Extrahepatic Portal Vein Research Articles

The Choice of Surgical Management in Patients with Portal Hypertension

Aim. To improve the results of surgical treatment of patients with syndrome of portal hypertension. Material and Methods. One hundred and sixty nine patients with syndrome of portal hypertension who underwent elective surgery were examined. 100 patients had liver cirrhosis and 69 patients – extrahepatic portal vein obstruction. Portocaval shunting was scheduled for all patients. Selective portocaval shunting was preferred in cirrhotic patients; maximal shunting using “side to side” or “H-type” mesentericocaval or splenorenal anastomoses was chosen for those who had extrahepatic portal vein obstruction. Results. Selective portocaval shunting was performed in 26 of 100 patients with liver cirrhosis. Seventy six patients underwent gastrotomy with direct oesophagogastric varices ligation. In case of extrahepatic portal vein obstruction portocaval shunting was applied in 69 patients and direct oesophagogastric varices ligation – in 26 patients. Shunt surgery was rejected due to high activity of the pathological process in liver, presence of hepatic encephalopathy, heart failure; and also according to ultrasonography and computed tomography of portal system. Satisfactory short-term results were obtained in 97% of patients. Overall mortality was 0,8%. Conclusion. Phased preoperative examination protocol should be applied for the optimization of surgical management in patients with portal hypertension: patients’ selection based on clinical and laboratory data, assessment of central hemodynamics and neurological status. It is necessary to determine hemodynamic and topographic features using ultrasonography and 3D computed tomography of portal and caval systems to forecast the ability to perform different types of portocaval shunting; to define type and extent of surgical intervention depending on intraoperative data

Extrahepatic Portal Hypertension and Portal Vein Thrombosis (Review)

Extrahepatic portal vein thrombosis is the second most frequent cause of portal hypertension after liver cirrhosis. Risk factors of the thrombosis are divided into systemic: hereditary and acquired thrombophilia (60–70%); and local: abdominal inflammation, trauma, surgery, etc. (20–40%). Acute thrombosis episode is often olygo- or asymptomatic and remains undiagnosed until the development of portal hypertension and its complications: gastroesophageal variceal bleeding, portal biliopathy, etc. Early onset of anticoagulation is recommended and should be prescribed at least for 3 months. In patients with portal cavernoma a long-term (even lifelong) anticoagulation is directed to prevent recurrence and extension of thrombosis, especially in case of thrombophilia. Portocaval shunting is the most radical way to correct portal hypertension and to deliver a patient from variceal bleedings and other complications of the disease. Hepatic encephalopathy is a rare condition and generally subclinical. Endoscopic band ligation and sclerotherapy are effective in acute variceal bleeding, primary and secondary prophylaxis of bleeding and should be supplied by non-selective β-adrenoblockers administration. Patients with extrahepatic portal vein thrombosis are well tolerated to variceal bleeding episodes and have high rates of survival during long-term follow-up due to normal liver function. Mortality in these patients is low (5-year mortality is less than 10%) and generally is associated with comorbidities rather complications of portal hypertension

A Computational Analysis of Blood Flow through Portal Vein under Normal and Extrahepatic Obstructions

Portal hypertension is an alarming disease globally whereas Extrahepatic portal vein obstruction (EHPVO) is an important cause of portal hypertension among children in most of the Asian countries. The frequent occurrence of this disease is due to the formation of thrombosis in the portal vein. To acquire insights into the behaviour of hemodynamics, EHPVO case is studied by the technique of computational fluid dynamics (CFD) by considering partial block formation with different sizes inside the main portal vein. On the basis of block sizes, three cases namely normal portal vein (without block in the portal vein) case, 40% block in the portal vein case and 85% block in the portal vein case are simulated by assuming a 2D steady, laminar and fully developed flow with no-slip wall condition using AnsysTM Fluent. Our study reveals that the flow distribution between the left and right branches of the portal vein is significantly influenced by the formation of the block in the main portal vein. It is concluded that Extrahepatic portal vein obstruction leads to the possibility of Hypertrophy and Atrophy complex to the liver and could heighten the risk of liver diseases. Our numerical examination will play a great role to the Liver Specialist for understanding the flow behavior with obstructed flow path as well as the diagnosis of associated liver disease and their treatments.Journal of Bangladesh Academy of Sciences, Vol. 41, No. 2, 183-199, 2017

