儿童门静脉海绵样变胆道病变:病例系列报道并简要文献回顾  

Portal cavernoma cholangiopathy in children:a case series and a brief literature review

作　　者：王增萌[1] 闫俊[1] 彭春辉[1] 吴东阳[1] 沈秋龙[2] 王凯[1] 陈亚军[1] Wang Zengmeng;Yan Jun;Peng Chunhui;Wu Dongyang;Shen Qiulong;Wang Kai;Chen Yajun(Department of General Surgery,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China;Department of Emergency Surgery,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院普外科,北京100045 [2]国家儿童医学中心,首都医科大学附属北京儿童医院急症外科,北京100045

出　　处：《中华小儿外科杂志》2025年第1期20-25,共6页Chinese Journal of Pediatric Surgery

摘　　要：目的总结儿童门静脉海绵样变胆道病变(portal cavernoma cholangiopathy,PCC)的临床特征,探讨患儿磁共振胰胆管成像(magnetic resonance cholangiopancreatography,MRCP)下的胆道形态,以提高临床对该疾病的诊疗认识水平。方法回顾性分析2021年1月至2022年12月首都医科大学附属北京儿童医院收治的11例门静脉海绵样变患儿的临床资料,所有患儿临床表现符合肝外门静脉阻塞,患儿均接受MRCP检查。分析所有患儿的临床表现、肝生化功能指标以及MRCP胆道形态学改变,采用印度国家肝脏研究协会简化"标准化命名"方法描述患儿的胆道形态特点。结果本组11例患儿中男4例,女7例;入院年龄为(91.3±11.8)个月;所有患儿均诊断为PCC。1例患儿存在梗阻性黄疸症状,患儿继发肝功能损害;另1例患儿存在肝功能指标异常,丙氨酸转氨酶及γ-谷氨酰转移酶轻微升高,提示极早期和轻微的胆道淤滞;其余9例均不具有胆绞痛、胆管炎、胆道结石或梗阻性黄疸的胆道系统疾病症状,无肝功能异常。按照简化"标准化命名"方法,胆道形态异常表现包括外在压迹(3/11)、浅压迹(1/11)、胆管形态不规则(6/11)、胆管狭窄并上游扩张(4/11)、胆管成角(4/11),未见充盈缺损(0/11)和原发胆管扩张(0/11)表现者。结论儿童PCC常见,但很少有症状;无症状者无须胆道治疗。压迹、胆管形态不规则、狭窄伴近端扩张、胆管成角是PCC的常见表现,而充盈缺损、原发胆管扩张少见。Objective To summarize the clinical features of children with portal cavernoma cholangiopathy(PCC)and the biliary tract morphology on the magnetic resonance cholangiopancreatography(MRCP),thus improving its clinical recognition.MethodsFrom January 2021 to December 2022,clinical data of 11 children with cavernous transformation of portal vein diagnosed with extrahepatic portal vein obstruction by MRCP in Beijing Children's Hospital,Capital Medical University were retrospectively analyzed.Clinical manifestations,liver function indicators,and biliary morphological changes visualized on MRCP were analyzed.A simplified"standardized nomenclature"proposed by the Indian National Association for Study of the Liver(INASL)was used to describe the bile duct morphological changes.ResultsEleven patients were included with the diagnosis of PCC.There were four males and seven females,with a median age of 91.3±11.8 months.One child had symptoms of obstructive jaundice,and secondary liver function damage.Another child had abnormal liver function indicators,with slight elevations of alanine aminotransferase(ALT)andγ-glutamyl transferase(γ-GGT)suggestive of very early and mild symptoms of biliary stasis.The remaining 9 children did not present symptoms of biliary system disease such as biliary colic,cholangitis,biliary stones or obstructive jaundice,and the liver function was normal.According to the simplified"standardized nomenclature",the abnormal bile duct morphology included extrinsic impression(3/11),shallow impression(1/11),irregular ductal contour(6/11),stricture with upstream dilatation(4/11),bile duct angulation(4/11).No filling defects(0/11)or ectasia(0/11)were observed.ConclusionsPCC in children is very common but rarely symptomatic.No specific treatment is required for asymptomatic patients.Impression,irregular ductal contour,stricture with upstream dilatation,and bile duct angulation are common morphological presentations,while filling defects,and ectasia are rarely seen.

关 键 词：门静脉 肝外门静脉阻塞 门静脉海绵样变 门静脉高压 磁共振胰胆管成像 儿童 

分 类 号：R725.7[医药卫生—儿科]