Chapter 1 of 7 · Diagnosis & Workup
Menstrual obstruction
Menstrual Obstruction Risk and Intraoperative Assessment of Müllerian Structures
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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
Intraoperative assessment of Müllerian structures can be accomplished by gently inserting a 3 or 3.5 pediatric feeding tube (with additional holes removed) into the fallopian tube and instilling saline in antegrade fashion to test patency - a 'test menstruation'.
Assessment of reproductive structures should begin 6 to 9 months after the onset of puberty to evaluate estrogen effects on the Müllerian system.
When one uterus is not draining well through a unified vaginal pathway, medical management can be used to suppress continued menstruation while planning the best approach for drainage or removal.
The vaginal switch procedure involves resecting one hemi-uterus while preserving ovarian blood supply, removing the vaginal septum, tubularizing both hemivaginas into a single one, and bringing the dome of one hemivagina down to the perineum.
In vaginal switch cases, approximately 30% had distal vaginal tissue with poor blood supply requiring reoperation, leading to more aggressive approach: if tissue looks dusky, proceed to partial vaginal replacement.
The vaginal switch procedure is only useful in specific anatomic setups and requires preparation to deal with each anatomic variant in cloaca patients.
Vaginal switch is particularly relevant when hemi-uteri are very widely spaced apart, which allows the vaginas to be widely spaced as well, enabling switching of native vagina down.
In a review of 60 vaginal switch patients (58 cloaca cases) from a center with 568 total cloaca patients, the procedure was only applicable in 10% of patients, with mean common channel length of 5.2 cm.
Of 51 patients who had vaginal switch with initial surgery, 27 patients (approximately 45%) acquired some degree of vaginal stenosis.
Among vaginal switch patients, 11 required subsequent vaginal replacement, 6 required enteroidoplasty, and 4 required incidental oophorectomy despite attempts to preserve ovarian blood supply.
Native vagina is always superior to graft tissue for vaginal reconstruction.
Vaginal switch may be appropriate in unique situations where there is a well-developed vagina on one side and a rudimentary structure on the opposite side, thus not compromising fertility.
In a review of 134 neovaginal replacements, rectum was the most commonly used segment historically, but practice has shifted away from using rectum to preserve bowel control, favoring other colon segments.
Enteroidoplasty stenosis occurred in 8-18% of patients across different bowel segment types (small bowel, rectum, sigmoid, remainder of colon) used for neovaginal replacement.
The introital size created in infancy is likely not adequate for sexual intimacy in the future, requiring reassessment at puberty.
Neovaginal prolapse ranged from 8% to as high as 20% of patients depending on the bowel segment used.
Only patients with rectal neovagina complained of mucus secretion; patients with colonic neovagina did not have significant concerns with mucus secretion.
Buccal mucosa grafts are primarily used for augmentation vaginoplasty in patients who had previous procedures and need enlargement at puberty, requiring a stent (called 'rocket ship') worn for about a week postoperatively with patient at bed rest.
Buccal mucosa is just mucosa without a foundation, making it suitable for augmentation where there is structured tissue to bridge between, but not appropriate for wide open spaces with nothing to bridge.
The Cincinnati Children's team does not include vaginal dilation at the time of neovaginal reconstruction, even with bowel replacement; assessment for dilation needs is made later after puberty.
Examination for introital adequacy should be performed after onset of puberty and menstruation, but definitely before sexual debut.
When enteroidoplasty is performed, dilation is recommended at that time; native perineal tissue responds to estrogen stimulation, becoming more pliable and healing better, making post-puberty the optimal time for enteroidoplasty.
Assessment of vaginal and introital adequacy is performed 6-8 weeks after definitive repair at time of colostomy closure to note adequacy and provide indicators for future needs.
Patency of the reproductive tract should be assessed at definitive repair, followed by serial ultrasound monitoring of endometrial stimulation, then post-menstruation ultrasound, ensuring patency before menstruation occurs.
For patients with ring-like introital stricture at adolescence, repair is relatively easy with a small operation using Z-plasty or other technique.
For patients with patent but fibrotic, non-elastic vagina that is narrow in entire length, rectal patches can be used: selecting a piece of redundant rectum, opening it, and patching the vagina from behind posterosagittally, allowing sexual activity.
Rectal patch technique is particularly useful for patients who do not rely on rectal musculature for bowel control and are already performing daily enemas, as it avoids major procedure of removing vagina and bringing another one.
In long-term outcome survey of 82 adult patients (18+ years) from 192 eligible, there were 40 pregnancies in 25 patients, with 35 live births, 1 neonatal death, 22 C-sections, 7 vaginal deliveries, and 5 miscarriages.
All 9 cloaca patients in the pregnancy cohort delivered by C-section.
Women diagnosed with cloaca were more likely to have additional gynecologic conditions such as PID, infertility, endometriosis, or PCOS than women with other anorectal malformation diagnoses.
Patients with neovaginal grafts should not be considered for vaginal delivery and should undergo C-section.
For patients catheterizing via Mitrofanoff, using enemas daily, or with bladder augmentation, vaginal delivery may carry less risk than C-section, requiring individualized assessment.
Laparoscopy allows assessment and mobilization of Müllerian structures in patients where structures cannot be accessed from the perineum, and can minimize incision size during definitive procedure.
Laparoscopic drainage of hydrocolpos allows assessment of entire reproductive tract to determine if additional drainage is needed.
Laparoscopy enables dissection of vagina away from posterior bladder and rectum, potentially performed during colostomy procedures for early assessment and family counseling.
In patients with cloaca, approximately 41% develop pelvic collection of menstrual blood requiring reoperation, based on Dr. Pena's group's review of cloacal anomaly patients.
Girls will not start menstruation until approximately 1.5 to 3 years after the onset of breast development, providing a safe period for assessment.
In a literature review, there were 23 reported cases of neovaginal malignancies, with majority being squamous cell carcinomas, mostly in vaginal agenesis patients rather than complex neovaginal replacements.
Of 6 adenocarcinoma cases in neovaginal tissue, only 4 had identified bowel segments: 2 sigmoid, 1 colon, 1 ileum.
Mean time to development of any neovaginal malignancy was 19.2 years; for bowel segment malignancies specifically, mean time was 24 years.