The Colorectal Quiz Episode 9: Motility Disorders Part 2

The patient is showing massively dilated colon as a result of colonic dysfunction, although the dilatation is predominantly in the rectal sigmoid.

A Sitz marker study is performed by having the patient take the markers and then obtaining an X-ray at day 5.

If a lot of Sitz markers are scattered throughout the colon or predominantly on the right side, it increases interest in performing colonic manometry.

If all the markers have disappeared on the X-ray, the patient did stool even if they reported not stooling, because the markers do not dissolve.

A Sitz marker study should be available pretty much anywhere, but colonic manometry is absolutely not available everywhere.

Sitz markers can be utilized as a colonic transit study by obtaining X-rays at day 0, 1, 2, and 4 to see the transit of markers in the colon.

Nuclear scintigraphy is a nice way to assess colonic motility when colonic manometry is not available.

From a surgeon's perspective, there are three colonic motility scenarios: diffusely slow but functional, normal motility with a segmental problem, or the entire colon being severely slow and dysmotile.

Colonic manometry provides information on peristaltic activity, which is the motion of the colon.

Throughout the day, a person should have two really strong contractions throughout the colon, called high-amplitude propagating contractions (HAPCs).

If a patient has two HAPCs during an 18 or 24 hour study period, they do not have colonic dysmotility, because the presence of HAPCs rules out colonic slowness or problems.

Some patients truly have an outlet issue and their colon is actually normal on manometry.

Manometry can characterize whether contractions are well coordinated (neuropathic problem) or low amplitude (myopathic problem).

The rectum does not have the same contractions as the rest of the colon.

In Hirschsprung patients status post pull-through, who no longer have the rectal-sigmoid brake, contractions may be seen going all the way from the right colon down to the sphincters.

Even if HAPCs are present and colonic motility appears normal, if there is no response to stimulants, the patient does not have normal colonic motility by definition.

The case was concluded not to be Hirschsprung disease; it was a sampling error, and the surgeon was convinced by calretinin positivity and did not re-biopsy.

Over time, the colon could probably rehabilitate, and the patient may eventually need only laxatives alone, but a mechanically emptying colon is a perfectly good endpoint.

Enemas from below or from above (through a Malone or cecostomy) are acceptable management; if patients fail that therapy, they may then need a resection.

The vast majority of patients with segmental disease respond to antegrade therapy only and never need a resection.

In Columbus, a multidisciplinary team (Richard Wood, Mark Levitt, Carlo Di Lorenzo, and Desiaco) decided that as of January 1st, every patient regardless of motility would receive antegrade therapy first, and the vast majority responded without needing resection.