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Development of portal vein thrombosis complicating idiopathic portal hypertension. A case report.

A 58-yr-old woman with biopsy-proven idiopathic portal hypertension presented with ascites and pretibial pitting edema. On admission, ultrasonic Doppler flowmetry demonstrated hepatopetal flow of a markedly reduced velocity in the portal vein, hepatofugal flow in the splenic vein, and a large spontaneous splenorenal shunt. The patient spontaneously developed hepatic encephalopathy 1 mo later. Percutaneous transhepatic portography demonstrated mural thrombi at the porta hepatis after the catheter had penetrated the mural thrombi without resistance; there was also a long retention of contrast medium in the portal vein. 99mTc-Macroaggregated albumin instilled into the superior mesenteric vein was caught in the lungs, and no activity entered the liver. Measurements of ammonia and immunoreactive insulin clearly indicated that superior mesenteric venous blood was shunted through the splenic vein and the splenorenal shunt. Subsequent ultrasonic examination with Doppler flowmetry suggested further growth of the thrombi and lack of blood flow in the portal vein. Although the procedure of percutaneous transhepatic catheterization could have contributed to the growth of thrombi, it is more likely that the thrombosis in the portal vein was a sequela to idiopathic portal hypertension, and was growing at the time of catheterization. This case may be of significance in the understanding of the relationship between idiopathic portal hypertension and extrahepatic portal obstruction.

Female↗

Abdominal computed tomography in systemic lupus erythematosus.

Sixty-five abdominal computed tomography (CT) scans of 54 systemic lupus erythrematosus (SLE) patients were retrospectively evaluated together with their clinical records. This was to assess the spectrum of CT findings and to determine the value of abdominal CT in this group of patients. Over a 3.5-year period, abdominal CT scans had been requested for suspected renal vein or inferior vena cava thrombosis (n = 52, 80%), sepsis, mesenteric ischaemia, Conn's syndrome, evaluation of hepatosplenomegaly, portal hypertension and hydronephrosis. The most frequent indication for CT was suspected renal vein thrombosis (RVT). An SLE patient with previously stable renal function who rapidly develops nephrotic syndrome with deteriorating renal function has an increased risk of thromboembolic phenomenon. Also, renal vein thrombosis is difficult to diagnose clinically and prompt anticoagulation can help preserve remaining renal function. Of these with suspected RVT, two had RVT only and five had thrombosis in both renal veins and inferior vena cava. Two patients had CT features strongly suggestive of mesenteric ischaemia, one had bilateral hydronephrosis thought to be secondary to lupus cystitis and CT confirmed two abdominal abscesses. Other incidental CT findings were: subscapular renal haematoma, overall enlargement or diminution of renal size, serositis, bowel wall thickening, splenic, hepatic and pancreatic enlargement and mild para-aortic lymphadenopathy. Abdominal CT revealed many diverse findings and aided the management of these SLE patients.

Adolescent↗

Surgical methods for the prevention of first and recurrent variceal bleeding.

Variceal bleeding should be managed by sclerotherapy for control of acute bleeding, and as the initial method in preventing recurrence. Surgery is required for the 30-40% who fail sclerotherapy. The surgical choices are selective variceal decompression, total portal systemic shunt, devascularization procedures, liver transplantation. Distal splenorenal shunt (DSRS) combines good control (greater than 90%) and maintenance of hepatic function. Survival after DSRS is significantly better in nonalcoholic, 75% at 5 years, compared to alcoholic cirrhotics, 45% at 5 years: this difference is associated with improved maintenance of portal flow in the former. Loss of portal flow in 60% of alcoholics has led to modification of DSRS with total disconnection of the splenic vein from the pancreas, resulting in improved portal flow maintenance. Patients with bleeding secondary to portal vein thrombosis and schistosomiasis are excellent candidates for DSRS. Total shunts will stop bleeding, and may be required emergently for uncontrolled hemorrhage. Portacaval H-graft is easily performed, but loss of portal flow accelerates liver failure. Devascularization procedures have a 20-40% rebleeding rate, but do not accelerate liver failure. Liver transplantation is increasingly indicated for patients with end-stage liver disease and variceal bleeding. However, it is the degree of hepatic failure, rather than the variceal bleeding per se, which dictates the need for this therapy. Optimal patient care requires that a center should be able to offer all such therapies.

