Pediatr Radiol (1991) 21:529-530
Pediatric Radiology 9 Springer-Verlag 1991
Ultrasound diagnosis of congenital intrahepatic portosystemic venous shunt A. A. Jabra and G. A. Taylor Russel H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA Received: 31 May 1991; accepted: 11 July 1991
Abstract. There are few radiologic descriptions of intrahepatic portosystemic venous shunts and all in adults. A case is reported of a newborn where the diagnosis of shunt was made with color Doppler and where the shunt resolved spontaneously at one year of age. Proposed etiologies for intrahepatic portovenous shunts are discussed.
We report an unusual case of intrahepatic portosystemic venous shunt occurring in a newborn to emphasize its benign course and the use of color Doppler ultrasound in its noninvasive evaluation.
Case report A healthy, 3-day-old baby girl was referred to us for follow up of a cystic mass in the right lobe of the liver detected on a prenatal sonogram. Physical examination and laboratory data, including liver function studies were normal. There was no evidence of congestive heart failure or thrombocytopenia. Sonography revealed a 3.5 x 2.0 cm lobular anechoic area in the right lobe of the liver which contained a i cm echogenic nodule in the periphery (Fig. i a,b). Color Doppler imaging showed a swirling pattern of flow within the lesion. The varix received its blood supply exclusively from a dilated portal vein via the main right portal vein (Fig. 1 c), and entered through multiple small veins into the intrahepatic inferior vena cava (Fig. i d). Pulsed Doppler imaging showed a venous pattern of flow in all feeding and draining vessels. The patient continued to be asymptomatic and a follow up ultrasound examination 6 months later showed a decrease in the size of the lesion to 1 cm in diameter. At one year sonography showed complete resolution with no residual hepatic parenchymal abnormality.
Discussion Communications between the hepatic venous and portal venous system via the hepatic sinusoids have been demonstrated by magnification wedge hepatic venograms in normal individuals [1]. Small portosystemic venous shunts, typically less than 2 mm in size have been shown in patients with cirrhosis via the latter technique [1] as well as via corrosion injection studies [2]. Shunts of larger size detected by ultrasound, CT or arteriography have only been reported in 18 adults [3-6]. Park et al. reviewed 14 reported cases and classified them into four types [3]: 9 A single large vessel communicating the right portal vein to the inferior v. cava. 9 Peripheral localized communication(s) between peripheral portal and hepatic vein branches in one segment of a hepatic lobe. 9 Aneurysmal communication of peripheral portal and hepatic veins. 9 Diffuse communications between peripheral portal and hepatic veins in both lobes. The etiology of these large intrahepatic portosystemic venous shunts is unknown. Both congenital as well as acquired causes have been proposed. The vitelline veins in the embryologic stages of fetal development break into intrahepatic sinusoid whose afferent branches give rise to the intrahepatic portal veins and whose efferent branches give rise to the hepatic veins [7]. Embryologic remnants from stages of development where these communications exist may account for the second, third and fourth types of shunts in Park's classification. Persistence of the ductus venosus has
been proposed as a cause for portosystemic shunts of the left lobe of the liver. It has also been proposed that portosystemic venous shunts develop as collateral pathways in the presence of portal hypertension. Abnormalities in the microscopic appearance of the walls of these shunts, namely the lack of the muscular layer and the elastic lamellae, is cited as evidence [8]. The first type of shunt, i. e. between right portal vein and inferior v. cava, may be the result of persistent fetal communications between the right subcardinal and right vitelline venous systems that both give rise to parts of the intrahepatic inferior v. cava [7]. The right vitelline venous system also gives rise to the portal venous system. Our case represents a congenital aneurysmal type of portosystemic communication. There have been 3 previously described cases in adults [4, 9, 10]. Only one patient had liver cirrhosis and it was postulated that portal hypertension may have caused a portal vein aneurysm that subsequently ruptured into the hepatic venous system [9]. The other 2 cases had no evidence for hepatic cirrhosis, no history of abdominal trauma or liver biopsy, and were considered congenital in origin. A b o u t 50 % of reported cases with intrahepatic portosystemic venous shunts presented with hepatocerebral syndrome and were placed on dietary restrictions or underwent surgical intervention. The infant we present had a benign course and the shunt spontaneously resolved, presumably following thrombosis. Intrahepatic portosystemic venous shunts should be differentiated from hemangioendotheliomas. The latter are neoplasms and are seen as vascular masses rather than aneurysms and their
530
A.A. Jabra and G. A. Taylor: Congenital intrahepatic portosystemic venous shunt
Fig. 1. a Sagittal view of right lobe of liver with color Doppler reveals a lobulated flow containing lesion b Focal solid appearing area (arrow)is noted at the periphery of the lesions and most probably represents clot. One drainingvein is seen posteriorly (openarrow) e Prominent portal vein is seen that supplied lesion through its right sided branch d Two other draining veins (whitearrows)are seen flowing towards the inferior vena cava (black arrow)and were noted to join it on other images
vascular supply is hepatic arterial rather than portovenous.
