Long-Term Bladder Management and Continence in Girls With Cloacal Malformation

Long-Term Bladder Management and Continence in Girls With Cloacal Malformation 1024 683 Pam Georgiana
Molly Fuchs, MD

New insights on outcomes and quality of life from Nationwide Children’s comprehensive study of pediatric patients.

Over the last decade, surgical innovation has revolutionized the treatment of cloacal malformation, a congenital anomaly affecting the development of the gastrointestinal, urinary and reproductive systems in girls. This condition occurs when the rectum, vagina and urinary tracts fail to separate properly during fetal development, resulting in a single channel, often called the “common channel.” Cloacal malformation is very rare, with only 40 to 50 girls born with it in the United States each year.

Little research exists on continence outcomes and bladder management for these girls in the long term. Recently, doctors at Nationwide Children’s Hospital took a significant step towards changing that with a study published in the Journal of Pediatric Surgery.

“Now that we can predictably do a safe and successful surgery with few complications and a good recovery process, it is time to look more closely at outcomes,” explains Molly E. Fuchs, MD, surgeon in the Department of Pediatric Urology and Department of Pediatric Colorectal and Pelvic Reconstructive Surgery at Nationwide Children’s.

The Nationwide Children’s Center for Colorectal and Pelvic Reconstruction (CCPR) provides multidisciplinary care to children with colorectal conditions. Because many of these conditions are complex, most patients require multifaceted medical care throughout their lives.

“After a decade of treating patients with cloacal malformation, our data is mature enough to study outcomes, which had really never been done with this degree of detail and follow up before. We were specifically looking to uncover insights about continence and bladder management,” Dr. Fuchs says.

Nationwide Children’s has built a database of approximately 300 patients with cloacal malformation. This is likely the largest database with detailed information that currently exists, given the rarity of the condition. However, the database is still relatively limited.

“There are many factors that can affect urologic outcomes that cannot be collected in a database,” Dr. Fuchs says. “But ours provides a good overview of patient and family experiences.”

Dr. Fuchs and team reviewed the treatment and outcomes of 152 patients aged three and older. Dryness is defined as patients either not wearing diapers or utilizing intermittent catheterization. Continence is defined as patients having less than one daytime accident per week. Patients with incontinent diversions through either vesicostomy (an opening from the bladder to the abdominal wall) or enterovesicostomy (an intestinal conduit) are considered wet.

Of the 152 patients, 61% are completely dry; 47% voided via urethra, 65% of those patients are dry; and 40% voided via clean intermittent catheterization, the vast majority of whom are dry. In addition,19 patients required bladder augmentation, a surgical procedure that increases bladder capacity.

“We can assume that the rates of dryness in this study are underestimated, because there are a number of kids included who are over age three and are not yet potty trained. There are also a portion who we know will require surgery and catheterization to be dry, but are not yet ready for surgery,” Dr. Fuchs explains. “We also need to do further research on other medical conditions and social situations that may affect continence, dryness, and the ability to perform intermittent catheterization.”

In the study, the length of the common channel was not associated with dryness or the need for intermittent catheterization. Also, the presence of an abnormal spinal cord was associated with the need for intermittent catheterization and a normal spine was associated with the ability to be dry.

“Urinary continence and dryness are not that simple,” Dr. Fuchs explains. “There are many complex factors that contribute to a patient’s continence outcomes. We have only scratched the surface of understanding this condition.”

“This study is a significant first step in guiding patients and their families about what to expect when diagnosed with cloacal malformation. However, because incredibly complex and nuanced factors are involved, more research is needed to truly predict outcomes,” Dr. Fuchs concludes.

 

 

Reference:

Morin JP, Srinivas S, Wood RJ, Dajusta DG, Fuchs ME. Bladder Management and Continence in Girls With Cloacal Malformation After 3 Years of Age. J Pediatr Surg. Published online April 20, 2024. doi:10.1016/j.jpedsurg.2024.04.010

About the author

Pam Georgiana is a brand marketing professional and writer located in Bexley, Ohio. She believes that words bind us together as humans and that the best stories remind us of our humanity. She specialized in telling engaging stories for healthcare, B2B services, and nonprofits using classic storytelling techniques. Pam has earned an MBA in Marketing from Capital University in Columbus, Ohio.