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Portal vein thrombosis (PVT) is one of the common complications of cirrhosis. The incidence of PVT correlates with liver disease severity—higher incidence in patients with Child–Turcotte–Pugh (CTP) C, large spontaneous portosystemic shunts, hepatofugal portal flow, and in the presence of hepatocellular carcinoma. PVT may worsen ascites, increase the risk and poor control of variceal bleeding. The occurrence of PVT may increase morbidity and lower survival after a liver transplant. Using statins prevents the occurrence of PVT, whereas beta-blockers may aggravate its occurrence. Cross-sectional imaging is mandatory for the precise diagnosis and classification of PVT. Symptomatic, occlusive PVT and candidacy for liver transplantation are the main indications for anticoagulation. Vitamin K antagonists, low-molecular-weight heparin, and newer anticoagulants are effective and safe in cirrhosis. Direct-acting oral anticoagulants are agents of choice in early cirrhosis (CTP A, B). The duration of anticoagulant therapy, predictors of response, and management of complications of cirrhosis while on therapy require in-depth knowledge and individualized treatment. Transjugular intrahepatic porto-systemic shunt can be considered in nonresponsive cases or when anticoagulants are contraindicated. This manuscript reviews the latest updated knowledge about managing PVT in cirrhosis. [ABSTRACT FROM AUTHOR]

Liver diseases complicate approximately 3% of pregnancies worldwide, and require specialized care to ensure optimal pregnancy outcomes. Extra-hepatic portal venous obstruction (EHPVO), even though asymptomatic in a majority of the cases, has a higher risk of variceal bleeding in pregnancy and adverse pregnancy outcomes. In this series, we describe five cases of EHPVO presenting during pregnancy at our tertiary care hospital, their antenatal management, and delivery outcomes. In our case series on EHPVO in pregnancy, a majority of the women were diagnosed during childhood and adequately asymptomatic before pregnancy. Two women underwent Endoscopic band ligation for esophageal varices and one woman underwent splenectomy before pregnancy for disease control. One woman was Anti - nuclear antibody (ANA) positive and another was Anti-phospholipid antibody (APLA) positive, while the other three did not have pre-existing thrombotic tendencies. One patient required Endoscopic band ligation during pregnancy. Pregnancy complicated by hypertension was noted in two of the five women (40%). Four of the five women (80%) had thrombocytopenia requiring blood products during delivery. Two women underwent preterm vaginal delivery, and one woman underwent preterm Caesarean section because of placental abruption. Three women had a post - partum hemorrhage, which was effectively managed with uterotonics and blood products. All five women and their neonates were discharged in good health. Multi - disciplinary approach with standardized antenatal care can give positive pregnancy outcomes in women with EHPVO. [ABSTRACT FROM AUTHOR]

Abstract Pregnancy in non-cirrhotic portal hypertension (NCPH) is an uncommon condition. Its management is challenging both to the obstetricians as well as to the gastroenterologists due to the lack of more extensive studies and standard clinical practice guidelines. These patients are at increased risk of portal hypertension (PTH) complications, especially variceal bleeding, and with an increased incidence of adverse maternal and fetal outcomes. Hence, a multidisciplinary approach is required for management of pregnancy in NCPH. This short review describes the different aspects of pregnancy with NCPH, emphasizing specific strategies for preventing and managing PTH from the preconceptional period to postpartum.

