| 王驰,闫淯淳,王子含,李三林,郭向峰,李嘉雯,申刚.儿童先天性肝内门体分流临床及影像学表现[J].中国介入影像与治疗学,2026,23(7):399-402 |
| 儿童先天性肝内门体分流临床及影像学表现 |
| Clinical and imaging manifestations of congenital intrahepatic portosystemic shunt in children |
| 投稿时间：2026-06-13 修订日期：2026-07-01 |
| DOI：10.13929/j.issn.1672-8475.2026.07.004 |
| 中文关键词: 儿童 自发性门体分流 体层摄影术，X线计算机 血管造影术，数字减影 |
| 英文关键词:child spontaneous portosystemic shunt tomography, X-ray computed angiography, digital subtraction |
| 基金项目:首都儿科研究所及首都儿童医学中心青年基金（QN-2026-23）。 |
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| 中文摘要: |
| 目的 观察儿童先天性肝内门体分流（CIPSS）的临床及影像学表现。方法 回顾性选取24例CIPSS患儿，包括合并肝纤维化4例、肝脏局灶性结节性增生（FNH）及肺部动静脉瘘各2例、肝肺综合征1例；其中21例接受介入治疗、2例接受外科结扎手术、1例未接受任何治疗。观察CIPSSCT及数字减影血管造影（DSA）间接门静脉造影表现，评估Park分型、测量分流道宽度；比较治疗前、后谷丙转氨酶（GPT）、谷草转氨酶（GOT）、血氨及胆汁酸水平，分析分流道宽度与血氨及胆汁酸水平的相关性。结果 24例CIPSS中，Park Ⅰ型10例、Ⅱ型3例、Ⅲ型4例、Ⅳ型3例、V型2例，余2例存在畸形血管团而分型不明；其中21例的分流道宽度为4.8～18.1 mm、平均（10.67±3.31）mm，3例Ⅳ型存在多发分流道而未测量其宽度。24例基线胆汁酸均升高，其中20例GPT升高、4例GOT升高、13例血氨升高；治疗后15例接受随访者血氨及胆汁酸水平均显著下降（P均＜0.05）。CIPSS分流道宽度与血氨（r=0.067，P=0.672）及胆汁酸水平（r=0.293，P=0.065）均无明显相关。结论 儿童CIPSS临床及影像学表现均具有一定特征性，可合并肝纤维化、FNH、肺部动静脉瘘及肝肺综合征。 |
| 英文摘要: |
| Objective To observe the clinical and imaging manifestations of congenital intrahepatic portosystemic shunt （CIPSS） in children. Methods Totally 24 CIPSS children were retrospectively enrolled， including 4 cases complicated with liver fibrosis， 2 cases complicated with focal nodular hyperplasia of the liver （FNH）， 2 cases complicated with pulmonary arteriovenous fistulas and 1 case complicated with hepatopulmonary syndrome. Among 24 cases， 21 cases underwent interventional embolization therapy， 2 cases underwent surgical ligation and 1 case received no treatment. CT and digital subtraction angiography （DSA） indirect portal vein angiography findings were observed， Park classification of CIPSS was assessed， and shunt tract width was measured. The glutamic-pyruvic transaminase （GPT）， glutamic-oxaloacetic transaminase （GOT）， blood ammonia and bile acids were compared before and after treatment.The correlations of shunt tract width with blood ammonia and bile acid levels were observed. Results Among 24 cases of CIPSS， Park Ⅰwas detected in 10 cases， Park Ⅱ in 3 cases， Park Ⅲ in 4 cases ， Park Ⅳ in 3 cases and Park Ⅴ in 2 cases， while unclassified type was detected in the rest 2 cases with malformed vascular clusters. The shunt tract widths ranged from 4.8 to 18.1 mm， with an average of （10.67±3.31）mm in 21 cases， which was not measured in 3 cases of Park Ⅳwith multiple shunt channels. Baseline elevated bile acids were found in all 24 cases， with elevated GPT in 20 cases， elevated GOT in 4 cases and elevated blood ammonia in 13 cases. After treatment， blood ammonia and bile acid levels of 15 cases who underwent follow-up significantly decreased （both P＜0.05）. No significant correlation was found between CIPSS shunt tract width with blood ammonia （r=0.067， P=0.672） nor bile acid levels （r=0.293， P=0.065）. Conclusion Clinical and imaging manifestations of CIPSS in children had certain characteristics， which might complicate with liver fibrosis， FNH， pulmonary arteriovenous fistula and hepatopulmonary syndrome. |
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