Colorectal Quizzes: Episode 39 – Mullerian Anomalies in Patients with ARM
Podcast22 min·Published Sep 2024Older

Colorectal Quizzes: Episode 39 – Mullerian Anomalies in Patients with ARM

With Dr. Marc Levitt & Dr. Jason Frischer & Dr. Leslie Breach & Dr. Veronica Gomez-Lobo · hosted by Dr. Lisa Bokova
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What the experts said32 expert statements · 2 host summaries
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.
ClinicalVeronica Gomez-Lobo
Before performing vaginoscopy, you should grab the labia and pull them out so the vagina opens up to ensure 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.
ClinicalVeronica Gomez-Lobo
If you see a cervix in the midline during vaginoscopy, usually there might be just one uterine structure, but you can still have a second vagina that you might be missing.
ClinicalVeronica Gomez-Lobo
If you see two cervixes during vaginoscopy, then you know the anatomy: there are two uteruses with two vaginas and two cervixes.
ClinicalVeronica Gomez-Lobo
If you see one cervix and one vagina during vaginoscopy, you don't really know what the anatomy is—duplication may still be present.
ClinicalVeronica Gomez-Lobo
Anytime a baby with an anorectal malformation is under anesthesia, she should be evaluated for Mullerian anatomy.
GuidelineVeronica Gomez-Lobo
The majority of cloaca patients will have duplication of Mullerian structures.
EpidemiologicalLeslie Breach
Just because you see one cervix, don't be satisfied—be thinking, is there another, and how can I best assess for that?
ClinicalLeslie Breach
Saline perturbation (test menstruation) can be performed either retrograde (from the cervix) or antegrade (from the distal fallopian tube) to assess patency of the system.
ClinicalLeslie Breach
A gentle perturbation carries relatively low risk.
ClinicalLeslie Breach
Hysteroscopy can be performed using small cystoscopy equipment to go into the cervix and look at the uterine cavity, which shows whether there are one or two ostia (fallopian tube openings).
ClinicalVeronica Gomez-Lobo
Cloaca patients have the highest association of Mullerian anomalies, followed by rectal vestibular fistula, then perineal fistula (which is still slightly above the general population).
EpidemiologicalLeslie Breach
The closer the vagina and rectum are together, the higher the chances of a Mullerian anomaly.
EpidemiologicalLeslie Breach
When babies are first born, they are under the influence of mother's hormones, and then they go through a mini-puberty. That is a great time to look at Mullerian structures because they get really small after that, once the hormones are gone.
ClinicalVeronica Gomez-Lobo
The reason Mullerian anomalies declare themselves at puberty is because the hormones kick in again.
ClinicalVeronica Gomez-Lobo
An ultrasound after birth is very valuable because the Mullerian structures are more visible under maternal hormonal influence.
ClinicalVeronica Gomez-Lobo
Do not diagnose an absent uterus when a child is 7 years old—many girls told they don't have a uterus at age 7 then get a period at age 13.
ClinicalVeronica Gomez-Lobo
Ultrasound is extremely good for assessing Mullerian structures in the neonatal period, and MRI is extremely bad in prepubertal children because the structures are very tiny.
ClinicalMarc Levitt
All you really want to know in prepubertal imaging is whether anything is dilated, and at that age there is nothing really dilated except in the very early stage when they are still under maternal hormonal effects.
ClinicalMarc Levitt
If a patient has symptoms and is 12 years old, then MRI is an awesome modality to see what is dilated.
ClinicalMarc Levitt
When performing hysteroscopy in a patient with a unicornuate uterus and non-communicating horn, you will see only one ostium opening into the fallopian tube, which tells you the two uterine bodies are not communicating into that one cervix.
ClinicalLeslie Breach
If there is endometrium within a non-communicating uterine horn, that is a problem because menstrual blood has no way out.
ClinicalLeslie Breach
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.
ClinicalVeronica Gomez-Lobo
Removing the fallopian tube not only prevents ectopic pregnancies but also prevents ovarian cancer, because what was previously thought to be ovarian cancer (serous cystadenocarcinoma) is actually coming from the tube.
ClinicalVeronica Gomez-Lobo
The main blood supply to the ovary is the ovarian vessels, so you want to protect those at all costs when removing a fallopian tube and uterine horn.
ClinicalLeslie Breach
To remove a fallopian tube while preserving the ovary, stay close to the fallopian tube itself in the mesosalpinx, but the ovarian vessels are the highest priority to protect.
ClinicalLeslie Breach
In patients with renal anomalies on the side of the Mullerian anomaly, ureteral stents can be placed to help protect the ureter, which can be in the admixture near the ovarian vessels going down into the pelvis.
ClinicalLeslie Breach
Mullerian remnants and anomalies are often found on the side of a pelvic kidney or absent kidney.
ClinicalLeslie Breach
Patients with a unicornuate uterus should have normal reproductive potential if the fallopian tube is not damaged by previous surgery, but there is a higher rate of premature deliveries.
ClinicalVeronica Gomez-Lobo
10% of vestibular fistulas have an absent vagina.
EpidemiologicalJason Frischer
Approximately 5% of vestibular fistulas have a vaginal septum, and approximately 1% (which might be high) have distal vaginal atresia.
EpidemiologicalMarc Levitt
Vaginal septa and distal vaginal atresia can occur in perineal fistulas, but are much more uncommon than in vestibular fistulas.
EpidemiologicalMarc Levitt
You lose the opportunity for good imaging after the neonatal period until puberty, where MRI is a little more helpful but requires sedation.
Host summaryJason Frischer · not cited in answers
Reproductive endocrinology colleagues remind us to be very gentle and careful with the fallopian tubes during laparoscopy, to avoid causing trauma that could be subsequently associated with hydrosalpinx or scarring of the tube.
Host summaryLeslie Breach · not cited in answers