Children's Hospital Colorado
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Care Considerations and Outcomes for Adolescents and Young Adults with Cloacal Anomalies

3/6/2024

Key takeaways

  • This scoping review summarizes the literature on the issues facing adolescents and young adults with cloacal anomalies.

  • It provides important guidance for urologists who did not receive training for pediatric cloacal anomalies but may treat these patients as adults.

  • Data was categorized into four domains: urologic, colorectal, gynecologic/obstetric and sexual/psychosocial.

  • With proper care and management, patients with cloacal anomalies can have a good prognosis for social continence and maintained kidney health.

  • More substantial research is needed for this population, especially in the areas of sexual function and transitioning to adult care.


Research study background

Cloacal anomalies are rare birth defects where the urethra, vagina and rectum are joined in a common channel. In the past half-century, advances in surgical reconstruction have shifted the focus from childhood survival to quality of life for adolescents and adults with this condition.

Researchers in the Department of Pediatric Urology at Children’s Hospital Colorado published a scoping review summarizing available literature on adolescents and young adults with cloacal anomalies. In part, the review serves to inform adult urologists without specialized pediatric training who may care for this population. Lead author Dan Wood, PhD, MB.BS, FRCS (Urol), who helped pioneer transitional urology care in London, joined Children’s Colorado in 2021 to establish a dedicated transitional urologic care program.

“It’s very important that these patients have lifelong care within a service that has appropriate expertise.”

– Dan Wood, PhD, MB.BS, FRCS (Urol)

Urologic considerations

Women with cloacal anomalies are at high risk for urologic dysfunction, including chronic kidney disease, which should be continually monitored. Through proper medical and surgical management, these patients can maintain kidney health and ~80% to 90% will achieve social continence.

Colorectal considerations

Both fecal incontinence rates and constipation rates in this population are wide-ranging, suggesting current data does not help predict bowel function based on a cloacal anomaly diagnosis. Fecal incontinence is one of the most common concerns of patients with anorectal malformations born after 1990. When compared to other anorectal malformations, patients with cloacal malformation were found to more likely to need bowel management.

A recent study of adolescents who used long-term treatment of Malone antegrade continence enemas, found significant improvements in overall bowel control and function. Existing studies about the MACE procedure are specific to adolescents and may not reflect the experience of adults with cloacal anomalies.

Gynecologic and obstetrical considerations

This review also emphasized the higher volume of Müllerian anomalies, and as such, menstruation and pregnancy are possible, but often require surgical intervention. Surgical options for these patients include vaginoplasty and introitoplasty, though there is not sufficient outcomes data to recommend either approach. Pre-pubertal vaginoplasty patients were found to experience a higher rate of complications during childbirth but researchers noted if other genital surgery is indicated, the intervention should still be performed before puberty. Women who underwent other types of vaginal replacement surgery did not achieve pregnancy, but it is unknown if they attempted pregnancy.

Sexual and psychosocial considerations

Given the lack of literature addressing sexual and psychosocial outcomes in this population, our team identified a strong need for more research to improve sexual health care for affected women. Available data suggests only half of adults with cloacal anomalies are sexually active and another study found frequent bowel and bladder incontinence was significantly correlated with higher sexual anxiety. In addition, adolescents with a history of anorectal malformations were found to face significant psychosocial barriers to transitioning to adult health care.

Clinical implications

Study authors noted these outcomes underscore the importance of reassessing this population at the onset of puberty and continuing care throughout adulthood. Our team is reviewing specific outcomes in additional detail and prospectively collecting data to help better understand the patient experience, optimize patient care and improve outcomes. As treatment improves at an earlier age for this patient population, it is becoming more important to provide education and training so that medical professionals can provide quality care when these patients reach adulthood.