OBJECTIVES: Megacalycosis or Puigvert's disease is a clinical entity characterized by dilatation of all renal calyces without dilatation of the renal pelvis or ureter in the absence of any obstructive factor. To date, its pathogenesis remains unclear. The rarity of this disease and the importance of making a correct differential diagnosis prompted us to report on the present case. METHODS/ RESULTS: Two patients with urolithiasis associated with ipsilateral megacalyces who were successfully treated by ESWL are described. The clinical, diagnostic and therapeutic aspects reported in the literature are briefly reviewed. CONCLUSIONS: There is a greater lithogenic predisposition in these patients. Intravenous urography has proved to be very accurate in making the differential diagnosis from other disorders such as obstructive uropathy and intermittent hydronephrosis. The efficacy of ESWL in the treatment of lithiasis associated with megacalycosis is underscored. A yearly control follow up by analyses and intravenous urography is advocated.
OBJECTIVES:Megacalycosis or Puigvert's disease is a clinical entity characterized by dilatation of all renal calyces without dilatation of the renal pelvis or ureter in the absence of any obstructive factor. To date, its pathogenesis remains unclear. The rarity of this disease and the importance of making a correct differential diagnosis prompted us to report on the present case. METHODS/ RESULTS: Two patients with urolithiasis associated with ipsilateral megacalyces who were successfully treated by ESWL are described. The clinical, diagnostic and therapeutic aspects reported in the literature are briefly reviewed. CONCLUSIONS: There is a greater lithogenic predisposition in these patients. Intravenous urography has proved to be very accurate in making the differential diagnosis from other disorders such as obstructive uropathy and intermittent hydronephrosis. The efficacy of ESWL in the treatment of lithiasis associated with megacalycosis is underscored. A yearly control follow up by analyses and intravenous urography is advocated.