Colorectal Channel · ERN eUROGEN ARM Webinar Series: Management of Cloacal Malformations – what is new in 2021?
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Video48 min·Published Dec 2022Older

ERN eUROGEN ARM Webinar Series: Management of Cloacal Malformations – what is new in 2021?

With Dr. Marc Levitt · hosted by Dr. Michelle Batty · Colorectal Channel
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What the experts said55 expert statements · 3 host summaries
A patient with no anal opening and a single perineal orifice has a cloaca, not ambiguous genitalia or urogenital sinus. If the patient has a normal anus, then urogenital sinus or ambiguous genitalia can be discussed.
ClinicalMarc Levitt
Cloacal patients are normal females with normal typical ovarian anatomy, though a variety of Mullerian anomalies can occur.
ClinicalMarc Levitt
The common channel in cloaca emanates just below the clitoral hood, which is not a typical location for the female urethra.
ClinicalMarc Levitt
Leaving the urethral opening in the clitoral location is suboptimal both cosmetically and functionally if the patient needs intermittent catheterization.
OpinionMarc Levitt
In utero ascites in cloaca occurs when urine flows from the bladder into the vagina, cannot exit the common channel, and backs up through the fallopian tubes into the peritoneal cavity.
ClinicalMarc Levitt
The vast majority of hydrocolpos can be drained perineally; many times abdominal surgery is not needed to drain a hydrocolpos.
ClinicalMarc Levitt
When hydrocolpos is decompressed perineally, the bladder will suddenly dilate because there is less pressure on the distal ureters, allowing them to empty into the bladder.
ClinicalMarc Levitt
A vesicostomy in almost all cloacas is not the correct treatment because it will not solve the distal ureteral obstruction.
ClinicalMarc Levitt
About 40% of cloacal patients have a bifid vaginal system.
EpidemiologicalMarc Levitt
Hydrocolpos only needs to be managed if it is causing hydronephrosis.
ClinicalMarc Levitt
The measurements vital in cystoscopy are the bladder neck location, the length of the common channel, and the length of the urethra. The urethral length must not be forgotten.
ClinicalMarc Levitt
Cloacas form in two groups: those with a low confluence and those with a high confluence.
ClinicalMarc Levitt
Historically, total urogenital mobilization was done based on common channel measurement alone, but this was done in some cases for patients with inadequately lengthed urethra.
ClinicalMarc Levitt
Most low confluence cloacas have a long urethra, and when long means greater than 1.5 centimeters, which is needed for bladder function.
ClinicalMarc Levitt
You do not want to disrupt the urogenital diaphragm or pull the bladder neck down out of the urogenital diaphragm, as this will result in urinary leakage.
ClinicalMarc Levitt
For a patient with a 3.5 cm common channel and only 1.5 cm native urethra, the preference is to do rectal mobilization, vaginal mobilization, repair the back of the common channel, and leave the urethra to become 5 cm (native urethra plus common channel), which is more likely to result in a dry patient.
ClinicalMarc Levitt
The distinction between TUM versus UG separation is based on: common channel length (3 cm or less is low confluence, likely TUM; greater than 3 cm is high confluence, likely UG separation) and urethral length (if urethra cannot be guaranteed to be 1.5 cm or greater, must do UG separation).
ClinicalMarc Levitt
A study of patients who underwent VCUG because of UTI found them to have at least 1.5 cm, if not greater, urethral length, which is where the recommendation for minimum urethral length comes from.
ClinicalMarc Levitt
If you split a long common channel and there is very little urethra on the other side, you are essentially bringing the bladder neck down to the perineum, which must be avoided.
ClinicalMarc Levitt
For a hypospadic urethra 1 cm away, you could consider leaving it hypospadic and doing only vaginal mobilization and introitoplasty, but must recognize the urethra might need catheterization one day, particularly if the patient has a spinal issue like tethered cord.
ClinicalMarc Levitt
The TUM itself may be needed just to mobilize the posterior vagina to get the introitus to reach comfortably.
ClinicalMarc Levitt
A new technique involves making an incision in the posterior common channel to recess the urethral meatus below the clitoral hood for better cosmesis and catheterization access.
ClinicalMarc Levitt
Occasionally the vagina does not reach and requires vaginal replacement, ideally using a segment of sigmoid colon.
ClinicalMarc Levitt
A nice trick for vaginal replacement is to use the sigmoid colostomy site itself, taking a segment needed for vaginal replacement and then recreating the colostomy slightly more proximal.
ClinicalMarc Levitt
Tissue engineering for vaginal replacement is a key future endeavor; in theory, a tissue engineered vagina could be created from the patient's own stem cells over 3 months in the lab.
OpinionMarc Levitt
The best time for cloacal correction is somewhere between 2 months and 1 year, with most repairs done at about 6 to 8 months of age.
ClinicalMarc Levitt
Fine needle cautery at low setting (usually 10, pure and spray for cut and coag) staying full thickness outside the TUM plane has not caused problems with stricture or fistula.
ClinicalMarc Levitt
Complications of PSARP are quite minimal with good technique, but rectal stricture can occur. When the vagina is mobilized and separated from the common channel, if under tension, vaginal stenosis can result.
ClinicalMarc Levitt
For vaginal stenosis after repair, as long as there is an opening, would not intervene at that point; would let the patient go through puberty and maybe consider an introitoplasty much later in life.
ClinicalMarc Levitt
For patients with greater than 3 cm common channel who undergo UG separation, 4 out of 5 (80%) will need intermittent catheterization. For those with 3 cm or less common channel, 1 out of 5 need intermittent catheterization.
