Primary Budd-Chiari syndrome: outcome of endovascular management for suprahepatic venous obstruction

OBJECTIVE: Primary Budd-Chiari syndrome (BCS) is a rare form of hepatic venous outflow obstruction at the suprahepatic inferior vena cava (IVC), the hepatic veins, or both. We assessed our 4-year experience in the management of BCS to evaluate the results of our methods of care. METHODS: We conducted a retrospective review of a nonrandomized clinical trial conducted in three teaching hospitals. Among 28 primary BCS patients, 9 remained in medical treatment only, and 19 who failed to respond to medical treatment received additional endovascular (n = 17) or surgical therapy (n = 2). Nine underwent IVC balloon angioplasty alone, 6 had angioplasty plus stents, and 2 had transjugular intrahepatic portosystemic shunts (TIPS) for hepatic vein lesions. One patient had a mesoatrial bypass; another had liver transplantation. Immediate response to the therapy was assessed with angiography and ultrasonography based on anatomic and/or hemodynamic correction or reduction of the lesion. Subsequent assessment of portal hypertension status was made with periodic clinical and laboratory evaluation (eg, ultrasonography, liver biopsy). RESULTS: Twenty-six patients had had IVC stenosis or occlusion by focal or segmental lesion. Two patients had hepatic vein outlet obstruction. There was no evidence of coagulopathy as the pathogenesis; all were related to membranous obstruction of the vena cava. Excellent immediate response to the endovascular therapy and subsequent relief of portal hypertension were achieved in 14 patients. Four patients had restenosis or progression of the residual lesion within 2 years; three responded to repeated stenting. Primary patency was 76.5%, and primary assisted patency was 94.1%. Two patients with TIPS and two with surgical therapy maintained excellent results. The medical treatment remained effective only in a limited group of 6 (21.4%) of the 28 patients. CONCLUSIONS: In BCS, both endovascular and surgical interventions provide excellent results and potentially halt liver parenchymal deterioration caused by portal hypertension. Liver transplantation remains the ultimate solution for advanced liver failure.     

Duplex ultrasonography in portal vein thrombosis

Summary Duplex ultrasonography (US) was performed in 23 patients with portal vein thrombosis. In 7 children and 16 adults, duplex US demonstrated intraluminal thrombi (4 cases), thrombus and periportal collaterals (2 cases), and cavernomatous transformation of the portal vein (17 cases). Real-time US is a suitable means of demonstrating the anatomy of the portal venous system; pulsed Doppler provided information on the patency and direction of portal flow. In a few cases, Doppler color flow imaging was used, which was helpful in the examination of branches of the portal vein and the superior mesenteric and splenic veins. Portal duplex US is suggested in selected groups of patients before surgery to demonstrate the presence or absence of portal vein thrombosis.     

Intraoperative stent placement in the portal vein during or after liver transplantation.

The purpose of this research was to evaluate the intermediate effectiveness of intraoperative portal vein stent placement for portal venous stenosis in liver transplantation. We attempted intraoperative portal vein stent placement in 44 portal venous anastomotic stenoses in 36 patients. All patients underwent stent placement via either the inferior or superior mesenteric vein. A total of 22 patients underwent portal vein stent placement simultaneously with liver transplantation, and 14 patients underwent stent placement 1-25 days (mean 5.93 days) after liver transplantation. Of the 22 patients, there was portal vein occlusion in 3 patients and small portal vein (<6 mm) in 10 patients (2.5-5.7 mm; mean size 3.9 mm). Patient follow-up included clinical and laboratory data collection, Doppler ultrasonography (US), and computed tomography (CT). Intraoperative portal vein stent placement was technically successful in all of our study patients, even in 6 patients with total occlusion of the portal vein. A total of 10 study patients underwent thrombectomy of the portal vein, 1 underwent patient portosystemic shunt ligation, and 7 patients had both procedures simultaneously. Portal venous patency has been maintained for 0-56 months (mean 16 months) in 42 (95%) of the 44 stent placements. In conclusion, intraoperative portal vein stent placement is an effective and long lasting treatment modality for treat portal venous stenosis, especially in patients with portal vein occlusion or small sized portal vein.     

Effect of portosystemic shunting on PGI2 and glucagon levels in humans.

