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1.  Recipient and donor thrombophilia and the risk of portal venous thrombosis and hepatic artery thrombosis in liver recipients 
BMC Gastroenterology  2011;11:130.
Background
Vascular complications, such as HAT, are an important cause of graft loss and recipient mortality. We aimed to characterize post-transplant thrombotic events in a cohort of liver transplant recipients, and identify independent risk factors for these complications.
Methods
We conducted a thrombophilic study of 293 orthotopic liver transplants performed in the Digestive Surgery Department of the 12 de Octubre Hospital (Madrid, Spain) between January 2001 and December 2006.
Results
The most frequent post-transplant thrombotic events were HAT (9%) and PVT (1.7%). The one variable associated with post-transplant thrombotic event was a high fibrinogen level in the global cohort of liver transplantation. But toxicity as event post-OLT has been associated with post-transplant thrombotic event in the retrospective group and high fibrinogen level and low protein C levels were associated post-transplant thrombotic event in the prospective group. Liver disease relapse (HR 6.609, p < 0.001), high levels of FVIII (HR 1.008, p = 0.019)) and low levels of antithrombin (HR 0.946, p < 0.001) were associated with poor overall survival (OS).
In conclusion, high fibrinogen and decreased protein C levels were associated with allograft thrombosis. Further studies are required in order to assess the clinical relevance of these parameters in prospective studies and to study the effect of anticoagulation prophylaxis in this group of risk.
doi:10.1186/1471-230X-11-130
PMCID: PMC3287260  PMID: 22123067
2.  Blood loss, predictors of bleeding, transfusion practice and strategies of blood cell salvaging during liver transplantation 
World Journal of Hepatology  2013;5(1):1-15.
Blood loss during liver transplantation (OLTx) is a common consequence of pre-existing abnormalities of the hemostatic system, portal hypertension with multiple collateral vessels, portal vein thrombosis, previous abdominal surgery, splenomegaly, and poor “functional” recovery of the new liver. The intrinsic coagulopathic features of end stage cirrhosis along with surgical technical difficulties make transfusion-free liver transplantation a major challenge, and, despite the improvements in understanding of intraoperative coagulation profiles and strategies to control blood loss, the requirements for blood or blood products remains high. The impact of blood transfusion has been shown to be significant and independent of other well-known predictors of posttransplant-outcome. Negative effects on immunomodulation and an increased risk of postoperative complications and mortality have been repeatedly demonstrated. Isovolemic hemodilution, the extensive utilization of thromboelastogram and the use of autotransfusion devices are among the commonly adopted procedures to limit the amount of blood transfusion. The use of intraoperative blood salvage and autologous blood transfusion should still be considered an important method to reduce the need for allogenic blood and the associated complications. In this article we report on the common preoperative and intraoperative factors contributing to blood loss, intraoperative transfusion practices, anesthesiologic and surgical strategies to prevent blood loss, and on intraoperative blood salvaging techniques and autologous blood transfusion. Even though the advances in surgical technique and anesthetic management, as well as a better understanding of the risk factors, have resulted in a steady decrease in intraoperative bleeding, most patients still bleed extensively. Blood transfusion therapy is still a critical feature during OLTx and various studies have shown a large variability in the use of blood products among different centers and even among individual anesthesiologists within the same center. Unfortunately, despite the large number of OLTx performed each year, there is still paucity of large randomized, multicentre, and controlled studies which indicate how to prevent bleeding, the transfusion needs and thresholds, and the “evidence based” perioperative strategies to reduce the amount of transfusion.
doi:10.4254/wjh.v5.i1.1
PMCID: PMC3562721  PMID: 23383361
Transplantation surgery; Liver dysfunction; Liver transplant; Intraoperative bleeding; Intraoperative transfusion; Autotransfusion; Autologous transfusions; Transfusion requirements; Blood salvage; Cell salvage
3.  Portal vein thrombosis is a potentially preventable complication in clinical islet transplantation 
Percutaneous transhepatic portal access avoids surgery, but is rarely associated with bleeding or portal venous thrombosis. We herein report our large, single-center experience of percutaneous islet implantation, and evaluate risk factors of portal vein thrombosis and graft function.
Prospective data was collected on 268 intraportal islet transplants (122 subjects). A portal venous Doppler ultrasound was obtained on Days 1 and 7 days posttransplant.
