Severe Portal Hypertension Secondary to Arterio-portal Fistula
Severe Portal Hypertension Secondary to Arterio-portal Fistula
Background: Arterio-portal fistulas (APFs) are rare vascular disorders of various origins that can lead to severe portal hypertension. Even if surgery was initially the treatment of choice, more recently, interventional radiological procedures have been considered as the first line therapeutic option.
Case Report: A man with no history of liver disease was admitted for abdominal pain and distension. Abdominal ultrasonography with Doppler, magnetic resonance imaging and computed tomography (CT) scan showed ascites, splenomegaly and a probable APF between the left branches of both the hepatic artery and portal vein, associated with hepatofugal portal flow. Upper gastrointestinal endoscopy revealed large oesophageal varices without bleeding. A celiac and mesenteric arteriography and a splenic arteriography were performed and confirmed the existence of multiple intrahepatic APFs. The initial treatment consisted of two sessions of percutaneous transcatheter endovascular embolization. Unfortunately, ascites worsened, and the patient did not respond to diuretic treatment. Therefore, a surgical treatment was considered to be the only suitable treatment because of the absence of improvement after embolization procedures. A left hepatectomy with hepatic artery ligation was performed. Clinical evolution was favourable; an improvement of ascites was obtained; control ultrasonography and CT scan disclosed no residual haemodynamic abnormality; and the portal vein was normal with a hepatopedal flow. Currently, 12 months after surgery, the clinical condition of the patient is good.
Conclusion: Percutaneous treatment of portal hypertension by embolizing multiple large APF has been described to be an effective method. Nevertheless, failure of such conservative treatment is possible and must lead to a salvage surgery.
Arterio-portal fistulas (APFs) are rare vascular disorders of various origins. The clinical spectrum of presentation ranges from symptom-free individuals to patients with severe portal hypertension. In the past, the majority of patients have required a surgical treatment. However, interventional radiological procedures are now being performed with increasing frequency. Angiography confirms the diagnosis and in many cases allows definitive interventional radiological treatment. Here, we report the case of a patient in whom after failure of an interventional radiological treatment, salvage surgical treatment was required.
A 63-year-old man with no history of liver disease, nor a remote history of trauma or liver biopsy, was first admitted for abdominal pain and distension in 2005. In the last month, he had lost 5 kg. Laboratory data included a serum albumin level of 3.0 g/dl; a haemoglobin value of 105 g/l; haematocrit level of 28%; a blood platelet count of 80 000/ml; a leucocyte count of 3500 cells/ml; and a prothombin rate of 61%; total bilirubin was 2 mg/dl, and aspartate aminotransferase and alanine aminotransferase were within the normal range. Abdominal ultrasonography with Doppler, magnetic resonance imaging (Figure 1) and computed tomography (CT) scan showed ascites, splenomegaly and a probable APF between the left branches of both the hepatic artery and portal vein, associated with hepatofugal portal flow. Upper gastrointestinal endoscopy revealed large oesophageal varices without bleeding. A celiac and mesenteric arteriography and a splenic arteriography were performed and confirmed the existence of multiple intrahepatic APFs arising from an enlarged hepatic artery, with regurgitation of the contrast medium into the main portal vein (Figure 2). In view of these findings, the portal hypertension was considered as caused by idiopathic multiple large APFs. The initial treatment consisted in a first session of percutaneous transcatheter endovascular embolization in an attempt to obtain occlusion of the fistulas. A 5 Fr catheter was placed into the origin of the hepatic artery, followed by coaxial passage of a 0.018-in. infusion microcatheter into the left hepatic artery. However, selective exploratory angiography revealed that the APF was actually formed by multiple small communications between the left arterial and portal branches. Embolization with a detachable balloon was therefore considered not to be feasible during the procedure. Embolization with N-butyl-cyanoacrylate (NBCA) and microcoils was performed. After embolization, the control celiac arteriography confirmed the occlusion of all APF. A control Doppler performed 24 h later showed a markedly reduced hepatopedal portal flow as compared with the pre-embolization hepatofugal flow. There was no complication of the radiological procedure; liver function was consistently normal after embolization. A new control Doppler performed 1 week after embolization showed a situation very similar to that observed during the first examinations, before any attempt at treatment. The flow was hepatofugal. A second angiography was therefore