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Case report: Enormous intrahepatic communication between the portal vein and the hepatic vein
ClinicalRadiology(1986) 37, 513-514 © 1986 Royal College of Radiologists
0009-9260/86/743513 $02.00
Case Report" Enormous Intrahepatic Communication between the Portal Vein and the Hepatic Vein. KUNI OHTOMO, MASAHIRO IIO
SHIGERU
FURUI*, MITSUNORI
SAITO?, TAKASHI KOKUBO,
YUJI ITAI and
Department of Radiology, University of Tokyo, *Department of Radiology, Defence Force Medical College, Saitama and ?Department of Neurology, Kohnodai Notional Hospital, Chiba, Japan. A case of enormous intrahepatic shunt between the portal vein and the hepatic vein is described. The lesion was successfully treated by embolisation using stainless steel coils on two occasions, after demonstration by percutaneous transhepatic portography (PTP).
A l t h o u g h in t h e c i r r h o t i c liver, m i c r o s c o p i c c o m m u n i c a t i o n s b e t w e e n b r a n c h e s o f t h e p o r t a l vein a n d o f the h e p a t i c vein a r e f r e q u e n t l y o b s e r v e d ( P o p p e r et al., 1952; M i l t o n et al., 1959), e n o r m o u s i n t r a h e p a t i c s h u n t s b e t w e e n t h e two s y s t e m s a r e v e r y r a r e , o n l y f o u r cases having b e e n r e p o r t e d in t h e English a n d J a p a n e s e l i t e r a t u r e to t h e b e s t o f o u r k n o w l e d g e ( R a s k i n et al., 1964; K o z u k a et al., 1975; T a g u c h i et al., 1983). In this r e p o r t w e d e s c r i b e o n e a d d i t i o n a l case in which e m b o l i s a t i o n using stainless steel coils markedly improved hepatic encephalopathy.
CASE REPORT A 42-year-old man was referred to our institution from an outside hospital for further evaluation of hepatic encephalopathy. The patient gave a history of consciousness disturbance for about seven years and had noticed tremor of the hands and fingers five years before. Abnormalities of physical examination included slight dementia, scanning speech, spastic tetraplegia and ataxia. Abnormal laboratory test results included: serum total protein 5.1 g/dl (normal 6.7-8.1g/dl), albumin 3.1g (4.0-5.0), prothrombin time 40.8% (70-100), and cholinesterase 218U/1 (355-705). Indocyanine green test showed retention of 53.6% (<10) at 15min. Two samples Request for reprints: Kuni Ohtomo, Department of Radiology, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan 113.
showed a high concentration of plasma ammonia of 237 and 133 g/dl (15-49). Liver biopsy disclosed mild infiltration by lymphocytes without cirrhosis. Arterial portography via the superior mesenteric artery showed multiple abnormal channels between the portal venous system and the hepatic veins within the liver. Percutaneous transhepatic portography (PTP) clearly demonstrated these abnormal communications from the 3rd or 4th order branches of the portal vein to hepatic vein branches in both lobes of the liver (Fig. la). To improve hepatic encephalopathy, embolisation therapy using stainless steel coils was attempted. The catheter was introduced via an ileocolie vein through a small abdominal incision. Seven stainless steel coils Occluding Spring Embolus (Cook, IN 47402, USA) were successfully delivered into the 3rd or 4th order portal vein branches and six boils were added at a second embolisation 11 months later (Fig. lb). A portogram obtained after the embolisation showed a decrease in shunt flow. The post-embolisation course was uneventful and the patient has been symptom-free without special medical therapy for seven months since the second embolisation.
DISCUSSION O f t h e f o u r p r e v i o u s l y r e p o r t e d cases, two h a d s y m p t o m s of h e p a t i c e n c e p h a t o p a t h y d u e to e n o r m o u s intrahepatic communication between portal and hepatic veins. T h e first case, r e p o r t e d by R a s k i n et al. (1964) was a 5 6 - y e a r - o l d m a n with m u l t i p l e i n t r a h e p a tic shunts b e t w e e n p o r t a l and h e p a t i c veins. T h e y c o n s i d e r e d it was a c o n g e n i t a l m a l f o r m a t i o n , n e v e r theless l e a v i n g u n a n s w e r e d the q u e s t i o n o f w h y 53 y e a r s e l a p s e d b e f o r e the a p p e a r a n c e o f t h e c e r e b r a l s y m p t o m s . K o z u k a et al., (1975) d e s c r i b e d t h e case of a 5 7 - y e a r - o l d m a n with h e p a t o c e r e b r a l d i s o r d e r in which a t u b e - s h a p e d shunt b e t w e e n a p o r t a l v e i n a n d h e p a t i c vein in t h e right l o b e was d e m o n s t r a t e d b y
(a) (b) Fig. 1 - Case 1. (a) PTP demonstrates multiple intrahepatic porto-systemic shunts. (b) Radiograph after the second embolisation. A total of 13 stainless steel coils were introduced into the portal vein branches of the right lobe and medial segment of the left lobe.
