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Autosomal Recessive Polycystic Kidney Disease: Radiologic-Pathologic Correlation¹

(CME available in print version and on RSNA Link)

¹ From the Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md (G.J.L.); the Department of Radiologic Pathology (G.J.L.) and Pediatric Pathology (E.S.S.), Armed Forces Institute of Pathology, Bldg 54, Rm M-121, 14th and Alaska Sts, NW, Washington, DC 20306-6000; and the Department of Radiology, National Naval Medical Center, Bethesda, Md (R.R.R.). Received December 20, 1999; revision requested February 14, 2000; revision received February 28; accepted March 2. Address reprint requests to G.J.L. (e-mail: lonergan@afip.osd.mil).

View larger version (124K): [in a new window]   Figure 1a.   Potter facies in ARPKD. (a) Frontal photograph of an infant demonstrates a snubbed nose, low-set and flattened ears, and deep eye creases. (b) Lateral photograph of another infant shows micrognathia and low-set ears. (c) Fetal US scan demonstrates a varus deformity of the foot (arrow), compatible with clubfoot.

View larger version (132K): [in a new window]   Figure 1b.   Potter facies in ARPKD. (a) Frontal photograph of an infant demonstrates a snubbed nose, low-set and flattened ears, and deep eye creases. (b) Lateral photograph of another infant shows micrognathia and low-set ears. (c) Fetal US scan demonstrates a varus deformity of the foot (arrow), compatible with clubfoot.

View larger version (112K): [in a new window]   Figure 1c.   Potter facies in ARPKD. (a) Frontal photograph of an infant demonstrates a snubbed nose, low-set and flattened ears, and deep eye creases. (b) Lateral photograph of another infant shows micrognathia and low-set ears. (c) Fetal US scan demonstrates a varus deformity of the foot (arrow), compatible with clubfoot.

View larger version (45K): [in a new window]   Figure 2.   Collecting duct dilatation in ARPKD. Diagram of a renal medullary ray depicts both normal and abnormal collecting ducts. The left side of the diagram (A) depicts a normal nephron draining into a normal (nondilated) collecting duct. The right side (B) depicts a normal nephron draining into an ectatic collecting duct in ARPKD.

View larger version (182K): [in a new window]   Figure 3.   Histologic features of the kidney in ARPKD. Low-power photomicrograph (original magnification,  x 20; hematoxylineosin stain) of a renal specimen involved with ARPKD shows radially oriented, dilated collecting ducts (*) abutting the renal capsule (arrowheads). Normal glomeruli are interspersed among the dilated collecting ducts (arrows).

View larger version (123K): [in a new window]   Figure 4.   Photograph of a bivalved kidney involved with ARPKD reveals multiple ectatic collecting ducts, radially oriented from the center of the kidney to the surface. The corticomedullary junction is obliterated by the numerous abnormal ducts.

View larger version (115K): [in a new window]   Figure 5a.   Nephromegaly in a newborn with ARPKD. (a) Frontal chest and abdominal radiograph shows bilateral flank masses that displace gas-filled bowel loops centrally (arrows). (b) Lateral chest and abdominal radiograph shows large retroperitoneal masses (*) that displace the gas-filled bowel loops anteriorly. (c) Autopsy photograph of the opened abdomen shows nephromegaly (curved arrows) and centrally displaced bowel. In the liver are multiple small yellow foci of portal tract fibrosis (straight arrows).

View larger version (103K): [in a new window]   Figure 5b.   Nephromegaly in a newborn with ARPKD. (a) Frontal chest and abdominal radiograph shows bilateral flank masses that displace gas-filled bowel loops centrally (arrows). (b) Lateral chest and abdominal radiograph shows large retroperitoneal masses (*) that displace the gas-filled bowel loops anteriorly. (c) Autopsy photograph of the opened abdomen shows nephromegaly (curved arrows) and centrally displaced bowel. In the liver are multiple small yellow foci of portal tract fibrosis (straight arrows).

View larger version (121K): [in a new window]   Figure 5c.   Nephromegaly in a newborn with ARPKD. (a) Frontal chest and abdominal radiograph shows bilateral flank masses that displace gas-filled bowel loops centrally (arrows). (b) Lateral chest and abdominal radiograph shows large retroperitoneal masses (*) that displace the gas-filled bowel loops anteriorly. (c) Autopsy photograph of the opened abdomen shows nephromegaly (curved arrows) and centrally displaced bowel. In the liver are multiple small yellow foci of portal tract fibrosis (straight arrows).

View larger version (102K): [in a new window]   Figure 6a.   Pulmonary hypoplasia in an infant with ARPKD. (a) Frontal chest and abdominal radiograph shows bilateral pneumothoraces (note the deep costophrenic sulci bilaterally [arrows] and unusually sharp mediastinal borders), bilateral flank masses, and centrally located bowel. (b) Autopsy photograph of the chest shows a small thorax, compatible with pulmonary hypoplasia.