Esophageal and Gastric Varices↗

Percutaneous transhepatic pancreatic islet cell transplantation in type 1 diabetes mellitus: radiologic aspects.

PURPOSE: To report our experience with percutaneous transhepatic pancreatic islet cell transplantation in patients with type 1 diabetes mellitus. MATERIALS AND METHODS: Between March 1999 and May 2002, 34 patients underwent 68 islet cell transplantation procedures. Patients with C-peptide-negative type 1 diabetes were selected on the basis of poor metabolic control (hypoglycemia or lability) despite compliance with optimal medical therapy. Islet cells were isolated from brain-dead donors. Access to the portal vein was gained from a right percutaneous transhepatic approach, and islet cells were infused with intermittent pressure monitoring. Twenty patients underwent two transplantations, seven patients underwent three transplantations, and seven patients underwent one transplantation. Complications during and after the procedure and postprocedural diabetic status were monitored. RESULTS: Successful portal vein cannulation and islet cell infusion were achieved in all cases. Fluoroscopy was used as the primary guidance modality in 58 of 68 (85%) procedures, and ultrasonography was used in 10 of 68 (15%). Total recorded fluoroscopy time varied from 0.6 to 103 minutes, with a median of 6.9 minutes. Potentially serious complications occurred in six of 68 (9%) procedures. Two patients developed portal venous thrombosis, and with subsequent anticoagulation therapy, one of the two developed an expanding hepatic hematoma that required surgery. Clinically important hemorrhage occurred in four patients, three of whom required blood transfusions. Of 26 patients who received completed transplants, all became insulin independent, and 81% (21 of 26) remained insulin free at 1 year. CONCLUSION: The percutaneous transhepatic approach for the implantation of islet cells into the portal vein is a safe procedure, and together with use of current cell separation techniques and an immunosuppressive regimen, offers a marked advance in the treatment of type 1 diabetes mellitus.

Adult↗

[Doppler echography in the intraoperative assessment of the liver circulation: an experimental study in swine].

In this experimental study the authors report an experience in the evaluation of hepatic blood flow with intraoperative echo-Doppler during orthotopic liver transplantation and side-to-side or end-to-side portacaval shunt. Doppler ultrasonography studied the flow of portal vein, hepatic artery and inferior vena cava before the recipient hepatectomy, and after reperfusion during liver grafting. Furthermore echo-Doppler of the portal system was performed to confirm portacaval shunt efficacy. Usually intraoperative Doppler ultrasonography may give informations about the patency of the shunt and regarding the development of early hepatic artery thrombosis during liver transplantation, but often unclear is the exact evaluation of the velocity of the blood flow through the liver. Further experimental studies and clinical evaluations need to find safe parameters and markers of vascular alteration using this superior diagnostic procedure.

Animals↗

Efficacy of an implanted drug delivery system for advanced hepatocellular carcinoma using 5-fluorouracil, epirubicin and mitomycin C.

BACKGROUND: In order to assess the efficacy of repeated hepatic arterial infusion (HAI) therapy for advanced hepatocellular carcinoma, we administered HAI therapy using 5-fluorouracil, epirubicin and mitomycin C, with an implanted drug delivery system. METHODS AND RESULTS: Thirty-seven patients received HAI therapy with a response rate of 21.6%. The 6-month and 1-year survival rates were 67.6 and 29.7%, respectively. Responders to HAI therapy showed significantly longer survival than non-responders, while patients with mild liver dysfunction tended to survive longer than those with severe dysfunction. Neither tumour thrombosis of the main trunk of the portal vein nor extrahepatic metastases were considered to constitute contraindications for HAI therapy. Catheter-related trouble occurred in eight patients and biloma in one patient, but other side effects were transient and tolerable. These patients received HAI therapy as outpatients for an average of 79.6% of the entire course of therapy. CONCLUSIONS: Hepatic arterial infusion therapy proved effective for patients with advanced hepatocellular carcinoma in terms of improving survival and outpatient rates.

Adult↗

A comparison of MR and duplex Doppler ultrasound for vascular assessment prior to orthotopic liver transplantation.

