretty sure this was a suave pull-through, although we did not have the operative notes.

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There was no evidence of a twist.

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So no evidence of any kind of mechanical problem.

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Did you do a rectal biopsy?

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We also did a rectal biopsy.

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And that rectal biopsy showed abundant ganglion cells,

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big nerves, and a normal calretinine staining pattern.

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So the reason for the biopsy was to determine if there was a retained Hirschsprung segment, correct?

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Yes.

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And of course, it's important to remember to look not only at the biopsy,

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but also how obstructed the patient is.

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For example, this patient does not have recurrent enterocrylitis, does not have failure to drive,

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is not chronically distended, and is really behaving more like functional constipation.

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Dr. Geier, let's get back to your case.

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Your next step was a contrast enema.

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Dr. Levitt, can you describe those findings?

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Thank you for that detailed description.

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What happened next?

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This patient was actually initially referred to our motility team,

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and so came with some motility testing completed as well.

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So anal rectomonometry was done, which showed normal resting pressures,

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an absent rare that was consistent with the previous diagnosis of Hirschsprungs.

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And they commented that the first sensation the patient had was when the balloon was filled to 70 billion years.

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An absent recto-anal inhibitory reflex means that the internal anal sphincter doesn't relax

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when the rectum is distended, which can contribute to constipation.

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Just to throw something out there, it's possible that this patient has a sphincter problem,

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perhaps for their whole life, given that he never emptied well,

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which could have led to the nerve hypertrophy and constipation behavior.

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Can I ask one question about your guys' opinion on anal rectomonometry and Botox?

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Do you guys routinely need monometry to show a high resting pressure before you would use Botox?

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And then for a non-cooperative patient, you'd have to empirically treat. Great discussion!

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Let's get back to our patient. This patient also had colonic manometry to evaluate how well the colon muscles are contracting and moving the stool along. What did his tests show?

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We have a pretty robust manometry motility program here, and a lot of patients have encountered this, especially in the post-Hirschsprungs patient,

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where they come with a colonic manometry done that suggests good contraction.

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Thank you.

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Those cases are rare though, where everything has anatomically checked out and it's still not empty.

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That would be a case for a colonic manometry. For this patient, you have the data now. What do you do with it?

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Just to take a step back, HIPCs are high amplitude propagating contractions, which aid in the transfer colonic contents over the long distance and often proceed emptying.

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This is useful information when treating functional constipation. When medical management is exhausted, these patients can undergo a Malone procedure, which creates a pathway directly into the colon through the abdominal walls for enemas.

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Thank you.

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Thank you.

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Dr. Geier, was this ultimately your plan for the patient?

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That's fantastic. It seems like you were able to look past the diagnosis of Hirschsprung disease, which had already been addressed surgically, and really focus on what was causing this patient's current symptoms.

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What advice would you give to other physicians managing patients with persistent constipation after surgery for Hirschsprung disease?

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Thank you.

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Thank you all for being here today and participating in this robust discussion. I think the most important thing is to remember that while the surgery can correct the underlying anatomical problem in Hirschsprung disease, many other factors can contribute to constipation.

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It's essential to take a thorough history, perform a physical exam, and consider all potential causes of constipation, including utility disorders, pelvic floor dysfunction, and behavioral issues. A multidisciplinary approach involving gastroenterologists, surgeons, and pelvic floor therapists is often necessary to provide optimal care for these patients.

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Thank you.