A case of repeated bleeding of ectopic varices associated with secondary extrahepatic portal vein obstruction treated with a surgical portosystemic shunt

A case of repeated bleeding of ectopic varices associated with secondary extrahepatic portal vein obstruction treated with a surgical portosystemic shunt

Anaesthetic Management in a Case of Wilson's Disease in Pregnancy

Wilson’s disease (WD) is an inherited autosomal recessive disease results due to deficiency of ceruloplasmin leading to copper deposition mainly liver, brain and cornea. This accumulation can cause liver cirrhosis, ataxia and kayser Fleischer ring (KF) in cornea. I present a case of 21-year-old primigravida diagnosed WD 3 years back on treatment with Zinc was referred to our hospital for elective caesarean section (CS). She had thrombocytopenia, extrahepatic portal vein obstruction with hypersplenism and mild ascites. She has undergone CS under general anaesthesia (GA) with successful outcome. The anaesthetic management in presence of hepatic dysfunction and thrombocytopenia was challenging and there are only few case reports on WD patients undergoing CS under GA.

Evaluation of Splenic Stiffness in Patients of Extrahepatic Portal Vein Obstruction Using 2D Shear Wave Elastography: Comparison with Intra-Operative Portal Pressure

To compare splenic stiffness (SS) with intra-operative portal pressures (PPs) in patients of extrahepatic portal vein obstruction (EHPVO). Twenty-one patients (14 males; 7 females) of mean age 20.4 years with clinical and sonographic diagnosis of EHPVO were included in this approved prospective study. Endoscopy for esophageal varices (EV) was done in all patients followed by ultrasonographic 2D shear wave elastography (SWE) of spleen. Three values were taken at different areas of spleen avoiding major vessels and mean was calculated. Intra-operative PP was measured from an omental vein during proximal spleno-renal shunt surgery. The PP was compared and correlated with SS along with other parameters. The mean SS was 46.04±8.0kPa and the mean PP was 33.29±4.1mmHg. There was no significant correlation between PP and SS (P=0.61) and between grades of EV and SS (P=0.38). Significant correlation was seen between grades of EV and PP (0.04). SS also did not show significant correlation with splenic size or duration of disease. SS measured by 2D SWE did not correlate with PP and thus may not help in predicting gastrointestinal bleed in patients of EHPVO.

Metabolic profile of children with extrahepatic portal vein obstruction undergoing meso-Rex bypass

BackgroundExtrahepatic portal vein obstruction (EHPVO) in children is often associated with growth restriction, which improves after the restoration of portal venous flow with a meso-Rex bypass, but the physiologic mechanism is unknown. The purpose of this study was to investigate the mechanism of growth delay in children with EHPVO by detailing the metabolic and nutritional profile before and after meso-Rex bypass. MethodsTwenty consecutive children with EHPVO were prospectively studied before and 1 year after meso-Rex bypass. Caloric balance was determined by investigating caloric intake via a calorie count, total energy expenditure via a doubly labeled water isotope assay and stool caloric loss by bomb calorimetry. Laboratory markers of nutrition and growth hormone resistance were tested. ResultsFifteen of the 20 children underwent successful meso-Rex bypass at a median age of 4.3 years. Prealbumin level was abnormally low (14.6 ± 3.0 mg/dL) at surgery but improved (17.0 ± 4.3 mg/dL) 1 year later (P = 0.026). Mean insulin-like growth factor 1 (IGF-1) level at baseline was 1.57 standard deviations below normal. IGF-1 levels increased from 88.3 ± 38.9 to 117.3 ± 54.5 ng/mL in the year after surgery (P = 0.047). Caloric intake divided by basal metabolic rate (1.90 ± 0.61), total energy expenditure (97.2 ± 15.0% of expected), and stool caloric losses (3.7 ± 1.8% of caloric intake) were all normal at baseline. ConclusionsChildren with EHPVO suffer from malnutrition and growth hormone resistance, which may explain their well-established finding of growth restriction. Prealbumin and IGF-1 levels improve after a successful meso-Rex bypass.