OBJECTIVE: This study determined if the proposed mediators of splanchnic blood flow, prostacyclin and glucagon, were elevated in patients with portal hypertension undergoing portal systemic shunts. SUMMARY BACKGROUND DATA: Chronic portal hypertension results in increased portal venous pressure and increased splanchnic blood flow. Animal studies have suggested prostacyclin or glucagon, potent vasodilators, as potential mediators of this increased flow. Correlative clinical studies have been difficult to perform due to the wide variation in degree of portal-systemic shunting and the frequent association of parenchymal liver disease in patients with cirrhosis. METHODS: The authors measured portal and systemic hemodynamics in PGI2 and glucagon levels in patients with portal hypertension due to cirrhosis (partial portal systemic shunt) and Budd-Chiari syndrome (complete portal systemic shunt) undergoing portal systemic shunts and in porto normotensive patients undergoing exploratory laparotomies. RESULTS: PGI2 levels in portal hypertension were significantly increased over normal, and prostacyclin in Budd-Chiari patients were increased significantly over patients with cirrhosis. Both PGI2 and portal venous pressure decreased significantly after portal systemic shunting, and prostacyclin levels correlated directly with portal venous pressure (R = 0.37, p < 0.05). CONCLUSIONS: This is the first evidence in humans supporting the hypothesis that PGI2 is elevated in portal hypertension and is related to both the degree of portal venous obstruction and portal pressure. PGI2 probably has a role in the abnormal splanchnic hemodynamics of human portal hypertension.     

Intraoperative ultrasound guided portal venous thrombectomy in living donor liver transplantation recipient surgery.

Portal vein reconstruction in liver transplantation from live donor grafts has major challenging factors in cases with portal venous thrombosis (PVT). To overcome this critical surgical challenge, we devised a novel technique, intraoperative ultrasonography (IOUS)-guided thrombectomy of the portal vein. IOUS-guided thrombectomy was applied to the 10 patients whose PVT extended to the splenomesenteric junction. In these patients, closed thin scissors were inserted from the stump of the recipient portal vein under ultrasound guidance and the thrombus was dissected from the venous wall. The application of IOUS-guided thrombectomy in patients with moderate to severe PVT led to a 3-year patency rate of 83%, comparable to that of simple thrombectomy applied to partial or minimal (grade I-II) PVT (83%). IOUS-guided thrombectomy is helpful to adequately remove severe thrombi from the deep lumen of the portal vein, provided the procedure was carried out by an experienced surgeon with adequate preparation for unexpected venous injuries.     

Liver transplantation using cavoportal transposition: an effective treatment in patients with complete splanchnic venous thrombosis

Complete venous thrombosis of the splanchnic system remains a major challenge in liver transplantation surgery. Some of these patients have been treated successfully by multivisceral transplantation. Cavoportal transposition is another alternative to treat these patients. We reviewed our single-center experience with this technique. Five patients with operatively confirmed complete splanchnic thrombosis were transplanted with the cava portal transposition technique. All survived the procedure; 60% survived long term. This technique is a useful salvage procedure in moribund patients with diffuse portal thrombosis who would otherwise rapidly succumb.     

Living Donor Liver Transplantation for Congenital Absence of the Portal Vein

The congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. Liver transplantation (OLT) may be indicated for patients with symptomatic CAPV refractory to medical treatment, especially due to hyperammonemia, portosystemic encephalopathy, hepatopulmonary syndrome, or hepatic tumors. Because portal hypertension and collateral circulation do not occur with CAPV, significant splanchnic congestion may occur when the portocaval shunt is totally clamped during portal vein (PV) reconstruction in OLT. This phenomenon results in severe bowel edema and hemodynamic instability, which negatively impact the patient's condition and postoperative recovery. We have successfully reconstructed the PV in living donor liver transplantation (LDLT) using a venous interposition graft, which was anastomosed end-to-side to the portocaval shunt by a partial side-clamp, using a patent round ligament of the liver, which was anastomosed end-to-end to the graft PV with preservation of both the portal and caval blood flows. Owing to the differences in anatomy among patients, at LDLT for CAPV liver transplant surgeons should seek to preserve both portal and caval blood flows.     

