Exploiting Phenotypic Plasticity for the Treatment of Hepatopulmonary Shunting in Abernethy Malformation
Exploiting Phenotypic Plasticity for the Treatment of Hepatopulmonary Shunting in Abernethy Malformation
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DOI：
10.1016/j.jvir.2010.01.038
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发表时间：
2010-06-01
影响因子：
2.9
通讯作者：
Finch, Michael
中科院分区：
文献类型：
--
作者：
Kuo, Michael D.;Miller, Franklin J.;Finch, Michael
一名11岁男孩因慢性低氧血症出现运动不耐受。检查后诊断为继发于先天性肝外门静脉分流（阿伯内西畸形）的肝肺综合征（HPS）。利用发育中肝脏的可塑性作为治疗HPS的一种策略。通过采用分期血管内介入方法，对门体血管回路进行了改造，以促进严重发育不良和未发育的肝内门静脉系统逐渐生长和发育。在最后一次手术完成后，患者的肝内门静脉外观正常；2个月后，HPS的体征和症状完全消失。2年后患者仍无肝肺综合征的痕迹。
An 11-year-old boy presented with exercise intolerance due to chronic hypoxemia. Work-up revealed a diagnosis of hepatopulmonary syndrome (HPS) secondary to a congenital extrahepatic portal-venous shunt (Abernethy malformation). Plasticity in the developing liver was exploited as a strategy for the treatment of HPS. With use of a staged endovascular approach, the portosystemic vascular circuitry was modified in a manner that facilitated progressive growth and development of the severely hypoplastic and underdeveloped intrahepatic portal venous system. After completion of the final procedure, the patient's intrahepatic portal veins were normal in appearance; 2 months later, signs and symptoms of HPS completely resolved. The patient remains free of UPS stigmata after 2 years.