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[Imaging of complications of liver transplantation in children].

The authors report their experience of imaging of 286 liver transplantations (LT) in children. Hepatic artery thrombosis is the most serious complication (9%), with a maximum risk during the first two weeks. Its clinical presentation is very variable and its diagnosis is based on Doppler ultrasonography. Emergency surgical disobstruction prevented the development of biliary or ischaemic complications in one half of cases. Portal thrombosis (4.5%) is due, in the majority of cases, to hypoplasia of the recipient's portal vein. Patency of intrahepatic portal branches with inversion of flow can delay the ultrasonographic diagnosis. Biliary complications are frequent (20%) and occur after a very variable interval. Their diagnosis is ultrasonographic, but sometimes delayed compared to the histological signs. Transhepatic cholangiography visualises the lesions and allows external drainage or even percutaneous dilatation. In 1 out of 4 cases, the aetiology was hepatic artery thrombosis. The imaging protocol after LT consists of ultrasonography with daily Doppler (or even twice-daily) in the patient's bed for the first two weeks and then as required. Computed tomography is useful to demonstrate parenchymal ischaemia and intraperitoneal abscess in multi-operated children. Angiography and cholangiography confirm the ultrasonographic signs. Interventional Radiology plays an increasingly important role.

Aortography↗

Autoimmune complications associated with hepatitis A virus infection in children.

Hepatitis A virus infection is usually a self-limited disease during childhood. Autoimmune manifestations are rarely reported among patients. We describe two children with acute hepatitis A infection who developed immune thrombocytopenia and hepatic venous thrombosis during the course of acute infection. Antiphospholipid antibodies were increased in both of them during the thrombocytopenic and thrombotic complications and decreased during the resolution of these events.

Acute Disease↗

Obstruction of the hepatic veins or suprahepatic inferior vena cava.

Obstruction of the main hepatic veins or suprahepatic inferior vena cava is caused mainly by thrombosis or its fibrous sequela. One or several underlying thrombogenic disorders are usually present, the most common of which is an overt or occult primary myeloproliferative disorder. The major complications are ascites and gastrointestinal bleeding. A sizeable proportion of the cases are asymptomatic. Diagnosis is usually made at ultrasound or magnetic resonance imaging when collateral veno-venous circulation or endoluminal hepatic venous material are demonstrated. Natural history is poorly known. Overall, mortality is high in the early phase of the disease but once the acute episode is terminated, prognosis is good. Treatment includes anticoagulation to prevent recurrence or extension of thrombosis, nonspecific measures to control ascites and gastrointestinal bleeding, and procedures aiming to restore hepatic blood outflow. Portal-caval shunts are preferred when possible. In patients with obstructed inferior vena cava, the first choice is percutaneous angioplasty, and the second choice is portal-systemic shunts with the right atrium or superior vena caval circulation. Transplantation is proposed for patients with severe liver failure.

Budd-Chiari Syndrome↗

[Sinistral portal hypertension with bleeding gastric varices as initial manifestation of renal-cell carcinoma].

We report a clinic case of renal-cell carcinoma presenting as sinistral portal hypertension; a clinical syndrome consisting of esplenic vein thrombosis manifested as isolated gastric varices with patent portal vein and normal hepatic function. The most frequent cause of this syndrome is pancreatic pathology. Renal-cell carcinoma is characterized by a wide variety of symptoms as initial manifestation. In our case, the patient developed a massive gastrointestinal bleeding secondary to isolated gastric varices caused by splenic vein thrombosis due to extrinsic compression by a hypernephroma that infiltrated the pancreas.

Carcinoma, Renal Cell↗

[Malignant-like angiomyolipoma of the liver: report of one case and review of the literature].