Simultaneous Extrahepatic Portal and Iliac Veins Thrombosis After Abdominal Surgery - A Case Report and Review of Literature

Abstract Introduction: Extrahepatic portal vein thrombosis (EPVT) is the most frequent cause that leads to portal hypertension in non-cirrhotic patients. This condition is related to systemic and local risk factors (such as inflammatory lesions, injuries to portal venous system by surgery, vascular procedures). Case presentation: A case of extended extrahepatic portal vein thrombosis and simultaneous thrombosis of left common iliac vein and inferior vena cava, appeared after abdominal surgery in a hypertensive, diabetic, 50 y.o. man is presented. An acute episode of abdominal pain was interpreted as an emergency and a surgical (initially laparoscopic and then open) procedure was planned in order to perform an appendectomy. Discharge diagnosis was hemoperitoneum secondary to iatrogenic rupture of sigmoid mesocolon provoked by trocar manipulation. Repeated imaging studies performed later revealed the thrombosis of portal vein with extension into right portal branch associated with superior mesenteric thrombosis and free-floating thrombus into left common iliac vein extended towards inferior vena cava. Surgical manoeuvres are considered as triggers of these thrombotic events. After 4 weeks of parenteral anticoagulation a partial recanalization of thrombi was identified, without bleedings. Conclusions: Acute EPVT needs a carefully management. Case is linked to abdominal surgery and requires prolonged anticoagulation related to simultaneous portal and iliac vein thrombosis. Associated conditions (hypertension and diabetes mellitus) must have an appropriate approach. After our knowledge this is the first case published in literature.

Pediatric Transjugular Intrahepatic Portosystemic Shunts

AbstractTransjugular intrahepatic portosystemic shunt (TIPS) creation offers potentially life-saving portal decompression regardless of patient age or size, but has been underutilized in pediatric patients to date. Experience has therefore been limited, and the full clinical benefit in this population is yet to be realized. Those wishing to embark on pediatric TIPSs would benefit not only from significant experience in adult TIPSs, but also from an awareness of challenges posed by the unique pediatric population. We herein review the etiologies and manifestations of portal hypertension more common in children, highlight some of the technical nuances of creating TIPSs in smaller anatomy, and summarize the existing literature on the topic. As extrahepatic portal vein occlusion (EHPVO) occurs with greater frequency in pediatric patients, special attention is paid to this condition and its associated challenges.

Clinical value of preventative surgical resection in the treatment of extra-hepatic portal vein aneurysm

