| disease | Idiopathic Non-cirrhotic Portal Hypertension |
| alias | Idiopthic Noncirrhotic Portal Hypertension Syndrom |
Idiopathic Noncirrhotic Portal Hypertension Syndrome. Also known as intrahepatic presinusoidal obstructive portal hypertension.
bubble_chart Etiology
(1) Irregular thickening of the liver capsule membrane; (2) Fibrous tissue proliferation in the portal area, with fibrous septa extending into the lobules. The fibrous septa are very fine and can only be detected with reticulin staining; (3) Grade I lobular structural distortion, with central veins compressed or irregular.
bubble_chart Clinical Manifestations
Clinical manifestations of portal hypertension include recurrent hematemesis and melena, with good tolerance to gastrointestinal bleeding and no signs of decompensation such as ascites, jaundice, or unconsciousness; laboratory tests may show normal liver function.
1. Clinical and radiological examinations confirm definite portal hypertension, including significant splenomegaly, esophageal varices, recurrent upper gastrointestinal bleeding, with normal or essentially normal liver function.
2. Splenoportography demonstrates no obstruction or stenosis in the extrahepatic portal vein.
3. Diagnosis can be established 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 superior to portacaval shunt, as the latter has a higher incidence of portal systemic encephalopathy.
1. Other causes of presinusoidal obstruction, such as congenital or recurrent hepatic fibrosis, often manifest in children under 10 years old and are frequently associated with polycystic kidney disease. Liver biopsy reveals extensive fibrous tissue proliferation in the portal area, with cord-like extensions into the lobules, serving as the primary diagnostic basis.
2. Postsinusoidal obstruction-induced portal hypertension, such as hepatic vein obstruction or intrahepatic hepatic vein branch obstruction, presents with sudden liver pain, progressive hepatomegaly, and refractory ascites.3. Portal hypertension caused by sinusoidal or intrahepatic postsinusoidal obstruction, such as long-term use of azathioprine, 6-MP, or excessive vitamin A, leads to abnormal collagen fiber proliferation in the Disse space and perisinusoidal fibrosis. A detailed medical history should be obtained for differential diagnosis.
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.
