Extrahepatic Portal System Research Articles

Multistage closure of a congenital extrahepatic portosystemic shunt

BackgroundCongenital extrahepatic portosystemic shunts (CEPS) are rare shunts connecting the extrahepatic portal system with the inferior vena cava. Shunt dimensions and the risk of portal hypertension determines the closure strategy. Endovascular treatment is indicated for single stage occlusion of longer length shunts, whereas the remaining shunt types are preferentially surgically occluded. Herein we describe the technical details of a novel endovascular treatment for short length CEPS.Case presentationA 15-years-old male with a short length CEPS complicated with multinodular liver disease was submitted to a multistage closure, as indicated by the high portal pressure values during shunt balloon occlusion venography. Initially a transjugular intrahepatic portosystemic shunt (TIPS) was created and the CEPS occluded with an atrial septal defect occluder. In a second procedure the TIPS was embolized with a flow reductor stent and an amplatzer vascular plug II. At a 1 year follow up the liver nodules size reduced, the patient remains asymptomatic, and the shunt adequately closed.ConclusionThis paper outlines the potential use of a TIPS and an atrial septal defect occluder combination in complex CEPS, supporting its usage as an alternative to the standard surgical treatment.Level of Evidence: Level 4, Case report.

Les thromboses veineuses splanchniques : étude monocentrique de 31 cas

Splanchnic vein thrombosis (SVT) denotes thrombosis of the hepatic venous system and of the extrahepatic portal system. They are often the manifestation of one or more underlying prothrombotic states and can sometimes present problems of therapeutic care. We report a monocentric study of 31 cases of SVT observed in an internal medicine department between January 2006 and June 2012. Epidemiological, clinical, laboratory, therapeutic and outcome data were analyzed. There was a slight female predominance (sex-ratio: 1.2). Median patient age at diagnosis was 37 years. Most cases of SVT were chronic (61.2%). Abdominal pain was the predominant symptom in acute cases while the predominant signs in chronic forms were signs of portal hypertension. Extrasplanchnic thrombosis was noted in seven patients (22.5%). Behçet's disease was the most common etiology (32%). The treatment was based on anticoagulants and corticosteroids and immunosuppressants when an inflammatory etiology was found. The average follow-up was 17 months. There were five deaths (16%). SVT often reflects the existence of a disease and/or an underlying prothrombotic state that should be identified to adapt the treatment and to avoid fatal complications.

CT Findings of Afferent Loop Varices After Bilioenteric Anastomosis in Patients With Malignant Disease

The purpose of this study was to determine the prevalence and CT findings of afferent jejunal loop varices after bilioenteric anastomosis in patients with malignancy. The study included 99 patients who underwent bilioenteric anastomosis for malignancy and follow-up CT examinations. Two abdominal radiologists reviewed CT images for the presence of afferent loop varices. They also assessed for the presence of extrahepatic portal occlusion or stenosis, other collateral vessels, ascites, splenomegaly, and recurrent tumor. Clinical data regarding age, sex, and histories of pancreaticoduodenectomy and surgery on the portal vein, radiation therapy, and postoperative gastrointestinal bleeding were collected. Univariate and multivariate analyses were performed to discern significant factors associated with afferent loop varices. Cumulative incidence was estimated with the Kaplan-Meier method. Afferent loop varices were found in 22 of the 99 patients (22.2%), and the 48-month cumulative incidence of afferent loop varices was 28.0%. Three of the 22 patients (13.6%) eventually experienced gastrointestinal bleeding. Obstruction in the extrahepatic portal system was the only feature that exhibited a significant difference in multivariate analysis (relative risk, 71.4; 95% CI, 7.4-1000). Afferent loop varices detected after bilioenteric anastomosis on CT images had a prevalence of 22.2% and can result in gastrointestinal bleeding. The presence of obstruction in the extrahepatic portal system was significantly associated with afferent loop varices.

Isolated hepatic metastasis with hepatic venous invasion and tumor thrombus in right atrium.