performed 2 weeks later and a second attempt of treatment by microcoils and NBCA was made. Unfortunately, ascites worsened, and the patient did not respond to diuretic treatment. It was therefore necessary to perform several abdominal paracenteses. Therefore, a surgical treatment was considered to be the only suitable treatment because of the absence of improvement after embolization procedures. In September 2005, a left hepatectomy with hepatic artery ligation was performed. During the procedure, the patient received 28 red blood cell (RBC) units and 1700 ml of autologous transfusion (using the Haemonetics Cellsaver). Histopathological examination of the liver showed the existence of large malformative vessels in the hepatic hilum; the portal vein was enlarged and its wall was modified by lesions of intimal hyperplasia and muscular proliferation; the hepatic arteries were occluded by recent thromboses; and numerous large and muscularized venous vessels, forming a densely interconnected network, were running along the main hepatic vessels. Liver parenchyma showed a normal architecture, without fibrosis or cirrhosis, without evidence of nodular hyperplasia or microcirculatory changes. Clinical evolution was favourable; an improvement of ascites was obtained; control ultrasonography and CT scan disclosed no residual haemodynamic abnormality; and the portal vein was normal with a hepatopedal flow. Currently, 12 months after surgery, the clinical condition of the patient is good (Figure 3).
(Enlarge Image)
Figure 1.
Magnetic resonance imaging appearance of intrahepatic arterio-portal fistulas (APFs) arising from an enlarged hepatic artery (HA), with regurgitation of the contrast medium into the main portal vein (PV).
(Enlarge Image)
Figure 2.
Angiographic appearance of multiple intrahepatic arterio-portal fistulas (APFs) (arrowhead) arising from an enlarged hepatic artery before treatment.
(Enlarge Image)
Figure 3.
Computed tomography scan appearance of the liver after surgery, showing no arterio-portal fistula left.
Well-known aetiologies of APF include blunt or penetrating trauma, rupture of the hepatic artery, iatrogenic causes (especially liver biopsy, ... ), hepatitis, liver cirrhosis, neoplasm and infections. However, as reported here, congenital or idiopathic arterio-portal venous fistulas may constitute up to one-third of the cases. In the case of portal hypertension secondary to a large APF, closure of the shunt is considered as an efficient therapy. Historically, the first treatments performed were the surgical ligation of the artery supplying the arterio-portal venous fistula or the simple resection of the vascular anomaly. More recently, because of the quite high operative mortality of such surgical procedures, together with the rapid development of various endovascular techniques, percutaneous embolization of APF has been increasingly performed in place of surgery. Hepatic infarction is one of its potential complications. As there are intrahepatic collateral circulation pathways, the embolization of all hepatic artery branches communicating with portal branches from the distal to the proximal part of the fistula can be necessary. Embolic agents include steel coils, detachable balloons, NBCA, lipiodol and polyvinyl alcohol particles. In some cases, several embolization sessions are necessary before clinical improvement is achieved. This was attempted in our patient. The failure of this therapeutic approach was likely to be owing to the presence of a very large APF, with multiple collateral branches that precluded complete embolization. Such an outcome has been reported very rarely. In our case, the continuous worsening of the clinical condition required a second-line surgical treatment, which was eventually able to achieve the restoration of a normal haemodynamic situation and the control of portal hypertension. Moreover, as reported here, surgical resection of APF alone may be unsuccessful, and surgical ligation of the hepatic artery may be necessary. In some rare cases, a liver transplantation represents the last therapeutic resource. Very recently, a classification with therapeutic implications has been proposed. A detailed radiologic evaluation can lead to differentiate small peripheral intrahepatic APFs (type 1) and large central APFs (type 2), as observed in our patient. Type 1 usually resolves spontaneously, whereas type 2 can cause severe complications including portal hypertension and hepatic parenchymal changes. This latter type of APFs always needs treatment and in the first possible instant by transcatheter embolization with a good prognosis. Nevertheless, our case strongly illustrates that surgical approaches should be reserved for complex cases. Congenital APFs (type 3) are diffuse and intrahepatic and can be the most difficult to manage.
In conclusion, percutaneous treatment of portal hypertension by embolizing multiple large APF has been described to be an effective method. Nevertheless, failure of such a conservative treatment is possible and may lead to a salvage surgery.
