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Successful coil embolization of the congenital intrahepatic arteriovenous fistula associated with intrahepatic portal vein aneurysm in an infant
European Journal of Radiology Extra 45 (2003) 17 /19 http://intl.elsevierhealth.com/journals/ejrx
Successful coil embolization of the congenital intrahepatic arteriovenous fistula associated with intrahepatic portal vein aneurysm in an infant Tanzer Sancak *, Sadık Bilgic Section of Angiography and Interventional Radiology, Department of Radiology, School of Medicine, Ankara University, Ankara, Turkey Received 27 December 2001; received in revised form 26 March 2002; accepted 27 March 2002
Abstract Fistula between the hepatic artery and portal vein, known as hepatoportal arteriovenous fistula, most commonly occur secondary to trauma or malignancy. Congenital hepatoportal arteriovenous fistula is a rare anomaly which causes portal hypertension with the reversal of flow in the portal circulation, leading to hyperaemia and congestion of the bowel, hepatosplenomegaly, causing severe ascites, gastrointestinal bleeding, anaemia and malabsorption. We describe herein a case of a 7-month-old child diagnosed to have congenital high-flow hepatoportal arteriovenous fistula by digital subtraction angiography, who was successfully managed by percutaneous transcatheter embolization of the feeding hepatic artery branches. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Digital subtraction angiography; Liver diseases; Ultrasound
1. Introduction Portal hypertension due to shunting of hepatic arterial blood into the portal circulation is most commonly due to an acquired arterial-portal fistula (APF) [1]. Congenital arterial-portal shunts are rare, with only a limited number having been described [2,3].
2. Case report A 7-month-old boy was referred to our department, initially evaluated for failure to thrive. He had upper and lower gastrointestinal bleeding. Ultrasonography demonstrated anechoic lesion in the right lobe of the liver, which was interpreted as a portal vein aneurysm. Echo-Doppler examination showed intrahepatic hepatofugal portal slow blood flow (SS-A-270A, Toshiba, * Corresponding author. Present address: Mesa Camyolu Sitesi, B1 Blok A17, Yenikent, Cayyolu, Ankara, Turkey. Tel.: /90-312-2414421; fax: /90-312-4473-727 E-mail address: [email protected] (T. Sancak).
Japan). Hepatic arterial dilatation, thrombosis of extrahepatic portal vein and hepatosplenomegaly were noted. The blood biochemical determinations, coagulation studies and leucocyte and platelet counts were normal. Urine analysis was normal. Chemical testing of stool specimen was positive for occult blood. Anaesthesia was carried out by intravenous injection of ketamine (1 mg/kg). Digital subtraction angiography (DSA) was performed via right femoral artery (Multistar Plus/T.O.P., Siemens AG, Forchheim, Germany). A 0.035ƒ, 180-cm hydrophilic guide wire (Urolix, Urtech, GmbH, Germany) was then advanced to the hepatic artery with a 4F coeliac catheter (RC2, infinity, Cordis Europa N.V., Roden, The Netherlands). By using small amounts of contrast medium, coeliac and hepatic arterial DSA demonstrated a hypertrophic and tortuous hepatic artery which was feeding a very active intrahepatic arterioportal fistula located centrally in the right lobe, with immediate filling of the portal vein aneurysms and reflux to the portal vein (Fig. 1a). The portal hypertension was manifested by retrograde flow through the portal vein and into the superior mesenteric vein during the venous phase of hepatic angiography (Fig. 1b). Late phase of splenic arteriography showed
1571-4675/03/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. doi:10.1016/S1571-4675(03)00016-6
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T. Sancak, S. Bilgic / European Journal of Radiology Extra 45 (2002) 17 /19
splenomegaly and thrombosis of the splenic vein. In order to protect the remaining hepatic parenchyma, multiple hepatic arterial branches catheterised with
microcatheters (Rapidtransit, Roden, Cordis Europa N.V.) and then occluded with coils (Detachable Coil System, Detach-18-3-9-Spiral soft platinum, William Cook Europe, Bjaeverskov, Denmark). After this procedure, hepatic angiogram revealed that there was no filling of the portal vein aneurysms and reflux to the portal vein (Fig. 1c). Late phase of superior mesenteric artery angiogram showed the hepatopedal filling of the superior mesenteric vein and the portal vein.
