肝窦阻塞综合征八例临床分析

目的探讨肝窦阻塞综合征（SOS）的临床诊治方法。方法回顾性分析8例SOS的临床资料,并进行随访。结果8例SOS主要临床表现为腹胀（8例）、肝区疼痛（7例）、腹水征（8例）及肝肿大（7例）等。8例的肝功能损害程度大多较轻,血清-腹水白蛋白梯度均大于11．1g／L,血清与腹水CA125均显著升高。超声检查均见肝脏肿大、胆囊壁水肿或增厚、门静脉增宽且流速缓慢、肝静脉变细以及腹腔积液等;MRI表现为门脉期及延迟期肝实质不均匀片状强化,肝叶、段静脉腔内造影剂充盈不良。经皮肝穿刺活检均见肝窦扩张、淤血及肝细胞变性、坏死,3例发现小静脉管腔狭窄、管壁增厚伴纤维组织增生。8例中1例行肝移植术后痊愈,4例经内科治疗后逐渐康复,3例死亡。结论SOS的临床表现以突出的门脉高压症为特点,CA125常显著升高,超声及MRI对本病的诊断与鉴别诊断有重大价值,而经皮肝穿刺活检的价值有限,联合应用影像学方法与病理活检可提高诊断正确率。早期应用抗凝药物是治疗本病的关键,严重病例可行肝移植术。

Clinical characteristics of hepatic sinusoidal obstruction syndrome: analysis of 8 cases

Objective To investigate the diagnosis and treatment of sinusoidal obstruction syndrome (SOS).Methods The data of 8 patients with SOS,including clinical manifestations,laboratory results, imaging,pathology,and the course of diagnosis and treatment were reviewed.All cases were followed up. Results The main clinical manifestations included abdominal distention,hepatalgia and signs of ascites and hepatomegaly.There were mild or moderate hepatocellular injury in 6 patients and heavy injury in 2.All patients'serum-ascites albumin gradient exceeded 11.1 g/L.The levels of CA125 in both serum and ascites elevated significantly.All patients'ultrasonography showed hepatomegaly,appearance of portal hypertension and attenuated hepatic veins.Reverse blood flow in portal vein was observed in 5 cases.Magnetic resonance imaging showed that contrast agent accumulated unevenly in liver in both portal period and lag period,but filled poorly in hepatic veins.Per cutem liver biopsy showed that all patients'hepatic sinusoids were congested,but venular occlusion was observed in only 3 cases.Five cases had been misdiagnosed.One patient healed after liver transplantation,4 patients recovered gradually by treatment with heparin and so on and 3 patients died.Conclusions Signs of outstanding portal hypertension with mild hepatocellular injury is the main clinical feature of SOS.Both serum and ascites CA125 levels in SOS patients are elevated significantly.The misdiagnosis rate of SOS is quite high,ultrasonography and magnetic resonance imaging have significant value in diagnosis and differential diagnosis,while the value of per cutem liver biopsy is limited.Combination of imaging and pathology should contribute to correct diagnosis of SOS.Application of anticoagulant in early course is vital,liver transplantation should be considered in severe cases.