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Bladder Exstrophy Treatment India

What is Bladder Exstrophy?

Best Cost Bladder Exstrophy Treatment in India Top Hospital Surgeon Delhi, Mumbai, Chennai, Hyderabad, Banglore INDIAAn abnormality of formation of the bladder and the bony pelvis. The bladder does not form into its normal round shape but instead is flattened and exposed on the abdominal wall. The pelvic bones are also widely separated. The remainder of the lower urinary tract may also be flattened and exposed, with abnormal formation of the prostate and penis. This congenital birth defect is seen in one of 10,000 to 50,000 live births. In a family with a child with exstrophy, the likelihood of a second child being born with exstrophy is one in 100. The risk of having a child with exstrophy is one in 70, if the parents have exstrophy.


Diagnosis

Diagnosis can be made on careful repeated ultrasounds done before delivery, but usually the diagnosis is not made until the baby is born. The finding of the exposed bladder is typical.


Treatment

Advances in surgery in the last 15 years have allowed reconstruction of the penis and bladder so that a more "normal" and functional lifestyle can be maintained by the patient and family. Current state-of-the-art treatment for exstrophy involves reconstruction of the various aspects of the deformity (i.e. closing the bladder, repair of the penis, and prevention of urine leakage. This usually involves separate operations at various times in the life of the child to obtain the best results.

  • Initial Closure: This is usually done soon after the baby is born. At this first operation, the pelvic bones are reformed into their normal ring shape (by a Pediatric Orthopedic surgeon), the bladder, abdominal wall, and posterior urethra are closed and the belly button is reconstructed. This procedure takes between 4-6 hours. Following surgery, the baby is placed with the lower legs in traction to prevent separating the pelvic bones. Babies are observed carefully and may be in the Intensive Care Unit to be monitored for the first day or two. The baby usually stays in the hospital for 3-4 weeks of healing. Antibiotics are given following the operation to prevent infection. The tube in the bladder is removed four weeks following surgery. The size of the bladder gradually increases over time. In very special circumstances with an excellent bladder plate and good sized penis, bladder exstrophy closure and epispadias repair can be combined. 
    However, this is only for very experienced exstrophy surgeons.

  • Epispadias repair: This repair occurs around 6-12 months of age. Time of surgery and the extent of the surgery is dependent on the size of the bladder and the deformity of the penis. At this stage, the urethra on top of the flattened penis is closed and transferred to below the corporal bodies as in the normal penis.

  • Continence Procedure: At this time the control of urine leakage is repaired and then further enlargement of the bladder is preformed if necessary. The timing of this procedure depends totally on the capacity of the bladder and the child's emotional and developmental status. The child must "want to be dry" and able to participate in a voiding program.


Special Voiding Improvement Program for the Exstrophy - Epispadias Patient

Children with bladder exstrophy face a combination of medical and emotional challenges as they work with their urology team. Developing a continence management program is an ongoing process that is often stressful for children and their families. The voiding improvement program provides hands-on one-on-one assistance to the child and family before and after bladder neck repair.

The clinic is staffed by a specially trained, multidisciplinary team including a pediatric urologist, pediatric nurse practitioner, pediatric behavioral psychologist, and pediatric clinical nurse.

The Voiding Improvement Team also assists the pediatric urologist in the evaluation of readiness for bladder neck reconstruction to help the child and family prepare for the post-surgical work that will allow for favorable continence outcome.

After bladder neck reconstruction surgery we work with children and families using both behavior modification and muscle retraining procedures to teach the child, family, and the child's bladder musculature to function at their maximum potential for long-term continence.

After bladder neck surgery, frequent daily phone consultations occur until the child is voiding well and tube free.

Visit usually last one hour and may include the following treatment components:

  • Bladder ultrasound

  • Urine culture

  • Medication management

  • Biofeedback for bladder muscle retraining

  • Establishment of a voiding urine

  • Assessment of barrier to adherence

  • Nutrition education

  • Behavior therapy

  • Relapse prevention recommendations

Progress is evaluated at each visit and communicated to the pediatric urologist.


Follow-up

Experience indicate that 72-75% of patients are free of urine leakage following reconstruction by the above-mentioned stages. The deformity of the penis was corrected to the satisfaction of the patient and the family in most instances. This, however, requires dedicated and intensive treatment and long-term follow up into adolescence and adulthood by the exstrophy team.


