CT Diagnosis and Differentiation Between Primary Hepatic Vein Budd-Chiari Syndrome and Hepatic Sinusoidal Obstructive Syndrome
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摘要:
目的：原发性肝静脉型布−加综合征（BCS）与肝窦阻塞综合征（SOS）的临床表现及实验室检查缺乏特异性，影像学表现相似。本研究通过收集BCS及SOS的临床及影像特点，探讨增强CT在两种疾病鉴别诊断的应用。材料与方法：回顾分析经DSA确诊的26例BCS和经肝活检确诊的20例SOS患者的临床及影像资料，比较其差异。结果：BCS患者门脉高压相关表现较明显，肝动脉直径、门脉主干直径及脾静脉增宽程度均超过SOS患者；CT增强扫描16例BCS患者呈现出门脉期以第1肝门及尾状叶为主出现斑片状强化方式；11例SOS患者门脉期则呈现出以第2肝门为中心的“鸦爪样”不均质强化方式；BCS肝功能损伤相对较轻于SOS患者，SOS患者的碱性磷酸酶增高、总胆固醇增高、白蛋白减低程度较BCS患者相对明显。两者病例影像的共同点是静脉期三支肝静脉不显影或显影纤细。结论：CT平扫及增强检查对BCS和SOS有重要意义，尾状叶体积改变、侧支循环开放程度等都是二者CT鉴别诊断要点，特别是增强CT所示的BCS呈现以第1肝门为中心不均匀强化方式及SOS以第2肝门为中心的不均匀强化方式有助于疾病早期的鉴别诊断。
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关键词:
- 体层摄影术 /
- X线计算机 /
- 原发性肝静脉型布−加综合征 /
- 肝窦阻塞综合征 /
- 鉴别诊断
Abstract:Objective: The clinical and laboratory specificity for diagnosing primary hepaticveins Budd-Chiari syndrome (BCS) and hepatic sinusoidal obstructive syndrome (SOS) is limited. This study aims to explore the application of contrast-enhanced CT in the differential diagnosis of BCS and SOS by examining their clinical and imaging features. Materials and Methods: Clinical and imaging data from 26 patients diagnosed with BCS via DSA and 20 patients diagnosed with SOS via liver biopsy were retrospectively analyzed. The differences between the two groups were compared. Results: Portal hypertension-related symptoms were more prominent in 16 patients with BCS, and the diameter of the hepatic artery, portal trunk, and degree of splenic vein dilation were all greater than those in 11 patients with SOS. On contrast-enhanced CT, BCS exhibited a patchy enhancement pattern in the portal phase, primarily localized to the first hepatic portal and caudate lobe. In contrast, SOS showed a “crow's claw-like” heterogeneous enhancement pattern centered on the second hepatic division. Liver function injury in BCS patients was less severe than in SOS patients. Elevated alkaline phosphatase, increased total cholesterol, and decreased albumin levels were more pronounced in SOS patients compared to those with BCS. A common feature in both groups was the absence or faint visibility of the three hepatic veins in the venous phase. Conclusion: The CT findings of BCS and hepatic SOS exhibit certain specificities. The enlargement of the caudate lobe and the degree of collateral circulation are key factors in the CT-based differential diagnosis. Notably, the heterogeneous enhancement pattern of BCS centered on the first hepatic portal and the heterogeneous enhancement pattern of SOS centered on the second hepatic portal in contrast-enhanced CT are valuable for the early differential diagnosis of these conditions.
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图 1 BCS CT增强检查特征性表现，男19岁，经DSA检查确诊为BCS。CT增强扫描动脉期肝实质轻度均匀强化，门脉期肝实质以第一肝门和尾状叶为主明显强化，延迟期强化范围较前增大
Figure 1. Characteristic findings of Budd-Chiari syndrome (BCS) on contrast-enhanced CT. 19-year-old male, diagnosed with BCS via DS, underwent enhanced CT. The arterial phase showed mild nomogeneous enhancement in the hepatic parenchyma, while the portal phase revesled significant enhanoement in the first hepatic portal and eaudate lobe. In the delayed phase, the enhancement range was norably increased
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图 2 SOS CT增强检查特征性表现。男49岁，经肝穿确诊为SOS，CT增强扫描动脉期肝实质轻度均匀强化，门脉期肝实质以第二肝门为中心的“鸦爪样”不均质强化，延迟期强化范围较前增大
Figure 2. Characteristic findings of hepatic sinusoidal destructive syndrome (SOS) on contrast-enhanced CT. A 49-year-old male, diagnosed with SOS via liver biopsy, underwent contrast-enhanced CT. The arterial phase showed mild homogeneous enhancement in the liver parenchyma, while the portal phase revealed a “raven’s paw” heterogeneous enhancement pattern centered on the second hepatic hilum
表 1 两组患者的实验室检查（%）
Table 1 Laboratory tests between the two groups (%)
分组 天冬氨酸
转氨酶 > 35 U/L丙氨酸
转氨酶 > 40 U/L碱性磷
酸酶 > 135 U/Lγ-谷氨酰
转肽酶 > 45 U/L总胆红素 > 17.1 umol/L 总胆固醇＞5.7 mm/L 白蛋白 < 35 g/L 凝血酶
原时间 > 13 S）血小板 < 100×
109CA125
(升高)＞
正常值2 倍尿蛋白
（+）原发性
肝静脉型
布−加综合征44 8 31 73 85 0 50 77 36 45 0 肝静脉
闭塞综合征45 30 60 90 90 15 85 70 35 65 5 
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表 2 两种疾病的影像学特征
Table 2 Imaging features of the two groups
类别 原发性肝静脉型布−加综合征 肝窦阻塞综合征 CT平扫 肝脏肿大，肝实质密度不均匀减低，多伴腹水形成 肝脏肿大，肝实质密度不均匀减低，多伴腹水形成 CT增强动脉期 肝实质均未见明显强化或呈轻度强化方式 肝实质均未见明显强化或呈轻度强化方式 门脉期 以第一肝门及尾状叶为主出现斑片状强化 第二肝门为中心的“鸦爪样”不均质强化 延迟期 肝实质仍呈斑片状强化，强化范围较门脉期进一步增大 肝实质仍呈“鸦爪样”强化，强化范围较门脉期进一步增大 肝静脉显影 未见显影、部分显影或纤细 未见显影、部分显影或纤细 肝动脉直径/mm 4.695±0.70 4.00±0.83 门静脉直径/mm 12.42±2.17 9.73±1.93
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