Graft interposition splenocaval shunt for total or selective decompression of portal hypertension

A new operation for selective or total decompression of the portal venous system in cases of intrahepatic portal hypertension is described. It involves interposition of a large-caliber Dacron graft between the splenic vein and the inferior vena cava. The graft-interposition splenocaval shunt is performed readily and quickly, satisfying the variable hemodynamic needs of patients with portal hypertension. It can be either selective (S-SCS) or total (T-SCS) from the beginning, or a T-SCS may be converted subsequently to a S-SCS should surgically induced hepatic decompensation supervene. It is less demanding technically than distal splenorenal shunt (D-SRS). The S-SCS conserves portal venous perfusion of the liver, preserves hepatocellular function and architecture at the preoperative levels, avoids precipitation of postshunt portal-systemic encephalopathy, and decompresses gastric-esophageal varices with prevention of further variceal bleeding even better than D-SRS. One hundred percent graft patency has been obtained, and the surgical results have been superior to those following portacaval shunt in patients with large liver blood flow and relative benignity of the liver disease, be it cirrhosis or noncirrhotic portal fibrosis. In patients with advanced cirrhosis, variceal bleeding, and small liver blood flows, T-SCS would be indicated. Patients of this category obtained inferior surgical results and had operative deaths (16.7%) following S-SCS. The concept of the operation has merits and deserves further evaluation.     

Successful Treatment of a Patient With Diffuse Portosplenomesenteric Thrombosis Using a Pericholedochal Varix for Portal Flow Reconstruction During Deceased Donor Liver Transplantation: A Case Report

Portal vein thrombosis remains a challenging issue in liver transplantation. When thrombectomy is not feasible due to diffuse portosplenomesenteric thrombosis, other modalities are adapted such as the use of a jump graft or portal tributaries or even multivisceral transplantation. For patients with diffuse thrombosis of the splanchnic venous system, a large pericholedochal varix can be a useful vessel for providing splanchnic blood flow to the graft and for relieving portal hypertension. We report our experience of successfully treating a patient with diffuse portosplenomesenteric thrombosis using a pericholedochal varix for portal flow reconstruction during deceased donor liver transplantation and eventually preventing unnecessary multivisceral transplantation. A 56-year-old man diagnosed with liver cirrhosis due to hepatitis B underwent deceased donor liver transplantation due to refractory ascites. Preoperative imaging revealed diffuse portosplenomesenteric thrombosis with large amount of ascites. During the operation, dissection of the main portal vein was not possible due to the development of multiple large pericholedochal varices and cavernous change of the main portal vein. After outflow reconstruction, portal inflow was restored by anastomosing the graft portal vein to a large pericholedochal varix. Postoperatively, although abdominal computed tomography scan showed stenosis of portal vein anastomosis site, liver function tests improved, and Doppler sonogram revealed no flow disturbance. During follow-up, the patient repeatedly developed hydrothorax and ascites. In addition, stenosis of the portal vein anastomosis and thrombosis of the portomesenteric system still remained. The patient underwent transhepatic portal vein stent insertion. After portal vein stent insertion, hydrothorax and ascites improved and the extent of thrombosis of the portomesenteric system decreased without anticoagulation therapy. In conclusion, enlarged pericholedochal varix in patients with totally obliterated splanchnic veins can be a source of useful inflow to restore portal flow and decrease the extent of thrombosis, thereby preventing unnecessary multivisceral transplantation.     

Renoportal anastomosis in right lobe living donor liver transplantation: report of a case

End-stage liver disease is often accompanied by thrombosis of the portal vein and the formation of splanchnic collateral vessels. Successful liver transplantation in such situations is more likely if the surgeon uses a strategy to establish a graft inflow. A 59-year-old male with a decompensated liver secondary to idiopathic portal hypertension underwent living donor liver transplantation (LDLT) using a right lobe liver graft donated from his son. His portal venous trunk was atrophied and a splenorenal shunt drained the mesenteric venous flow into the systemic circulation. LDLT was performed with renoportal anastomosis (RPA) using his right internal jugular vein as an interposed venous graft, without dissecting the collateral vessels. Although he developed temporary functional hyperbilirubinemia, he was discharged from the hospital 23 days after LDLT. This case suggests that RPA is a useful technique to manage patients with an obstructed portal vein and a splenorenal shunt.     

