Exstrophy

Exstrophy is a rare birth defect of the abdominal wall. The skin, muscles and other parts of the abdominal wall do not form properly and are turned inside out. This condition is also known as bladder exstrophy when it involves the bladder.

Overview

Exstrophy is a complex condition that affects several body systems. It is part of a spectrum of birth defects called the exstrophy-epispadias complex. In addition to the bladder, the intestines and reproductive organs may also be affected. The severity of the condition can vary widely among individuals.

Causes

The exact cause of exstrophy is unknown. It is thought to occur as a result of a disruption in the development of the abdominal wall and bladder early in pregnancy. This disruption causes the bladder to form outside the body and the abdominal wall to be turned inside out.

Symptoms

The most obvious symptom of exstrophy is the presence of the bladder on the outside of the body. Other symptoms can include a wide set pubic bone, a small penis or clitoris, and a short, small vagina. Some individuals with exstrophy may also have an omphalocele, a birth defect in which the intestines or other abdominal organs protrude through a hole in the belly button.

Diagnosis

Exstrophy can often be diagnosed before birth through a routine ultrasound. After birth, the condition is usually obvious due to the presence of the bladder on the outside of the body.

Treatment

Treatment for exstrophy typically involves surgery to repair the bladder and abdominal wall. The goal of treatment is to create a functioning bladder and to improve the appearance of the genitalia. Some individuals with exstrophy may require additional surgeries or other treatments later in life.

Prognosis

The prognosis for individuals with exstrophy is generally good. With proper treatment, most individuals with this condition can lead normal, healthy lives. However, they may have an increased risk of bladder and kidney problems, as well as fertility issues.

See also

• Birth defect
• Bladder
• Abdominal wall
• Omphalocele

Exstrophy Resources
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