Conclusion A n e u r y s m a l portosystemic intrahepatic venous shunts are rare in infancy. Our report suggests that these lesions are congenital, may be asymptomatic, and may regress spontaneously without specific intervention or treatment. Duplex and color D o p p l e r help to differentiate them from the more c o m m o n hemangioendothelioma.
References 1. Bookstein J J, Appelman HD, Waiter JF, Foley WD, Turcotte JG, Lambert M
(1975) Histological-venographic correlates in portal hypertension. Radiology 116:565-573 2. Hales MR, Allan JS, Hall EM (1959) Injection-corrosion studies of normal and cirrhotic livers. Am J Patho135:909-941 3. Park JH, Cha SH,Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. A JR 155:527-528 4. Wittich G, Jantsch H, Tscholakoff D (1985) Congenital portosystemic shunt diagnosed by combined real-time and DoppIer sonography. J Ultrasound Med 4:315-318 5. Charnsangavej C, Soo C-S, Bernardino ME, Chuang VR Wallace S (1983) Portalhepatic venous malformation: ultrasound, computed tomographic, and angiographic findings. Cardiovasc Intervent Radiol 6:109-111 6. Mori H, Hayashi K, Amamoto Y (1986) Membranous obstruction of the inferior vena cava associated with intrahepatic
portosystemic shunt. Cardiovasc Intervent Radiol 9:209413 7. Arey LB (1954) Developmental anatomy, 6. edn. Saunders, Philadelphia, pp 377386 8. Kozuka S, Sassa R, Kakumu S (1975) An enormous intrahepatic shunt between portal vein and hepatic one. Angiology26: 365-371 9. Takaysasu K, Moriyama N, Shima Y, et al. (1984) Spontaneous portal-hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology 86:945-948 10. Chagnon SF, Vallee CA, Barge J, Clevalier LJ, LeGal J, Blery MV (1986) Aneurysmal portahepatic venous fistula: report of 2 cases. Radiology 159:693-695 A. A. Jabra, M.D. Divisionof Pediatric Imaging The Johns Hopkins Hospital 600 N. Wolfe Street Baltimore, MD 21205, USA
Pediatric Radiology 9 Springer-Verlag 1991
Ultrasound diagnosis of congenital intrahepatic portosystemic venous shunt A. A. Jabra and G. A. Taylor Russel H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA Received: 31 May 1991; accepted: 11 July 1991
Abstract. There are few radiologic descriptions of intrahepatic portosystemic venous shunts and all in adults. A case is reported of a newborn where the diagnosis of shunt was made with color Doppler and where the shunt resolved spontaneously at one year of age. Proposed etiologies for intrahepatic portovenous shunts are discussed.
We report an unusual case of intrahepatic portosystemic venous shunt occurring in a newborn to emphasize its benign course and the use of color Doppler ultrasound in its noninvasive evaluation.
Case report A healthy, 3-day-old baby girl was referred to us for follow up of a cystic mass in the right lobe of the liver detected on a prenatal sonogram. Physical examination and laboratory data, including liver function studies were normal. There was no evidence of congestive heart failure or thrombocytopenia. Sonography revealed a 3.5 x 2.0 cm lobular anechoic area in the right lobe of the liver which contained a i cm echogenic nodule in the periphery (Fig. i a,b). Color Doppler imaging showed a swirling pattern of flow within the lesion. The varix received its blood supply exclusively from a dilated portal vein via the main right portal vein (Fig. 1 c), and entered through multiple small veins into the intrahepatic inferior vena cava (Fig. i d). Pulsed Doppler imaging showed a venous pattern of flow in all feeding and draining vessels. The patient continued to be asymptomatic and a follow up ultrasound examination 6 months later showed a decrease in the size of the lesion to 1 cm in diameter. At one year sonography showed complete resolution with no residual hepatic parenchymal abnormality.