3. Discussion Shunts between the hepatic arterial and portal venous circulation are usually acquired due to trauma, surgical and percutaneous hepatobiliary procedures, cirrhosis, tumours or rupture of a hepatic artery aneurysm into the portal vein [3]. APF and arterial-portal venous malformation (APVM) produce hyperdynamic portal hypertension with its potential sequela of variceal bleeding and ascites and occasionally, features of the mesenteric steal syndrome [3 /6]. When compared with arteriovenous shunts, hepatic arterial-to-portal venous shunts are unique in their lack of propensity to cause high output heart failure. This is likely due to flow restriction by hepatic sinusoids that are interposed between the lesion and the right heart. Attempts at treating the resultant portal hypertension with a portosystemic shunt may actually precipitate heart failure by redirecting arterialised portal blood into the systemic venous circulation [3]. Thus, the therapy should be aimed at obliteration of the shunt. Definitive surgical therapy would have necessitated resection of the nidus of the APVM. Alternative surgical approaches include partial hepatectomy and hepatic artery ligation. The latter may be ineffective due to the usual presence of a rich intrahepatic collateral arterial circulation. Successful surgical ligation of an intrahepatic APF has been reported, but is generally not feasible for deep parenchymal lesions [3]. Transcatheter embolotherapy has been successfully used to close APF and APVM [3,4,6]. The choice of appropriate embolic material or vascular occlusive device for effective therapy of an arterial-portal shunt should be dictated by the vascular anatomy of the lesion, especially the size and the number of arteriovenous communications and feeding arteries. An APF
Fig. 1. (a) Hepatic arterial angiogram demonstrated hypertrophied hepatic artery feeding an intrahepatic arterioportal fistula and early filling of the portal vein aneurysms. (b) The venous phase of hepatic angiography showed that retrograde flow through the superior mesenteric vein via the portal vein. (c) Right hepatic artery branches catheterized selectively with microcatheters and occluded with coils. Control coeliac angiogram showed that there was no filling of the portal vein.
T. Sancak, S. Bilgic / European Journal of Radiology Extra 45 (2002) 17 /19
may be successfully treated by occluding the single feeding artery or the draining vein or the fistula itself with an occlusive device, such as a detachable balloon, stainless steel coil or polyvinyl alcohol particles. The goal in treating an APVM is the obliteration of the vascular nidus because proximal occlusion of feeding arteries alone is usually ineffective [3]. In our case, stainless steel coil springs were used to embolize the main branches of right hepatic artery. Revascularisation of intrahepatic fistula after hepatic artery ligation or after successful angiographic embolization or portal vein occlusion should be considered [4]. Transcatheter embolotherapy should be considered as a preferable alternative for the treatment of an intrahepatic APF or APVM in infants.
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References [1] Balci NC, Semelka RC, Sandhu JS. Intrahepatic arterioportal fistula: gadolinium-enhanced 3D magnetic resonance angiography findings and angiographic embolization with steel coils. Magn Res Imag 1999;17:475 /558. [2] Fulton RL, Wolfel DA. Hepatic artery-portal vein arterio-venous fistula. Arch Surg 1970;100:307 /9. [3] Routh WD, Keller FS, Cain WS, Royal SA. Transcatheter embolization of a high-flow congenital intrahepatic arterial-portal venous malformation in an infant. J Pediatr Surg 1992;27:511 /4. [4] Romeo JM, Herrera N, Picouto LP. Angiographic embolization of intrahepatic arterioportal fistula. Br J Surg 1991;78:736 /7. [5] Altuntas B, Erden A, Karakurt C, Kut A, Senbil N, Yurdakul M. Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm. J Clin Ultrasound 1998;26:357 /60. [6] Agawala S, Dutta H, Bhatnagar V, Gulathi M, Paul S, Mitra D. Congenital hepatoportal arteriovenous fistula: report of a case. Surg Today 2000;30:268 /71.