Frequently found in kidney, angiomyolipoma is a rare mesenchymal tumor when diagnosed in the liver and usually benign composed of proliferative blood vessels, fatty tissue and smooth muscle. We report the case of a 67-year-old woman who underwent a left hepatectomy for a 4th segment tumor unidentified after imaging and fine needle biopsy. Final anatomopathologic examination revealed an epithelioïd hepatic angiomyolipoma with signs of malignant behaviour as vascular and lymphatics embolus and invaded left portal vein thrombosis. During the subsequent 24-month follow-up, no recurrence was observed. A review of the literature found only two cases of malignant hepatic angiomyolipoma with fatal issue, however, their incidence must be underrated because of their scarcity and the difficulty of their diagnosis, which needs immunohistochemical confirmation with HMB 45 in particularly. Advances in imaging and anatomopathology in particular with the concept of PEComa (Perivascular-Epithelioïd Cell) as the unifying feature should lead to the recognition of the various variant patterns and cell types. The latter which are important for a correct diagnosis, in order to obtain reliable data about frequency, possible malignant behaviour and therefore consensus management for hepatic angiomyolipoma.

Aged↗

[Mesenteric-portal thrombosis after hematologic splenectomy].

Venous thrombosis of the portal system following splenectomy for haematological diseases is uncommon, being reported with an incidence of 0.2 to 6%. Diagnosis may be difficult and the clinical presentation varies greatly. We report 4 cases, included in a consecutive series of 350 splenectomies for hematological diseases. Case 1: a man of 22 years, operated on for autoimmune hemolytic anemia developed severe and generalized abdominal pain 20 days after splenectomy. The coeliac arteriography showed a thrombosis of the portal system. The laparotomy revealed segmentary small bowel necrosis. Outcome after intestinal resection was uneventful. Case 2: a man of 56 years, operated on for essential thrombocythemia had the laboratory findings of acute hepatic failure 28 days after splenectomy. Doppler ultrasonography revealed a portal vein thrombosis. He was treated with heparin. Case 3: a man of 69 years, operated on for acquired idiopathic anemia, developed asthenia and fever 23 days after splenectomy. The ultrasonography showed a portal vein thrombosis. He was successfully treated with heparin. Case 4: a man of 20 years, operated on for Minkowsky-Chauffard hemolytic anemia developed a severe and generalized abdominal pain and fever 13 days after splenectomy. The CT-scan showed a thrombosis of the portal system. Outcome after thrombectomy and regional thrombolysis was uneventful. The aim of this presentation is to review the pathophysiological diagnosis, therapeutic and prophylactic aspects of this serious complication of splenectomy.

Adult↗

Plasma levels of D-dimer and soluble fibrin polymer in patients with hepatocellular carcinoma: a possible predictor of tumor thrombosis.

INTRODUCTION: Fibrin formation and removal occurs continuously during the development of malignancy. Moreover, plasma D-dimer is indicative of ongoing fibrinolysis, and soluble fibrin polymer (Thrombus precursor protein, TpP) represents thrombogenic activity. We evaluated the relationship between the levels of plasma D-dimer and TpP and tumor thrombosis in patients with hepatocellular carcinoma (HCC), and examined these markers as possible predictors of tumor thrombus in the portal or the hepatic vein. MATERIALS AND METHODS: Plasma levels of D-dimer and TpP were measured in 66 HCC patients (38 without tumor thrombosis, 28 with tumor thrombosis) and 29 healthy controls, by enzyme immunoassay using an Asserachrom D-Di kit (Diagnostica Stago, France) and a TpP kit (American Biogenetic Sciences, USA). RESULTS: The plasma levels of D-dimer and TpP in HCC patients were found to be significantly higher than those in healthy controls, and these values were also significantly higher in patients with tumor thrombosis than those without tumor thrombosis. Positive D-dimer (>367 ng/ml) correlated weakly with the presence of tumor thrombosis, whereas positive TpP (>5.4 microg/ml) correlated strongly with the presence of tumor thrombosis. By multivariant logistic analysis, positive TpP level was found to be a significant predictor of the presence of tumor thrombosis. In contrast, positive D-dimer level was not found to be a significant predictor for predicting tumor thrombosis. CONCLUSIONS: Increased D-dimer and TpP levels in HCC may suggest that fibrinolysis and coagulation occur continuously during tumor progression. This study shows that a positive TpP level is a predictor of tumor thrombosis in HCC, which suggests that TpP may be useful for identifying tumor thrombus in the portal and hepatic veins.

