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Transhepatic Embolization of a Portohepatic Venous Fistula
Case Report
Transhepatic Embolization of a Portohepatic Venous ~istula' Peter Stroz, MD Murray Asch, MD, FRCPC Shia Salem, MD, FRCPC Kenneth Sniderman, MD, FRCPC Steven Gallinger, MD, MSc, FRCPC
Index terms: Aneurysm, therapy, 957.1264, 957.732 Shunts, portohepatic, 957.494 Trauma, 957.494
JVIR 1996; 7:705-707
INTRAHEPATIC shunts between the portal and systemic venous system are extremely rare, with less than 20 cases reported in the literature (1,2). Intrahepatic shunts can be caused by congenital abnormalities and acquired secondary to hepatic cirrhosis or as a complication of percutaneous biliary drainage (3). Our review of the literature failed to find a hepatic venous shunt that has definitely been attributed to trauma. We describe a n intrahepatic portohepatic venous shunt with a n associated pseudoaneurysm in which a traumatic origin is suggested. With the use of widely available technology and percutaneous techniques, this uncommon pathologic condition was successfully treated.
I CASE REPORT
From the Departments of Medical Imaging (P.S., M.A., S.S.) and Surgery (S.G.), Mount Sinai Hospital, 600 University Ave, Toronto, Ontario, Canada, M5G 1x5, and the Department of Medical Imaging (K.S.), The Toronto Hospital, University of Toronto, Toronto, Ontario. Received February 2, 1996; revision requested March 21; revision received April 2; accepted April 13. Address correspondence to M.A. O SCVIR, 1996
A previously healthy 50-year-old woman presented 4 months after a motor vehicle accident. Her medical history was remarkable only for pelvic pain evaluated with abdominal ultrasound (US) 3 years earlier; the liver was normal. Durina " the accident. the vehicle was severely damaged, but the patient was not aware of any personal injury and did not seek medical attention. Several days after the accident, the patient began experiencing intermittent left-upper-quadrant pain, which then migrated to the right upper quadrant. After 4 months of persistent pain, she was referred for abdominal sonography. The sonogram revealed a 5-cm, anechoic mass in the anterior segment of the right lobe of the liver. Color Doppler and spectral Doppler interrogation demonstrated a fistulous communication between the right portal vein and the hepatic vein with a subcapsular pseudoaneurysm (Fig la), which was confirmed with
contrast-enhanced spiral computed tomography (CT) (Fig lb). As a result of the unusual nature of this patient's imaging abnormalities and syrnptomatology, the case was discussed a t a multidisciplinary conference. The patient's symptoms were attributed to the portal vein-tohepatic venous shunt. In addition, it was believed that the subcapsular position of the pseudoaneurysm might increase her risk for bleeding related to minor trauma. For these reasons, it was decided to attempt to treat this lesion with use of percutaneous techniaues. The procedure was performed on an outpatient basis with use of intravenous sedation and local anesthesia. Venous access was achieved through the right internal jugular vein, and after placement of a 9-F vascular sheath, a 5-F multipurpose (Cook Canada, Stouffville, Ontario) catheter was directed into the of the right hepatic vein. The left branch of the portal vein was accessed with a 21gauge needle from a n anterior abdominal approach, under real-time US control. The tract was dilated with a Neff introducer set over a 0.018-inch guide wire. A 5-F multipurpose catheter was then introduced. Portal venography demonstrated normal portal venous anatomy except for a single large vein arising from an anterior branch of the right portal vein, which supplied the large pseudoaneurysm and which drained into a single enlarged branch of the right hepatic vein (Fig 2). The pseudoaneurysm was selectively cannulated from both portal and hepatic venous approaches. From the hepatic venous approach, an 11.5-mm-diameter occlusion balloon (Medi-teclBoston Scientific, Watertown, MA) was inflated within the pseudoaneurysm and was pulled against the hepatic vein, oc-
706
Journal of Vascular and Interventional Radiology
September-October 1996
Figure 1. Imaging studies demonstrate a single portal vein branch supplying the pseudoaneurysm. (a)Color Doppler US; (b) arterial phase of spiral CT. cluding venous outflow. Three 15-mm Gianturco (Cook Canada) coils were placed within the pseudoaneurysm completely filling it, and two 10-mm Gianturco coils were placed within the feeding branch of the portal vein. After coil placement, the balloon was deflated, and a venogram confirmed both an absence of flow within the aneurysm and an absence of flow across the fistula (Fig 3a). The hepatic parenchymal tract was embolized with a single 3-mm Gianturco coil. Moderate postprocedural left-upperquadrant pain resulted in overnight observation in the short stay unit. Recurrent right-upper-quandrant pain, 5 days after the procedure, prompted repeat US and CT examinations, which revealed persistent thrombosis of the fistula and pseudoaneurysm (Fig 3b). Three-month follow-up revealed no change in the character of the patient's right-upperquadrant pain and the fistula and pseudoaneurysm remained thrombosed.