Therapeutic heparinization, complete ablation of the portal catheter tract with Avitene paste, and limiting packed cell volume to < 5 ml completely prevented any portal thrombosis in the most recent 101 islet transplant procedures over the past 5 years. In the previous cumulative experience, partial thrombosis did not affect islet function. Standard liver volume correlated negatively (r=−0.257, P<0.001), and packed cell volume correlated positively with portal pressure rise (r=0.463, P<0.001). Overall, partial portal thrombosis occurred after 10 procedures (overall incidence 3.7%, most recent 101 patient incidence 0%). There were no cases of complete thrombosis, and no patient developed sequelae of portal hypertension.
In conclusion, portal thrombosis is a preventable complication in clinical islet transplantation, provided therapeutic anticoagulation is maintained, and packed cell volume is limited to <5 ml.
doi:10.1111/j.1600-6143.2011.03717.x
PMCID: PMC3226916  PMID: 21883914
Islet transplantation; risk factors; portal vein thrombosis; standard liver volume
4.  A High Incidence of Native Portal Vein Thrombosis in Veterans Undergoing Liver Transplantation 
The Journal of surgical research  1996;60(2):333-338.
The incidence of native portal vein thrombosis (PVT) in liver transplant recipients has been reported to range from 2.1 to 13.8%. We have identified an inordinately high incidence of PVT in a consecutive series of U.S. veterans receiving liver transplants. Between October 1989 and February 1994, 88 consecutive U.S. veterans received 99 orthotopic liver transplants under primary Tacrolimus (Prograf, formerly FK506) based immunosuppression. A number of clinical features were examined in an effort to identify risk factors for PVT and outcome was compared to patients without PVT. Native PVT was present in 23/88 (26%) patients. All of these patients were male U.S. veterans with a mean age of 47 years. When compared to the 65 patients without PVT, we found no significant difference with respect to underlying liver disease, age, Childs-Pugh score (mean = 12), UNOS status as defined prior to April 1995 (95% UNOS 3 or 4), previous abdominal surgery, or liver volume. Median blood loss for patients with PVT (21 units of packed red blood cells) was greater than for those without PVT (14 units, P = 0.04). Portal thrombectomy was performed in 11 patients, 11 patients required mesoportal jump grafts, and 1 patient had an interposition graft. Standard veno-venous bypass was used in 10 patients with single bypass utilized for the remainder. Actuarial patient survival for all patients at 1, 2, and 4 years was 88, 85, and 79%, respectively. There was no significant difference in patients with or without PVT. Patients with PVT had poorer graft survival than patients without PVT (86% vs 65%, 1 year; 81% vs 65%, 2 years; 81% vs 61%, 4 years; P = 0.03); however, this was not related to technical problems with the portal venous inflow. PVT occurred in 26% of U.S. veterans undergoing liver transplantation. These patients had significantly higher operative blood loss and poorer graft survival. The high incidence of postnecrotic cirrhosis in a predominantly male group of patients with advanced disease, as is evident by the high mean Childs-Pugh score and UNOS status, perhaps accounts for our observations.
doi:10.1006/jsre.1996.0053
PMCID: PMC2950617  PMID: 8598664
5.  The Incidence of Portal Vein Thrombosis at Liver Transplantation 
Hepatology (Baltimore, Md.)  1992;16(5):1195-1198.
The incidence of portal vein thrombosis was examined in 885 patients who received orthotopic liver transplantations for various end-stage liver diseases between 1989 and 1990. The thrombosis was classified into four grades. Grade 1 was thrombosis of intrahepatic portal vein branches, grade 2 was thrombosis of the right or left portal branch or at the bifurcation, grade 3 was partial obstruction of the portal vein trunk, and grade 4 was complete obstruction of the portal vein trunk. Among the 849 patients without previous portosystemic shunt, 14 patients (1.6%) had grade 1, 27 patients (3.2%) had grade 2, 27 patients (3.2%) had grade 3 and 49 patients (5.8%) had grade 4 portal vein thrombosis. The incidence of portal vein thrombosis was highest (34.8%) in the patients with hepatic malignancy in the cirrhotic liver, followed by those with Budd-Chiari syndrome (22.2%) and postnecrotic cirrhosis of various causes (15.7%). The patients with encephalopathy, ascites, variceal bleeding, previous splenectomy and small liver had significantly higher incidences of portal vein thrombosis than the others. The total incidence of portal vein thrombosis among the 36 patients with previous portosystemic shunt was 38.9%, which was significantly higher than that (13.8%) of those without shunt.
PMCID: PMC2989675  PMID: 1427658
6.  Splanchnic vein thrombosis in candidates for liver transplantation: usefulness of screening and anticoagulation 
Gut  2005;54(5):691-697.