A 78-year-old man was transferred to our hospital for detailed examination and treatment of sudden-onset and rapidly increasing general edema. Routine blood counts and laboratory test results were normal. His medical history included chronic heart failure over a very long period under medical treatment and a sigmoidectomy for 4-cm sigmoid colon cancer carried out 3 years previously. At that time, an isolated small (about 2 cm) liver metastasis (segment 4) was also detected. Simultaneous segmentectomy and sigmoidectomy was initially considered, but the patient’s cardiac problem prevented it, and ultrasound (US)-guided radio-frequency ablation (RFA) was selected as the most appropriate treatment for the hepatic metastasis. During the following three years, the patient visited the outpatient clinic regularly, and several whole-body computed tomography (CT) studies at intervals of 6 months showed no abnormalities. They also showed the treated hepatic metastasis to be completely necrosed (Fig. 1). The patient was symptom-free for a long time, but very recently he suddenly became edematous. Abdominal US performed in our hospital using a Toshiba Aplio XG unit (Tokyo, Japan) revealed an approximately 6 9 5-cm irregular and ill-margined slightly hypoechoic tumor in S4, which extended posteriorly and directly invaded the left, middle, and right hepatic veins. The left and middle hepatic veins were completely occluded and were not recognized on B-mode US, and the right hepatic venous lumen was partially recognized. The tumor extended towards the right atrium, and a huge tumor thrombus was found there (Fig. 2). The intrahepatic and extrahepatic portal system was normal, but a large amount of ascites was also present throughout the whole abdominal cavity. Contrast-enhanced US using Sonazoid solution (GE Health Care), performed to observe blood flow in the liver, provided more detailed information about the hemodynamics of the lesion and the surrounding hepatic veins (mechanical index *0.25). Soon R. Satoyoshi (&) H. Kotanagi Department of Surgery, Akita Red Cross Hospital, 222-1 Kamikitade Saruta, Nawashirosawa, Akita 010-1495, Japan e-mail: rika-s@alto.ocn.ne.jp

Maximizing Use of Marginal Liver Grafts for Transplantation: A Case Report of Absent Donor Extrahepatic Portal Vein

Maximizing Use of Marginal Liver Grafts for Transplantation: A Case Report of Absent Donor Extrahepatic Portal Vein

A case of infarction of small bowel caused by thrombophilia secondary to hyperhomocysteinaemia

Dear Editor: Patients with coronary heart disease, stroke, peripheral arterial occlusive disease or venous thrombosis show a higher prevalence of hyperhomocysteinaemia (HHCY), between 20% and 50%. The meta-analysis of Homocysteine Studies Collaboration underlines the connection between HHCY and degenerative vascular disease. For a homocysteine (HCY) increase of 5 μmol/l, the odds ratio for ischemic heart disease was calculated to be 1.32, for venous thrombosis 1.60, and 1.59 for stroke. Bostom et al. demonstrate that starting from a plasma concentration threshold of 10 μmol/l, the vascular risk progressively increases following a linear dose-response relationship. We report a case of mesenteric vein thrombosis (MVT) with segmental transmural intestinal infarction of small bowel secondary to HHCY associated with portal vein thrombosis (PVT) at 1 month from the infarct onset. A 45-year-old man was admitted for diffuse colicky abdominal pain dating back to the previous 8 days. The pain progressively worsened particularly in the lower abdominal quadrants and became continuous at admission time. The patient was affected by mild hypertension which was not on medication. The clinical history was characterized by a splenectomy performed 2 months before for a voluminous splenic organized hematoma due to a motorcycle accident. Before splenectomy the patient underwent a MR and a CT scan, which did not show any alteration in the extrahepatic portal system. Moreover, since at discharge the platelet count was within normal limits, the patient was not treated with antiplatelet agents (dipyridamole, aspirin), but only with low-molecular-weight heparin (LMWH), that he carried out until the 30th post-operative day from splenectomy. On examination, the abdomen was tender and guarded. Plain abdominal film showed some small air-fluid levels and the abdominal ultrasonography revealed significant intraperitoneal fluid and no alteration of other abdominal organs, with no evidence of PVT. Laboratory tests showed a neutrophilic hyperleukocytosis. Because of the clinical, radiological, and laboratory findings, we decided for an emergency laparotomy. Intraoperatively, 28 cm of nonviable infarcted small bowel distal to the mid-jejunum with thickened mesentery was detected. No mechanical factors or strangulation were identified. The infarcted segment was resected but bowel continuity was not restored because of the severe intestinal conditions. Therefore, a terminal ileostomy was performed. The histological report showed an acute hemorrhagic necrosis of the intestinal loop involved. Immediately after the surgical approach, the patient was put on infusion, broad-spectrum antibiotics, and anticoagulant therapy with LMWH. The intraoperative findings and the patient’s young age, associated with the lack of anamnestic cardiovascular or thrombotic risk factors, suggested us to test the patient for coagulation disorders. The screening for primary thrombophilia was only significant for a raised serum HCY of 37 μmol/l. In the review by Herrmann et al., this value is classified as intermediary HHCY and it consists of heterozygous mutations of enzymes or severe vitamin Int J Colorectal Dis (2009) 24:1471–1473 DOI 10.1007/s00384-009-0729-8

