肝静脉型布-加综合征与肝窦阻塞综合征的CE-MRA鉴别诊断

The Differential Diagnosis of BCS(type Ⅱ) and Hepatic Sinusoidal Obstruction Syndrome by CE-MRA

目的提高肝静脉型布-加综合征和肝窦阻塞综合征的影像诊断水平。方法回顾性分析经DSA证实的30例肝静脉型布-加综合征及经病理证实的8例肝窦阻塞综合征的CE-MRA特点,归纳两者影像表现的差异。结果本组肝静脉型布-加综合征和肝窦阻塞综合征门静脉内径分别为1.53±0.33cm、1.47±0.39cm,差异无统计学意义(P=0.104)。(1)肝静脉型布-加综合征主要表现为:肝大、肝叶比例失调、尾状叶体积增大、脾大、腹水;30例肝静脉型布-加综合征患者,35支肝静脉显示不清、28支肝静脉闭塞(38.89%)、19支合并血栓(21.11%)、8支通畅(8.89%);30例患者均显示的肝内侧支;静脉期肝脏强化分布表现为反"爪"型强化(40%)、内高外低强化(36.67%)、内低外高强化(10%)、均匀强化(10%)及不规则强化(3.33%)。(2)肝窦阻塞综合征主要表现为肝脾大、腹水;8例肝窦阻塞综合征患者,有18支(6例)肝静脉显示清楚、管腔通畅,余6支(2例)显示不清;8例肝窦阻塞综合征患者均未示肝内侧支血管;静脉期肝脏强化呈"爪"型分布(87.5%),余1例强化不规则。结论 CE-MRA在鉴别肝静脉型布-加综合征和肝窦阻塞综合征中可提供可靠的信息,通过仔细观察影像表现,对两者诊断准确性会有进一步提高。

Objective To raise the imaging diagnostic levle of BCS （type II） and Hepatic Sinusoidal Obstruction Syndrome （HSOS）. Methods The CE-MRA of 30 cases with BCS （type II） proved by DSA and 8 cases with HSOS diagnosed by histology were analyzed retrospectively. Results The mean diameter of portal vein of BCS and HSOS were 1.53±0.33cm and 1.47±0.39cm, and there was no significant difference between them （P=0.104）. （1） The clinical manifestations of BCS were hepatomegaly （70%）, caudate lobe enlargement （86.67%）, splenomegaly（100%）, ascites（70%） and hepatic lobes with irregular shape （93.33%）; 35 hepatic veins were not showed（38.89%）, 19 with thrombosis （21.11%）, 28 with obstructed veins;8 cases with patency; 30 cases showed Intra-hepatic collateral vessels; 36.67% cases showed diminished enhancement in peripheral liver, and 10% cases showed diminished enhancement in central liver, and 40% cases represented＂claw-shaped＂ diminished enhancement around hepatic veins, and 10% cases showed homogeneous enhancement and 3.33% cases showed Irregular enhancement. （2） The clinical manifestations of HSOS were hepatomegaly （100%）, splenomegaly（12.5%）, ascites（100%）; 6 hepatic veins（2cases） were not showed; all cases were not show intra-hepatic collateral vessels; 87.5% cases represented ＂claw-shaped＂ enhancement around hepatic veins, and 12.5% cases showed irregular enhancement. Conclusion CE-MRA is helpful in differentiating BCS （type II） from HSOS. The accuracy of differential diagnosis will be further improved by observing the imaging findings carefully.