Biomarkers↗

CT findings in chronic post-thrombotic obstruction of the inferior vena cava.

Four patients suffering from chronic post-thrombotic inferior vena caval obstruction were examined by CT scan. The CT appearance of the various venous collateral channels is presented. Other findings observed were bulky seminal vesicles and bilateral compressed bladder. Two unique findings for this entity were observed: 1) the disappearance of the long-since thrombosed IVC, or as we refer to it, the "lonely aorta" sign; and 2) absence of the posterior low-density area of the liver on the unenhanced cuts, representing the hepatic segment of the IVC. This noninvasive method facilitates differentiation among chronic post-thrombotic occlusion of the IVC, acute thrombosis, tumor thrombosis, and occlusion by tumor compression.

Adult↗

[Thrombolysis of an extensive venous thrombosis of the lower body in an anomaly of the vena cava inferior].

HISTORY AND CLINICAL FINDINGS: A 41-year-old man was admitted to hospital for acute pain in the right flank. At first urolithiasis was suspected but excluded by sonography and excretion urography. Computed tomography (CT) was performed because of increasing pain and swelling of the left leg. It showed bilateral thrombosis of the deep leg and pelvic veins as well as of the infrarenal inferior vena cava (IVC) and the right renal vein. The patient's general condition was impaired. The circumference of the left leg was greater than that of the right by 5 cm 15 cm above the knee joint space and both legs had marked varicosities with congestive dermatitis. INVESTIGATIONS: The concentration of the thrombin-antithrombin complex was 13.4 micrograms/l, D-dimer 124 micrograms/l, prothrombin fragment F1+2 3,5 nmol/l and fibrin monomere 27.7 micrograms/ml, as expression of a manifest thrombosis. CT with contrast medium demonstrated the previously sonographically shown extent of the thrombosis. In addition the hepatic IVC segment was absent. The azygos vein was enlarged and there was an extensive collateral circulation. TREATMENT AND COURSE: Systemic thrombolysis (streptokinase for 3 days, then urokinase) for 11 days dissolved the thrombi and CT now demonstrated complete recanalization of the caudal deep vein system and the IVC abnormality (absence of hepatic segment). After intravenous thrombolysis oral anticoagulation with phenprocoumon was started and will be continued all the patient's life.

Adult↗

A metastasising ameloblastoma associated with renal calculi and hypercalcaemia.

A young male had an assumed dentigerous cyst marsupialized and later a recurrent ameloblastoma resected. Eleven years later he was admitted with renal stones and hypercalcemia and metastases of the ameloblastoma in the left lung were discovered. Death occurred as a result of spinal and hepatic spread of his tumor and thrombosis of the renal veins. Renal calcification was demonstrated. The possible causes of the hypercalcaemia which was not associated with a raised serum parathormone or affected by parathyroidectomy is discussed.

Adolescent↗

Multiphase helical CT findings after percutaneous ablation procedures for hepatocellular carcinoma.

