14例Abernethy畸形患者多层螺旋CT及MRI影像学表现分析

Analysis of multislice helical CT and MRI imaging findings in 14 patients with Abernethy malformations

目的分析Abernethy畸形患者多层螺旋CT(multislice helical CT,MSCT)及MRI影像资料,以提高对该类疾病的认识。方法选取2015年1月至2022年1月中山大学附属第一医院及韶州人民医院符合诊断标准的l4例具有完整MSCT或MRI影像资料的Abernethy畸形患者作为研究对象,分析其影像学表现。结果14例患者中,4例Ⅰ型Abernethy畸形患者影像学表现为肝门静脉缺如,1例Ⅰa型门静脉缺如脾静脉-左肾静脉,肠系膜上静脉-腔静脉,3例Ⅰb型Abernethy畸形患者中,肠系膜上静脉及脾静脉-左肾静脉2例,门脉缺如,肠系膜上静脉及脾静脉-腔静脉;肝动脉增粗1例;10例Ⅱ型Abernethy畸形患者影像学特点为门静脉主干或分支细小,部分分支缺如,其中肠系膜上静脉及脾静脉-左肾静脉7例,肠系膜上静脉及脾静脉汇入肠系膜下静脉再分支并与双侧髂内静脉沟通1例,门脉右支-腔静脉2例。结论Abernethy畸形是较罕见的先天性疾病,MSCT及MRI增强扫描是诊断本病的主要影像学手段,对疾病的分型及确定门体分流途径起到重要作用,提高对该病的认识是实现准确诊断的关键。

Objective To analyze the multislice helical CT(MSCT)and MRI imaging data of Abernethy malformation to improve the understanding of this diseases.Methods 14 patients with Abernethy malformation with complete MSCT or MRI imaging data who met the diagnostic criteria in the First Affiliated Hospital of Sun Yat-sen University and Shaozhou People's Hospital from January 2015 to January 2022 were selected as the research subjects,and their imaging findings were analyzed.Results Of 14 patients,the imaging findings of 4 patients with typeⅠAbernethy malformation were absence of hepatic portal vein,1 patient with typeⅠa portal vein absence of splenic vein-left renal vein,superior mesenteric veincaval vein,among the 3 patients with typeⅠb Abernethy malformation,there were 2 cases of superior mesenteric vein and splenic vein-left renal vein,absence of portal vein,superior mesenteric vein and splenic vein-caval vein;hepatic artery thickening in 1 case;the imaging features of 10 patients with typeⅡAbernethy malformation were small main or branch of portal vein and absence of some branches,including 7 cases of superior mesenteric vein and splenic vein-left renal vein,and 1 case of superior mesenteric vein and splenic vein merging into inferior mesenteric vein and 2 cases of communicating with bilateral internal iliac vein;right portal vein-caval vein.Conclusion Abernethy malformation is a rare congenital disease,and MSCT and MRI scan are the main imaging methods for the diagnosis of the disease,which plays an important role in the classification and phytoum shunt pathway,improving the understanding of the disease is the key to achieve accurate diagnosis.