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All About

Bladder Exstrophy and Epispadias

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Symptoms

Bladder exstrophy is the most common in the larger group of birth defects called the bladder exstrophy-epispadias complex (BEEC). If your child has BEEC, he or she will have one of the following:

  • Epispadias. This is the least severe form of BEEC, in which the tube to expel urine (urethra) doesn't fully develop.
  • Bladder exstrophy. This defect causes the bladder to form on the outside of the body. The bladder is also turned inside out. Usually, bladder exstrophy will involve organs of the urinary tract, as well as the digestive and reproductive systems. Defects of the abdominal wall, bladder, genitals, pelvic bones, final section of the large intestine (rectum) and opening at the end of the rectum (anus) can occur.Children with bladder exstrophy also have vesicoureteral reflux. This condition causes urine to flow the wrong way from the bladder back up into the tubes that connect to the kidneys (ureters). Children with bladder exstrophy also have epispadias.
  • Cloacal exstrophy. Cloacal exstrophy (kloe-A-kul EK-stroh-fee) is the most serious form of BEEC. In this condition, the rectum, bladder and genitals don't fully separate as the fetus develops. These organs may not be correctly formed, and the pelvic bones are affected as well.

 

The kidneys, backbone and spinal cord also may be affected. Most children with cloacal exstrophy have spinal abnormalities, including spina bifida. Children born with protruding abdominal organs probably also have cloacal exstrophy or bladder exstrophy.

Causes

  1. Doctors aren't sure what causes bladder exstrophy to develop. Researchers think that a combination of genetic and environmental factors likely plays a role.
  2. What is known is that as the fetus grows, a structure called the cloaca (klo-A-kuh) where reproductive, urinary and digestive openings all come together doesn't develop properly in babies who develop bladder exstrophy. Defects in the cloaca can vary a lot depending on the age of the fetus when the developmental error occurs.

More about Treatment

Bladder exstrophy (EK-stroh-fee) is a rare birth defect in which the bladder develops outside the fetus. The exposed bladder can't store urine or function normally, resulting in urine leakage (incontinence).

 

Problems caused by bladder exstrophy vary in severity. They can include defects in the bladder, genitals and pelvic bones, as well as defects in the intestines and reproductive organs.

 

Bladder exstrophy may be spotted on a routine ultrasound during pregnancy. Sometimes, though, the defect isn't visible until the baby is born. Babies born with bladder exstrophy will need surgery to correct the defects.

 

FAQ on this Treatment

What is treatment for Bladder Exstrophy and Epispadiasis?

Treatment is Surgical Intervention i.e, Staged Reconstructive Approach.Medical treatment is ineffective to treat this defect.Surgeries are performed in following steps:
i. Closure of bladder plate after birth immediately
ii.Epispadias repair at 6-9 month or sometimes with bladder closure after birth
iii.Bladder neck repair at 5 years of age.

What care one has to take after Surgery?

Postoperative care includes:
i. wound and skin care to prevent infection
ii.Child must lie on back or side
iii.Medicines are generally given to reduce spasm and suprapubic catheter is inserted after procedure.
iv. Nutrition of child must be continued.

What are the advantages of this treatment?

Advantages of treatment include:
i.Defect is permanently closed so there will be no function as well anatomical abnormality of Bladder and Urethra as well as Genital organs.
ii.Infertility is prevented
iii. Quality of life is improved.

Can I prevent this disease from occuring?

If anyone in your family has this condition then it is wise to do Genetic testing but as it is costly hence USG during pregnancy can be diagnosed so careful examination during pregnancy is necessary. Termination of pregnancy is option but as this condition is surgical treatable so there is no harm in continuing pregnancy.

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