先天性肝外门体分流的研究进展

王莹; 徐声润; 余荻; 杨晋辉; 常江

doi:10.3969/j.issn.1001-5256.2023.02.032

先天性肝外门体分流的研究进展

DOI: 10.3969/j.issn.1001-5256.2023.02.032

昆明医科大学第二附属医院消化内科，昆明 650101

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： 王莹负责查阅文献，撰写文章；徐声润、余荻提供修改意见并参与修改；杨晋辉指导选题及写作思路；常江提供修改意见并最终定稿。

详细信息

通信作者:
常江，cjcjchangjiang@sina.com (ORCID: 0000-0002-8678-3979)

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出版历程
- 收稿日期: 2022-06-29
- 录用日期: 2022-07-30
- 出版日期: 2023-02-20

Research advances in congenital extrahepatic portosystemic shunt

Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, Kunming 650101, China

More Information

Corresponding author: CHANG Jiang, cjcjchangjiang@sina.com (ORCID: 0000-0002-8678-3979)

摘要

摘要: 先天性肝外门体分流(CEPS)也称Abernethy畸形，表现为先天性门静脉与下腔静脉之间存在异常吻合。该病在临床上极为罕见，加之其症状表现多样，易漏诊误诊。本文就CEPS的发生机制、分型特点、临床表现、诊断及治疗等研究进展作一综述，以期提高对CEPS的认识，并为其诊治流程的进一步规范提供参考。
- 先天性肝外门体分流 /
- Abernethy畸形 /
- 诊断 /
- 治疗学
Abstract: Congenital extrahepatic portosystemic shunt (CEPS) is also called Abernethy malformation, with the manifestation of congenital abnormal anastomosis between the portal vein and the inferior vena cava. CEPS is extremely rare in clinical practice and has diverse clinical symptoms, which often leads to missed diagnosis and misdiagnosis. This article reviews the pathogenesis, classification, clinical manifestation, diagnosis, and treatment of CEPS, so as to improve the awareness of this disease and provide a reference for further standardization of its diagnosis and treatment process in the future.
- Congenital Extrahepatic Portosystemic Shunt /
- Abernethy Malformation /
- Diagnosis /
- Therapeutics

HTML全文

图 1 CEPS分型

注：SV，脾静脉；SMV，肠系膜上静脉；IVC，下腔静脉；PV，门静脉。

Figure 1. Classification of CEPS

下载: 全尺寸图片幻灯片

图 2 CEPS诊治流程

Figure 2. Diagnosis and treatment process of CEPS

下载: 全尺寸图片幻灯片

参考文献(42)

