StayCurrentMD · 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 · StayCurrentMD
Cued at 9:14 · stops at 9:59 · press play
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What the experts said32 expert statements
The most common prenatal ultrasound finding in cloaca is a pelvic mass, often initially thought to be the bladder but actually representing a dilated vagina (hydrocolpos).
ClinicalMarc Levitt
Fetal intervention for cloaca is unlikely but may be necessary for massive hydronephrosis with impending renal loss; one case report from Japan described hydrocolpos drainage similar to bladder drainage for urethral valves.
ClinicalMarc Levitt
In newborn examination, cloaca presents as one hole below the clitoris with no anus; this is distinct from urogenital sinus which has one hole but a normal anus.
ClinicalMarc Levitt
Cloaca is not ambiguous genitalia and has no adrenal problem; the baby is a normal female with two normal ovaries and will be hormonally normal.
ClinicalMarc Levitt
Many patients considered cloacas are actually vestibular fistulas; with better examination pulling the labia up and out, you can see three distinct holes (urethra, vagina, and vestibular fistula).
ClinicalMarc Levitt
Initial workup includes ultrasound for hydronephrosis and kidney number, spinal ultrasound for tethered cord, plain X-ray of spine for hemivertebrae and sacral quality, and echocardiogram at most centers.
GuidelineMarc Levitt
For bilateral hydrocolpos, 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 tube placement, use an 8 or 10 French pigtail catheter from interventional radiology; a curled catheter prevents tube fallout as the hydrocolpos recedes, unlike straight catheters which fall out.
ClinicalMarc Levitt
Vesicostomy is rarely needed in cloaca because the problem is not the bladder but the hydrocolpos compressing the trigone and distal ureters; draining the hydrocolpos relieves the obstruction.
ClinicalMarc Levitt
Vesicostomy or suprapubic tube is indicated only when the common channel is very narrow or absent, preventing bladder drainage even after hydrocolpos decompression.
ClinicalMarc Levitt
Massive bilateral ureteral reflux is an exception where vesicostomy may be needed to decompress the system until later in life.
ClinicalMarc Levitt
Hydrocolpos forms because the bladder preferentially fills the vagina through a fistula rather than exiting the common channel; the vagina also contains mucus, and maternal estrogen can increase mucus production.
ClinicalMarc Levitt
The urethra in cloaca often requires a steep turn (scope on floor pointing to ceiling) to reach the bladder, explaining why urine does not drain easily and instead fills the vagina.
ClinicalMarc Levitt
Cystoscopy is not performed in the newborn period; it is delayed until 2-3 months when a larger scope can be used, visualization is better, and the baby is healthier.
ClinicalMarc Levitt
Urogenital sinus (one hole with normal anus) requires workup for adrenal problems and virilization; if virilized, likely has adrenal hyperplasia requiring electrolyte monitoring.
ClinicalMarc Levitt
Cloacal repair timing is typically 2-3 months for diagnostic endoscopy and cloacogram, with definitive repair anytime thereafter within the first year; Dr. Levitt aims for before 6 months if managing from birth, before 1 year if referred.
ClinicalMarc Levitt
The two critical endoscopic measurements are common channel length (traditional measure, with 3 cm or less being straightforward) and urethral length from urethral takeoff to bladder neck (newly emphasized measure that determines surgical approach).
ClinicalMarc Levitt
Endoscopy can falsely suggest the rectum is reachable when it is actually a long narrow fistula with healthy rectum in the abdomen; a contrast study (cloacogram) is needed to determine this.
ClinicalMarc Levitt
The cloacogram technique involves leaving catheters in the bladder, distal colostomy, and perineal orifice during endoscopy, then having interventional radiology inject these structures and create a 3D reconstruction.
ClinicalMarc Levitt
Studies comparing 2D cloacogram, 3D reconstruction, 3D printed models, and virtual reality showed that more complex modalities led to more correct anatomic descriptions; 3D is definitely better than 2D.
ClinicalMarc Levitt
Urogenital mobilization, first described by Alberto Pena in 1996, mobilizes the urethra and vagina as a unit without operating on the wall between them, eliminating the 10% urethral-vaginal fistula rate from prior techniques.
ClinicalMarc Levitt
Urogenital mobilization is appropriate when common channel is 3 cm or less AND there is at least 1.5-2 cm of urethra above the urethral takeoff; this leaves adequate urethral length after splitting the common channel.
ClinicalMarc Levitt
When urethral length is inadequate (less than 1.5 cm from takeoff to bladder neck), the common channel must be left alone to become the urethra, and the vagina must be separated from the common channel—a technically demanding operation.
ClinicalMarc Levitt
If urogenital mobilization is attempted with inadequate urethral length and the complex does not reach, the only option is abdominal delivery of the urogenital complex, which often does not work and can lead to urethral devascularization and loss.
ClinicalMarc Levitt
Leaving a patient with inadequate urethral length after urogenital mobilization results in leakage with no way to gain control 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
For type 1 cloaca (common channel of 1 cm with adequate urethral length), mobilizing the vagina alone and leaving a slightly hypospadiac urethra is acceptable if the patient will void and not require intermittent catheterization.
ClinicalMarc Levitt
Many cloaca patients have neurogenic bladder component whether or not they have tethered cord; a visible urethral orifice that is easily catheterized is needed if intermittent catheterization will be required.
ClinicalMarc Levitt
When native vagina does not reach after full mobilization, options include vaginal switch (disconnecting one side while preserving ovarian blood supply) or vaginal replacement with colon (preferred), small bowel, or rectum.
ClinicalMarc Levitt
Tissue engineering of vaginas using patient stem cells is on the horizon, with work done at Wake Forest and in Mexico; this could revolutionize cloaca care by eliminating the need for vaginal replacement.
OpinionMarc Levitt
The most common problem in redo cloaca surgery is that the surgeon never realized they were dealing with 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