Formação do sistema venoso portal em Chinchilla lanígera

O estudo referente à formação do sistema venoso portal (VP) em chinchila (Chinchilla lanigera) foi obtido através da análise de 10 animais dos quais o sistema porta extra-hepático sofreu injeção de látex corado, seguidos de dissecação em estereolupa. Logo após foram confeccionados esquemas a partir dos resultados observados, onde se averiguou que a VP é originada sempre através da confluência de duas raízes. A primeira destas raízes é o tronco mesentérico comum (TM), formado a partir da união das veias mesentérica cranial e mesentérica caudal. O TM recebe ainda como tributárias as veias pancreaticoduodenal cranial e gastroepiplóica direita. A segunda raiz, que apresenta menor calibre, é a veia lienal. Esta descrição foi observada em 100% dos espécimes mantendo-se constante, sugestionando que os resultados obtidos possam ser considerados como modelo padrão.

Research progress of vasculopathy in portal hypertension

Portal hypertension, one of the vascular diseases, not only has lesions in liver, but also changes in vascular structures and functions of extrahepatic portal system, systemic system and pulmonary circulation. The pathological changes of vasculopathy in portal hypertension include remodeling of arterialized visceral veins, intimal injury of visceral veins and destruction of contractile structure in visceral arterial wall. The mechanisms of vasculopathy in portal hypertension may be attributed to the changes of hemodynamics in portal system, immune response, gene modulation, vasoactive substances, and intrahepatic blood flow resistance. Portal hypertension can cause visceral hyperdynamic circulation, and the development and progression of visceral vasculopathy, while visceral vasculopathy can promote the development and progression of portal hypertension and visceral hyperdynamic circulation in turn. The aforementioned three factors interact in the pathogenesis of hepatic cirrhosis-induced portal hypertension and are involved in hemorrhage due to varicose vein rupture.

Le cavernome portal : diagnostic, étiologies et conséquences

Portal cavernoma is a network of veins whose caliber, initially millimetric or microscopic, is increased and which contain hepatopedal portal blood. It results from occlusion, thrombotic and always chronic, of the extra-hepatic portal system. Diagnosis is mainly done by imaging. Clinical signs of portal cavernoma are usually related to extra-hepatic portal hypertension (hematemesis due to rupture of oeso-gastric varices, splenomegaly, rectal bleeding from ano-rectal varices, growth retardation in children) and sometimes to the cause of portal hypertension (abdominal pain, venous bowel infarction). Occurrence of portal thrombosis is often the conjunction of a local cause and a prothrombotic disorder which must be systematically detected. Biliary consequences of cavernoma are related to compression of common bile duct and are usually asymptomatic. In case of jaundice or cholangitis, portal decompression by portosystemic shunt can be performed to treat both biliary symptoms and portal hypertension.