514
CLINICAL RADIOLOGY
autopsy. They speculated that the shunt was not a congenital malformation but a result of parenchymal collapse of the liver because histopathology showed that the wall of the shunt lacked both the muscular layer and the elastic lamellae, and the fibrous granulation tissues of the wall were histologically similar to the fibrous septa of liver cirrhosis. Our case had a close resemblance to Raskin's case in the clinical history and portographic findings. Recently Taguchi et al. (1983) reported two cases of intrahepatic portosystemic shunt discovered by ultrasound examination for chronic liver disease. They had no neurological symptoms and PTP demonstrated bead-like fistulae from the enlarged portal vein branch to the hepatic vein. In our case arterial portography via the superior mesenteric artery faintly showed the abnormal communication between the portal and hepatic veins within the liver: However, PTP was indispensable for the final diagnosis. No standard treatment exists for symptomatic intrahepatic porto-systemic communication since the lesion is so unusual. In Raskin's case, although ligation of the left branch of the portal vein and partial resection of the right lobe of the liver were performed, the patient died on the second day after operation. We elected to embolise the lesion using a technique similar to the one advocated by Ido et al. (1985) for
embolisation of gastro-oesophageal varices. The satisfactory result obtained following embolisation suggests that this may be the treatment of choice for symptomatic giant intrahepatic porto-systemic communication.
Acknowledgements. We would like to thank Holly Korsvik Wysocki, M.D. for her helpful suggestions and assistance in preparing this manuscript.
REFERENCES Ido, K. & Hiramatsu, K. (1985) Trans-ileocolic vein obliteration of the gastroesophageal varices. Nichidoku-iho, 30, 155-163. Kozuka, S., Sassa, R. & Kakumu, S. (1975). An enormous intrahepatic shunt between portal vein and hepatic one. Angiology, 26, 365-371. Milton, R., Hales, M.D, Allan, J.S., Hall, E.H. (1959). Injectioncorrosion studies of normal and cirrhotic livers. American Journal of Pathology, 35, 909-928. Popper, H., Elias, H. & Petty DE. (1952) Vascular pattern of the cirrhotic liver. American Journal of Clinical Pathology. 22, 717-729. Raskin, N. H., Price, J. B. & Fishman, R. A. (1964) Portalsystemic encephalopathy due to congenital intrahepatic shunts. New England Journal of Medicine, 270 225-229. Taguchi, H., Horiguchi, Y. & Kitano, T. (1983) Two cases of intrahepatic portosystemic shunt. Japanese Journal of Medical Ultrasonics, 43, 71-72.
0009-9260/86/743513 $02.00
Case Report" Enormous Intrahepatic Communication between the Portal Vein and the Hepatic Vein. KUNI OHTOMO, MASAHIRO IIO
SHIGERU
FURUI*, MITSUNORI
SAITO?, TAKASHI KOKUBO,
YUJI ITAI and
Department of Radiology, University of Tokyo, *Department of Radiology, Defence Force Medical College, Saitama and ?Department of Neurology, Kohnodai Notional Hospital, Chiba, Japan. A case of enormous intrahepatic shunt between the portal vein and the hepatic vein is described. The lesion was successfully treated by embolisation using stainless steel coils on two occasions, after demonstration by percutaneous transhepatic portography (PTP).
A l t h o u g h in t h e c i r r h o t i c liver, m i c r o s c o p i c c o m m u n i c a t i o n s b e t w e e n b r a n c h e s o f t h e p o r t a l vein a n d o f the h e p a t i c vein a r e f r e q u e n t l y o b s e r v e d ( P o p p e r et al., 1952; M i l t o n et al., 1959), e n o r m o u s i n t r a h e p a t i c s h u n t s b e t w e e n t h e two s y s t e m s a r e v e r y r a r e , o n l y f o u r cases having b e e n r e p o r t e d in t h e English a n d J a p a n e s e l i t e r a t u r e to t h e b e s t o f o u r k n o w l e d g e ( R a s k i n et al., 1964; K o z u k a et al., 1975; T a g u c h i et al., 1983). In this r e p o r t w e d e s c r i b e o n e a d d i t i o n a l case in which e m b o l i s a t i o n using stainless steel coils markedly improved hepatic encephalopathy.