Background and aims: Splanchnic vein thrombosis is a significant source of complications in candidates for liver transplantation. The aims of this study were: (a) to determine the prevalence of and risk factors for splanchnic vein thrombosis in cirrhotic patients awaiting transplantation and (b) to assess the usefulness of anticoagulation.
Methods: A total of 251 cirrhotic patients listed for transplantation were analysed. All underwent systematic screening for thrombosis with Doppler ultrasonography. During the second period of the study, all patients with thrombosis received anticoagulation up to transplantation while during the first period none had received anticoagulation.
Results: The incidence of splanchnic vein thrombosis at evaluation was 8.4%. Seventeen additional patients (7.4%) developed de novo thrombosis after evaluation. Independent risk factors for thrombosis were low platelet count (77.4 (36.3) v 111.6 (69.2) 109/l; p = 0.001), a past history of variceal bleeding (47.4% v 29.1%; p = 0.003), and a prolonged interval from listing to transplantation (8.5 (6.8) v 4.8 (4.4) months; p = 0.002). The proportion of partial or complete recanalisation was significantly higher in those who received (8/19) than in those who did not receive (0/10, p = 0.002) anticoagulation. Survival was significantly lower in those who had complete portal vein thrombosis at the time of surgery (p = 0.04).
Conclusion: These results support a systematic screening for splanchnic vein thrombosis in patients awaiting transplantation. They suggest that in these patients, anticoagulation is safe and has a significant impact on recanalisation as well as prevention of extension of thrombosis.
doi:10.1136/gut.2004.042796
PMCID: PMC1774501  PMID: 15831918
portal vein thrombosis; cirrhosis; portal hypertension; vitamin K antagonists; liver transplantation
7.  Portal Interventions in Liver Transplant Recipients 
Portal vein interventions in liver transplant recipients represent a group of interventions in the management of several disease entities including portal vein stenosis, portal vein thrombosis, and recurrent liver cirrhosis with portal hypertension with and without gastric varices. The procedures performed in these patient populations include portal vein angioplasty with or without stent placement for portal vein stenosis, portal vein thrombolysis with or without stent placement for portal vein thrombosis, transjugular intrahepatic portosystemic shunts or splenic embolization for cirrhosis, and balloon-occluded retrograde transvenous obliteration for gastric varices. This article discusses these disease entities and the minimal invasive procedures used in their management.
doi:10.1055/s-0032-1312570
PMCID: PMC3444876  PMID: 23729979
transplant; liver; vascular complications; endovascular; portal; portal hypertension; TIPS; BRTO
8.  Hepatic artery thrombosis following pediatric liver transplantation: Assessment of blood flow measurement in allografts 
Clinical transplantation  1989;3:184-189.
The purpose of this study was to define parameters which could be predictive of hepatic artery thrombosis, which continues to be a major complicating factor in pediatric liver transplantation. The hepatic blood flow of 14 pediatric liver patients (15 grafts) who weighed less than 15 kg was measured electromagnetically during orthotopic liver transplantation. The results of blood flow determination and the clinical data in 7 patients (8 grafts) who developed hepatic artery thrombosis were compared with those of 7 control patients. All patients with a hepatic arterial flow of less than 60 ml/min developed hepatic artery thrombosis (4/8 vs. 0/7; p < 0.05), and the patients with hepatic artery thrombosis exhibited higher total hepatic and portal vein flow per 100 gram of liver tissue (262 vs. 136 ml/min; p < 0.001 and 222 vs. 80 ml/min; p < 0.025, respectively) as well as longer cold preservation time (384 vs. 326 min; p < 0.025). The results of our study suggest that hepatic arterial flows of less than 60 ml/min are critical for the development of hepatic artery thrombosis, and that portal venous overflow and increased preservation times may contribute to the development of hepatic artery thrombosis.
PMCID: PMC3000218  PMID: 21151746
liver transplantation; hepatic artery thrombosis; flowmeter
9.  Sixty-four-slice computed tomography in surgical strategy of portal vein cavernous transformation 
AIM: To investigate the role of 64-slice computed tomography (CT) in portal vein cavernous transformation to determine surgical strategy.
METHODS: The site of lesions and extent of collateral circulation in 12 pediatric cases of cavernous transformation of the portal vein with surgical treatment were analyzed.
RESULTS: Eleven of 12 children had esophageal varices and were treated with lower esophageal and gastric devascularization and splenectomy, and the other case was only treated with splenectomy. There were eight cases with spontaneous spleen/stomach-renal shunt, four with Retzius vein opening, which was reserved during surgery. Three cases of lesions involving the intrahepatic portal vein (PV) were treated with living donor liver transplantation. One patient died from PV thrombosis after liver transplantation, and the rest had no significant complications.
