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A case of inflammatory pseudotumor of the liver hilum successfully treated with aggressive hepatectomy.

Inflammatory pseudotumor (IPT) of the liver is a rare, benign tumor. When the tumor involves the hepatic hilum, however, the clinical course of the patient becomes problematic because of obstructive jaundice and portal hypertension. Complete removal of the tumor sometimes is difficult when the hepatic hilum is extensively involved, and liver transplantation becomes necessary for such an unresectable tumor. This report concerns a 7-year-old boy who presented with obstructive jaundice and portal hypertension owing to an inflammatory pseudotumor of the hepatic hilum and was treated successfully with aggressive hepatectomy, vascular reconstructions of both the portal vein and the hepatic artery, and biliary reconstruction. The patient was discharged after 39 days of hospitalization. Eight months later, portal vein obstruction was detected and treated successfully with percutaneous transhepatic balloon dilatation of the obstructed site. The liver has continued to function well for 11 months after the tumor resection.

Bile Ducts↗

[Fulminant liver failure after administration of the herbal antidepressant Kava-Kava].

HISTORY AND CLINICAL FINDINGS: A 60 year-old woman was admitted to hospital because of jaundice, fatigue, weight loss over several months and icteric skin. Because of progressive liver failure, concomitant renal failure and progressive encephalopathy she was transferred to an intensive care unit. INVESTIGATIONS: Biochemical tests revealed acute liver failure with high levels of total and conjugated bilirubin (30 mg/dl) as well as aspartate aminotransferase (921 IU/l) and alanine aminotransferase (1350 IU/l) concentrations. Prothrombin time was less than 10 %. Serological tests could rule out viral hepatitis, metabolic or autoimmune causes of liver failure. On abdominal computed tomography and ultrasonography no pathological changes were detected. Above all portal vein thrombosis, ascites, focal lesions of the liver and extrahepatic cholestasis could be excluded. Liver histology showed extensive hepatocellular necrosis with intrahepatic cholestasis. TREATMENT AND CLINICAL COURSE: The patient's physical condition deteriorated. She had to be intubated because of respiratory insufficiency and encephalopathy stage IV. Because of progressive liver failure under conservative treatment the patient received an orthotopic liver transplant 11 days after admission. CONCLUSIONS: The exclusion of other causes and the histological diagnosis made Kava-Kava as the cause of acute liver failure most likely. This is the 18th case of Kava-Kava induced liver failure reported to the European regulatory authorities.

Alanine Transaminase↗

Long-term neuropsychological outcome in children undergoing portal-systemic shunts for portal vein obstruction without liver disease.

Forty-two children with portal vein obstruction (cavernoma) and without liver disease had a portosystemic surgical shunt performed at different ages. All of them had severe gastrointestinal hemorrhage prior to surgery. To evaluate the long-term neurological and psychological effects of shunt procedures in children with normal liver function, they were studied up to 24 years postoperatively with psychometric testing. A comparison with two control groups as to the impact of surgery, family situation, and neonatal events was considered. Twenty-four children with portal vein obstruction who were not operated on and 19 children splenectomized for hematological reasons acted as the control groups. No differences were observed in schooling and employment. Psychometric tests were almost identical in shunted patients and in the control groups. Minimal portal systemic encephalopathy appeared to be undetectable clinically either by electroencephalogram or by venous ammonia levels. The results of psychometric tests appear to show no loss of intellectual and psychological capacity in patients operated on for portal obstruction. Only visual memory and spatiotemporal tests showed slightly worse results in those children who underwent this procedure. Further longitudinal studies are required to substantiate these findings.

Adolescent↗

Metastatic hepatic neoplasms: angiographic features of portal vein involvement.

Hepatic metastases produce a spectrum of angiographic alterations of the portal vein that mimicks that of primary liver neoplasms. In 11 patients with varied primary malignancies, hepatic arteriograms revealed seven instances of opacification of the portal vein from arterioportal shunting, two cases of intraluminal tumor thrombus, and two cases with intrahepatic portal vein occlusion. Another patient with liver metastases developed an arterioportal fistula following percutaneous liver biopsy. Although uncommon, metastatic involvement of the portal vein is not necessarily indicative of a poor prognosis since one patient's arterioportal shunting resolved following intraarterial chemotherapy infusion and tumor regression.