View larger version (121K): [in a new window]   Figure 6b.   Pulmonary hypoplasia in an infant with ARPKD. (a) Frontal chest and abdominal radiograph shows bilateral pneumothoraces (note the deep costophrenic sulci bilaterally [arrows] and unusually sharp mediastinal borders), bilateral flank masses, and centrally located bowel. (b) Autopsy photograph of the chest shows a small thorax, compatible with pulmonary hypoplasia.

View larger version (147K): [in a new window]   Figure 7a.   Fetal kidneys in ARPKD. (a) Longitudinal US scan of the left kidney in a 3rd-trimester fetus shows an enlarged, echogenic kidney (arrows) with loss of corticomedullary differentiation. Note the paucity of amniotic fluid, indicating oligohydramnios. (b) Transverse US scan of a 26-week-old fetus shows enlarged kidneys (arrows) that are more echogenic than the liver (*).

View larger version (147K): [in a new window]   Figure 7b.   Fetal kidneys in ARPKD. (a) Longitudinal US scan of the left kidney in a 3rd-trimester fetus shows an enlarged, echogenic kidney (arrows) with loss of corticomedullary differentiation. Note the paucity of amniotic fluid, indicating oligohydramnios. (b) Transverse US scan of a 26-week-old fetus shows enlarged kidneys (arrows) that are more echogenic than the liver (*).

View larger version (134K): [in a new window]   Figure 8a.   Urinary tract in ARPKD. (a) Longitudinal US scan of the upper portion of the right kidney (straight arrows) shows an enlarged, heterogeneous kidney that is more echogenic than the liver. The thin hypoechoic rim of tissue at the renal periphery (*) most likely represents compressed cortex. The liver (curved arrows) demonstrates heterogeneous echogenicity. (b) Excretory urogram demonstrates striated nephrograms in bilaterally enlarged kidneys, hypodense rims of parenchyma (*), and a small bladder (arrow). (c) Photograph of the autopsy specimen shows the enlarged kidneys with persistent fetal lobation and the small bladder (arrow).

View larger version (112K): [in a new window]   Figure 8b.   Urinary tract in ARPKD. (a) Longitudinal US scan of the upper portion of the right kidney (straight arrows) shows an enlarged, heterogeneous kidney that is more echogenic than the liver. The thin hypoechoic rim of tissue at the renal periphery (*) most likely represents compressed cortex. The liver (curved arrows) demonstrates heterogeneous echogenicity. (b) Excretory urogram demonstrates striated nephrograms in bilaterally enlarged kidneys, hypodense rims of parenchyma (*), and a small bladder (arrow). (c) Photograph of the autopsy specimen shows the enlarged kidneys with persistent fetal lobation and the small bladder (arrow).

View larger version (124K): [in a new window]   Figure 8c.   Urinary tract in ARPKD. (a) Longitudinal US scan of the upper portion of the right kidney (straight arrows) shows an enlarged, heterogeneous kidney that is more echogenic than the liver. The thin hypoechoic rim of tissue at the renal periphery (*) most likely represents compressed cortex. The liver (curved arrows) demonstrates heterogeneous echogenicity. (b) Excretory urogram demonstrates striated nephrograms in bilaterally enlarged kidneys, hypodense rims of parenchyma (*), and a small bladder (arrow). (c) Photograph of the autopsy specimen shows the enlarged kidneys with persistent fetal lobation and the small bladder (arrow).

View larger version (108K): [in a new window]   Figure 9a.   Macrocysts in ARPKD. (a) Longitudinal US scan of an enlarged, echogenic right kidney shows several rounded, anechoic areas (*). (b) Photograph of the bivalved kidney specimen helps confirm the macrocysts (arrows). The radially arrayed, fusiform, dilated spaces throughout the remainder of the renal parenchyma are dilated collecting ducts.

View larger version (156K): [in a new window]   Figure 9b.   Macrocysts in ARPKD. (a) Longitudinal US scan of an enlarged, echogenic right kidney shows several rounded, anechoic areas (*). (b) Photograph of the bivalved kidney specimen helps confirm the macrocysts (arrows). The radially arrayed, fusiform, dilated spaces throughout the remainder of the renal parenchyma are dilated collecting ducts.

View larger version (107K): [in a new window]   Figure 10.   Striated nephrograms in ARPKD. Excretory urogram of a newborn with enlarged, smooth kidneys shows striation of the kidneys bilaterally, representing pooling of contrast material in dilated, radially oriented collecting ducts.