A prospective blinded comparison of duplex Doppler ultrasound (DDU) and a magnetic resonance angiography (MRA) 2-D time-of-flight technique was performed to assess the hepatic vasculature in 23 adult patients referred for orthotopic liver transplantation. Both techniques confirmed patency of the inferior vena cava in all patients, demonstrated a complete portal vein occlusion in one patient, and accurately defined the vascular and organ anatomy in a patient with situs inversus. The results were confirmed at surgery in 18 of the patients who have subsequently undergone liver transplantation. In one patient a partial portal vein thrombosis was found at surgery which was not detected by either imaging technique. MRA was more sensitive overall in detecting porto-systemic collaterals (31 locations in 17 patients) compared with ultrasound (24 locations in 17 patients), although ultrasound detected more patients with recanalized para-umbilical veins (DDU: 10 patients; MRA: 8 patients). These results indicate that a simple MRA technique can equal duplex Doppler ultrasound for the non-invasive assessment of patency of the hepatic vasculature and should be considered when DDU examination is equivocal or technically inadequate. MRA may provide additional information on the presence and size of porto-systemic collaterals which may be of value in monitoring portal hypertension.

Adult↗

A 27-year experience with surgical treatment of Budd-Chiari syndrome.

OBJECTIVE: To determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. SUMMARY BACKGROUND DATA: Experiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. METHODS: From 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation. RESULTS: In the 32 patients with BCS resulting from hepatic vein occlusion alone, SSPCS had a surgical death rate of 3%, and 94% of the patients were alive 3.5 to 27 years after surgery. All 31 survivors remained free of ascites and almost all had normal liver function. No patient with a patent shunt had encephalopathy. The SSPCS remained patent in all but one patient. Liver biopsies showed no evidence of congestion or necrosis, and 48% of the biopsies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thrombosis. All patients survived surgery, but five subsequently developed thrombosis of the synthetic graft and died. Because of the poor results, mesoatrial shunt was abandoned. Instead, a high-flow combination shunt was introduced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patients who underwent combined SSPCS and CAS, and the shunts functioned effectively during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for orthotopic liver transplantation. Three patients died of liver failure while awaiting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively. CONCLUSIONS: SSPCS in BCS with hepatic vein occlusion alone results in reversal of liver damage, correction of hemodynamic disturbances, prolonged survival, and good quality of life when performed early in the course of BCS. Similarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has been discontinued in the authors' program because of an unacceptable incidence of graft thrombosis and death. In patients with advanced cirrhosis from long-standing, untreated BCS, orthotopic liver transplantation is the only hope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression.

Adult↗

Hepatic arterial buffer response fails to restore hepatic oxygenation after temporary liver dearterialization in canines.

BACKGROUND: Hepatic artery thrombosis is a rare but extremely troublesome condition after liver transplantation. Recently, urgent arterial revascularization has been used as rescue therapy, leading to improved graft and patient survivals. Hepatic artery ligation produces a progressive reduction in portal vein blood flow. Theoretically, a hyperemic response may be expected following hepatic artery reperfusion (hepatic artery buffer response, HABR). In this study, we tested the hypothesis that HABR can maintain adequate liver oxygenation after temporary liver dearterialization. METHODS: Seven dogs (19.7 +/- 1.2 kg) subjected to 60 minutes of hepatic artery occlusion were observed for 120 minutes thereafter. Systemic hemodynamics was evaluated through Swan-Ganz and arterial catheters, and splanchnic perfusion by portal vein and hepatic artery blood flows (PVBF and HABF) via an ultrasonic flowprobe. Liver enzymes (ALT and LDH) and systemic and hepatic oxygen delivery (DO2hepat) were calculated using standard formulae. RESULTS: Hepatic artery occlusion induced a progressive reduction in PVBF and DO2hepat. A complete restoration of HABF after hepatic artery declamping was observed; however, the DO2hepat (33.3 +/- 5.9 to 16.5 +/- 5.9 mL/min) did not return to the baseline levels. CONCLUSION: Temporary hepatic artery occlusion induced a progressive decrease in portal vein blood flow during ischemia, an effect that continued during the reperfusion period. The hepatic artery blood flow was promptly restored after declamping. However, HABR was not able to restore hepatic oxygen delivery to baseline levels during the reperfusion period.

Animals↗

Duplex Doppler ultrasound for the detection of vascular occlusion following liver transplantation in children.

