Colorectal Quiz: Episode 47 - Megarectosigmoid

Cloaca patients require assessment of three systems: urologic, gynecologic, and colorectal.

Kurt Sheldon, a urologist and pediatric surgeon, established that spina bifida and cloaca bladders need to stay empty to prevent kidney damage.

Keeping spina bifida and cloaca bladders empty prevents kidney damage; prior to this approach, many 13-year-olds showed kidney damage and required renal transplant.

Every patient with an anorectal malformation needs a gynecologist colleague to ensure they are doing well.

Even with anatomically perfect anorectal malformation repairs, patients may still have soiling.

Anorectal manometry is not part of the standard initial workup for anorectal malformation patients at Cincinnati Children's.

Anal position can be determined by physical exam and electrical stimulation during exam under anesthesia.

Megarectosigmoid in ARM patients can be both inherent (motility problem) and acquired from failure to aggressively treat constipation over many years.

Most anorectal malformation patients need constipation treatment.

In anorectal malformation patients, the rectum is vital for bowel control because they lack anal canal sensation and internal sphincter; rectal distension (proprioception) provides the cue to squeeze the external sphincter.

If the rectum is removed in anorectal malformation patients, the capacity for proprioception is lost.

Prior to the PSARP (1980), abdominal-perineal pull-throughs were performed that discarded the rectum and pulled sigmoid down, which was wrong.

Older patients who had abdominal-perineal pull-throughs can be recognized on contrast studies by haustral markings at the anus in the pelvis, indicating sigmoid was pulled through.

Rectal tapering technique involves anti-mesenteric stapling over a dilator (like sleeve gastrectomy or proximal jejunal atresia repair), sometimes with oversewing.

After rectal tapering, bowel management becomes more manageable and anatomy studied one year post-op has not shown re-dilation.

A sacral ratio of 0.45 indicates the sacrum and perineal musculature are not great, making the patient likely a bowel management candidate who will probably not achieve successful bowel control.

For extreme megarectosigmoid with poor sacral ratio, one option is to remove the entire rectosigmoid, pull through proximal sigmoid, and perform Malone appendicostomy simultaneously.

In 99+% of megarectosigmoid cases, sigmoid resection is usually not necessary; Malone alone is often sufficient.

Colons can empty surprisingly well with antegrade enemas only, potentially avoiding resection entirely.

If anoplasty is strictured, mislocated, or prolapsed requiring redo PSARP, and megarectosigmoid is present, complete resection with sigmoid pull-through and diversion should be considered.

If only a stricture is present, enlarging the anus may allow decompression and the colon might improve significantly without resection.