ABSTRACT
Unilateral renal cystic disease (URCD) of kidney is a non-familial, extremely rare condition, characterized by replacement of the renal parenchyma of one kidney by a cluster of multiple cysts of varying size with a normal contralateral kidney. It is morphologically indistinguishable from autosomal dominant polycystic kidney disease (ADPKD); as such, hepatic and pancreatic cysts is not seen and shows no progressive deterioration in renal function; thus, differentiating ADPKD from URCD becomes important. We report a case of URCD documented by clinical and radiological imaging. A 21 year-old female, presented with history of mild lancinating pain in the left flank for 6 years which aggravated in the past 3 days, with no history of lower urinary tract symptoms. No significant family illnesses reported. Examination showed normal vitals and ballotability present and associated tenderness on deep palpation in left lumbar region. Laboratory findings were within normal limits. Ultrasonography of abdomen and pelvis showed left hydronephrosis with multiple cysts. CECT Abdomen revealed an enlarged left kidney (∼15×16×10 cm) filled with variable sized round, well-marginated multiple cysts. Renal ultrasound was performed on patient’s parents and her siblings and ruled out cystic renal disease. Hence, authors considered the diagnosis of URCD in this patient. In conclusion, treatment and managing guidelines of URCD have not been mentioned in any of the medical literatures. There is little information regarding the progression of URCD. Hence there is a need for further understanding of pathogenesis, progression and management of these patients.
ABSTRACT
Unilateral renal cystic disease (URCD) is a multicystic disease, characterized by varying sized cysts localized in a diffusely enlarged kidney without forming a distinct encapsulated mass. Except for its unilaterality, gross and histological findings of URCD are indistinguishable from those of autosomal dominant polycystic kidney disease (ADPKD). However, unlike ADPKD, URCD patients show neither a genetic background nor progressive deterioration in renal function; thus, the differential diagnosis of URCD from ADPKD is important, especially from the genetic and prognostic standpoint. Usually, URCD patients have no cardiac valvular abnormalities and no cystic diseases in other intraperitoneal organ, except simple cyst. Intracranial aneurysm can be found in 5-10% of ADPKD patients, but not in previous reported fourteen URCD cases yet. We report a 41- year-old patient with URCD and cerebral artery aneurysm. Careful family history taking and ultrasonographic examination of his parents and siblings revealed that they have no renal cystic disease. We followed the patient for 13 months and his renal function did not deteriorate.