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Congenital Hepatic Shunts¹

¹ From the Department of Radiology, Hospital Universitario 12 de Octubre, Carretera de Andalucía km 5,400, 28041 Madrid, Spain (C.G., M.M., G.G., E.G.H.); the Department of Radiology, Hospital Universitario del Niño Jesús, Madrid, Spain (C.M.); and the Department of Radiology, Hospital Madrid, Madrid, Spain (P.M.). Presented as an education exhibit at the 2002 RSNA scientific assembly. Received February 28, 2003; revision requested June 10 and received August 5; accepted August 11. All authors have no financial relationships to disclose. Address correspondence to C.G. (e-mail: mamengallego@terra.es).

View larger version (49K): [in a new window]   Figure 1.  Drawings illustrate the embryologic development of the pulmonary venous system. By day 19, the pulmonary venous plexus (1) drains both the cardinal (2) and umbilicovitelline (3, 4) venous systems. Next, the pulmonary venous plexus gradually loosens its connections with the umbilicovitelline venous system while connecting with the emerging common pulmonary vein (7), whose origin is still controversial. The pulmonary vein is incorporated into the left atrium (6), resulting in the final configuration of the normal pulmonary venous return. 5 = common sinoatrial chamber, 8 = left ventricle, 9 = right cardiac chambers, 10 = superior vena cava.

View larger version (50K): [in a new window]   Figure 2.  Drawings illustrate the embryologic development of the hepatic venous system. Initially, the vitelline veins (1) enter the embryo with the yolk stalk and anastomose with each other around the duodenum (2) before passing through the septum transversum (3) on their way to the sinus venosum (4). The umbilical veins (5) course on either side of the septum transversum and come into contact with the sinusoids. The proximal part of the left vitelline vein involutes, and all the blood coming from the left side of the liver is redistributed to the right vitelline vein. The entire right umbilical vein, part of the left umbilical vein, and the left sinus venosus also degenerate. In the final configuration of the fetal hepatic venous system, the left umbilical vein brings all the oxygenated blood to the embryo. The ductus venosus (6) connects the umbilical vein with the inferior vena cava (IVC) (7). The portal venous system (8) originates from a selective involution of the anastomotic network around the duodenum.

View larger version (154K): [in a new window]   Figure 3a.  Localized infantile hemangioma in a newborn with hepatomegaly and CHF. (a) Transverse US image of the liver depicts a localized heterogeneous mass in the right hepatic lobe (arrowheads) with coarse calcifications (arrow). (b) Color Doppler US image demonstrates the highly vascular nature of the mass. (c) Color duplex US image of the enlarged hepatic artery (arrowhead) demonstrates high-velocity flow and a low resistivity index (RI). (d) Coronal single-shot fast spin-echo MR image of the abdomen shows a hyperintense mass in the right hepatic lobe (arrows) with flow voids within and around the lesion (arrowheads). (e) Axial contrast material-enhanced gradient-echo T1-weighted MR image of the liver (arterial phase) depicts intense peripheral uptake with early filling of the draining veins (arrows), a finding that indicates the presence of arteriovenous shunting in the lesion.

View larger version (117K): [in a new window]   Figure 3b.  Localized infantile hemangioma in a newborn with hepatomegaly and CHF. (a) Transverse US image of the liver depicts a localized heterogeneous mass in the right hepatic lobe (arrowheads) with coarse calcifications (arrow). (b) Color Doppler US image demonstrates the highly vascular nature of the mass. (c) Color duplex US image of the enlarged hepatic artery (arrowhead) demonstrates high-velocity flow and a low resistivity index (RI). (d) Coronal single-shot fast spin-echo MR image of the abdomen shows a hyperintense mass in the right hepatic lobe (arrows) with flow voids within and around the lesion (arrowheads). (e) Axial contrast material-enhanced gradient-echo T1-weighted MR image of the liver (arterial phase) depicts intense peripheral uptake with early filling of the draining veins (arrows), a finding that indicates the presence of arteriovenous shunting in the lesion.