EpidemiologicalMarc Levitt
There is definitely increased mucus production with a bowel neovagina, so if it can be avoided, it should be, but it should be done if the vagina doesn't reach successfully to the perineum.
ClinicalMarc Levitt
There is some literature on pregnancy in cloacal patients. A few patients have become pregnant, and C-section is definitely advised because of the extensive perineal dissection.
ClinicalMarc Levitt
For stenotic vagina post-op, if the orifice is there, would leave the patient alone, let them go through puberty, and maybe do an introitoplasty later in life.
ClinicalMarc Levitt
If the vagina has disappeared post-op, a very good time to correct it might be at the time of colostomy closure when you can take the colostomy site itself and bring down a neovagina independently.
ClinicalMarc Levitt
The number of redo operations has dramatically reduced because surgeons are doing anatomy analysis first, doing it well, and not attempting complex cases they don't feel comfortable with.
OpinionMarc Levitt
In the speaker's hands, TUM is a very straightforward case with very good results, virtually no vaginal stenosis, and a very good urethral repair.
OpinionMarc Levitt
From a technical point of view, if dissecting the rectum and perineal body, vaginal replacement if needed should be done then, though it is much more difficult to do as a teenager.
ClinicalMarc Levitt
Vaginal switch operation is no longer done; those patients ended up with a lot of stenosis.
ClinicalMarc Levitt
Neovaginal dilatation is not done; would rather allow for a skin-level stenosis and later do an introitoplasty rather than subject the patient to vaginal dilatation.
ClinicalMarc Levitt
The perineal sphincter muscle complex is absolutely preserved during a posterior sagittal repair.
ClinicalMarc Levitt
For perineal vaginal drainage, the common channel may need catheterization 2 or 3 times per day. The baby may start to void between catheterizations, which can be followed on ultrasound.
ClinicalMarc Levitt
Families are taught how to catheterize and brought to radiology to confirm by ultrasound that they are putting the perineal catheter in the correct location. Sometimes it needs to be directed right or left if there is bilateral hydrocolpos. A Coude catheter is useful because it can be twisted and directed.
ClinicalMarc Levitt
When doing UG separation, dissection starts on the back of the vagina as it enters the common channel, lifting it up off the common channel and dissecting the plane between vagina and posterior urethra. Do not touch the common channel at all to avoid getting into spongiosum tissue.
ClinicalMarc Levitt
Personal preference is to always start posterior sagittally if the confluence is low, below the peritoneal reflection. In the rare case where vagina and rectum are in the abdomen already, would start in the abdomen.
ClinicalMarc Levitt
With laparoscopy or robotics, you can go much lower than with laparotomy, but you want to get to the very end of vaginal insertion onto the common channel, which is hard to do. It's easier to start posterior sagittally and then go into the abdomen to continue that dissection.
ClinicalMarc Levitt
A TUM can always be done in prone position. If a TUM doesn't reach, can go into the abdomen, mobilize the confluence together, and pull through, but in such a case it would have been better to do a separation, not a TUM.
ClinicalMarc Levitt
The key to saving kidneys in cloaca is keeping the bladder empty through aggressive intermittent catheterization and bladder management, with vesicostomy in appropriate patients, particularly those with grade 4 or 5 reflux. This is learned from spina bifida management.
ClinicalMarc Levitt
Small bowel is the second choice after colon for neovagina. The blood supply of small bowel is quite tenuous and not as forgiving as that of colon.
ClinicalMarc Levitt
Personal preference is to do the entire cloacal operation together rather than staging the rectum first and doing UG mobilization later, though it is reasonable to do TUM later if it can be done perineally without touching the rectum.
OpinionMarc Levitt
Perineal body length is individualized, measured from the bottom of the labia where they meet in the middle to the anterior limit of the anal sphincter. Everything in between is the perineal body.
ClinicalMarc Levitt
Hydrocolpos can recur after procedure if vaginal stenosis occurs. In that case, would dilate up the vaginal opening to allow flow. Usually vaginal stenosis is quite skin level and does allow drainage of mucus.
ClinicalMarc Levitt
There is rarely an occasion to close the bladder neck because most urethras are salvageable if you respect the principle of keeping the common channel intact to become the neourethra. They all have a smooth, catheterizable common channel, but you need to get the vagina off of it.
ClinicalMarc Levitt
Only in very rare circumstances of congenital urethral atresia, where the patient never was able to drain urine and drained urine out the fallopian tubes with in utero ascites, do those patients need vesicostomy at birth and ultimately a Mitrofanoff.
ClinicalMarc Levitt
Colon is preferred over small bowel for vaginal replacement for its more sturdy blood supply and because using the left colon at the colostomy site saves an anastomosis by taking the colostomy down and making a more proximal colostomy.
ClinicalMarc Levitt
Patients are plugged into the concept of transition at about age 12 because that's when girls are going through puberty and need gynecology colleagues. Officially at age 21 they no longer are seen at Children's Hospital, though can flex till about age 30 if needed.
ClinicalMarc Levitt
Colleagues in Seattle taught that most hydrocolpos, including bilateral cases, can be drained with perineal catheterization.
Host summaryMarc Levitt · not cited in answers
Doctor Hendren historically did only urogenital separations, and then Doctor Pena in 1996 showed the total urogenital mobilization, which was brilliant but overused for patients who did not have an adequately length urethra.
Host summaryMarc Levitt · not cited in answers
Laparoscopic or robotic UG separation has been done by colleagues including Doctor Wood in Columbus and Belinda Dickey in Boston. Cases take a long time but are elegant and beautiful, and are a good approach for patients who would require laparotomy.
Host summaryMarc Levitt · not cited in answers