[1]	ABERNETHY J. Account of two instances of uncommon formation in the viscera of the human body: From the philosophical transactions of the royal society of London[J]. Med Facts Obs, 1797, 7: 100-108.
[2]	LIN XQ, RAO JY, XIANG YF, et al. Case report: A rare syncope case caused by Abernethy Ⅱ and a review of the literature[J]. Front Cardiovasc Med, 2021, 8: 784739. DOI: 10.3389/fcvm.2021.784739.
[3]	XU Y, JIE LL, GAO X, et al. A case of ultrasound diagnosed fetal congenital portal vein deficiency[J]. Chin J Ultrasonogr, 2016, 25(11): 1007, 1011. DOI: 10.3760/cma.j.issn.1004-4477.2016.11.022. 许燕, 接连利, 高翔, 等. 超声诊断胎儿先天性门静脉缺如1例[J]. 中华超声影像学杂志, 2016, 25(11): 1007, 1011. DOI: 10.3760/cma.j.issn.1004-4477.2016.11.022.
[4]	MENG XZ, KANG T, QIAN W, et al. A case of elderly Abernethy malformations caused by hepatic encephalopathy[J]. Chin J Difficult Comp Cases, 2020, 19(9): 953, 962. DOI: 10.3969/j.issn.1671-6450.2020.09.022. 孟祥柱, 康婷, 钱伟, 等. 老年Abernethy畸形致肝性脑病1例[J]. 疑难病杂志, 2020, 19(9): 953, 962. DOI: 10.3969/j.issn.1671-6450.2020.09.022.
[5]	WHITE JJ, BRENNER H, AVERY ME. Umbilical vein collateral circulation: the caput medusae in a newborn infant[J]. Pediatrics, 1969, 43(3): 391-395.
[6]	LESHEN MA, DEVANAGONDI R, SAUL D, et al. Physiological fetal vascular shunts and failure to regress: what the radiologist needs to know[J]. Pediatr Radiol, 2022, 52(7): 1207-1223. DOI: 10.1007/s00247-022-05302-0.
[7]	LISOVSKY M, KONSTAS AA, MISDRAJI J. Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation[J]. Am J Surg Pathol, 2011, 35(9): 1381-1390. DOI: 10.1097/PAS.0b013e3182230ce4.
[8]	LEMOINE C, NILSEN A, BRANDT K, et al. Liver histopathology in patients with hepatic masses and the Abernethy malformation[J]. J Pediatr Surg, 2019, 54(2): 266-271. DOI: 10.1016/j.jpedsurg.2018.10.083.
[9]	WANG YM, ZHONG L, TAO YH. Comprehensive review of 18 cases with Abernethy malformation in China from 2001 to 2012[J]. J Appl Clin Pediatr, 2013, 28(19): 1478-1482. DOI: 10.3969/cma.j.issn.2095-428X.2013.19.012. 王亚妹, 钟琳, 陶于洪. 2001年至2012年中国18例Abernethy畸形综合报道[J]. 中华实用儿科临床杂志, 2013, 28(19): 1478-1482. DOI: 10.3969/cma.j.issn.2095-428X.2013.19.012.
[10]	KOBAYASHI N, NIWA T, KIRIKOSHI H, et al. Clinical classification of congenital extrahepatic portosystemic shunts[J]. Hepatol Res, 2010, 40(6): 585-593. DOI: 10.1111/j.1872-034X.2010.00667.x.
[11]	MORGAN G, SUPERINA R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies[J]. J Pediatr Surg, 1994, 29(9): 1239-1241. DOI: 10.1016/0022-3468(94)90812-5.
[12]	DIPAOLA F, TROUT AT, WALTHER AE, et al. Congenital portosystemic shunts in children: Associations, complications, and outcomes[J]. Dig Dis Sci, 2020, 65(4): 1239-1251. DOI: 10.1007/s10620-019-05834-w.
[13]	BAIGES A, TURON F, SIMÓN-TALERO M, et al. Congenital extrahepatic portosystemic shunts (Abernethy malformation): An international observational study[J]. Hepatology, 2020, 71(2): 658-669. DOI: 10.1002/hep.30817.
[14]	TANG H, SONG P, WANG Z, et al. A basic understanding of congenital extrahepatic portosystemic shunt: incidence, mechanism, complications, diagnosis, and treatment[J]. Intractable Rare Dis Res, 2020, 9(2): 64-70. DOI: 10.5582/irdr.2020.03005.
