Bladder exstrophy (Ectopia vesicae)

Bladder exstrophy (Ectopia vesicae) is a rare midline congenital anomaly that exists as a part of larger spectrum of the exstrophy-epispadias complex and involves protrusion of the open urinary bladder through a defect in the lower abdominal wall. Its presentation is variable, often including abnormalities of the bony pelvis, pelvic floor, and genitalia. The underlying embryologic mechanism leading to bladder exstrophy is unknown, though it is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm.[1]

Incidence

Occurring at a rate between 1 in 10,000 to 1 in 50,000 [2] with a male-to-female ratio of 2.3-4:1.

Clinical presentation

In general children born with bladder exstrophy are full term, with everted posterior bladder wall seen in the midline of the lower abdomen. Mucosa of the exposed bladder is typically smooth and pink. Umbilical cord exists from the superior-most border of the bladder plate along with small umbilical hernia. There is significant widening of the pubic symphysis and anus is anteriorly placed. There may be association of inguinal hernia upto 82% in boys and upto 10% in females. Females frequently have a displaced and narrowed vaginal orifice, a bifid clitoris, and divergent labia

Prenatal diagnosis

Ultrasonography:

• Inability to visualize the bladder on ultrasound
• A lower abdominal bulge
• A small penis with anteriorly displaced scrotum
• A low set umbilical insertion
• Abnormal widening of the iliac crests

Initial management at birth

Upon delivery, umbilical cord is ligated with silk suture than applying traditional plastic clip.The exposed bladder is irrigated and a non-adherent film is placed to prevent as much contact with the external environment as possible. In the event the child was not born at a medical center with an appropriate exstrophy support team then transfer to teritiary care center should be done as early as possible.

Treatment

Modern therapy is aimed at surgical reconstruction of the bladder and genitalia. Three stage approach is followed.
Stage-1: Functional closure.Objective to place bladder and prostate deep within pelvis to achieve a more normal position.It often requiring a pelvic osteotomy. This leaves the patient with penile epispadias and urinary incontinence.
Stage-2: Epispadias repair. At approximately 6-12 months of age the patient undergoes repair of the epispadias after testosterone stimulation.
Stage-3: Bladder neck reconstruction and ureteroneocystostomy. Bladder neck repair usually occurs around the age of 4–5 years, though this is dependent upon a bladder with adequate capacity and, most importantly, an indication that the child is interested in becoming continent.

Prognosis:

The most important criterion for improving long-term prognosis is success of the initial closure . If a patient requires more than one closure their chance of continence drops off precipitously with each additional closure – at just two closures the chance of voiding continence is just 17%.s