Portal vein thrombosis (PVT), particularly the presence of portal cavernoma, was traditionally considered a relative contraindication for transjugular intrahepatic portosystemic shunting (TIPS) due to the technical difficulties in accessing and maneuvering the portal vein and avoiding the high risk for bleeding periportal collaterals. However, the last decade has seen a surge in the number of studies—mostly case reports and small series of patients—demonstrating that TIPS is not only technically feasible in the vast majority of these patients but also provides effective and long-term control of symptoms associated with portal hypertension in cases refractory to the standard line of therapy. The present article aims to provide a concise but exhaustive overview of the role and the standard and technically difficult TIPS placement scenarios in patients with chronic non-malignant PVT and with and without underlying liver disease. The review is strategically punctuated by exemplary instances from the authors' experience. [ABSTRACT FROM AUTHOR]

A variety of complications and associated clinical presentations may be seen in patients with cirrhotic and non-cirrhotic portal hypertension. We present one such case of Upper GI hemorrhage from ectopic duodenal varices in a case of pre-hepatic portal hypertension due to Extrahepatic Portal Venous Obstruction (EHPVO). The case was managed successfully with endovascular Portal Vein Recanalization (PVR) and metallic stent deployment. With adequate technical success, improved symptoms, and laboratory parameters, the patient was discharged on long-term anticoagulation and interval follow-up. [ABSTRACT FROM AUTHOR]

Background: Women with Extra hepatic portal vein obstruction (EHPVO) are mostly young and belong to Asian countries. In the Indian subcontinent, 20–30% variceal bleeds are caused by EHPVO. Hence pregnancy is a concern in such patients. The objective of this study is to observe the maternal and neonatal outcomes in women with EHPVO. Materials and Method: Extra hepatic portal vein obstruction was studied retrospectively in 28 pregnancies in 20 women from Jan 2011 to July 2018 at a tertiary hospital in South India and the pregnancy outcomes were observed during this period. Institutional Review Board approval obtained. Results: The mean age of the women was 24.3 years and the mean age of diagnosis was 18.5 years. Splenomegaly, thrombocytopenia and anaemia were seen in 22 (78.5%), 17 (60.7%) and 8 (28.5%) of pregnancies, respectively. Rate of abortions and preterm deliveries were 2 (7.1%,n=28) and 10 (35.7%,n=28). There was one stillbirth (3.6%) in the study group. EHPVO was diagnosed in 25 (89.3%) women prenatally in our series. During pregnancy only one woman had variceal bleed, which was managed conservatively. Blood and blood product transfusion was required in 7(25%) of women and there was no maternal mortality. Conclusion: Pregnancies in EHPVO have good maternal and neonatal outcomes, provided they are taken care of by a multidisciplinary approach in a tertiary care centre.

Proximal splenorenal shunt (PSRS) is one of the most commonly performed portosystemic shunt (PSS) in extrahepatic portal venous obstruction (EHPVO) for portal decompression. Sometimes various anatomical and surgical factors related to the splenic vein and/or left renal vein may make the construction of a PSRS difficult or impossible. Unconventional shunts are required to tide over such conditions. From January 2008 to December 2018, 189 patients with EHPVO underwent PSS, of which, the 10 patients who underwent unconventional shunts form the study group of this paper. The ten unconventional shunts included 8 proximal splenoadrenal shunts, one collateral-renal shunt, and one inferior mesenteric vein to inferior vena cava (IMV-Caval) shunt. The mean percentage drop in omental pressure was 34.2% post-shunt with a mean anastomotic diameter of 13.7 ± 3.1 mm. Three patients experienced some form of postoperative complication. With a mean follow-up period of 32.3 months (maximum of 111 months) all patients had patent shunts on follow-up Doppler. None of the patients had variceal bleed, or features of biliopathy and hepatic encephalopathy in follow-up. Unconventional shunts can be used safely and effectively with good postoperative outcomes in EHPVO. [ABSTRACT FROM AUTHOR]

Extrahepatic portal vein obstruction (EHPVO) is characterized by cavernomatous transformation of the extrahepatic portal vein leading to portal hypertension and its sequelae. This article reviews the etiology, clinical features, and the management of its sequelae. Most common presentation being about of hematemesis or growth retardation in children and in later childhood/early adult life, patients commonly present with variceal bleed, growth retardation, and hypersplenism. Asymptomatic EHPVO patients can be followed up. Patients with variceal bleed need endoscopic management, and surgery is indicated in those who fail endoscopy or who are not amenable to endoscopic management. Patients with symptomatic hypersplenism can be treated with interventional radiological procedures. Symptomatic portal biliopathy can be managed with ERCP in the acute setting and a decompressive shunt procedure with or without a biliary diversion as an elective procedure. [ABSTRACT FROM AUTHOR]