hepatic lobe, although there are two reports of left-sided shunts (3,4). The anterior inferior portal vein is situated closest to the middle hepatic vein in the periphery of the liver, which may account for the predominance of right-sided lesions ( 5 ) . Left-sided shunts may be related to persistence of the ductus venosus (6). In a review of the literature, we could not identify a case of intrahepatic portohepatic venous shunt that definitely has been attributed to blunt trauma. Although not definitely proven, the temporal relationship of a shunt and pseudoaneurysm with the documented recent motor vehicle accident is strongly suggestive of a
traumatic intrahepatic portohepatic venous shunt in our patient. Shearing forces that occurred during the accident may have resulted in an intrahepatic laceration, causing communication between the portal and hepatic venous systems, which may then have developed into a pseudoaneurysm. It is doubtful that this lesion was the cause of the patient's pain, particularly because she remained symptomatic after successful embolization of the shunt. To date, no other cause for her pain has been identified. Aside from pain control, another reason for intervention in her case was the concern that the subcapsular position of the pseudoaneurysm would have
I DISCUSSION Intrahepatic portohepatic venous shunt is extremely rare. Although most revorts sueeest .,- that these shunts are congenital in origin, symptoms do not usually develop until the fifth or sixth decade. These lesions are most commonly found in the right
b. a. Figure 2. (a) Portal vein catheter injection demonstrates a single portal vein
branch supplying the pseudoaneurysm. (b) Retrograde injection through the right hepatic vein demonstrates a single draining vein.
Stroz et a1
707
Volume 7 Number 5
Figure 3. Postembolization images show coils within pseudoaneurysm and absence of flow across fistula. (a)Portal vein catheter injection; (b) venous phase spiral CT image. p u t h e r a t r i s k for bleeding related t o s u b s e q u e n t minor t r a u m a . C e r t a i n technical factors were considered before t h e procedure. T h e 5-cm lesion required t h e largest coils available a t o u r institution t o completely fill t h e pseudoaneurysm a n d reduce t h e risk of distal embolization of t h e coils. An occlusive balloon i n t h e hepatic vein w a s a f u r t h e r precautionary m e a s u r e a g a i n s t distal embolization. A n a d d e d benefit of increasi n g venous s t a s i s i n t h e lesion w a s believed t o increase t h e probability of a successful outcome. T h e probability of bleeding from t h e pseudoaneurysm m a d e direct p u n c t u r e of t h e lesion a n u n a t t r a c t i v e option. Little is known a b o u t t h e n a t u r a l history of t h e s e lesions. Most lesions a r e entirely asymptomatic a n d a r e discovered incidentally (1,3,9,10). P a t i e n t s w i t h acquired i n t r a h e p a t i c portohepatic venous s h u n t , secondary t o cirrhosis a n d portal hypertension, usually p r e s e n t with encephalopathy (7,8). Decreasing tolerance t o toxic metabolites w i t h increasing a g e m a y explain t h e l a t e clinical manifestation (4,6,7). T h e r e h a s been a case report of a lesion associated w i t h abdominal pain (8). O u r p a t i e n t did not h a v e a n y symptoms suggestive of cirrhosis or encephalopathy, a n d all laboratory investigations w e r e normal. Abdominal sonography w i t h color Doppler is a n excellent method t o identify t h e s e lesions a n d t o identify t h e direction of flow. Confirmation
w i t h C T i s valuable w h e n suboptimal sonographic visualization of t h e liver occurs a n d t o identify associated abnormalities ( 8 , l l ) . T r e a t m e n t options include surgical resection a n d percutaneous embolization. T h e r e is a single report of a case of i n t r a h e p a t i c portohepatic venous s h u n t t r e a t e d w i t h coil embolization, which w a s performed t h r o u g h a n ileocolic vein d u r i n g laparotomy (12). Advantages of percutaneous embolization include reduced morbidity a n d preservation of hepatic parenchyma. T h i s is of particular importance w h e n faced w i t h a lesion i n t h e s e t t i n g of cirrhosis a n d portal hypertension w h e r e preservation of hepatic parenc h y m a would be a n i m p o r t a n t consideration.
