Peng Yuming,Yin Qiang,Yuan Miaoxian,et al.Treatment strategy of portal vein cavernous degeneration shunt and liver transplantation in children[J].Journal of Clinical Pediatric Surgery,,21():431-436.[doi:10.3760/cma.j.cn101785-202203043-007]
Treatment strategy of portal vein cavernous degeneration shunt and liver transplantation in children
- Keywords:
- Portal Vein/PP; Cavernous Transformation of Portal Vein; Splenorenal Shunt; Surgical; Por- tasystemic Shunt; Surgical; Liver Transplantation; Surgical Procedures; Operative/MT; Treatment Outcome
- Abstract:
- Objective To explore the clinical characteristics and surgical treatment of spongiosis of por- tal vein in children at a single center. Methods From May 2010 to January 2022, clinical data were retrospec- tively reviewed for 27 children with portal cavernous degeneration. There were 12 boys and 15 girls with an age range of (19-143) months. Form of informed consent was signed. Cavernous portal vein degeneration was di- agnosed by abdominal ultrasound and computed tomography (CT). Digital subtraction angiography (DSA) was employed for further evaluating the patency of portal vein in and outside liver. Finally surgical approach was se- lected according to the pathological findings. Results All of them underwent surgery, including 19 cases of Rex operation,19 cases of high preoperative portal vein pressure, pressure declined markedly after Rex bypass. In 2 cases, large lateral hepatic branch vein was anastomosed with sagittal part of portal vein. And intrahepatic portal vein was perfused with satisfactory amount. In 1 child, splenic vein was used for classical Rex surgery af- ter splenic splenectomy. In 2 cases, thrombosis was formed in bypass vessels. Despite anticoagulant therapy, vas- cular occlusion occurred. Three children underwent liver transplantation due to severe portal spongiform degen- eration and recurrent gastrointestinal bleeding. One child of early hepatic artery thrombosis became recanalized after anticoagulant therapy. Distal splenorenal shunt was performed in 3 cases and shunt vessels stayed unobstr- ucted. In two children of proximal splenorenal shunt, one case developed mural thrombosis of splenic vein and improved after anticoagulant therapy. Conclusion Children’s portal cavernous degeneration is often character- ized by symptoms related to portal hypertension. The operation method should be selected according to the com- prehensive analysis of the development and compensation of the portal vein inside and outside the liver. The by- pass vessel can be selected according to the characteristics of the children’s own blood vessels. The reason for the first choice of Rex surgery is that it can restore the normal anatomical channel and physiological function of the portal vein system, improve liver perfusion and growth and development, and avoid the occurrence of hepatic encephalopathy.
References:
[1] Ruan Z, Wu M, Shao C, et al. Effects of Rex-bypass shunt on the cavernous transformation of the portal vein in children:evaluation by the color Doppler ultrasonography[J]. Insights Imaging, 2020,11(1):4. DOI:10. 1186/s13244-019-0815-6.
[2] Yonem O, Bayraktar Y. Is portal vein cavernous transformation a component of congenital hepatic fibrosis?[J]. World J Gastro- enterol,2007, 13(13):1928-1929. DOI:10. 3748/wjg. v13. i13. 1928.
[3] 孙蕊, 李龙. 儿童门静脉海绵样变性的外科手术治疗研究进展[J]. 临床小儿外科杂志, 2020, 19(12):1145-1151. DOI:10. 3969/j. issn. 1671-6353. 2020. 12. 016. Sun R, Li L. Research advances of surgery for cavernous transfor- mation of portal vein in children[J]. J Clin Ped Sur, 2020, 19(12):1145-1151. DOI:10. 3969/j. issn. 1671-6353. 2020. 12. 016.
[4] Yamoto M, Chusilp S, Alganabi M, et al. Meso-Rex bypass versus portosystemic shunt for the management of extrahepatic portal vein obstruction in children:systematic review and meta-analysis[J]. Pediatr Surg Int, 2021, 37(12):1699-1710. DOI:10. 3748/wjg. v13. i13. 1928.
[5] Guérin F, Bidault V, Gonzales E, et al. Meso-Rex bypass for ex- trahepatic portal vein obstruction in children[J]. Br J Surg, 2013, 100(12):1606-1613. DOI:10. 1002/bjs. 9287.
[6] Sarma MS, Ravindranath A, Ravindranath. Portal cavernoma cholangiopathy in children and the management dilemmas[J]. J Clin Transl Hepatol, 2020, 8(1):61-68. DOI:10. 14218/JCTH. 2019. 00041.