View larger version (116K): [in a new window]   Figure 3c.  Localized infantile hemangioma in a newborn with hepatomegaly and CHF. (a) Transverse US image of the liver depicts a localized heterogeneous mass in the right hepatic lobe (arrowheads) with coarse calcifications (arrow). (b) Color Doppler US image demonstrates the highly vascular nature of the mass. (c) Color duplex US image of the enlarged hepatic artery (arrowhead) demonstrates high-velocity flow and a low resistivity index (RI). (d) Coronal single-shot fast spin-echo MR image of the abdomen shows a hyperintense mass in the right hepatic lobe (arrows) with flow voids within and around the lesion (arrowheads). (e) Axial contrast material-enhanced gradient-echo T1-weighted MR image of the liver (arterial phase) depicts intense peripheral uptake with early filling of the draining veins (arrows), a finding that indicates the presence of arteriovenous shunting in the lesion.

View larger version (155K): [in a new window]   Figure 3d.  Localized infantile hemangioma in a newborn with hepatomegaly and CHF. (a) Transverse US image of the liver depicts a localized heterogeneous mass in the right hepatic lobe (arrowheads) with coarse calcifications (arrow). (b) Color Doppler US image demonstrates the highly vascular nature of the mass. (c) Color duplex US image of the enlarged hepatic artery (arrowhead) demonstrates high-velocity flow and a low resistivity index (RI). (d) Coronal single-shot fast spin-echo MR image of the abdomen shows a hyperintense mass in the right hepatic lobe (arrows) with flow voids within and around the lesion (arrowheads). (e) Axial contrast material-enhanced gradient-echo T1-weighted MR image of the liver (arterial phase) depicts intense peripheral uptake with early filling of the draining veins (arrows), a finding that indicates the presence of arteriovenous shunting in the lesion.

View larger version (142K): [in a new window]   Figure 3e.  Localized infantile hemangioma in a newborn with hepatomegaly and CHF. (a) Transverse US image of the liver depicts a localized heterogeneous mass in the right hepatic lobe (arrowheads) with coarse calcifications (arrow). (b) Color Doppler US image demonstrates the highly vascular nature of the mass. (c) Color duplex US image of the enlarged hepatic artery (arrowhead) demonstrates high-velocity flow and a low resistivity index (RI). (d) Coronal single-shot fast spin-echo MR image of the abdomen shows a hyperintense mass in the right hepatic lobe (arrows) with flow voids within and around the lesion (arrowheads). (e) Axial contrast material-enhanced gradient-echo T1-weighted MR image of the liver (arterial phase) depicts intense peripheral uptake with early filling of the draining veins (arrows), a finding that indicates the presence of arteriovenous shunting in the lesion.

View larger version (99K): [in a new window]   Figure 4a.  Infantile hemangioma in a newborn with hepatomegaly. (a) Unenhanced abdominal CT scan depicts a well-defined mass with heterogeneous low attenuation and coarse calcifications (arrowheads) in the left hepatic lobe. (b) Tc-99m red blood cell scintigram demonstrates a large varix within the tumor. (c) Selective celiac axis arteriogram shows that the mass is highly vascularized and is supplied by the left hepatic artery (black arrow) and the anterosuperior and anteroinferior pancreaticoduodenal arteries (arrowheads). A central varix (white arrow) is observed at the arterial phase, a finding that reflects the presence of arteriovenous shunting.

View larger version (142K): [in a new window]   Figure 4b.  Infantile hemangioma in a newborn with hepatomegaly. (a) Unenhanced abdominal CT scan depicts a well-defined mass with heterogeneous low attenuation and coarse calcifications (arrowheads) in the left hepatic lobe. (b) Tc-99m red blood cell scintigram demonstrates a large varix within the tumor. (c) Selective celiac axis arteriogram shows that the mass is highly vascularized and is supplied by the left hepatic artery (black arrow) and the anterosuperior and anteroinferior pancreaticoduodenal arteries (arrowheads). A central varix (white arrow) is observed at the arterial phase, a finding that reflects the presence of arteriovenous shunting.

View larger version (89K): [in a new window]   Figure 4c.  Infantile hemangioma in a newborn with hepatomegaly. (a) Unenhanced abdominal CT scan depicts a well-defined mass with heterogeneous low attenuation and coarse calcifications (arrowheads) in the left hepatic lobe. (b) Tc-99m red blood cell scintigram demonstrates a large varix within the tumor. (c) Selective celiac axis arteriogram shows that the mass is highly vascularized and is supplied by the left hepatic artery (black arrow) and the anterosuperior and anteroinferior pancreaticoduodenal arteries (arrowheads). A central varix (white arrow) is observed at the arterial phase, a finding that reflects the presence of arteriovenous shunting.

