Liver histopathology in patients with hepatic masses and the Abernethy malformation

Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases

“…Treatment with LT has been proposed, but is unnecessary in the vast majority of children because of modern endovascular shunt closure techniques. ( 324,325 ) A significant proportion of these patients may have severe congenital cardiac, genitourinary, and musculoskeletal defects. ( 323 ) Acquired portopulmonary hypertension (15% of cases) and hepatopulmonary syndrome (3%) require careful management ( 323 ) of pulmonary hypertension preoperatively and postoperatively.…”

Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt

“…This is supported by the observation that CEPS-associated liver nodules typically arise in liver tissue, which is supplied with predominantly arterial blood due to CEPS type l malformations (6,21,22). Also, excessive capillarization appears to be more common in the liver of children with CEPS and coexisting liver masses (17). Lack of portal flow may contribute to enlarging arterial branches, hypertrophy of hepatocytes, and eventually, liver tumors.…”

“…HAs are generally classified into three different subtypes (12): (1) adenomas with mutations in the tumor suppressor HNF1alpha (≈45%) (9), (2) adenomas with mutations in ß-catenin (≈10%) and (3) adenomas with inflammatory features and no specific mutations (≈40%). In patients with CEPS, liver nodules are typically associated with rarefication of venous vessels within the portal tracts (6,13,14), hypertrophy of hepatic arterial branches and remodeling of liver architecture (15)(16)(17). Progression of HA into HCC has been described in patients with CEPS, including occasional children (18).…”

Hepatopulmonary Syndrome in an Adolescent With Insidious Hypoxia and Small Intrahepatic Portal Venous Shunts: Posttransplant Benefit From Sildenafil

“…4 venous circulation bypassing the liver and may lead to HPS. [4][5][6] They are often discrete vessels large enough to be visualized on angiography. 7 In contrast to well described cases of CPSS, our patient exhibited several anatomic differences.…”