Additional pediatric resources: GeneralPediatrics.com | PediatricEducation.org | SearchingPediatrics.com
Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
Etiology/Pathophysiology:
Etiology is unknown.Twenty percent are still born or die as neonates,
and an additional 50% die in the first 2 years of life. Other
associated anomalies include hip dislocations / club foot / limb
deformities (40% ), cardiac defects(30% ), and malrotation (30% ).
Pathology:
Aplasia of the muscles of the lower medial abdominal wall. The
kidneys have a decreased number of functioning nephrons with variable
cystic dysplasia. The dilated ureters and bladder have a patchy
absence of smooth muscle.
Imaging Findings:
The ureters and bladder are dilated. Urethral abnormalities that can
be seen include posterior urethral dilation, diverticula, stenosis /
atresia, and megaurethra. They can have umbilical-bladder attachment
or a patent urachus.
DDX:
References:
See References Chapter.
Additional pediatric resources: GeneralPediatrics.com | PediatricEducation.org | SearchingPediatrics.com
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