View larger version (138K): [in a new window]   Figure 5a.  Congenital hepatic AVM in a neonate with CHF. (a) Transverse US image of the liver shows a nest of dilated, tortuous anechoic structures (arrowheads) with normal intervening liver parenchyma. No mass effect is seen within the liver. (b) Color Doppler US image demonstrates the vascular nature of the mass. (c) Duplex US image of an intrahepatic arterial vessel demonstrates high-velocity flow and a low RI related to arteriovenous shunting. (d) Duplex US image shows a hyperpulsatile pattern of the portal vein at an intrahepatic portal venous branch.

View larger version (136K): [in a new window]   Figure 5b.  Congenital hepatic AVM in a neonate with CHF. (a) Transverse US image of the liver shows a nest of dilated, tortuous anechoic structures (arrowheads) with normal intervening liver parenchyma. No mass effect is seen within the liver. (b) Color Doppler US image demonstrates the vascular nature of the mass. (c) Duplex US image of an intrahepatic arterial vessel demonstrates high-velocity flow and a low RI related to arteriovenous shunting. (d) Duplex US image shows a hyperpulsatile pattern of the portal vein at an intrahepatic portal venous branch.

View larger version (118K): [in a new window]   Figure 5c.  Congenital hepatic AVM in a neonate with CHF. (a) Transverse US image of the liver shows a nest of dilated, tortuous anechoic structures (arrowheads) with normal intervening liver parenchyma. No mass effect is seen within the liver. (b) Color Doppler US image demonstrates the vascular nature of the mass. (c) Duplex US image of an intrahepatic arterial vessel demonstrates high-velocity flow and a low RI related to arteriovenous shunting. (d) Duplex US image shows a hyperpulsatile pattern of the portal vein at an intrahepatic portal venous branch.

View larger version (106K): [in a new window]   Figure 5d.  Congenital hepatic AVM in a neonate with CHF. (a) Transverse US image of the liver shows a nest of dilated, tortuous anechoic structures (arrowheads) with normal intervening liver parenchyma. No mass effect is seen within the liver. (b) Color Doppler US image demonstrates the vascular nature of the mass. (c) Duplex US image of an intrahepatic arterial vessel demonstrates high-velocity flow and a low RI related to arteriovenous shunting. (d) Duplex US image shows a hyperpulsatile pattern of the portal vein at an intrahepatic portal venous branch.

View larger version (134K): [in a new window]   Figure 6a.  Suspected hepatic AVM. (a) Selective hepatic angiogram demonstrates a highly vascular mass with poor regional demarcation and puddling of contrast material in vascular spaces (arrows). (b) Late arterial phase angiogram shows obvious arteriovenous shunting with early filling of the IVC (arrows).

View larger version (136K): [in a new window]   Figure 6b.  Suspected hepatic AVM. (a) Selective hepatic angiogram demonstrates a highly vascular mass with poor regional demarcation and puddling of contrast material in vascular spaces (arrows). (b) Late arterial phase angiogram shows obvious arteriovenous shunting with early filling of the IVC (arrows).

View larger version (130K): [in a new window]   Figure 7a.  Arterioportal fistula in a 3-month-old infant with biliary atresia. (a) Longitudinal color Doppler US image of the liver demonstrates color aliasing in the portal vein and neighboring liver parenchyma, a finding that reflects the presence of a fistula. (b) Color duplex US image demonstrates pulsatile hepatofugal high-velocity flow in the main portal vein. (c) Axial contrast-enhanced fat-suppressed gradient-echo T1-weighted MR image of the liver depicts a dilated main portal vein with marked enhancement (arrow) similar to that of the aorta (arrowhead) and hepatic artery (not shown). Ascites due to portal hypertension is also seen (*). (d) Selective hepatic arteriogram demonstrates retrograde filling of the portal (solid arrow), splenic (open arrow), and superior mesenteric (arrowhead) veins. Feathered arrow indicates the gastroduodenal artery. (e) Celiac axis angiogram obtained after embolization of the right hepatic artery with a microcoil (arrow) shows complete resolution of the fistula. One week later, the fistula recurred through arterial collateralization, and the patient underwent liver transplantation.