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Abstract and Introduction
Abstract
Background: Arterio-portal fistulas (APFs) are rare vascular disorders of various origins that can lead to severe portal hypertension. Even if surgery was initially the treatment of choice, more recently, interventional radiological procedures have been considered as the first line therapeutic option.
Case Report: A man with no history of liver disease was admitted for abdominal pain and distension. Abdominal ultrasonography with Doppler, magnetic resonance imaging and computed tomography (CT) scan showed ascites, splenomegaly and a probable APF between the left branches of both the hepatic artery and portal vein, associated with hepatofugal portal flow. Upper gastrointestinal endoscopy revealed large oesophageal varices without bleeding. A celiac and mesenteric arteriography and a splenic arteriography were performed and confirmed the existence of multiple intrahepatic APFs. The initial treatment consisted of two sessions of percutaneous transcatheter endovascular embolization. Unfortunately, ascites worsened, and the patient did not respond to diuretic treatment. Therefore, a surgical treatment was considered to be the only suitable treatment because of the absence of improvement after embolization procedures. A left hepatectomy with hepatic artery ligation was performed. Clinical evolution was favourable; an improvement of ascites was obtained; control ultrasonography and CT scan disclosed no residual haemodynamic abnormality; and the portal vein was normal with a hepatopedal flow. Currently, 12 months after surgery, the clinical condition of the patient is good.
Conclusion: Percutaneous treatment of portal hypertension by embolizing multiple large APF has been described to be an effective method. Nevertheless, failure of such conservative treatment is possible and must lead to a salvage surgery.
Introduction
Arterio-portal fistulas (APFs) are rare vascular disorders of various origins. The clinical spectrum of presentation ranges from symptom-free individuals to patients with severe portal hypertension. In the past, the majority of patients have required a surgical treatment. However, interventional radiological procedures are now being performed with increasing frequency. Angiography confirms the diagnosis and in many cases allows definitive interventional radiological treatment. Here, we report the case of a patient in whom after failure of an interventional radiological treatment, salvage surgical treatment was required.
A 63-year-old man with no history of liver disease, nor a remote history of trauma or liver biopsy, was first admitted for abdominal pain and distension in 2005. In the last month, he had lost 5 kg. Laboratory data included a serum albumin level of 3.0 g/dl; a haemoglobin value of 105 g/l; haematocrit level of 28%; a blood platelet count of 80 000/ml; a leucocyte count of 3500 cells/ml; and a prothombin rate of 61%; total bilirubin was 2 mg/dl, and aspartate aminotransferase and alanine aminotransferase were within the normal range. Abdominal ultrasonography with Doppler, magnetic resonance imaging (Figure 1) and computed tomography (CT) scan showed ascites, splenomegaly and a probable APF between the left branches of both the hepatic artery and portal vein, associated with hepatofugal portal flow. Upper gastrointestinal endoscopy revealed large oesophageal varices without bleeding. A celiac and mesenteric arteriography and a splenic arteriography were performed and confirmed the existence of multiple intrahepatic APFs arising from an enlarged hepatic artery, with regurgitation of the contrast medium into the main portal vein (Figure 2). In view of these findings, the portal hypertension was considered as caused by idiopathic multiple large APFs. The initial treatment consisted in a first session of percutaneous transcatheter endovascular embolization in an attempt to obtain occlusion of the fistulas. A 5 Fr catheter was placed into the origin of the hepatic artery, followed by coaxial passage of a 0.018-in. infusion microcatheter into the left hepatic artery. However, selective exploratory angiography revealed that the APF was actually formed by multiple small communications between the left arterial and portal branches. Embolization with a detachable balloon was therefore considered not to be feasible during the procedure. Embolization with N-butyl-cyanoacrylate (NBCA) and microcoils was performed. After embolization, the control celiac arteriography confirmed the occlusion of all APF. A control Doppler performed 24 h later showed a markedly reduced hepatopedal portal flow as compared with the pre-embolization hepatofugal flow. There was no complication of the radiological procedure; liver function was consistently normal after embolization. A new control Doppler performed 1 week after embolization showed a situation very similar to that observed during the first examinations, before any attempt at treatment. The flow was hepatofugal. A second angiography was therefore performed 2 weeks later and a second attempt of treatment by microcoils and NBCA was made. Unfortunately, ascites worsened, and the patient did not respond to diuretic treatment. It was therefore necessary to perform several abdominal paracenteses. Therefore, a surgical treatment was considered to be the only suitable treatment because of the absence of improvement after embolization procedures. In September 2005, a left hepatectomy with hepatic artery ligation was performed. During the procedure, the patient received 28 red blood cell (RBC) units and 1700 ml of autologous transfusion (using the Haemonetics Cellsaver). Histopathological examination of the liver showed the existence of large malformative vessels in the hepatic hilum; the portal vein was enlarged and its wall was modified by lesions of intimal hyperplasia and muscular proliferation; the hepatic arteries were occluded by recent thromboses; and numerous large and muscularized venous vessels, forming a densely interconnected network, were running along the main hepatic vessels. Liver parenchyma showed a normal architecture, without fibrosis or cirrhosis, without evidence of nodular hyperplasia or microcirculatory changes. Clinical evolution was favourable; an improvement of ascites was obtained; control ultrasonography and CT scan disclosed no residual haemodynamic abnormality; and the portal vein was normal with a hepatopedal flow. Currently, 12 months after surgery, the clinical condition of the patient is good (Figure 3).
