Bladder exstrophy (also known as ectopia vesicae) refers to a herniation of the urinary bladder through an anterior abdominal wall defect. The estimated incidence of bladder exstrophy is ,, live births 4,6. Bladder exstrophy is thought to be caused by a developmental. We have described an operation the aim of which is to reduce the gap at the pubic symphysis which accompanies ectopia vesicae. 2. The operation. as it is now. What is Bladder Exstrophy? Bladder exstrophy â€“ also called Ectopia vesicae, is a congenital anomaly o.
|Published (Last):||5 September 2014|
|PDF File Size:||18.9 Mb|
|ePub File Size:||8.98 Mb|
|Price:||Free* [*Free Regsitration Required]|
Bladder exstrophy-epispadias-cloacal exstrophy complex is a spectrum of anomalies involving the urinary tract, genital tract, musculoskeletal system and sometimes the intestinal tract. In classic bladder exstrophy, most anomalies are related to defects of the abdominal wall, bladder, genitalia, pelvic bones, rectum and anus. Bladder exstrophy is a rare developmental abnormality that is present at birth congenital in which the bladder and related structures are turned inside out.
The rear portion of the bladder wall posterior vesical wall turns outward exstrophy through an opening in the abdominal wall and urine is excreted through this opening. The extent of the exstrophy depends on how large the opening is.
Extrophy of bladder Diwakar RK, Khurana O – West Afr J Radiol
The mildest form is when there is a defect or opening in the tube that carries urine out of the body from the bladder urethra and is termed vezicae. The most severe form is when there is a defect in the urethra, bladder and bowel cloacal exstrophy. Classic bladder exstrophy is when there is a defect in the urethra and bladder and is intermediate in severity. The underlying cause of this complex is not known.
The physical characteristics are the result of a developmental abnormality during embryonic growth in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles.
The bladder-exstrophy-epispadias-cloacal exstrophy complex can take many forms depending on the extent of the vesicqe abnormality that causes it. The mildest form is when there is an opening in the urethra epispadias.
The most severe form is when there is an opening in the urethra, bladder and bowel cloacal exstrophy. The most common form is classic bladder exstrophy in which the bladder and related structures are turned inside out through an opening in the abdominal wall. Classic bladder exstrophy is intermediate in severity and the bladder is open from the top of the bladder through the urethra and to the tip vesicxe the penis.
Boys with epispadias have ectopi urethra that is extremely short and split and the opening vezicae on the upper surface of the penis. Girls with epispadias have a urethral opening located between a split clitoris and labia minor.
Cloacal exstrophy is a severe birth defect in which there is usually a membrane-covered area on the abdominal wall that contains the abdominal contents omphalocele. The bladder is divided in two halves vesivae males have a penis split in two halves.
Females have a clitoris divided in two halves and may have two vaginal openings. The opening of the rectum to the outside of the body is usually missing or abnormally small. Other abnormalities are sometimes associated with the complex. These include a separation of the pubic ectoppia, absence of the lower portion of the bladder ectoppia lack of bladder control incontinence and abnormal position of the tubes that carry urine from the kidneys to the bladder ureters causing back up of urine in the kidneys reflux.
In normal development, the cloacal membrane temporarily separates the urogenital and echopia structures and them breaks when tissue that will form etopia muscles begins to grow in its place.
The bladder exstrophy-epispadias-cloacal exstrophy complex is caused by a developmental abnormality that occurs weeks after conception in which the cloacal membrane is not replaced by tissue that will form the abdominal muscles.
The underlying cause of this error in development is not known. The birth prevalence of classic bladder exstrophy has been estimated to be between 1 in 10, and 1 in 50, livebirths. Males are affected times more often than females. Isolated epispadias occurs in approximately 1 inlive male births and 1 inlive female births. Cloacal exstrophy occurs in approximately 1 inlive births. Prenatal ultrasound examination of a fetus with the complex may reveal absence of bladder filling, low-set umbilical cord, separation of pubic bones, small genitals and an abdominal mass that increases in size as the pregnancy progresses.
The treatment of bladder exstrophy consists of a series of corrective surgeries performed over several years. The first surgery is closure of the bladder to allow it to hold urine, placement of the bladder inside the pelvis and closure of the abdominal wall. In some cases, children with bladder exstrophy may also require a series of surgical procedures to reconstruct the external genitalia.
These surgeries are usually performed before the age of 2 years. Bladder neck reconstruction is performed at approximately 5 years of age to allow control of urine and ureters are repositioned to prevent urine from backing up into the kidneys. The outlook for maintaining normal kidney function after surgical correction and reconstruction is good.
However, some individuals with this disorder may experience long-term urinary problems such as kidney stones, kidney infections, and varying degrees of urinary incontinence. Other treatment is symptomatic and supportive. The use of three-dimensional CT to evaluate the bony pelvis and pelvic floor is being explored. This may provide new insight for long-term issues such as urinary and fecal incontinence and pelvic organ prolapse and help in developing better techniques for reconstruction of the pelvic bones and pelvic floor osteotomy in these patients.
Researchers at the Johns Hopkins Institute of Genetic Medicine and the Brady Urology Institute are seeking to identify genes and environmental factors that cause exstrophy and epispadias. For more information on this study, please contact:. Information on current clinical trials is posted on the Internet at www. All studies receiving U.
Lippincott, Williams and Wilkins, Bladder and cloacal exstrophy. Lippincott Williams and Wilkins.
State-of-the-art reconstructive surgery of bladder exstrophy at the Johns Hopkins Hospital. Ecopia J Dis Child. Sonographic findings in the prenatal diagnosis of bladder exstrophy.
Am J Obstet Gynecol. Gearhart JP, et al.
Rare Disease Database
The failed exstrophy closure: Csontai A, et al. Results of surgical treatment in children with bladder exstrophy. Connor JP, et al.
Long-term follow-up of patients with bladder exstrophy: Closure of the exstrophic bladder: The content of the website and databases of the National Organization for Rare Disorders NORD is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD.
About News Events Contact. General Discussion Bladder exstrophy-epispadias-cloacal exstrophy complex is a spectrum of anomalies involving the urinary tract, genital tract, musculoskeletal system and sometimes the intestinal tract.
Causes In normal development, the cloacal membrane temporarily separates the urogenital and anal structures and them breaks when tissue that will form abdominal muscles begins vesicwe grow in its place. Affected Populations The birth prevalence of classic bladder exstrophy has been estimated to be between 1 in 10, and 1 in 50, livebirths. Diagnosis Prenatal ultrasound examination of a fetus with the complex may reveal absence of bladder filling, low-set umbilical cord, separation of pubic bones, small genitals and an abdominal mass that increases in size as the pregnancy progresses.
Standard Therapies Treatment The treatment of bladder exstrophy consists of a series of corrective surgeries performed over several years.
Investigational Vewicae The use of three-dimensional CT to evaluate the bony pelvis and pelvic floor is being explored. For more information on this study, please contact: Assistant Professor of Genetic Medicine and Pediatrics sboyd mail. Years Published, Alone we are rare. Together we are strong.