Successful coil embolization of the congenital intrahepatic arteriovenous fistula associated with intrahepatic portal vein aneurysm in an infant Tanzer Sancak *, Sadık Bilgic Section of Angiography and Interventional Radiology, Department of Radiology, School of Medicine, Ankara University, Ankara, Turkey Received 27 December 2001; received in revised form 26 March 2002; accepted 27 March 2002
Abstract Fistula between the hepatic artery and portal vein, known as hepatoportal arteriovenous fistula, most commonly occur secondary to trauma or malignancy. Congenital hepatoportal arteriovenous fistula is a rare anomaly which causes portal hypertension with the reversal of flow in the portal circulation, leading to hyperaemia and congestion of the bowel, hepatosplenomegaly, causing severe ascites, gastrointestinal bleeding, anaemia and malabsorption. We describe herein a case of a 7-month-old child diagnosed to have congenital high-flow hepatoportal arteriovenous fistula by digital subtraction angiography, who was successfully managed by percutaneous transcatheter embolization of the feeding hepatic artery branches. # 2002 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Digital subtraction angiography; Liver diseases; Ultrasound
1. Introduction Portal hypertension due to shunting of hepatic arterial blood into the portal circulation is most commonly due to an acquired arterial-portal fistula (APF) [1]. Congenital arterial-portal shunts are rare, with only a limited number having been described [2,3].
2. Case report A 7-month-old boy was referred to our department, initially evaluated for failure to thrive. He had upper and lower gastrointestinal bleeding. Ultrasonography demonstrated anechoic lesion in the right lobe of the liver, which was interpreted as a portal vein aneurysm. Echo-Doppler examination showed intrahepatic hepatofugal portal slow blood flow (SS-A-270A, Toshiba, * Corresponding author. Present address: Mesa Camyolu Sitesi, B1 Blok A17, Yenikent, Cayyolu, Ankara, Turkey. Tel.: /90-312-2414421; fax: /90-312-4473-727 E-mail address: [email protected] (T. Sancak).
Japan). Hepatic arterial dilatation, thrombosis of extrahepatic portal vein and hepatosplenomegaly were noted. The blood biochemical determinations, coagulation studies and leucocyte and platelet counts were normal. Urine analysis was normal. Chemical testing of stool specimen was positive for occult blood. Anaesthesia was carried out by intravenous injection of ketamine (1 mg/kg). Digital subtraction angiography (DSA) was performed via right femoral artery (Multistar Plus/T.O.P., Siemens AG, Forchheim, Germany). A 0.035ƒ, 180-cm hydrophilic guide wire (Urolix, Urtech, GmbH, Germany) was then advanced to the hepatic artery with a 4F coeliac catheter (RC2, infinity, Cordis Europa N.V., Roden, The Netherlands). By using small amounts of contrast medium, coeliac and hepatic arterial DSA demonstrated a hypertrophic and tortuous hepatic artery which was feeding a very active intrahepatic arterioportal fistula located centrally in the right lobe, with immediate filling of the portal vein aneurysms and reflux to the portal vein (Fig. 1a). The portal hypertension was manifested by retrograde flow through the portal vein and into the superior mesenteric vein during the venous phase of hepatic angiography (Fig. 1b). Late phase of splenic arteriography showed
1571-4675/03/$ - see front matter # 2002 Elsevier Science Ireland Ltd. All rights reserved. doi:10.1016/S1571-4675(03)00016-6
18
T. Sancak, S. Bilgic / European Journal of Radiology Extra 45 (2002) 17 /19
splenomegaly and thrombosis of the splenic vein. In order to protect the remaining hepatic parenchyma, multiple hepatic arterial branches catheterised with
microcatheters (Rapidtransit, Roden, Cordis Europa N.V.) and then occluded with coils (Detachable Coil System, Detach-18-3-9-Spiral soft platinum, William Cook Europe, Bjaeverskov, Denmark). After this procedure, hepatic angiogram revealed that there was no filling of the portal vein aneurysms and reflux to the portal vein (Fig. 1c). Late phase of superior mesenteric artery angiogram showed the hepatopedal filling of the superior mesenteric vein and the portal vein.
