Ye Qiao-Bo, Huang Qin-Feng, Luo Yao-Chang, Wen Yi-Lei, Chen Zi-Kun, Wei Ai-Ling
Basic Medical College, Chengdu University of Traditional Chinese Medicine, Chengdu 611137, Sichuan Province, China.
Department of Oncology, The First Affiliated Hospital, Guangxi University of Chinese Medicine, Nanning 530023, Guangxi Zhuang Autonomous Region, China.
World J Clin Cases. 2021 Apr 26;9(12):2937-2943. doi: 10.12998/wjcc.v9.i12.2937.
Budd-Chiari syndrome (BCS) is a rare heterogeneous liver disease characterized by obstruction of the hepatic venous outflow tract. The incidence of BCS is so low that it is difficult to detect in general practice and difficult to include within the scope of routine diagnosis. The clinical manifestations of BCS are not specific; hence, BCS tends to be misdiagnosed.
We report the case of a 33-year-old Chinese woman who presented with progressive distension in the upper abdomen. She was initially misdiagnosed with liver cirrhosis (LC) due to abnormalities on an upper abdominal computed tomography scan. Although she was taking standard anti-cirrhosis therapy, her symptoms did not improve. Magnetic resonance imaging showed caudate lobe hypertrophy; and dilated lumbar and hemiazygos veins. Venography revealed membranous obstruction of the inferior vena cava owing to congenital vascular malformation. A definitive diagnosis of BCS was made. Balloon angioplasty was performed to recanalize the obstructed inferior vena cava and the patient's symptoms were completely resolved.
BCS lacks specific clinical features and can eventually lead to LC. Clinicians and radiologists must carefully differentiate BCS from LC. Correct diagnosis and timely treatment are vital to the patient's health.
布加综合征(BCS)是一种罕见的异质性肝脏疾病,其特征为肝静脉流出道梗阻。BCS的发病率极低,在普通医疗实践中难以发现,也难以纳入常规诊断范围。BCS的临床表现不具有特异性;因此,BCS容易被误诊。
我们报告了一名33岁中国女性的病例,她出现进行性上腹部膨隆。由于上腹部计算机断层扫描异常,她最初被误诊为肝硬化(LC)。尽管她接受了标准的抗肝硬化治疗,但其症状并未改善。磁共振成像显示尾状叶肥大;以及腰静脉和半奇静脉扩张。静脉造影显示由于先天性血管畸形导致下腔静脉膜性梗阻。最终确诊为BCS。进行了球囊血管成形术以再通梗阻的下腔静脉,患者症状完全缓解。
BCS缺乏特异性临床特征,最终可导致LC。临床医生和放射科医生必须仔细鉴别BCS和LC。正确诊断和及时治疗对患者健康至关重要。