Colorectal Quiz Episode 35: Absent Vagina
With Dr. Mark Levitt & Dr. Jason Fisher & Dr. Juan Calisto · hosted by Dr. Laura Tusaba
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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
In a two-hole perineum with normal urethra and vestibular opening, differential diagnosis includes vestibular fistula with distal vaginal atresia or urogenital sinus (exceedingly rare cloacal variant).
Vestibular fistula with distal vaginal atresia is less rare than urogenital sinus with vestibular fistula.
Cystoscopy or laparoscopy are options to define vaginal anatomy in cases of absent visible vagina.
Patient had sacral ratio of 0.8 with no sacral defect or hemisacrum and normal spine by ultrasound.
Ultrasound and MRI showed absence of uterus and cervix; ovaries were not visualized on either study.
MRI in babies this small is usually pretty useless because nothing's going to be dilated.
Imaging studies were performed at 5-6 months of age, when maternal hormones would no longer be on board, making cross-sectional imaging less useful.
It is rare for patients to truly lack ovaries, but not rare for ultrasound to fail to visualize ovaries.
Laparoscopy provides comprehensive information by visualizing Mullerian anatomy and confirming absence of urogenital sinus.
Three scenarios exist for Mullerian anatomy in absent vagina: (1) no structures except ovaries and tubes (most common), (2) upper vagina with no lower vagina (vaginal atresia), (3) complete vagina with distal stenosis (least common).
Do not perform vaginal surgery until intraabdominal vaginal anatomy is defined, because a native vagina may be present and usable for pull-through.
Laparoscopy in this case revealed ovaries and fallopian tubes but no uterus and no upper vagina.
When no uterus or upper vagina is present, the vaginal anatomy will be for sexual purposes only.
Reconstruction used the rectal fistula as neovagina and distal rectum for anoplasty.
Vestibular fistula has egressive stool, so there is no urgency to proceed with reconstruction.
Advantages to addressing vagina at time of rectal repair include avoiding dissection through scarred perineal body in the future.
If introitus is good and potentially dilatable, no vaginal surgery should be done at this stage; dilation is very effective and gynecologists prefer it over bowel neovagina.
If upper vagina is present but blind-ending, current approach is to leave everything alone, allow puberty, then pull through the native vagina once it enlarges.
Colostomy is not mandatory for vestibular fistula; family can be taught to dilate the visible fistula and anoplasty can be performed at appropriate timing.
Using distal rectum as vaginal graft may compromise continence by removing bowel tissue that potentially helps with bowel control.
In patients with good prognostic factors (sacral ratio 0.8, normal spine, no neurologic abnormalities), alternative grafts such as proximal left colon or sigmoid should be considered to preserve distal rectum.
The concept that distal rectum is essential for continence in patients with excellent sphincters, normal spine, and normal sacrum is unproven; rectum 6 centimeters above may work adequately.
Using distal rectum as vaginal graft and proximal rectum for anoplasty is technically elegant and solves both problems purely posterior sagittally, particularly when vagina is for sexual purposes only.
There is no data to support which graft (distal rectum vs. proximal colon) improves functional outcome.
Using colostomy site (left colon) as vaginal graft has advantages: well-vascularized pedicled mesentery, can reach vagina easily, and allows opening more proximal colostomy for later closure.
Using colostomy site for neovagina means dissecting perineal body has already occurred at time of colostomy closure, which is a disadvantage.
Small Mullerian remnants (hemiuteri) that are clearly not useful should be left in place; there is no rush to remove them.
If one side has patent Mullerian anatomy and vaginal pull-through is planned, the contralateral Mullerian remnant should be removed along with the fallopian tube while preserving ovarian blood supply.
Some ovarian cancers develop in the fallopian tube; removing the tube reduces risk of ovarian cancer when resecting a Mullerian remnant.
In anorectal malformations in females, gynecologic anatomy must be assessed by looking into the introitus or performing vaginoscopy.
Incidence of vaginal septum in vestibular fistula is about 5%.
Incidence of distal vaginal atresia is a little under 1%.
Mullerian anomalies associated with perineal fistula are very rare.
Gynecologist should be involved in all female anorectal malformation cases.
Every opportunity in the OR should be used to confirm gynecologic anatomy, ensure no vaginal septum is left behind, and at any abdominal operation (colostomy creation, closure, laparoscopic Malone), Mullerian structures should be examined.
If bilateral Mullerian remnants are present without lumens, gynecologists advise not touching them initially and obtaining ultrasound 6 months after menarche (breast budding) to ensure nothing is dilating.