原発性肝外門脈閉塞症

Extrahepatic portal vein obstruction (EHPVO) is a major cause of noncirrhotic portal hypertension, particularly in children and young adults. Traditionally, definitive management relied on surgical shunts such as proximal splenorenal shunt and meso-Rex bypass; however, extensive porto-mesenteric thrombosis, unfavorable anatomy, perioperative morbidity, and limited surgical expertise may restrict surgical feasibility in many patients. Over the last decade, advances in interventional radiology have substantially expanded therapeutic options beyond surgery. Portal vein recanalization (PVR) is increasingly considered a physiological endovascular treatment strategy in anatomically suitable patients because it restores hepatopetal portal flow while preserving native hepatic perfusion. In patients with diffuse intrahepatic involvement, persistent portal hypertension, or inadequate portal inflow, adjunctive transjugular intrahepatic portosystemic shunt (TIPS) may improve decompression and long-term patency. Transjugular extrahepatic portosystemic shunt (TEPS) serves as an advanced salvage option in complex anatomy unsuitable for conventional TIPS, while partial splenic embolization and variceal embolization complement management of hypersplenism and refractory variceal bleeding. This review provides an overview of endovascular interventions in EHPVO, highlighting the underlying rationale, patient selection, techniques, outcomes, and complications.

Acute cholangitis is a potentially life-threatening infection of the biliary tract that usually results from biliary obstruction, most commonly secondary to choledocholithiasis. Although prompt diagnosis and treatment often lead to favorable outcomes, uncommon complications such as pylephlebitis (septic thrombosis of the portal vein) may significantly increase morbidity and mortality. We report the case of a 47-year-old woman with no significant past medical history who presented with right upper quadrant abdominal pain, fever (38.2°C), asthenia, and nausea. Physical examination revealed diffuse abdominal tenderness and mild jaundice. Abdominal point-of-care ultrasound (POCUS) made by the emergency physician on call showed multiple gallbladder calculi with sludge but without visible biliary dilatation. Laboratory evaluation demonstrated an inflammatory syndrome with C-reactive protein of 72 mg/L, normal leukocyte count, total bilirubin of 87 IU/L, and direct bilirubin of 56 IU/L, and cholestatic liver enzyme abnormalities, including alkaline phosphatase (ALP) of 190 IU/L and gamma-glutamyl transferase (GGT) of 108 IU/L. The diagnosis of acute angiocholitis was thus suspected. Abdominal imaging (computed tomography (CT) with contrast) showed intrahepatic and extrahepatic biliary dilatation caused by a distal common bile duct stone, multiple gallbladder calculi, and segment II left portal vein thrombosis consistent with pylephlebitis. A diagnosis of acute cholangitis secondary to choledocholithiasis complicated by pylephlebitis was established. The patient was treated with intravenous (IV) antibiotics, fluid resuscitation, and supportive care, with planned biliary decompression and definitive surgical management. This case highlights a rare but serious vascular complication of biliary stone disease. Clinicians should maintain a high index of suspicion for portal venous involvement in patients with cholangitis and persistent systemic symptoms. Early imaging, timely antimicrobial therapy, and coordinated multidisciplinary management are essential to reduce the risk of severe complications and improve outcomes.

Perihilar cholangiocarcinoma (Klatskin tumor) remains one of the most technically challenging hepatopancreatobiliary malignancies because of its close association with the portal vein and hepatic artery within the hepatic hilum. Current operative management of Bismuth type I and II lesions remains controversial, particularly regarding the role of bile duct resection alone versus combined hepatectomy to achieve oncologically adequate R0 margins while minimizing operative morbidity. While traditionally managed through an open approach, robotic surgery has emerged as a minimally invasive option for selected complex biliary and vascular resections. We present the robotic resection of a Bismuth type II Klatskin tumor in a 72-year-old male who presented with obstructive jaundice and a mid-bile duct stricture. Following preoperative optimization, the patient underwent robotic extrahepatic biliary resection, radical lymphadenectomy, portal vein lateral venorrhaphy, and Roux-en-Y biliary reconstruction. Preoperative imaging and intraoperative assessment demonstrated localized hilar involvement without significant longitudinal extension into the right or left hepatic ducts, allowing bile duct resection without formal hepatectomy while still achieving negative proximal and distal margins. Tumor resection was completed without conversion to open surgery. Portal vein cross-clamp time was 10 minutes. Unification ductoplasty between the right anterior and posterior sectoral ducts enabled dual hepaticojejunostomy reconstruction. At 2-year surveillance follow-up, the patient remained without evidence of recurrence. This case demonstrates the technical feasibility of robotic portal vein lateral venorrhaphy and complex biliary reconstruction in selected Bismuth type II perihilar cholangiocarcinoma requiring dual hepaticojejunostomy reconstruction.

Portal cavernoma cholangiopathy (PCC), also referred to as portal biliopathy, encompasses a heterogeneous spectrum of biliary tract abnormalities that develop as a consequence of extrahepatic portal vein obstruction (EHPVO) with subsequent cavernous transformation of the portal vein. Pseudotumoral portal cavernoma represents a distinct subtype of PCC and is characterized by the presence of numerous fine collateral venous channels that encase and simulate the course of the common bile duct (CBD). We report 2 cases of pseudotumoral portal cavernoma secondary to portal vein thrombosis in patients with underlying myeloproliferative disorders. These cases illustrate the diagnostic challenges associated with this rare entity and underscore the central role of cross-sectional imaging in establishing an accurate diagnosis and preventing misinterpretation as a biliary neoplasm.

BACKGROUND: Extrahepatic portal vein obstruction (EHPVO) can be idiopathic or secondary to congenital abnormalities and other conditions in the pediatric population. The severity of the disease can be accurately determined by the precise degree of hepatic fibrosis. Most cases result from portal vein thrombosis (PVT). This study aimed to evaluate the accuracy of liver stiffness measurement (LSM) compared with liver biopsy for assessing liver fibrosis in patients with PVT. METHODS: This retrospective cross-sectional study included children aged 3-18 years with EHPVO/PVT who were referred to the Cairo University Children's Hospital between 1 July 2016 and 31 December 2018. All patients underwent clinical examination, routine laboratory studies, and Doppler ultrasound of the portal system. Fibrosis was staged on liver biopsy using the METAVIR scoring system. Logistic regression was used in an exploratory manner to assess factors associated with hepatic fibrosis. RESULTS: A total of 20 children were included. Males predominated, accounting for 85%. Splenomegaly was present in 70% of patients and hepatomegaly in 20%. Hepatic fibrosis was absent in 25%, mild in 30%, and moderate in 45% of patients. FibroScan (transient elastography, TE) values were the most strongly associated with fibrosis stage, had good discriminative ability (AUROC 0.912), and appeared to outperform platelet count and liver function tests. Clinical features, including bleeding history, did not correlate with fibrosis stage. CONCLUSION: Platelet count and FibroScan (TE) may serve as useful indicators of hepatic fibrosis in children with PVT, with FibroScan (TE) being the strongest associated factor in this sample.