View larger version (96K): [in a new window]   Figure 11a.   Pattern of contrast material enhancement in ARPKD in an adolescent with mild renal insufficiency. (a) Axial unenhanced CT scan shows a normal-sized right kidney with a small calcification (arrow). (b) Axial CT scan obtained immediately after intravenous injection of contrast material shows prompt peripheral enhancement. (c) A 15-minute delayed image reveals streaks of high attenuation in the renal parenchyma, oriented radially from the renal pelvis. This finding represents excreted contrast material pooling in dilated collecting ducts.

View larger version (106K): [in a new window]   Figure 11b.   Pattern of contrast material enhancement in ARPKD in an adolescent with mild renal insufficiency. (a) Axial unenhanced CT scan shows a normal-sized right kidney with a small calcification (arrow). (b) Axial CT scan obtained immediately after intravenous injection of contrast material shows prompt peripheral enhancement. (c) A 15-minute delayed image reveals streaks of high attenuation in the renal parenchyma, oriented radially from the renal pelvis. This finding represents excreted contrast material pooling in dilated collecting ducts.

View larger version (117K): [in a new window]   Figure 11c.   Pattern of contrast material enhancement in ARPKD in an adolescent with mild renal insufficiency. (a) Axial unenhanced CT scan shows a normal-sized right kidney with a small calcification (arrow). (b) Axial CT scan obtained immediately after intravenous injection of contrast material shows prompt peripheral enhancement. (c) A 15-minute delayed image reveals streaks of high attenuation in the renal parenchyma, oriented radially from the renal pelvis. This finding represents excreted contrast material pooling in dilated collecting ducts.

View larger version (111K): [in a new window]   Figure 12.   Severe ARPKD in a newborn. Axial CT scan obtained after intravenous administration of contrast material shows large, smooth, low-attenuation kidneys (arrowheads). There is faint, streaky enhancement secondary to the infant's renal failure, resulting in poor perfusion and excretion of contrast material.

View larger version (87K): [in a new window]   Figure 13.   Mild ARPKD in a 16-year-old adolescent with normal renal function. Excretory urogram shows kidneys of normal size but some striation of the nephrograms bilaterally.

View larger version (50K): [in a new window]   Figure 14.   Drawings illustrate the development of the ductal plate. The ductal plate initially forms as a single layer of cells around the developing portal vein (A). Another layer of cells develops, creating a double sleeve around the portal vein (B). In the next stage (C), the double-layered sleeve coalesces and remodels around the portal vein (*). The ductal plate in a normal portal tract (D) remodels into several connected bile ducts around the portal vein (*). The ductal plate in a patient with CHF (E) remodels abnormally, forming bile ducts around the portal vein (*) that are dilated, abnormally branching, and increased in number. There is fibrosis of the tissue separating the bile ducts in the portal tract.

View larger version (186K): [in a new window]   Figure 15a.   Histologic features of the liver in ARPKD. (a) Low-power photomicrograph (original magnification,  x 75; hematoxylineosin stain) of a liver specimen shows an enlarged portal tract (curved arrows) with increased numbers of irregular, branching bile ducts (straight arrows), surrounded by lighter pink staining portal fibrosis. The hepatic parenchyma (*) is normal. (b) Medium-power photomicrograph (original magnification,  x 140; trichrome stain) shows blue-staining periportal fibrosis in a portal tract (arrows), within which are multiple dilated bile ducts (*).

View larger version (185K): [in a new window]   Figure 15b.   Histologic features of the liver in ARPKD. (a) Low-power photomicrograph (original magnification,  x 75; hematoxylineosin stain) of a liver specimen shows an enlarged portal tract (curved arrows) with increased numbers of irregular, branching bile ducts (straight arrows), surrounded by lighter pink staining portal fibrosis. The hepatic parenchyma (*) is normal. (b) Medium-power photomicrograph (original magnification,  x 140; trichrome stain) shows blue-staining periportal fibrosis in a portal tract (arrows), within which are multiple dilated bile ducts (*).

View larger version (154K): [in a new window]   Figure 16.   Photograph of a cut liver section removed from an adolescent with ARPKD as part of a liver transplantation shows macroscopically dilated intrahepatic bile ducts (arrows) and a puckered surface, due to retractile bands of periportal fibrosis.

View larger version (116K): [in a new window]   Figures 17a.   Mild intrahepatic biliary dilatation. (a) Axial contrast material-enhanced CT scan shows mild intrahepatic biliary dilatation (arrows). (b) Corresponding radiograph obtained during cholangiography helps confirm the mildly and irregularly dilated intrahepatic biliary tree.

View larger version (128K): [in a new window]   Figures 17b.   Mild intrahepatic biliary dilatation. (a) Axial contrast material-enhanced CT scan shows mild intrahepatic biliary dilatation (arrows). (b) Corresponding radiograph obtained during cholangiography helps confirm the mildly and irregularly dilated intrahepatic biliary tree.

