Chronic Portal Vein Research Articles

Diagnostic challenges in non-cirrhotic portal hypertension - porto sinusoidal vascular disease.

Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension, which differ from cirrhosis through histological alterations, hemodynamic characterization and, clinical outcome. Because of the similarities in clinical presentation and imaging signs, frequently these patients, and particularly those with porto-sinusoidal vascular disease (PSVD), are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis. The most challenging differentiation to be considered is between PSVD and cirrhosis and, although not pathognomonic, liver biopsy is still the standard of diagnosis. Although they still require extended validation before being broadly used, new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease, like transient elastography, contrast-enhanced ultrasound or metabolomic profiling, have shown promising results. Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction, especially now when it is known that 40% of patients suffering from PSVD develop portal vein thrombosis. In this particular case, once the portal vein thrombosis occurred, the diagnosis of PSVD is impossible according to the current guidelines. Moreover, so far, the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances. In this review we highlighted the diagnostic challenges regarding the PSVD, as well as the current techniques used in the evaluation of these patients.

PORTAL CAVERNOMA AS A CAUSE OF HEMATEMESIS IN A YOUNG UNMARRIED FEMALE: A CASE REPORT

Thrombotic incidents are usually uncommon in unusual sites like portal vein and further in the adolescent age group which presents a potential diagnostic dilemma. Chronic non-cirrhotic non-malignant portal vein thrombosis (PVT) can present at any age group, usually as acute upper gastrointestinal (UGI) bleed. Diagnosis depends on imaging modalities involving Doppler, computed tomography (CT), magnetic resonance imaging, and portography. We report the case of a 20-year-old unmarried girl presented with multiple episodes of hematemesis. Ultrasonography abdomen and CT abdomen revealed PVT with portal cavernous transformation of the portal vein. Her prothrombin time and activated partial thromboplastin time were normal. Her connective tissue workup was normal. A diagnosis of PVT secondary to umbilical vein sepsis during infancy was made. Adolescent patients presented with acute hematemesis should not be neglected for some local cause of UGI bleed but should be evaluated for other rare causes like PVT, for early diagnosis and management to reduce feared complications.

What is the accuracy of platelet count and platelet count/spleen length ratio for the diagnosis of esophageal varices in adults with chronic liver disease or portal vein hypertension?

What is the accuracy of platelet count and platelet count/spleen length ratio for the diagnosis of esophageal varices in adults with chronic liver disease or portal vein hypertension?

Sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including bleeding (haemorrhage) from oesophageal and gastrointestinal varices. Variceal bleeding commonly occurs in children with chronic liver disease or portal vein obstruction. Therefore, prevention is important. Primary prophylaxis of variceal bleeding in adults is the established standard of care because of the results of numerous randomised clinical trials demonstrating the efficacy of non-selective beta-blockers or endoscopic variceal ligation in decreasing the incidence of variceal bleeding. In children, band ligation, beta-blockers, and sclerotherapy have been proposed as alternatives for primary prophylaxis of oesophageal variceal bleeding. However, it is unknown whether those treatments are of benefit or harm when used for primary prophylaxis in children. To assess the benefits and harms of sclerotherapy compared with sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase Elsevier, and two other registers in February 2019. We scrutinised the reference lists of the retrieved publications, and performed a manual search of the main paediatric gastroenterology and hepatology conference (NASPGHAN and ESPGHAN) abstracts from January 2008 to December 2018. We searched four registries for ongoing clinical trials. There were no language or document type restrictions. We included randomised clinical trials irrespective of blinding, language, or publication status assessing sclerotherapy versus sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We used standard Cochrane methodology to perform this systematic review. We used the intention-to-treat principle to analyse outcome data, and GRADE to assess the certainty of evidence per outcome. We found only one randomised clinical trial that fulfilled our inclusion criteria. The trial was at high risk of bias. The trial included 108 Brazilian children with median age of 4.3 years (range 11 months to 13 years). Fifty-six children were randomised to prophylactic sclerotherapy (ethanolamine oleate 2%) and 52 children to no intervention (control). Children were followed up for a median of 4.5 years. Eight children (six from the sclerotherapy group versus two from the control group) dropped out before the end of the trial. The follow-up was from 18 months to eight years. Mortality was 16% (9/56 children) in the sclerotherapy group versus 15% (8/52 children) in the control group (risk ration (RR) 1.04, 95% confidence interval (CI) 0.44 to 2.50; very low-certainty evidence). Upper gastrointestinal bleeding occurred in 21% (12/56) of the children in the sclerotherapy group versus 46% (24/52) in the control group (RR 0.46, 95% CI 0.26 to 0.83; very low-certainty evidence). There were more children with congestive hypertensive gastropathy in the sclerotherapy group than in the control group (14% (8/56) versus 6% (3/52); RR 2.48, 95% CI 0.69 to 8.84; very low-certainty evidence). The incidence of gastric varices was similar between the sclerotherapy group and the control group (11% (6/56) versus 10% (5/52); RR 1.11, 95% CI 0.36 to 3.43; very low-certainty evidence). The incidence of bleeding from gastric varices was higher in the sclerotherapy group than in the control group (4% (3/56) versus 0% (0/52); RR 6.51, 95% CI 0.34 to 123.06; very low-certainty evidence). The study did not assess health-related quality of life. Oesophageal variceal bleeding occurred in 5% (3/56) of the children in the sclerotherapy group versus 40% (21/52) of the children in the control group (RR 0.13, 95% CI 0.04 to 0.42; very low-certainty evidence). The most prevalent complications (defined as non-serious) were pain and fever after the procedure, which promptly resolved with analgesics. However, numerical data on the frequency of these adverse events and their occurrences in the two groups were lacking. No funding information was provided. We found no ongoing trials. The evidence, obtained from one randomised clinical trial at high risk of bias, is very uncertain on whether sclerotherapy has an influence on mortality and if it may decrease first upper gastrointestinal or oesophageal variceal bleeding in children. The evidence is very uncertain on whether sclerotherapy has an influence on congestive hypertensive gastropathy, incidence on gastric varices, and incidence of bleeding from gastric varices. Health-related quality of life was not measured. There were no serious events caused by sclerotherapy, and analysis of non-serious adverse events could not be performed due to lack of numerical data. The GRADE assessment of each outcome showed a very low-certainty evidence. The results of the trial need to be interpreted with caution. Larger randomised clinical trials, following the SPIRIT and CONSORT statements, assessing the benefits and harms of sclerotherapy compared with sham or no intervention for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis are needed. The trials should include important clinical outcomes such as death, failure to control bleeding, and adverse events.

Clinical case of chronic portal vein thrombosis

Portal vein thrombosis (PVT) is a form of venous thrombosis that causes impaired or terminated blood flow in the portal vein. PVT is the main reason for the development of extrahepatic portal hypertension. The article presents a clinical case of chronic PVT with signs of severe portal hypertension and ascites. A feature of the case is that in a patient with suspected decompensated cirrhosis of the liver with the ineffectiveness of standard treatment, PVT was detected. The cause of PVT was mechanical trauma of the portal vein and hemocoagulation thrombophilia. For health reasons, the patient was prescribed rivaroxaban. Rivaroxaban is an oral anticoagulant from the group of Xa inhibitors. Against the background of treatment, recanalization of PVT was noted in the patient. Our experiment has shown that rivaroxaban is a promising drug for treatment of PVT.