[15]	SOOTA K, KLAIR JS, LABRECQUE D. Confusion for fifteen years: A case of Abernethy malformation[J]. Clin Gastroenterol Hepatol, 2018, 16(10): A50. DOI: 10.1016/j.cgh.2018.01.003.
[16]	ALONSO-GAMARRA E, PARRÓN M, PÉREZ A, et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review[J]. Radiographics, 2011, 31(3): 707-722. DOI: 10.1148/rg.313105070.
[17]	ALVI AA, PICHARDO J, GUPTA S, et al. An interesting case of congenital intrahepatic porto-hepatic shunt as a cause of unexplained encephalopathy[J]. Cureus, 2020, 12(4): e7639. DOI: 10.7759/cureus.7639.
[18]	FU L, WANG Q, WU J, et al. Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome[J]. Eur J Pediatr, 2016, 175(2): 195-201. DOI: 10.1007/s00431-015-2623-4.
[19]	ZHAO S, WANG J, HAN B, et al. Liver transplantation for Abernethy malformation complicated with hepatopulmonary syndrome in children: report of one case and literature review[J/OL]. Chin J Hepat Surg (Electronic Edition), 2022, 11(3): 288-293. DOI: 10.3877/cma.j.issn.2095-3232.2022.03.015. 赵帅, 王健, 韩冰, 等. 肝移植治疗儿童Abernethy畸形合并肝肺综合征一例并文献复习[J/OL]. 中华肝脏外科手术学电子杂志, 2022, 11(3): 288-293. DOI: 10.3877/cma.j.issn.2095-3232.2022.03.015.
[20]	LIN KY, CHEN H, YU L. Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report[J]. BMC Cardiovasc Disord, 2019, 19(1): 141. DOI: 10.1186/s12872-019-1124-1.
[21]	KONG Y, ZHANG H, LIU C, et al. Abernethy malformation with multiple aneurysms: incidentally found in an adult woman with Caroli's disease[J]. Ann Hepatol, 2013, 12(2): 327-331. DOI: 10.1016/S1665-2681(19)31373-0
[22]	PEČEK J, FISTER P, HOMAN M. Abernethy syndrome in Slovenian children: Five case reports and review of literature[J]. World J Gastroenterol, 2020, 26(37): 5731-5744. DOI: 10.3748/wjg.v26.i37.5731.
[23]	HE X, ZHU Y, FU H, et al. Case report: Membranoproliferative glomerulonephritis, a rare clinical manifestation of Abernethy malformation type Ⅱ[J]. Front Pediatr, 2021, 9: 647364. DOI: 10.3389/fped.2021.647364.
[24]	FRANCHI-ABELLA S, GONZALES E, ACKERMANN O, et al. Congenital portosystemic shunts: diagnosis and treatment[J]. Abdom Radiol (NY), 2018, 43(8): 2023-2036. DOI: 10.1007/s00261-018-1619-8.
[25]	GUO C, ZHONG YM, WANG Q, et al. Diagnostic accuracy of multi-slice computed tomography in children with Abernethy malformation[J]. BMC Med Imaging, 2019, 19(1): 97. DOI: 10.1186/s12880-019-0396-3.
[26]	BLANC T, GUERIN F, FRANCHI-ABELLA S, et al. Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy[J]. Ann Surg, 2014, 260(1): 188-198. DOI: 10.1097/SLA.0000000000000266.
[27]	SOKOLLIK C, BANDSMA RH, GANA JC, et al. Congenital portosystemic shunt: characterization of a multisystem disease[J]. J Pediatr Gastroenterol Nutr, 2013, 56(6): 675-681. DOI: 10.1097/MPG.0b013e31828b3750.
[28]	UCHIDA H, SAKAMOTO S, KASAHARA M, et al. Longterm outcome of liver transplantation for congenital extrahepatic portosystemic shunt[J]. Liver Transpl, 2021, 27(2): 236-247. DOI: 10.1002/lt.25805.
[29]	ZHANG B, WU KT, LI L, et al. Catheter embolization of type Ⅱ congenital extrahepatic portosystemic shunt with hematochezia: A case series and review of the literature[J]. Cardiovasc Intervent Radiol, 2018, 41(7): 1121-1127. DOI: 10.1007/s00270-018-1972-6.