3. Discussion Shunts between the hepatic arterial and portal venous circulation are usually acquired due to trauma, surgical and percutaneous hepatobiliary procedures, cirrhosis, tumours or rupture of a hepatic artery aneurysm into the portal vein [3]. APF and arterial-portal venous malformation (APVM) produce hyperdynamic portal hypertension with its potential sequela of variceal bleeding and ascites and occasionally, features of the mesenteric steal syndrome [3 /6]. When compared with arteriovenous shunts, hepatic arterial-to-portal venous shunts are unique in their lack of propensity to cause high output heart failure. This is likely due to flow restriction by hepatic sinusoids that are interposed between the lesion and the right heart. Attempts at treating the resultant portal hypertension with a portosystemic shunt may actually precipitate heart failure by redirecting arterialised portal blood into the systemic venous circulation [3]. Thus, the therapy should be aimed at obliteration of the shunt. Definitive surgical therapy would have necessitated resection of the nidus of the APVM. Alternative surgical approaches include partial hepatectomy and hepatic artery ligation. The latter may be ineffective due to the usual presence of a rich intrahepatic collateral arterial circulation. Successful surgical ligation of an intrahepatic APF has been reported, but is generally not feasible for deep parenchymal lesions [3]. Transcatheter embolotherapy has been successfully used to close APF and APVM [3,4,6]. The choice of appropriate embolic material or vascular occlusive device for effective therapy of an arterial-portal shunt should be dictated by the vascular anatomy of the lesion, especially the size and the number of arteriovenous communications and feeding arteries. An APF
Fig. 1. (a) Hepatic arterial angiogram demonstrated hypertrophied hepatic artery feeding an intrahepatic arterioportal fistula and early filling of the portal vein aneurysms. (b) The venous phase of hepatic angiography showed that retrograde flow through the superior mesenteric vein via the portal vein. (c) Right hepatic artery branches catheterized selectively with microcatheters and occluded with coils. Control coeliac angiogram showed that there was no filling of the portal vein.
T. Sancak, S. Bilgic / European Journal of Radiology Extra 45 (2002) 17 /19
may be successfully treated by occluding the single feeding artery or the draining vein or the fistula itself with an occlusive device, such as a detachable balloon, stainless steel coil or polyvinyl alcohol particles. The goal in treating an APVM is the obliteration of the vascular nidus because proximal occlusion of feeding arteries alone is usually ineffective [3]. In our case, stainless steel coil springs were used to embolize the main branches of right hepatic artery. Revascularisation of intrahepatic fistula after hepatic artery ligation or after successful angiographic embolization or portal vein occlusion should be considered [4]. Transcatheter embolotherapy should be considered as a preferable alternative for the treatment of an intrahepatic APF or APVM in infants.
19
References [1] Balci NC, Semelka RC, Sandhu JS. Intrahepatic arterioportal fistula: gadolinium-enhanced 3D magnetic resonance angiography findings and angiographic embolization with steel coils. Magn Res Imag 1999;17:475 /558. [2] Fulton RL, Wolfel DA. Hepatic artery-portal vein arterio-venous fistula. Arch Surg 1970;100:307 /9. [3] Routh WD, Keller FS, Cain WS, Royal SA. Transcatheter embolization of a high-flow congenital intrahepatic arterial-portal venous malformation in an infant. J Pediatr Surg 1992;27:511 /4. [4] Romeo JM, Herrera N, Picouto LP. Angiographic embolization of intrahepatic arterioportal fistula. Br J Surg 1991;78:736 /7. [5] Altuntas B, Erden A, Karakurt C, Kut A, Senbil N, Yurdakul M. Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm. J Clin Ultrasound 1998;26:357 /60. [6] Agawala S, Dutta H, Bhatnagar V, Gulathi M, Paul S, Mitra D. Congenital hepatoportal arteriovenous fistula: report of a case. Surg Today 2000;30:268 /71.