Setting the Standard for Cloacal Malformation Management

The team in the Center for Colorectal and Pelvic Reconstruction at Nationwide Children’s Hospital has transformed the profession’s approach to surgical care and follow-up, dramatically altering patient outcomes in the process.

To say that cloacal malformation management has been historically understudied may be an understatement.

“At the time we published our surgical protocol, it was the first time in 22 years anyone had suggested a new technique,” says Molly Fuchs, MD, pediatric urologist and surgeon for the Center for Colorectal and Pelvic Reconstruction (CCPR) at Nationwide Children’s Hospital.

In the past 10 years, the team has introduced a handful of groundbreaking advancements that have transformed cloacal malformation surgical practices internationally and provided a roadmap for ongoing improvement in patient outcomes.

“Cloacal malformation is very rare and is also one of the most complex malformations we come across, so patients have historically had poor outcomes,” says Richard Wood, MBChB, FCPS(SA), chief of the CCPR at Nationwide Children’s. “We recognized a critical gap in knowledge surrounding the management of these patients and knew we had an opportunity to meaningfully improve their care.”

With the support of hospital administration since the opening of the CCPR in 2014, Dr. Wood and colleagues planned and implemented multidisciplinary management and regular data analysis to improve cloacal outcomes. Their first step? Gathering evidence surrounding the best approach to surgical technique for different patients.

A Personalized Approach to Procedures

In cloacal malformations, the urethra, vagina and rectum all share a single tube called the “common channel.” The goal of operating is to separate the three channels physically while preserving their unique functions.

For decades, standard practice for surgical management involved measuring the common channel to inform whether attempting the simplest approach, called total urogenital mobilization, could work, or whether a more complex repair, called urogenital separation, would be required. Many surgeons would attempt the easier technique first and, if they found intraoperatively that it would not work, they would switch mid-operation to the other approach. Switching often leads to damage or ultimately loss of the patient’s urethra in the process, resulting in a lifetime of catheterization.

The Nationwide Children’s team felt that measurement of only the common channel oversimplified patient anatomy and proposed a new algorithm for selecting surgical techniques — one that added in consideration of the length of the patient’s urethra. They hypothesized that patients with short urethras would be better candidates for urogenital separation, while those with longer urethras and short common channels would do well with total urogenital mobilization.

The team performed preoperative imaging studies to get common channel and urethra lengths in order to select procedures in advance and published their outcomes, showing that their method resulted in 97% of patients having a viable urethra postoperatively.

“The important thing is that if you choose the right operation in advance and can avoid a switch during the middle of the operation, patients should do fine,” says Daniel DaJusta, MD, pediatric urologist and medical director of the CCPR. “That was our first major breakthrough.”

Improved urethral function enabled the team to begin looking beyond the basics. For example, after seeing a few cases of urethra-vaginal fistula in patients undergoing urogenital separation, the team implemented the use of interposing tissue as a barrier between urethra repair and vaginal tissue, reducing fistula rates to almost zero. By natural extension, the team’s surgical protocol also reduces the need for re-do surgeries and preserves anatomy to allow multiple solutions for future catheterization if full urinary control cannot be achieved.

“Once we figured out what worked best in each operation, it became a matter of following up with patients over time and figuring out their longer-term outcomes,” says Dr. DaJusta.

Girls born with cloacal malformations have high rates of bladder dysfunction, kidney damage, incontinence and sexual or reproductive health challenges. The CCPR team wanted to change that.

“We started at the beginning, asking questions about what we don’t know about this group of patients, and thinking of the whole picture of how we should be taking care of them,” Dr. Fuchs says of the team’s comprehensive approach to creating new evidence- based practices for cloacal care.

Bladder Outcomes and Renal Function

According to the literature, about 9 in every 10 girls born with cloacal malformation experience some form of bladder dysfunction, but the exact cause of dysfunction is unclear. Many patients have spinal cord or vertebral abnormalities that can independently cause bladder dysfunction, but surgery to separate the common channels — specifically the total urogenital mobilization approach — has also been suspected of introducing damage.

To determine the effect of surgical technique on bladder function, the CCPR team reviewed 48 cloacal malformation cases with pre- and postoperative urodynamics testing. The children had procedures selected according to the team’s algorithm based on the length of both the urethra and the common channel.

In contrast to studies done elsewhere, nearly 80% of the CCPR patients had stable or improved bladder function following surgery. Rather than surgical technique or spine status, the team found that having a common channel measuring 3 cm or longer was the only significant predictor of worsening bladder status, although a higher percentage of patients undergoing urogenital separation (30%) were among those with worsening function compared to those undergoing total urogenital mobilization (9.5%). They published their findings in the Journal of Pediatric Urology.

The team has also reported continence outcomes for 152 patients aged 3 years or older, of whom 93 (61.2%) were dry (<1 daytime accident per week): 82% of those using clean intermittent catheterization and 65% of those voiding via urethra. Although the team plans to do further follow-up to better understand the connection between patient anatomy and urinary outcomes, common channel length was not significantly associated with dryness, while spinal cord abnormality was associated with dryness, while spinal cord abnormality was associated with clean intermittent catheterization and normal spine anatomy was associated with the ability to void via urethra and be dry. The team published the results in the Journal of Pediatric Surgery. 