Effect of prostaglandin E1 and nitroglycerin on portal venous flow during hypothermic extracorporeal circulation: Assessment by transesophageal echography

BACKGROUND: Although several vasodilators are used to control vascular resistance during cardiac surgery, their effects on splanchnic circulation during extracorporeal circulation are unknown. We designed the present noninvasive study to evaluate the effect of prostaglandin E1 and nitroglycerin on portal venous flow during extracorporeal circulation using transesophageal echography. METHODS: We included 26 patients undergoing cardiac surgery with moderate hypothermic extracorporeal circulation in this study. After obtaining hemodynamic stability under extracorporeal circulation, we measured portal venous diameter, mean flow velocity and the velocity time integral using transesophageal echography and calculated portal venous flow. The patients were assigned to two groups where either prostaglandin E1 (N = 13) or nitroglycerin (N = 13) was administered intravenously to maintain perfusion pressure at the level of 70 mmHg. We measured the same parameters 20 and 40 min following administration of the drug. RESULTS: Visualization of the portal vein was obtained by transesophageal echography in anesthetized patients. Calculated portal venous flow significantly increased in the prostaglandin E1 group, while it did not alter in the nitroglycerin group. CONCLUSION: The present results indicate that transesophageal echography may be a feasible tool to assess portal venous flow, and that prostaglandin E1 may improve the blood distribution to the splanchnic area and the liver during hypothermic extracorporeal circulation.     

Preeminence of lesser splanchnic blood flow in selected patients with generalized portal hypertension

Although restricted transhepatic portal flow is necessary for development of generalized portal hypertension (GPH), increased splanchnic arterial inflow also contributes to GPH and its clinical sequelae. In this context, we describe 7 male and 6 female patients (mean age 48 years) in whom the lesser splanchnic (gastrosplenic) system played a key role in the signs and symptoms of GPH. These 13 patients (9 with hepatic cirrhosis, 3 with primary myeloproliferative disorder, and 1 with extrahepatic portal block) shared common features of massive splenomegaly, huge splenofundic gastric varices, often with a prominent natural shunt to the left renal vein. Total or near total splenectomy alone or combined where appropriate with coronary vein ligation was effective in controlling varix hemorrhage (10 patients), ascites (3), or complications of an enlarged spleen-anorexia and abdominal pain (3), hemolytic anemia (1) and profound thrombocytopenia with severe epistaxis (1). Intraoperative jejunal portal venography was crucial in operative management in order to establish definitively the presence or absence of coronary venous collaterals, and when present, to verify their operative ligation. These distinctive patients illustrate: 1) GPH is a heterogeneous syndrome of divergent splanchnic circulatory patterns, a feature which should be taken into account in selecting operative treatment; 2) one well-defined subgroup displays prominent hyperdynamic lesser splanchnic and specifically, splenic blood flow as a major contributor to clinical complications; and 3) within this subgroup, splenectomy combined with documented absence or surgical interruption of coronary venous collaterals as corroborated by intraoperative portography is effective alternative treatment.     

Reappraisal of intraoperative ultrasonography for pancreatobiliary carcinomas: assessment of malignant portal venous invasion

BACKGROUND: Conventional preoperative imaging modalities are unreliable for assessing portal venous invasion by pancreatobiliary carcinoma. We evaluated the usefulness of intraoperative ultrasonography for detecting pancreatobiliary carcinoma and assessing portal venous invasion, compared with other imaging modalities. METHODS: Ninety-one patients with pancreatic carcinoma (n = 66) or bile duct carcinoma (n = 25) underwent ultrasonography, computed tomography, angiography, and endoscopic ultrasonography preoperatively. All these patients underwent tumor resection, with (n = 23) or without (n = 68) portal vein resection, after intraoperative ultrasonography. Portal venous invasion was histologically examined in all patients. RESULTS: Intraoperative ultrasonography was significantly more sensitive (100%) than ultrasonography (79%), computed tomography (81%), and angiography (54%) for detecting carcinomas, especially bile duct carcinomas and small (< or = 2.0 cm) tumors. Portal venous invasion was confirmed histopathologically in 25 patients. For diagnosing portal venous invasion, intraoperative ultrasonography was more sensitive (92%) and specific (92%) than ultrasonography (56% and 73%), computed tomography (64% and 79%), and angiography (76% and 83%), respectively. Endoscopic ultrasonography showed a 95% detectability for carcinomas and a 92% accuracy for assessing portal venous invasion. CONCLUSIONS: Intraoperative ultrasonography is a simple and accurate procedure for detection of pancreatobiliary carcinomas and assessment of portal venous invasion.     