Objective To investigate the clinical value of preventative surgical resection in the treatment of extra-hepatic portal vein aneurysm. Methods The method of retrospective descriptive study was conducted. The clinical data of 1 patient with extra-hepatic portal vein aneurysm who was admitted to the Guangzhou General Hospital of Guangzhou Military Command of PLA in May 2014 were collected. The patient suffered from dull pain in his epigastrium, with no obvious causative factors. The patient recieved abdominal B-ultrasound, CT scans abdominal and superior mesenteric artery, angiographies after admission in May 2014, and was diagnosed as with extra-hepatic portal vein aneurysm. The preventative surgical resection was selected according to the patient′s individual health status. Extra-hepatic portal vein aneurysm resection was conducted under the guidance of intraoperative ultrasonography after abdominal exploration. A double-cavity drainage tube was placed in the venturis hole when portal vein aneurysm was dissected and removed. Then the abdomen was closed. The patient received regular perioperative management. The operation time, volume of intraoperative blood loss, time for diet recovery, time of drainage tube removal, postoperative complications, results of laboratory tests, results of imaging findings, healing of incision, time of postoperative discharge from hospital, results of pathologic examination and follow-up were observed. Follow-up using outpatient examination or telephone interview was performed to detect the inspect status of portal vein and inspect recurrence of portal vein aneurysm by abdominal B-ultrasound and CT examinations till December 2014. Results The patient underwent successful extra-hepatic portal vein aneurysm resection. The operation time was 85 minutes and the volume of intraoperative blood loss was around 150 mL. The patient gradually returned to normal diet and self-care ability at postoperative day 3, and the abdominal drainage tube was removed at postoperative day 3. No postoperative complications including hemorrhage, portal vein thrombosis and dysfunction of liver were detected after operation. There were normal levels of blood routine and blood biochemical test. Postoperative B-ultrasound examination showed smooth blood flow in portal vein and the abdominal blood vessel CT angiography presented clear image of the portal vein, with smooth blood flow and normal lumen. The surgical incision recovered very well and the patient was discharged from hospital at postoperative day 9. Results of postopeartive pathologic examination showed that there were cystic-like tissues with simple squamous epithelium cells on the interior, the wall of which consisted of smooth muscle was in accordance of the structure of vein. The patient didn′t receive any other special treatment or take medicines postoperatively. The patient had good physical health and can take care of himself during six-month follow-up, without narrow portal vein, recurrence of portal vein aneurysm and other complications. Conclusion Preventative surgical resection is safe and feasible for extra-hepatic portal vein aneurysm, with a good efficacy. Key words: Portal vein aneurysm, extra-hepatic; Surgical procedures, operative; Preventability; Portal vein aneurysm resection

Portal-to-right portal vein bypass for extrahepatic portal vein obstruction

ObjectiveRex shunt (mesenteric-to-left portal vein bypass) is considered a more physiologically rational treatment for EHPVO than other portosystemic systemic shunts in children. However, about 13.6% of children with EHPVO do not have usable left portal veins and up to 28.1%. Rex operations in children are not successful. Hence, a Rex shunt in these children was impossible. This study reports a novel approach by portal-to-right portal vein bypass for treatment of children with failed Rex shunts. Material and methodsEight children (age 6.1years, range 3.5–8.9years) who underwent Rex shunts developed recurrent gastrointestinal bleeding and hypersplenism 13months (11–30months) postoperatively. After ultrasound confirmation of blocked shunt, they underwent exploration. Three patients were found to have right portal vein agenesis. Five patients (62.5%) were found to have the patent right portal vein, with the diameter of 3–6mm. Four patients underwent bypass between the main portal vein in the hepatoduodenal ligament and the right portal vein by interposing an inferior mesenteric vein autograft, whereas the remaining patient underwent a bypass using ileal mesenteric vein autograft. ResultsThe operations took 2.3h (1.9–3.5h). The estimated blood loss was 50ml (30–80ml), with no complication. The portal venous pressure dropped from 34.6cmH2O (28–45 cmH2O) before the bypass to 19.6cmH2O (14–24cmH2O) after the bypass. The 5 patients were followed up for 10.2months (4–17months) and the post-operative ultrasound and CT angiography confirmed the patency of all the grafts and disappearance of the portal venous cavernova in all five patients. ConclusionThe portal-to-right portal vein bypass technique is feasible and safe for treatment of children with EHPVO who have had failed Rex shunts. Our preliminary result indicates that this technique extends the success of Rex shunt from left portal vein to right portal vein and open a new indication of physiological shunt for some of the children who not only have had failed Rex shunts or but also are not suitable for the Rex shunts. Type of studyTreatment study. Level of evidenceLevel IV.