Transjugular intrahepatic portosystemic shunt is an effective and safe treatment of cirrhotic patients with portal vein thrombosis or cavernoma

TIPS is a well established treatment in selected patients with refractory ascites and/or gastrointestinal bleeding (GIB). Whether TIPS may also be a safe and effective therapeutic option for cirrhotic patients with chronic portal vein thrombosis (PVT) or cavernoma is still not clear. We Investigated the feasibility, safety and efficacy of TIPS in cirrhotic patients with PVT. All consecutive patients with PVT or cavernoma undergoing TIPS placement from April 2014 to April 2019 in GOM Niguarda are here considered. All patients completed at least 6 months of follow-up after TIPS. Of 153 patients undergoing TIPS insertion, 50(32%) had cirrhosis complicated by PVT or cavernoma. In 11 cases(22%) PVT was associated with GIB, in 16 (32%) with ascites. 32(64%) patients had failed recanalization while on anticoagulation, 18 could not be anticoagulated for contraindications. Majority of patients was male (72%), leading etiologies were post-viral and post-alcohol cirrhosis. Median age was 57 years. Median MELD was 14 (range 8–18). TIPS placement was technically feasible in 96%(48/50)of patients. PVT extension is described in table 1. Trans-splenic approach was necessary in 8/50(16%). Early post-TIPS complications were: hemoperitoneum (3 cases,6%), sovra-hepatic vein occlusion (1 case,2%) and systemic infection (6 cases, 12%). During follow-up 94%(15/16) recovered ascites and 15 patients(30%) had at least 1 portosystemic-encephalopathy episode; 33/48 (68%) patients had a complete recanalization and 11/48 (22%) had a partial recanalization. Liver related mortality was 6%. 18 patients were listed to transplantation after achieving portal patency, 6 have been already transplanted, 8 are on waiting list and 4 could be delisted for clinical improvement. TIPS in cirrhotic patients with PVT with or w/o cavernoma is effective and well tolerated. Complete recanalization was achieved in 2 patients out of three, obtaining a clinical beneficial effect on symptomatic PH. Recanalization allowed patients to be listed for LT when needed

Sclerotherapy versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including bleeding (haemorrhage) from oesophageal and gastrointestinal varices. Variceal bleeding commonly occurs in children with chronic liver disease or portal vein obstruction. Prevention is therefore important. Primary prophylaxis of variceal bleeding in adults is the established standard of care because of the results of numerous randomised clinical trials demonstrating the efficacy of non-selective beta-blockers or endoscopic variceal ligation in decreasing the incidence of variceal bleeding. However, sclerotherapy is the only endoscopic prophylactic option currently available in infants weighing less than 10 kg of bodyweight due to the size of the endoscopic ligator. To assess the benefits and harms of sclerotherapy versus any type of beta-blocker for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, PubMed, Embase Elsevier, LILACS (Bireme), and Science Citation Index Expanded (Web of Science) in February 2019. We scrutinised the reference lists of the retrieved publications and performed a manual search from the main paediatric gastroenterology and hepatology conferences (NASPGHAN and ESPGHAN) abstract books from January 2008 to December 2018. We searched ClinicalTrials.gov, FDA, EMA, and WHO, for ongoing clinical trials. There were no language or document type restrictions. We planned to include randomised clinical trials irrespective of blinding, language, or publication status, assessing sclerotherapy versus any type of beta-blocker for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. We planned to include quasi-randomised and other observational studies retrieved with the searches for randomised clinical trials for report of harm. We planned to collect and summarise data from randomised clinical trials as described in our protocol, using standard Cochrane methodologies. We found no randomised clinical trials assessing sclerotherapy versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis. Randomised clinical trials assessing the benefits or harms of sclerotherapy versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children and adolescents with chronic liver disease or portal vein thrombosis are lacking. Therefore, trials with adequate power and proper design, assessing the benefits and harms of sclerotherapy versus beta-blockers on patient-relevant clinical outcomes such as mortality, failure to control bleeding, and adverse events are needed. Unless such trials are conducted and the results become published, we cannot make any conclusions regarding the benefits or harms of the two interventions.