CASE REPORT A 42-year-old man was referred to our institution from an outside hospital for further evaluation of hepatic encephalopathy. The patient gave a history of consciousness disturbance for about seven years and had noticed tremor of the hands and fingers five years before. Abnormalities of physical examination included slight dementia, scanning speech, spastic tetraplegia and ataxia. Abnormal laboratory test results included: serum total protein 5.1 g/dl (normal 6.7-8.1g/dl), albumin 3.1g (4.0-5.0), prothrombin time 40.8% (70-100), and cholinesterase 218U/1 (355-705). Indocyanine green test showed retention of 53.6% (<10) at 15min. Two samples Request for reprints: Kuni Ohtomo, Department of Radiology, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, Japan 113.
showed a high concentration of plasma ammonia of 237 and 133 g/dl (15-49). Liver biopsy disclosed mild infiltration by lymphocytes without cirrhosis. Arterial portography via the superior mesenteric artery showed multiple abnormal channels between the portal venous system and the hepatic veins within the liver. Percutaneous transhepatic portography (PTP) clearly demonstrated these abnormal communications from the 3rd or 4th order branches of the portal vein to hepatic vein branches in both lobes of the liver (Fig. la). To improve hepatic encephalopathy, embolisation therapy using stainless steel coils was attempted. The catheter was introduced via an ileocolie vein through a small abdominal incision. Seven stainless steel coils Occluding Spring Embolus (Cook, IN 47402, USA) were successfully delivered into the 3rd or 4th order portal vein branches and six boils were added at a second embolisation 11 months later (Fig. lb). A portogram obtained after the embolisation showed a decrease in shunt flow. The post-embolisation course was uneventful and the patient has been symptom-free without special medical therapy for seven months since the second embolisation.
DISCUSSION O f t h e f o u r p r e v i o u s l y r e p o r t e d cases, two h a d s y m p t o m s of h e p a t i c e n c e p h a t o p a t h y d u e to e n o r m o u s intrahepatic communication between portal and hepatic veins. T h e first case, r e p o r t e d by R a s k i n et al. (1964) was a 5 6 - y e a r - o l d m a n with m u l t i p l e i n t r a h e p a tic shunts b e t w e e n p o r t a l and h e p a t i c veins. T h e y c o n s i d e r e d it was a c o n g e n i t a l m a l f o r m a t i o n , n e v e r theless l e a v i n g u n a n s w e r e d the q u e s t i o n o f w h y 53 y e a r s e l a p s e d b e f o r e the a p p e a r a n c e o f t h e c e r e b r a l s y m p t o m s . K o z u k a et al., (1975) d e s c r i b e d t h e case of a 5 7 - y e a r - o l d m a n with h e p a t o c e r e b r a l d i s o r d e r in which a t u b e - s h a p e d shunt b e t w e e n a p o r t a l v e i n a n d h e p a t i c vein in t h e right l o b e was d e m o n s t r a t e d b y
(a) (b) Fig. 1 - Case 1. (a) PTP demonstrates multiple intrahepatic porto-systemic shunts. (b) Radiograph after the second embolisation. A total of 13 stainless steel coils were introduced into the portal vein branches of the right lobe and medial segment of the left lobe.
514
CLINICAL RADIOLOGY
autopsy. They speculated that the shunt was not a congenital malformation but a result of parenchymal collapse of the liver because histopathology showed that the wall of the shunt lacked both the muscular layer and the elastic lamellae, and the fibrous granulation tissues of the wall were histologically similar to the fibrous septa of liver cirrhosis. Our case had a close resemblance to Raskin's case in the clinical history and portographic findings. Recently Taguchi et al. (1983) reported two cases of intrahepatic portosystemic shunt discovered by ultrasound examination for chronic liver disease. They had no neurological symptoms and PTP demonstrated bead-like fistulae from the enlarged portal vein branch to the hepatic vein. In our case arterial portography via the superior mesenteric artery faintly showed the abnormal communication between the portal and hepatic veins within the liver: However, PTP was indispensable for the final diagnosis. No standard treatment exists for symptomatic intrahepatic porto-systemic communication since the lesion is so unusual. In Raskin's case, although ligation of the left branch of the portal vein and partial resection of the right lobe of the liver were performed, the patient died on the second day after operation. We elected to embolise the lesion using a technique similar to the one advocated by Ido et al. (1985) for
embolisation of gastro-oesophageal varices. The satisfactory result obtained following embolisation suggests that this may be the treatment of choice for symptomatic giant intrahepatic porto-systemic communication.
Acknowledgements. We would like to thank Holly Korsvik Wysocki, M.D. for her helpful suggestions and assistance in preparing this manuscript.
REFERENCES Ido, K. & Hiramatsu, K. (1985) Trans-ileocolic vein obliteration of the gastroesophageal varices. Nichidoku-iho, 30, 155-163. Kozuka, S., Sassa, R. & Kakumu, S. (1975). An enormous intrahepatic shunt between portal vein and hepatic one. Angiology, 26, 365-371. Milton, R., Hales, M.D, Allan, J.S., Hall, E.H. (1959). Injectioncorrosion studies of normal and cirrhotic livers. American Journal of Pathology, 35, 909-928. Popper, H., Elias, H. & Petty DE. (1952) Vascular pattern of the cirrhotic liver. American Journal of Clinical Pathology. 22, 717-729. Raskin, N. H., Price, J. B. & Fishman, R. A. (1964) Portalsystemic encephalopathy due to congenital intrahepatic shunts. New England Journal of Medicine, 270 225-229. Taguchi, H., Horiguchi, Y. & Kitano, T. (1983) Two cases of intrahepatic portosystemic shunt. Japanese Journal of Medical Ultrasonics, 43, 71-72.