Are there any related issues?
A baby with exstrophy of the bladder will usually also have all, or some, of these other associated abnormalities:
- Epispadias: In boys, the urethra (the tube through which urine is funneled out of the body from the bladder) has not formed completely, and it may be extremely short and split. Because of this, it opens on the upper surface rather than on the end of the penis. The split may be small or involve the entire length of the penis. In the later case, the penis will be flat and short looking like a small shovel. In girls, the urethral opening is located between a split clitoris and labia minora.
- Dorsal chordee: This means that the penis is pulled up against the abdomen with a slight downward curve. This is caused by a shorter than normal tendon in the upper part of the penis.
- Absence of a bladder neck and sphincter: The bladder neck is the funnel shaped lower portion of the bladder which contains the ring of muscles that open and close the bladder outlet. It is this muscle ring (sphincter) which makes it possible to achieve bladder control or continence. When it is absent there is no ability for bladder control.
- Small bladder capacity: This means that the bladder is unable to hold a normal quantity of urine.
- Abnormally positioned ureters: Ureters (the tubes which channel urine from the kidneys to the bladder) enter the bladder abnormally resulting in urine backing up toward the kidneys (reflux).
- Separation (diastasis) of the pubic bones: The large bones which make up the pelvic cavity normally develop as a ring. The pelvis supports the bladder, the reproductive and the lower intestinal organs. The pubic bones normally join to form the front portion of the pelvis and to protect the bladder, the urethra, and the muscles of the abdominal wall. In bladder exstrophy the pubic bones have failed to join, leaving a wide opening. This open pelvic ring also results in the hips being turned outward (rotated). This causes the child with exstrophy to “toe-out”, or to have their feet point away from one another when they stand or walk. However, children with exstrophy should be able to run, jump, and play normally despite the pelvic deformity, and there is no evidence that exstrophy results in arthritis of the hips in later years.
- An anus positioned further forward than usual: The opening of the rectum is positioned more anterior (closer to the scrotum /vagina). This positioning is typically not accompanied by problems with bowel function.
- A low positioned belly button (umbilicus): The umbilicus is not usually visible and is removed when the bladder is surgically closed.