Intraoperative estimation of liver blood flow in man.

The intraoperative measurement of the afferent circulation of the liver, namely the hepatic artery flow and portal venous flow was carried out upon 14 anaesthetized patients having carcinoma of the splanchnic area, mainly in the head of the pancreas, by means of transit time ultrasonic volume flowmeter. The hepatic artery flow, portal venous flow and total hepatic flow were 0.377 +/- 0.10; 0.614 +/- 0.21; 0.992 +/- 0.276 l/min, respectively. The ratio of hepatic arterial flow to portal venous flow was 0.66 +/- 0.259. There was a sharp, significant increase in hepatic arterial flow (29.8 +/- 6.1%, p < 0.01) after the temporary occlusion of portal vein, while the temporary occlusion of hepatic artery did not have any significant effect on portal venous circulation. The interaction between hepatic arterial flow and portal venous flow is a much disputed question, but according to the presented data here, it is unquestionable, that the decrease of portal venous flow immediately results a significant increase in hepatic artery circulation.     

Surgical strategy for hepatocellular carcinoma originating in the caudate lobe

BACKGROUND: The prognosis of hepatocellular carcinoma originating in or mainly involving the caudate lobe (caudate HCC) is generally poor. We reviewed the clinicopathologic findings of patients who underwent liver resection of caudate HCC and correlated the outcome with the surgical strategy. METHODS: Records of 402 patients who underwent liver resection for HCC were reviewed. The patients were divided into 2 groups. One group consisted of 15 patients who underwent liver resection for caudate HCC. The other group included 387 patients with HCC in a site other than the caudate lobe. RESULTS: Anatomic resection of Couinaud segment I or IX (a partial caudate lobectomy), conforming to portal anatomy, was performed in 13 patients with caudate HCC, and segmentectomies of segments I and IX (a total caudate lobectomy) were performed in 2 patients with caudate HCC. The incidence of postoperative complications was similar in the caudate HCC group and HCC in other sites group, with no operative deaths in the caudate HCC group. Tumor-free survival and cumulative survival were similar in the 2 groups. However, among patients with caudate HCC, tumor-free and cumulative survival were lower in patients with than without microscopic portal venous involvement (P<.01). CONCLUSIONS: Partial caudate lobectomy (anatomic resection of segment I or IX) along the portal system is an appropriate procedure for caudate HCC, especially in patients with impaired liver function or a small HCC. Patients with caudate HCC who have microscopic portal venous involvement may require adjuvant therapy as early recurrence is likely.     

Portal venous velocity in biliary atresia

From December 1986 to April 1989, 38 patients with biliary atresia (eight newly diagnosed) were evaluated with doppler ultrasound of the portal venous system. Peak and mean velocities were computer derived from the spectral waveform. Good velocity was greater than 15 cm/s, intermediate velocity was 8 to 14 cm/s, abnormal velocity was less than 7 cm/s or hepatofugal. Patients were grouped according to clinical status: group 1 (n = 14), normal liver function; group 2 (n = 15), recurrent cholangitis; group 3 (n = 2), established bile drainage but complicated cirrhosis; group 4 (n = 7), failed portoenterostomy. All patients with normal liver function (group 1) had good or intermediate velocities. Thirteen of 15 patients with recurrent cholangitis (group 2) had good or intermediate velocities. Both patients in this group with abnormal velocities required transplantation. In group 3 the patient with abnormal velocity is on the transplant waiting list. In group 4, abnormal velocities preceded or coincided with deterioration of liver function in five of seven patients. Doppler ultrasound provides useful anatomic information, determines direction of flow, quantitates velocity of flow, and, when performed serially, provides adjunctive information on liver status in children with biliary atresia. These preliminary results suggest that patients with abnormal or significantly decreasing velocity are destined for transplantation. Patients with good portal venous velocity warrant ongoing, aggressive surgical management.     

