Colorectal Channel · Cloaca Management with Dr. Marc Levitt & Dr. Aaron Garrison
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Podcast43 min·Published Dec 2016Older

Cloaca Management with Dr. Marc Levitt & Dr. Aaron Garrison

With Dr. Mark Levitt & Dr. Aaron Garrison · hosted by Dr. Todd Ponsky · Colorectal Channel
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What the experts said43 expert statements
The most common prenatal ultrasound finding in cloaca is a pelvic mass, often representing a dilated vagina (hydrocolpos).
ClinicalMarc Levitt
Fetal intervention for cloaca is unlikely to be necessary; babies should typically go to term.
ClinicalMarc Levitt
Fetal hydrocolpos drainage has been performed at least once (case report from Japan) for massive hydronephrosis with impending renal loss, similar to bladder drainage for urethral valves.
ClinicalMarc Levitt
In cloaca, there is one perineal hole below the clitoris and no anus; this is not ambiguous genitalia, there is no adrenal problem, and the baby is a normal female with two normal ovaries.
ClinicalMarc Levitt
A urogenital sinus (single hole with normal anus present) may be associated with virilization and requires evaluation for adrenal hyperplasia and electrolyte abnormalities.
ClinicalMarc Levitt
To examine a newborn for cloaca, grab the labia and lift them up and out with very good lighting to see if there is a single hole or distinct urethral, vaginal, and rectal orifices.
ClinicalMarc Levitt
Many patients considered cloacas actually have vestibular fistulas; with better examination you can see three holes (urethra, vagina, and rectal opening in vestibule).
ClinicalMarc Levitt
Initial workup for cloaca includes ultrasound for hydronephrosis and kidney number, spinal ultrasound for tethered cord, cardiac auscultation, and plain X-ray of spine to assess sacrum.
ClinicalMarc Levitt
Intermittent catheterization of the common channel may decompress hydrocolpos, but is not reliable because the catheter may enter the urethra, right or left vagina, or rectum; success should be confirmed by ultrasound.
ClinicalMarc Levitt
If hydrocolpos is bilateral, open into both vaginas at the dome, remove some of the common wall (septum), and one tube will drain both sides.
ClinicalMarc Levitt
For vaginostomy, use an 8 or 10 French pigtail catheter (not a straight catheter) because as hydrocolpos recedes, straight catheters fall out but curled catheters do not.
ClinicalMarc Levitt
Vesicostomy is rarely needed in cloaca; the problem is usually hydrocolpos compressing the trigone and distal ureters, not bladder drainage. Draining the hydrocolpos relieves the ureteral obstruction.
ClinicalMarc Levitt
Vesicostomy is indicated only when the bladder does not drain after successful hydrocolpos decompression, which occurs in very rare circumstances with very long narrow common channels or absent urethra.
ClinicalMarc Levitt
Vesicostomy is also indicated in the rare circumstance of massive bilateral ureteral reflux, where decompressing the system protects the ureters until later repair.
ClinicalMarc Levitt
Hydrocolpos develops because urine preferentially fills the vagina through the vaginal fistula rather than exiting the common channel, likely due to mechanical factors (steep urethral angle).
ClinicalMarc Levitt
Hydrocolpos fluid is typically a turbid combination of mucus and urine; maternal estrogen effect can increase mucus production and rarely cause blood in the hydrocolpos.
ClinicalMarc Levitt
Hydronephrosis in cloaca is caused by hydrocolpos pressing forward on the trigone and compressing the distal ureters where they enter the bladder.
ClinicalMarc Levitt
Cystoscopy in the newborn period is not advantageous; the required scope is tiny, visualization is poor, the perineum is swollen, and it is better to minimize OR time in newborns.
OpinionMarc Levitt
Laparoscopic approach to colostomy and hydrocolpos drainage (described by Michigan group) provides excellent visualization and is a valuable technique.
ClinicalMarc Levitt
For massive hydrocolpos extending above the umbilicus, use a lower midline incision to access the dome, and consider a tubeless vaginostomy sutured to the abdominal wall like a G-tube.
ClinicalMarc Levitt
Urogenital sinus (single perineal opening with normal anus) requires workup for adrenal problems causing virilization, though it can occur without virilization.
