Idiopathic Noncirrhotic Portal Hypertension Syndrome, also known as intrahepatic presinusoidal obstructive portal hypertension.

bubble_chart Pathological Changes

① Irregular thickening of the liver capsule membrane; ② Fibrous tissue hyperplasia in the portal area, with fibrous septa extending into the lobules. The septa are extremely fine and can only be detected with reticulin staining; ③ Grade I lobular structural deformation, with central veins being compressed or irregular.

bubble_chart Clinical Manifestations

Clinical manifestations of portal hypertension include recurrent hematemesis and melena, with good tolerance to gastrointestinal bleeding and absence of decompensation signs such as ascites, jaundice, or unconsciousness. Laboratory tests may show normal liver function.

bubble_chart Diagnosis

1. Clinical and radiological examinations reveal definite portal hypertension, including significant splenomegaly, esophageal varices, recurrent upper gastrointestinal bleeding, with normal or essentially normal liver function.

2. Splenoportography shows no obstruction or stenosis in the extrahepatic portal vein.

3. Diagnosis can be made based on the characteristic pathological changes observed in liver tissue biopsy. {|102|}

bubble_chart Treatment Measures

For patients with little or no bleeding, conservative treatment may be adopted; for those requiring surgery, splenectomy combined with splenorenal venous anastomosis is preferable to portacaval shunt, as the latter has a higher incidence of portal shunt encephalopathy.

bubble_chart Differentiation

1. Pre-sinusoidal obstruction caused by other reasons, such as congenital or recurrent hepatic fibrosis, is often accompanied by polycystic kidney in children under 10 years old. Liver biopsy reveals a large amount of fibrous tissue proliferation in the portal area, extending in cord-like patterns into the lobules, which is the main diagnostic basis.

3. Portal hypertension caused by sinusoidal or intrahepatic post-sinusoidal obstruction, such as long-term use of azathioprine, 6-MP, or excessive vitamin A leading to abnormal collagen fiber proliferation in the Disse space and perisinusoidal fibrosis, requires careful medical history inquiry for differentiation.

4. Extrahepatic portal vein thrombosis or stenosis is often accompanied by abdominal distension and fullness, abdominal pain, hematochezia, and ascites. Splenoportography can confirm the diagnosis.