Extrahepatic portal venous obstruction (EHPVO) usually presents with upper gastrointestinal bleed in the first decade of life. Symptomatic portal hypertensive biliopathy is seen in a minority of patients with EHPVO. With use of endoscopic intervention, biliary drainage is maintained in these patients. Various procedural complications have been linked while performing endoscopic retrograde cholangiography and stenting; however, these are managed conservatively. Here, we are highlighting a case of EHPVO with symptomatic portal biliopathy in which the patient bled from paracholedochal collateral after biliary stenting and was managed successfully with a multidisciplinary approach.

Purpose: To evaluate role of 3-T magnetic resonance portovenography (MRPV) in children with extra hepatic portal venous obstruction (EHPVO) and compare unenhanced and contrast-enhanced sequences. Methods: 20 Children with EHPVO underwent MRPV using unenhanced [T2, T1 pre-contrast, Balanced turbo field echo (BTFE)] and contrast-enhanced (CE) modified DIXON (mDIXON) sequences. The images were evaluated for the patency of abdominal veins, hepatic and splenic parenchyma by two radiologists for interobserver agreement. Results: BTFE and post-contrast mDIXON sequences performed best for evaluation of abdominal veins. Concordance between the BTFE and CE sequences was good to very good for both the radiologists for splenic vein (SV) evaluation. The concordance between the two sequences for evaluation of superior mesenteric vein and its confluence with the SV was less remarkable and varied from fair to good, while the interobserver agreement was very good to perfect. The interobserver agreement between the BTFE and CE sequence for the evaluation of left renal vein was very good, while that for IVC was perfect. The agreement between BTFE and CE sequence was perfect for the evaluation of right and middle hepatic veins, while the interobserver agreement was good to perfect. The interobserver agreement was poor for evaluation of hepatic parenchyma on BTFE sequence as compared to CE sequence, and moderate for splenic parenchyma. Conclusion: BTFE sequence is the single best unenhanced MR pulse sequence to detect all the vascular structures in children with EHPVO. CE-MRI is not superior to BTFE sequence and should be used at the discretion of the radiologist. [ABSTRACT FROM AUTHOR]

Background: Women with Extra hepatic portal vein obstruction (EHPVO) are mostly young and belong to Asian countries. In the Indian subcontinent, 20–30% variceal bleeds are caused by EHPVO. Hence pregnancy is a concern in such patients. The objective of this study is to observe the maternal and neonatal outcomes in women with EHPVO. Materials and Method: Extra hepatic portal vein obstruction was studied retrospectively in 28 pregnancies in 20 women from Jan 2011 to July 2018 at a tertiary hospital in South India and the pregnancy outcomes were observed during this period. Institutional Review Board approval obtained. Results: The mean age of the women was 24.3 years and the mean age of diagnosis was 18.5 years. Splenomegaly, thrombocytopenia and anaemia were seen in 22 (78.5%), 17 (60.7%) and 8 (28.5%) of pregnancies, respectively. Rate of abortions and preterm deliveries were 2 (7.1%,n=28) and 10 (35.7%,n=28). There was one stillbirth (3.6%) in the study group. EHPVO was diagnosed in 25 (89.3%) women prenatally in our series. During pregnancy only one woman had variceal bleed, which was managed conservatively. Blood and blood product transfusion was required in 7(25%) of women and there was no maternal mortality. Conclusion: Pregnancies in EHPVO have good maternal and neonatal outcomes, provided they are taken care of by a multidisciplinary approach in a tertiary care centre. [ABSTRACT FROM AUTHOR]

