Colorectal Quiz: Episode 47
With Dr. Chris Geyer · hosted by Dr. Filipe Jaldes · Marc Levitt
Cued at 13:29 · stops at 14:14 · 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
A 13-year-old cloaca patient would often show up with kidney damage in the past, and many unfortunately ended up with renal transplant.
Cloaca patients require cesarean section for childbirth.
Every patient with an anorectal malformation needs a gynecology colleague to ensure they are doing well.
Even with an excellent anatomical repair, anorectal malformation patients may still have soiling.
The first thing to check in a soiling ARM patient is whether the anus is okay (well-located, without stricture, without prolapse).
Anorectal manometry is not part of the standard initial workup for anorectal malformation patients.
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 ARM patients even when the anus is not strictured.
Mega-rectosigmoid in ARM can be both inherent (motility problem) and acquired from failure to aggressively treat constipation over many years.
Most ARM patients need constipation treatment.
In general, you want to keep the rectum in ARM patients because the rectum is vital for bowel control.
ARM patients don't really have anal canal sensation or an anal canal.
ARM patients don't really have any form of internal sphincter unless the very distal aspect of the rectum was saved.
Distention of the rectum (proprioception) provides the cue for ARM patients to squeeze their external sphincter and hold in stool.
If you remove the rectum in an ARM patient, you lose the capacity for proprioception.
Prior to the PSARP, an abdominal perineal pull-through was done for ARM (same concept as Hirschsprung's disease): throw the rectum away and pull the sigmoid down. That was wrong.
In older ARM patients who had abdominal perineal pull-through, you see haustral markings at the anus and in the pelvis on contrast study because they pulled through sigmoid (the rectum has no haustral markings).
Dr. Geyer has tapered mega-rectosigmoids in ARM patients both laparoscopically and open, at time of colostomy closure and after failed bowel management, with relatively good success in fewer than ten cases.
After rectal tapering, Dr. Geyer's patients' bowel management became more manageable, and anatomy studied one year after showed no re-dilation.
In many ARM kids, even with the best operative plans, they still have trouble with incontinence, frequently due to their anatomy and musculature.
A patient with sacral ratio of 0.45 has poor sacral development, so the perineum and musculature are probably not great, making them likely a bowel management candidate who will probably never achieve successful bowel control.
Dr. Levitt's approach for this unique case would be to remove the entire mega-rectosigmoid, pull through the proximal sigmoid, make a well-sized anaplasty, and do a Malone at the same time.
Some patients with sigmoid pull-through are able to feel distension in the sigmoid and achieve continence, but it is not the expectation.
For a redo PSARP with mega-rectosigmoid, Dr. Levitt would remove all the perineal rectum, dissect it out, go into the abdomen, dissect out the dilated segment, pull through the proximal sigmoid, close the posterior sagittal incision, and do a Malone. This is a 0.1% case.
In 99+ percent of ARM cases, a sigmoid resection is usually not necessary; usually a Malone only is all you need.
Colons can empty surprisingly well with antegrade enemas only, potentially avoiding resection.
Dr. Geyer's plan is to try Malone only first, telling the family it might not succeed and more definitive surgery (removing colon) may be needed.
Very often a mega-rectosigmoid patient's anaplasty is not good (strictured, mislocated, or prolapsed), requiring redo of the anaplasty.
If the anus has only a stricture, making the anus bigger might give the colon a chance to decompress and improve.
If the mega-rectosigmoid is not strictured and is enormous, it was probably an inherent problem present since birth, and resection is appropriate.
Cloaca patients require systematic evaluation of three systems: urologic, gynecologic, and colorectal.
Cystatin C is helpful to check GFR (renal function) in cloaca patients.
Attention to renal function is a relatively new advancement in care for cloaca patients to ensure they do not require renal transplant.
Kurt Sheldon taught that spina bifida bladders and cloaca bladders need to stay empty, which made a huge difference in preventing kidney damage.
The most common cause for redoing anorectal malformation patients is that the anus was placed in the wrong position.
There are many ways to tell if the anus is in the proper position, such as electrical stimulation and rectal ultrasound or MRI.
The PSARP operation for anorectal malformations was introduced in 1980, which is relatively new from a surgical point of view.
The tapering technique involves going in laparoscopically or open, finding the large rectosigmoid, and tapering on the anti-mesenteric side with a stent or dilator in the rectum to ensure good lumen size, using stapling and sometimes over-sewing.