Ces Urol 2019, 23(1):19-29 | DOI: 10.48095/cccu2019002

Diagnosis, therapy and urological complications of cloacal malformations

Jan Trachta¹, Martin Kynčl², Richard Škába¹

¹ Klinika dětské chirurgie 2. LF UK a FN Motol, Praha

² Klinika zobrazovacích metod 2. LF UK a FN Motol, Praha

Trachta J, Kynčl M, Škába R. Diagnosis, therapy and urological complications of cloacal malformations. 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.

Keywords: Cloacal malformation, hydrocolpos, mobilisation of urogenital sinus.

Received: October 10, 2018; Accepted: January 25, 2019; Prepublished online: February 26, 2019; Published: March 25, 2019