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Budd-Chiari syndrome after pregnancy is an extremely rare disease. Reported here is a case of postpartum Budd-Chiari syndrome with unusual features of prolonged hypercoagulability state. The disease occurred 2 weeks after delivery and despite massive anticoagulation treatment the patient developed severe hepatic vein occlusion, renal vein thrombosis, inferior vena cava thrombosis, and femoral artery thrombosis.
Objections to portal systemic shunting in children with life-threatening hemorrhage from esophageal varices include the high incidence of postshunt encephalopathy with neurologic and psychiatric sequelae and the inability to provide an adequate shunt in very young children. We have operated on eight children in the past 4 years for bleeding varices. The causes were: portal vein thrombosis (3), congenital hepatic fibrosis (2), chronic active hepatitis (2), and cystic fibrosis (1). The ages at operation were between 2 and 17 years. These children underwent various modifications of an operation described by Sugiura. The operation we have developed is done through a single thoracoabdominal incision, dividing and anastomosing the esophagus with a stapler, preserving the vagal innervation to the pylorus and antrum, and wrapping the fundus around the distal esophagus at the site of the anastomosis. The venous drainage of the lower esophagus and of the upper stomach is divided. The operation is therefore shorter and simpler, but adheres to the principles enunciated by Sugiura. Complications include one significant postoperative anastomotic leak and one symptomatic esophageal stricture. Longterm results have been gratifying with no evidence of rebleeding from esophageal varices. We believe that our modification of the original Sugiura operation is the preferred therapy of bleeding esophageal varices when surgical intervention is indicated because it preserves the normal structure and function of the upper gastrointestinal tract as well as the portal venous drainage to the liver.
BACKGROUND: The shortage of cadaveric donor livers is the rate-limiting step in clinical liver transplantation. Split liver transplantation provides a means to expand the cadaveric donor pool. However, this concept has not reached its full potential because of inferior patient and graft survival and high complication rates when traditional ex vivo split techniques are used. Therefore we sought to evaluate the safety, applicability, and effectiveness of a new technique for split liver transplantation. METHODS: This study consists of 15 in situ split liver procurements, which resulted in 28 liver transplants. In situ splitting of selected livers from hemodynamically stable cadaveric donors was performed at the donor hospital without any additional work-up or equipment being needed. In situ liver splitting is accomplished in a manner identical to the living-donor procurement. This technique for liver splitting results in a left lateral segment graft (segments 2 and 3) and a right trisegmental graft (segments 1 and 4-8). This procedure required the use of the donor hospital operating room for an additional 1.5-2.5 hr and did not interfere with the procurement of 30 kidneys, 12 hearts, 7 lungs, and 9 pancreata from these same donors. RESULTS: The 6-month and 1-year actuarial patient survival rates were 92% and 92%, respectively, while the 6-month and 1-year actuarial graft survival rates were 86% and 86%, respectively. The 6-month and 1-year actuarial patient survival rate of patients who received a left lateral segment graft was 100% and 100%, respectively, while those who received a right trisegmental graft had 6-month and 1-year rates of 86% and 86%, respectively. The actuarial death-censored graft survival rates at 6 months and 1 year were 80% and 80%, respectively, for the left lateral segment grafts, and 93% and 93%, respectively, for the right trisegmental grafts. Alograft and patient survival was independent of United Network for Organ Sharing status at the time of liver transplantation. No patient developed a biliary stricture, required re-exploration for intra-abdominal hemorrhage, or suffered from portal vein, hepatic vein, or hepatic artery thrombosis CONCLUSIONS: In situ split liver transplantation can be accomplished without complications and provides results that are superior to those obtained previously with ex vivo methods. It abolishes ex vivo benching and prolonged ischemia times and provides two optimal grafts with hemostasis accomplished. This technique decreases pediatric waiting time and allows adult recipients to receive right-sided grafts safely. In situ splitting is the method of choice for expanding the cadaveric liver donor pool.