Acknowledgment: We wish to t h a n k Guglielmina Picano for her assistance i n preparing this manuscript. References 1. Gheorghiu D, Leibowits 0, Bloom RA. Case report: asymptomatic aneurysmal intrahepatic porto-hepatic venous shunt-diagnosis by ultrasound. Clin Radiol 1994; 49:64-65. 2. Kerlan RK, Sollenberger RD, Palubinskas AJ, Raskin NH, Callen PW, Ehrenfeld WK. Portal-systemic encephalopathy due to a congenital portocaval shunt. AJR 1982; 139: 1013-1015. 3. Bezzi M, Mitchell DG, Needleman L, Goldberg BB. Iatrogenic aneurysmal portal-hepatic venous fistula. J Ultrasound Med 1988; 7:457-461.
4. Chagnon SF, Vallee CA, Barge J , Chevalier LJ, Le Gal J, Blery MV. Aneurysmal portahepatic venous fistula: report of two cases. Radiology 1986; 159:693-698. 5. Takayasu K, Moriyama N, Shima Y, et al. Spontaneous portal-hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology 1984; 86:945-948. 6. Raskin NH, Price JB, Fishman RA. Portal-systemic encephalopathy due to congenital intrahepatic shunts. N Engl J Med 1964; 270:225-229. 7. Park J H , Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR 1990; 155:527-528. 8. Jeng LBB, Chen MF. Intrahepatic portohepatic venous shunt: a case report of successful surgical resection. Arch Surg 1993; 128:349-352. 9. Wittich G, Jantsch H, Tscholakoff D. Congenital portosystemic shunt diagnosed by combined real-time and Doppler sonography. J Ultrasound Med 1985; 4:315-318. 10. Charnsangavej C, Soo CS, Bernardino ME, Chuang VP, Wallace S. Portalhepatic venous malformation: ultrasound, computed tomographic, and angiographic findings. Cardiovasc Intervent Radiol 1983; 6:109-111. 11. Mori H, Hayashi K, Fukuda T, et al. Intrahepatic portosystemic venous shunt: occurrence in patients with and without liver cirrhosis. AJR 1987; 149:711-714. 12. Ohtomo K, Furui S, Mitsunori S, Takashi K, Yuji I, Masahiro I. Case report: enormous intrahepatic communication between the portal vein and the hepatic vein. Clin Radiol 1986; 37513-514.
Transhepatic Embolization of a Portohepatic Venous ~istula' Peter Stroz, MD Murray Asch, MD, FRCPC Shia Salem, MD, FRCPC Kenneth Sniderman, MD, FRCPC Steven Gallinger, MD, MSc, FRCPC
Index terms: Aneurysm, therapy, 957.1264, 957.732 Shunts, portohepatic, 957.494 Trauma, 957.494
JVIR 1996; 7:705-707
INTRAHEPATIC shunts between the portal and systemic venous system are extremely rare, with less than 20 cases reported in the literature (1,2). Intrahepatic shunts can be caused by congenital abnormalities and acquired secondary to hepatic cirrhosis or as a complication of percutaneous biliary drainage (3). Our review of the literature failed to find a hepatic venous shunt that has definitely been attributed to trauma. We describe a n intrahepatic portohepatic venous shunt with a n associated pseudoaneurysm in which a traumatic origin is suggested. With the use of widely available technology and percutaneous techniques, this uncommon pathologic condition was successfully treated.