BACKGROUND: Multiple-phase helical computed tomography (CT) has been regarded as the method of choice in the evaluation of patients with hepatocellular carcinoma (HCC) treated by nonsurgical procedures. The aim of this article was to report our experience in the assessment of nodular and parenchymal changes recognizable after various percutaneous ablation therapies. METHODS: We reviewed the studies of 116 consecutive patients with HCC treated with multisession percutaneous ethanol injection (56 patients, 98 nodules), single-session percutaneous ethanol injection (14 patients, 31 nodules), radiofrequency thermal ablation (32 patients, 48 nodules), and interstitial laser photocoagulation (14 patients, 25 nodules). CT had been performed 3-28 days after the last session (mean = 18 days) with unenhanced helical acquisition and with contrast-enhanced double- or triple-phase helical acquisition. RESULTS: Persisting neoplastic tissue was identified within 54.5% of the nodules. It was located centrally in 4.5% of these nodules, peripherally in 11%, and eccentrically in 84.5%, and its shape was crescent in 58%, globular in 24.5%, and other in 16%. On arterial phase scans, viable tumor was hyperdense in 97% of the lesions and isodense in 3%; on portal phase scans, the tumor was hyperdense in 20%, isodense in 28%, and hypodense in 52%; on delayed phase scans, the tumor was consistently hypodense. Tumor necrosis was always hypodense on contrast-enhanced scans. On unenhanced images, 7.4% of the nodules were undetectable. Nodule diameter appeared as unchanged in 53% of the nodules and as larger in 47%; its shape was unchanged in 54% and modified in 46%; its margins were unchanged in 36% and modified in 64%. A rim of granulation tissue was detected around 15% of the nodules, and a perilesional transient attenuation difference was detected in 21%. Perihepatic effusion was seen in 13% of the patients, segmental biliary duct dilation and local atrophy each in 9%, arterioportal fistula in 6%, portal vein thrombosis, subcapsular collection and pleural effusion each in 7%, hepatic infarction in 5%, and inferior vena cava thrombosis in 2%. CONCLUSION: Percutaneous ablation of HCC may cause several changes. Knowledge of their CT appearance is mandatory to correctly assess and manage this tumor.

Adult↗

Hepatic hemangiosarcoma: imaging findings and differential diagnosis.

Primary hepatic angiosarcoma is a rare mesenchymal tumor of the liver that usually presents with nonspecific symptoms in elderly men. We present four cases of hepatic hemangiosarcoma and discuss the imaging characteristics of this entity. Our series shows that this tumor is not uncommon in younger patients with no associated risk factors such as previous exposure to thorotrast or vinyl chloride. Our experiences on a limited number of patients suggests that the combined use of angiography and dual-phase helical CT provides a better identification of the tumor and its complications. Analysis of imaging studies in patients with hepatic hemangiosarcoma reveals hypervascular lesions. Common complications were portal vein thrombosis, Budd-Chiari syndrome, as well as arterio-venous or arterio-portal shunts. Due to the vascularity of the tumor, percutaneous liver biopsy is hazardous.

Adult↗

Endoscopic Management of Biliary Complications After Orthotopic Liver Transplantation.

After orthotopic liver transplantation (OLT), biliary duct complications can occur in as many as 10% to 35% of patients. In the early medical and surgical literature, surgical therapy was the primary mode of management of biliary tract complications and was the eventual course of operative intervention in up to 70% of cases. However, with recent advances in therapeutic biliary endoscopy, the current endoscopic and transplantation literature suggests that endoscopic management with techniques such as endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic sphincterotomy, biliary stenting, and stone removal techniques can be successfully applied for the majority of post-OLT biliary complications. The most common biliary complications after OLT include biliary strictures (anastomotic and nonanastomotic); bile duct leaks, common bile duct stones, and biliary casts; sphincter of Oddi/ampullary muscle dysfunction/spasm; and disease recurrence (eg, primary sclerosing cholangitis). Predisposing factors for biliary complications after OLT include hepatic artery thrombosis, impaired perfusion of the biliary tree, portal vein thrombosis, and preservation or harvesting injuries, which can increase the incidence of complications as much as 40%. Use of immunosuppressive agents such as cyclosporine can lead to cholesterol/bile stasis and stone formation. Outside of endoscopic therapy, there is little medical or dietary management that can be applied for post-OLT biliary complications. Ursodiol (ursodeoxycholic acid) has often been used as a neoadjuvant to ERCP therapy in the setting of common bile duct stones/casts, and low-fat diets may be recommended in this setting, but no large, randomized trials have advocated medical or conservative management alone.