Budd-Chiari syndrome is a rare condition characterized by thrombosis within the hepatic veins and inferior vena cava. A case of Budd-Chiari syndrome is presented in a patient who presented acutely 3 days following laparoscopic cholecystectomy for acalculous cholecystitis. A discussion of pathology and findings on calcium colloid scintigram, CT scan and Doppler ultrasound is provided.
Budd-Chiari syndrome is thrombosis of the hepatic veins, and associated conditions vary. Budd-Chiari and celiac sprue association is a rare condition. A 24-year-old woman was admitted to the clinic with complaints of weakness, distended abdomen and weight loss for four months. Results of investigation showed Budd-Chiari syndrome and malabsorption with endomysial antibodies and intestinal villous atrophy. All known etiological factors for Budd-Chiari syndrome were negative. Three months after initiation of a gluten-free diet and replacement therapy, she died from infection of ascitic fluid and renal failure following umbilical hernia rupture. Celiac sprue must be remembered especially in the event of Budd-Chiari syndrome and malabsorption.
INTRODUCTION: We describe an unusual case of acute hepatitis leading rapidly to acute liver failure. CLINICAL PICTURE: Our patient had known chronic hepatitis B with a regenerating nodule confirmed on imaging and histology. He was admitted initially for abdominal discomfort after a trip to China, and investigations showed acute hepatitis with alanine transaminase (ALT) and aspartate transaminase (AST) at 678 and 557 U/L, respectively. Initial differential diagnoses were acute exacerbations of chronic hepatitis B, and viral hepatitis A or E. However, acute Budd- Chiari syndrome was diagnosed on computed tomography (CT) scan of the abdomen, which showed extensive thrombosis of the hepatic vein, all the intrahepatic branches, inferior vena cava, up to the right atrium. TREATMENT: Due to the extensive nature of the thrombus, radiological or surgical intervention could not be performed. OUTCOME: The patient was managed conservatively but progressed rapidly and died of acute liver failure 16 days after presentation. CONCLUSION: Our case highlights the rapidity of progression of acute Budd-Chiari syndrome. Diagnosis and management of Budd-Chiari syndrome are discussed.
We report a case of hypereosinophilic syndrome (HES) which later evolved into acute lymphoblastic leukemia (ALL). A 37-year-old man showed typical clinical manifestations of HES: pulmonary infiltrates, erythematous skin rash, deep vein thrombosis, endomyocardial fibrosis, and diffuse central nervous system dysfunctions. Although he was treated with prednisolone and hydroxyurea, marked eosinophilia persisted and lymphoblasts gradually increased in the bone marrow. He died of severe disseminated fungal infection after anti-leukemic therapy. Autopsy revealed marked fibrous thickening of the endocardium, bilateral common iliac vein thrombosis, and chronic hepatitis with fibrosis. Neither eosinophilic nor leukemic cell infiltration was seen in any tissue at autopsy. Including this case, 24 patients with ALL and hypereosinophilia have been reported in English-language literature. We discuss the relationship between eosinophilia and ALL, and the mechanisms, particularly the role of eosinophil cationic protein (ECP), in causing various organ system dysfunctions in HES.
Among the typical complications of oral contraceptive use are intrahepatic cholestasis, occurring in women with a special and probably genetical predisposition only, and a thrombotic diathesis secondary to increased hepatocellular synthesis of coagulation factors, both effects induced by the estrogens. This state of hypercoagulability may lead to thrombosis of the hepatic veins and may thus cause the Budd-Chiari syndrome. A tendency to gallstone formation is explained by a derangement in the synthesis of bile acids and by changes in the permeability of liver cell membranes and of epithelial cells of the gallbladder, an effect again due to the estrogen component of the contraceptives. After long-term use, an increased incidence of benign liver cell adenomas is observed; malignant liver tumors do occur very rarely. A causal relationship with focal nodular hyperplasia, however, is unlikely. Only the typical vascular changes of this entity are probably due to the effects of the contraceptives.