ClinicalMarc Levitt
Most urogenital sinuses can be managed with perineal urogenital mobilization without touching the rectum; high UG sinus cases may require a transrectal (Astra) approach.
ClinicalMarc Levitt
Definitive cloaca repair timing: perform endoscopy and cloacography at 2–3 months of age, then repair anytime thereafter within one year, ideally before 6 months if managing from birth.
ClinicalMarc Levitt
The two critical endoscopic measurements are common channel length (from perineum to urethral takeoff) and urethral length (from urethral takeoff to bladder neck); urethral length determines the surgical approach.
ClinicalMarc Levitt
Traditional classification uses 3 cm common channel length (≤3 cm straightforward, >3 cm complicated), but urethral length is equally important and not mentioned in published papers.
ClinicalMarc Levitt
Endoscopy can falsely suggest the rectum is reachable when it is actually a long narrow fistula with healthy rectum high in the abdomen; contrast study (cloacogram) is needed to assess this.
ClinicalMarc Levitt
3D cloacogram reconstruction is superior to 2D fluoroscopy; experienced surgeons answer anatomy questions more correctly with 3D imaging, and printed 3D models may be even better.
ClinicalMarc Levitt
Cloaca patients benefit from collaborative multidisciplinary approach; the days of a single surgeon handling these cases alone are over.
OpinionMarc Levitt
Urogenital mobilization, first described by Alberto Pena in 1996, mobilizes urethra and vagina as a unit without operating on the wall between them, eliminating the 10% urethral-vaginal fistula rate of prior techniques.
ClinicalMarc Levitt
Urogenital mobilization is appropriate when common channel is ≤3 cm AND urethral length above the takeoff is at least 1.5–2 cm; this leaves adequate urethral length after splitting the common channel.
ClinicalMarc Levitt
With inadequate urethral length, do not perform total urogenital mobilization; instead leave the common channel to become the urethra and separate the vagina from it—a technically demanding operation.
ClinicalMarc Levitt
After separating vagina from common channel, repair the common channel and cover with anorectal fat pad and possibly SIS to ensure well-healed urethra and avoid urethral-vaginal fistula.
ClinicalMarc Levitt
If urogenital mobilization is attempted but the complex does not reach, the only option is abdominal delivery of the urogenital complex; if this fails, separating the vagina from a circumferentially dissected common channel risks devascularizing and losing the urethra.
ClinicalMarc Levitt
Urogenital mobilization with inadequate urethral length leaves the patient with urinary leakage that cannot be controlled without tightening or closing the bladder neck.
ClinicalMarc Levitt
Separating the vagina from the common channel and leaving the common channel as the entire urethra gives the patient about a 4 cm urethra, allowing intermittent catheterization and continence.
ClinicalMarc Levitt
Type 1 cloaca (common channel ~1 cm with adequate urethral length): mobilize the vagina and leave the urethra slightly hypospadiac; patient will void if no neurogenic bladder component.
ClinicalMarc Levitt
Patients with tethered cord or neurogenic bladder need a visible urethral orifice that is easily catheterized; slightly hypospadiac urethra is acceptable only if certain the patient will void and not need intermittent catheterization.
ClinicalMarc Levitt
When native vagina does not reach after full mobilization, options include vaginal switch (disconnect one side preserving ovarian blood supply, switch dome down, remove septum) or vaginal replacement.
ClinicalMarc Levitt
For vaginal replacement, left colon is the preferred option; sigmoid may be used depending on the vascular arcade.
OpinionMarc Levitt
Tissue engineering of vaginas using patient stem cells is on the horizon (work at Wake Forest and Mexico); this would revolutionize cloaca care by eliminating the need for vaginal replacement.
ClinicalMarc Levitt
Complex cloacas requiring specialized expertise include those with common channel >3 cm or urethral length (takeoff to bladder neck) <1.5 cm.
ClinicalMarc Levitt
The most common problem in redo cloacas is the surgeon never realized it was a cloaca and only fixed the rectum, leaving the urogenital sinus untouched.
ClinicalMarc Levitt
The second most common redo problem is inadequate mobilization of structures, leaving the patient with a stenosed or lost vagina.
ClinicalMarc Levitt