I CASE REPORT
From the Departments of Medical Imaging (P.S., M.A., S.S.) and Surgery (S.G.), Mount Sinai Hospital, 600 University Ave, Toronto, Ontario, Canada, M5G 1x5, and the Department of Medical Imaging (K.S.), The Toronto Hospital, University of Toronto, Toronto, Ontario. Received February 2, 1996; revision requested March 21; revision received April 2; accepted April 13. Address correspondence to M.A. O SCVIR, 1996
A previously healthy 50-year-old woman presented 4 months after a motor vehicle accident. Her medical history was remarkable only for pelvic pain evaluated with abdominal ultrasound (US) 3 years earlier; the liver was normal. Durina " the accident. the vehicle was severely damaged, but the patient was not aware of any personal injury and did not seek medical attention. Several days after the accident, the patient began experiencing intermittent left-upper-quadrant pain, which then migrated to the right upper quadrant. After 4 months of persistent pain, she was referred for abdominal sonography. The sonogram revealed a 5-cm, anechoic mass in the anterior segment of the right lobe of the liver. Color Doppler and spectral Doppler interrogation demonstrated a fistulous communication between the right portal vein and the hepatic vein with a subcapsular pseudoaneurysm (Fig la), which was confirmed with
contrast-enhanced spiral computed tomography (CT) (Fig lb). As a result of the unusual nature of this patient's imaging abnormalities and syrnptomatology, the case was discussed a t a multidisciplinary conference. The patient's symptoms were attributed to the portal vein-tohepatic venous shunt. In addition, it was believed that the subcapsular position of the pseudoaneurysm might increase her risk for bleeding related to minor trauma. For these reasons, it was decided to attempt to treat this lesion with use of percutaneous techniaues. The procedure was performed on an outpatient basis with use of intravenous sedation and local anesthesia. Venous access was achieved through the right internal jugular vein, and after placement of a 9-F vascular sheath, a 5-F multipurpose (Cook Canada, Stouffville, Ontario) catheter was directed into the of the right hepatic vein. The left branch of the portal vein was accessed with a 21gauge needle from a n anterior abdominal approach, under real-time US control. The tract was dilated with a Neff introducer set over a 0.018-inch guide wire. A 5-F multipurpose catheter was then introduced. Portal venography demonstrated normal portal venous anatomy except for a single large vein arising from an anterior branch of the right portal vein, which supplied the large pseudoaneurysm and which drained into a single enlarged branch of the right hepatic vein (Fig 2). The pseudoaneurysm was selectively cannulated from both portal and hepatic venous approaches. From the hepatic venous approach, an 11.5-mm-diameter occlusion balloon (Medi-teclBoston Scientific, Watertown, MA) was inflated within the pseudoaneurysm and was pulled against the hepatic vein, oc-
706
Journal of Vascular and Interventional Radiology
September-October 1996
Figure 1. Imaging studies demonstrate a single portal vein branch supplying the pseudoaneurysm. (a)Color Doppler US; (b) arterial phase of spiral CT. cluding venous outflow. Three 15-mm Gianturco (Cook Canada) coils were placed within the pseudoaneurysm completely filling it, and two 10-mm Gianturco coils were placed within the feeding branch of the portal vein. After coil placement, the balloon was deflated, and a venogram confirmed both an absence of flow within the aneurysm and an absence of flow across the fistula (Fig 3a). The hepatic parenchymal tract was embolized with a single 3-mm Gianturco coil. Moderate postprocedural left-upperquadrant pain resulted in overnight observation in the short stay unit. Recurrent right-upper-quandrant pain, 5 days after the procedure, prompted repeat US and CT examinations, which revealed persistent thrombosis of the fistula and pseudoaneurysm (Fig 3b). Three-month follow-up revealed no change in the character of the patient's right-upperquadrant pain and the fistula and pseudoaneurysm remained thrombosed.
hepatic lobe, although there are two reports of left-sided shunts (3,4). The anterior inferior portal vein is situated closest to the middle hepatic vein in the periphery of the liver, which may account for the predominance of right-sided lesions ( 5 ) . Left-sided shunts may be related to persistence of the ductus venosus (6). In a review of the literature, we could not identify a case of intrahepatic portohepatic venous shunt that definitely has been attributed to blunt trauma. Although not definitely proven, the temporal relationship of a shunt and pseudoaneurysm with the documented recent motor vehicle accident is strongly suggestive of a
traumatic intrahepatic portohepatic venous shunt in our patient. Shearing forces that occurred during the accident may have resulted in an intrahepatic laceration, causing communication between the portal and hepatic venous systems, which may then have developed into a pseudoaneurysm. It is doubtful that this lesion was the cause of the patient's pain, particularly because she remained symptomatic after successful embolization of the shunt. To date, no other cause for her pain has been identified. Aside from pain control, another reason for intervention in her case was the concern that the subcapsular position of the pseudoaneurysm would have
I DISCUSSION Intrahepatic portohepatic venous shunt is extremely rare. Although most revorts sueeest .,- that these shunts are congenital in origin, symptoms do not usually develop until the fifth or sixth decade. These lesions are most commonly found in the right
b. a. Figure 2. (a) Portal vein catheter injection demonstrates a single portal vein
branch supplying the pseudoaneurysm. (b) Retrograde injection through the right hepatic vein demonstrates a single draining vein.