[30]	ZHANG J, DUAN W, FANG Z, et al. Efficacy and safety of surgical ligation versus endovascular embolization for type Ⅱ congenital extrahepatic portosystemic shunt[J]. Biomed Res Int, 2021, 2021: 9951393. DOI: 10.1155/2021/9951393.
[31]	HARING M, CUPERUS F, DUIKER EW, et al. Scoping review of clinical practice guidelines on the management of benign liver tumours[J]. BMJ Open Gastroenterol, 2021, 8(1): e000592. DOI: 10.1136/bmjgast-2020-000592.
[32]	VIRGILIO E, CAVALLINI M. Managing focal nodular hyperplasia of the liver: Surgery or minimally-invasive approaches? A review of the preferable treatment options[J]. Anticancer Res, 2018, 38(1): 33-36. DOI: 10.21873/anticanres.12188.
[33]	HAPPAERTS S, FOUCAULT A, BILLIARD JS, et al. Combined hepatocellular-cholangiocarcinoma in a patient with Abernethy malformation and tetralogy of Fallot: A case report[J]. Hepatology, 2016, 64(5): 1800-1802. DOI: 10.1002/hep.28656.
[34]	BENEDICT M, RODRIGUEZ-DAVALOS M, EMRE S, et al. Congenital extrahepatic portosystemic shunt (Abernethy malformation type Ib) with associated hepatocellular carcinoma: Case report and literature review[J]. Pediatr Dev Pathol, 2017, 20(4): 354-362. DOI: 10.1177/1093526616686458.
[35]	RAJESWARAN S, JOHNSTON A, GREEN J, et al. Abernethy malformations: Evaluation and management of congenital portosystemic shunts[J]. J Vasc Interv Radiol, 2020, 31(5): 788-794. DOI: 10.1016/j.jvir.2019.08.007.
[36]	LIU MK, CHEN XH, FANG YF, et al. Type Ⅱ Abernethy malformation combined with focal nodular hyperplasia of the liver: a case report and literature review[J]. Chin J Hepatobiliary Surg, 2021, 27(12): 941-942. DOI: 10.3760/cma.j.cn113884-20210330-00116. 刘明坤, 陈兴海, 方一凡, 等. Ⅱ型Abernethy畸形合并肝脏局灶性结节性增生一例并文献复习[J]. 中华肝胆外科杂志, 2021, 27(12): 941-942. DOI: 10.3760/cma.j.cn113884-20210330-00116.
[37]	MATSUURA T, TAKAHASHI Y, YANAGI Y, et al. Surgical strategy according to the anatomical types of congenital portosystemic shunts in children[J]. J Pediatr Surg, 2016, 51(12): 2099-2104. DOI: 10.1016/j.jpedsurg.2016.09.046.
[38]	ACHIRON R, GINDES L, KIVILEVITCH Z, et al. Prenatal diagnosis of congenital agenesis of the fetal portal venous system[J]. Ultrasound Obstet Gynecol, 2009, 34(6): 643-652. DOI: 10.1002/uog.7460.
[39]	SINKOVSKAYA E, KLASSEN A, ABUHAMAD A. A novel systematic approach to the evaluation of the fetal venous system[J]. Semin Fetal Neonatal Med, 2013, 18(5): 269-278. DOI: 10.1016/j.siny.2013.05.009.
[40]	YAGEL S, COHEN SM, VALSKY DV, et al. Systematic examination of the fetal abdominal precordial veins: a cohort study[J]. Ultrasound Obstet Gynecol, 2015, 45(5): 578-583. DOI: 10.1002/uog.13444.
[41]	SORKIN T, STRAUTNIEKS S, FOSKETT P, et al. Multiple β-catenin mutations in hepatocellular lesions arising in Abernethy malformation[J]. Hum Pathol, 2016, 53: 153-158. DOI: 10.1016/j.humpath.2016.02.025.
[42]	DELDAR ABAD PASKEH M, MIRZAEI S, ASHRAFIZADEH M, et al. Wnt/β-catenin signaling as a driver of hepatocellular carcinoma progression: An emphasis on molecular pathways[J]. J Hepatocell Carcinoma, 2021, 8: 1415-1444. DOI: 10.2147/JHC.S336858.