THE SO-CALLED MEGAURETER-MEGACYSTIS SYNDROME: TWO CASE REPORTS

Abstract

The megaureter-megacystis syndrome was first described by Williams in 1954 in relation to infants who had very large bladders and grossly dilated ureters with free reflux.
We have encountered 2 cases that fit the description of the megaureter-megacystis syndrome. Case 1 was a 6-year-old boy and case 2 was a 7-year-old boy. They visited our clinic with the same chief complaint of high fever. The urinary examinations revealed pyuria in both cases especially manifest in the second case, and they were admitted for treatments and further examinations.
In each case IVP and DIP findings showed dilated calyces, pelves and ureters, and cystogram demonstrated a large bladder, grossly bilateral dilated ureters with free vesicoureteral refluxes. Voiding cystourethrograms showed normal urethras and complete emptying of the bladders in two cases. By cysstoscopy it was found that the trigone was wide and trabeculation was not present in each case. Cystometric examinations found no definite abnormalities except the large capacities in both cases. (about 900ml in case 1, (450ml in case 2.) Their total renal functions were greatly decreased similarly in two cases.
We performed preliminary nephrostomies in both cases and then the antireflux operation using the Paquin procedure in case 1. We would treat radically after the improvement of the renal function in case 2.
Some discussions were made on the clinical entities of the megaureter-megacystis syndrome, the megacystis syndrome and primary reflux.