Hepatic myelopathy is a rare complication of hepatic insufficiency, causing progressive spastic paraparesis. There are few reports detailing the clinical and diagnostic aspects of this uncommon cause of neurological deterioration in patients with liver disease. Early recognition of this disorder will become more important as patients with liver disease survive longer due to medical advances, including liver transplantation. We present an additional patient with hepatic myelopathy associated with infantile portal vein thrombosis and review the previous reports of hepatic myelopathy in the English literature.
Duplex Doppler US is a procedure that enables confirmation--and in many instances--detection of portal hypertension by visualizing the presence of collateral blood vessels and determining the direction of the portal flow. In addition, absence of Doppler signal into the veins allows assessment of venous thrombosis both in the portal and hepatic veins.
Retransplantation (re-TX) is the only available therapy for irreversible liver graft dysfunction. The outcome of a second procedure depends upon several factors, some of which are not defined at the time of the decision to retransplant. This study is an analysis of all re-TX of the liver performed at our unit between January 1995 and January 2004. Among the 474 liver TX were 55 (11.6%) re-TX in 47 patients. We studied (1) diagnosis at first TX; (2) indication for re-TX and time lapse; (3) donor age and cold ischemia time (CIT); (4) duration of operation, peroperative bleeding, and complications; (5) ICU and ward periods; and (6) patient and graft survivals. Patients who underwent re-TX did not differ from those transplanted once with regard to age, gender, or diagnosis. The indications for re-TX were roughly one-third biliary tract complications/chronic rejection, one-third hepatic artery thrombosis, and one-third others, including primary nonfunction, acute rejection, portal vein thrombosis, sepsis, and B/C hepatitis. The re-TX were "urgent" in 29 and "elective" in 26 cases. Complications were common; about half of the patients were reoperated due to bleeding or biliary problems. To date (May 2004), 15 patients have died (12 "urgent" and 3 "elective"), of whom 5 had well functioning grafts. In summary, liver re-TX is a complicated procedure associated with significant mortality and morbidity, but considering that the actual patient group has a poor prognosis without re-TX, the results are nevertheless encouraging.
Antiphospholipid antibody syndrome (APS) is now recognized as one of the most important causes of hypercoagulability. The most common site for venous thrombosis in APS is deep venous thrombosis of the lower extremities. Other sites of venous thrombosis include retinal veins, renal veins, and hepatic veins. The authors report a case of splenic vein thrombosis disclosing antiphospholipid syndrome in which also the cytolytic effect of aPL may play a role of "cofactor" in the genesis of thrombosis through the release of thromboplastin from the lysis of red cells, granulocytes and platelets, making them vulnerable to clearance by splenic macrophages. Important considerations are stressed about differential diagnosis, etiopathogenetic factors, therapy and follow-up of the patient.
A 27-year-old primipara with severe preeclampsia and primary antiphospholipid syndrome developed right upper quadrant pain, massive ascites, HELLP syndrome, and disseminated intravascular coagulation shortly following vaginal delivery. Computed tomography and color Doppler studies were compatible with complete thrombosis of the right hepatic veins, the Budd-Chiari syndrome. Anticoagulation was initiated, along with supportive measures, and the patient recovered completely. Imaging studies 6 months later were normal. This case demonstrates that nearly fatal forms of venous thrombosis may complicate preeclampsia in women with antiphospholipid syndrome; Doppler studies of the hepatic vein are of value in establishing the diagnosis.
Portal vein thrombosis is one of the main prehepatic causes of portal hypertension. The most frequent causes of thrombosis in this localization, apart from hepatic cirrhosis, are the following: acute inflammatory diseases and abdominal cancers, traumas, proliferative diseases of the hematopoietic system. In recent years attention was given to disorders in hemostasis, such as thrombophilia, in the course of which thrombosis development is particularly common. The authors present 10 patients after an incident of portal vein thrombosis, in which primary hepatic pathology was excluded and tests directed at thrombophilia were performed. In seven patients abnormalities in the examined parameters were found, and what is more, in two cases they had a complex character and involved more than one parameter. In five patients hyperhomocysteinemia was found. Among them, in two patients there was also a decreased protein S activity and in one of them there was also APC-resistance. In the next two patients there were abnormalities in one of the examined parameters - APC-resistance. Hyperhomocysteinemia was found in all patients with idiopathic thrombosis, and in one of them there were concurrent changes in protein S activity and APC-resistance. In patients with the history of portal vein thrombosis diagnostics of thrombophilia should be performed.