CONCLUSION: The PV, its branches and collateral circulation were clearly seen by 64-slice spiral CT angiography, which helped with preoperative surgical planning.
doi:10.3748/wjg.v17.i38.4334
PMCID: PMC3214709  PMID: 22090790
Cavernous transformation; Portal vein; 64-slice computed tomography; Liver transplantation; Portal hypertension; Esophageal devascularization; Gastric devascularization
10.  Pediatric Liver Transplantation 
Radiology  1985;157(2):339-344.
The postoperative diagnostic imaging examinations of 44 children who underwent 59 orthotopic liver transplantations were reviewed. The imaging modalities used for the evaluation of suspected complications include plain roentgenography, ultrasonography (US), computed tomography (CT), nuclear scintigraphy, arteriography, percutaneous and operative cholangiography, and endoscopic retrograde cholangiopancreatography. The main postoperative complications included ischemia, thrombosis (hepatic artery and portal vein), infarction, obstruction or leakage of the biliary anastomosis, hepatic and perihepatic infection, and allograft rejection. US, the most frequently used abdominal imaging modality, was best suited for detection of biliary duct dilatation, fluid collections in or around the transplanted liver, and hepatic arterial, inferior vena caval, and portal vein thrombosis. CT was especially helpful in corroborating findings of infection and in locating abscesses. Technetium 99m sulfur colloid (early- and late-phase imaging) provided a sensitive, although nonspecific, means of assessing allograft vascularization and morphology. Angiography showed vascularity most clearly, and cholangiography was the most useful In the assessment of bile duct patency. A diagnostic imaging algorithm is proposed for evaluation of suspected complications.
PMCID: PMC3006431  PMID: 3901104
Liver, transplantation, 76.45; Surgery, complications
11.  Portal Vein Thrombosis and Liver Transplant Survival Benefit 
Background
Portal vein thrombosis (PVT) complicates the liver transplant operation and potentially affects waiting list survival. The implications on calculations of survival benefit, which balance both waiting list and posttransplant survival effects of PVT, have not been determined. The objective of this study is to describe the effect of PVT on the survival benefit of liver transplantation.
Methods
Using Scientific Registry of Transplant Recipients (SRTR) data on adult liver transplant candidates wait-listed between September 2001 and December 2007, Cox proportional hazards models were fitted to estimate the covariate-adjusted effect of PVT on transplant rate, waiting list survival and posttransplant survival. We then used sequential stratification to estimate liver transplant survival benefit by cross-classifications defined by MELD score and PVT status.
Results
The prevalence of reported PVT among 22,291 liver transplant recipients was 4.02% (N = 897). PVT was not a predictor of waiting list mortality (HR=0.90; p=0.23), but was a predictor of posttransplant mortality (HR=1.32; p=0.02). Overall, transplant benefit was not significantly different for patients with PVT vs. without PVT (p=0.21), but there was a shift in the benefit curve. Specifically, the threshold for transplant benefit among patients without PVT was MELD > 11, compared to MELD > 13 for PVT patients.
Conclusions
PVT is associated with significantly higher posttransplant mortality, but does not affect waiting list mortality. Among low-MELD patients, PVT is associated with less transplant survival benefit. Clinicians should carefully consider the risks of liver transplantation in clinically stable patients who have PVT.
doi:10.1002/lt.22105
PMCID: PMC2915450  PMID: 20677291
mesenteric thrombosis; liver transplant survival; waiting list mortality; posttransplant survival; MELD score
12.  Splanchnic vein thrombosis, the onset manifestation in JAK positive Chronic Myeloproliferative Disorders Neoplasms  
Journal of Medicine and Life  2011;4(1):97-101.
Background: Patients with Myeloproliferative Neoplasms–(MPN) have a high risk of thrombotic complications. Portal vein thrombosis is a severe complication, which in many cases, appears at the onset of the disease; the risk factors are related to the presence of qualitatively altered thrombocytes and leucocytes, leading to their activation and appearance of leukocytes–platelet–aggregates; anomalies of portal vein endothelial cells are also implicated. The presence of JAK2V617F mutation increases the risk for splahnic thrombosis.
