Real-World Urinary Management and Outcomes in Cloacal Exstrophy Among Children and Adults

Real-World Urinary Management and Outcomes in Cloacal Exstrophy Among Children and Adults 1024 683 Jessica Nye, PhD
Molly Fuchs, MD

Most (94%) older children and adults with cloacal exstrophy are managed by incontinent diversion or clean intermittent catheterization.

“The published literature about cloacal exstrophy can be misleading, and there is an impression that with surgical bladder reconstruction, normal bladder function and voiding can be restored. That is to say, a child will be able to potty train and to control their urine without having accidents. However, this is probably an oversimplification, and my colleagues and I believe it is unrealistic,” says Molly E. Fuchs, MD, urologist at Nationwide Children’s Hospital.

To evaluate real-world incontinence management in cloacal exstrophy, the Pediatric Urology Midwest Alliance (PUMA) group analyzed data from children and adults cared for at five institutions.

The study population comprised 40% boys or men and 60% girls or women aged <10 years (n=57), 10-17.9 years (n=35), or >18 years (n=68).

Overall, no child or adult had volitional voiding through their urethra with at least a three-hour dry interval.

More adults (68%) and older children (83%) were managed with clean intermittent catheterization (CIC) than younger children (32%; P <.001) whereas fewer adults (15%) or older children (11%) emptied urine via urethra (e.g., CIC per urethra, Valsalva, incontinence) compared with younger children (46%; P <.001).

“Historically, bladder reconstruction was performed to try to achieve voiding via the urethra by trying to reconstruct the bladder to look normal, hoping it would also function normally. This would often require numerous surgical procedures and have poor success rates. Perhaps, instead of trying to achieve normal bladder function, we should prioritize preserving renal function and creating a urinary system that allows for good quality of life, which may require a urinary stoma or catheterization,” says Dr. Fuchs.

Bladder augmentation occurred at a median age of 7 years among 87 patients. The rates of augmentation increased with age from 21% among younger children to 71%-74% among older children and adults (P <.001).

The study authors noted that most of the patients (79%) also had intestinal diversion. The management strategy for bowel diversion differed significantly on the basis of institution (P =.001) whereas urinary management strategies did not (P =.31).

In the future, Dr. Fuchs and colleagues want to focus on kidney health, the patient experience and ensuring that patients have maximal function and comfort while minimizing time spent in the health care setting.

Dr. Fuchs concluded, “We need more information about patients’ experience and satisfaction. Are patients happy with how they manage their bladder? Is there any decisional regret? The reality is we’re making these decisions for children, and we don’t really know how adults who have this condition feel. My goal is to create a way to manage the bladder that’s durable, that hopefully doesn’t require a lot of surgical procedures, particularly early in life, so we can keep these children at home to develop and thrive and not in the hospital their whole childhood.”



Fuchs ME, Ahmed M, Dajusta DG, Gargollo P, Kennedy UK, Rosoklija I, Strine AC, Whittam B, Yerkes E, Szymanski KM, on behalf of PUMA. Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study. Journal of Pediatric Urology. 2022;S1477-5131(22)00400-4.

About the author

Jessica Nye, PhD, is a freelance science and medical writer based in Barcelona, Spain. She completed her BS in biology and chemistry and MS in evolutionary biology at Florida State University. Dr. Nye studied population genetics for her doctorate in biomedicine at University of Pompeu Fabra. She conducted her postdoctoral research on the inheritance of complex traits at the Autonomous University of Barcelona.