Liver of the “visible man”

Endoscopic surgery, also called minimally invasive surgery, is presumed to drastically reduce postoperative morbidity and thus to offer both human and economic benefits. For the surgeon, however, this approach leads to a number of gestural challenges that require extensive training to be mastered. In order to replace experimentation on animals and patients, we decided to develop a simulator for endoscopic surgery. To achieve this goal, a firm step was to develop a working prototype, a 'standard patient', on which the informatic and microengineering tools could be validated. We used the visible man dataset for this purpose. The external shape of the visible man's liver, his biliary passages and his extrahepatic portal system turned out to be fully within the standard pattern of normal anatomy. Anatomic variations were observed in the intrahepatic right portal vain, the hepatic veins and the arterial blood supply to the liver. Thus, the visible man dataset reveals itself to be very well suited for the simulation of minimally invasive surgical operation such as endoscopic cholecystectomy.

Computed tomography in the evaluation of cirrhosis and portal hypertension.

Patients with cirrhosis present a continuing diagnostic and therapeutic challenge. The status of their disease frequently changes, necessitating intensive serial evaluation. CT is an invaluable tool in the management of these patients because it can noninvasively provide vital information concerning liver size, contour, and occasionally hepatic parenchyma. More importantly, CT can demonstrate superficial and deep varices, assess the patency of the extrahepatic portal system, and detect other complications including ascites, hepatic steatosis, hemochromatosis, and hepatocellular carcinoma.

Gastrointestinal and Abdominal Manifestations of Agnogenic Myeloid Metaplasia

Gastrointestinal and abdominal manifestations of agnogenic myeloid metaplasia (AMM) were reviewed from the records of 122 patients with this disorder. Twenty-five percent of patients had abdominal pain of splenic origin. Hepatomegaly was present in 92 patients but excretory function of the liver generally remained good. Liver biopsies, carried out on 13 patients, showed AMM in all. The degree of hepatomegaly was paradoxically great in comparison to the relatively mild pathologic changes observed in liver biopsies. Nine patients had portal hypertension with esophageal varices and one of these also had ascites. Liver biopsies from three patients with symptomatic portal hypertension without obstruction of the extra-hepatic portal system showed only mild or moderate AMM. Our findings support the concept that increased portal blood flow is important in the pathogenesis of portal hypertension in patients wih AMM.

OBSTRUCTION TO THE EXTRAHEPATIC PORTAL SYSTEM IN CHILDHOOD

OBSTRUCTION TO THE EXTRAHEPATIC PORTAL SYSTEM IN CHILDHOOD

Roentgen demonstration of the venous circulation in the liver; portal venography.

Vasography has proved to be one of the most fruitful fields of roentgen investigation in recent years. With the hope of further extending its scope, a study of the roentgen demonstration of the portal circulation of the liver has been instituted at the University of Minnesota. This demonstration has been given the descriptive designation portal venography. The literature contains several references to portal venography, but these are concerned for the most part with the extra-hepatic portal system. Little interest has been evidenced in the intrahepatic portal circulation, which is the subject of this paper. Due to its relative inaccessibility, the various methods of approach to the portal circulation present difficulties from a diagnostic standpoint. An external approach is possible under certain specialized circumstances, such as a pre-existing portacaval anastomosis or portal obstruction with secondary dilatation of the periumbilical veins. The great majority of cases, however, require an internal appro...

Observations on the major radicals of the extrahepatic portal systems.

IN REVIEWING the literature on the anatomy of the extrahepatic portal system in the light of present day surgical procedures for the relief of portal hypertension, we were able to find little information on the length and caliber of the major vessels making up the extrahepatic portal systems. 1 In the performance of anastomoses between the portal system and the systemic circulation, it is important that the vessels used should have sufficient caliber to be able to transport enough blood in a given time to relieve the hypertension in the portal system. Because there is so little known about the length of these veins in the average person and because there is virtually nothing recorded in the literature concerning the caliber of the veins that are available for these anastomoses, this study was undertaken. Since the study was begun, Douglass, Baggenstoss and Hollinshead, 2 of the Mayo Clinic, have reported