bladder exstrophy is a severe congenital malformation in which the children had bladder no front wall and the corresponding wall of the abdomen.This defect is extremely rare - in one case, by 40-50 thousand babies.Boys are born with exstrophy 2 times more often girls.
What is the cause of the children of this severe defect medical science has not yet been found.
How does a child with exstrophy of the bladder?
child with exstrophy of the bladder is born with a gaping hole in his stomach.The rear wall of this hole is a bladder wall.The hole diameter range from 3 to 8 cm., The divergence pubic bone can reach 4-7 cm. Because of this discrepancy child exstrophy patient acquires the characteristic "duck" gait.
inner surface of the bladder is lined with a mucous bright red.On mucosa seen two slots - a ureteral orifice.From the mouths of streams jerky follows urine flowing on the perineum, the area of the genitals, inner thighs.
When a child is in tension (crying, screaming, laughing), there is a bulgi
bladder neck and sphincter of these children are absent.The bladder is very small in size, ureters disposed properly and therefor are part of the urine is thrown back into the kidney.A gaping hole of the bladder mucosa and continuous contact with the environment leads to the development of such children pyelonephritis.
Caring for such patients is very difficult for the kids.Because of his defect, they can not communicate with their healthy peers.From sick children always smells of urine.In addition, bladder exstrophy, and is often accompanied by other developmental defects, such as for example cryptorchidism or inguinal hernia.
bladder exstrophy is different severity
first degree exstrophy - when the hole within 4 cm discrepancy pubic bone up to 4 cm, and a minimum of related developmental defects.
exstrophy second degree - the size of the hole in the stomach varies between 5 and 7 cm, and the pubic bone at odds of 6-9 cm. This pathology is aggravated by multiple concurrent developmental defects.
third degree exstrophy - associated with severe malformations.Pubic bone in this case, spending more than 9 cm.
Treatment of children with bladder exstrophy
treat children with this congenital pathology is possible only by surgery.Carrying out such an operation it is recommended in the first days of a baby born.If you put off the operation at a later time, the surgeon will not be able to form a complete pelvic ring.
However, some medical sources recommend the operation at a later 2-3 months of age.
1. If the open hole of the bladder is not more than 3 cm in diameter, while exstrophy is not accompanied by other developmental disabilities, and the child feels fine, then surgery is reconstructive surgical character.The surgeon from the surrounding tissues will form the front wall of the bladder and close the anterior abdominal wall.In most cases, the operation results are satisfactory, but the likelihood of incontinence remains high in most cases.
2. In a more complex case, the problem is the surgeon transplant ureters in the sigmoid colon and ensuring antireflux mechanism throw urine into the ureter.Such surgical treatment of bladder exstrophy is currently most prevalent.If the operation is successful, then the child has urinary incontinence stops.If it is not the end of the operation is stored urine reflux and leads to the development of pyelonephritis and other diseases of the kidneys.
3. Another option transactions - transplant ureters in a separate segment of the intestine.Usually it uses the rectum.The surgeon performing the resection of the rectum at the point where it enters the sigmoid.The hole is sutured.The lower sector of the sigmoid colon surgeon sews to the rectum near the anus.The resulting isolated segment of the rectum is moved both ureters.Thus obtained artificial bladder.
In general, treatment of children with bladder exstrophy - this is an extremely complex, time-consuming and expensive process.However, if a timely appeal to the clinic, where the accumulated successful experience of such operations, there is a chance that a child born with this severe pathology, be able to live a normal life.