Renal relevant radiology: radiologic imaging in autosomal dominant polycystic kidney disease

Abstract

Autosomal-dominant polycystic kidney disease is a systemic disorder and the most common hereditary renal disease, which is characterized by cyst growth, progressive renal enlargement, and development of renal failure. The cystic nature of autosomal dominant polycystic kidney disease and its renal and extrarenal complications (kidney stones, cyst hemorrhage, intracerebral aneurysm, liver cysts, cardiac valve abnormalities, etc.) give radiologic imaging studies a central role in the management of these patients. This article reviews the indications, comparative use, and limitation of various imaging modalities (ultrasonography, magnetic resonance imaging, computerized tomography scan, Positron emission tomography scan, and renal scintigraphy) for the diagnosis and management of complications in autosomal dominant polycystic kidney disease. Finally, this work provides evidence for the value of total kidney volume to predict disease progression in autosomal dominant polycystic kidney disease.

Figure 1.

Ultrasonography and magnetic resonance imaging (MRI) imaging of patients with autosomal dominant polycystic kidney disease (ADPKD) compared with bilateral simple acquired cysts. (A) Longitudinal ultrasonographic view of the right kidney showing multiple dark hypoechoic lesions throughout the kidney with kidney enlargement, consistent with ADPKD. (B) Coronal T2-weighted MRI images show multiple tiny T2 high signal lesions in both kidneys with well preserved renal parenchyma in a patient with bilateral acquired cysts (arrows). (C) Coronal T2-weighted and (D) coronal unenhanced three-dimensional T1-weighted gradient echo MRIs of a patient with ADPKD and conserved renal function show multiple cystic lesions. Lesions with high T2 signal and low T1 signal represent simple cysts (arrow), whereas hemorrhagic cysts show low T2 signal and high T1 signal (star). Preserved renal parenchyma is seen between the cysts (double arrow).

Figure 2.

Correlation between kidney length and body height in adults. Solid line, ANOVA; dotted line, 95% confidence limits. Reprinted from reference , with permission.

Figure 3.

Calcifications in autosomal dominant polycystic kidneys. A shows a noncontrast computerized tomography scan of a patient with autosomal dominant polycystic kidney disease and cyst wall calcifications (arrow). B represents the noncontrast computerized tomography scan of a 27-year-old man with autosomal dominant polycystic kidney disease and multiple calcified stones in the right kidney (arrows).

Figure 4.

Complex cyst in a patient with autosomal dominant polycystic kidney disease. (A) Axial T2-weighted and axial (B) unenhanced and (C) enhanced three-dimensional T1-weighted gradient echo magnetic resonance images depict a mass in the lower pole of the left kidney with (A, arrow) heterogeneous T2 signal intensity and (B, arrow) T1 high signal. After administration of Gadobenate Dimeglumine, there is high signal on (C, arrow) an enhanced image similar to the (B) unenhanced T1-weighted image. The absence of enhancement on the (D, arrow) subtraction image supports the diagnosis of complex hemorrhagic cyst and rules out a tumor.

Figure 5.

Mercaptoacetyltriglycerine-3 (MAG-3) renal scintigraphy without and with injection of furosemide in a 33-year-old patient with autosomal dominant polycystic kidney disease who presented with massive gross hematuria and acute renal failure. A, top, represents the perfusion phase, and the bottom images show the excretion phase before injection of furosemide. (B) The postfurosemide images show asymmetric excretion and a higher intensity signal on the right side. The times to peak height of the cortical renogram curves are 2.72 for the left kidney and 16 for the right kidney. The cortical 20 minutes to maximum ratios are 0.48 for the left kidney and 0.98 for the right kidney. These results suggest obstruction on the right side, which in this case, was caused by ureteral clots.

Figure 6.

Distribution of liver cysts based on Couinaud’s hepatic segments. Couinaud’s classification of (left) hepatic segmentation with the (right) distribution of liver cysts in autosomal-dominant polycystic kidney disease patients per each segment.