The team’s published cohorts represent some of the largest patient samples in the literature.

“We perform a large number of cloacal surgeries per year,” says Dr. Wood of the CCPR team, which performs dozens of procedures annually. “This obviously translates to considerable clinical experience and the ability to answer a lot of research questions that we couldn’t do without prospective data collection from so many patients.”

Renal protection became the next target for the team, because as many as 50% of all patients born with cloacal malformations end up with kidney problems — many of whom eventually need transplantation or die of renal failure.

“Baseline renal function at the time of surgery is normal for most of these children, suggesting future renal insufficiency must be explained with things happening after surgery over the long term, such as recurring infections or poor bladder emptying,” says Dr. DaJusta. “That means renal insufficiency in these patients could be preventable.”

The team developed a strict renal protection protocol involving regular laboratory evaluation, personalized surgical procedure selection, proactive imaging for signs of impending kidney damage or urinary retention, and early introduction of catheterization when required.

Over a median follow-up period of 4.2 years, only 2.9% (n=3) of 105 girls progressed from normal kidney function to renal dysfunction. Although 3 additional patients who started with kidney dysfunction progressed as well, none of the cohort with normal kidneys at study initiation required dialysis or transplantation. The outcomes, also published in the Journal of Pediatric Urology, represent a major improvement over the historical renal dysfunction incidence of 50%.

Comprehensive Care and Follow-up

In many institutions, cloacal malformation care sits under the remit of a single specialty. General surgeons perform the primary operation and bid farewell to patients until the need for follow-up or revision surgeries arises. Not so at Nationwide Children’s.

“Right from the beginning we saw this as a complex problem with a multispecialty solution,” says Dr. Wood, who leads a team of colorectal surgeons, urologists, genitourinary and reproductive health specialists, and specially trained nurses, crafting a robust, collaborative program. “Shared clinics often aren’t the most profitable, so we are very fortunate that leadership supported us and knew this was the best way to make a difference for these patients.”

Now, preoperative, surgical and follow-up care — including long-term monitoring and counseling — involve experts from multiple teams, as well as dedicated nurse practitioners. Urologists manage bladder, kidney and urethral health while gynecologists consider menstruation, sexual health and childbearing potential at appropriate ages. Colorectal experts work on maximizing fecal continence and addressing behavior-related and functional gastrointestinal issues.

“Getting urology involved early on has been really critical,” explains Dr. Fuchs. “Generalists might think that if a baby has wet diapers, they’re peeing fine, but we know as urologists that may not be true — they could have a full bladder that is overflowing and causing the kidneys to suffer. Kidney disease is silent at first, so you won’t know about it if you’re not following patients and looking for it.”

As the team’s original cohort of patients advances toward adulthood, the team is also preparing a smooth transition to a prepared adult team at The Ohio State University Wexner Medical Center, including Transition Director Alessandra Gasior, DO, who sees patients at both institutions and straddles transitional care for adolescents and young adults.

“All of this is part of a cohesive plan to meeting the needs of this patient population,” says Dr. Wood. “We have one of the largest cohorts and some of the best long-term follow-up data out there, and it’s only going to get better.”

There are many questions about long-term health for these patients that the team hopes to shed light on, especially in their population of patients with procedures selected based on personal anatomy and follow-up with renal protection protocols and ongoing bladder and bowel care.

The team continues to monitor patients for durability of kidney outcomes and continence beyond 5 years, as well as long-term integrity of the surgically created channels. They also share results via publications and consortia dedicated to improving the management of cloacal malformations — the primary drivers of their techniques’ adoption globally.

“These kids require a lifetime of care, and we’re trying our best to support them on their journey through our research and collaboration,” says Dr. Fuchs of the team’s ongoing efforts to revolutionize the outcomes of patients with cloacal malformations. “It’s rare to see research impact the care of kids so much in such a short period of time, and it’s something we are very proud of.”

This story also appeared in the Spring/Summer 2025 print issue. Download the PDF of the full issue.

References:

1. Srinivas S, Morin JP, Bergus KC, Armon N, Griffin KL, Jayanthi V, Wood RJ, Dajusta DG, Fuchs ME. Following a strict renal protection protocol in cloacal malformations: A descriptive analysis. Journal of Pediatric Urology. 2024;20(4):725.e1–725.e7.
2. Morin JP, Srinivas S, Wood RJ, Dajusta DG, Fuchs ME. Bladder management and continence in girls with cloacal malformation after 3 years of age. Journal of Pediatric Surgery. 2024;59(8):1647–1651.
3. Fuchs ME, Srinivas S, Quintanilla Amoros R, Jayanthi VR, Wood RJ, Dajusta DG. The effect of surgical reconstruction on bladder function in cloacal malformation: A study of urodynamics. Journal of Pediatric Urology. 2024; S1477-5131(24)00356-5. Advance online publication.
4. Skerritt C, Wood RJ, Jayanthi VR, Levitt MA, Ching CB, DaJusta DG, Fuchs ME. Does a standardized operative approach in cloacal reconstruction allow for preservation of a patent urethra? Journal of Pediatric Surgery. 2021;56(12):2295–2298.

Image Credit: Nationwide Children’s