小打卡 每日打卡：0010、腹腔含脂肪病变—疾病谱及鉴别诊断要点 小程序
来源：双语学影像；病例选自《Mayo Clinic Body MRI Case Review》
19-year-old woman with midabdominal bloating and discomfort
19岁女性，中腹部饱胀、不适。
Fig 2.17.1
Fig 2.17.2
Fig 2.17.3
Fig 2.17.4
Coronal SSFSE (Figure 2.17.1) and axial fat-suppressed T2-
weighted FSE (Figure 2.17.2) images demonstrate marked hepatomegaly with subtle hypointensity of the caudate lobe compared with the remainder of the liver.
Axial portal venous phase postgadolinium 3D SPGR images (Figure 2.17.3) show a nutmeg appearance of diffuse heterogeneous enhancement with relative sparing of the caudate lobe. Axial oblique and coronal oblique minimum-intensity projection images (Figure 2.17.4) reconstructed from equilibrium phase data demonstrate hepatic vein thrombosis.
冠状位SSFSE序列(Figure 2.17.1)、横断位脂肪抑制T2WI FSE序列(Figure 2.17.2)示肝脏明显增大，肝尾叶信号略低。
3D SPGR增强扫描门静脉期(Figure 2.17.3)示肝实质弥漫性不均匀强化，呈肉豆蔻样。尾叶强化信号较均匀。（a nutmeg appearance？肉豆蔻样？小编没见过肉豆蔻，sorry）
平衡期图像的斜横断和斜冠状位最小密度投影重建(Figure 2.17.4)可见肝静脉血栓形成。
Diagnosis
Budd-Chiari syndrome（BCS）
布加综合征
Comment
BCS is characterized by hepatic venous outflow obstruction, which can occur at the level of the intrahepatic veins, the IVC, or the right atrium. It is named in honor of George Budd, a British internist who described 3 cases of hepatic vein thrombosis in 1845, and Hans Chiari, an Austrian pathologist who published the first pathologic description of BCS in 1899 (and is also responsible for the Arnold-Chiari malformation and the Chiarinetwork).
BCS的特点是肝静脉流出道梗阻，可发生于肝内静脉、下腔静脉、右心房。其命名是为了纪念1845年首先描述了3例肝静脉血栓形成的英国学者George Budd，和1899年首次发表BCS病理报道的奥地利病理学家Hans Chiari。（Hans Chiari还首次报道了Arnold-Chiari畸形和Chiari网）
BCS has been divided into primary and secondary causes.Primary BCS involves intrinsic venous abnormalities, such as thrombosis, stenosis, or webs, and secondary BCS results from extrinsic compression by tumors or other masses.Obstruction of the IVC with or without involvement of the hepatic veins is predominantin Asia; pure hepatic vein obstruction is more commonly seen in Western countries.Risk factors include inherited and acquired hypercoagulable states, and this patient’s only risk factor was oral contraceptive use. Clinical presentation depends on the rapidity of onset and can range from fulminant hepatic failure to nearly asymptomatic disease, althoughmost patients have the classic triad of abdominal pain, ascites, and hepatomegaly. Over time,portal hypertension and cirrhosis develop in nearly all untreated patients, and mortality is increased without interventional or surgical treatment.
BCS可以分为原发性和继发性。原发性BCS指病因为血管本身的异常，包括血栓形成、管腔狭窄、膜性狭窄。继发性BCS可以由外部的肿瘤或肿块压迫所致。在亚洲，下腔静脉阻塞伴有或不伴有肝静脉阻塞较为多见；而在西方国家，单纯肝静脉阻塞多见。BCS的高风险因素包括先天遗传和后天性血液高凝状态，本例患者的高危因素为口服避孕药史。
BCS的临床症状根据起病缓急不同，可以呈爆发性肝功能衰竭，也可以没有明显症状。大多数患者可表现为腹痛、腹水、肝肿大的三联征。如果患者没有接受治疗，随时间推移，可以发生门静脉高压、肝硬化等症状。介入治疗或手术治疗可以降低BCS的死亡率。
Dynamic postgadolinium 3D SPGR images typically reveal the most striking findings in patients with BCS, demonstrating thrombosis or stenosis of hepatic veins or the IVC, or both; enlargement of the caudate lobe; and heterogeneous enhancement of the periphery of the liver (nutmeg liver), with more uniform enhancement centrally and particularly in the caudate lobe, which generally has separate venous drainage. Prominent collateral veins may be visualized.
3D SPGR动态增强扫描可以发现BCS患者的病理变化，包括肝静脉或下腔静脉的血栓形成或管腔狭窄，肝尾叶增大，肝脏周边不均匀强化（肉豆蔻样肝），肝脏中央均匀强化、尤其是肝尾叶（有独立的静脉引流）。有时可以看到侧支循环的形成。
BCS is well known for stimulating the development of regenerative nodules or FNH-like lesions that are mildly hyperintense on precontrast T1-weighted images and show intense uniform hyperenhancement in the arterial phase before becoming isointense on portal venous and equilibrium phase images (Figure 2.17.5, from a follow-up examination performed 3 years after initial diagnosis). Development of regenerative nodules is thought to be related to the increased hepatic arterial flow stimulated by elevated portal venous pressures.
众所周知，BCS可以刺激RN或类似FNH病灶的生长，表现为T1WI略高信号，增强扫描动脉期明显强化，门脉期及平衡期呈等信号(Figure 2.17.5是一个随访了三年的病例)。RN的生长被认为与门静脉高压刺激肝动脉供血增加有关。
Several studies have reported an increased risk of HCC in patients with BCS; however, the range of reported HCC prevalence is extremely wide and the true risk uncertain. Nevertheless, routine surveillance of these patients for development of HCC is probably warranted.
很多文献报道了BCS患者发生肝癌的几率增加，但报道中提到肝癌的发病率数据变化范围很大，所以真正的发病几率并不确定。尽管如此，BCS患者仍需要做常规的随访和检查，防止发生HCC。
Surgical treatment for BCS patients includes membrane resection, IVC reconstruction, surgical creation of portosystemic or mesoatrial shunts, and hepatic transplantation. Interventional therapy also has proved successful in many patients and includes angioplasty, stent placement, thrombolysis, and TIPS creation.
BCS的手术治疗方法包括膜切除术、IVC重建、门体分流术、mesoatrial分流术、肝脏移植等。
介入治疗也被证实对很多患者有很好的效果，比如血管成形术、支架植入、溶栓、经颈静脉肝内门体支架分流术（TIPS）。
Fig 2.17.5
Precontrast 3D SPGR image (Figure 2.17.5A) demonstrates 3 hyperintense nodules in the liver. Arterial phase postgadolinium 3D SPGR image (Figure 2.17.5B) following Multihance administration shows intense enhancement of the nodules,which become nearly isointense to liver on the portal venous phase image (Figure 2.17.5C). A 90-minute delayed hepatobiliary phase image (Figure 2.17.5D) reveals contrast retention in the nodules, an appearance consistent with regenerative nodules or FNH. Note also metallic artifact in the portal vein consistent with TIPS stent.
3D SPGR平扫(Figure 2.17.5A) 肝内可见三个高信号结节。肝胆特异对比剂Multihance增强扫描动脉期(Figure 2.17.5B) 可见结节强化，门静脉期(Figure 2.17.5C)呈等信号。90分钟延迟的肝胆相位图像(Figure 2.17.5D)可见结节内造影剂滞留，提示RN或HCC。
另外，门静脉内可见TIPS支架的金属伪影。
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