Duplex sonography accurately assesses portacaval shunt patency

Among 42 patients who had undergone portacaval shunt (PCS) to treat bleeding esophageal varices, shunt patency was assessed with duplex sonography 1 month to 5 years postoperatively. Patency was confirmed in all patients (100%). Correlative angiograms confirming the sonographic findings were obtained in 24 patients. Duplex scanning showed hepatofugal or stagnant flow in the distal portal vein in all 42 patients. Very low rates of liver-failure-related mortality (6%) and morbidity (6% incidence of encephaloparthy) in this series despite loss of portal perfusion of the liver in patients incriminate factors other than magnitude and direction of portal vein flow as the cause of complications occurring after PCS. Duplex sonography offers accurate and relevant clinical and physiologic data about shunt hemodynamics in patients who have undergone PCS.     

Measurement of portal blood flow in man by a continuous local thermodilution method. III. Effects of dopamine on systemic and portal hemodynamics after hepatectomy

We gave dopamine (3 micrograms/kg/min, 30 min) intravenously on the third day after hepatectomy to 19 patients, and studied the effects of the drug on systemic and portal hemodynamics. In another 42 patients, administration of dopamine at the same rate was started soon after hepatectomy and continued for about 2 weeks; the clinical results were evaluated. After hepatectomy, the systemic hemodynamics were hyperdynamic and the portal hemodynamics were hypodynamic. After 30 min of dopamine administration, the oxygen pressure in portal blood increased, and because portal blood flow also increased, the oxygen delivery to the liver increased. The mechanism involved an increased proportion of portal venous flow to cardiac output, and a decrease in the splanchnic resistance, not portal venous resistance. Probably, specific dopamine receptors played important role in the increase in the superior mesenteric arterial blood flow. Among 42 patients given small dosage of dopamine, the clinical symptoms of five of seven who had developed liver failure improved. None of the other 35 patients given dopamine preventively developed liver failure. Dopamine in small doses is useful for the management of liver failure after liver resection.     

Improving hepatic and portal venous flows using tissue expander and Foley catheter in liver transplantation

BACKGROUND AND OBJECTIVE: Vascular reconstruction is important in liver transplantation because its obstruction causes graft failure and eventual loss. Vascular outflow obstruction may be due to graft malposition. We describe our experience with liver allograft repositioning using tissue expander and Foley catheter to improve hepatic and portal venous outflows. PATIENTS AND METHODS: A total of seven patients who received liver transplantation at our institution developed hepatic and/or portal venous obstruction during final graft positioning detected by Doppler ultrasonography (hepatic vein flow <10 cm/s; portal vein flow <12 cm/s). Chart and operative records of these patients were reviewed. Technique of operation, donor-recipient characteristics, use of tissue expander or Foley catheter to improve venous outflow, complications, and outcome were analyzed. RESULTS: Hepatic and/or portal venous obstruction were detected after portal reperfusion. We used commercially available tissue expander used in plastic surgery and Foley catheter to reposition the graft. Tissue expanders were used in three recipients (age: 27-46 yr). Foley catheters were used in four recipients (age: 7 months-53 yr). One recipient used both tissue expander and Foley catheter. Expanders were filled with 300-770 mL saline and placed into the right subphrenic space. Foley catheters were filled with 15-75 mL saline. Significant improvements in hepatic and/or portal venous outflow were detected by Doppler ultrasonography post-graft repositioning. Aspiration of expander and Foley catheter contents was started from 6th to 27th postoperative day under sonographic guidance. All expanders and catheters were removed by the 19th-56th postoperative day (mean: 38 d). Complications included chylous ascites (1/7), bile leak (1/7), tube drain infection (2/7), septicemia (2/7). All complications were successfully managed by non-operative interventions. There was no outflow obstruction detected by ultrasonography before and after removal of expanders and catheters. One- and two-year graft and patient survivals were both 100%. CONCLUSION: The use of tissue expanders and Foley catheters to improve hepatic and portal venous outflow in malposed liver allografts is a simple and safe method after liver transplantation.     

Portal vein obstruction in patients requiring hepatic resection or transplantation

Four patients with chronic portal vein occlusion (or near occlusion) who required major hepatic resection or transplantation are described. In each case, portal vein patency was restored, and objective evidence of long-term patency is presented in three patients. These patients demonstrate the feasibility of restoring and indefinitely maintaining portal venous flow coincident to major hepatic surgery.