Transsplenic portal vein recanalization and direct intrahepatic portosystemic shunt placement to optimize liver transplantation

BackgroundPercutaneous trans-splenic portal vein recanalization (PVR) has been reported for facilitation of transjugular intrahepatic portosystemic shunts (TIPS), however has not been applied to patients undergoing direct intrahepatic portosystemic shunt (DIPS). We report the utilization of trans-splenic-PVR with DIPS creation in a patient with chronic portal and hepatic vein occlusions undergoing liver transplantation evaluation.Case presentationA 48-year-old male with decompensated alcoholic cirrhosis complicated by refractory ascites, hepatic encephalopathy, and variceal bleeding underwent CT that demonstrated chronic occlusion of the hepatic veins (HV), extrahepatic portal vein (PV), and superior mesenteric vein (SMV). Due to failed attempts at TIPS at outside institutions, interventional radiology was consulted for portal vein recanalization (PVR) with TIPS to treat the portal hypertension and ascites and also facilitate an end-to-end PV anastomosis at transplantation. After an initial hepatic venogram confirmed chronic HV occlusion, a DIPS with trans-splenic PVR was planned. The splenic vein was accessed under sonographic guidance using a micropuncture set and subsequently upsized to a 6 French sheath over a stiff guidewire. A splenic venogram via this access confirmed occlusion of the PV with drainage of the splenic vein (SV) through gastric varices. The thrombosed PV was then recanalized and angioplastied to restore PV flow via the transsplenic approach. A transjugular liver access kit with a modified 21-gauge needle was advanced into the IVC through the internal jugular vein (IJV) sheath and directed towards the target snare in PV. The needle was used to subsequently puncture the PV through the caudate lobe and facilitate placement of a wire into the SV. The initial portosystemic gradient (PSG) was 20 mmHg. The IJV sheath was advanced through the hepatic parenchymal tract into the main-PV and a stent-graft was placed across the main PV and into the IVC. A portal venogram demonstrated brisk blood flow through the DIPS, resolution of varices and a PSG of 8 mmHg. One month after the procedure, the patient had a significant reduction in ascites and MELD-NA score. Patient is currently listed and awaiting transplantation.ConclusionsIn the setting of chronically occluded portal and hepatic veins, trans-splenic PVR DIPS may serve as an effective bridge to liver transplantation by facilitating an end to end portal vein anastomosis.

Long-term results of variceal bleeding management in 302 patients with chronic extrahepatic portal vein obstruction.

Treatment modalities of variceal bleeding or rebleeding for extrahepatic portal vein obstruction (EHPVO) are limited, and their long-term results and prognostic factors are unclear. This study aimed at assessing the long-term results of EHPVO treated with current recommendations and investigating the influencing factors. Between 2009 and 2016, 302 consecutive patients with EHPVO were included. Watch-and-wait policy was applied for those with no (n=59) or small varices (n=55), nonselective beta-adrenergic blocker and nonselective beta-adrenergic blocker plus endotherapy were for primary (n=115) and secondary prophylaxis (n=87), transjugular intrahepatic portosystemic shunt (TIPS) or combination therapy was for those with recurrent bleeding (n=92). The median follow up was 58.8months. The 1-, 3-, and 5-year cumulative rates were 3.6%, 19.2%, 32.3% for small varices development and 4.5%, 30.9%, 53.4% for large varices development. The 1-, 3-, and 5-year cumulative rates were 11.1%, 20.9%, and 34.9% for first variceal bleeding and 16.0%, 26.9%, and 33.6% for variceal rebleeding. For those with recurrent variceal bleeding, only TIPS (n=37, technical success rate: 90.2%) was associated with a reduced risk of variceal rebleeding (1-, 3-, and 5-year: 5.6%, 11.7%, and 21.9%). The 1-, 3-, and 5-year survival rates were 96.9%, 95.8%, and 91.9%. Prothrombotic factors and anticoagulation did not influence the risk of variceal bleeding, rebleeding, and survival. By applying the same variceal management as recommended for patients with liver cirrhosis, patients with non-cirrhotic EHPVO showed a similar development of varices and variceal bleeding. This is also true for the beneficial effect of TIPS to prevent rebleeding.

