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
When a baby in Georgia gets diagnosed with prenatal cardiac disease, the Sibley cardiology group gets called early and involved, including reviewing echocardiogram and meeting with high-risk OB.
An anal dimple with raised area and good color change indicates there is probably a good sphincter.
White beads along the scrotal raphe with meconium smear indicate the anorectal malformation is one of the less complicated lesions with an opening somewhere along the perineal body.
For ARM with external opening and no cardiac defect, primary repair in the neonatal period would be the optimal choice.
Dilation and sending baby home is a suboptimal choice in a baby with no cardiac defect, but might be a good choice in a baby you don't want to take to the OR.
If you could dilate a perineal fistula patient, you don't need to go to the OR at all and can let them deal with the heart.
There is no rush on a vestibular fistula in a female patient or a perineal fistula, allowing time to address cardiac issues first.
In a male perineal fistula, the 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 get egressive stool and never go to the OR.
In a cardiac patient with an external opening from ARM, the typical approach is to dilate as long as they're evacuating okay.
For perineal fistula or vestibular fistula in a female, one can consider just dilating with good evacuation and letting the cardiac situation play itself out.
In a blue baby with significant cardiac lesion requiring early surgery where patient isn't oxygenating well, there is concern about healing of an ARM repair.
There is 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.
Colostomy is not any more or less risky than a one-hour mini-PSARP for perineal fistula.
A conus at L2 is normal.
A baby is too young to calculate a sacral ratio in the neonatal period.
VCUG is obtained if there are renal anomalies in ARM patients.
A turnable loop ostomy with 95-5 percentage behaves like an end colostomy, and no one knows except the surgeon that there's another side where you can do a contrast study.
For laparoscopy in cardiac babies, initial insufflation pressure should start around 8 mmHg if possible.
Irrigating the distal rectum and evacuating stool helps keep laparoscopic pressures low in ARM patients.
Starting flow rate at 1 liter per minute for laparoscopy in babies is a cautious approach.
If the baby has an umbilical line, consider going into Palmer's Point instead of accessing through the umbilicus, using a Hasson technique.
For umbilical access in laparoscopy, dissect in with a mosquito, ensure you're in the peritoneum without touching any vessel before insufflation, and clear the line of air.
Important factors for continence include sensation in the anal canal, absence of the dentate line, quality of the spine, type of anorectal malformation, and sacral anatomy.
Sacral ratio measurement should wait until 3 months of age.
A baby with a low ARM lesion (closely approximated perineal fistula to anal muscular complex) should do really well with continence.
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 white beads along the raphe should be scraped off during repair, as they can persist into teenage years and young adulthood if left alone.
The perineal fistula tract is only one millimeter deep; do not dive in to find it as it will disappear with good anoplasty and anterior rectal wall mobilization.
For a 50-50 perineal 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 of the sphincteric ellipse, then full mobilization is required.
For hospitals that take care of many pediatric cardiac patients, an ostomy is probably the standard choice for ARM with really significant cardiac anomaly.
Dr. Tim Jackson's technique involves looking laparoscopically while passing a tube into the distal segment to perform irrigation under direct visualization.