并发消化道出血的先天性门体分流畸形8例影像学特征病例系列报告

摘要目的：探讨合并消化道出血的先天性门体分流畸形的影像学特征，以提高对该畸形的认识。方法：纳入复旦大学附属儿科医院病历系统中先天性门体分流且病史中有消化道出血症状的病例，采集性别、年龄、临床表现、CTA和MRA影像学资料、外科手术结局。CTA为GE Light speed 64排螺旋CT扫描仪，MRA为Siemens Avanto 1.5 T 扫描仪，提取纳入病例的CTA原始图像，以1.25 mm层厚/0.625 mm层间隔重建，传入后处理工作站进行多平面重建；提取纳入病例的MRA原始图像，传入MR工作站进行后期多平面重建；由2名从事儿科影像诊断10年以上医生独立读片，观察门静脉及其属支的形态及走行，异常门体分流位置、途径，髂静脉、下腔静脉及肠管、肠壁血管分布。结果：2008年3月至2017年1月符合本文病例纳入标准的连续病例8例，男6例，年龄3月至10岁，7例贫血，7例便血，1例呕血。8例均行CTA检查，其中2例同时行MRA检查。截止出院时，6例行手术治疗，2例随访观察。8例先天性门体分流畸形患儿均为肝外型门体分流，2例肝外型Ⅰ型，6例为Ⅱ型。以便血为表现的7例中，髂内静脉、直肠上静脉及结肠静脉扩张扭曲；以呕血为表现的1例，脾静脉和肠系膜上静脉汇合后通过胃冠状静脉汇入左肾静脉，胃冠状静脉显著扩张、扭曲。结论：合并便血的先天性门体分流大部分通过肠系膜下静脉与髂静脉异常沟通，发生便血的比例高。

Imaging features of 8 cases of congenital portosystemic shunt with gastrointestinal hemorrhage: Case series report

AbstractObjective：To describe the imaging features of congenital portosystemic shunt presenting in 8 children with gastrointestinal bleeding.Methods：This retrospective, single center study was approved by our institutional review board. We searched the cases of congenital portosystemic shunt with history of gastrointestinal bleeding in hospital information system of Children's Hospital of Fudan University. All patients' medical records including sex, age, clinical manifestations, CTA and MRA imaging data, and surgical outcome were reviewed. The raw CT data were reconstructed into 1.25 mm slice thickness and 0.625 mm slice interval, and then were sent into a CT workstation for the post processing. Also, the raw MR date were sent into an MR workstation for post processing. After the data were post processed, all of the images were sent to the Picture Archiving and Communication System and were reviewed by 2 radiologists with more than 10 years experience in pediatric abdominal CT and MR imaging; both radiologists were blinded to the clinical data and identified the main portal vein, intrahepatic portal vein, internal iliac vein and superior rectal vein, as well as the portosystemic shunts. A consensus interpretation of image findings between them was accepted.Results：A total of 8 consecutive cases with the inclusion criteria were included in this study from March 2008 to January 2017. 6 were male, 2 were female, age was ranged from 3 months to10 years. 7 patients presented with lower gastrointestinal bleeding, 1 with hematochezia, 7 patients with anemia. All patients underwent CTA examination, 2 of them underwent MRA examination at the same time. By the time of discharge, 6 cases received surgical treatment, and 2 cases were followed up. All of the 8 cases were congenital extrahepatic portosystemic shunt, 6 were type Abernethy Ⅱ and 2 were type Abernethy Ⅰ. The images of the seven patients with hematochezia showed that the shunts were from the splenomesenteric junction (n=6) or splenic vein (n=1) to the internal iliac vein via an inferior mesenteric vein .One patient with hematemesis showed the shunt from the splenomesenteric junction to the left renal vein via the venae coronaria ventriculi.Conclusion：Most of congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was not common in congenital portosystemic shunt, but the prevalence of gastrointestinal bleeding in patients with this type was high.