PDF(Pubmed)
Abstract：
UNASSIGNED: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic portal veinthrombosis as the portal vein may not be visualized in either entities.
METHODS: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences.
UNASSIGNED: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS.
CONCLUSIONS: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis.
METHODS: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences.
UNASSIGNED: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS.
CONCLUSIONS: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis.
摘要：
■先天性肝外门体分流术（CEPS）是一种罕见的血管畸形，其中内脏和门静脉血液被分流到肝脏的体循环中。1型CEPS有时很难与慢性门静脉血栓形成等病理区分开，因为门静脉可能无法在任何一个实体中可视化。
方法：一名3岁男性儿童因腹痛一周被诊断为慢性门静脉血栓形成。在我们的机构进行了重复的腹部超声检查，我们能够观察到门静脉终止到肾上肝下下腔静脉的端到侧模式和肝脏第八段的局灶性低回声肝病变。没有海绵状变性或门静脉高压症的迹象的证据。随后，三相计算机断层扫描显示了类似的发现，门静脉终止于肾上下腔静脉。局灶性肝脏病变在动脉期显示出外周对比增强，并表现为中央非增强区域，随后的序列均有增强的证据。
■1型CEPS很容易与慢性门静脉血栓形成混淆，因为门静脉可能不可见，肝动脉在两个实体中均显示代偿性扩大。然而,门静脉血栓形成通常与潜在的诱发因素有关,并可导致门静脉高压症和海绵体转化的继发性征象的发展,这对于将门静脉血栓与CEPS区分开来至关重要.
结论：慢性门静脉血栓形成是一种很好的模拟物，应与超声检查的CEPS相区别。用彩色流进行细致的扫描有助于仔细检查血管解剖结构，确定与CEPS相关的发现，如肝脏病变，并排除慢性门静脉血栓形成的征象。
方法：一名3岁男性儿童因腹痛一周被诊断为慢性门静脉血栓形成。在我们的机构进行了重复的腹部超声检查，我们能够观察到门静脉终止到肾上肝下下腔静脉的端到侧模式和肝脏第八段的局灶性低回声肝病变。没有海绵状变性或门静脉高压症的迹象的证据。随后，三相计算机断层扫描显示了类似的发现，门静脉终止于肾上下腔静脉。局灶性肝脏病变在动脉期显示出外周对比增强，并表现为中央非增强区域，随后的序列均有增强的证据。
■1型CEPS很容易与慢性门静脉血栓形成混淆，因为门静脉可能不可见，肝动脉在两个实体中均显示代偿性扩大。然而,门静脉血栓形成通常与潜在的诱发因素有关,并可导致门静脉高压症和海绵体转化的继发性征象的发展,这对于将门静脉血栓与CEPS区分开来至关重要.
结论：慢性门静脉血栓形成是一种很好的模拟物，应与超声检查的CEPS相区别。用彩色流进行细致的扫描有助于仔细检查血管解剖结构，确定与CEPS相关的发现，如肝脏病变，并排除慢性门静脉血栓形成的征象。