A Rare Case of Chronic Extra-Hepatic Portal Vein Obstruction (EHPVO) Presenting With Variceal Bleed

EHPVO is a vascular disorder of the liver defined by obstruction of the extra-hepatic portal vein with or without involvement of the intra-hepatic portal or splenic or superior mesenteric veins. Underlying hypercoagulable and prothrombotic states are commonly reported from the West. 21-year-old female with history of Acute lymphoblastic leukemia diagnosed 2002 requiring chemotherapy currently in remission, splenectomy 2009 presents with large volume hematemesis. Denied alcohol use, NSAID use, anticoagulation, drug use or herbal medications. Hemoglobin (Hb) 4.8, Liver function tests (LFTs) normal. EGD showed 3 columns of large esophageal varices with high-risk stigmata s/p 8 bands. Ultrasound (US) Doppler showed Heterogeneous liver echotexture otherwise normal. She presented 2 weeks later with abdominal Pain, fever and chills. Hb 4.1 (8.6 at discharge). Normal platelets, INR and LFTs. Peripheral smear unremarkable. Reticulocyte % 2.8. CT Abdomen showed occlusive thrombosis of the main portal vein extending into the right portal vein and SMV with collateral vasculature. EGD/Colonoscopy showed small Esophageal and rectal varices. Parvovirus, Ebstein bar virus, Cytomegalovirus, Bone marrow biopsy, Factor 5 Leiden, F2 202110G Variant, JAK2 V617F, ANA, Beta-2 glycoprotein and Cardiolipin negative. Hepatic venous pressure gradient (HVPG) was 6. Liver biopsy showed some enlarged and peripherally displaced portal vein branches, mild sinusoidal dilatation. No significant fibrosis, steatosis or iron accumulation. Patient was diagnosed with Idiopathic or splenectomy induced EHPVO. Chronic EHPVO presents commonly with repeated, well-tolerated bleeding episodes from esophageal varices. Gastric varices are present in about one-third of the patients with EHPVO. Moderate to massive splenomegaly is universal, and may be a presenting feature. HVPG is normal. EHPVO is diagnosed by imaging as Doppler US, CT or MRI, which demonstrate portal vein obstruction, presence of intraluminal thrombus in the portal vein and/or portal vein cavernoma. Liver biopsy is necessary in a patient with EHPVO if the liver functions are deranged. We need to pursue Investigations to evaluate for a Prothrombotic State. Upper endoscopy is warranted in these patients. For the control of acute variceal bleeding, endoscopic therapy with banding is effective. In a patient with established chronic EHPVO, anti-coagulants are recommended if there is a history of recurrent thrombotic episodes.

Recurrent Biliary Obstruction Secondary to Portal Vein Thrombosis With Cavernous Transformation and the Role of Cholangioscopy in Diagnosis: Case Report

Portal vein thrombosis with cavernous transformation is a rare cause of biliary obstruction. Portal biliopathy is a term that refers to biliary abnormalities associated with portal vein hypertension and is most commonly caused by extrahepatic portal vein thrombosis. Patients may be asymptomatic, but can also present with abdominal pain, jaundice, and fever. We describe a case of biliary obstruction secondary to portal vein thrombosis with cavernous transformation who presented with recurrent jaundice. A 61 year-old female with history of coronary artery disease presented with generalized weakness and jaundice of three days duration. She had associated nausea and vomiting. Physical examination was notable for scleral icterus. Initial work-up was revealed markedly elevated total bilirubin and alkaline phosphatase. Abdominal and MRCP showed extrahepatic and intrahepatic biliary ductal dilatation due to short segment stenosis of the distal common bile duct. CT scan of the abdomen with IV contrast demonstrated a large tortuous splenic vein as well as numerous dilated veins extending into the region of the porta hepatis. Bilirubin and alkaline phosphatase levels rapidly improved and the patient was discharged with a plan for follow-up MRCP. Two weeks later, she presented with recurrence of her jaundice. ERCP was performed which showed the common bile duct and intrahepatic ducts to be dilated, and a plastic stent was placed after which the total bilirubin and alkaline phosphatase levels decreased. She then developed cholangitis and underwent ERCP with cholangioscopy, which showed extrinsic compression at the mid bile duct likely from the portal venous cavernoma, establishing the diagnosis of portal biliopathy. A metal stent was placed. Subsequent Doppler ultrasound of the liver revealed multiple venous collaterals seen in the porta hepatis, consistent with cavernous transformation. The patient was started on warfarin with resolution of her symptoms and normalization tests. She has had no recurrence at 4 months of follow-up. Workup was negative for an underlying hypercoagulablity. Portal vein thrombosis with cavernous transformation may rarely be associated with the development of portal biliopathy. There should be high suspicion for this diagnosis in people who present with obstructive jaundice and have risk factors for thrombosis. Cholangioscopy may be helpful in making the diagnosis. Endoscopic biliary stenting may be uitilized for treatment.Figure: CT abdomen with contrast suggestive of portal vein thrombosis.Figure: MRCP showing a marked extrahepatic and intrahepatic ductal dilatation.Figure: Doppler ultrasound of the liver revealed multiple venous collaterals seen in the porta hepatis, consistent with cavernous transformation.

