Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient
Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient
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DOI：
10.1055/s-0035-1566097
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发表时间：
2016-02-01
影响因子：
1.8
通讯作者：
Lopez Santamaria, Manuel
中科院分区：
文献类型：
--
作者：
Chocarro, Gloria;Virginia Amesty, Maria;Lopez Santamaria, Manuel
引言先天性门体分流（CPSS）是一种罕见的疾病，治疗问题不存在不足。本文的目的是显示我们的经验，在异质性的这种condition.Material和MethodsA回顾性研究25 CPSS在1995年至2014年期间进行。描述的形态，临床影响，和treatment given.ResultsAccordingly的成像技术（IT），分流明显肝内14例，肝外10例，混合1例。14例显示肝门循环。在进行放射学检查的全部分流中，侵入性放射学技术能够显示肝内门静脉。在其他患者中，由于无症状，因此未对其进行调查。一名儿童在出生时出现多器官衰竭伴暴发性肝功能衰竭。分流管在放射学上闭合，临床损害迅速逆转。他现在无症状，超声扫描控制中不再有CPSS图像。此外，7名儿童目前没有症状，并定期监测。7名儿童进行了产前诊断，在5个分流关闭自发。9名儿童在其进展过程中出现症状（高氨血症、再生结节、胆汁淤积、胃肠道出血）。其中，我们对5例患者进行了球囊试验闭塞，所有患者均可耐受，随后进行放射学闭合。在我们的经验中，介入放射学技术的进步，避免了手术关闭shunt.ConclusionsMorphologically，CPSS是非常异质性的，建立了多种可能的连接。CPSS有多种临床表现，从无症状患者到急性肝功能衰竭。治疗方法应该是个体化的，因此应该在过度专业化的中心进行。
IntroductionCongenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition.Material and MethodsA retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given.ResultsAccording to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt.ConclusionsMorphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers.