Robert L. Lebowitz, M.D.
Children' s Hospital, Harvard Medical School
Boston, Massachusetts


FRALEY SYNDROME

This patient presented at 13 years of age with right-sided pain and hematuria. An IVP showed stones in markedly diluted upper calyces of the right single system kidney (fig 1 & 2). A retrograde ureterogram with a drainage film confirmed retention from obstruction due to narrowing of the upper infundibulum (fig 3 & 4). At surgery, the upper infundibulum was "sandwiched" between the artery and vein, and she underwent right upper pole partial nephrectomy. Fraley, in a 1966 paper in the New England Journal of Medicine entitled "Vascular Obstruction of the Superior Infundibulum Causing Nephralgia", described the condition that bears his name. It is comon to see dilation of the upper calyces of a child's kidney, but unusual to have this be the cause of signs or symptoms. In the Journal of the Canadian Association of Radiologists, Vol. 19, Dec. 1968, Rusiewicz and Reilly reviewed 2000 IVPs performed in children over a 5-year period. Fifty-five had dilated upper calyces, 42 moderate, and 15 severe. Only one child had nephralgia.

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