Disorders Associated with Bladder Exstrophy

Epispadius - The urethra, which carries urine out of the body, has not formed completely. In boys, the penis is flattened and is pulled up toward the abdomen with the urethra open on the upper surface of the penis. In girls, the urethral opening is located between a divided clitoris and labia minora.

Vesicoureteral Reflux - Urine is made by the kidneys and travels down tubes called ureters to be stored in the bladder. Normally urine flows one way. Reflux is a condition where urine can back up from the bladder into the kidneys. Reflux becomes serious when bacteria-infected urine in the bladder travels to the kidneys, which can lead to kidney damage or worse: loss of kidney function. Many children outgrow this condition, but some require surgery to repair this problem. Some children are placed on a daily low-dose antibiotic for a period of time.

Diastasis - Separation of the front pubic bones which does not allow the bladder to remain inside the body

Small Bladder Capacity - All exstrophy bladders are small at birth, some smaller than others. The extent to which the bladder will grow cannot be definitely determined. Successful bladder closure and epispadius repair provide conditions for the bladder to grow. Good urethral resistance can help "stretch" the bladder. Time will tell.

Missing Bladder Neck and Sphincter - The lower portion of the bladder, a funnel-shaped bladder neck and sphincter, consists of muscles that open and close the bladder outlet to control urine flow.


The Normal Urinary System

The urinary system consists of two kidneys, two ureters, the bladder, the bladder sphincter (the muscle that acts as a binder to hold urine in the bladder) and the urethra. The kidneys clean waste and remove excess fluids from the blood, as well as help to manage blood pressure. The ureters are the tubes that drain the waste, the urine, into the bladder. The urine is stored in the bladder. When the bladder is full, it contracts, and the sphincter opens allowing the drainage of urine into the urethra to the outside of the body.


What Surgeries Will My Child Need?

Please be aware that each case is different and your doctor will decide which surgery and treatment plan is best for your child. In most cases more than one surgery will be necessary and your doctor will discuss this with you. The following information is generalized and not specific to your child.

The first surgery is primary closure of the exstrophied bladder and proper placement of the pubic bones. This is best done within the first seventy-two hours after birth. After the bladder closure the infant will be placed in a position called modified Bryant's traction. This is a position in which the hips have 90 degrees of flexion, the knees and ankles are held together, and the buttocks are slightly elevated off the bed. This traction may be needed for three to four weeks. In some cases more time may be necessary.

The bladder size may be very small. Surgery will be needed to increase the size of the bladder. This will allow the bladder to hold an ample volume of urine. Most patients will need to empty the bladder of urine with a tube/catheter. The catheter is inserted into the bladder, the urine is drained and then the catheter is removed. This procedure is called clean intermittent urinary catheterization. This will be done several times a day. This will be taught to the patients by the pediatric urology nurse or another qualified professional.


The Male Patient

The male may have a short, curved penis which may appear somewhat flat at the top. The urethral opening is epispadiac (on the upper surface of the penis). There is usually a space between the base of the penis and the scrotum. The patient may have bilateral inguinal hernias. The testes may be undescended (not in the scrotum) or retractile (capable of going back into the scrotum). If hernias are present they will be repaired. Reconstructive surgery will be done to repair the penis. This surgery will result in functional and cosmetically acceptable genitalia.


The Female Patient

The female patient usually presents with a normal uterus, fallopian tubes, and ovaries. The vagina may be slightly higher in placement and somewhat narrowed. The clitoris is separated into two parts; the labia and mons pubis (hair-bearing skin) are spread apart. Reconstructive surgery will be performed to bring the clitoris, mons pubis and labia (if necessary) together. This surgery will provide functional and cosmetically acceptable genitalia.

The adult female patient will be able to have normal sexual intercourse. Almost all females are able to have children. When the female is ready to start a family her doctor should be consulted since she will be prone to prolapse of the uterus. This is not dangerous when monitored carefully. Her doctor will determine if a cesarean section will be necessary.


Potential Issues Facing Exstrophy

  • Urinary Tract Infections

  • Latex Allergy

  • Intermittant Catheterization

  • Bladder Stones

  • Inguinal Hernias


What to Expect in the Hospital

  • Catheters

  • Intravenous (IV)

  • NG Tube

  • Antibiotics

  • ICU

  • Traction

 

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