Bladder - Persistent cloaca

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Bladder

Congenital anomalies

Persistent cloaca

Reviewers: Alcides Chaux, M.D., Johns Hopkins University School of Medicine (see Reviewers page)
Revised: 4 April 2011, last major update April 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

Definition
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● 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
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● Also called cloacogenic bladder

Epidemiology
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● Occurs in 1 per 20,000 births, only in girls

Etiology
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● 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)

Clinical features
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● 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 Mullerian 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
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● Prenatal diagnosis of persistent cloaca associated with VATER (Tohoku J Exp Med 2007;213:291)

Treatment
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● Reconstruction of cloaca by total urogenital mobilization (J Urol 2009;182:2455, eMedicine)

Clinical images
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Normal embryo at 25-27 days

Normal embryo at weeks 5-7

Diagram of persistent cloaca

Gross images
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Persistent cloaca serves as convergence of rectum and both ureters

Additional references
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● Series of 490 cases with cloacal malformations (Semin Pediatr Surg 2010;19:128)

End of Bladder > Congenital anomalies > Persistent cloaca

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