Aim of the study: To determine the correlation of the endoscopic findings with portal Doppler and ultrasound (USG) in children with suspected portal hypertension (PHT). Material and methods: Eighty children with extrahepatic portal vein obstruction (EHPVO) and chronic liver disease (CLD) were included in this retrospective study conducted over a period of 1 year. All patients underwent upper gastrointestinal (GI) endoscopy and Doppler. Results: The etiology was EHPVO in 30 (37.5%) patients, biliary atresia in 12 (15%), Budd-Chiari syndrome in 11 (13.7%), Wilson's disease in 10 (12.5%), idiopathic CLD in 8 (10%), autoimmune hepatitis in 4 (5%), glycogen storage disease (GSD) in 3 (3.8%), non-alcoholic liver disease (NAFLD) in 1 (1.3%) and systemic lupus erythematosus (SLE) in 1 (1.3%) patient. Fifty-three (66.25%) patients had esophageal varices on endoscopy, of whom 3 (3.8%) had associated gastric varices. Portal hypertensive gastropathy (PHG) was present in 30 (37.5%) patients, of whom 10 (12.5%) had severe PHG. Forty-one (51.3%) patients had PHT on Doppler (κ correlation 0.43). Kappa correlation was 0.43 in patients with biliary atresia, 0.31 in Budd-Chiari syndrome, 0.23 in idiopathic CLD, 0.21 in CLD, and 0.05 in Wilson's disease. All (100%) EHPVO patients and 39 (78%) CLD patients had PHT on USG. Endoscopic findings of PHT were seen in 24 (80%) EHPVO patients and 29 (58%) CLD patients. All patients with EHPVO had cavernous transformation of the portal vein on Doppler. For patients with CLD, the common Doppler findings were collaterals seen in 35 patients and reversal of flow in 12 patients. Conclusions: Doppler ultrasound followed by endoscopy should be used to diagnose PHT in children. In children with biliary atresia, Doppler ultrasound may miss changes of PHT. [ABSTRACT FROM AUTHOR]

In acute portal vein thrombosis (PVT), a six-month anticoagulation treatment achieves complete recanalization in only 35%–45% of patients, but the predictors of poor treatment responses are unclear. We examined treatment outcomes in PVT and aimed to identify predictors of incomplete recanalization and portal hypertensive complications. This retrospective study comprised patients diagnosed with PVT between 2006 and 2015. Key exclusion criteria were liver cirrhosis, malignancy, and age <18. The final cohort comprised 145 patients, of whom 132 (92%) were primarily treated with anticoagulation. The 5-year cumulative incidence of complete recanalization was 42% and of portal hypertensive complications, 31%. Independent predictors of insufficient recanalization were sub-acute or chronic thrombosis (hazard ratio (HR) 3.1, 95% CI 1.6–5.8), while acute pancreatitis was a protective factor (HR 0.3, 95% CI 0.2 − 0.7). Independent predictors of incident portal hypertensive complications were as cites at baseline (HR 3.3, 95% CI 1.7–6.7), sub-acute or chronic thrombosis (HR 2.9, 95% CI 1.6–5.3), extension of thrombosis to the splenic or mesenteric vein (HR 2.6, 95% CI 1.2–5.7), myeloproliferative disease (HR 3.0, 95% CI 1.4–6.5), and anemia (HR 2.1, 95% 1.1–3.9), while acute pancreatitis was a protective factor (HR 0.1, 95% CI 0.03–0.5). Etiology and age of thrombosis are associated with treatment responses in PVT. The presence of ascites at baseline, etiology, and extent of thrombosis, a non-acute thrombosis and anemia, are associated with the risk of portal hypertensive complications. Etiology and extent of thrombosis should be taken into account when determining the treatment (method) for PVT. [ABSTRACT FROM AUTHOR]

EHPVO in individual with solitary left kidney is rare occurance. Though there is no etiological association between EHPVO and solitary left kidney but the solitary left kidney decides the modality of treatment. Eighteen year lady referred to our institute with menorrhagia for 5 years and ultrasonography finding of splenomegaly and atretic right kidney. Investigations revealed Extra Hepatic Portal Vein Obstruction with multiple cavernoma formation with oesophagogastric varices with right renal agenesis. She successfully underwent splenectomy with devascularisation. Patient with EHPVO present mainly with recurrent episodes of variceal bleeding, splenomegaly and hypersplenism. Splenectomy and esophagogastric devascularisation is an effective modality of treatment for patient with EHPVO with solitary kidney.

