Bladder exstrophy is a congenital condition where varying degrees of abdominal wall defects are observed in the midline of the lower abdominal wall at birth, causing the bladder to protrude outward. In severe cases, the entire bladder is everted, exposing both ureteral orifices, with a defect in the pubic symphysis and severe epispadias formation. Approximately one case of bladder exstrophy is found in every 1,000 pediatric autopsies, but clinical statistics report about one case per 50,000 live births, indicating that most affected individuals die shortly after birth. The condition is more common in males than females, with a ratio of approximately 8:1.

bubble_chart Pathogen

From an embryological perspective, it is generally believed that the failure of mesoderm development in the lower abdominal region, partial defect of the abdominal wall, and anterior displacement of the cloaca lead to bladder exstrophy. (Figure 1, 2)

Figure 1 Bladder exstrophy and epispadias

Patient Xing ××, male, 7 years old, hospital number 79694

Figure 2 Bladder exstrophy (schematic diagram)

bubble_chart Clinical Manifestations

Partial defect of the lower abdominal wall, with the bladder mucosa everted and appearing bright red. In severe cases, the ureteral orifices on both sides are exposed, and urine can be seen intermittently spurting from them. In even more severe cases, there is a defect in the pubic symphysis, severe epispadias, and penile deformity. Due to constant urine soaking of the pants, the surrounding skin becomes eroded with a strong odor of urine. Patients often have other congenital malformations, such as spina bifida or chapped lips. Generally speaking, patients are highly prone to upper urinary tract infections. However, in reality, there are cases of severe bladder exstrophy where the patient lived nearly 30 years before seeking medical attention, and upon examination, their growth, development, and daily life were found to be in good condition.

The diagnosis of this disease can be made at a glance without special examinations. However, since it is often accompanied by other congenital malformations, a thorough and detailed examination of the whole body and various systems is necessary.

bubble_chart Treatment Measures

For mild cases, where only a small portion of the bladder mucosa is everted and the abdominal wall defect is minimal, local repair methods can be successful. For more severe cases, local repairs often fail, typically after the age of ten. Ureteral-rectal transplantation and sigmoid colostomy yield more favorable outcomes.

bubble_chart Complications

Spina bifida; chapped lips