Colorectal Quiz Episode 18: Cloaca Part 2
With Dr. Jason Fisher & Dr. Richard Wood & Dr. Mark Levitt · hosted by Dr. Amanda Jensen · Colorectal Channel
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
Definitive diagnostic workup for cloaca is typically performed at 5 to 6 months of age.
The workup includes multidisciplinary team evaluation (urology, gynecology, colorectal), cystovaginoscopy and examination under anesthesia, preoperative urodynamics catheter placement, and 3D cloacogram.
During cystoscopy of the common channel, the easiest structure to enter is the vagina or vaginas; entering the urethra and bladder is challenging because the scope must point far upward to take the turn.
The 3D cloacogram is acquired by injecting contrast into bladder, vagina(s), and rectal fistula, then using a vascular C-arm in radiology (or hybrid OR) with reconstruction software to create three-dimensional images.
The major advantage of 3D reconstruction is spatial understanding of anatomy, because patients do not always present with textbook anatomy.
During cystoscopy it is important to look for cervices to understand Müllerian development (one vagina vs. two, presence of uterus) and to identify ureteral orifices, which in complex malformations may attach anomalously low to the bladder or bladder neck.
The common channel takes a significant turn as it passes behind the pubis, especially in longer common channel cases.
A multi-institutional study showed that cystoscopy significantly undermeasures common channel and urethral structures compared to 3D reconstruction, because a straight scope cannot measure the turn behind the pubis.
Relying only on cystoscopy may result in significantly underreading the length of the common channel.
Endoscopy performed by a general pediatric surgeon without extensive cloaca experience has value in distinguishing straightforward from complex cloacas and identifying cases that should be referred to specialized centers.
A major change in cloacal management occurred when surgeons began evaluating complexity before attempting repair and referring difficult cases to high-volume centers, reducing the need for reoperations that were common 10-15 years ago.
Lower confluence cloacas, if the surgeon knows the technique, are a beautiful and elegant operation; higher confluence cloacas requiring vaginal replacement and management of ectopic ureters should be done at specialized centers.
The algorithm published in 2017 ('Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning,' Journal of Pediatric Surgery) helps identify patients amenable to reproducible reconstruction vs. those needing complex reconstruction.
Type 1 cloaca is defined as common channel length <1 cm; it is essentially a hypospadic urethra with a rectovaginal fistula.
In type 1 cloaca, the hypospadic urethral orifice is not touched; the plan is vaginoplasty, anorectoplasty, and PSARP.
Even in type 1 cloaca, the true rectum can still be high, so imaging is important to determine rectal position.
For common channel length 1-3 cm, a normal urethra should be at least 1.5 cm long.
If urethral length is >1.5 cm and common channel is 1-3 cm, the patient is amenable to total urogenital mobilization (TUM) and PSARP.
If urethral length is <1.5 cm, urogenital separation (UGS) is advocated, because performing TUM on a 1 cm urethra would place the bladder neck near the perineum and could render the patient incontinent.
The majority of 1-3 cm common channel cloacas have a normal length urethra and are amenable to TUM.
For common channel >3 cm, patients often have urethral length <1.5 cm; in either case, UGS is advocated with repair of the common channel (left as the urethra), mobilization of the vagina to the perineum, and PSARP.
If the vagina or vaginas cannot reach the perineum, the patient may need vaginal replacement to bridge the gap.
If the rectum is high, consider an abdominal approach (open or laparoscopic-assisted PSARP) to mobilize rectal length.
Hardy Hendren was the father of cloacal management in the late 1960s and 1970s, with specific focus on urology and urethral reconstruction.
Alberto Peña made a major advance in 1996 with the development of total urogenital mobilization (TUM), which kept the urethra and vagina together as a unit and mobilized them forward; prior to that, all patients had urogenital separation.
The next major change in the cloaca protocol occurred 21 years later, in 2017, when the algorithm incorporating urethral length measurement was presented at ABSA.
At the 2017 ABSA presentation, 91-year-old Hardy Hendren stated from the microphone that he agreed with everything presented and had no questions.
The 2017 algorithm is the first reproducible approach to cloacal management after 50 years of work on this challenging problem.
The algorithm has been validated in 116 consecutive patients without a single intraoperative plan change.
Following the algorithm using the 3 cm and 1.5 cm thresholds allows surgeons to stay out of trouble; it provides a guide for which cases are reproducible and which require referral.
The major change in the 2017 algorithm was the addition of urethral length measurement; previously the decision was based only on common channel length (less than or greater than 3 cm).
Measuring urethral length is critical because the patient needs an appropriately lengthed urethra at the end of the operation.
Urethral length is defined as the distance from where the common channel splits (urethra separates from vagina) to where the urethra enters the bladder, not from the single perineal orifice to the bladder neck.
Measuring urethral length accurately with a cystoscope is difficult because the scope must navigate the curve behind the pubis, leading to significant under- or over-reading; this is especially important in longer common channel cases.
The goal is to position the bladder neck above the urogenital diaphragm, where the external sphincter complex and urethra lie, so that intraabdominal pressure does not compromise continence.
Urethral length can be measured using a ureteric catheter under fluoroscopy or with a scope, but the most accurate measurement comes from 3D imaging because it does not straighten structures and falsely measure them.
The study 'Measure twice and cut once: comparing endoscopy and 3D cloacogram for common channel and urethral measurements in patients with cloacal malformations' was published in the Journal of Pediatric Surgery, October 2019.