Extrahepatic portal vein obstruction

BACKGROUND AND OBJECTIVES: More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency...

Non-cirrhotic portal hypertension (NCPH) is a well-recognized clinico-pathological entity, which is associated with clinical signs and symptoms, imaging, and endoscopic features of portal hypertension (PHT), in absence of cirrhosis. In patients with NCPH without known risk factors of PHT or extrahepatic portal vein thrombosis, the condition is called idiopathic non-cirrhotic portal hypertension (INCPH). There are multiple infectious, immune related causes, systemic diseases, drug and toxin exposures, haematological disorders, and metabolic risk factors that have been associated with this INCPH...

This report describes the understudied co-occurrence of temporomandibular joint ankylosis (TMJA) and extrahepatic portal vein obstruction (EHPVO), exploring a shared pathway involving hypercoagulability. TMJA is an acquired pathology where joint surfaces fuse, causing restricted mouth opening and facial asymmetry. Globally, TMJA is prevalent among 1.5 to 5 patients/million, with a higher incidence in developing countries. While trauma and infections often cause TMJA, the pathogenesis remains unclear in many cases...

Extrahepatic portal vein obstruction (EHPVO) is a rare disease. Most EHPVO patients are usually referred to a gastroenterologist for intestinal bleeding and hypersplenic thrombocytopenia; however, hypercoagulative diseases may be occult in these patients and require anticoagulation. The purpose of this study was to elucidate the clinical characteristics of EHPVO. We conducted a retrospective analysis of the hospital database, evaluating the medical records of 15 patients (7 males, 8 females, mean age of onset 42...

Biliary stent insertion is a well-established treatment of bile duct obstruction. Stent migration is a late-onset complication and can be life-threatening. We present a case of proximal biliary stent migration to the portal vein. An upper endoscopy was performed, and the biliary stent was retracted. Bleeding was seen from the papilla of Vater. The hepatoduodenal ligament was explored, and the common bile duct was found fully adherent to the portal vein, with a fistula between the common bile duct and the portal vein...

INTRODUCTION: Extrahepatic Portal Vein Obstruction is the most common cause of portal hypertension in children. However, it has a very low prevalence. Esophageal varices due to portal hypertension in children can lead to recurrent episodes of upper gastrointestinal bleeding, which can have a sinister outcome if timely diagnosis and treatment are not initiated. CASE PRESENTATION: A 7-year-old male child presents with recurrent episodes of upper gastrointestinal bleeding for 3 years...

AIM: There are few data regarding the safety and effectiveness of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines in patients with intractable hepatobiliary diseases. We conducted a multicenter, questionnaire-based, cross-sectional study to determine the safety and effectiveness of the SARS-CoV-2 vaccines in Japanese patients with intractable hepatobiliary disease. METHODS: Patients aged ≥18 years with autoimmune hepatitis (AIH), primary biliary cholangitis, primary sclerosing cholangitis, Budd-Chiari syndrome, idiopathic portal hypertension, and extrahepatic portal vein obstruction at each center were consecutively invited to join the study...

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...

PURPOSE: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. METHODS: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed...

BACKGROUND: This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. METHODS: EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV...

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BACKGROUND: Concerned studies with respect to the outcome of pregnant patients with non-cirrhotic portal hypertension are limited. Thus, a systematic review and meta-analysis of the available literature was conducted. METHODS: A literature search was conducted from 1999 to December 2021 for studies evaluating pregnancy outcomes in patients with non-cirrhotic portal hypertension. RESULTS: Twelve studies were included in the meta-analysis. The pooled rate of variceal bleeding, ascites and severe anemia requiring blood transfusion were 9...

BACKGROUND: As an emerging standard of care for portal vein cavernous transformation (PVCT), Meso-Rex bypass (MRB) has been complicated and variated. The study aim was to propose a new classification of PVCT to guide MRB operations. METHODS: Demographic data, the extent of extrahepatic PVCT, surgical methods for visceral side revascularization, intraoperative blood loss, operating time, changes in visceral venous pressure before and after MRB, postoperative complications and the condition of bypass vessels after MRB were extracted retrospectively from the medical records of 19 patients...

Atypical variceal bleeding, which primarily stems from extrahepatic portal vein obstruction (EHPVO), is a severe complication of pancreatic hepatobiliary surgery. This review provides insights into this condition's incidence, diagnosis, and management strategies. The treatment modalities for atypical variceal bleeding resulting from EHPVO range from endoscopic intervention to surgical procedures, including direct variceal ligation and shunt surgery. Here, we discuss the efficacy and potential limitations of each treatment approach...

BACKGROUND: Hepatic encephalopathy, (HE) although commonly associated with cirrhosis, has also been reported in non-cirrhotic portal hypertension (NCPH). The importance of identifying and treating HE in NCPH lies in the fact that many patients may be wrongly diagnosed as having psychiatric or neurologic disorders. Hence, we aimed to systematically review the prevalence of HE in NCPH. METHODS: A comprehensive search of three databases (Medline, Embase and Scopus) was conducted from inception to November 2022 for studies reporting on the prevalence of minimal HE (MHE) and overt HE (OHE) in patients with NCPH...

Extrahepatic portal vein obstruction (EHPVO) is a very rare disease-causing portal hypertension. Myeloproliferative neoplasm (MPN) including essential thrombocythemia (ET) is reported as a risk factor for EHPVO due to underlying persistent thrombophilia.A Japanese woman in her 40s was referred to our hospital with a one-month history of gastric variceal bleeding due to EHPVO. Laboratory investigation demonstrated thrombocytosis despite portal hypertension. She had a mutation of clonal marker JAK2V617F with EHPVO, which prompted us to consult a hematologist...

•Most data on the natural history of portal hypertension come from studies in adults. •The morbidity rate of upper gastrointestinal bleeding in children with portal hypertension tend to be underestimated. •This study showed the relevance of morbidity rates after variceal hemorrhage in pediatric patients, especially those with cirrhosis. •Patients with hemodynamic instability requiring blood transfusion or expansion on admission are at increased risk of complications secondary to upper gastrointestinal bleeding and should be closely monitored...

We present a 27-year-old man with a 2-year history of extrahepatic portal vein obstruction and selective immunoglobulin A deficiency, referred for acute cholangitis from portal cavernoma cholangiopathy (PCC). Because recurrent cholangitis rapidly led to liver failure, orthotopic liver transplantation (OLT) was successfully performed. To date, this is one of the few cases of patients with symptomatic PCC who required OLT and the first case who had a successful 6-year follow-up. Thus, OLT can be used for symptomatic PCC associated with nonshuntable anatomy, ineffective biliary drainage, and progressive liver damage...

BACKGROUNDS/AIMS: Biliary surgery in patients with extrahepatic portal vein obstruction with portal cavernoma (PC) is technically challenging, and associated with the risk of bleeding. Therefore, prior portal vein decompression is usually recommended before definitive biliary surgery. Only a few studies have so far reported the safety of isolated laparoscopic cholecystectomy. We aimed to evaluate our experience of laparoscopic cholecystectomy in patients with PC without prior portal decompression...

INTRODUCTION:  Umbilical vein catheterization (UVC) can cause portal venous thrombosis, leading to the development of extrahepatic portal venous obstruction (EHPVO) and portal hypertension (PHT). The feasibility of the Meso-Rex bypass (MRB) for the treatment of EHPVO in patients with a history of UVC has been questioned. We compared the feasibility of performing an MRB in patients with or without a history of previous UVC. METHODS:  A retrospective review of patients with EHPVO and known UVC status explored for a possible MRB at our institution was performed (1997-2022)...