Bladder, ureter & renal pelvis
Congenital anomalies
Persistent cloaca
Author: Alcides Chaux, M.D.
Last author update: 1 April 2011
Last staff update: 23 November 2020
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Persistent cloaca
Table of Contents
Definition / general | Terminology | Epidemiology | Etiology | Diagrams / tables | Clinical features | Case reports | Treatment | Additional referencesCite this page: Chaux A. Persistent cloaca. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bladdercloacogenic.html. Accessed October 4th, 2023.
Definition / general
- Defined as confluence of rectum, vagina and urethra into a single common chamber that opens into a single orifice where the normal urethral opening is located
- Represents a wide spectrum of defects with the common denominator of a single perineal orifice
- The length of the common chamber varies from 1-10 cm (average 3 cm)
Terminology
- Also called cloacogenic bladder
Epidemiology
- Occurs in 1 per 20,000 births, only in girls
Etiology
- Cloaca is single canal from which the urinary, genital and intestinal tracts arise at gestational weeks 5-6
- Persistent cloaca is due to failure of urogenital septum to divide rectum from urogenital sinus
- May be related to B-class Eph/ephrin signaling (J Pediatr Urol 2007;3:354)
Diagrams / tables
Images hosted on other servers:
Normal embryo at 25 - 27 days
Normal embryo at weeks 5 - 7
Diagram of persistent cloaca
Clinical features
- Dilated sac like structure in center of abdomen
- 80% have an associated anogenital anomaly, including imperforate anus, absent genital or urinary orifices, renal agenesis, absence of prostate or seminal vesicles or poorly developed foregut derivatives
- 30% of patients present with hydrocolpos (dilated vagina filled with fluid, urine or mucus) and 40% have a duplicated Müllerian system
- May be identified prenatally by ultrasound or MRI in a female fetus presenting with hydronephrosis and a large cystic lesion arising from the pelvis (Congenit Anom (Kyoto) 2009;49:116)
Case reports
- Prenatal diagnosis of persistent cloaca associated with VATER (Tohoku J Exp Med 2007;213:291)
Treatment
- Reconstruction of cloaca by total urogenital mobilization (J Urol 2009;182:2455, eMedicine)
Additional references
- Series of 490 cases with cloacal malformations (Semin Pediatr Surg 2010;19:128)