Methods and results: We present three patients with portal vein thrombosis and Budd Chiari syndrome, who were further diagnosed with MPN–the thrombosis was the onset event of the disease
Conclusion: Patients were diagnosed with thrombosis of the portal vein before being diagnosed with MPN. Splenectomy was not associated with risk of thrombosis for the two cases in which it was performed; for one case, splenectomy was a therapeutic method to resolve portal hypertension. All patients had homozygous JAK2 mutation, which is associated in recent studies with increased risk of portal, mesenteric thrombosis. The high number of platelet was difficult to control for all patients. Bone marrow biopsy and determination of JAK status are valuable investigations for patients who have splenoportal thrombosis, with no apparent identifiable cause.
PMCID: PMC3056429  PMID: 21505581
portal thrombosis; chronic myeloproliferative disorders; JAK positive
13.  Angiography of Liver Transplantation Patients 1 
Radiology  1985;157(2):305-311.
Over 45 months, 119 angiographic examinations were performed in 95 patients prior to liver transplantation, and 53 examinations in 44 patients after transplantation. Transplantation feasibility was influenced by patency of the portal vein and inferior vena cava. Selective arterial portography, wedged hepatic venography, and transhepatic portography were used to assess the portal vein if sonography or computed tomography was inconclusive. Major indications for angiography after transplantation included early liver failure, sepsis, unexplained elevation of liver enzyme levels, and delayed bile leakage, all of which may be due to hepatic artery thrombosis. Other indications included gastrointestinal tract bleeding, hemobilia, and evaluation of portal vein patency in patients with chronic rejection who were being considered for retransplantation. Normal radiographic features of hepatic artery and portal vein reconstruction are demonstrated. Complications diagnosed using results of angiography included hepatic artery or portal vein stenoses and thromboses and pancreaticoduodenal aneurysms. Intrahepatic arterial narrowing, attenuation, slow flow, and poor filling were seen in five patients with rejection
PMCID: PMC2962557  PMID: 3901102
Liver; angiography; 952.122; 957.124; Liver; transplantation; 761.45
14.  Contrast-enhanced ultrasound with SonoVue in the evaluation of postoperative complications in pediatric liver transplant recipients 
Journal of Ultrasound  2007;10(2):99-106.
Purpose
To evaluate the utility of contrast-enhanced sonography in the study of pediatric liver transplant recipients and its potential impact in reducing the need for invasive diagnostic procedures.
Materials and methods
From October 2002 to December 2003 we performed routine color Doppler ultrasound and contrast-enhanced ultrasound studies on 30 pediatric patients who had undergone liver transplantation. Findings indicative of complications were confirmed with invasive studies (angiography, computed tomography, and PTC).
Results
Contrast-enhanced sonography correctly identified four of the five cases of hepatic artery thrombosis and all those involving the portal (n = 6) and hepatic vein (n = 3) thrombosis. It failed to identify one case of hepatic artery thrombosis characterized by collateral circulation arising from the phrenic artery and the single case of hepatic artery stenosis. The latter was more evident on color Doppler, which revealed a typical tardus parvus waveform. The use of contrast offered no significant advantages in the study of biliary complications although it did provide better visualization of bile leaks.
Conclusions
Contrast-enhanced sonography improves diagnostic confidence and reduces the need for more invasive imaging studies in the postoperative follow-up of pediatric liver transplant recipients.
doi:10.1016/j.jus.2007.02.008
PMCID: PMC3478709  PMID: 23396596
Split liver; Pediatric liver transplantation; Complications; Contrast-enhanced sonography
15.  Splanchnic vein thrombosis following renal transplantation: a case report 
BMC Nephrology  2013;14:161.
Background
Recurrent episodes of venous thrombosis have been closely correlated with JAK2 V617F mutation. Upto date, JAK2 gene mutation has not been defined as a prothrombic risk factor in renal transplant recipients. Herein; we present a case of portosplenic vein thrombosis in a primary renal transplant recipient with JAK2 V617F mutation who had no history of prior venous thromboembolism or thrombophilia.
Case presentation
A 59 year old female caucasian patient with primary kidney transplant admitted with vague abdominal pain at left upper quadrant. Abdominal doppler ultrasound and magnetic resonance imaging angiography demonstrated splanchnic vein thrombosis (SVT). The final diagnosis was SVT due to MPD (essential thrombocytosis, ET) with JAK2 V617F mutation. After 3 months of treatment with warfarin (≥5 mg/day, to keep target INR values of 1.9-2.5), control MRI angiography and doppler USG demonstrated partial (>%50) resolution of thrombosis with recanalization of hepatopedal venous flow. The patient is still on the same treatment protocol without any complication.
