Colorectal Quiz: Episode 47
With Dr. Chris Geyer & Dr. Jason Frischer · hosted by Dr. Filipe Jaldes · Marc Levitt
Cued at 16:16 · stops at 17:01 · press play
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Every patient with cloacal history requires evaluation of three systems: urologic, gynecologic, and colorectal.
Checking cystatin C is helpful to assess GFR and renal function in cloacal patients.
Before aggressive bladder emptying protocols, 13-year-old cloacal patients often showed up with kidney damage and many required renal transplant.
Cloacal patients require cesarean section for childbirth.
Every patient with an anorectal malformation needs a gynecologist colleague to ensure they are doing well.
The most common cause for redoing anorectal malformation patients is that the anus was placed in the wrong position.
Anorectal manometry is not part of the standard initial workup for anorectal malformation patients; it is saved for circumstances that are difficult to figure out.
There are many ways to tell if the anus is in the proper position, such as electrical stimulation and rectal ultrasound or MRI.
You should be able to tell if the anus is properly positioned by looking and by electrical stimulation during exam under anesthesia.
The rectosigmoid can be inert in anorectal malformation patients even when the anus is not strictured.
Mega-rectosigmoid in anorectal malformation can be both inherent (problematic motility) and acquired from failure to aggressively treat constipation over many years.
Most anorectal malformation patients need constipation treatment.
In general, you want to keep the rectum in anorectal malformation patients because the rectum is vital for bowel control.
Anorectal malformation patients don't really have anal canal sensation, don't really have an internal sphincter unless the very distal rectum was saved, and external sphincter quality depends on how well-formed the pelvis is (determined by sacral measurements).
Distention of the rectum (proprioception) provides the cue to squeeze the external sphincter and hold in stool in anorectal malformation patients.
If you remove the rectum in an anorectal malformation patient, you lose the capacity for proprioception and the patient will never have bowel control.
In older patients who had abdominal perineal pull-through, you can recognize it on contrast study by seeing haustral markings at the anus and in the pelvis, because they pulled through sigmoid (the rectum has no haustral markings).
Dr. Geyer has tapered mega-rectosigmoids in anorectal malformation patients both laparoscopically and open, at time of colostomy closure and after failed bowel management, with relatively good success in fewer than ten cases.
The rectal tapering technique involves going in laparoscopically or open, tapering on the anti-mesenteric side with a stent or dilator in the rectum to ensure good lumen size, stapling and sometimes over-sewing.
After rectal tapering, patients' bowel management becomes more manageable and anatomy studied one year after has not shown re-dilation.
A sacral ratio of 0.45 indicates the sacrum is not great and the perineal musculature is probably not great.
A patient with sacral ratio 0.45 is likely to be a bowel management candidate and will probably never achieve successful bowel control given the quality of their pelvis and amount of caudal regression.
For this specific case (0.1% scenario), Dr. Levitt would do a redo PSARP, remove all perineal rectum, dissect out the mega-rectosigmoid, pull through proximal sigmoid, and perform a Malone procedure.
For 99+ percent of mega-rectosigmoid cases, a Malone procedure alone is all you need, and you can be surprised how well colons empty with antegrade enema only.
Very often patients with mega-rectosigmoid have an analplasty that is not good (strictured, mislocated, or prolapsed) and need a redo analplasty anyway.
If the anus is just strictured, making it bigger might give the colon a chance to decompress and get better.
Kurt Sheldon established that spina bifida bladders and cloacal bladders need to stay empty to prevent kidney damage.
Prior to the PSARP (posterior sagittal anorectoplasty) in 1980, the surgical approach for anorectal malformations was an abdominal perineal pull-through that discarded the rectum and pulled the sigmoid down, which was wrong.
Dr. Levitt has had some patients with good success with continence where the sigmoid is able to feel some distension after resection and sigmoid pull-through, but it is not the expectation.