Bladder exstrophy
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| Bladder exstrophy Classification and external resources | ||
| ICD-10 | Q64.1 | |
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| ICD-9 | 753.5 | |
| OMIM | 600057 | |
| DiseasesDB | 33377 | |
| eMedicine | ped/704 | |
| MeSH | C12.740.700.132 | |
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Bladder exstrophy, more properly, the exstrophy-epispadias complex is a rare congenital anomality occurring once every 40,000-50,000 live births with a 2:1 male:female ratio. The diagnosis involves a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis, and external genitalia. It occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal wall.
Treatment is with surgical correction of the defect, but patients can still have long term issues with urinary tract infections and sexual dysfunction.
Pathogenesis
The cause of bladder exstrophy is maldevelopment of the lower abdominal wall, leading to a rupture which causes the bladder to communicate with the amniotic fluid.
Spectrum of anomalies
The typical manifestation of exstrophy-epispadias complex is
- bladder everted through a midline lower abdominal wall defect
- widening of the pubic symphysis
- epispadias in males (dorsal cleft in the penis, exposing the urethral mucosa)
- bifid clitoris in females, with a short "urethral strip" indistinguishable from bladder mucosa.
The spectrum of disease extends from spade penis and epispadias on one hand, to exstrophy with cloaca (also known as cloacal exstrophy).
Treatment
Modern therapy is aimed at surgical reconstruction of the bladder and genitalia
Prognosis
Even with successful surgery, patients may have long-term problems with
- incontinence
- urinary reflux
- repeated urinary tract infections
- sexual dysfunction
External links
Congenital malformations and deformations of genital organs and urinary system (Q50-Q64, 752-753) | |
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| Genital organs (overview) | female: Uterine malformation - Mullerian agenesis - Uterine didelphys - Clitoromegaly
male: Cryptorchidism - Hypospadias - Chordee - Monorchism - Micropenis - Penile agenesis Pseudohermaphroditism |
| Urinary system | kidney/ureter: Renal agenesis/Potter syndrome, Papillorenal syndrome - cystic (Polycystic kidney disease, Meckel syndrome, Multicystic dysplastic kidney) - Ectopic ureter - Horseshoe kidney - Renal ectopia
bladder/urethra: Epispadias - Bladder exstrophy urachus: Urachal cyst |
| See also non-congenital reproductive and urinary conditions (N, 580-629) | |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