Sixty-three children and adolescent patients who received 78 consecutive orthotopic liver transplants and had serial duplex ultrasound monitoring were retrospectively reviewed for episodes of occlusion of the hepatic artery or portal vein. There were 13 documented episodes of complete occlusion of the main hepatic artery or a major branch, of which nine were diagnosed by the absence of flow during duplex ultrasound examination. Four occlusions were not detected, two affected hepatic artery branches, one an accessory hepatic artery, and one the main hepatic artery. The overall sensitivity of the method was 69% and specificity 100%. Six portal vein occlusions were documented in five patients and all six were successfully diagnosed on ultrasound examination, giving a sensitivity and specificity of 100%. Three of the patients had simultaneous occlusions of both vessels. In the early post-operative period duplex Doppler ultrasound examination has proved an effective non-invasive method for the detection of vascular occlusion, but is not yet sensitive enough to replace angiography fully for the detection of hepatic artery occlusion, particularly when this occurs distal to the main hepatic artery.

Adolescent↗

Auxiliary partial liver transplantation: imaging evaluation in 10 patients.

Auxiliary partial liver transplantation involves placement of a heterotopic graft in the subhepatic space, with the liver of the recipient left unaltered. We studied the values of three imaging procedures (Doppler sonography, diisopropyl iminodiacetic acid [DISIDA] scintigraphy, and cholangiography) to evaluate the transplant and the native liver after surgery in 10 patients. Special attention was paid to the Doppler waveform of the hepatic artery of the graft and to the resistive index. An increase of the index above 0.80 was not specific for, but was always associated with graft dysfunction (rejection, primary nonfunction, multiple organ failure, or portal vein thrombosis). DISIDA scintigraphy also lacked specificity and was of limited value in the detection of postoperative complications. It proved useful in routine imaging to monitor atrophy of the native liver and hypertrophy of the transplant. Cholangiography was useful for visualization of the biliary tree. Our experience suggests that these three noninvasive imaging procedures are sufficient for the proper postoperative evaluation of patients after auxiliary partial liver transplantation.

Adolescent↗

Acute pancreatitis and deep vein thrombosis associated with HELLP syndrome.

The HELLP syndrome (HS) belongs to the list of obstetric complications believed to be associated with coagulation disorders. It was formerly thought that chronic intravascular clotting (DIC) in the placental vessels was the main cause. A hypercoagulable state has been reported in cases of severe HS associated with microvascular abnormalities that may involve cerebral, placental, hepatic and renal vessels. A case of acute pancreatitis and DVT of inferior cava in a pregnant woman, presenting with HS at 29 weeks, who was found to have a R506Q mutation, is reported. Preeclampsia-associated pancreatitis and DVT have rarely been reported. It is hypothesized that APC-R and Factor V Leiden mutation may prove to be new and more important markers capable of predicting a more significant maternal morbidity associated with HS. Thrombosis prophylaxis may be considered during pregnancy in order to reduce hazardous multiorgan failure (MOF) in women who are heterozygous for Factor V Leiden mutation.

Acute Disease↗

Computed tomography during arterial portography under temporary balloon occlusion of the hepatic artery: evaluation of pseudolesions caused by arterio-portal venous shunts.

BACKGROUND: Computed tomography during arterial portography (CTAP) under temporary balloon occlusion of the hepatic artery (BOHA-CTAP) was introduced to evaluate pseudolesions caused by portal venous impairments such as arterioportal shunt and tumor thrombus. METHODS: BOHA-CTAP was performed in seven patients with hepatocellular carcinoma and correlated with clinical outcomes. For patients with wedge-shaped defects suggestive of pseudolesions, BOHA-CTAP was obtained by a 5-F balloon occlusion catheter into the proper hepatic artery through the second 5-F introducer inserted into the common femoral artery a few centimeters below the first 5-F sheath for CTAP. RESULTS: Eight pseudolesions were determined clinically on follow-up CT, ultrasonography, or magnetic resonance imagings. On BOHA-CTAP, five of the eight pseudolesions were eliminated, and two were diminished in comparision with conventional CTAP. One wedge-shaped defect due to tumor thrombus in the portal vein did not show any change. CONCLUSION: BOHA-CTAP can reduce pseudolesions caused by portal venous impairments and enable the demarcation of the true tumors.

Aged↗