View larger version (146K): [in a new window]   Figure 7b.  Arterioportal fistula in a 3-month-old infant with biliary atresia. (a) Longitudinal color Doppler US image of the liver demonstrates color aliasing in the portal vein and neighboring liver parenchyma, a finding that reflects the presence of a fistula. (b) Color duplex US image demonstrates pulsatile hepatofugal high-velocity flow in the main portal vein. (c) Axial contrast-enhanced fat-suppressed gradient-echo T1-weighted MR image of the liver depicts a dilated main portal vein with marked enhancement (arrow) similar to that of the aorta (arrowhead) and hepatic artery (not shown). Ascites due to portal hypertension is also seen (*). (d) Selective hepatic arteriogram demonstrates retrograde filling of the portal (solid arrow), splenic (open arrow), and superior mesenteric (arrowhead) veins. Feathered arrow indicates the gastroduodenal artery. (e) Celiac axis angiogram obtained after embolization of the right hepatic artery with a microcoil (arrow) shows complete resolution of the fistula. One week later, the fistula recurred through arterial collateralization, and the patient underwent liver transplantation.

View larger version (110K): [in a new window]   Figure 7c.  Arterioportal fistula in a 3-month-old infant with biliary atresia. (a) Longitudinal color Doppler US image of the liver demonstrates color aliasing in the portal vein and neighboring liver parenchyma, a finding that reflects the presence of a fistula. (b) Color duplex US image demonstrates pulsatile hepatofugal high-velocity flow in the main portal vein. (c) Axial contrast-enhanced fat-suppressed gradient-echo T1-weighted MR image of the liver depicts a dilated main portal vein with marked enhancement (arrow) similar to that of the aorta (arrowhead) and hepatic artery (not shown). Ascites due to portal hypertension is also seen (*). (d) Selective hepatic arteriogram demonstrates retrograde filling of the portal (solid arrow), splenic (open arrow), and superior mesenteric (arrowhead) veins. Feathered arrow indicates the gastroduodenal artery. (e) Celiac axis angiogram obtained after embolization of the right hepatic artery with a microcoil (arrow) shows complete resolution of the fistula. One week later, the fistula recurred through arterial collateralization, and the patient underwent liver transplantation.

View larger version (87K): [in a new window]   Figure 7d.  Arterioportal fistula in a 3-month-old infant with biliary atresia. (a) Longitudinal color Doppler US image of the liver demonstrates color aliasing in the portal vein and neighboring liver parenchyma, a finding that reflects the presence of a fistula. (b) Color duplex US image demonstrates pulsatile hepatofugal high-velocity flow in the main portal vein. (c) Axial contrast-enhanced fat-suppressed gradient-echo T1-weighted MR image of the liver depicts a dilated main portal vein with marked enhancement (arrow) similar to that of the aorta (arrowhead) and hepatic artery (not shown). Ascites due to portal hypertension is also seen (*). (d) Selective hepatic arteriogram demonstrates retrograde filling of the portal (solid arrow), splenic (open arrow), and superior mesenteric (arrowhead) veins. Feathered arrow indicates the gastroduodenal artery. (e) Celiac axis angiogram obtained after embolization of the right hepatic artery with a microcoil (arrow) shows complete resolution of the fistula. One week later, the fistula recurred through arterial collateralization, and the patient underwent liver transplantation.

View larger version (99K): [in a new window]   Figure 7e.  Arterioportal fistula in a 3-month-old infant with biliary atresia. (a) Longitudinal color Doppler US image of the liver demonstrates color aliasing in the portal vein and neighboring liver parenchyma, a finding that reflects the presence of a fistula. (b) Color duplex US image demonstrates pulsatile hepatofugal high-velocity flow in the main portal vein. (c) Axial contrast-enhanced fat-suppressed gradient-echo T1-weighted MR image of the liver depicts a dilated main portal vein with marked enhancement (arrow) similar to that of the aorta (arrowhead) and hepatic artery (not shown). Ascites due to portal hypertension is also seen (*). (d) Selective hepatic arteriogram demonstrates retrograde filling of the portal (solid arrow), splenic (open arrow), and superior mesenteric (arrowhead) veins. Feathered arrow indicates the gastroduodenal artery. (e) Celiac axis angiogram obtained after embolization of the right hepatic artery with a microcoil (arrow) shows complete resolution of the fistula. One week later, the fistula recurred through arterial collateralization, and the patient underwent liver transplantation.