Conclusion
JAK2 V617F mutation analysis should be a routine procedure in the diagnosis and treatment of kidney transplant patients with thrombosis in uncommon sites.
doi:10.1186/1471-2369-14-161
PMCID: PMC3723440  PMID: 23876158
Thrombosis; Portosplenic vein thrombosis; JAK2 gene mutation; Myeloproliferative disorders; Renal transplantation
16.  Huge extrahepatic portal vein aneurysm as a late complication of liver transplantation 
World Journal of Hepatology  2010;2(5):201-202.
A 60-year-old male underwent orthotopic liver transplantation because of hepatitis C virus related cirrhosis. After 12 d, the patient underwent re-transplantation due to primary graft non function. One year later the patient developed a thrombosis of the main portal vein needing a surgical revision. After 11 years the patient was operated on because of a clinical picture of intestinal occlusion. As an incidental finding, a large aneurysm of the main portal vein was diagnosed. The incidence of intra- and extrahepatic Portal vein aneurysms (PVAs) is not clear. To the best of our knowledge, only one case of intrahepatic PVA in a liver transplant has been reported in the literature. In addition, we have found no documented cases of extrahepatic PVAs in liver transplanted patients.
doi:10.4254/wjh.v2.i5.201
PMCID: PMC2999282  PMID: 21160997
Portal vein aneurysm; Late complication; Liver transplantation; Portal hypertension; Doppler ultrasound
17.  Liver transplantation for “mass-forming” sclerosing cholangitis after laparoscopic cholecystectomy☆ 
INTRODUCTION
Chronic biliary obstruction consequence of a bile duct injury may require liver transplantation (LT) in case of secondary biliary cirrhosis, intractable pruritus or reiterate episodes of cholangitis. “Mass-forming” sclerosing cholangitis leading to secondary portal vein thrombosis and pre-sinusoidal portal hypertension has not been reported so far.
PRESENTATION OF CASE
We present the case of a patient who underwent laparoscopic cholecystectomy for Mirizzi syndrome. The persistent bile duct obstruction due to a residual gallstone fragment was treated by a prolonged biliary stenting. Following repeated bouts of cholangitis, a fibrous centrohepatic scar developed, conglobating and obstructing the main branches of the portal vein and of the biliary tree. The patient developed secondary portal vein thrombosis and portal hypertension. After an extensive diagnostic work-up, including surgical exploration to rule out malignancy, the case was successfully managed by liver transplantation.
DISCUSSION
Mass-forming sclerosis of the bile duct and biliary bifurcation may develop as a consequence of chronic biliary obstruction and prolonged stenting. Secondary portal vein thrombosis and pre-sinusoidal portal hypertension represents an unusual complication, mimicking Klatskin tumor.
CONCLUSION
A timely and proper management of post-cholecystectomy complications is of mainstay importance. Early referral to a specialized hepato-biliary center is strongly advised.
doi:10.1016/j.ijscr.2013.07.021
PMCID: PMC3785926  PMID: 23995476
Biliary obstruction; Biliary stent; Cholangiocarcinoma; Gallstones; Portal hypertension
18.  Portal Cavernoma during Pregnancy 
Portal vein thrombosis (PVT) is characterized by the obstruction of the portal venous system. The venous obstruction can be partial or complete and it is caused by thrombogenic conditions (acquired or hereditary) or nonthrombotic factors. The acquired conditions include abdominal inflammation, infections, surgery, myeloproliferative disorders, obesity, oral contraceptive intake, pregnancy, and postpartum period. Occasionally, it is not possible to recognize any overt cause of PVT. During pregnancy there is an increased venous thromboembolism risk mainly in the systemic venous system and the PVT can occur, but there are no data about its exact prevalence, etiology, and outcome. The portal cavernoma is the cavernomatous transformation of the portal vein. It is a consequence of chronic PVT and occurs when myriads of collateral channels develop to bypass the occlusion. The clinical presentation includes hematemesis due to variceal bleeding, ascites or anaemia, and splenomegaly. The cavernous transformation of the portal vein is easily diagnosed by sonography. We report our case of a 32-year-old, gravida 3 para 2, pregnant woman admitted to our hospital at 13 weeks and 1 day of gestation, clinically asymptomatic. Laboratory test, ultrasound, and endoscopic evaluation were negative. After a detailed counseling, the patient decided on termination of pregnancy at 15 weeks and 1 day of gestation.