Portal Cavernoma Mimicking Pancreatic Malignancy

Portal Cavernoma Colangiopathy (PCC) is characterized by biliary tract abnormalities due to extrahepatic portal vein obstruction (EHPVO). EHPVO can also cause pre-hepatic portal hypertension. Portal cavernoma refers to the expanded network of collateral vessels, sometimes mimicking a neoplasm. We report a 65-year-old man with past medical history of portal hypertension, who was admitted due to jaundice and abdominal distension. Laboratory tests were impressive for alkaline phosphatase 674 U/L, total bilirubin 6.8 mg/dL and transaminases 60 U/L. Computed tomography (CT) of the abdomen and pelvis revealed biliary ductal dilatation and a hepatic lesion suggestive of hemangioma. Magnetic resonance cholangiopancreatography (MRCP) revealed intra and extra hepatic biliary dilatation, and an irregular narrowing of the common bile duct (CBD) at the level of the porta hepatis surrounded by an ill-defined soft tissue lesion. To better asses the stricture at the CBD and unclear mass at porta hepatis, endoscopic ultrasonography (EUS) was performed. A hypoechoic mass was seen at the pancreatic head region with associated distal CBD obstruction and extensive collateral circulation. Given biliary dilation seen on CT and EUS with CBD stricture, endoscopic retrograde cholangio-pancreatography (ERCP) was performed and demonstrated a localized biliary stricture with malignant appearance. Sphincterotomy was performed and a metal stent was placed into the CBD. Fine needle aspiration from the EUS and brush cytology from ERCP were negative for malignancy. A repeat EUS showed stable vascular lesion in the peripancreatic area. A liver biopsy showed mild nodular regenerative hyperplasia (NRH) with minimal fibrosis. These cholangiographic findings were determined to be due to chronic PVT with cavernous transformation. Our paper illustrates a patient with non-cirrhotic portal hypertension associated with PVT and cavernous transformation, known as PCC. The hypoechoic mass at the peripancreatic region and biliary stricture were radiologic features of PCC. Symptomatic patients with PCC are at high-risk for sustained biliary obstruction complications, including secondary biliary cirrhosis. An association between PCC and NRH has not been previously described and likely contributed to the portal hypertension in this case. One must be aware of PCC as an important differential diagnosis with malignancy and a cause of pre-cirrhotic portal hypertension.Figure

Portal vein aneurysm in thalassaemia

Arterial aneurysms are more common than visceral venous aneurysms. Portal vein aneurysms being the most common type of visceral venous aneurysms. Here, we present an 18-year-old young woman with thalassaemia...

Research advances in diagnosis and treatment of duodenal varices

Duodenal varices are an uncommon disease which occurs along the entire gastrointestinal tract outside the esophagus or stomach. Among ectopic varices, duodenal varices are the most common one. Cirrhosis, hepatic portal hypertension, extrahepatic portal vein occlusion and vasculitis contribute to the pathogenesis of ectopic variceal formation. The unusual pathophysiology, location, diversity and uncertain curative effect of DV are associated with its low detection rate and high mortality of patients. Therefore, the diagnosis and treatment of DV is one of the tough problems in clinical practice. This article reviews the current research advances in pathophysiology, diagnosis and treatment of duodenal varices to provide reference for clinical practice. Key words: Varicose veins; Duodenum; Diagnosis

