Huang Zongwei,Gao Zhigang,Zhang Lifeng,et al.Clinical analysis of cavernous transformation of portal vein in children:a report of 18 cases[J].Journal of Clinical Pediatric Surgery,2020,19(09):810-815.[doi:10.3969/j.issn.1671-6353.2020.09.010]
儿童门静脉海绵样变18例诊疗分析
- Title:
- Clinical analysis of cavernous transformation of portal vein in children:a report of 18 cases
- 关键词:
- 门静脉/病理学; 门静脉海绵样变/诊断; 门静脉海绵样变/治疗; 外科手术/方法; 儿童
- Keywords:
- Portal Vein/PA; Cavernous Transformation of Portal Vein/DI; Cavernous Transformation of Portal Vein/TH; Surgical Procedures; Operative/MT; Child
- 分类号:
- R726.5;R657.3
- 摘要:
- 目的 探讨儿童门静脉海绵样变的临床特点及治疗方式。方法 回顾性分析2012年至2018年浙江大学医学院附属儿童医院收治的18例门静脉海绵样变患儿临床资料、手术方式及预后情况,并进行总结分析。结果 本组18例均手术成功。其中5例行Rex手术,选择的分流血管包括肠系膜上静脉、胃冠状静脉、胃右静脉以及门静脉扩张最显著的侧支血管。行Rex手术的患儿有1例术后因消化道出血、脾功能亢进而再次接受手术,发现为吻合口狭窄,术中切除狭窄段,取管径较大的一段空肠系膜血管作为补片搭桥,同时行食管胃底静脉结扎术。5例行Warren手术的患儿术后恢复良好,无一例消化道出血,脾功能亢进、脾大现象均明显改善,术后无一例并发症。4例行近端脾肾分流术(其中1例术后反复消化道出血,于二次手术时行肠系膜上静脉-下腔静脉分流术)。3例行肠系膜上静脉-下腔静脉分流术,同时切除脾脏,术后出现消化道出血,保守治疗后病情得到控制。结论 儿童门静脉海绵样变需早期诊断,选择恰当的治疗措施对改善患儿的预后非常重要。外科治疗方法的选择应根据患儿的病情及门静脉系统血管的条件来决定,其中Rex手术是儿童门静脉海绵样变的首选治疗方法,此外Warren手术和近端脾肾分流术也是较为理想的术式。
- Abstract:
- Objective To explore the clinical characteristics and treatment modalities of cavernous transformation of portal vein (CTPV) in children. Methods The clinical data,surgical approaches and prognoses were analyzed retrospectively for 18 CTPV children from 2012 to 2018. Results All of them were operated successfully.Five of them underwent Rex shunting.The selected shunt vessels included superior mesenteric vein,gastric coronal vein,right gastric vein and the most significantly dilated collateral vessels of portal vein.One of them was reoperated due to gastrointestinal hemorrhage and hypersplenism.During re-operation,the cause was anastomotic stenosis.Another five children underwent Warren shunting.All of them recovered well without any postoperative complication.Four of them underwent proximal splenorenal shunting.However,one child was re-operated for recurrent gastrointestinal hemorrhage.Three of them underwent superior mesenteric vein-inferior vena cava shunting.Two children with postoperative hematemesis could be controlled by conservative measures. Conclusion CTPV should be diagnosed early in children.Appropriate treatment is essential for a decent prognosis.Surgical approach should be determined according to the status of disease and the vascular conditions of portal system.Rex shunt is recommended for CTPV in children.And Warren and proximal splenorenal shunts are also ideal.
参考文献/References:
1 Yonem O,Bayraktar Y.Is portal vein cavernous transformation a component of congenital hepatic fibrosis?[J].World J Gastroenterol,2007,13(13):1928-1929.DOI:10.3748/wjg.v13.i13.1928.
2 王果,冯杰雄.小儿腹部外科学[M].北京:人民卫生出版社,2011:732-734.Wang G,Feng JX.Pediatric Abdominal Surgery[M].Beijing:People’s Health Publishing House,2011:732-734.
3 Shinohara T,Ando H,Watanabe Y,et al.Extrahepatic portal vein morphology in children with extrahepatic portal hypertension assessed by 3-dimensional computed tomographic portography:a new etiology of extrahepatic portal hypertension[J].J Pediatr Surg,2006,41(4):812-816.DOI:10.1016/j.jpedsurg.2005.12.027.
4 Guimaraes H,Castelo L,Guimaraes J,et al.Does umbilical vein catheterization to exchange transfusion lead to portal vein thrombosis?[J].Eur J Pediatr,1998,157(6):461-463.DOI:10.1007/s004310050853.
5 Kathemann S,Lainka E,Ludwig JM,et al.Imaging of the intrahepatic portal vein in children with extrahepatic portal vein thrombosis-comparison of magnetic resonance imaging and retrograde portography[J].J Pediatr Surg,2019,54(8):1686-1690.DOI:10.1016/j.jpedsurg.2018.10.049.
6 Monroe EJ,Speir EJ,Hawkins CM,et al.Transsplenic splenoportography and portal venous interventions in pediatric patients[J].Pediatric Radiology,2018,48(10):1441-1450.DOI:10.1007/s00247-018-4157-1.
