Congenital porto-sinusoidal vascular disorder-induced portal hypertension: A comprehensive review based on a classic clinical case

Figure 1 Occlusive portal vein lesions accompanied by nodular regenerative hyperplastic changes. A: Hepatic sinusoidal lymphocytic infiltration and stromal fibrosis in the confluent zone of the portal vein; B: Portal vein was partially narrowed and occluded, partially tortuous and dilated; C: Peri-sinusoid fibrosis (Masson trichrome); D: Nodular disorganization with hepatocyte plate atrophy (reticulin); E: Immunohistochemical staining showed positivity for CD10, CD38, CK19, and CK7. Scale bars = 100 μm.

Figure 2 The potential pathophysiological mechanisms of congenital portal hypertension essentially involve abnormalities in the structure or hemodynamics of the portal venous system. Including: Congenital venous developmental defects, congenital hepatic fibrosis, perinatal venous thrombosis, molecular genetic factors such as gene mutations, and environmental factors such as perinatal infections and drug exposure. Additionally, complications arising in related sites due to congenital portal hypertension may occur. FBN1: Fibrillin 1; NOTCH1: Notch receptor 1; ACVRL1: Activin A receptor like type 1; MRI: Magnetic resonance imaging.

Figure 3 Treatment approaches for congenital portal hypertension. Including pharmacological interventions, endoscopic therapy during acute bleeding episodes, and surgical/interventional procedures to improve long-term prognosis.