View larger version (148K): [in a new window]   Figure 8a.  Extrahepatic portosystemic shunt in a 14-year-old boy with rectal bleeding and congenital absence of the portal vein. (a) Transverse color Doppler US image of the liver demonstrates a single vessel at the hepatic hilum (arrow) that represents the hepatic artery. A huge venous vessel (arrowheads) is seen behind the pancreatic gland (*). This vessel was seen to course toward the pelvis and to have hepatofugal flow. (b) Contrast-enhanced CT scan of the abdomen shows absence of the portal vein. A single hepatic artery (arrow) is seen at the hepatoduodenal ligament. (c) On a contrast-enhanced CT scan of the abdomen, the portosystemic shunting vessel (arrows) is seen coursing behind the pancreas. (d) Selective superior mesenteric artery angiogram (venous phase) depicts a huge inferior mesenteric vein (black arrow) that drains all the mesenteric venous flow through the iliac vein into the IVC (arrowhead). Note the absence of the main and intrahepatic portal veins. White arrow indicates the superior mesenteric vein.

View larger version (124K): [in a new window]   Figure 8b.  Extrahepatic portosystemic shunt in a 14-year-old boy with rectal bleeding and congenital absence of the portal vein. (a) Transverse color Doppler US image of the liver demonstrates a single vessel at the hepatic hilum (arrow) that represents the hepatic artery. A huge venous vessel (arrowheads) is seen behind the pancreatic gland (*). This vessel was seen to course toward the pelvis and to have hepatofugal flow. (b) Contrast-enhanced CT scan of the abdomen shows absence of the portal vein. A single hepatic artery (arrow) is seen at the hepatoduodenal ligament. (c) On a contrast-enhanced CT scan of the abdomen, the portosystemic shunting vessel (arrows) is seen coursing behind the pancreas. (d) Selective superior mesenteric artery angiogram (venous phase) depicts a huge inferior mesenteric vein (black arrow) that drains all the mesenteric venous flow through the iliac vein into the IVC (arrowhead). Note the absence of the main and intrahepatic portal veins. White arrow indicates the superior mesenteric vein.

View larger version (110K): [in a new window]   Figure 8c.  Extrahepatic portosystemic shunt in a 14-year-old boy with rectal bleeding and congenital absence of the portal vein. (a) Transverse color Doppler US image of the liver demonstrates a single vessel at the hepatic hilum (arrow) that represents the hepatic artery. A huge venous vessel (arrowheads) is seen behind the pancreatic gland (*). This vessel was seen to course toward the pelvis and to have hepatofugal flow. (b) Contrast-enhanced CT scan of the abdomen shows absence of the portal vein. A single hepatic artery (arrow) is seen at the hepatoduodenal ligament. (c) On a contrast-enhanced CT scan of the abdomen, the portosystemic shunting vessel (arrows) is seen coursing behind the pancreas. (d) Selective superior mesenteric artery angiogram (venous phase) depicts a huge inferior mesenteric vein (black arrow) that drains all the mesenteric venous flow through the iliac vein into the IVC (arrowhead). Note the absence of the main and intrahepatic portal veins. White arrow indicates the superior mesenteric vein.

View larger version (165K): [in a new window]   Figure 8d.  Extrahepatic portosystemic shunt in a 14-year-old boy with rectal bleeding and congenital absence of the portal vein. (a) Transverse color Doppler US image of the liver demonstrates a single vessel at the hepatic hilum (arrow) that represents the hepatic artery. A huge venous vessel (arrowheads) is seen behind the pancreatic gland (*). This vessel was seen to course toward the pelvis and to have hepatofugal flow. (b) Contrast-enhanced CT scan of the abdomen shows absence of the portal vein. A single hepatic artery (arrow) is seen at the hepatoduodenal ligament. (c) On a contrast-enhanced CT scan of the abdomen, the portosystemic shunting vessel (arrows) is seen coursing behind the pancreas. (d) Selective superior mesenteric artery angiogram (venous phase) depicts a huge inferior mesenteric vein (black arrow) that drains all the mesenteric venous flow through the iliac vein into the IVC (arrowhead). Note the absence of the main and intrahepatic portal veins. White arrow indicates the superior mesenteric vein.

