Bladder exstropy, more properly, the exstrophy-epispadias complex is a rare congenital anomaly 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. Treatment is with surgical correction of the defect, but patients can still have long term issues with urinary tract infections and sexual dysfunction. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // Q00-Q99 - Congenital malformations, deformations and chromosomal abnormalities (Q00-Q07) Congenital malformations of the nervous system (Q00) Anencephaly and similar malformations (Q01) Encephalocele (Q02) Microcephaly (Q03) Congenital hydrocephalus (Q04) Other congenital malformations of brain (Q05) Spina bifida (Q06) Other congenital malformations of spinal cord (Q07) Other congenital malformations of nervous... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... A congenital disorder is a medical condition or defect that is present at or before birth (for example, congenital heart disease). ...

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). The pubic symphysis is the midline cartilaginous joint uniting the superior rami of the left and right pubic bones (pubis). ... Hypospadias is a birth defect of the urethra in the male that involves an abnormally placed urethral meatus (opening). ... A womans clitoris extends from the visible portion to a point below the pubic bone. ... Hypospadias is a birth defect of the urethra in the male that involves an abnormally placed urethral meatus (opening). ... Cloacal exstrophy is a severe birth defect wherein much of the abdominal organs (the bladder and intestines) are exposed. ...

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

• Bladder exstrophy - Geneva Foundation for Medical Education and Research
• Bladder exstrophy page at The Children's Hospital Boston