Thrombectomy and Thrombolysis by Catheter Through Right Gastroepiploic Vein of Acute Superior Mesenteric Venous Thrombosis with Peritoneal Signs

Thrombectomy and Thrombolysis by Catheter Through Right Gastroepiploic Vein of Acute Superior Mesenteric Venous Thrombosis with Peritoneal Signs

Portal Hypertensive Cholangiopathy, Which Masquerades as an Acute Portal Vein Thrombosis

Portal hypertensive cholangiopathy is a rare complication of extrahepatic portal vein obstruction and portal cavernoma. It is usually asymptomatic but may be associated with jaundice, biliary colic, and fever. Abdominal sonography and Doppler are ideal initial diagnostic modalities, followed by magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. These imaging modalities are important if an intervention is planned for the management of portal hypertension and for relieving biliary obstruction. If computed tomography (CT) is performed in isolation, it may be challenging to distinguish this entity from acute on chronic portal vein thrombosis. The diagnostic results should be interpreted cautiously. This case report of an abdomen CT performed on a patient with a history of portal vein thrombosis masqueraded as an acute on chronic portal vein thrombosis with cavernous collaterals. Doppler confirmed the diagnosis of portal hypertensive cholangiopathy. Correlation with clinical symptoms and Doppler may be necessary to distinguish portal hypertensive cholangiopathy from acute portal vein thrombus.

2337 Secondary Sclerosing Cholangitis and Nodular Regenerative Hyperplasia Caused by Hereditary Spherocytosis

INTRODUCTION: Hereditary spherocytosis is a hemolytic anemia that leads to premature cell destruction due to red cell membrane defect. It causes release of unconjugated bilirubin, and subsequent bilirubin gallstone formation occurs. Chronic recurrent choledocholithiasis can cause secondary sclerosing cholangitis (SSC). Hemolysis can lead to chronic vascular injury and portal vein thrombosis, resulting in Nodular Regenerative Hyperplasia (NRH). Here, we report a case of HS causing SSC with concurrent NRH. CASE DESCRIPTION/METHODS: A 37-year-old female with a history of hereditary spherocytosis with thrombocytosis presented to the office for abnormal liver function tests (LFTs). History of splenectomy and cholecystectomy in 2002. Patient was noted to have abnormal LFTs in 2009 (Figure 1). Her LFTs were routinely monitored until 2016 when she presented with epigastric pain, jaundice, and abnormal LFTs. Viral and autoimmune hepatitis serologies were negative. Iron studies were normal. Right upper quadrant (RUQ) ultrasound (US) showed mildly dilated intrahepatic ducts. Magnetic resonance cholangiopancreatography(MRCP) was performed which showed a questionable stricture in the proximal CBD and mild dilatation of the central intrahepatic duct. ERCP showed normal caliber CBD with multiple filling defects. A biliary sphincterotomy was performed and the stone was removed. ERCP performed in 2016 and 2018 revealed multiple stones in the biliary ductal system, which were removed and the CBD was dilated. Liver biopsy showed mild portal and lobular inflammation with mild portal fibrosis and changes suggestive of NRH. DISCUSSION: HS caused the patient's recurrent choledocholithiasis and contributed to SSC. Pathologically, SSC causes inflammation, obliterative fibrosis, stricture formation, and destruction of the biliary tree, which leads to biliary cirrhosis. Although often reversible, outcomes may not be as favorable if it is not recognized quickly, particularly in critical illness induced SSC. NRH is a liver disease characterized by many hyperplastic nodules without separation by fibrotic tissue. It is associated with any systemic disease that causes vascular injury, in this case our patient’s HS. Pathology in our case revealed only “mild” changes suggestive of NRH, so the patient hasn’t yet developed portal hypertension. There are no cases in literature that document HS as a factor contributing to either of these disease states. Early suspicion and liver biopsy are necessary for prompt diagnosis and improved outcomes.

