CONGENITAL megaloureter is a disease of infants and children. Most of these patients die before 20 years of age. The cause of the disease remains obscure. There is no conclusive evidence that infection and chronic inflammation may actually cause or predispose toward its development. It is difficult also to explain the causal factor responsible for the extreme ureteral dilation and hydronephrosis in this condition on a nonobstructive basis; yet no obstruction is demonstrable at the ureterovesical juncture or bladder neck. If obstructive uropathy is demonstrated, the ureteral dilation is usually termed secondary, and the case is not classified as primary or congenital megaloureter. To date, the most reasonable causal factor proposes a faulty neuromuscular mechanism producing ureteral atony, frequently in association with dilation of the bladder. A comparison has been made between congenital megaloureter and Hirschsprung's disease. There is, in fact, probably more than chance association of the high incidence
ROGER BAKER. URETERAL ELECTROMYOGRAPHY IN CONGENITAL MEGALOURETER. AMA Am J Dis Child. 1954;87(1):7–15. doi:10.1001/archpedi.1954.02050090007002
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