View larger version (155K): [in a new window]   Figure 9a.  Extrahepatic portosystemic venous shunt in an asymptomatic 8-year-old boy with portal vein agenesis. (a) Abdominal radiograph shows the liver with a relatively small volume (arrows). (b) Contrast-enhanced CT scan of the abdomen demonstrates a single vessel at the hepatic hilum (arrowhead) that represents the hepatic artery. No intra- or extrahepatic portal vein was identified. (c) Contrast-enhanced CT scan of the abdomen obtained at a lower level shows a portosystemic venous shunt (arrowheads) between the splenomesenteric confluence and the suprarenal IVC. (d) Selective splenic artery angiogram (venous phase) depicts absence of the portal vein and the presence of an extrahepatic portosystemic venous shunt (black arrow) to the IVC (arrowhead). White arrow indicates the splenic vein.

View larger version (127K): [in a new window]   Figure 9b.  Extrahepatic portosystemic venous shunt in an asymptomatic 8-year-old boy with portal vein agenesis. (a) Abdominal radiograph shows the liver with a relatively small volume (arrows). (b) Contrast-enhanced CT scan of the abdomen demonstrates a single vessel at the hepatic hilum (arrowhead) that represents the hepatic artery. No intra- or extrahepatic portal vein was identified. (c) Contrast-enhanced CT scan of the abdomen obtained at a lower level shows a portosystemic venous shunt (arrowheads) between the splenomesenteric confluence and the suprarenal IVC. (d) Selective splenic artery angiogram (venous phase) depicts absence of the portal vein and the presence of an extrahepatic portosystemic venous shunt (black arrow) to the IVC (arrowhead). White arrow indicates the splenic vein.

View larger version (126K): [in a new window]   Figure 9c.  Extrahepatic portosystemic venous shunt in an asymptomatic 8-year-old boy with portal vein agenesis. (a) Abdominal radiograph shows the liver with a relatively small volume (arrows). (b) Contrast-enhanced CT scan of the abdomen demonstrates a single vessel at the hepatic hilum (arrowhead) that represents the hepatic artery. No intra- or extrahepatic portal vein was identified. (c) Contrast-enhanced CT scan of the abdomen obtained at a lower level shows a portosystemic venous shunt (arrowheads) between the splenomesenteric confluence and the suprarenal IVC. (d) Selective splenic artery angiogram (venous phase) depicts absence of the portal vein and the presence of an extrahepatic portosystemic venous shunt (black arrow) to the IVC (arrowhead). White arrow indicates the splenic vein.

View larger version (100K): [in a new window]   Figure 9d.  Extrahepatic portosystemic venous shunt in an asymptomatic 8-year-old boy with portal vein agenesis. (a) Abdominal radiograph shows the liver with a relatively small volume (arrows). (b) Contrast-enhanced CT scan of the abdomen demonstrates a single vessel at the hepatic hilum (arrowhead) that represents the hepatic artery. No intra- or extrahepatic portal vein was identified. (c) Contrast-enhanced CT scan of the abdomen obtained at a lower level shows a portosystemic venous shunt (arrowheads) between the splenomesenteric confluence and the suprarenal IVC. (d) Selective splenic artery angiogram (venous phase) depicts absence of the portal vein and the presence of an extrahepatic portosystemic venous shunt (black arrow) to the IVC (arrowhead). White arrow indicates the splenic vein.

View larger version (179K): [in a new window]   Figure 10a.  Intrahepatic portosystemic venous shunt seen incidentally in a neonate. (a) Transverse US image of the right hepatic lobe shows a communication (arrowhead) between a branch of the right portal vein (curved arrow) and a hepatic vein (straight arrow). (b) Color Doppler US image demonstrates the vascular nature of the communication. The shunt disappeared the following year.

