The exstrophy of the urinary bladder is a complex malformation, it includes a disordered closure of the anterior wall of the bladder, muscles and skin of the abdominal wall and the symphysis (pubic bone); the child is born with an open bladder. It is always associated with malformations of the external genitalia; in boys epispadias, i.e. the urethra, which is fully split (open) at its entire length, is seen on the dorsal side of the short and dorsally curved penis. In girls the clitoris is divided and the labia majora are dislocated to a lateral direction.
I./4.4.2.: Symptoms
The urine of an infant with an exstrophy of the urinary bladder is leaking from the open bladder, through the ureteral orifices opening to the outside world, and a permanent inflammation of the bladder is present. The associated epispadias will cause complaints usually only later; after the infancy it may be a problem that the child is unable to or can only difficultly control urination (although the majority is completely unable to retain urine, i.e. incontinent); in puberty and adult age, in addition to the cosmetic problems, the curved penis and open urethra cause difficulties in the processes of coitus and generation of children, respectively.
I./4.4.3.: Diagnostics
The diagnosis is usually based on a thorough physical examination of the infant, during which the extent of the above mentioned abnormities and their potential consequences should also be considered. The examination of the upper urinary tract with US or possibly another imaging procedure (e.g. intravenous urography) is necessary in order to assess any possible other anomalies (see the previous part). Because of the defective closure of symphysis and other pelvic anomalies, it is helpful to make an X-ray picture as well.
I./4.4.4.: Differential diagnosis
Differentiating exstrophy from other pathologic states is usually no problem.
I./4.4.5.: Therapy
Its treatment is surgical: in order to avoid severe infections and secondary damage, an attempt to reconstruct the integrity of the bladder’s anterior wall, neck and the open urethra should be made as early as possible, already in the first few days of life. Malformation of all tissues of the anterior abdominal wall poses a problem, as after plastic surgery the abdominal wall often opens again, or fistulas develop. Therefore the reconstruction is usually made in several steps; reoperations are often necessary.