Evaluation of growth and nutritional status in children and adolescents with extrahepatic portal vein obstruction and portal hypertension

ABSTRACT Objective: This study aims to evaluate dietary intake, nutritional status, and growth rate in children and adolescents with extrahepatic portal vein obstruction and portal hypertension. Methods: Outpatients aged 1-18 years, diagnosed with extrahepatic portal vein obstruction and portal hypertension, who had no associated diseases, and who had not been subjected to a venous shunt were included in this study. Two evaluations were carried out in this study: an initial (evaluation 1) and a final evaluation (evaluation 2), with a three-month minimum interval between them. In each evaluation, dietary intake was analyzed comparing the results with recommended energy intake using the Harris & Benedict equation and participants’ anthropometric data, such as weight, height, mid-arm muscle circumference, weight-for-age, height-for-age, and body mass index-for-age, based on the World Health Organization 2006 standards. Results: A total of 22 patients participated in this study. There was a significant improvement in weight, height, body mass index, and mid-arm muscle circumference measurements (p<0.001; p<0.001; p<0.017; p=0.0018 respectively) and in the relationship between dietary intake and energy recommended energy intake, according to the Harris & Benedict equation (p=0.0001) from the first and second evaluation. Conclusion: Extrahepatic portal vein obstruction and portal hypertension were not shown to be factors predisposing to malnourishment.

Extrahepatic Portal Venous Obstruction: What Should be the Mainstay of Treatment?

The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system. While endotherapy obliterates natural porto-systemic collaterals in the gastroesophageal region, persistently raised portal pressures manifest as an increase in secondary isolated gastric varices, ectopic varices, portal hypertensive vasculopathy, issues related to massive splenomegaly, portal biliopathy, growth retardation and hence impaired quality of life (QOL). An ideal management strategy should address both bleed and non-bleed consequences of EHPVO and translate into a near normal QOL. Further, MRB has opened up new dimensions to the management philosophy of EHPVO. This review article critically evaluates the role of surgery and endotherapy based on available literature and authors' own experience.Surgery and endotherapy are complementary. However, with increasing duration of follow-up post EEEV, it is evident that there is resurgence in the role of surgery (PSS/MRB) as a single one time definitive procedure for alleviating all bleed and delayed non bleed sequelae of EHPVO.Surgery for EHPVO (PSS/MRB) should not be allowed to become a dying art and future generations of surgeons should continue to receive training in this specialized area of surgery.

Imaging in portal cavernoma cholangiopathy: current understanding and future perspectives

Portal cavernoma cholangiopathy (PCC) as the name suggests refers to the spectrum of biliary changes that occur secondary to cavernomatous transformation of the portal vein in patients with extrahepatic portal vein obstruction (EHPVO) (1-5). PCC has previously been widely referred to as ‘portal biliopathy’ in medical literature but the current terminology is perhaps more readily comprehensible.

Portal vein thrombosis as a rare cause of abdominal pain: When to consider?

Extrahepatic portal vein thrombosis (PVT) is a rare condition that is characterized by the presence of thrombus within any segment of the portal vein, including the right and left intrahepatic branches. It may also extend to the splenic or superior mesenteric veins. Portal vein thrombosis may be related to cirrhosis or liver malignancy as well as to local inflammatory conditions in the abdomen and genetic or acquired thrombophilic diseases. Currently, PVT is being increasingly diagnosed due to advances in modern imaging techniques. The clinical presentation has a wide range, from an asymptomatic lesion to a potentially life-threatening situation. In this study, we present three patients with PVT. The diagnosis was made by radiologic and clinical findings. In the first patient, genetic testing revealed factor V Leiden mutation as the cause of PVT. The second patient was diagnosed with lupus anticoagulant syndrome as the cause of PVT. Portal vein thrombosis was associated with intra abdominal infection due to anastomotic leakage in the third patient. Two patients were successfully treated with anticoagulant therapy. This report emphasizes that even though PVT is a rare cause of abdominal pain, timely diagnosis and appropriate management is vital due to its lethal complications such as mesenteric ischemia and mesenteric infarct.