Journal Article↗

Pediatric liver transplantation with cadaveric or living related donors: comparative results in 90 elective recipients of primary grafts.

STUDY DESIGN: Between July 1993 and March 1997, 110 children were listed for primary elective liver transplantation with cadaveric (Cad: n = 68) or living-related (LR: n = 42) donors. Pregraft mortality, post-transplant survival, and surgical and immunologic complications were retrospectively compared in both groups. RESULTS: The pregraft mortality rate was 10 (15%) of 68 versus 1 (2%) of 42 in the Cad and LR groups, respectively (P =.049). Postliver transplantation 1-year patient and graft survival rates were 87% and 75% in the Cad group (n = 49) versus 92% and 90% in the LR group (n = 41), respectively (NS). The incidence of post-transplant complications was as follows: hepatic artery thrombosis (Cad: 16%; LR: 0%, P =.020), portal vein thrombosis (Cad: 8%; LR: 2%, NS), and biliary complications (Cad: 14%; LR: 34%, P =.044). The overall incidence of acute rejection was similar in both groups; however, a lower incidence of acute rejection occurred in LR graft recipients treated with tacrolimus. CONCLUSIONS: The introduction of an LR donor liver transplantation program allowed a significant decrease in the pretransplant mortality rate, with a consequent overall improvement in patient survival compared with the Cad series. The incidence of biliary complications was higher in the LR series, whereas better human leukocyte antigen matching in this subgroup did not result in a lower rejection incidence.

Adolescent↗

Doppler sonography of the native liver.

Doppler sonography is being used routinely in evaluating the vascular structures of the native liver because of its ease of use, lower cost, easier availability, lack of need for X-ray and accuracy. Doppler sonography can well demonstrate the vascularization of liver tumors, portal vein thrombosis, portal vein abnormalities in patients with portal hypertension and hepatic venous findings in patients with Budd Chiari syndrome. The purpose of this article is to present information about Doppler sonography of the native liver and to show its usefulness in the evaluation of hepatic vascular diseases.

Hepatic Artery↗

Autopsy findings following gastric bypass surgery for morbid obesity.

BACKGROUND: Roux-en-Y gastric bypass, currently the most frequently performed surgical procedure for morbid obesity, has a low but significant mortality rate. There are limited data documenting the findings at necropsy in patients who have died following this procedure. OBJECTIVE: To determine cause of death and pathologic processes present in obese subjects dying after gastric bypass surgery. PATIENTS: We studied 10 patients who underwent autopsy following gastric bypass surgery for morbid obesity between the years 1994 and 2000. RESULTS: There were 6 men and 4 women. The mean age of the patients was 48 years (range, 28-62 years). The mean preoperative weight was 162 kg (range, 112-245 kg), and the mean body mass index was 54 kg/m(2) (range, 39-76 kg/m(2)), similar to all patients undergoing gastric bypass at our institution during the same period. Five deaths were directly attributable to technical complications. Five deaths were attributed to underlying comorbid conditions. One patient died of cirrhosis and one of pulmonary hemorrhage. Three patients died from pulmonary embolism. However, 8 of 10 patients had microscopic evidence of pulmonary emboli, despite prophylaxis for deep vein thrombosis. Most patients had some degree of steatohepatitis and hepatic fibrosis (80% and 70%, respectively). There were no deaths from primary cardiac events. CONCLUSIONS: In patients who die after Roux-en-Y gastric bypass, half die due to technical complications, whereas the other half die of complications of their obesity. Clinically, only 20% of patients were suspected to have pulmonary emboli, yet at autopsy, 80% of patients had pulmonary emboli. In morbidly obese patients undergoing Roux-en-Y gastric bypass, there is an unexpectedly high rate of clinically silent pulmonary emboli contributing to morbidity and mortality.

Adult↗