Clinical features of hepatic sinusoidal syndrome

Objective To improve the understanding of diagnosis and treatment of hepatic sinusoidal syndrome(HSOS) by analyzing its clinical features. Methods Clinical data about 23 HSOS patients admitted to Chinese PLA General Hospital were retrospectively analyzed. Results Of the 23 HSOS patients, 8(34.7%) were treated with Chinese medicines containing Gynura Rhizom, 2(8.7%) received chemotherapy and immunosuppressive therapy, 2(8.7%) were given analgesics including nonsteroidal anti-inflammatory agents, and 11(47.9%) had no clear predisposing cause. Their main clinical manifestations were ascites(78.3%), abdominal distension(73.9%), hepatomegaly(21.7%), abdominal pain at the right upper quadrant(26.1%) and jaundice(17.4%), with varying degrees of impaired liver function. Type-B ultrasonography(US) showed uneven distribution of enhanced echo in liver, unclear plaque or very fine hepatic vein, decreased or disappeared blood flow in portal vein, and flat posterohepatic vena cava. Enhanced CT scanning showed map-like uneven distribution of contrast agent in liver. Angiography revealed dry branch like or spider weblike changes in liver. Pathology demonstrated hepatic sinusoid congestion and dilatation, degeneration and necrosis of hepatic cells at different degrees, endothelial oedema or fibrosis of central vein or sublobular vein. Of the 23 patients after medical treatment, 18(78.3%) were improved, 1(4.3%) received no treatment, 2(8.7%) failed to treatment, and 3(8.7%) died. Conclusion Take administration of certain Chinese herbal medicine, chemotherapy and immunosuppressive therapy are the main predisposing cause of HSOS. Its diagnosis can be established according to its pathology, history, clinical manifestations and characteristic imaging. Early use of liver-protective, anticoagulant and microcirculation drugs can improve the prognosis of HSOS patients.