A case of bilateral multicysitic dysplastic kidneys

Abstract

 Bilateral multicystic dysplastic kidney (MCDK) can cause Potter's sequence and most of them are fatal in utero. Here, we report a rare case of bilateral MCDK who remains alive and well 14 months later.
 Her mother had been diagnosed as diabetes mellitus and took insulin treatment, however her level of fasting plasma glucose was not controlled. Antenatal sonography at 18 week gestation confirmed that both kidneys contained several cysts of varying sizes. Liquor volume was normal. However, the liquor volume decreased at 33 week gestation, and the baby was born by caesarean delivery. Urine and blood examinations, including serum creatinine levels, remained within the normal range.
 Postnatal sonography and enhanced-MRI revealed that the right kidney had the typical appearances of MCDK with multiple non-communicating cysts. Similarly, the left kidney comprised several cysts of varying sizes, however renal parenchyma was observed in the left. A static scintigraphy with 99 mtechnetium-dimercaptosuccinic acid (DMSA) scan showed the preserved function within the lower moiety of the left kidney as well as a non-functioning right kidney. Therefore, the left kidney was diagnosed as segmental MCDK.
 Her renal function became deteriolated, so peritoneal dialysis was commenced at 4 months of age. She continues to thrive and is doing well at 14 months old. Her long-term survival appears to be caused by the preserved left renal function. Because her mother had diabetes mellitus, it is possible that the occurrence of MCDK might be associated with the sustained exposure to hyperglycemia in uterine. In addition, it might be caused by genetic disorder, for example, the TCF-2 gene mutation which is a cause of MODY type 5.