View larger version (158K): [in a new window]   Figure 10b.  Intrahepatic portosystemic venous shunt seen incidentally in a neonate. (a) Transverse US image of the right hepatic lobe shows a communication (arrowhead) between a branch of the right portal vein (curved arrow) and a hepatic vein (straight arrow). (b) Color Doppler US image demonstrates the vascular nature of the communication. The shunt disappeared the following year.

View larger version (150K): [in a new window]   Figure 11a.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (134K): [in a new window]   Figure 11b.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (94K): [in a new window]   Figure 11c.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (152K): [in a new window]   Figure 11d.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (159K): [in a new window]   Figure 11e.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (156K): [in a new window]   Figure 11f.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (148K): [in a new window]   Figure 11g.  Multiple peripheral intrahepatic portosystemic venous shunts in an asymptomatic neonate. (a) Transverse US image demonstrates liver heterogeneity and multiple tubular structures (arrows) with a cystic component (arrowheads). (b) Color Doppler US image of one of the lesions demonstrates its vascular nature, with feeding (white arrow) and draining (black arrow) vessels. (c) Color duplex US image shows a turbulent triphasic pattern in the portal vein, a finding that raises suspicion for portosystemic venous shunting. Normal flow velocities and RI in the hepatic artery exclude an arteriovenous shunt. (d) Coronal half-Fourier single-shot spin-echo train image of the liver shows that the largest lesions have flow voids (arrows). (e) Coronal half-Fourier single-shot spin-echo train image of the liver demonstrates a communication between a lesion (arrow) and the middle hepatic vein (arrowhead). (f) Axial two-dimensional time-of-flight MR image (arterial phase) reveals that there is no arterial flow in the lesions (arrowhead). Solid arrow indicates the aorta, feathered arrow indicates the hepatic artery. (g) Axial two-dimensional time-of-flight MR image (venous phase) depicts venous flow in the lesions (arrowhead). Solid arrow indicates the IVC, feathered arrow indicates the portal vein. The shunts resolved spontaneously at age 6 months.

View larger version (149K): [in a new window]   Figure 12a.  Patent aneurysmal ductus venosus in a 12-year-old girl with mild neurologic disturbances. (a) Longitudinal color Doppler US image of the left hepatic lobe shows an aneurysmal communication (arrow) between the left hepatic (arrowhead) and portal veins. * = IVC. (b) Color duplex US image of the left portal vein (white arrow) demonstrates triphasic flow, a finding that reflects the presence of a portosystemic shunt. Black arrow indicates a patent aneurysmal ductus venosus. (c) Contrast-enhanced CT scan of the liver (early portal venous phase) depicts early asymmetric enhancement of the left hepatic vein (arrow). (d) On a contrast-enhanced CT scan obtained at a lower level, the ductus venosus demonstrates aneurysmal dilatation (arrowhead) and communication with the left portal vein (arrow).

View larger version (102K): [in a new window]   Figure 12b.  Patent aneurysmal ductus venosus in a 12-year-old girl with mild neurologic disturbances. (a) Longitudinal color Doppler US image of the left hepatic lobe shows an aneurysmal communication (arrow) between the left hepatic (arrowhead) and portal veins. * = IVC. (b) Color duplex US image of the left portal vein (white arrow) demonstrates triphasic flow, a finding that reflects the presence of a portosystemic shunt. Black arrow indicates a patent aneurysmal ductus venosus. (c) Contrast-enhanced CT scan of the liver (early portal venous phase) depicts early asymmetric enhancement of the left hepatic vein (arrow). (d) On a contrast-enhanced CT scan obtained at a lower level, the ductus venosus demonstrates aneurysmal dilatation (arrowhead) and communication with the left portal vein (arrow).

View larger version (125K): [in a new window]   Figure 12c.  Patent aneurysmal ductus venosus in a 12-year-old girl with mild neurologic disturbances. (a) Longitudinal color Doppler US image of the left hepatic lobe shows an aneurysmal communication (arrow) between the left hepatic (arrowhead) and portal veins. * = IVC. (b) Color duplex US image of the left portal vein (white arrow) demonstrates triphasic flow, a finding that reflects the presence of a portosystemic shunt. Black arrow indicates a patent aneurysmal ductus venosus. (c) Contrast-enhanced CT scan of the liver (early portal venous phase) depicts early asymmetric enhancement of the left hepatic vein (arrow). (d) On a contrast-enhanced CT scan obtained at a lower level, the ductus venosus demonstrates aneurysmal dilatation (arrowhead) and communication with the left portal vein (arrow).

