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.
More about tetralogy of Fallot
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What the experts said
In Georgia, when a baby gets diagnosed with prenatal cardiac disease, the cardiac group gets called early and gets involved, including reviewing echocardiograms and meeting with high-risk OB
Missed anorectal malformations are a topic for a separate podcast
An anal dimple with raised area and good color change indicates there is probably a good sphincter
Tiny white beads in the scrotal raphae indicate the ARM lesion is one of the less complicated lesions with an opening somewhere along the perineal body
For a perineal fistula ARM without cardiac defect, primary repair would be the optimal choice
Dilation without going to the OR might be a good choice in a baby you don't want to take to the operating room
For patients with ARM and really significant cardiac anomaly, an ostomy is probably the standard choice
In a female patient with vestibular or perineal fistula, there is no rush to operate
In a male, the perineal hole is not always easy to see and dilation is more dangerous because it is near the urethra
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 are evacuating okay
The concern with significant cardiac lesions requiring early surgery in a blue baby is worry about healing of the repair
There is no need to do a colostomy in a baby with perineal fistula and cardiac disease; you can dilate and do the repair primarily later
A conus at L2 is normal
A baby is too young in the neonatal period to calculate a sacral ratio
VCUG is obtained if there are renal anomalies
Every time they tried to dilate this baby, the baby would cry and desat down to the 60s due to TET spells
A turnable loop ostomy behaves like an end ostomy with a 95-5 percentage split, and no one knows except the surgeon that there is another side where you can do a contrast study
For cardiac babies undergoing laparoscopy, initial insufflation pressure is set around 8 mmHg if tolerated
Starting flow rate for insufflation in babies is set at 1 L/min
If the anus can be irrigated, evacuating stool helps keep insufflation pressures low during laparoscopy
If the baby has an umbilical line, consider Palmer's Point access rather than umbilical access, using a Hasson technique
When accessing the umbilicus, dissect in with a mosquito, make sure you are in without touching any vessel before insufflation, and clear the line of air
It is important to irrigate the distal bowel as much as possible during colostomy creation
A technique for distal irrigation involves having someone look laparoscopically while passing a tube into the distal segment and performing irrigation under direct visualization
This baby's PDA completely closed postnatally
Despite maneuvers, this baby continued to have hypercyanotic spells and required heart surgery sooner than the initially planned 6 months
Emergent tetralogy of Fallot repair was performed on day of life 5
PSARP was planned for about 3 months after colostomy
A baby with a low ARM lesion where the perineal fistula is closely approximated to the anal muscular complex should do really well for continence
Important factors for continence include sensation in the anal canal, absence of the dentate line, quality of the spine, quality of the anorectal malformation, and quality of the sacrum
Sacral ratio measurement should wait until 3 months of age
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 pearls along the median raphae should be scraped off at about one millimeter depth; do not dive in to find the fistula tract as it will disappear with good anoplasty and anterior rectal wall mobilization
Pearls along the raphae can persist into teenage years and young adulthood if not addressed
When 50% of the fistula is within the muscle complex and 50% is anterior, leave the anterior wall (the danger zone) and mobilize posteriorly to fill the anal sphincter, creating an 80-20 reconstruction
If the fistula is completely outside the sphincteric ellipse, a full mobilization must be performed