Adult↗

Intrahepatic Osler's disease: report of two cases and review of the literature.

Osler-Weber-Rendu disease is a hereditary vascular disease with multiple manifestations. The liver is involved rarely. Vascular abnormalities include telangiectasis and arteriovenous fistulas, sometimes associated with fibrosis and cirrhosis. Hepatic arteriovenous shunting may include secondary portal hypertension, reduced liver function and high cardiac output. Two cases of Osler-Weber-Rendu disease with extensive hepatic arteriovenous fistulation were described in detail and we report on their clinical features. In the first patient, treatment was symptomatic since liver transplantation is not indicated because the patient was asymptomatic. Embolization treatment of hepatic arteries was indicated in the second patient because he had biliary disease and recurrent cholangitis secondary to vasculo-biliary shunts. Therapy with arterial embolization, banding, or ligature of hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers now another therapeutic option for patients with intrahepatic high shunting and secondary pulmonary hypertension.

Adolescent↗

[A study on the etiology of Budd-Chiari syndrome].

OBJECTIVE: To investigate the etiology of Budd-Chiari syndrome (BCS) with finding in surgical operation. METHODS: Thirteen patients with BCS undertook direct vision membranectomy and reconstruction of inferior vena cava. The specimens were studied with light microscopy, electron microscope and immunohistochemistry. RESULTS: Membranous occluding changes, which thickness varying from 2 to 20mm, were found in the posthepatic segment of inferior vena cava during the operation. Thrombi in inferior vena cava were found in 7 cases, 3 of them with thrombi evenly extending to the root of hepatic vein. The compromised venous wall showed proliferation of fibrous connective tissue, hyalinization or mucoid degeneration under light microscopy, 9 of the 13 cases showed chronic infiltration of inflammatory cells. Copious collagenous fibers, few endothelial cells and mitochondria swelling were found with electron microscope. No immune complex (IgA, IgG, IgM) was observed by immunohistochemistry. CONCLUSION: It is concluded that in these cases of BCS, occluding and stenosing lesions in the inferior vena cava were the outcome of thrombosis and its sequelae and local inflammation might be one of the reasons of thrombosis. There was no evidence of congenital malformation.

Adult↗

Three-dimensional multislice helical computed tomography with the volume rendering technique in the detection of vascular complications after liver transplantation.

BACKGROUND: Hepatic artery stenosis and thrombosis are common complications in liver transplant patients. Digital subtraction angiography (DSA) has served as the gold standard to make this diagnosis. More recently, three-dimensional helical computed tomographic arteriography (3D CTA) with maximum intensity projection and shaded surface display techniques has been compared with DSA. The purpose of this study was to determine whether 3D CTA with the volume rendering technique is a useful and accurate tool in the detection of vascular complications after liver transplantation. METHODS: Thirty-five consecutive liver transplant patients underwent 3D CTA with volume rendering technique. The standard of reference was DSA for 20 patients and imaging and clinical follow-up for 15 patients. Two blinded reviewers evaluated the axial and 3D CTA images in consensus. RESULTS: 3D CTA with volume rendering technique detected 10 hepatic artery stenoses, six hepatic artery thromboses, two hepatic artery pseudoaneurysms, two splenic artery aneurysms, two portal vein stenoses, and four redundant hepatic arteries. In one case computed tomography (CT) detected a moderate hepatic artery stenosis, while conventional angiography showed a normal artery. The sensitivity of CT for detecting vascular lesions was 100%, specificity was 89% (8 of 9), accuracy was 95% (19 of 20), positive predictive value was 92% (11 of 12), and negative predictive value was 100% (8 of 8). CONCLUSIONS: 3D CTA is a useful and accurate noninvasive technique for detection of vascular complications in liver transplant patients.

Adult↗

Successful percutaneous pulse spray thrombolysis of extensive acute portocaval hemitransposition thrombosis.