Transjugular intrahepatic portosystemic shunting (TIPS) is an established strategy for the management of complications of portal hypertension. Endoprosthetic infection ("endotipsitis") is a rare but serious and difficult-to-treat complication of TIPS placement. Here we report the occurrence of an infected thrombus complicating TIPS placement in a patient with extra-hepatic portal vein obstruction, recurrent variceal bleeding and portal biliopathy accompanied by recurrent cholangitis. Infected thrombotic material within TIPS could be removed only by employing rotational thrombectomy. This procedure revealed the presence of a biliary fistula which carried pathogens in the systemic circulation. The multiple episodes of sepsis did no longer recur following exclusion of the biliary fistula. This case highlights the possibility to use rotational thrombectomy for the management of complex cases of TIPS dysfunction., Competing Interests: FV, FM, and FF have received lecture fees from Gore., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Institute of Radiology.)

Cavernomatous transformation of the portal vein, seen in extrahepatic portal venous obstruction (EHPVO), can cause impingement or ischemic insult on bile ducts manifesting as "portal cavernoma cholangiopathy" (PCC). Bile duct wall calcification in portal biliopathy is a rare occurrence and has not been reported in the literature to the best of our knowledge. We report a 59-year-old male, a known case of EHPVO, who had undergone laparoscopic cholecystectomy, splenectomy, and splenorenal shunt in the past. The patient had now presented to us in view of recurrent episodes of cholangitis for which a bilioenteric bypass was planned. Intraoperatively, dilated and densely thickened bile ducts with multiple pericholedochal collaterals were noted. Incision of common hepatic duct and left hepatic duct showed completely calcified ductal wall with no visible healthy mucosa. Calcifications were removed partially from the bile duct walls near choledochotomy site. With the anticipation of futile benefit from bilioenteric bypass, Roux-en-Y HJ was abandoned. Hepaticoduodenostomy was done to prevent bile leak from choledochotomy site. [ABSTRACT FROM AUTHOR]

Extrahepatic portal venous obstruction (EHPVO) usually presents with upper gastrointestinal bleed in the first decade of life. Symptomatic portal hypertensive biliopathy is seen in a minority of patients with EHPVO. With use of endoscopic intervention, biliary drainage is maintained in these patients. Various procedural complications have been linked while performing endoscopic retrograde cholangiography and stenting; however, these are managed conservatively. Here, we are highlighting a case of EHPVO with symptomatic portal biliopathy in which the patient bled from paracholedochal collateral after biliary stenting and was managed successfully with a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Introduction: Cardiac functional abnormalities are common in patients with cirrhosis of the liver. Nonetheless, the effect of portal hypertension and liver disorder on cardiac abnormalities is yet to be investigated. The current study evaluated the contribution of cirrhotic and non-cirrhotic portal hypertension as the potential cause of cardiac abnormalities., Methods: The present study was a cross-sectional observational study. After excluding known heart diseases, 128 patients with portal hypertension from different causes were enrolled in the study. Cardiac functional activity was assessed by electrocardiogram (ECG) and transthoracic echocardiography (TTE).  Results: This study included a total of 128 patients, out of which 24 had extrahepatic portal vein obstruction (EHPVO), four patients had Budd-Chiari syndrome and 100 had liver cirrhosis. Normal ventricular function was observed in patients with EHPVO and Budd-Chiari syndrome. Sixty-eight percent of cases had liver cirrhosis diastolic abnormalities. The mean QTc interval in patients with cirrhotic cardiomyopathy (CCM) was 0.49 ± 0.05 sec which was significantly increased when compared to patients without CCM with 0.432 ± 0.07 at p =0.0016. The Child Turcotte Pugh (CTP) score and MELD (Model for End-Stage Liver Disease) score in patients with CCM were significantly higher as compared to patients without CCM. All alcoholic cirrhotic and non-alcoholic cirrhotic patients had equal prevalence of diastolic dysfunction ( p -value >0.05)., Conclusion:  Patients with Child class C or a high MELD score are associated with a higher prevalence rate of CCM while normal cardiac function was observed among patients having portal hypertension due to extrahepatic causes. We recommend cardiac evaluation by echocardiography in all cirrhotic patients. Institution of specific medical therapy and early referral for liver transplantation should be considered to improve survival in patients with decompensated cirrhosis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Behera et al.)

