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目的探讨Abernethy畸形的CT和MRI表现,以提高对本病的认识。方法符合诊断标准的Abernethy畸形6例,其中3例行CT平扫及增强检查,3例行MRI平扫及增强检查。全部病例行最大密度投影(MIP)和/或容积再现(VR)后处理。检索1990年~2013年Pub Med上的英文文献,收集具有完整CT和/或MR资料的Abernethy畸形60例,一并纳入分析。结果本组6例均为Ⅱ型Abernethy畸形。文献60例中37例为Ⅰ型,23例为Ⅱ型。29例Ⅱ型Abernethy畸形中,门腔分流部位:脾静脉与肾静脉直接或间接交通15例(51.72%),肠系膜上静脉与下腔静脉直接或间接交通5例(17.24%),门静脉与下腔静脉单支直接沟通5例(17.24%),门静脉与下腔静脉多支沟通1例(3.45%);另外3例表现为门静脉与下腔静脉系统的单支沟通,分别为肠系膜上静脉与右侧精索静脉2例(6.90%)、肠系膜上静脉与左髂总静脉1例(3.45%)。Ⅰ型Abernethy畸形中,Ⅰa型20例,Ⅰb型17例,均表现为门静脉干血完全向腔静脉分流而不回流到肝脏。66例Abernethy畸形中,17例合并肝内结节(本组3例,文献14例),其中12例单发,5例多发;其中15例为良性结节(88.24%),2例为恶性结节(11.76%)。28例伴发其他器官畸形,其中27例为Ⅰ型患者。结论 CT、MRI可准确对Abernethy畸形进行分型诊断并确定肝外门腔静脉分流的部位。Ⅰ型患者易合并其他脏器畸形;Abernethy畸形患者肝脏结节多为良性结节,少数可转变为恶性结节。
Objective To investigate the CT and MRI findings of Abernethy deformity to improve the understanding of this disease. Methods Abernethy deformity met the diagnostic criteria in 6 cases, of which 3 cases underwent plain and enhanced CT examination and 3 cases underwent plain MRI and enhanced examination. All cases were maximal density projection (MIP) and / or volumetric rendering (VR) post-processing. English literature from Pub Med from 1990 to 2013 was retrieved and 60 Abernethy deformities with complete CT and / or MR data were collected and included in the analysis. Results The group of 6 cases were type Ⅱ Abernethy deformity. Among the 60 cases, 37 cases were type Ⅰ and 23 cases were type Ⅱ. In 29 Abernethy malformations of type Ⅱ, there were 15 cases (51.72%) with direct or indirect splenic and renal vein traffic, 5 cases (17.24%) with direct or indirect superior mesenteric vein and inferior vena cava communicating with portal vein There were 5 cases (17.24%) directly communicating with the inferior vena cava and 1 case (3.45%) communicating with the inferior vena cava. The other 3 cases showed the single branch communicating with the inferior vena cava and the superior mesenteric vein Right spermatic vein in 2 cases (6.90%), superior mesenteric vein and left common iliac vein in 1 case (3.45%). Type Ⅰ Abernethy deformity, type Ⅰ a 20 cases, type Ⅰ b 17 cases were manifested as portal venous dry shunt completely to the vena cava but not to the liver. In 66 Abernethy deformities, 17 had intrahepatic nodules (3 in this group, 14 in literature), of which 12 were single and 5 were multiple. Among them, 15 were benign nodules (88.24%) and 2 were malignant Nodules (11.76%). 28 cases associated with other organ deformities, of which 27 cases were type Ⅰ patients. Conclusion CT and MRI can accurately diagnose Abernethy’s deformity and determine the location of extrahepatic portal venous shunt. Type Ⅰ patients with other organ deformities easily combined; Abernethy abnormal liver nodules mostly benign nodules, a few can be transformed into malignant nodules.