Radiology Resident Case of the Week
Etiology/Pathophysiology
Defeciency in development of the lower abdominal wall musculature. The bladder is open and the mucosa of the bladder is continous with the skin. occurs in 500,000 births. males are affected twice as often as females. It is also associated with entire epispadias. In males the penis is short and deformed. a bifid scrotum and unilateral cryptorchidism may also be associated. Omphalocele, vertebral anomalies and myelomenigoceles have also been noted.
In females the urethra is short and buried in the exstrophy. the labias may be widely separed with a bifid clitoris. upper urinary tract anomalie are uncommon.
Pathology:
Miscellaneous
Imaging
On plain films 60% of cases show separated symphysis with extrophy-epispadic anomaly. 25% are associated with epispadic anomalies alone. Radiologic evaluation is usually done postoperatively to exclude adencarcinoma at the anastomosis site years after ureterosigmoidostomy.
Ultrasound, nuclear cystography and IVU are helpful tools .
DDX
Bladder Extrophy, Cleidocranial dysostosis
Key references
N. R. Dunnick, R.W. McCallum, C.M. Sandler. Textbook Of Uroradiology. 1991, pge.30 Williams & Wilkins . Baltimore.
S.N. Siverman, J.P. Kuhn. Caffey's Essentials of Pediatric X-ray Diagnosis. 1990, pge. 704-705. Yearbook Medical Publisher, Inc.
ACR Code
838.1464
Keywords:
Exstrophy