Great Ormond Street Hospital for Children NHS Foundation Trust: Information for Families Bladder exstrophy and epispadias This leaflet explains about bladder exstrophy and epispadias and what to expect when your child comes to Great Ormond Street Hospital (GOSH) for treatment. GOSH is one of only two hospitals in the UK carrying out bladder exstrophy repair operations, as research has shown that the outlook is better when a child is cared for in a specialist treatment centre. How does the urinary What is bladder exstrophy? system work? ‘Exstrophy’ means ‘turned inside out’. The urinary system consists of the kidneys, Bladder exstrophy is a congenital (present at the bladder and ureters. The kidneys birth) abnormality of the bladder. It happens filter the blood to remove waste products when the skin over the lower abdominal and form urine. The urine flows from wall (bottom part of the tummy) does not the kidneys down through the ureters to form properly, so the bladder is open and the bladder. From here it passes through exposed on the outside of the abdomen. another tube called the urethra to the outside when urinating (peeing). What is epispadias? In epispadias, the urethra does not form properly. All boys with bladder exstrophy also have epispadias, but it can also occur on it own. kidneys In boys, the urethra may be very short and split, and as a result it emerges on the top surface of the penis rather than in its usual position at the end of the penis. ureters The split may be small, or, when it occurs in boys born with bladder exstrophy, it may involve the full length of the penis, making the penis short and broad. In girls, the opening of the urethra is higher and wider than usual, the labia bladder (the lip-like folds encircling the vaginal sphincter opening) are further apart than normal urethra and the clitoris (a small, very sensitive part of the female genitalia) is split in two. Sheet 1 of 6 Ref: 2013F0031 © GOSH NHS Foundation Trust February 2014 What features are How are bladder associated with bladder exstrophy and epispadias exstrophy? diagnosed? As well as the bladder being exposed, Bladder exstrophy is sometimes diagnosed babies with bladder exstrophy may also before birth using ultrasound scans. have related problems affecting their However, it is often not picked up urinary system and pelvic bones. These before birth, but will be obvious once related problems vary in severity and your baby is born. Epispadias in boys is do not affect every baby. These will be usually identified at birth, but in girls confirmed using ultrasound scans and the diagnosis is usually made later when x-rays, and may be corrected during a they develop bladder control problems or series of operations. They include: infections. Problems with the neck of the bladder and sphincter (ring of muscle that What causes bladder squeezes and relaxes to let urine flow from the bladder) exstrophy and epispadias? The bladder has a smaller capacity than We do not know why bladder exstrophy usual, so cannot hold much urine occurs. It affects the developing baby during very early stages of development, The ureters join the bladder in a at about four to six weeks into the different place to normal pregnancy. This is when organs, muscles The middle part of the pelvic bones are and other tissues start to form. It is not separated the result of anything either parent did or Bladder exstrophy can be associated did not do, and is not simply an inherited with other problems, but the doctor will condition. examine your child closely to see if this is the case. Some may need to be corrected with an operation, but others do not. For How common is more information about the operations, bladder exstrophy? please see our leaflets. The more common Bladder exstrophy occurs in 1 in every problems include: 40,000 births, affecting two to three The anus is further forward than usual times more boys than girls. If you have a The belly button is lower down than baby with bladder exstrophy the chance usual of having another baby with bladder exstrophy is increased to about 1 in 100. Umbilical and inguinal hernia, where People born with bladder exstrophy part of the abdominal lining and who go on to have children have a 1 sometimes a section of intestine in 70 chance of giving birth to a baby bulges out through a weak area in the with bladder exstrophy. If you have any abdominal wall muscles concerns, we can arrange for you to see a Undescended testes, where the testicles genetic counsellor. are not in their usual place in the scrotum Sheet 2 of 6 Ref: 2013F0031 © GOSH NHS Foundation Trust February 2014 How are bladder drain from the bladder through a number exstrophy and associated of catheters (plastic tubes) placed in the bladder. problems treated? Your child will come back to the ward Babies born with bladder exstrophy and to recover. For the first day or so, they epispadias are admitted to GOSH in the will have an intravenous infusion (drip) first few days after birth by ambulance giving fluids and medications until the from where he or she was born. His or her bladder starts to recover. Your child will abdomen will be covered in ‘cling film’, need to have regular pain relief after which protects the bladder surface, and also the operation. Initially, pain relief will be allows staff to observe the area closely too. given through an epidural. Bladder exstrophy and epispadias are They may also have ureteric stents in corrected in a series of operations over the place, which are thin tubes inserted first few years of life. The overall aim of through the abdomen into your child’s treatment is to prevent any kidney damage bladder and up each ureter. These drain and correct the abnormalities so that your away urine while the bladder recovers child’s urinary system and genitals work from surgery. A urethral stent will also properly and look as normal as possible. have been inserted into the urethra to Your child’s urology surgeon (specialist in keep it open while the area heals. problems affecting the urinary system) will After the first week, some of the tubes explain the treatment plan for your child; will be removed. The drip will be removed this can vary from child to child. Before when your child starts feeding again. The each operation, your child’s surgeon will epidural is usually removed three to five visit you to explain about the operation in days after the operation. Seven days after more detail, discuss any worries you might the operation, the nurses will remove have and ask you to give your permission the ureteric stents if they were inserted. for the operation, by signing a consent This will be done on the ward – we will form. An anaesthetist will also visit you to give your child pain relief beforehand explain about the anaesthetic and pain although it may still be uncomfortable. relief after the operation. You and your child will be able to go home once your child is recovering and has been reviewed by the doctors. What do the operations involve? Around three months later, your child will have a cystoscopy to check how Bladder and abdominal wall repair the bladder is healing. Please see our operation – first few days after birth Cystoscopy information sheet for further This operation closes the bladder and details. abdominal wall, so that that the bladder is inside the body and in the correct position. After the operation, urine will Sheet 3 of 6 Ref: 2013F0031 © GOSH NHS Foundation Trust February 2014 Kelly procedure – at one Are there any risks to two years old with these operations? After the initial closure of the bladder All surgery carries a small risk of bleeding exstrophy, there is no sphincter at the during or after the operation. Every junction of the bladder and urethra. The anaesthetic carries a risk of complications, Kelly procedure (also called a soft tissue but this is small. There is a small risk of reconstruction of the bladder neck) uses infection, but your child may be given existing muscle and soft tissue to create antibiotics as a precaution. a ring of muscle that acts like a sphincter. After the first operation to repair the This holds urine in the bladder allowing it bladder and abdominal wall, there is a risk to stretch and gain more capacity and also that the wound will not heal properly and helps form a strong stream of urine when open up again. This can cause the bladder weeing. to move out of position. This happens The surgeon can create a tummy button more often if the area to be repaired is (omphaloplasty) during the same large, as the skin needs to stretch to cover operation if preferred. it. If the wound opens up again, your child will need another operation to repair the In boys, the Kelly procedure may also bladder and abdominal wall. The surgeons involve a reconstruction of the urethra may correct the pelvic bones during this and penis or it may be done in a separate operation as well. operation at a later stage. There is also a risk of kidney damage During the same operation, the ureters in children with bladder exstrophy. The may be re-positioned within the bladder abnormal join between the ureters and if they are not joining the bladder bladder allows urine to flow backwards in the correct place. This can cause a towards the kidneys.