(Enlarge Image)
Figure 1.
Magnetic resonance imaging appearance of intrahepatic arterio-portal fistulas (APFs) arising from an enlarged hepatic artery (HA), with regurgitation of the contrast medium into the main portal vein (PV).
(Enlarge Image)
Figure 2.
Angiographic appearance of multiple intrahepatic arterio-portal fistulas (APFs) (arrowhead) arising from an enlarged hepatic artery before treatment.
(Enlarge Image)
Figure 3.
Computed tomography scan appearance of the liver after surgery, showing no arterio-portal fistula left.
Well-known aetiologies of APF include blunt or penetrating trauma, rupture of the hepatic artery, iatrogenic causes (especially liver biopsy, ... ), hepatitis, liver cirrhosis, neoplasm and infections. However, as reported here, congenital or idiopathic arterio-portal venous fistulas may constitute up to one-third of the cases. In the case of portal hypertension secondary to a large APF, closure of the shunt is considered as an efficient therapy. Historically, the first treatments performed were the surgical ligation of the artery supplying the arterio-portal venous fistula or the simple resection of the vascular anomaly. More recently, because of the quite high operative mortality of such surgical procedures, together with the rapid development of various endovascular techniques, percutaneous embolization of APF has been increasingly performed in place of surgery. Hepatic infarction is one of its potential complications. As there are intrahepatic collateral circulation pathways, the embolization of all hepatic artery branches communicating with portal branches from the distal to the proximal part of the fistula can be necessary. Embolic agents include steel coils, detachable balloons, NBCA, lipiodol and polyvinyl alcohol particles. In some cases, several embolization sessions are necessary before clinical improvement is achieved. This was attempted in our patient. The failure of this therapeutic approach was likely to be owing to the presence of a very large APF, with multiple collateral branches that precluded complete embolization. Such an outcome has been reported very rarely. In our case, the continuous worsening of the clinical condition required a second-line surgical treatment, which was eventually able to achieve the restoration of a normal haemodynamic situation and the control of portal hypertension. Moreover, as reported here, surgical resection of APF alone may be unsuccessful, and surgical ligation of the hepatic artery may be necessary. In some rare cases, a liver transplantation represents the last therapeutic resource. Very recently, a classification with therapeutic implications has been proposed. A detailed radiologic evaluation can lead to differentiate small peripheral intrahepatic APFs (type 1) and large central APFs (type 2), as observed in our patient. Type 1 usually resolves spontaneously, whereas type 2 can cause severe complications including portal hypertension and hepatic parenchymal changes. This latter type of APFs always needs treatment and in the first possible instant by transcatheter embolization with a good prognosis. Nevertheless, our case strongly illustrates that surgical approaches should be reserved for complex cases. Congenital APFs (type 3) are diffuse and intrahepatic and can be the most difficult to manage.
In conclusion, percutaneous treatment of portal hypertension by embolizing multiple large APF has been described to be an effective method. Nevertheless, failure of such a conservative treatment is possible and may lead to a salvage surgery.
CLICK HERE for subscription information about this journal.
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