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StayCurrentMD
ARMs in Female Patients: Pediatric Colorectal Controversies 2014
hosted by Dr. Marc Levitt
Chapter 1 of 7 · Diagnosis & Workup
Fistula diagnosis
Diagnosis and Classification of Female Perineal and Vestibular Fistulas
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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
Don's goal for mobilization is to pull down on the rectum until it reaches the perineal skin with a little bit of tension, then quit, rather than completely separating it from the vagina.
In every single female redo, the surgeon finds areolar tissue that had never been dissected by the original surgeon, suggesting inadequate anterior rectal wall mobilization led to perineal body disruption.
Many newborn female vestibular fistulas that required redo were done without a colostomy, which may have contributed to the need for reoperation.
The common perineal groove is often associated with an anorectal malformation and the vast majority will become normal skin over time if observed.
Coming in from lateral to anterior is key for separating the common wall, and starting more proximally makes separation easier than starting right at the perineum.
Marc Levitt performs vestibular repairs primarily without a colostomy, either in the newborn period or within the first 3-4 months depending on the child's condition.
Marc Levitt waits until the perineal body is healed (around day 6 or 7) before feeding after primary vestibular repair without colostomy, based on experience with redos in patients who were fed early.
By watching the perineal body carefully and keeping the patient NPO, the surgeon can intervene and re-suture the perineal body on day 6 or 7 if needed, preventing complete dehiscence.
In parts of the world without hyperalimentation, 10% dextrose can be used for NPO periods up to 7 days in healthy, robust children, though not in undernourished infants.
About 2 to 5% of vestibular fistulas have a vaginal septum that should be identified at the time of primary repair.
The ideal time to remove a vaginal septum is when the rectum is being mobilized and the perineal body is open, though many patients have had rectal repairs with the septum ignored.
Don's preferred method for vestibular fistula with absent vagina is to use sigmoid colon as a vaginoplasty, delaying repair while dilating the rectal fistula without doing a colostomy initially to preserve the sigmoid blood supply.
The repair can be done laparoscopically, harvesting sigmoid and bringing it to the perineum to do both the imperforate anus repair and sigmoid vaginoplasty in one stage, followed by a backup colostomy.
The alternative approach is to use the rectum as vagina and mobilize more proximal rectum down as neo-rectum, but this should only be done if the patient is unlikely to be continent due to spinal anomaly or absent sacrum, since rectum has value for continence.
Of 33 patients with vestibular fistula and absent vagina, 75% had urologic problems including neurogenic bladder, and 50% had CKD stage 3 or greater.
Patients with vestibular fistula and absent vagina require aggressive urologic screening due to high rates of solitary kidneys, reflux, hydronephrosis, neurogenic bladder, and urinary tract infections.
In vestibular fistula with absent vagina, the rectum separates very nicely from the urethra with thick fibrous tissue that is not nearly as adherent as rectum to posterior vagina, creating space for the neo-vagina.
The ideal time to create a neo-vagina in ARM patients is when fixing the rectum because the perineal body is open, and it's technically easier when children are younger because the sigmoid pedicle reaches more easily in a shorter pelvis.
There are two different types of cloacas that should be distinguished: lower ones (13 centimeters or less common channel) and complicated ones (13 centimeters or greater), and making this distinction helps avoid trouble.
About 50% of cloacas have a duplicated gynecologic system.
Marc Levitt recommends just diverting the patient initially and dealing with vaginostomy without scoping, then doing endoscopy at 2-3 months of age when visualization is better and there's no rush.
For tube vaginostomy, a curled tube should be used rather than a straight tube, because as hydrocolpos inflammation resolves and it recedes from the abdominal wall, straight tubes fall out at about 2 months.
Much of the fluid in hydrocolpos can be urine refluxing up, not just vaginal secretions, so urine is often as big a problem as the vaginal dilatation.
If using intermittent catheterization for hydrocolpos, the teaching should be done under ultrasound guidance because the tube can go into right vagina, left vagina, bladder, or rectum, and you may go three days without draining the correct structure.
In most patients with cloaca and hydrocolpos, draining the hydrocolpos alone is sufficient and bladder drainage is rarely needed.
When there is a duplicated vaginal system in cloaca, both sides must be drained or only one side's hydronephrosis will improve.
Eva prefers complete separation of rectum from vagina to the areolar plane because incomplete separation may lead to more retraction and wound problems, though this results in losing some rudimentary internal sphincter tissue.
Michael agrees with more mobilization to bring the rectum down without tension, and warns that even in seemingly straightforward perineal fistulas, staying too far from the rectal wall can lead to entering the posterior vagina.
If a common perineal groove produces significant mucus, it can be treated with a simple procedure to excise the mucosa and close it.
The rectal blood supply is intramural, so the last thing you want to do is injure the rectal wall because then you're hurting its blood supply.
A systematic review on perioperative nutrition in ARM showed that giving early enteral nutrition seems better than later nutrition, but all studies were retrospective and poor quality.
Women with longitudinal vaginal septa often learn to work around them and may be asymptomatic with intercourse, but menstrual hygiene is a major reason to remove the septum as patients report needing tampons in each side or needing both tampon and pad.
Resecting a vaginal septum in an adolescent is not a hard operation and can be done using electrocautery, getting as close to the cervix as possible without damaging it.
If a vaginal septum is discovered in a 6-year-old after ARM repair, there is no rush to repair it before menarche unless the child is having another operation.
Michael's approach to newborn cloaca with hydrocolpos is to do an open divided colostomy and decompress the vagina with a pigtail catheter rather than creating a formal vaginostomy.
Creating a colostomy in patients with massive hydrocolpos can be challenging and may require decompressing the vagina first.
Attempting cystoscopy at the time of colostomy creation in a patient with cloaca makes the colostomy creation very difficult.
A laparoscopic approach to hydrocolpos can be used, placing the laparoscope through left upper quadrant after initial decompression, then placing a right lower quadrant vaginostomy tube percutaneously and a left lower quadrant diverting colostomy.
An alternative to tube vaginostomy is having the family intermittently catheterize the cloaca 2-3 times daily, which drains the urine that refluxes into the vagina and perpetuates the distension cycle.
Bedside ultrasound can guide tube placement for hydrocolpos drainage in the ICU, and after drainage the bladder will fill beautifully, demonstrating that the hydrocolpos was compressing the ureters and preventing bladder filling.