高龄先天性肝内门体分流合并肝性脑病1例报告

张俊君; 高欣; 吴莹; 程翌; 徐维田

doi:10.3969/j.issn.1001-5256.2021.06.040

高龄先天性肝内门体分流合并肝性脑病1例报告

DOI: 10.3969/j.issn.1001-5256.2021.06.040

中部战区总医院消化内科，武汉 430070

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：张俊君负责课题设计，资料分析，撰写论文；高欣、吴莹、程翌参与收集数据，修改论文；徐维田负责拟定写作思路，指导撰写文章并最后定稿。

详细信息

作者简介:
张俊君(1985—)，女，主治医师，主要从事胃肠动力以及肝病学研究

通信作者:
徐维田，xuwt0219@163.com

中图分类号: R575
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- 文章访问数: 459
- HTML全文浏览量: 123
- PDF下载量: 37
- 被引次数: 0
出版历程
- 收稿日期: 2020-10-24
- 录用日期: 2021-01-29
- 出版日期: 2021-06-20

A case of congenital intrahepatic portosystemic venous shunt with hepatic encephalopathy in the elderly

Department of Gastroenterology, General Hospital of Central Theater Command, Wuhan 430070, China

摘要

- 肝性脑病 /
- 先天性肝内门体分流 /
- 老年人, 80以上
- Hepatic Encephalopathy /
- Congenital Intrahepatic Portosystemic Venous Shunt /
- Aged, 80 and over

HTML全文

图 1 患者头颅MRI+DWI+MRA结果

注：a，冠状位；b，横状位；c，矢状位；d，MRA。

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图 2 动态脑电图结果

注：中重度异常脑电图(全部导联可见稍多散发中波幅1.5~ 2.5 Hz三相波，背景波为30~60 μV、4~5 Hz的波)。

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图 3 腹部增强CT及增强MRI结果

注：a/e，动脉期，肝左叶迂曲扩张血管团相对肝实质呈低密度影/低信号影；b/f，门静脉期，门静脉左支与肝左叶迂曲扩张血管团强化程度一致；c/g，门静脉期，门静脉左支汇入肝左叶迂曲扩张静脉团；d/h，门静脉期，肝左静脉由肝左叶迂曲扩张静脉团引出，二者强化程度一致，且扭曲增粗。

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参考文献(21)

