数字减影血管造影对儿童Abernethy畸形的诊断

Value of digital subtraction angiography in the diagnosis of Abernethy malformation in children

目的探讨儿童Abernethy畸形的DSA及CT表现并探讨其诊断价值。方法对我院共11例Abernethy畸形的影像学检查结果进行回顾性分析,所有病例均行DSA及CT检查,CT检查均行平扫及增强多期扫描,并行多平面重组(MPR)、最大密度投影(MIP)及容积再现(VR)后处理。结果本组病例中,3例为AbernethyⅠb型,8例为AbernethyⅡ型,其中CT结果误诊2例(均为Ⅱ型误诊为Ⅰb型)。门体分流部位:门静脉与下腔静脉交通9例,脾静脉与右髂总静脉交通1例,脾静脉与左髂总静脉交通1例。本组病例其他主要临床资料包括先天性心脏病、肺动脉高压、弥漫性肺动静脉瘘、肝功能异常、肝内结节、血氨增高、肝性脑病等。结论多层螺旋CT可对Abernethy畸形进行诊断并明确分流部位,但DSA对于儿童Abernethy畸形诊断、分型及指导治疗仍具有不可取代的作用。

Objective To study the imaging manifestations and value in the diagnosis of Abernethy malformation in children.Methods Imaging findings of 11 children with Abernethy malformation were retrospectively analyzed.All patients had digital subtraction angiography and contrast-enhanced CT.Results 6 patients with type Ⅱ and 3 children with type Ⅰ b Abernethy malformation were correctly diagnosed on CT.2 with type Ⅱ were misdiagnosed as type Ⅰ b.Among the 11 patients,9 presented with single vessel shunt between portal vein and IVC,1 with shunt between splenic vein and right iliac vein,1 with shunt between splenic vein and left iliac vein.Other clinical findings included congenital heart disease,pulmonary hypertension,diffuse pulmonary arteriovenous fistula,abnormal liver function,hepatic nodules,elevated blood ammonia,and hepatic encephalopathy.Conclusion CT can demonstrate Abernethy malformation and the exact location of the portacaval shunt.DSA still plays an irreplaceable role in diagnosing and classifying the Abernethy malformation in children to guide treatment planning.