View larger version (129K): [in a new window]   Figure 12d.  Patent aneurysmal ductus venosus in a 12-year-old girl with mild neurologic disturbances. (a) Longitudinal color Doppler US image of the left hepatic lobe shows an aneurysmal communication (arrow) between the left hepatic (arrowhead) and portal veins. * = IVC. (b) Color duplex US image of the left portal vein (white arrow) demonstrates triphasic flow, a finding that reflects the presence of a portosystemic shunt. Black arrow indicates a patent aneurysmal ductus venosus. (c) Contrast-enhanced CT scan of the liver (early portal venous phase) depicts early asymmetric enhancement of the left hepatic vein (arrow). (d) On a contrast-enhanced CT scan obtained at a lower level, the ductus venosus demonstrates aneurysmal dilatation (arrowhead) and communication with the left portal vein (arrow).

View larger version (49K): [in a new window]   Figure 13.  Drawing illustrates the anatomic disposition of the pulmonary veins in infradiaphragmatic TAPVR. The pulmonary veins drain into the left hepatic or left portal vein through a large common channel. Flow is almost always inhibited by one or more stenoses at this channel.

View larger version (110K): [in a new window]   Figure 14a.  Infradiaphragmatic TAPVR in a newborn with cyanosis and respiratory distress. (a) Longitudinal midline US image of the upper abdomen shows a large vascular channel (arrow) coming from the thorax, with flow moving in the same direction as in the aorta (arrowhead). (b) Transverse US scan of the liver demonstrates a stenotic segment (white arrowheads) of the common pulmonary vein (black arrows) just before it enters the left hepatic vein (white arrow). Black arrowhead indicates the aorta. (c) Oblique color Doppler US image of the liver shows color aliasing in the stenotic segment and left hepatic vein (white arrow). Black arrow indicates the common pulmonary vein, arrowhead indicates the aorta.

View larger version (130K): [in a new window]   Figure 14b.  Infradiaphragmatic TAPVR in a newborn with cyanosis and respiratory distress. (a) Longitudinal midline US image of the upper abdomen shows a large vascular channel (arrow) coming from the thorax, with flow moving in the same direction as in the aorta (arrowhead). (b) Transverse US scan of the liver demonstrates a stenotic segment (white arrowheads) of the common pulmonary vein (black arrows) just before it enters the left hepatic vein (white arrow). Black arrowhead indicates the aorta. (c) Oblique color Doppler US image of the liver shows color aliasing in the stenotic segment and left hepatic vein (white arrow). Black arrow indicates the common pulmonary vein, arrowhead indicates the aorta.

View larger version (122K): [in a new window]   Figure 14c.  Infradiaphragmatic TAPVR in a newborn with cyanosis and respiratory distress. (a) Longitudinal midline US image of the upper abdomen shows a large vascular channel (arrow) coming from the thorax, with flow moving in the same direction as in the aorta (arrowhead). (b) Transverse US scan of the liver demonstrates a stenotic segment (white arrowheads) of the common pulmonary vein (black arrows) just before it enters the left hepatic vein (white arrow). Black arrowhead indicates the aorta. (c) Oblique color Doppler US image of the liver shows color aliasing in the stenotic segment and left hepatic vein (white arrow). Black arrow indicates the common pulmonary vein, arrowhead indicates the aorta.

View larger version (140K): [in a new window]   Figure 15.  Infradiaphragmatic TAPVR. Pulmonary arteriogram (venous phase) demonstrates abnormal confluence of the pulmonary veins into a common channel (arrow) that traverses the diaphragm and courses toward the abdomen.

View larger version (27K): [in a new window]   Figure 16.  Chart illustrates a suggested algorithmic approach that can be used when a congenital hepatic vascular shunt is suspected at US. AP = arterioportal, AV = arteriovenous, CAPV = congenital absence of the portal vein, EPSS = extrahepatic portosystemic shunt, IPSS = intrahepatic portosystemic shunt, iTAPVR = infradiaphragmatic TAPVR, RBCTc99 = Tc-99m red blood cell scintigraphy.