View larger version (113K): [in a new window]   Figures 18a.   Severe intrahepatic biliary dilatation. (a) Axial contrast-enhanced CT scan demonstrates multiple large, rounded, low-attenuation areas in the liver. The spleen is also enlarged. (b) Photograph of a resected liver specimen shows that these spaces are dilated intrahepatic bile ducts. The yellow material within the dilated ducts is bile.

View larger version (145K): [in a new window]   Figures 18b.   Severe intrahepatic biliary dilatation. (a) Axial contrast-enhanced CT scan demonstrates multiple large, rounded, low-attenuation areas in the liver. The spleen is also enlarged. (b) Photograph of a resected liver specimen shows that these spaces are dilated intrahepatic bile ducts. The yellow material within the dilated ducts is bile.

View larger version (94K): [in a new window]   Figure 19a.   Moderate intrahepatic biliary dilatation. (a) Transverse US scan of the liver (arrows) shows large, elongated anechoic spaces (*). (b) Axial contrast-enhanced CT scan shows rounded, low-attenuation areas in the liver (arrows). (c) Delayed (60-minute) frontal image from an iminodiacetic acid radionuclide study demonstrates patchy distribution and excretion of the radiopharmaceutical in the liver (arrowheads), which has flowed into the small intestine (arrows), a finding which confirms that the biliary dilatation is nonobstructive. The patchy appearance probably represents excretion into dilated bile ducts, with pooling of the radiopharmaceutical. (d) Radiograph of the resected liver specimen, obtained after its biliary tree was injected with iodinated contrast material, reveals the intrahepatic biliary dilatation.

View larger version (111K): [in a new window]   Figure 19b.   Moderate intrahepatic biliary dilatation. (a) Transverse US scan of the liver (arrows) shows large, elongated anechoic spaces (*). (b) Axial contrast-enhanced CT scan shows rounded, low-attenuation areas in the liver (arrows). (c) Delayed (60-minute) frontal image from an iminodiacetic acid radionuclide study demonstrates patchy distribution and excretion of the radiopharmaceutical in the liver (arrowheads), which has flowed into the small intestine (arrows), a finding which confirms that the biliary dilatation is nonobstructive. The patchy appearance probably represents excretion into dilated bile ducts, with pooling of the radiopharmaceutical. (d) Radiograph of the resected liver specimen, obtained after its biliary tree was injected with iodinated contrast material, reveals the intrahepatic biliary dilatation.

View larger version (113K): [in a new window]   Figure 19c.   Moderate intrahepatic biliary dilatation. (a) Transverse US scan of the liver (arrows) shows large, elongated anechoic spaces (*). (b) Axial contrast-enhanced CT scan shows rounded, low-attenuation areas in the liver (arrows). (c) Delayed (60-minute) frontal image from an iminodiacetic acid radionuclide study demonstrates patchy distribution and excretion of the radiopharmaceutical in the liver (arrowheads), which has flowed into the small intestine (arrows), a finding which confirms that the biliary dilatation is nonobstructive. The patchy appearance probably represents excretion into dilated bile ducts, with pooling of the radiopharmaceutical. (d) Radiograph of the resected liver specimen, obtained after its biliary tree was injected with iodinated contrast material, reveals the intrahepatic biliary dilatation.

View larger version (104K): [in a new window]   Figure 19d.   Moderate intrahepatic biliary dilatation. (a) Transverse US scan of the liver (arrows) shows large, elongated anechoic spaces (*). (b) Axial contrast-enhanced CT scan shows rounded, low-attenuation areas in the liver (arrows). (c) Delayed (60-minute) frontal image from an iminodiacetic acid radionuclide study demonstrates patchy distribution and excretion of the radiopharmaceutical in the liver (arrowheads), which has flowed into the small intestine (arrows), a finding which confirms that the biliary dilatation is nonobstructive. The patchy appearance probably represents excretion into dilated bile ducts, with pooling of the radiopharmaceutical. (d) Radiograph of the resected liver specimen, obtained after its biliary tree was injected with iodinated contrast material, reveals the intrahepatic biliary dilatation.

View larger version (106K): [in a new window]   Figure 20a.   Varices in ARPKD. (a) Esophagogram demonstrates several serpiginous filling defects (arrows) in the lower esophagus, consistent with esophageal varices. (b) Axial contrast-enhanced CT scan obtained through the upper abdomen shows enlarged and tortuous splenic veins (arrows), indicating portal hypertension.

View larger version (110K): [in a new window]   Figure 20b.   Varices in ARPKD. (a) Esophagogram demonstrates several serpiginous filling defects (arrows) in the lower esophagus, consistent with esophageal varices. (b) Axial contrast-enhanced CT scan obtained through the upper abdomen shows enlarged and tortuous splenic veins (arrows), indicating portal hypertension.