Colorectal Quiz Episode 32: Anorectal Malformations and Cardiac Anomalies
With Dr. Jason Fisher & Dr. Mark Levitt & Dr. Megan Durham · hosted by Dr. Rod Gerardo
This podcast is for verified healthcare professionals.Sign in to watch — the rest of this page is open.Sign in
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
same diagnosisPodcast
Colorectal Quiz: Episode 40
Marc Levitt · 18 min · Published Nov 2024
Podcast
Colorectal Quiz Episode 29: Female ARM-Post Op Management
25 min · Published Mar 2022
Podcast
The Colorectal Quiz Episode 23: Hirschsprung Disease - The Soiling Patient...
26 min · Published Dec 2021
Podcast
The Colorectal Quiz Episode 22: Hirschsprung Disease - the Soiling Patient Part 1
19 min · Published Nov 2021
Podcast
The Colorectal Quiz Episode 21: The History of Hirschsprung Disease
15 min · Published Sep 2021
Podcast
The Colorectal Quiz Episode 20: Hirschsprung Disease Obstruction Part 2
21 min · Published Sep 2021
What the experts said
In Georgia, whenever a baby gets diagnosed with prenatal cardiac disease, the pediatric cardiology group (SIBL) gets called early and gets involved, including reviewing echocardiograms and meeting with high-risk OB.
The patient had tetralogy of Fallot with a large VSD with bidirectional shunt, moderate pulmonary valve stenosis, and right ventricular outflow tract obstruction.
Prenatally, there was mild right ventricular hypertrophy and a very small PDA.
An anal dimple (raised area with good color change) indicates there is probably a good sphincter.
White beads in the scrotal raphae indicate a fistula tract along the median raphae.
Dr. Durham routinely gets echocardiograms on all ARM patients to stay consistent with prenatal screening and check the box.
A good physical exam and listening to the heart with a stethoscope can bypass the need for an echocardiogram if necessary, particularly in resource-limited settings.
With pearls along the median raphae and meconium visible, the lesion is one of the less complicated ARM lesions, and primary repair in the neonatal period would be ideal.
There is an option to send the baby home and dilate, but primary repair is most likely the optimal choice for a perineal fistula without cardiac defect.
Dilation at home is a suboptimal choice in a baby with no cardiac defect, but it is important to know how to do it because it might be a good choice in a baby you don't want to take to the OR.
For ARM patients with really significant cardiac anomalies, colostomy is probably the standard choice.
If you can dilate a perineal fistula patient, you don't need to go to the OR at all and can let the cardiac team deal with the heart.
There is no rush on a vestibular fistula or perineal fistula in females.
In males, the perineal hole is not always easy to see, and dilation is a little more dangerous because it is near the urethra, but with care and Hagar dilators, you can get egress of stool and never go to the OR.
In a cardiac patient with an external opening from an ARM, Dr. Fisher typically dilates as long as they are evacuating OK, allowing the cardiac situation to play itself out.
In a blue baby with a significant cardiac lesion requiring early surgery, there is concern about healing of an ARM repair due to poor oxygenation.
Dr. Levitt does not think there is a need to do a colostomy in this type of baby; you can dilate and then do the repair primarily later, and the colostomy is not more or less risky.
Dr. Durham gets a VCUG if there are renal anomalies.
The preference would have been to dilate, but this baby had significant tet spells, and cardiologists initially expected cardiac repair around 6 months of age.
Every time they tried to dilate, the baby would cry and oxygen saturation would drop to the 60s.
For cardiac babies undergoing laparoscopy, Dr. Durham initially starts at a pressure of 8 if possible.
Because the anus was able to be irrigated, they evacuated some stool, which probably helped keep pressures low during laparoscopy.
Dr. Durham always starts laparoscopy in babies at a flow rate of 1, which is slower than others, and sometimes increases it.
If the baby has an umbilical line, an alternative is to go to Palmer's Point and use a Hasson technique rather than accessing through the umbilicus.
Dr. Levitt uses an infra-umbilical approach, dissecting in with a mosquito, ensuring he is in the peritoneum without touching any vessel before insufflating, and clears the line of air.
Dr. Levitt has looked in laparoscopically while passing a tube into the distal segment and performing irrigation, watching the process laparoscopically.
On follow-up echocardiograms, the PDA completely closed, but the baby continued to have hypercyanotic spells.
On day 5, the baby underwent emergent tetralogy of Fallot repair.
The baby did really well post-operatively from cardiac surgery, and the cardiac team gave clearance to proceed with definitive ARM repair relatively quickly, at about 3 months.
This baby has a low lesion with a closely approximated perineal fistula to the anorectomuscular complex, so should do really well for continence.
The sacral ratio should be measured at 3 months of age.
Dr. Levitt believes that one day it will be possible to put the weight of the spine, the weight of the ARM, and the weight of the sacrum (and perhaps other factors) into some sort of analysis to predict: if you have good surgery, your potential for bowel control is X.
In this case, half of the perineal opening was anterior to the muscular complex, so the entire opening was formally moved back and centered around the anorectomuscular complex.
The fistula along the median raphae was left alone because the perineal fistula did not extend up into the raphae itself.
Dr. Levitt has seen teenagers and young adults with those beads (along the raphae) that never went away, so you have to scrape that off; it is 1 millimeter deep.
Do not dive in and try to find the fistula tract along the raphae; it will disappear provided you have a good anoplasty with good anterior rectal wall mobilization.
If you have a 50/50 situation (half of the fistula within the muscle complex, half just anterior), leave the anterior wall because that is the danger zone; you will still have a little bit of anal opening outside the sphincter complex.
Mobilize enough posteriorly so that you fill the anal sphincter and complete the reconstruction, becoming like an 80-20 rather than 50-50.
If the fistula is completely outside of the sphincter ellipse, then you have to do a full mobilization.
The patient had cross-fused ectopia of the left kidney with a normally positioned right kidney and conus at L2 (which is normal).
There was reported sacral dysplasia with a foreshortened sacrum, though the baby was too young to calculate a sacral ratio.
The VCUG showed a small diverticulum along the right bladder base, otherwise normal.
A laparoscopic terminal loop colostomy was performed, which behaves like an end colostomy (95% to 5% loop) but allows for distal contrast studies.
Important factors for continence include sensation in the anal canal and absence of the dentate line.