Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Pediatr. 2009 Oct;76(10):1059-60. doi: 10.1007/s12098-009-0202-2. Epub 2009 Nov 12.
Congenital intrahepatic portosystemic venous shunt (IHPSVS) is rare vascular anomaly. We present one case of a 14-month male child who presented with global developmental delay. Child had high ammonia levels with low glutamine and high bile salts on the previous investigations and had history of neonatal seizures since day 13 of life. On admission, serum ammonia levels were elevated to 112micromol/L. Other laboratory investigations including liver and renal function test, and electrolytes were normal. He was, diagnosed to have IHPSVS on the basis of Doppler and CT, and treated by embolization with n-butyl cyanoacrylate (glue). A brief review of diagnostic modalities and endovascular management for the IHPSVS is presented including the present case.
先天性肝内门体静脉分流（IHPSVS）是一种罕见的血管异常。我们报告了一例 14 个月大的男性患儿，其表现为全面发育迟缓。患儿之前的检查显示血氨水平升高，谷氨酰胺水平低，胆盐水平高，并在出生后第 13 天开始出现新生儿癫痫。入院时，血清氨水平升高至 112μmol/L。其他实验室检查包括肝功能、肾功能和电解质检查均正常。根据多普勒超声和 CT 检查，患儿被诊断为 IHPSVS，并接受了 n-丁基氰基丙烯酸酯（胶水）栓塞治疗。本文介绍了 IHPSVS 的诊断方法和血管内治疗，包括本病例。
