Kiganos In puberty, extropie and reproductive functions constitute increasingly important issues for both sexes. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Case report Etiology The underlying cause remains unknown: Currently, several methods for bladder reconstruction with creation of an outlet resistance either as a staged or a one-stage approach during the newborn period are favored worldwide. Download the free HONcode toolbar. Depending on severity, the EEC may involve the urinary system, the musculoskeletal system, the pelvis, the exttrophie floor, the abdominal wall, the genitalia and sometimes the spine and the anus.
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Shortening of a pubic rami External rotation of the pelvis. Cause[ edit ] The cause is not yet clinically established but is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm. Diagnosis[ edit ] In a small retrospective study of 25 pregnancies five factors were found to be strongly associated with a prenatal diagnosis of bladder exstrophy: Inability to visualize the bladder on ultrasound A lower abdominal bulge A small penis with anteriorly displaced scrotum A low set umbilical insertion Abnormal widening of the iliac crests While a diagnosis of bladder exstrophy was made retrospectively in a majority of pregnancies, in only three cases was a prenatal diagnosis made.
For this reason, patients have the best outcomes when the bladder closures are performed at high volume centers where surgical and nursing teams have extensive experience in caring for the disease.
In the event the child was not born at a medical center with an appropriate exstrophy support team then transfer will likely follow. Upon transfer, or for those infants born at a medical center able to care for bladder exstrophy, imaging may take place in the first few hours of life prior to the child undergoing surgery. Surgery[ edit ] Watercolour drawing of ectopia vesicae in a man aged 23 years, after operation Modern therapy is aimed at surgical reconstruction of the bladder and genitalia.
Both males and females are born with this anomaly. Treatment is similar. In males treatments have been: In the modern staged repair of exstrophy MSRE the initial step is closure of the abdominal wall, often requiring a pelvic osteotomy. This leaves the patient with penile epispadias and urinary incontinence. At approximately 2—3 years of age the patient then undergoes repair of the epispadias after testosterone stimulation.
Finally, bladder neck repair usually occurs around the age of 4—5 years, though this is dependent upon a bladder with adequate capacity and, most importantly, an indication that the child is interested in becoming continent.
In some of the bladder reconstructions, the bladder is augmented with the addition of a segment of the large intestines to increase the volume capacity of the reconstructed bladder. Surgical reconstruction to correct the split of the mons, redefine the structure of the bladder neck and urethra.
Vaginoplasty will correct the anteriorly displaced vagina. If the anus is involved, it is also repaired. Fertility remains and women who were born with bladder extrophy usually develop prolapse due to the weaker muscles of the pelvic floor.