Abstract
Major Statement: A review article about the diagnostic tools and management of persistent cloaca malformation that belongs to one of the most complex new-born anomalies with a need of the life-long follow-up by pediatric and general urologist. The majority of cloaca patients undergo multiple reconstructive procedures.
Cloacal malformation is the most severe form of anorectal malformation in girls. The complex treatment is possible only in specialized pediatric centers and life-long follow-up is needed.
Females with persistent cloaca suffer also from many associated anomalies, especially urinary tract and spinal malformations. Correct anatomical and functional reconstruction of persistent cloaca belongs to one of the most challenging tasks in pediatric surgery and urology.
The goal of the complex therapy is to help patients achieve urinary and fecal continence, regular menstruation at puberty and possibility of good quality of sexual life in adulthood. In this review article based on the current literature and our own experience we describe the spectrum of cloaca malformations and associated anomalies, possible techniques of reconstruction and expected outcomes after the treatment by multiple specialist team.
For achieving urinary continence these patients often need several urology procedures. However, despite the complex treatment half of the cloaca girls will develop chronic kidney disease before achieving adulthood and therefore detailed and long-term follow-up by dedicated pediatric and general urologist is mandatory.