Bladder exstrophy

Bladder Exstrophy

Bladder exstrophy is a rare congenital anomaly that involves the protrusion of the urinary bladder through a defect in the lower abdominal wall. This condition is part of the exstrophy-epispadias complex, which includes a spectrum of anomalies affecting the urinary tract, genitalia, and pelvic structures.

Pathophysiology

Bladder exstrophy occurs due to a failure in the closure of the infraumbilical midline structures during embryonic development. This results in the bladder being exposed outside the body, with the inner surface of the bladder visible externally. The condition is often associated with other anomalies such as epispadias, where the urethra does not develop properly, and a separation of the pubic symphysis.

Epidemiology

Bladder exstrophy is a rare condition, with an estimated incidence of 1 in 30,000 to 50,000 live births. It is more common in males than females, with a male-to-female ratio of approximately 2:1.

Clinical Presentation

Infants with bladder exstrophy present with a visible defect in the lower abdominal wall, where the bladder mucosa is exposed. The umbilicus is typically low-set, and the genitalia may be malformed. In males, the penis is often short and broad with epispadias, while females may have a bifid clitoris and a short, wide vagina.

Diagnosis

Diagnosis of bladder exstrophy is usually made at birth based on the characteristic physical findings. Prenatal ultrasound may suggest the diagnosis if there is an absence of a visible bladder and other associated findings such as a low-set umbilicus and abnormal genitalia.

Management

The management of bladder exstrophy involves surgical correction, which is typically performed in stages. The initial surgery aims to close the bladder and abdominal wall, and subsequent procedures may address the reconstruction of the genitalia and urinary continence. Multidisciplinary care involving pediatric urologists, surgeons, and other specialists is essential for optimal outcomes.

Prognosis

With advances in surgical techniques, the prognosis for individuals with bladder exstrophy has improved significantly. Many patients achieve urinary continence and have normal renal function. However, long-term follow-up is necessary to monitor for complications such as urinary tract infections and renal impairment.

Also see

• Epispadias
• Congenital anomalies
• Pediatric urology
• Urinary tract reconstruction