We report a case of extensive acute thrombosis of portal vein and cava in a portocaval hemitransposition liver graft that was treated successfully with percutaneous pulse spray thrombolysis through a femoral vein access. The patient subsequently developed descending and sigmoid colon ischemic necrosis because of the venous thrombosis necessitating emergency colon resection. The patient had prolonged postoperative intensive care stay, but was eventually discharged in a good condition with normal liver function. Three month follow up demonstrated persistent normalization of hepatic function and normal duplex ultrasound.

Acute Disease↗

Subcutaneous administration of hepatitis B immune globulin in combination with lamivudine following orthotopic liver transplantation: effective prophylaxis against recurrence.

Prophylaxis against recurrent hepatitis B virus (HBV) infection with hepatitis B immune globulin (HBIG), in combination with antiviral agents such as lamivudine, has allowed transplantation for this condition to become feasible and accepted. Current protocols allow for HBIG administration either intravenously or intramuscularly. To date, there has been no reported experience with the subcutaneous route of post-transplant HBIG delivery. We report our experience of a 60-yr-old man for whom liver transplantation was performed for chronic HBV. HBIG was administered intramuscularly during the anhepatic phase of surgery. The finding of a portal vein thrombosis requiring repeated thrombectomy necessitated chronic anticoagulation. Post-operatively, HBIG was administered subcutaneously, in four separate injections, for a daily dose of 2170 IU along with continued lamivudine dosing. Hepatitis B surface antibody (anti-HBs) titres reached a serum concentration of >500 IU/L by seven d post-transplant and approximately 1000 IU/L by nine d post-transplant. Five months post-transplant, with continued combination of subcutaneous HBIG and lamivudine, there has been no recurrent HBV infection and anti-HBs titres have been at target levels. Our experience suggests that subcutaneous delivery of HBIG may be a feasible consideration when intramuscular/intravenous dosing is not possible.

Antiviral Agents↗

High-attenuation recent thrombus of the portal vein: CT demonstration and clinical significance.

Attenuation characteristics of portal vein thrombi on nonenhanced computed tomographic (CT) scans were assessed in 122 patients with proved portal vein thrombosis. Portal vein thrombi of high attenuation were found in four patients with hepatocellular carcinoma. From pathologic and radiologic studies, it was concluded that the high attenuation was caused by blood clots of recent onset formed at the tip of tumor thrombus. Differentiation from choledocholithiasis, hematobilia, and calcification of thrombi could be easily made by means of ultrasonography (US). Although plain CT is usually considered noncontributory in the diagnosis of venous thrombosis, it enabled the differentiation of recent thrombus in these four patients. Tumor thrombus in the major branches or main trunk of the portal vein is indicative of poor prognosis. When hepatic mass and high-attenuation portal vein thrombi are demonstrated with plain CT and substantiated by US, enhanced CT and angiography may be unnecessary for treatment of patients with advanced hepatocellular carcinoma.

Aged↗

The causes and consequences of chronic hepatic venous outflow obstruction.

The causes of chronic hepatic venous outflow obstruction are membranous obstruction of the inferior vena cava (group 1), hepatic venous ostial obstruction (group 2) and partial hepatic venous ostial obstruction (group 3). At the H. F. Verwoerd and Kalafong Hospitals, Pretoria, 155 cases have been diagnosed, almost all in the first group. Over 50% of the patients in this series had hepatocellular carcinoma; this association appears to be a constant finding world-wide. The most likely cause is a chronic hepatocaval thrombosis with organisation, leading to fibrous stenosis or membranous occlusion of the involved veins.

Adult↗

Surgery for adult polycystic liver disease.

