From
StayCurrentMD
ARMs in Female Patients: Pediatric Colorectal Controversies 2014
Chapter 1 of 8 · Diagnosis & Workup
Fistula diagnosis
Diagnosis and Classification of 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
The forchette represents the boundary between perineal and vestibular fistula classifications, though this distinction can be ambiguous and 'Mother Nature is up there chuckling at us when we try to make these distinctions.'
Don's mobilization goal for perineal/vestibular fistula repair is to mobilize just enough so the rectum reaches perineal skin with a little bit of tension, not necessarily achieving complete separation from vagina.
In every redo of a female ARM, the surgeon finds areolar tissue that had never been dissected by the original surgeon, suggesting the redo was needed because the perineal body disrupted when the anterior rectal wall was not freed enough and pulled back.
Many female vestibular fistula redos were done in the newborn period without a backup colostomy, which may contribute to complications.
Common perineal groove is a mucosal-lined channel between vagina and anus in patients with otherwise normal urethra, vagina, and anus; the vast majority become normal skin over time if observed.
If common perineal groove causes mucus production, it is a simple fix to unroof the mucosa and suture it up; it is often associated with perineal fistula.
Patients with vestibular fistula and absent vagina should undergo clinical evaluation for VACTERL association features because of the association with this specific anomaly pattern.
The host performs vestibular repairs primarily without colostomy, either in newborn period or within 3-4 months depending on child's condition, but acknowledges surgeons who want diversion cannot be criticized.
If doing primary repair with simultaneous diversion, the host recommends repair plus colostomy at same time followed by colostomy closure, rather than newborn colostomy, delayed repair, then colostomy closure.
The host waits until perineal body is healed (around day 6-7) before feeding after primary vestibular repair without colostomy, admitting this is based on bias from doing many redos where early feeding preceded dehiscence.
Watching the perineal body carefully during NPO period allows intervention (re-suturing on day 6-7) before complete dehiscence, versus feeding early, sending home, and discovering at 3-4 week clinic visit that perineal body has fallen apart.
In settings without hyperalimentation, 10% dextrose can be used for NPO periods up to 7 days in healthy, robust children, allowing surgeons in resource-limited settings to attempt NPO protocols.
For delayed vestibular repairs, the host performs full GoLYTELY bowel prep until effluent is clear, plus or minus oral antibiotics (which recent evidence suggests may be advantageous), with patient admitted day before surgery.
The host's protocol for primary vestibular repair includes PICC line, hyperalimentation, and careful perineal body examination on day 7; if healed, patient is fed and discharged; if separation is beginning (1-2 times per year), patient returns to OR for reinforcing sutures.
The host does not use a Foley catheter during or after vestibular/perineal fistula repair, believing urine leaking on the perineum is not a big deal.
About 2-5% of vestibular fistulas have a vaginal septum that must be identified; the ideal time to address the septum is during initial rectal mobilization when the perineal body is open.
Formal vaginoscopy is not necessary for every vestibular fistula; visual inspection of the introitus with spreading is sufficient, but if a septum is seen, then vaginoscopy should be performed to evaluate cervix/cervices.
Don's preferred method for ARM with absent vagina is to use sigmoid colon as neovagina, delaying repair to dilate the rectal fistula without newborn colostomy (to preserve sigmoid blood supply), then performing laparoscopic sigmoid vaginoplasty with rectal repair and backup colostomy at older age.
The alternative approach to absent vagina is to use the existing rectum as neovagina and mobilize more proximal bowel as neo-rectum, but this should only be done if the patient is unlikely to be continent (spinal anomaly, absent sacrum) because rectum has value for continence.
Of 33 patients with ARM and absent vagina in one series, 75% had urologic problems including neurogenic bladder, and 50% had CKD stage 3 or greater, including solitary kidneys, reflux, hydronephrosis, neurogenic bladder, and UTIs.
Once the diagnosis of absent vagina is made in ARM, aggressive urologic screening must be employed; this is one of three ARM categories requiring mandatory urology collaboration (along with males with any urologic association and cloacas).
In vestibular fistula with absent vagina, the rectum separates from the urethra through very thick, fibrous tissue that is not nearly as adherent as rectum to posterior vagina, creating space to bring through the neovagina.
Gynecologists typically recommend delaying neovagina construction until teenage years, but pediatric surgeons prefer doing it at the time of rectal repair because the perineal body is open (ideal opportunity) and the sigmoid pedicle reaches more easily in younger children with shorter pelvises.
There are two distinct types of cloacas: lower ones (common channel ≤3cm) and complicated ones (common channel ≥3cm); making this distinction helps avoid trouble in management.
Hydrocolpos may obstruct the distal ureters and cause bilateral hydronephrosis; about 50% of cloacas have a duplicated gynecologic system.