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AIM: Hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) is associated with poor prognosis. The aim of this prospective study was to evaluate the efficacy of hepatic arterial infusion chemotherapy (HAIC) for patients with this disease. METHODS: Eighteen HCC patients with PVTT were treated with HAIC via a subcutaneously implanted injection port. A course of chemotherapy consisted of daily cisplatin (10 mg for one hour) followed by 5-fluorouracil (250 mg for five hours) for five continuous days within a given week. The patients were scheduled to receive four consecutive courses of HAIC. Responders were defined in whom either a complete or partial response was achieved, while non-responders were defined based on stable or progressive disease status. The prognostic factors associated with survival after treatment were analyzed. RESULTS: Six patients exhibited partial response to this form of HAIC (response rate=33%). The 3, 6, 9, 12 and 18-month cumulative survival rates for the 18 patients were 83%, 72%, 50%, 28%, and 7%, respectively. Median survival times for the six responders and 12 non-responders were 15.0 (range, 11-18) and 7.5 (range, 1-13) months, respectively. It was demonstrated by both univariate and multivariate analyses that the therapeutic response and hepatic reserve function were significant prognostic factors. CONCLUSION: HAIC using low-dose cisplatin and 5-fluorouracil may be a useful alternative for the treatment of patients with advanced HCC complicated with PVTT. There may also be survival-related benefits associated with HAIC.
OBJECTIVE: The objective of this study was to describe CT findings of changes in the liver associated with benign obstruction of the hepatic inferior vena cava (IVC). MATERIALS AND METHODS: For a 10-year period, 35 patients with benign obstruction of the hepatic IVC underwent contrast-enhanced CT of the abdomen. These patients were included in this retrospective study. CT scans were analyzed for morphologic changes and abnormal enhancement of the liver, changes in intrahepatic vessels, and additional findings that might be related to obstruction of the IVC. RESULTS: Morphologic changes of the liver included hypertrophy of the caudate lobe (91%) and the left lobe (57%), atrophy of the right lobe (49%), and a nodular surface (74%). The most common pattern of attenuation change was areas of linear, irregular, or wedge-shaped hypoattenuation predominantly located in the peripheral portion of the liver (63%). Diffuse hypoattenuation was seen in six patients (19%) and was frequently found in areas in which hepatic veins filled with hypoattenuated thrombosis (67%). On CT, segmental IVC obstruction (80%) was seen as an obliterated segment of the hepatic IVC. However, membranous IVC obstruction (20%) was not seen on CT. The IVC below the level of obstruction was often revealed as rounded (89%) and occasionally contained thrombus (37%) or calcification (26%). CONCLUSION: CT shows a broad spectrum of morphologic and attenuation changes of the liver and of the hepatic vessels in benign obstruction of the hepatic IVC.
Serious intestinal bleeding from vascular ectasia secondary to extrahepatic portal thrombosis is much less frequent than variceal bleeding, and its treatment is not clearly defined. We describe a 4-year-old girl with repeated intestinal bleeding from vascular ectasia, without any varix, with late extrahepatic portal vein thrombosis (PVT) and late hepatic artery thrombosis (HAT) after living-related liver transplantation. The bleeding stopped after simple splenectomy. She has presented neither bleeding nor any serious complications related to splenectomy for 1 year to date. We think uncontrollable hemorrhage from gastrointestinal vascular ectasia secondary to extrahepatic portal thrombosis in a pediatric patient can and should be treated by simple splenectomy, because patients with this complication usually have a normally functioning liver. However, it is not clear whether this procedure is effective for variceal bleeding.