Exstrophy-Epispadias Complex (EEC) referred as a spectrum of genitourinary malformations ranges in severity from epispadias (E) and classical bladder exstrophy (CEB) to the exstrophy of the cloaca (EC) as the most severe form. Based on the severity, the urinary system, the musculoskeletal system, the pelvis, the pelvic floor, the abdominal wall, the genitalia and sometimes the spine and the anus may involve in EEC.
EEC is characterized by a noticeable defect of the lower abdominal wall, both with an evaginated bladder plate (CEB) or with open urethral plate in males and a cleft in females (E). Urine drips from the ureteric orifices, visible on bladder surface or per urethram it may be visible. In the exceptional cases of CE spinal defects, 2 exstrophied hemibladders, imperforate anus, as well as omphalocele can be seen after birth. Some different forms of the EEC (pseudo-exstrophy, duplicated exstrophy and covered exstrophy) have also been described. EEC may also results from enlargement of the cloacal membrane or mechanical disruption, which prevent the invasion of mesodermal cells all along with the infraumbilical midline and thus results in exstrophy. The timing of the rupture will determine the severity of the malformation.
Appearance of Urinary incontinence is the major clinical symptom.
The main cause of EEC is unknown but in rare cases of familial occurrence is been reported and both environmental and genetic factors will play a main role in the etiology of EEC.
Diagnosis of EEC at the time of birth is made on the base of the clinical presentation but EEC may be predicted prenatally by careful ultrasound examinations with the index result of repeated non-visualization of a usually filled fetal bladder.
Wide counseling must be provided to parents but due to a commonly favorable outcome with suitable surgical management, termination of the pregnancy must no longer be systematically recommended.