There is no rush to scope the vagina in a newborn with cloaca; it is better to divert the patient and perform vaginoscopy at 2-3 months of age when visualization is better and the procedure is more pleasant.
For hydrocolpos drainage, if it is large and comfortably reaches the abdominal wall, a sutured tubeless vaginostomy can be performed; if lower, use a curled tube rather than straight tube because hydrocolpos recedes from abdominal wall as inflammation resolves and straight tubes fall out at about 2 months.
Much of the fluid in hydrocolpos can be vaginal secretions, but much can also be urine refluxing up, so urine is often as big a problem as vaginal dilatation.
If using intermittent catheterization for hydrocolpos, the teaching should be done under ultrasound guidance because the catheter can go into right vagina, left vagina, bladder, or rectum, and three days may pass without draining the correct structure; blind passage through the perineum often fails to drain intended structures.
In most cloaca patients, successful drainage can be achieved by draining the hydrocolpos only, but on rare occasion the bladder must also be drained; awareness that there is often a right and left hemivagina is critical because only draining one side will only improve one side of hydronephrosis.
During surgical opening of colostomy for cloaca, the dome of the hydrocolpos can be opened and a portion of the septum removed to create a single chamber for easier drainage.
If a tube vaginostomy is placed for hydrocolpos, it may be difficult to mobilize the vagina for definitive repair months later because it becomes fixed to skin, though this can be taken down if laparotomy is needed anyway for a large hydrocolpos.
Eva advocates complete separation of rectum from vagina to the areolar plane because incomplete mobilization may cause retraction and wound problems, though this results in losing more rudimentary internal sphincter tissue.
Common perineal groove may represent arrested embryologic development, analogous to an opened-up perineal fistula extending to the scrotum in males.
The rectal blood supply is intramural, so injuring the rectal wall during dissection hurts its blood supply, making vaginal injury preferable to rectal injury during separation.
Starting laterally before attempting anterior separation is key to safely creating two structures from the common wall; the lateral plane defines the anterior plane.
Mark (the host) advocates coming in from lateral to anterior, and notes that structures are easier to separate more proximally than right at the perineum, so starting higher and working proximal to distal helps find the right plane.
Eva's systematic review on perioperative nutrition in ARM found that giving early enteral nutrition seems better than later nutrition (as in adult surgery), but all existing studies are retrospective and poor quality.
About 1000 patients have been studied regarding perioperative nutrition in ARM, but they are all retrospective and bad quality studies; good prospective cohort studies are needed before attempting large multi-center randomized trials.
Rapid learning healthcare systems allow continual accrual of experience with rapid statistical modeling to provide real-time point-of-care results for rare diseases where traditional multi-center trials introduce excessive variability.
Jonathan uses a Foley catheter to keep alkaline urine away from the fresh wound, though acknowledging uncertainty about the actual benefit.
Jerry Pierce (gynecologist) explains that women with longitudinal vaginal septums are often asymptomatic with intercourse (learning to use one side) and can labor successfully (either blowing out the septum or having it resected during repair), but menstrual hygiene is a major problem requiring tampon in each side or tampon plus pad.
Vaginal septum resection in adolescents is not difficult; it can be done with electrocautery, removing as much as possible close to the cervix without damaging it.
If a vaginal septum is discovered at age 6 in a child whose anus was already repaired, Jerry recommends waiting until puberty unless the child needs another operation anyway, as there is no rush in the premenarchal period.
The primary determinant of whether a patient has a vaginal septum versus double vagina (uterine didelphys) is the number of cervices seen on vaginoscopy; an isolated longitudinal vaginal septum can exist with one müllerian system.
Michael's approach to newborn cloaca with hydrocolpos is open divided colostomy with pigtail catheter vaginal decompression rather than formal vaginostomy, and he avoids cystoscopy at the time of colostomy creation because it makes the procedure very difficult.
Doctor Speck describes a laparoscopic approach to cloaca with hydrocolpos: cystoscopy and vaginoscopy to decompress, left upper quadrant laparoscope port for visualization, percutaneous right lower quadrant vaginostomy tube, and left lower quadrant diverting colostomy.
Doctor Rosen (Curry) advocates intermittent catheterization of the cloaca 2-3 times daily to drain urine and avoid leaving a tube in the vagina for months; after the newborn period, most fluid is urine refluxing into the vagina because vaginal distension obstructs the urethra in a self-perpetuating cycle.
Steve Krauss describes bedside ultrasound-guided drainage of hydrocolpos in a critically ill newborn with creatinine of 4 and very echogenic fluid (probably meconium and urine); after drainage, the previously invisible bladder filled beautifully, demonstrating that hydrocolpos compresses ureters and prevents bladder filling.