Colorectal Quiz Episode 32: Anorectal Malformations And Cardiac Anomalies
With Dr. Mark Levitt & Dr. Jason Frischer & Dr. Megan Durham · hosted by Dr. Rod Gerardo · Marc Levitt
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
Whenever a baby in Georgia gets diagnosed with prenatal cardiac disease, the Sibley cardiac group gets called early and involvement means going over the echocardiogram and meeting with high risk OB.
An anal dimple with raised area and good color change means there's probably a good sphincter.
White beads in the scrotal raphe with meconium smear indicate the ARM lesion is one of the less complicated lesions with an opening somewhere along the perineal body.
For a perineal fistula with no cardiac defect, primary repair would be the optimal choice, though dilation and delayed repair is also an option.
For female patients with vestibular or perineal fistulas, there's no rush to operate; dilation can allow egressive stool without going to the OR.
In males, the perineal hole isn't always easy to see and dilation is more dangerous because it's near the urethra, but with care and Hagar dilators you can achieve egressive stool.
In a cardiac patient with an external ARM opening, the typical approach is to dilate as long as they're evacuating okay, allowing the cardiac situation to play itself out.
The concern with early ARM repair in a blue baby with significant cardiac lesion requiring early surgery is the healing of that repair when the patient isn't oxygenating well.
There's no need to do a colostomy in a baby with perineal fistula and cardiac disease; you can dilate and then do the repair primarily later, and colostomy is not more or less risky than a one-hour PSARP.
A conus at L2 is normal.
Dr. Durham gets a VCUG if there are renal anomalies.
Every time they tried to dilate this baby, he would cry and desat down to the 60s due to TET spells, leading to the decision to perform colostomy.
A turnable loop ostomy behaves like an end ostomy in a 95-5 percentage configuration, and no one knows except the surgeon that there's another side where you can do a contrast study.
For laparoscopy in cardiac babies, Dr. Durham starts with a pressure of eight if possible and a flow rate of one.
When accessing the umbilicus for laparoscopy, dissect in with a mosquito, make sure you're in without touching any vessel before insufflating, and clear the line of air.
This baby has a low lesion with closely approximated perineal fistula to the anal muscular complex and should do really well for continence.
Important factors for continence are sensation in the anal canal, absence of the dentate line, quality of the spine, quality of the anorectal malformation, and quality of the sacrum.
When half of the perineal fistula opening is anterior to the muscular complex, the entire opening should be formally moved back into the center around the anal muscular complex.
The raphe beads should be scraped off at one millimeter depth; do not dive in to find the fistula tract as it will disappear with good anoplasty and anterior rectal wall mobilization.
For a 50-50 fistula (half within muscle complex, half anterior), leave the anterior wall as it's the danger zone, mobilize posteriorly to fill the anal sphincter, creating an 80-20 reconstruction; if the fistula is completely outside the sphincteric ellipse, full mobilization is required.
In a baby with significant cardiac anomaly and ARM, an ostomy is probably the standard choice at hospitals that care for many pediatric cardiac patients.
If the baby has an umbilical line, consider going into Palmer's Point rather than accessing through the umbilicus, using a Hassan technique.
Dr. Levitt's technique for distal irrigation involves having someone look laparoscopically while he passes the tube into the distal segment and does the irrigation under direct visualization.