7 Chennapragada SM,Lord DJ,Wong CKF,et al.Abstract No.195:Transsplenic splenoportography in paediatric portal hypertension:a safe and useful tool[J].Journal of Vascular & Interventional Radiology,2008,19(2):S74.DOI:10.1016/j.jvir.2007.12.217.
8 Menon P,Rao KL,Bhattacharya A,et al.Extrahepatic portal hypertension:quality of life and somatic growth after surgery[J].Eur J Pediatr Surg,2005,15(2):82-87.DOI:10.1055/s-2004-830341.
9 Bambini DA,Superina R,Almond PS,et al.Experience with the Rex shunt (mesenterico-left portal bypass) in children with extrahepatic portal hypertension[J].J Pediatr Surg,2000,35(1):13-19.DOI:10.1016/s0022-3468(00)80005-6.
10 Zhang JS,Li L,Cheng W.A new procedure for the treatment of extrahepatic portal hypertension in children:portal cavernoma-rex shunt with interposition of grafted portal vessel[J].J Am Coll Surg,2016,222(6):e71-e76.DOI:10.1016/j.jamcollsurg.2016.03.020.
11 Zhao H,Tsauo J,Zhang X,et al.Regarding "The optimal procedure of modified Rex shunt for the treatment of extrahepatic portal hypertension in children"[J].J Vasc Surg Venous Lymphat Disord,2018,6(3):421-422.DOI:10.1016/j.jvsv.2017.11.008.
12 Kim HB,Pomposelli JJ,Lillehei CW,et al.Mesogonadal shunts for extrahepatic portal vein thrombosis and variceal hemorrhage[J].Liver Transpl,2005,11(11):1389-1394.DOI:10.1002/lt.20487.
13 张金山,李龙.小儿肝外门静脉高压Rex术后门静脉高压复发的临床分析[J].中华小儿外科杂志,2015,36(12):894-897.DOI:10.3760/cma.j.issn.0253-3006.2015.12.004.Zhang JS,Li L.Etiology and treatment for recurrent portal hypertension after Rex shunting in children[J].Chin J Pediatr Surge,2015,36(12):894-897.DOI:10.3760/cma.j.issn.0253-3006.2015.12.004.
14 Zhang W,Shao GR,Zhang Y,et al.Partial splenectomy and use of splenic vein as an autograft for meso-Rex bypass:a clinicaI observational study[J].Med Sci Monit,2014,20:2235-2242.DOI:1012659/MSM 892482.
15 孔艳霞,张大.分流手术治疗门脉海绵样变8例临床分析[J].临床小儿外科杂志,2015,14(5):414-416.DOI:10.3969/j.issn.1671-6353.2015.05.019.Kong YX,Zhang D.Shunting procedures for cavernous transformation of portal vein:a report of 8 cases[J].J Clin Ped Sur,2015,14(5):414-416.DOI:10.3969/j.issn.1671-6353.2015.05.019.
16 Wang RY,Wang JF,Sun XG,et al.Evaluation of Rex shunt on cavernous transformation of the portal vein in children[J].World Journal of Surgery,2017,41(4):1134-1142.DOI:10.1007/s00268-016-3838-x.
17 张金山,李龙.Rex手术治疗小儿肝外门静脉高压的应用进展[J].中华小儿外科杂志,2017,38(8):636-640.DOI:10.3760/cma.j.issn.0253-3006.2017.08.018.Zhang JS,Li L.Recent advances of Rex shunt for extra-hepatic portal venous obstruction[J].Chin J Pediatr Surg,2017,38(8):636-640.DOI:10.3760/cma.j.issn.0253-3006.2017.08.018.
18 Sretenovic AL,Peri?i? V,Krsti? Z,et al.Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension,massive splenomegaly and severe hypersplenism[J].Surgery Today,2013,43(5):521-525.DOI:10.1007/s00595-012-0405-4.
19 张金山,侯文英,李龙.Warren手术治疗小儿门脉高压症50例分析[J].临床小儿外科杂志,2014,13(1):26-29.DOI:10.3969/j.issn.1671-6353.2014.01.009.Zhang JS,Hou WY,Li L.Warren’s operation in the treatment of portal hypertension in children:a report of 50 cases[J].J Clin Ped Sur,2014,13(1):26-29.DOI:10.3969/j.issn.1671-6353.2014.01.009.
20 Rao KL,Goyal A,Menon P,et al.Extrahepatic portal hypertension in children:observations on three surgical procedures[J].Pediatric Surgery International,2004,20(9):679-684.DOI:10.1007/s00383-004-1272-x.
21 Zhang JS,Li L,Hou WY,et al.Spleen-preserving proximal splenic-left intrahepatic portal shunt for the treatment of extrahepatic portal hypertension in children[J].J Pediatr Surg,2015,50(6):1072-1075.DOI:10.1016/j.jpedsurg.2015.02.048.
备注/Memo
收稿日期:2019-05-13。
基金项目:2017年浙江省医药卫生科研项目(编号:2017KY441)
通讯作者:高志刚,Email:ebwk@zju.edu.cn