[1]	STRINGER MD. The clinical anatomy of congenital portosystemic venous shunts[J]. Clin Anat, 2008, 21(2): 147-157. DOI: 10.1002/ca.20574.
[2]	OHNISHI Y, UEDA M, DOIH, et al. Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess[J]. J Pediatr Surg, 2005, 40(5): e1-e3. DOI: 10.1016/j.jpedsurg.2005.02.011.
[3]	HOU YL, ZHENG B, YAN JY, et al. Analysis of color Doppler ultrasonographic findings of congenital intrahepatic portosystemic venous shunt[J]. Chin Imag J Integr Tradit Western Med, 2019, 17(6): 650-652. DOI: 10.3969/j.issn.1672-0512.2019.06.033 霍亚玲, 郑彬, 闫加勇, 等. 先天性肝内门-体静脉分流的彩色多普勒超声表现分析[J]. 中国中西医结合影像学杂志, 2019, 17(6): 650-652. DOI: 10.3969/j.issn.1672-0512.2019.06.033
[4]	PARK JH, CHA SH, HAN JK, et al. Intrahepatic portosystemic venous shunt[J]. AJR Am J Roentgenol, 1990, 155(3): 527-528. DOI: 10.2214/ajr.155.3.2117349.
[5]	ZHU LL, LYU ZB. Diagnosis and treatment of common portal vein malformations in children[J]. Chin J Pediatr Surg, 2010, 31(8): 627-629. DOI: 10.3760/cma.j.issn.0253-3006. 2010.08.016. 朱琳琳, 吕志葆. 常见儿童门静脉畸形的诊治现状[J]. 中华小儿外科杂, 2010, 31(8): 627-629. DOI: 10.3760/cma.j.issn.0253-3006.2010.08.016.
[6]	LIN ZY, CHEN SC, HSIEH MY, et al. Incidence and clinical significance of spontaneous intrahepatic portosystemic venous shunts detected by sonography in adults without potential cause[J]. J Clin Ultrasound, 2006, 34(1): 22-26. DOI: 10.1002/jcu.20176.
[7]	TAKAHASHI S, YOSHIDA E, SAKANISHI Y, et al. Congenital multiple intrahepatic portosystemic shunt: An autopsy case[J]. Int J Clin Exp Pathol, 2014, 7(1): 425-431.
[8]	BERNARD O, FRANCHI-ABELLA S, BRANCHEREAU S, et al. Congenital portosystemic shunts in children: Recognition, evaluation, and management[J]. Semin Liver Dis, 2012, 32(4): 273-287. DOI: 10.1055/s-0032-1329896.
[9]	FRANCHI-ABELLA S, BRANCHEREAU S, LAMBERT V, et al. Complications of congenital portosystemic shunts in children: Therapeutic options and outcomes[J]. J Pediatr Gastroenterol Nutr, 2010, 51(3): 322-330. DOI: 10.1097/MPG.0b013e3181d9cb92.
[10]	TAKENAGA S, NARITA K, MATSUI Y, et al. Hepatic encephalopathy due to congenital multiple intrahepatic portosystemic venous shunts successfully treated by percutaneous transhepatic obliteration[J]. Case Rep Gastroenterol, 2016, 10(3): 701-705. DOI: 10.1159/000452204.
[11]	ZANG GL, HUANG PT, XU WY, et al. Color doppler ultrasound and contrast-enhanced ultrasound model in congenital intrahepatic portosystemic venous shunt[J]. Chin J Ultrasonogr, 2012, 21(12): 1043-1047. DOI: 10.3760/cma.j.issn.1004-4477.2012.12.011. 臧国礼, 黄品同, 许伟莹, 等. 先天性肝内门体分流的彩色多普勒超声和超声造影模式研究[J]. 中华超声影像学杂志, 2012, 21(12): 1043-1047. DOI: 10.3760/cma.j.issn.1004-4477. 2012.12. 011.
[12]	ZANG GL, HUANG PT, ZHOU WP. Routine ultrasound and contrast-enhanced ultrasound findings of congenital hepatic portal venous fistula[J]. Chin J Ultrasonogr, 2010, 19(9): 821-823. DOI: 10.3760/cma.j.issn.1004-4477.2010.09.032. 臧国礼, 黄品同, 周维平. 先天性肝静脉门静脉瘘的常规超声及超声造影表现[J]. 中华超声影像学杂志, 2010, 19(9): 821-823. DOI: 10.3760/cma.j.issn.1004-4477.2010.09.032.
[13]	PLESTED MJ, ZWINGENBERGER AL, BROCKMAN DJ, et al. Canine intrahepatic portosystemic shunt insertion into the systemic circulation is commonly through primary hepatic veins as assessed with CT angiography[J]. Vet Radiol Ultrasound, 2020, 61(5): 519-530. DOI: 10.1111/vru.12892.
[14]	YI LL, DONG Z, CAI HS, et al. Clinical application of MSCTA in diagnosing congenital intrahepatic portosystemic venous shunts[J]. J Clin Radiology. 2015, 34(1): 79-83. DOI: 10.13437/j.cnki.jcr.2015.01.025. 衣利磊, 董帜, 蔡华崧, 等. MSCTA诊断先天性肝内门-体静脉分流[J]. 临床放射学杂志, 2015, 34(1): 79-83. DOI: 10.13437/j.cnki.jcr.2015.01.025.
[15]	GU XH, SHAO H, SUN AM, et al. Application value of DSA in the clinical classification and treatment of children's congenital extrahepatic portosystemic shunts[J]. China Med Devic, 2017, 32(7): 60-63. DOI: 10.3969/j.issn.1674-1633.2017.07.017. 顾小红, 邵虹, 孙爱敏, 等. DSA在儿童先天性肝外门体静脉分流的临床分型及治疗中的应用价值[J]. 中国医疗设备, 2017, 32(7): 60-63. DOI: 10.3969/j.issn.1674-1633.2017.07.017.
[16]	PAPAMICHAIL M, PIZANIAS M, HEATON N. Congenital portosystemic venous shunt[J]. Eur J Pediatr, 2018, 177(3): 285-294. DOI: 10.1007/s00431-017-3058-x.
[17]	LI XF, WU QQ, WANG L, et al. Ultrasound diagnosis of congenital intrahepatic portosystemic shuns in fetal stage and postpartum: Case report[J]. Chin J Perinat Med, 2016, 19(11): 833-835. DOI: 10.3760/cma.j.issn.1007-9408.2016.11.009. 李晓菲, 吴青青, 王莉, 等. 先天性肝内门体静脉分流胎儿期及产后超声诊断1例[J]. 中华围产医学杂志, 2016, 19(11): 833-835. DOI: 10.3760/cma.j.issn.1007-9408.2016.11.009.
[18]	LAUTZ TB, TANTEMSAPYA N, ROWELL E, et al. Management and classification of type Ⅱ congenital portosystemic shunts[J]. J Pediatr Surg, 2011, 46(2): 308-314. DOI: 10.1016/j.jpedsurg.2010.11.009.
[19]	BRADER RA, KIM KR. Transhepatic embolization of a congenital intrahepatic portosystemic shunt for the treatment of hepatic encephalopathy in a noncirrhotic patient using Amplatzer vascular plug device[J]. Radiol Case Rep, 2017, 12(2): 318-322. DOI: 10.1016/j.radcr.2016.12.006.
[20]	Chinese Society of Hepatology, Chinese Medical Association. Guidelines on the management of hepatic encephalopathy in cirrhosis[J]. J Clin Hepatol, 2018, 34(10): 2076-2089. DOI: 10.3969/j.issn.1001-5256.2018.10.007. 中华医学会肝病学分会. 肝硬化肝性脑病诊疗指南[J]. 临床肝胆病杂志, 2018, 34(10): 2076-2089. DOI: 10.3969/j.issn.1001-5256.2018.10.007.
[21]	LI XK, WANG S, LI ZG, et al. Updated key points of Guidelines for the diagnosis and management of hepatic encephalopathy in cirrhosis(2018)[J]. J Clin Hepatol, 2019, 35(7): 1485-1488. DOI: 10.3969/j.issn.1001-5256.2019.07.012. 李小科, 王姗, 李志国, 等. 2018年《肝硬化肝性脑病诊疗指南》更新要点解读[J]. 临床肝胆病杂志, 2019, 35(7): 1485-1488. DOI: 10.3969/j.issn.1001-5256.2019.07.012.