1920 Superior Mesenteric Arteriovenous Fistula Mimicking Hepatic Cirrhosis With Spontaneous Bacterial Peritonitis

INTRODUCTION: Arteriovenous fistula of the superior mesenteric vasculature is a rare entity that was reported for this first time by Movitz and Finne in 1960. The majority of these cases are iatrogenic after surgical bowel resection. We present a 52-year-old male patient who had a spontaneous AFV of the superior mesenteric vasculature that presented as a chronic portal vein thrombosis. CASE DESCRIPTION/METHODS: A 52-year-old male patient with a past medical history significant for active hepatitis C infection. The patient presented to our institution complaining of progressive abdominal distention and chronic abdominal pain for 3 months. He had been diagnosed with cirrhosis two months prior to admission and was started on furosemide and spironolactone for ascites and underwent multiple therapeutic paracentesis. At presentation, the patient was found to have a normal platelet count and INR which were not consistent with cirrhosis calling the diagnosis into question. Further workup with ultrasound examination of the liver revealed portal vein thrombosis with extension to the proximal part of the splenic vein. This was followed by CT of the abdomen and pelvis with IV contrast which showed smooth liver surface, enlarged portal, superior and inferior mesenteric veins with clot seen in the left portal vein extending into the main portal vein (Figure 1).it also showed a fistula of the mid superior mesenteric artery with the superior mesenteric vein (Figure 2) which explained the prominence and the enlargement of the portal venous system. Upon diagnosis, the patient was transferred to a tertiary center for endovascular closure of the fistula. DISCUSSION: Arteriovenous fistula of the superior mesenteric vasculature is a rare entity. Most cases are iatrogenic in origin occurring after bowel resection. The most common presentation is gastrointestinal bleeding due to portal hypertension and resulting variceal changes. Treatment with endovascular with coil embolization is often preferred over surgical repair. In summary, we discuss an unusual presentation of idiopathic arteriovenous fistula of the superior mesenteric vasculature with portal vein thrombosis, abdominal pain and ascites mimicking hepatic cirrhosis with spontaneous bacterial peritonitis.

Band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis.

Portal hypertension commonly accompanies advanced liver disease and often gives rise to life-threatening complications, including haemorrhage from oesophageal and gastrointestinal varices. Variceal haemorrhage commonly occurs in children with chronic liver disease or portal vein obstruction. Prevention is therefore important. Following numerous randomised clinical trials demonstrating efficacy of non-selective beta-blockers and endoscopic variceal ligation in decreasing the incidence of variceal haemorrhage, primary prophylaxis of variceal haemorrhage in adults has become the established standard of care. Hence, band ligation and beta-blockers have been proposed to be used as primary prophylaxis of oesophageal variceal bleeding in children. To determine the benefits and harms of band ligation compared with any type of beta-blocker for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register (February 2019), CENTRAL (December 2018), PubMed (December 2018), Embase Ovid (December 2018), LILACS (Bireme; January 2019), and Science Citation Index Expanded (Web of Science; December 2018). We scrutinised the reference lists of the retrieved publications and performed a manual search from the main paediatric gastroenterology and hepatology conferences (NASPGHAN and ESPGHAN) abstract books from 2009 to 2018. We searched ClinicalTrials.gov for ongoing clinical trials. There were no language or document type restrictions. We planned to include randomised clinical trials irrespective of blinding, language, or publication status for assessment of benefits and harms. We planned to also include quasi-randomised and other observational studies retrieved with the searches for randomised clinical trials for report of harm. We planned to summarise data from randomised clinical trials using standard Cochrane methodologies. We found no randomised clinical trials assessing band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Randomised clinical trials assessing the benefits or harms of band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis are lacking. There is a need for well-designed, adequately powered randomised clinical trials to assess the benefits and harms of band ligation versus beta-blockers for primary prophylaxis of oesophageal variceal bleeding in children with chronic liver disease or portal vein thrombosis. Those randomised clinical trials should include patient-relevant clinical outcomes such as mortality, failure to control bleeding, and adverse events.

Prevalence and impact of sarcopenia in non-cirrhotic portal hypertension.