Portosystemic shunt surgery is an established treatment option for preventing variceal rebleeding in patients with noncirrhotic portal hypertension (NCPH). The proximal splenorenal shunt is a widely performed procedure in these patients. In this study, the use of adrenal vein as an alternative conduit has been investigated. A retrospective analysis of patients with NCPH who underwent proximal splenoadrenal and splenorenal shunt between 2011 and 2015 was performed. Demographic findings, etiology of portal hypertension, clinical presentation, hematological parameters, liver function test, intraoperative findings, postoperative morbidity, and shunt patency were studied and compared between the two groups. All patients were followed up for a minimum of 12 months with Doppler study of the shunt to assess shunt patency and upper gastrointestinal endoscopy to assess regression of varices. During the study period, 97 patients with NCPH underwent shunt surgery (proximal splenoadrenal shunt, 8; proximal splenorenal shunt, 74; and interposition mesocaval shunt, 15). Anomalous anatomy of the left renal vein was the main indication (5/8 patients) for a splenoadrenal shunt. Median fall in portal pressure in patients who underwent splenoadrenal shunt was 11.5 mmHg (range, 2–14 mmHg). The median (range) operative time was 4.5 (3–6) hours and median (range) intraoperative blood loss was 160 (100–200) mL. During a median (range) follow-up of 32 (12–48) months, shunt thrombosis developed in one patient. Comparison of intraoperative parameters and postoperative outcomes showed no significant difference in median fall in portal pressure (p = 0.39), median operative time (p = 0.51), median blood loss (p = 0.80), Grade III/IV postoperative complications (p = 0.56), shunt thrombosis (p = 0.93), and varices regression rate (p = 0.72) between patients undergoing proximal splenorenal and splenoadrenal shunt. In conclusion, left adrenal vein is a suitable vascular conduit for porto-systemic shunt surgery. This is especially so if the performance of splenorenal shunt is precluded because of anatomic abnormality of the renal vein. [ABSTRACT FROM AUTHOR]

