Medullary cystic disease of the kidney (fam ilial ju venile nephron ophthisis) is characterized by an emi a, azo temia, excessive renal sodium loss, normal blood press ure and urinary sediment, and innumerable small renal cysts, primaril y at the corticornedullary junction . Selective renal an giogr aphy with 2X m agnification ena bled pr esumptive diagn osis in 4 of ,j case s. Characteristic features induded m arked cortical thinning and multiple cy sts (1-40 mm in diameter) which sp are d the ou te rmost cortex. Diagnostic Radiology INDEX TERM S : Anemia. Kidneys , ography • Uremia Radiology 110 : 277 -281, February 1974M E DU L L A R Y cystic disea se of the kidney is characterized by the presen ce of multiple cysts in the renal medulla and inner cortex and by the in sidious development of anemia in young p er sons, leading to d eath in uremia. R adiograp hic fea tures have not bee n previously considere d helpful in diagnosis. . In our experience angiographic features were some wh a t specific and u su ally enabled diff er entiation from other conditions, su ch a s medullary sp onge kidney , classical pol ycy stic kidney di sease , glome ru lonephritis, or p yelonephritis.
MA TERIAL A ND M E THODDuring a r ecent review of ] 43 cases of chronic renal parenchymal disease , angiograms from 5 patients with medullary cyst ic di sease were encounter ed. Also available for comparison were angiogra ms from 8 patients with polycy stic disease, 50 with ch ronic glomerulonephritis, 24 with pyelone p hrit is, and 2 with m edullary sponge kidney. Direct magnification arteri ography (2 : 1) using the sho r t -t a rget-film m ethod (2) was utilized in mo st cases. Proper volu me and rate of injection of cont rast medium were cruc ial and assured a den se n ephrogram which was the key to correc t di agn osis. Usu a lly 6-10 ml of R enografin 76 wer e injected a t a rate approximating renal blood flow, as es t imate d by prelimin ary injection with fluoroscopic observat ion . Injected a t a rate equivalen t t o blood flow , all the contrast medium r eaches the kidney in high conce n t ra t ion, thus maximizin g the density of the nephrogram.Histolo gic confirmation wa s obt ained in 4 of cysts • Kidneys, failure • Renal AngiFi g . 1. T he cut surface of the kidney re veals multiple cysts involving the parenchyma at the co rt ico m ed u lla ry junction. T he kidney me a sured 3 X 6 X IO cm .the patients with medullary cystic disease, with nephrectomy specimens from 3 cases and percutaneous needle biopsy from 1. The remaining case fulfilled all the clinical criteria of medullary cyst ic di sease. The parameters spe cifica lly eva lu a t ed were: uniformity of involvement within and between kidneys, kidney size, cortical thickness , caliber of the main renal artery , renal blood flow, interlobar and arcuate artery abnormalities (p r u n in g , tortu osity , crowd ing, stenosis, occlus ions, st ret ch ing and tapering) , visualization of the interlobular ar te ries, prominence of the ex traren a l arteries, integrity of the renal...