Little is known on nutritional parameters in patients with chronic portal vein thrombosis (PVT) and idiopathic non-cirrhotic portal hypertension (INCPH). The study aims to assess the prevalence and the clinical impact of sarcopenia in patients with non-cirrhotic portal hypertension (NCPH). A control group of cirrhotic patients was also studied. Both groups were followed up to establish the relationship between sarcopenia and clinical outcomes. Sixty-seven patients with NCPH (51 PVT and 16 INCPH) were included in the study group and 104 patients with liver cirrhosis in the control group. The axial plane passing through the intersomatic disk between L3 and L4 was evaluated for the quantitative analysis of muscle mass and the skeletal muscle index (SMI) was calculated. The presence of sarcopenia was established according to SMI validated cut off. Sarcopenia was present in the 38% of patients with INCPH, 35% of patients with chronic PVT, 32% of patients with compensated cirrhosis and 54% of decompensated cirrhotics. During a mean follow-up of 51±62months, there was no difference in sarcopenic and non-sarcopenic patients with NCPH for incidence of ascites, hepatic encephalopathy, esophageal varices, variceal bleeding and death. However, the incidence of refractory variceal bleeding requiring TIPS placement was significantly higher in comparison with the non-sarcopenic ones (29% vs 7%, P=0.01 at log-rank test). In patients with NCPH sarcopenia is similar to that observed in cirrhotic patients. Moreover, the rate of refractory variceal bleeding was higher in sarcopenic patients suggesting a clinical negative impact of muscle depletion.

FRI-439-Factor V and VIII may predict the risk of recurrent splanchnic thrombosis in patients with non-cirrhotic chronic portal vein thrombosis of local and idiopathic etiology not receiving anticoagulation

FRI-439-Factor V and VIII may predict the risk of recurrent splanchnic thrombosis in patients with non-cirrhotic chronic portal vein thrombosis of local and idiopathic etiology not receiving anticoagulation

Recanalization of Chronic Extrahepatic Portal Vein Obstruction in Pediatric Patients Using a Minilaparotomy Approach.

Extrahepatic portal vein obstruction (EHPVO) is the most frequent cause of portal hypertension in children. Some patients are not amenable to meso-Rex bypass and alternative surgeries do not restore physiologic flow. We aim to demonstrate the feasibility and safety of minilaparotomy for recanalization of chronic EHPVO. This 2013-2015 single-center, retrospective review included pediatric patients with chronic EHPVO who underwent minilaparotomy, mesenteric vein access, and attempted recanalization of the occluded portal vein. Outcomes included portal patency, resolution of variceal bleeding, size and number of varices, spleen size, and platelet count. There were 6 EHPVO patients. The median age was 9.9 years and median duration of EHPVO was 7 years (3-16 years). EHPVO etiologies were liver transplantation (50%), idiopathic (33%), and umbilical vein catheterization (17%). Four patients (67%) had successful portal vein recanalization and stenting. At last follow-up [median 3.1 years (2.2-4.3 years)] all successfully recanalized patients had patent portal vein stents and resolution of varices and variceal bleeding. The median reduction in spleen size was 26%, with improvement in platelet counts (50-310/μL). The 2 patients with an idiopathic etiology may have never had a main extrahepatic portal vein based on imaging, and both were unable to be recanalized. Recanalization and stenting of a prolonged occlusion of the portal vein via a minilaparotomy approach is feasible, safe, and may provide an alternative to shunt surgery or endoscopic therapy in selected patients.

Recanalization of Chronic Portal Vein Occlusion in Pediatric Liver Transplant Patients