Background Gastric varices (GV) are found in patients with portal hypertension. Incidence of bleeding from GV is relatively low, but is severe, and associated with higher mortality. Aims and objectives To compare the types of GV in cirrhosis vs. extrahepatic portal venous obstruction (EHPVO) and the results of endoscopic N -butyl-2-cyanoacrylate (NBC, glue) injection. Methods Four hundred and fifty-four patients undergone glue injection of GV for primary prophylaxis or control of bleeding for first episode of bleeding from GV between August 2010 and August 2015 were analyzed. Results Of 454 patients, 64% ( n = 292) were cirrhotics and 36% ( n = 162) had EHPVO. Types of GV were GOV1 in 16.4% ( n = 48) of cirrhotics vs. 7.4% ( n = 12) of EHPVO, GOV2 in 76.7% ( n = 224) of cirrhotics vs. 53.1% ( n = 86) of EHPVO, Isolated gastric Varices (IGV1) in 39.5% ( n = 64) of patients with EHPVO vs. 6.8% ( n = 20) cirrhotics. The patients were treated with NBC injections. The mean volume of glue injected was 2.89 ± 1.59 ml over a median of 1 session (range: 1–7). The total volume of glue required was lower in cirrhotics (2.44 ± 1.17 ml vs. 3.69 ± 1.91 ml, P < 0.05) than in EHPVO patients. One hundred and seventeen (40.1%) of cirrhotics required >1 sessions of glue injection as compared to 102 (63%) of EHPVO patients. Over mean follow up of 14.7 ± 6.46 months, rebleeding (10% vs. 13%) was similar in patients with cirrhosis and EHPVO and mortality (15.4% vs. 2.5%) was higher in cirrhotics than EHPVO. Conclusion In patients with bleeding from GV, GOV2 are more common in cirrhotics and IGV1 in patients with EHPVO. Patients with EHPVO required higher total volume of glue and more glue sessions for GV obturation. [ABSTRACT FROM AUTHOR]

Splenic artery aneurysm is one of the most common visceral aneurysms. Patients are usually asymptomatic. Splenic artery aneurysm if untreated has the potential for rupture and is therefore life-threatening. Its association with extrahepatic portal vein obstruction is rare. A 25-year female was incidentally diagnosed with a splenic artery aneurysm with extrahepatic portal vein obstruction with splenomegaly 8 years back during the 5th month of her second pregnancy. No intervention was done back then. Recently, she presented to the surgical gastroenterology outpatient department with an increasing abdominal mass. On examination, the patient was pale and splenomegaly was present. Hematological reports were suggestive of hypersplenism. The patient underwent splenectomy and aneurysmal resection with a proximal splenorenal shunt as the best course of treatment. Due to the rarity of the disease, the management is still challenging and needs further study. Diagnosis can be made clinically with support from imaging modalities. Surgical treatment has a good outcome in such patients. Even with the availability of less invasive procedures such as endovascular treatment, open surgery is preferred. Proximal splenorenal shunt is a well-accepted surgical procedure for extrahepatic portal vein obstruction. Splenectomy and aneurysmal resection can relieve hypersplenism and treat splenic artery aneurysm in patients with isolated splenic artery aneurysm at the splenic hilum. • SAA with EHPVO is especially common during pregnancy. • Albeit rare, due to its high risk of fatality, it is extremely important for clinicians to diagnose and manage it well. • Surgery is the best modality of treatment for SAA with EHPVO with hypersplenism. [ABSTRACT FROM AUTHOR]

Background and objective: Portal hypertension may affect intestinal functions, brush border enzymes and absorption parameters. Information about these in extrahepatic portal venous obstruction (EHPVO) in children is limited and poorly reported. We therefore studied the brush border enzymes and absorptive capacity in EHPVO in children. Methods: The study was conducted on 52 children of EHPVO. The diagnosis of EHPVO was made on the basis of clinical presentation and ultrasound examination. All patients underwent upper gastrointestinal endoscopy. Endoscopic tissue biopsies from duodenum were taken on aluminum foils and kept immediately at −20°C for estimation of brush border enzymes. Tissue biopsies were homogenized in sodium maleate buffer, 0.1 M pH 6.0, by a homogenizer and processed for the enzymes: lactase, maltase, and sucrase. Enzyme levels were compared to normal healthy controls ( n = 20). d-Xylose test, stool acid steatocrit for fat excretion in stools and stool alpha-1 antitrypsin were done to know about the absorptive parameters. Results: Enzyme levels of lactase (6.21 ± 5.67 IU/mg) and sucrase (37.07 ± 21.06 IU/mg) in EHPVO group were significantly lower as compared to lactase (23.32 ± 10.48 IU/mg) and sucrase (95.96 ± 46.55 IU/mg) in normal healthy controls. Maltase levels were lower, but difference was not statistically significant in EHPVO group (56.90 ± 28.65 IU/mg) as compared to normal controls (63.28 ± 22.88 IU/mg). There was no significant difference of urinary d-xylose and stool fat in patients with normal or short stature EHPVO patients. Conclusion: EHPVO leads to decrease in levels of brush border enzymes in small bowel but their absorption capacity remains normal. [ABSTRACT FROM AUTHOR]

