Appearance of autosomal recessive polycystic kidney disease in magnetic resonance imaging and RARE-MR-urography.
OBJECTIVE
To describe the appearance of autosomal recessive polycystic kidney disease (ARPKD) on MRI and RARE-MR urography.


METHODS
Seven boys and one girl (aged 3 months to 14 years, median 2.5 years) were evaluated. Images were obtained with 0.23-T and 1.5-T MR systems using T1-weighted (T1-W) spin-echo, T2-weighted (T2-W) turbo-spin-echo and RARE-MR-urography sequences. Signal intensities, morphological appearance of the affected kidneys and, specifically, the picture of the urinary tract on RARE-MR-urography were evaluated.


RESULTS
All children showed kidney enlargement, reniform but humpy kidney shape, homogeneously grainy renal parenchyma, normal renal pelvis and normal calyces. Signal intensity was hyperintense in T2-W images in all cases. In six cases (n = 7), T1-W images were hypointense. On RARE-MR urography a hyperintense, linear radial pattern was seen in the cortex and medulla which represents the characteristic microcystic dilatation of collecting ducts in ARPKD. Three boys and the girl presented with a few circumscribed small subcapsular cysts.


CONCLUSIONS
In order to confirm the diagnosis of ARPKD, RARE-MR urography seems to be a non-invasive imaging tool that shows directly the microcystic dilated water-filled collecting ducts.
Kern
S
S
Department of Diagnostic Radiology, University Hospital Freiburg, Germany.
Zimmerhackl
L B
LB
Hildebrandt
F
F
Ermisch-Omran
B
B
Uhl
M
M
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007089:Image Enhancement; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications / Q000175:diagnosis*; D012189:Retrospective Studies; D014567:Urography / Q000379:methods*