PurposeTo evaluate technical and clinical success and report long-term outcomes of portal vein (PV) recanalization in pediatric orthotopic liver transplant (OLT) patients with chronic PV occlusion. Materials and MethodsThis is a retrospective review of 15 OLT patients (5 males) with chronic PV occlusion who underwent PV recanalization (33 procedures) between October 2011 and February 2018. Median age was 4.5 years (range, 1–16 years); median weight was 16.6 kg (range, 11.5–57.3 kg). Median time interval from OLT to first intervention was 3.25 years (range, 0.6–15.7 years). Clinical presentations included hypersplenism (n = 12), gastrointestinal bleeding (n = 9), and ascites (n = 3). One patient had incidental diagnosis of PV occlusion. Primary, primary-assisted, and secondary patency at 3, 6, 12, and 24 months were evaluated. ResultsTechnically successful PV recanalization and reduction of PV pressure gradient to ≤ 5 mm Hg was performed in 13/15 patients (87%). Ten of 15 (67%) patients had successful recanalization with the first attempt. Clinical success, defined as improvement in signs and symptoms of portal hypertension, was achieved in 12/13 (92%) patients. Five of 33 (15%) major complications (Society of Interventional Radiology class C), including perisplenic hematoma (n = 2), hemoperitoneum (n = 2), and hepatic artery pseudo aneurysm (n = 1), were managed with pain medication and blood product replacement. Median follow-up was 22 months (range, 1–77 months). Median primary patency was 5 months. Primary patency at 3, 6, 12, and 24 months was 53.8%, 46.2%, 38.5%, and 30.8%, respectively. Primary-assisted patency was 84.6%, 76.9%, 53.8%, and 46.2%, respectively. Secondary patency was 92.3%, 84.6%, 53.8%, and 46.2%, respectively. ConclusionsPV recanalization is a safe and effective minimally invasive option in the management of chronic PV occlusion after pediatric OLT.

Acute Variceal Bleeding Causes Significant Morbidity.

The need for primary prophylaxis of variceal bleeding in children is unclear due to insufficient evidence of the efficacy of prophylactic therapy and the mortality and morbidity associated with the first bleeding event. Previous studies have provided estimates of mortality. We aimed to investigate the morbidity associated with acute variceal bleeding (AVB) in children and to identify contributing factors. We retrospectively reviewed children with chronic liver disease or portal vein thrombosis admitted with acute upper gastrointestinal bleeding between 2000 and 2015. Seventy AVB episodes in 57 children (median age 6 years, 52% girls) were included, 58% with cirrhosis and 30% portal vein thrombosis. Approximately 67% were the patient's first bleed. Post-AVB morbidity was present in 57% of all episodes and in 64% of first bleeds and included: ascites (34%), infection (30%), respiratory complications (24%), intensive care unit admission (20%), rebleed (11%), encephalopathy (7%), acute kidney injury (6%), and failure to control bleed (4%). Two patients died (4% of first bleeds, 8% of cirrhotics' first bleeds) within 6 weeks of bleeding. Median length of stay was 7 days. Overall morbidity was associated with total bilirubin (P = 0.001). Ascites after AVB was associated with pediatric end-stage liver disease (P = 0.0007), total bilirubin (P = 0.001), and cirrhosis (P = 0.006). Median length of stay was longer in patients with morbidities (18 vs 4 days, P < 0.0001). Children with AVB suffer significant morbidity but have a low risk of death. Morbidity should therefore be considered in future studies measuring the risks and benefits of primary prophylaxis of first AVB in children.

Long-term outcomes of transmesenteric portal vein recanalization for the treatment of chronic portal vein thrombosis after pediatric liver transplantation.

Portal vein thrombosis (PVT) may occur at any time following liver transplantation. We describe our experience with portal vein recanalization in cases of thrombosis after liver transplantation. Twenty-eight children (5%) out of 566 liver transplant recipients underwent portal vein recanalization using a transmesenteric approach. All children received left hepatic segments, developed PVT, and had symptoms or signs of portal hypertension. Portal vein recanalization was performed via the transmesenteric route in all cases. Twenty-two (78.6%) patients underwent successful recanalization and stent placement. They received oral anticoagulants after the procedure, and clinical symptoms subsided. Symptoms recurred due to portal vein restenosis/thrombosis in seven patients. On an intention-to-treat basis, the success rate of the proposed treatment was 60.7%. Only 17 out of 28 children with posttransplant chronic PVT retained stent patency (primary+assisted) at the end of the study period. In cases of portal vein obstruction, the transmesenteric approach via minilaparotomy is technically feasible with good clinical and hemodynamic results. It is an alternative procedure to reestablish the portal flow to the liver graft that can be performed in selected cases and a therapeutic addition to other treatment strategies currently used to treat chronic PVT.