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Bladder
Congenital anomalies
Exstrophy
Author: Nat Pernick, M.D. (see Authors page)
Revised: 2 January 2009, last major update – January 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
Definition
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● Bladder communicates with body surface or lies as an opened sac
Terminology
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● Part of exstrophy-epispadias complex
● Also called classic bladder exstrophy
Epidemiology
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● Prevalence at birth is 1 per 30,000; male predominance up to 3:1
● May be associated with maternal tobacco use (J Urol 2008;179:1539)
Sites
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Etiology
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● Developmental failure in lower abdominal wall or anterior wall of bladder due to failure of cloacal membrane to property differentiate
● Persistence or overgrowth of cloacal membrane on lower anterior abdominal area may prevent normal mesenchymal ingrowth, which causes divergence of lower abdominal muscular structures, and forces the genital ridges to fuse caudal to the cloacal membrane (J R Soc Med 1996;89:39P)
● The stage of ingrowth of the urorectal septum at the time of rupture determines whether one will produce an exstrophic urinary tract alone (classic bladder exstrophy or epispadias) or cloacal exstrophy with the hindgut interposed between the hemibladders
Clinical features
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● Usually diagnosed with prenatal scans
● Urine drips from ureteric orifices onto bladder surface (Orphanet J Rare Dis 2009 Oct 30;4:23)
● Bladder mucosa is red at birth, and mucosal polyps may be seen
● If closure is delayed, may have inflammatory changes of whitish coating, ulcerations and hyperplastic formations
● Thin skin stripes mark transition between normal skin and squamous metaplastic area
● Most patients have bilateral inguinal hernias
● Male newborns: open (epispadic) urethral plate covers whole dorsum of the penis from open bladder to glandular grove
● Female newborns: have completely split clitoris next to the open urethral plate; also narrow vaginal opening anterior to a shortened perineum
● Associated with glandular metaplasia and adenocarcinoma (<10% of exstrophied bladders) or squamous metaplasia and squamous cell carcinoma (~7% of patients)
● Also associated with infections and ulceration
Prognostic factors
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Case reports
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Treatment
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● Surgical correction with several procedures over time (Johns Hopkins), usually beginning in first week of life
● Rarely, adults present to physicians with exstrophy (Indian J Urol 2008;24:164)
Clinical images
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Diagrams of exstrophy
Gross description (Macroscopy)
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Gross images
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Exstrophy in
male infant
Micro description (Histopathology)
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Micro images
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Cytology description
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Cytology images
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Positive stains
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Negative stains
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Electron microscopy descriptions
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Electron microscopy images
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Molecular / cytogenetics description
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Molecular / cytogenetics images
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Differential Diagnosis
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Additional references
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End of Bladder > Congenital anomalies > Exstrophy
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