Abernethy畸形的CT和MR诊断

摘要

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PubMed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 analyze the clinical and imaging features of Abernethy malformation.Methods 6 patients with confirmed diagnosis of type II Abernethy malformation in our hospital and 60 patients with type I(37)and type II(23)Abernethy malformation and CT and/or MRI reported in the literature through Pubmed search were evaluated.Results Among the 29 patients with type II Abernethy malformation,15 presented with splenorenal shunt(51.72%),5 with shunt between inferior vena cava(IVC) and superior mesenteric vein and(17.24%),5 with single vessel shunt between portal vein and IVC(17.24%),1 with multi-vessel shunt between portal vein and IVC(3.45%),2 with shunt between superior mesenteric vein and right spermatic vein,and 1 with shunt between superior mesenteric vein and left common iliac vein.20 patients with type Ia and 17 patients with type Ib presented with reversed blood flow in the portal vein.Of the 66 patients,15 had benign liver nodules(88.24%)and 2 had malignant liver nodules (11.76%).Of 28 patients with other organ malformations,27 were type I.Conclusion CT and MRI can distinguish the types of Abernethy malformation and show the exact location of the portacaval shunt.Type I patients are more likely to have other organ malformations.Hepatic nodules tend to be benign.