[7] Hawkins CM, Shaw DW, Healey PJ, et al. Pediatric liver trans- plant portal vein anastomotic stenosis:correlation between ultra- sound and transhepatic portal venography[J]. Liver Transpl, 2015, 21(4):547-553. DOI:10. 1002/lt. 24077.
[8] Zhang JS, Li L. Imaging features and clinical relevance of portal venous systems shown by extrahepatic portal angiography in chil- dren with extrahepatic portal venous obstruction[J]. J Vasc Surg Venous Lymphat Disord, 2020, 8(5):756-761. DOI:10. 1016/j. jvsv. 2019. 11. 016.
[9] 曾道炳, 邸亮, 丁兢, 等. 断流术治疗肝硬化门静脉高压症食管胃静脉曲 张疗 效[J]. 中华 肝脏 外科 手术 学电 子杂 志, 2019, 8(4):306-310. DOI:10. 3877/cma. j. issn. 2095-3232. 2019. 04. 007. Zeng DB, Di L, Ding J, et al. Clinical efficacy of devascularization for esophageal and gastric varices in cirrhotics with portal hyper- tension[J]. Chinese Journal of Hepatic Surgery (Electronic Edi- tion), 2019, 8(4):306-310. DOI:10. 3877/cma. j. issn. 2095-3232. 2019. 04. 007.
[10] 张正筠, 陈其民, 徐敏, 等. 脾-左肾上腺静脉分流术治疗儿童门静 脉海 绵样 变性[J]. 中华 普通 外科 杂志, 2010, 25(1):17-19. DOI:10. 3760/cma. j. issn. 1007-631 X. 2010. 01. 006. Zhang ZJ, Chen QM, Xu M, et al. Spleno-left adrenal shunt for the treatment of portal spongiosis in children[J]. Chinese Jour- nal of General Surgery, 2010, 25(1):17-19. DOI:10. 3760/cma. j. issn. 1007-631 X. 2010. 01. 006.
[11] Lautz TB, Keys LA, Melvin JC, et al. Advantages of the meso-Rex bypass compared with portosystemic shunts in the management of extrahepatic portal vein obstruction in children[J]. J Am Coll Surg, 2013, 216(1):83-89. DOI:10. 1016/j. jamcollsurg. 2012. 09. 013.
[12] Lautz TB, Eaton S, Keys L, et al. Metabolic profile of children with extrahepatic portal vein obstruction undergoing meso-Rex by- pass[J]. J Surg Res, 2018, 223:109-114. DOI:10. 1016/j. jss. 2017. 10. 010.
[13] Zhang J, Li L. Rex shunt for extra-hepatic portal venous obstruc- tion in children[J]. Children (Basel), 2022, 9(2):297. DOI:10. 3390/children9020297.
[14] de Ville de Goyet J, Alberti D, Clapuyt P, et al. Direct bypassing of extrahepatic portal venous obstruction in children:a new tech- nique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension[J]. J Pediatr Surg, 1998, 33(4):597-601. DOI:10. 1016/s0022-3468(98) 90324-4.
[15] 张金山, 李龙. 小儿门静脉高压手术治疗新进展. 临床小儿外科杂志, 2019, 18(12):989-993. DOI:10. 3969/j. issn. 1671-6353. 2019. 12. 001. Zhang JS, Li L. Recent advances in surgery for portal hypertension in children[J]. J Clin Ped Sur, 2019, 18(12):989-993. DOI:10. 3969/j. issn. 1671-6353. 2019. 12. 001.
[16] Muratore S, Flanagan S, Hunter D, et al. Recanalization of chronic extrahepatic portal vein obstruction in pediatric patients using a minilaparotomy approach[J]. J Pediatr Gastroenterol Nutr, 2019, 68(3):384-388. DOI:10. 1097/MPG. 0000000000002206.
[17] Bruestle K, Griesemer A. Pediatric liver transplantation in a cen- ter that is neither east nor west[J]. Ann Surg, 2021, 273(2):e73-e74. DOI:10. 1097/SLA. 0000000000004525.
Memo
收稿日期:2022-3-13。
基金项目:湖南省卫生健康委员会技术创新项目(编号:湘卫医政医管处便函[2018]187号),湖南省儿童医院国际化人才项目
通讯作者:尹强,Email:qiangyin@hotmail.com