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
Cued at 0:12 · stops at 0:57 · press play
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 cloacal malformation is typically performed at 5-6 months of age
The diagnostic workup includes multidisciplinary team evaluation with urology, gynecology, and colorectal surgery, followed by cystovaginoscopy and examination under anesthesia
Preoperative urodynamics catheter placement is performed as part of the examination
During cystoscopy of the common channel, the vagina or vaginas are the easiest structures to enter, while accessing the urethra and bladder is challenging because it requires pointing far upward
The rectal fistula entrance is not always apparent during endoscopy and may require fluid injection through the mucous fistula to define it
When a septum is present, the rectal fistula is very often visible in the bottom of the septum on the rectal side
3D cloacogram is acquired by injecting contrast into all three structures (urethra, vagina, rectum) and using vascular C-arm imaging with specialized software
During cystoscopy it is important to identify cervices to understand Müllerian development and to look for ureteral orifices which may have anomalous attachments to the bladder or bladder neck
The common channel takes a very significant turn as it passes behind the pubis, particularly in longer common channel cases
Cystoscopy significantly undermeasures anatomical structures compared to 3D reconstruction because the straight scope cannot accurately measure the turn behind the pubis
Routine endoscopy allows general pediatric surgeons to differentiate straightforward from complex cloacas and identify cases that should be referred to specialized centers
As recently as 10-15 years ago, many cloacal repairs required revision because surgeons attempted repairs that were more complicated than anticipated
Lower confluence cloacas can be elegantly repaired by appropriately trained surgeons, while higher confluence cases with vaginal replacements and ectopic ureters should be done at specialized centers
Type 1 cloaca is defined as common channel length less than 1 centimeter and represents a hypospadiac urethra with rectovaginal fistula
For type 1 cloaca, the hypospadiac urethral orifice is not touched, and the repair consists of vaginoplasty, introitoplasty, and PSARP
Even in type 1 cloaca with short common channel, the true rectum can still be positioned high, making imaging important to determine rectal height
For common channel length between 1-3 cm with urethral length greater than 1.5 cm, the patient is amenable to total urogenital mobilization (TUM) and PSARP
If urethral length is less than 1.5 cm, urogenital separation is advocated because performing TUM would result in the bladder neck being sewn near the perineum, potentially rendering the patient incontinent
For common channel greater than 3 cm, patients often have urethral length less than 1.5 cm and require urogenital separation with repair of the common channel as the urethra
When vagina or vaginas cannot reach the perineum, vaginal replacement may be needed to bridge the gap
If the rectum is positioned high, an abdominal approach may be needed to mobilize length, 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 developed total urogenital mobilization (TUM) in 1996 as a major advance, keeping urethra and vagina together as a unit to mobilize forward
Prior to 1996, all cloacal patients underwent urogenital separation
The algorithm using common channel length of 3 cm and urethral length of 1.5 cm as decision points was presented in 2017 and represents the next major change after TUM
Hardy Hendren at age 91 endorsed the 2017 algorithm at APSA, stating he agreed with everything presented
The algorithm has been validated in 116 consecutive patients without needing to change the surgical plan in any case
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 perineal orifice to bladder neck
Accurate urethral length measurement is fundamentally important to ensure the bladder neck lands above the urogenital diaphragm where the external sphincter complex lies
3D imaging provides the most accurate urethral measurement because cystoscopy straightens structures and can falsely measure them due to the anatomical curve