Stroz et a1
707
Volume 7 Number 5
Figure 3. Postembolization images show coils within pseudoaneurysm and absence of flow across fistula. (a)Portal vein catheter injection; (b) venous phase spiral CT image. p u t h e r a t r i s k for bleeding related t o s u b s e q u e n t minor t r a u m a . C e r t a i n technical factors were considered before t h e procedure. T h e 5-cm lesion required t h e largest coils available a t o u r institution t o completely fill t h e pseudoaneurysm a n d reduce t h e risk of distal embolization of t h e coils. An occlusive balloon i n t h e hepatic vein w a s a f u r t h e r precautionary m e a s u r e a g a i n s t distal embolization. A n a d d e d benefit of increasi n g venous s t a s i s i n t h e lesion w a s believed t o increase t h e probability of a successful outcome. T h e probability of bleeding from t h e pseudoaneurysm m a d e direct p u n c t u r e of t h e lesion a n u n a t t r a c t i v e option. Little is known a b o u t t h e n a t u r a l history of t h e s e lesions. Most lesions a r e entirely asymptomatic a n d a r e discovered incidentally (1,3,9,10). P a t i e n t s w i t h acquired i n t r a h e p a t i c portohepatic venous s h u n t , secondary t o cirrhosis a n d portal hypertension, usually p r e s e n t with encephalopathy (7,8). Decreasing tolerance t o toxic metabolites w i t h increasing a g e m a y explain t h e l a t e clinical manifestation (4,6,7). T h e r e h a s been a case report of a lesion associated w i t h abdominal pain (8). O u r p a t i e n t did not h a v e a n y symptoms suggestive of cirrhosis or encephalopathy, a n d all laboratory investigations w e r e normal. Abdominal sonography w i t h color Doppler is a n excellent method t o identify t h e s e lesions a n d t o identify t h e direction of flow. Confirmation
w i t h C T i s valuable w h e n suboptimal sonographic visualization of t h e liver occurs a n d t o identify associated abnormalities ( 8 , l l ) . T r e a t m e n t options include surgical resection a n d percutaneous embolization. T h e r e is a single report of a case of i n t r a h e p a t i c portohepatic venous s h u n t t r e a t e d w i t h coil embolization, which w a s performed t h r o u g h a n ileocolic vein d u r i n g laparotomy (12). Advantages of percutaneous embolization include reduced morbidity a n d preservation of hepatic parenchyma. T h i s is of particular importance w h e n faced w i t h a lesion i n t h e s e t t i n g of cirrhosis a n d portal hypertension w h e r e preservation of hepatic parenc h y m a would be a n i m p o r t a n t consideration.
Acknowledgment: We wish to t h a n k Guglielmina Picano for her assistance i n preparing this manuscript. References 1. Gheorghiu D, Leibowits 0, Bloom RA. Case report: asymptomatic aneurysmal intrahepatic porto-hepatic venous shunt-diagnosis by ultrasound. Clin Radiol 1994; 49:64-65. 2. Kerlan RK, Sollenberger RD, Palubinskas AJ, Raskin NH, Callen PW, Ehrenfeld WK. Portal-systemic encephalopathy due to a congenital portocaval shunt. AJR 1982; 139: 1013-1015. 3. Bezzi M, Mitchell DG, Needleman L, Goldberg BB. Iatrogenic aneurysmal portal-hepatic venous fistula. J Ultrasound Med 1988; 7:457-461.
4. Chagnon SF, Vallee CA, Barge J , Chevalier LJ, Le Gal J, Blery MV. Aneurysmal portahepatic venous fistula: report of two cases. Radiology 1986; 159:693-698. 5. Takayasu K, Moriyama N, Shima Y, et al. Spontaneous portal-hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology 1984; 86:945-948. 6. Raskin NH, Price JB, Fishman RA. Portal-systemic encephalopathy due to congenital intrahepatic shunts. N Engl J Med 1964; 270:225-229. 7. Park J H , Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR 1990; 155:527-528. 8. Jeng LBB, Chen MF. Intrahepatic portohepatic venous shunt: a case report of successful surgical resection. Arch Surg 1993; 128:349-352. 9. Wittich G, Jantsch H, Tscholakoff D. Congenital portosystemic shunt diagnosed by combined real-time and Doppler sonography. J Ultrasound Med 1985; 4:315-318. 10. Charnsangavej C, Soo CS, Bernardino ME, Chuang VP, Wallace S. Portalhepatic venous malformation: ultrasound, computed tomographic, and angiographic findings. Cardiovasc Intervent Radiol 1983; 6:109-111. 11. Mori H, Hayashi K, Fukuda T, et al. Intrahepatic portosystemic venous shunt: occurrence in patients with and without liver cirrhosis. AJR 1987; 149:711-714. 12. Ohtomo K, Furui S, Mitsunori S, Takashi K, Yuji I, Masahiro I. Case report: enormous intrahepatic communication between the portal vein and the hepatic vein. Clin Radiol 1986; 37513-514.