Colorectal Quiz Episode 31: Müllerian Anomalies in patients with ARM
With Dr. Marc Levitt & Dr. Jason Friser & Dr. Leslie Breach & Dr. Veronica Gomez-Lobo · hosted by Dr. Lisa Bokova
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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
During vaginoscopy, the vagina does not have a sphincter, so you need to squeeze the labia around the scope so that the vagina fills up with water.
Before performing vaginoscopy, you should grab the labia and pull them out so the vagina opens up to assure there isn't a septum that goes all the way out, because sometimes you can put the scope into one vagina and never realize there's a second vagina there.
If you see a single cervix in the midline during vaginoscopy, you usually feel like there might be just one uterine structure, but you can have a second vagina that you might be missing.
If you see one cervix and one vagina during vaginoscopy, you don't really know what the anatomy is.
Anytime the baby is under anesthesia, she should be evaluated for Müllerian anatomy.
The majority of cloaca patients will have Müllerian duplication.
A gentle perturbation carries relatively low risk.
There is an increase in the association of Müllerian anomalies in all patients with anorectal malformations.
The closer the vagina and rectum are together, the higher the chances that there is a Müllerian anomaly.
Cloaca has the highest association of Müllerian anomalies, followed by rectal vestibular fistula, and then the least being perineal fistula, which is still slightly above the rest of the population.
When babies are first born, they're under the influence of mom's hormones, and then they go through a mini puberty. That's a great time to look at the Müllerian structures because they get really small after that, once the hormones are gone.
An ultrasound after birth is very valuable because the Müllerian structures are more visible due to maternal hormonal influence.
Do not diagnose an absent uterus when somebody's 7 years old, because many girls told they don't have a uterus at age 7 then get a period at age 13.
Ultrasound is extremely good and MRI is extremely bad for prepubertal Müllerian assessment because the structures are very tiny.
In prepubertal patients, all you really want to know is if anything is dilated, and at that age there's nothing really dilated except in the very early stage when they're still under mom's hormonal effects.
If patients have symptoms and they're 12 years old, then MRI is an awesome modality to see what's dilated.
Non-communicating uterine horns cause severe pain, much worse than other obstructive anomalies, and because these can be very small, sometimes people have a hard time identifying them.
Removing fallopian tubes not only prevents ectopic pregnancies but also prevents ovarian cancer.
What we used to think was ovarian cancer, serous cystadenocarcinoma, is actually coming from the fallopian tube.
The main blood supply to the ovary are the ovarian vessels, which must be protected at all costs during fallopian tube removal.
In patients with renal anomalies on the side of the Müllerian anomaly, stents can be placed to help protect the ureter which can be in that admixture near the ovarian vessels going down into the pelvis.
Müllerian remnants and anomalies are often found on the side of a pelvic kidney or absent kidney.
With a unicornuate uterus, patients should have normal reproductive potential if the fallopian tube is not damaged by previous surgery, but there is a higher rate of premature deliveries.
10% of vestibular fistulas have an absent vagina.
5% of vestibular fistulas have a septum and 1% have distal vaginal atresia.
Septa and distal vaginal atresia can occur in perineal fistulas but are much more uncommon than in vestibular fistulas.
Reproductive endocrinology colleagues remind us to be very gentle and careful with the fallopian tubes during laparoscopic surgery, not to cause any trauma that could be subsequently associated with hydrosalpinx or scarring of the tube.