儿童肝移植合并正中弓状韧带压迫综合征1例  

A case report of pediatric liver transplantation complicated with median arcuate ligament compression syndrome

作　　者：乐盈 张明满[1] 李英存[1] 戴小科[1] Le Ying;Zhang Mingman;Li Yingcun;Dai Xiaoke(Department of Hepatobiliary Surgery,Children's Hospital of Chongqing Medical University,the Ministry of Education Key Laboratory of Child Development Disease Research,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院肝胆外科,儿童发育疾病研究教育部重点实验室,儿童发育重大疾病国家国际科技合作基地,儿科学重庆市重点实验室,重庆400014

出　　处：《中华器官移植杂志》2025年第3期235-238,共4页Chinese Journal of Organ Transplantation

摘　　要：正中弓状韧带压迫综合征(median arcuate ligament compression syndrome,MALS)临床表现不典型,在儿童中罕有报道。对于肝移植受者,合并MALS是术后肝动脉血栓形成的独立危险因素。该例儿童受者因肝豆状核变性、肝硬化、急性肝衰竭入院,人工肝替代治疗后肝功能衰竭无明显好转,遂行肝移植治疗。移植术中因开放吻合肝动脉搏动弱,无搏动性血流波形,考虑出现MALS,后通过松解并离断弓状韧带,结扎脾动脉及胃左动脉成功处理。术前仔细阅读影像学资料,关注CT血管成像的典型表现,个体化制定手术预案,术中根据超声监测结果及动脉搏动情况调整术式是保证移植效果的关键。Median arcuate ligament compression syndrome(MALS)presents with atypical clinical manifestations.MALS is rarely reported in pediatric patients but is recognized as an independent risk factor for postoperative hepatic artery thrombosis in liver transplant recipients.We report a case of a pediatric liver transplant recipient with hepatolenticular degeneration,cirrhosis,and acute liver failure.Despite undergoing artificial liver support therapy,the patient showed no significant improvement in liver function and subsequently underwent liver transplantation.Intraoperatively,weak arterial pulsation and the absence of a pulsatile waveform in the hepatic artery anastomosis raised suspicion of MALS.The condition was successfully managed by releasing and transecting the median arcuate ligament,along with ligation of the splenic and left gastric arteries.To optimize transplantation outcomes,meticulous preoperative imaging assessment,particularly focusing on characteristic findings in CT angiography,is essential.Additionally,individualized surgical planning and intraoperative adjustments based on ultrasound monitoring and arterial pulsation assessments are critical for ensuring successful transplantation.

关 键 词：肝移植 正中弓状韧带 肝动脉血栓形成 CT血管成像 

分 类 号：R726.5[医药卫生—儿科]