doi:10.1155/2013/396083
PMCID: PMC3872388  PMID: 24392231
19.  Management of Anticoagulation for Portal Vein Thrombosis in Individuals with Cirrhosis: A Systematic Review 
Non-neoplastic portal vein thrombosis (PVT) is an increasingly recognized complication of liver cirrhosis. It is often diagnosed fortuitously and can be either partial or complete. The clinical significance of PVT is not obvious except in some situations such as when patients are on the waiting list for liver transplantation. The only known therapy is anticoagulation which has been shown to permit the disappearance of thrombosis and to prevent further extension. Anticoagulation is a challenging therapy in individuals with liver cirrhosis because of the well-recognized coagulation abnormalities observed in that setting and because of the increased risk of bleeding, especially from gastrointestinal tract caused by portal hypertension. We herein review the current knowledge on that topic in order to highlight the advantages and disadvantages of the currently proposed therapeutic attitudes in face of the diagnosis of PVT in individuals with cirrhosis.
doi:10.1155/2012/672986
PMCID: PMC3388284  PMID: 22778970
20.  Causes of Adult Splanchnic Vein Thrombosis in the Mediterranean Area 
The term splanchnic vein thrombosis encompasses Budd-Chiari syndrome (BCS), extrahepatic portal vein obstruction (EHPVO), and mesenteric vein thrombosis.
Risk factors can be local or systemic. A local precipitating factor is rare in BCS, while it is common in patients with portal vein thrombosis. Chronic myeloproliferative neoplasms (MPN) are the leading systemic cause of splanchnic vein thrombosis, and are diagnosed in half BCS patients and one-third of EHPVO patients; the somatic mutation JAK2 V617F is detectable in a large majority of patients with overt MPN, and up to 40% of patients without overt MPN. Inherited thrombophilia is present in at least one-third of patients, and the factor V Leiden or the prothrombin G20210A mutations are the most common mutations found in BCS or EHPVO patients, respectively. Multiple factors are present in approximately one-third of patients with BCS and two- thirds of patients with portal vein thrombosis.
In a few patient series from the Southern Mediterranean area the high prevalence of MPN and thrombophilia as underlying cause of BCS is confirmed, although the data should be considered preliminary. Peculiar risk factors present in the area are Behçet’s disease and hydatidosis; moreover, membraneous webs, typically found in Asian patients, are present in a significant portion of cases.
doi:10.4084/MJHID.2011.063
PMCID: PMC3248340  PMID: 22220260
21.  Portal vein thrombosis and outcomes in pediatric liver transplant candidates and recipients in the United States 
The effect of occlusive portal vein thrombosis (PVT) on the mortality of pediatric liver transplant candidates and recipients is poorly defined. Using standard multivariable techniques, we studied the relationship between PVT and waiting list and post-transplant survival using data from the Scientific Registry of Transplant Recipients between September 2001 and December 2007. A total of 5,087 candidates and 3,630 liver transplant recipients were evaluated during the period. PVT was found in 1.4% (n = 70) of liver transplant candidates and 3.7% (n = 136) of recipients. PVT was not associated with increased waiting list mortality (HR = 0.9; 95% CI, 0.8–1.1, p = 0.35). Conversely, PVT patients had significantly lower unadjusted survival in the post-transplant period (p = 0.01). PVT was independently associated with increased post-transplant mortality in multivariate models (HR = 2.9; 95%CI 1.6–5.3, p = 0.001 at 30 days and HR = 1.7; 95% CI, 1.1–2.4; p = 0.01 overall survival). The presence of PVT in pediatric liver candidates was not associated with increased waiting list mortality but was clearly associated with post-transplant mortality, especially in the immediate post-operative period.
doi:10.1002/lt.22371
PMCID: PMC3162124  PMID: 21744467
22.  Portal vein thrombosis: Insight into physiopathology, diagnosis, and treatment 
Portal vein thrombosis (PVT) is a relatively common complication in patients with liver cirrhosis, but might also occur in absence of an overt liver disease. Several causes, either local or systemic, might play an important role in PVT pathogenesis. Frequently, more than one risk factor could be identified; however, occasionally no single factor is discernable. Clinical examination, laboratory investigations, and imaging are helpful to provide a quick diagnosis, as prompt treatment might greatly affect a patient’s outcome. In this review, we analyze the physiopathological mechanisms of PVT development, together with the hemodynamic and functional alterations related to this condition. Moreover, we describe the principal factors most frequently involved in PVT development and the recent knowledge concerning diagnostic and therapeutic procedures. Finally, we analyze the implications of PVT in the setting of liver transplantation and its possible influence on patients’ future prognoses.
doi:10.3748/wjg.v16.i2.143
PMCID: PMC2806552  PMID: 20066733
Portal vein thrombosis; Portal hypertension; Thrombophilic factors; Liver cirrhosis; Liver transplantation; Anticoagulants
23.  Is thrombophilia a major risk factor for deep vein thrombosis of the lower extremities among Lebanese patients? 
Aim
Factor V Leiden (R506Q) mutation is the most commonly observed inherited genetic abnormality related to vein thrombosis. Lebanon has one of the highest frequencies of this mutation in the world with a prevalence of 14.4% in the general population. The aim of this study is to define risk factors including inherited genetic abnormalities among Lebanese patients with lower extremity deep vein thrombosis. We report the clinical outcome of patients with thrombophilia.