Discussion Communications between the hepatic venous and portal venous system via the hepatic sinusoids have been demonstrated by magnification wedge hepatic venograms in normal individuals [1]. Small portosystemic venous shunts, typically less than 2 mm in size have been shown in patients with cirrhosis via the latter technique [1] as well as via corrosion injection studies [2]. Shunts of larger size detected by ultrasound, CT or arteriography have only been reported in 18 adults [3-6]. Park et al. reviewed 14 reported cases and classified them into four types [3]: 9 A single large vessel communicating the right portal vein to the inferior v. cava. 9 Peripheral localized communication(s) between peripheral portal and hepatic vein branches in one segment of a hepatic lobe. 9 Aneurysmal communication of peripheral portal and hepatic veins. 9 Diffuse communications between peripheral portal and hepatic veins in both lobes. The etiology of these large intrahepatic portosystemic venous shunts is unknown. Both congenital as well as acquired causes have been proposed. The vitelline veins in the embryologic stages of fetal development break into intrahepatic sinusoid whose afferent branches give rise to the intrahepatic portal veins and whose efferent branches give rise to the hepatic veins [7]. Embryologic remnants from stages of development where these communications exist may account for the second, third and fourth types of shunts in Park's classification. Persistence of the ductus venosus has
been proposed as a cause for portosystemic shunts of the left lobe of the liver. It has also been proposed that portosystemic venous shunts develop as collateral pathways in the presence of portal hypertension. Abnormalities in the microscopic appearance of the walls of these shunts, namely the lack of the muscular layer and the elastic lamellae, is cited as evidence [8]. The first type of shunt, i. e. between right portal vein and inferior v. cava, may be the result of persistent fetal communications between the right subcardinal and right vitelline venous systems that both give rise to parts of the intrahepatic inferior v. cava [7]. The right vitelline venous system also gives rise to the portal venous system. Our case represents a congenital aneurysmal type of portosystemic communication. There have been 3 previously described cases in adults [4, 9, 10]. Only one patient had liver cirrhosis and it was postulated that portal hypertension may have caused a portal vein aneurysm that subsequently ruptured into the hepatic venous system [9]. The other 2 cases had no evidence for hepatic cirrhosis, no history of abdominal trauma or liver biopsy, and were considered congenital in origin. A b o u t 50 % of reported cases with intrahepatic portosystemic venous shunts presented with hepatocerebral syndrome and were placed on dietary restrictions or underwent surgical intervention. The infant we present had a benign course and the shunt spontaneously resolved, presumably following thrombosis. Intrahepatic portosystemic venous shunts should be differentiated from hemangioendotheliomas. The latter are neoplasms and are seen as vascular masses rather than aneurysms and their
530
A.A. Jabra and G. A. Taylor: Congenital intrahepatic portosystemic venous shunt
Fig. 1. a Sagittal view of right lobe of liver with color Doppler reveals a lobulated flow containing lesion b Focal solid appearing area (arrow)is noted at the periphery of the lesions and most probably represents clot. One drainingvein is seen posteriorly (openarrow) e Prominent portal vein is seen that supplied lesion through its right sided branch d Two other draining veins (whitearrows)are seen flowing towards the inferior vena cava (black arrow)and were noted to join it on other images
vascular supply is hepatic arterial rather than portovenous.
Conclusion A n e u r y s m a l portosystemic intrahepatic venous shunts are rare in infancy. Our report suggests that these lesions are congenital, may be asymptomatic, and may regress spontaneously without specific intervention or treatment. Duplex and color D o p p l e r help to differentiate them from the more c o m m o n hemangioendothelioma.
References 1. Bookstein J J, Appelman HD, Waiter JF, Foley WD, Turcotte JG, Lambert M
(1975) Histological-venographic correlates in portal hypertension. Radiology 116:565-573 2. Hales MR, Allan JS, Hall EM (1959) Injection-corrosion studies of normal and cirrhotic livers. Am J Patho135:909-941 3. Park JH, Cha SH,Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. A JR 155:527-528 4. Wittich G, Jantsch H, Tscholakoff D (1985) Congenital portosystemic shunt diagnosed by combined real-time and DoppIer sonography. J Ultrasound Med 4:315-318 5. Charnsangavej C, Soo C-S, Bernardino ME, Chuang VR Wallace S (1983) Portalhepatic venous malformation: ultrasound, computed tomographic, and angiographic findings. Cardiovasc Intervent Radiol 6:109-111 6. Mori H, Hayashi K, Amamoto Y (1986) Membranous obstruction of the inferior vena cava associated with intrahepatic
portosystemic shunt. Cardiovasc Intervent Radiol 9:209413 7. Arey LB (1954) Developmental anatomy, 6. edn. Saunders, Philadelphia, pp 377386 8. Kozuka S, Sassa R, Kakumu S (1975) An enormous intrahepatic shunt between portal vein and hepatic one. Angiology26: 365-371 9. Takaysasu K, Moriyama N, Shima Y, et al. (1984) Spontaneous portal-hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology 86:945-948 10. Chagnon SF, Vallee CA, Barge J, Clevalier LJ, LeGal J, Blery MV (1986) Aneurysmal portahepatic venous fistula: report of 2 cases. Radiology 159:693-695 A. A. Jabra, M.D. Divisionof Pediatric Imaging The Johns Hopkins Hospital 600 N. Wolfe Street Baltimore, MD 21205, USA