BACKGROUND: Occasionally patients with adult polycystic liver disease (APLD) have symptoms. For these patients surgery may represent a valuable therapeutic option to relieve symptoms. METHODS: From September 1977 to August 1993 at our institution, 10 women with APLD were examined and surgically treated. They underwent a partial hepatic resection together with cyst fenestration. The surgical outcome and long-term follow-up were retrospectively analyzed. RESULTS: Postoperative morbidity consisted of one case of pneumonia, and one case of acute pancreatitis with deep vein leg thrombosis. One patient died after acute Budd-Chiari syndrome developed as a result of liver collapse after fenestration of a posterior cyst. In the long term six of nine patients were symptom free. Late surgical complications included acute cholecystitis (one patient), small bowel obstruction (one), and incisional hernia (two). CONCLUSIONS: A combined surgical approach of hepatic resection and cyst fenestration has proved feasible for patients with highly symptomatic APLD. Extensive fenestration of posterior cysts should be avoided; transverse hepatic resection (frontal hepatectomy) up to the costal margin is proposed. This therapy provides good results at long-term follow-up.

Adult↗

Liver transplantation in patients with thrombosis of the portal, splenic or superior mesenteric vein.

Fourteen cases are presented of preoperative portal vein thrombosis complicating orthotopic liver transplantation from an experience of 195 transplants carried out between April 1986 and April 1991. In four patients who suffered rethrombosis of the portal vein, the mortality rate was 100 per cent from various causes. Overall there were six deaths; two of those who died had a patent portal vein at death. Three patients underwent retransplantation: one for primary non-function, two for rejection. It is concluded that: (1) portal vein thrombosis should not represent an absolute contraindication to liver transplantation; (2) preoperative screening of prospective transplant recipients for portal thrombosis should be routine; (3) postoperative anticoagulation therapy and periodic Doppler ultrasonographic assessment of portal vein flow are important elements of post-transplant management; and (4) with thrombectomy and portal vein resection an end-to-end portal anastomosis may be performed with good results.

Adult↗

Preoperative vascular imaging in pediatric liver transplantation.

BACKGROUND/PURPOSE: There is a considerable variation in the use of vascular imaging techniques in the preoperative assessment of children scheduled for liver transplantation. Duplex Doppler ultrasound scan (US), magnetic resonance angiography (MRA), and conventional angiography are used to varying extents. The authors compared the results of preoperative vascular imaging studies with operative findings to determine their accuracy and usefulness. METHODS: Results of preoperative vascular imaging in 37 consecutive children undergoing cadaveric liver transplantation were compared with operative findings. Those undergoing relatively elective transplantations were investigated by US and MRA (group 1), whereas those requiring urgent transplants were assessed only by US (group 2). RESULTS: The median age of the cohort (15 boys; 22 girls) was 4 years (19 days to 16 years) and the median weight was 17 kg (2.9 to 82 kg). In group 1 (n = 26), 20 children had a normal-caliber, patent portal vein at transplant and 6 had a narrow but patent portal vein requiring venous reconstruction in 4. The sensitivity and specificity of MRA in the detection of an abnormally narrow portal vein were 100% (6/6) and 95% (19/20), respectively. If reversed or absent flow in the portal vein on US was taken as an indication of a potentially abnormal vein, the sensitivity and specificity of Doppler US were 83% (5/6) and 95% (19/20), respectively. Magnetic resonance angiography revealed arterial anomalies in 4 children but failed to detect small accessory hepatic arteries in 5. The single patient with an aberrant vena cava was identified by MRA. In group 2 (n = 11), venous findings at operation and on US were concordant in 10 (91%) cases; one infant with reversed flow in the portal vein on US had a thrombosed vein at surgery. Magnetic resonance angiography was useful in 2 patient groups: those with reversed flow on Doppler US or suspected portal vein thrombosis in whom an abnormal portal vein was present in 86% (6/7) and infants with the biliary atresia splenic malformation syndrome who had multiple venous and arterial anomalies. CONCLUSIONS: A detailed Doppler examination of the hepatic vasculature by an experienced sonographer/radiologist provides sufficient vascular imaging for most children scheduled for cadaveric liver transplantation. Routine MRA is recommended in children with the biliary atresia splenic malformation syndrome and in those with abnormal duplex Doppler US findings. Although there are limited data in this study, MRA is also valuable in children with Budd-Chiari syndrome, liver tumors, or a previous portosystemic shunt.

Adolescent↗