Colorectal Quiz Episode 18: Cloaca Part 2
With Dr. Jason Fisher & Dr. Richard Wood & Dr. Mark Levitt · hosted by Dr. Amanda Jensen & Dr. Kira Ahmad · Marc Levitt
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 usually performed at about 5-6 months of age
The workup includes multidisciplinary team evaluation (urology, gynecology, colorectal) followed by cystovaginoscopy and examination under anesthesia
Preoperative urodynamics catheter is placed in the bladder as part of the examination
During cystoscopy of the common channel, the easiest structure to enter is usually the vagina or vaginas; entering the urethra and bladder is challenging because it requires pointing very far up
During endoscopy, surgeons measure the length of the urethra, common channel, and vagina, and assess for the presence of a septum and location of the rectal fistula
3D cloacogram is acquired by injecting contrast into all three structures (urethra, vagina, rectum) and using vascular C-arm software to reconstruct three-dimensional images
During cystoscopy it is important to look for cervices to determine if there is one or two vaginas and assess Müllerian development
Surgeons should look for ureteral orifices during cystoscopy because in complex malformations there can be anomalous ureteral attachments to the bladder or bladder neck that come in very low and could be treacherous
Endoscopy performed by general pediatric surgeons has value in determining whether a cloaca is straightforward or complex, helping surgeons decide whether to refer to a specialized center
As recently as 10-15 years ago, there was much more redoing of cloacas required because surgeons attempted repairs without adequate evaluation and realized the cases were more complicated than imagined
Lower confluence cloacas are elegant operations if the surgeon knows how to perform them, while higher confluence cases with vaginal replacements and ectopic ureters should be done at specialized centers
For common channel less than 1 cm (type one cloaca), the malformation is essentially a hypospadiac urethra with a rectovaginal fistula
In type one cloaca (common channel <1 cm), the hypospadiac urethral orifice is not touched, and the plan is vaginoplasty, introitoplasty, and PSARP
Even in type one cloaca with short common channel, the true rectum can still be high, so knowing rectal height is important
A normal urethra should be at least 1.5 cm in length
For common channel 1-3 cm with urethral length >1.5 cm, the patient is amenable to total urogenital mobilization (TUM) and PSARP
For common channel >3 cm, patients often have urethral length <1.5 cm, and urogenital separation with repair of the common channel as the urethra is advocated
If the rectum is high, surgeons may want to consider an abdominal approach to mobilize first, changing the PSARP approach to LARP (laparoscopic-assisted PSARP)
Hardy Hendren was the father of cloacal management in the late 1960s and 70s with specific focus on urology and urethral reconstruction
Alberto Pena made a major advance in cloacal care in 1996 with development of total urogenital mobilization (TUM), which kept the urethra and vagina together as a unit for mobilization
Prior to 1996, all cloaca patients had urogenital separation
The next major change in cloaca protocol after TUM came 21 years later in 2017 with the algorithmic approach incorporating urethral length measurement
Hardy Hendren at age 91 attended the 2017 APSA presentation of the new algorithm and stated he agreed with everything presented
The 2017 algorithm is the first time cloacal management has been reproducible
Following the algorithm, 116 consecutive patients have been managed without needing to change the surgical plan
The major change in the 2017 algorithm was ensuring measurement of urethral length, whereas previously only common channel length (<3 cm or >3 cm) was considered
Urethral length is defined as the distance from where the common channel splits (where urethra leaves the common channel) to where it enters the bladder, not from the single orifice to the bladder neck
Measuring urethral length accurately with cystoscopy is difficult because of the curve behind the pubis, which can lead to significant under- and over-reading
The goal is to position the bladder neck above the urogenital diaphragm where the external sphincter complex lies, so that intra-abdominal pressure does not compromise continence
The most accurate urethral measurement comes from 3D imaging rather than cystoscopy because imaging does not straighten the structures and falsely measure them
The common channel takes a very significant turn as it gets behind the pubis, which is visible on lateral view imaging
Cystoscopy significantly undermeasures anatomical structures compared to 3D reconstruction because the straight scope cannot measure the turn behind the pubis
A study comparing endoscopy to 3D cloacogram showed that cystoscopy significantly under-reads the length of the common channel
If urethral length is less than 1.5 cm, urogenital separation is advocated because performing TUM would result in the bladder neck sewn near the perineum, potentially rendering the patient incontinent
The majority of 1-3 cm common channel cloacas have normal length urethra and are amenable to TUM and PSARP
If the vagina or vaginas cannot reach the perineum after urogenital separation, the patient may need vaginal replacement to bridge the gap