Bladder exstrophy

Bladder exstrophy

Infobox_Disease
Name = Bladder exstrophy


Caption =
DiseasesDB = 33377
ICD10 = ICD10|Q|64|1|q|60
ICD9 = ICD9|753.5
ICDO =
OMIM = 600057
MedlinePlus =
eMedicineSubj = ped
eMedicineTopic = 704
MeshName = Bladder+Exstrophy
MeshNumber = C12.740.700.132

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.

pectrum 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 withcite journal |author=Gargollo PC, Borer JG |title=Contemporary outcomes in bladder exstrophy |journal=Current opinion in urology |volume=17 |issue=4 |pages=272–80 |year=2007 |pmid=17558272 |doi=10.1097/MOU.0b013e3281ddb32f]
*incontinence
*urinary reflux (see Vesicoureteral_reflux)
*repeated urinary tract infections
*sexual dysfunction

References

External links

* [http://www.childrenshospital.org/az/Site856/mainpageS856P0.html Bladder exstrophy page] at [http://www.childrenshospital.org Children's Hospital Boston]


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Look at other dictionaries:

  • exstrophy-epispadias complex — a group of congenital defects of the anterior abdominal wall, including exstrophy of the bladder, exstrophy of the cloaca, and epispadias, believed to be caused by failure of the cloacal membrane to be reinforced by ingrowth of mesoderm …   Medical dictionary

  • Exstrophy — Ex stro*phy, n. [Gr. ? to turn inside out; ? = ? out + ? to turn.] (Med.) The eversion or turning out of any organ, or of its inner surface; as, exstrophy of the eyelid or of the bladder. [1913 Webster] …   The Collaborative International Dictionary of English

  • exstrophy — [ek′strə fē] n. [< EX 1 + Gr strophē, a turning: see STROPHE] Med. the turning inside out of an organ; esp., such a congenital condition of the urinary bladder …   English World dictionary

  • Exstrophy — Eversion of a hollow organ at birth. An exstrophic bladder is one that is turned inside out like a rubber glove. In exstrophy of the cloaca (a primitive embryonic structure) an area of the intestine is interposed between two separate areas of the …   Medical dictionary

  • exstrophy of bladder — a developmental anomaly marked by absence of a portion of the lower anterior abdominal wall and the anterior bladder wall, with eversion of the posterior bladder wall through the defect and with an open pubic arch and widely separated ischia… …   Medical dictionary

  • exstrophy — /ek streuh fee/, n., pl. exstrophies. Pathol. a birth defect resulting in the eversion of an organ: exstrophy of the bladder. [1830 40; < Gk ekstroph(é) inversion of the uterus, lit., a turning inside out (see EC , STROPHE) + Y3] * * * …   Universalium

  • exstrophy of cloaca — cloacal exstrophy a developmental anomaly in which two segments of bladder (hemibladders) are separated by an area of intestine with a mucosal surface, resembling a large red tumor in the midline of the lower abdomen. Called also ectopia cloacae …   Medical dictionary

  • exstrophy — n. a severe congenital abnormality in which the bladder fails to close during development: the baby is born with an absent lower abdominal wall and the internal surface of the posterior bladder wall is exposed. It is associated with epispadias,… …   The new mediacal dictionary

  • exstrophy —   n. Medicine, having organ especially bladder, turned inside out …   Dictionary of difficult words

  • exstrophy — ˈekstrəfē noun ( es) Etymology: ex (I) + strophy (from Greek strophein to turn + English y) more at strophe : eversion of a part or organ; spe …   Useful english dictionary

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