- Rare malformation occurring
in 1:25,000-40,000 births (1).
- Usually sporadic with a male
to female ratio of 2:1 (1).
- Pathogenesis.
- Ventral defect in the infraumbilical
abdominal wall.
- Absent fetal bladder in its
normal position within the pelvis (2).
- Normal kidneys.
- Normal amniotic fluid volume.
- The urinary bladder is
exposed and opens anteriorly (the mucosal surface of the bladder is
everted through the abdominal defect, with the bladder margins continuous
with the abdominal wall resulting in a mucocutaneous junction) (3).
- Size of the exstrophied
bladder is variable.
- Widening of the symphysis
pubis (the degree of separation is proportional to the severity of the
epispadias-exstrophy malformation) (4).
- Umbilicus is low set (caudal
insertion) and may be involved in the area of extrophy.
- In males there is complete
epispadias.
- In females the corresponding
findings are bifid clitoris and wide labial separation.
- Engel RME. Exstrophy of the bladder
and associated anomalies. Birth Defects 1974;10:146.
- Barth RA, Filly RA,
Sondheimer FK. Prenatal sonographic findings in bladder exstrophy. J
Ultrasound Med 1990;9:359-361.
- Patten BM, Barry A. The
genesis of exstrophy of the bladder and epispadias. Am J Anat 1952;90:35.
- White P, Lebowitz RL.
Exstrophy of the bladder. Radiol Clin North Am 1977;15:93.