Methods
From January 1998 to January 2008, 162 patients (61 males and 101 females) were diagnosed with lower extremity deep vein thrombosis. Mean age was 61 years (range: 21 to 95 years).
Results
The most frequent risk factors for vein thrombosis were surgery, advanced age, obesity, and cancer. Twenty-five patients had thrombophilia, 16 patients had factor V Leiden (R506Q) mutation, and seven patients had MTHFR C677T mutation. Ninety-two percent of patients screened for thrombophilia were positive. Screening was requested in young patients (16), patients with recurrent (11), spontaneous (8), and extensive (5) venous thrombosis, familial history (5), pregnancy (4), estroprogestative treatment (3), and air travel (1). Nine patients had one, 11 patients had two, and five had three of these conditions. Follow-up (6 to 120 months) of these 25 patients treated with antivitamin K did not reveal recurrences or complications related to venous thromboembolism.
Conclusion
Factor V Leiden mutation followed by MTHFR mutation are the most commonly observed genetic abnormalities in these series. Defining risk factors and screening for thrombophilia when indicated reduce recurrence rate and complications. Recommendations for thrombophilia screening will be proposed.
PMCID: PMC2725795  PMID: 19688103
venous thrombosis; risk factors; genetics; factor V Leiden; prothrombin G20210A; MTHFR C677T
24.  Splanchnic Vein Thrombosis in the Mediterranean Area in Children 
Abdominal venous thrombosis may present as splanchnic venous thrombosis (SVT) (occlusion of portal, splenic, superior or inferior mesenteric veins) or Budd- Chiari Syndrome (BCS) (thrombosis of inferior vena cava and/or hepatic veins). The aim of this review is to report the scanty data available for SVT in the South Mediterranean area. In one Egyptian study, the possible circumstantial risk factors for portal vein thrombosis (PVT) were found in 30% of cases: 19% neonatal sepsis, 8.7% umbilical catheterization, 6% severe gastroenteritis and dehydration. Another Egyptian study concluded that hereditary thrombophilia was common in children with PVT (62.5%), the commonest being factor V Leiden mutation (FVL) (30%). Concurrence of more than one hereditary thrombophilia was not uncommon (12.5%). The first international publication on hepatic veno-occlusive disease (VOD) in Egypt was in 1965 in children who rapidly develop abdominal distention with ascites and hepatomegaly. This disease was more frequent in malnourished children coming from rural areas; infusions given at home may contain noxious substances that were hepatotoxic and infections might play a role. VOD of childhood is rarely seen nowadays. Data from South Mediterranean area are deficient and this may be attributable to reporting in local medical journals that are difficult to access. Medical societies concerned with this topic could help distribute this information.
doi:10.4084/MJHID.2011.027
PMCID: PMC3152449  PMID: 21869913
25.  Portal vein thrombosis following laparoscopic cholecystectomy complicated by dengue viral infection: a case report 
Introduction
Portal vein thrombosis is an uncommon post-operative complication following abdominal surgery. Although therapeutic anticoagulation is recommended, this treatment may be questionable when the patient has an associated bleeding diathesis.
Case presentation
We report a case of a 63-year-old woman of Asian Indian ethnicity who developed portal vein thrombosis following an uneventful laparoscopic cholecystectomy for symptomatic gallstones. Her condition was further complicated by dengue viral infection in the post-operative period, with thrombocytopenia immediately preceding the diagnosis of portal vein thrombosis. The etiological connections between dengue viral infection with thrombocytopenia, laparoscopic cholecystectomy, portal vein thrombosis as well as the treatment dilemmas posed in treating a patient with portal vein thrombosis with a bleeding diathesis are discussed.
Conclusion
When portal vein thrombosis occurs in patients with contraindications to anticoagulation, there is a role for initial conservative management without aggressive anticoagulation therapy and such patients must be approached on an individualized basis.
doi:10.1186/1752-1947-5-126
PMCID: PMC3073920  PMID: 21450059

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