Short Case ReDort
Unilateral Multicystic Kidney and Contralateral Hydronephrosis in the Newborn In 1936 Schwartz described for the first time an unusual unilateral multicystic kidney in a male infant, aged 4 months. Since then this condition has been described in several other reports. It was thought that the opposite kidney was always normal. Innes Williams (1958), however, found in one of his cases a partial atresia of the opposite ureter which had caused severe hydronephrosis. Recently we saw a newborn with a unilateral multicystic kidney and a severe hydronephrosis of the opposite ureter, which closely resembled the patient mentioned by Innes Williams. Case Report A 12-year-old girl was sent to our hospital because of anuria, vomiting and a palpable mass in the right loin. During the first week after birth there were no feeding problems and apparently there was a normal urine output. On palpation there were extensive nodular masses in the flanks. Blood urea and creatinine were considerably raised (urea 251 mg%, creatinine 6.5 mg%) and there was also a severe acidosis (pH 7.20, Base Excess-1 6). There was pronounced oliguria. Because of the impairment of renal function with anuria, intravenous pyelography could not be done. Infusions with glucose 5 % and sodium bicarbonate 4.2% were started, and cystography was performed. There was no vesico-ureteric reflux and therefore retrograde pyelography was indicated. On monitoring we saw bilateral slender ureters but visualisation of the pyela was impossible. The right ureter was displaced towards the midline. Because of a temporary fault in the apparatus X-rays could not be taken. Next the mass in the right flank was punctured and 10 ml clear yellow fluid was obtained and replaced by contrast medium. On monitoring, a cyst was seen. Further several cysts were punctured and emptied. The left kidney was not punctured as we assumed that there were bilateral multicystic kidneys in which case the prognosis was very poor. Afterwards there was a temporary production of urine but the acidosis remained unchanged and the child died after four weeks. At autopsy the right kidney showed cystic enlargement and measured 1 0 x 5 ~ 4cm. There was no communication between the several cysts, which measured up to 5 cm in diameter. The distal part of the right ureter was normal, but the proximal part was atretic. A normal cortex and medulla were not seen
The left kidney measured 6 x 3 x 2 cm, and the surface was smooth with some subcapsular cysts up to 0.3 cm. Post-mortem pyelography showed that the pelvis was dilated and there was a stenosis at the pyelo-ureteral junction. The rest of this ureter was normal. On cut surface the cortex, medulla and the number of calices were normal. There was no right or left sided uretero-vesical stenosis.
Comment In view of the autopsy findings, the child could perhaps have been saved by a temporary pyelocutaneostomy. It was an error to assume that the left kidney was also a multicystic kidney. In the event of such an unusual case of a multicystic kidney, it is imperative to examine the opposite kidney carefully in case there should be a possibility to treat the associated anomaly. R. J. SCHOLTMEIJER and J. J. VAN DER HARTEN, Departments of Urology and Pathology, Academic Hospital of the Free University, Anisterdant, The Netherlands
References SCHWARTZ, J. 11936). An unusual unilateral multicystic kidney in an infant. Journal of Urology, 35, 259-263. WILLIAMS, D. I. (1958). Urology in childhood (from Encyclopedia of Urology). Berlin: Springer Verlag. 176
Unilateral Multicystic Kidney and Contralateral Hydronephrosis in the Newborn In 1936 Schwartz described for the first time an unusual unilateral multicystic kidney in a male infant, aged 4 months. Since then this condition has been described in several other reports. It was thought that the opposite kidney was always normal. Innes Williams (1958), however, found in one of his cases a partial atresia of the opposite ureter which had caused severe hydronephrosis. Recently we saw a newborn with a unilateral multicystic kidney and a severe hydronephrosis of the opposite ureter, which closely resembled the patient mentioned by Innes Williams. Case Report A 12-year-old girl was sent to our hospital because of anuria, vomiting and a palpable mass in the right loin. During the first week after birth there were no feeding problems and apparently there was a normal urine output. On palpation there were extensive nodular masses in the flanks. Blood urea and creatinine were considerably raised (urea 251 mg%, creatinine 6.5 mg%) and there was also a severe acidosis (pH 7.20, Base Excess-1 6). There was pronounced oliguria. Because of the impairment of renal function with anuria, intravenous pyelography could not be done. Infusions with glucose 5 % and sodium bicarbonate 4.2% were started, and cystography was performed. There was no vesico-ureteric reflux and therefore retrograde pyelography was indicated. On monitoring we saw bilateral slender ureters but visualisation of the pyela was impossible. The right ureter was displaced towards the midline. Because of a temporary fault in the apparatus X-rays could not be taken. Next the mass in the right flank was punctured and 10 ml clear yellow fluid was obtained and replaced by contrast medium. On monitoring, a cyst was seen. Further several cysts were punctured and emptied. The left kidney was not punctured as we assumed that there were bilateral multicystic kidneys in which case the prognosis was very poor. Afterwards there was a temporary production of urine but the acidosis remained unchanged and the child died after four weeks. At autopsy the right kidney showed cystic enlargement and measured 1 0 x 5 ~ 4cm. There was no communication between the several cysts, which measured up to 5 cm in diameter. The distal part of the right ureter was normal, but the proximal part was atretic. A normal cortex and medulla were not seen
The left kidney measured 6 x 3 x 2 cm, and the surface was smooth with some subcapsular cysts up to 0.3 cm. Post-mortem pyelography showed that the pelvis was dilated and there was a stenosis at the pyelo-ureteral junction. The rest of this ureter was normal. On cut surface the cortex, medulla and the number of calices were normal. There was no right or left sided uretero-vesical stenosis.
Comment In view of the autopsy findings, the child could perhaps have been saved by a temporary pyelocutaneostomy. It was an error to assume that the left kidney was also a multicystic kidney. In the event of such an unusual case of a multicystic kidney, it is imperative to examine the opposite kidney carefully in case there should be a possibility to treat the associated anomaly. R. J. SCHOLTMEIJER and J. J. VAN DER HARTEN, Departments of Urology and Pathology, Academic Hospital of the Free University, Anisterdant, The Netherlands
References SCHWARTZ, J. 11936). An unusual unilateral multicystic kidney in an infant. Journal of Urology, 35, 259-263. WILLIAMS, D. I. (1958). Urology in childhood (from Encyclopedia of Urology). Berlin: Springer Verlag. 176