Among the various causes for lower gastrointestinal bleeding, ectopic varices constitute a small chunk. Though rare, these can pose a diagnostic challenge with recurrent bleed leading to multiple admission and blood transfusions. A 41-year-old male presented to our department with multiple episodes of melena. On further evaluation with CT angiography, a diagnosis of extrahepatic portal vein obstruction with moderate splenomegaly and ectopic jejunal varix was made. He underwent splenectomy with resection of involved jejunal segment with side to side anastomosis. The diagnosis of ectopic varices remains elusive in a large number of cases in view of the varied etiology. Various newer endoscopic and imaging modalities can play a diagnostic as well as therapeutic role but this also further complicates the management as there is a lack of substantial guidelines directing the treatment protocol. As a result, we have to resort to a case by case approach for the optimal management in these cases. The main modality of management for bleeding ectopic varices is percutaneous or endoscopic. Surgery is reserved for refractory cases, with decompressive shunts combined with segmental resection of involved intestine being at the forefront of surgical options. • Ectopic varices are an unusual cause of lower GI bleeding caused by porto-systemic collaterals. • Ectopic varices most commonly occur at jejuno-ileum, but duodenal varices are more prone to bleed. • The optimal management varies with each case due to paucity of treatment guidelines. • Decompressive shunt with splenectomy and segmental resection of intestine effectively prevents rebleeding. [ABSTRACT FROM AUTHOR]

Extrahepatic portal venous obstruction (EHPVO) and temporomandibular joint (TMJ) ankylosisis are significant problems in Asian countries. Both EHPVO and bilateral TMJ ankylosis may have rare association due to protein C and S deficiency which may cause hypercoagulability as well as reduced fibrinolytic activity. Ankylosis arising in early childhood is associated with facial asymmetry, feeding difficulty and speech development alterations. It is also associated with great challenges of endoscopic management in extra hepatic portal vein obstruction (EHPVO) with variceal bleed as well as air way management during surgical management and post-operative recovery. Recently a case series had shown association of TMJ ankylosis with EHPVO due to protein C deficiency which might be an etiological factor for both EHPVO as well as TMJ ankylosis. This case report documents a case of 14 year young girl who had TMJ ankylosis due to ear infection and EHPVO with esophageal varices had multiple episodes of upper GI bleed with mild deficiency of protein C and S, successfully managed with proximal splenorenal shunt to prevent further episodes of upper GI bleed, as endoscopic management is not feasible due to TMJ ankylosis.

NCPH is a heterogeneous group of liver disorders of vascular origin, leading to PHT with near normal HVPG. NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. Both disorders present with clinically significant PHT with preserved liver functions. Diagnosis is easy and can often be made clinically with support from imaging modalities. Management centers on control and prophylaxis of variceal bleeding. In EHPVO, there are additional concerns of growth faltering, portal biliopathy, MHE and parenchymal dysfunction. Surgical shunts are indicated in patients with failure of endotherapy, bleeding from sites not amenable to endotherapy, symptomatic hypersplenism or symptomatic biliopathy. Persistent growth failure, symptomatic and recurrent hepatic encephalopathy, impaired quality of life or massive splenomegaly that interferes with daily activities are other surgical indications. Rex-shunt or MLPVB is the recommended shunt for EHPVO, but needs proper pre-operative radiological assessment and surgical expertise. Both disorders have otherwise a fairly good prognosis, but need regular and careful surveillance. Hepatic schistosomiasis, CHF and NRH have similar presentation and comparable prognosis., (Copyright © 2013 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)