Abstract
From October 1981 to September 1992, seven patients with Beckwith-Wiedemann syndrome (BWS) were treated in our hospital. Among the seven patients, five (71%) had urological abnormalities; urethral stenosis with bilateral vesicoureteral reflux (VUR), bilateral ectopic ureter, cystinuria with bladder stone, bilateral hydronephrosis and unilateral abdominal testis. The co-existances of cystinuria and ectopic ureter with BWS has not been reported in literature. Four patients recieved urological interventions. The patient with urethral stenosis was treated with optic internal urethrotomy, and bilateral VUR disappeared. The patient with bilateral ectopic ureter was tredted by left percutaneous nephrostomy followed by bilateral uretero-cysto neostomy. Now she is continent, but suffering from chronic renal failure. The patient with cystinuria was treated by transurethral cystolithotripsy and urinary alkalization. No recurrence of urolithiasis is observed. Long-loop vas orchiopexy was performed for abdominal testis. High incidence of urological abnormalities in cases of BWS was documented.
