摘要
目的初步评估血管内治疗新生儿布‑加综合征的短期疗效,总结临床治疗经验。方法回顾性分析福建省妇幼保健院2022年6月收治的1例布‑加综合征新生儿的临床资料。患儿,男,生后5 d因彩色多普勒超声提示“下腔静脉肝段强回声(血栓可能)”入院治疗,经腹部计算机断层血管造影检查后诊断为布‑加综合征,遂行血管内治疗,对下腔静脉闭塞段予单纯球囊扩张。以“新生儿”“布‑加综合征”“Budd‑Chiari综合征”及其英文“neonate”“Budd‑Chiari syndrome”为关键词,检索中国知网、万方、维普及PubMed数据库中截至2024年5月收录的相关文献并行文献复习。结果本例患儿手术顺利,术中普通肝素用量为370 IU,造影剂用量为90 ml,X线曝光时间为21 min,辐射吸收剂量为15 mGy。术后继续接受补液、抗凝治疗,术后食欲逐渐改善,术后1周复查肝功能指标恢复至正常参考范围。术后1周复查腹部彩色多普勒超声提示下腔静脉血流通畅。患儿出院后予利伐沙班抗凝治疗,随访22个月,体重正常增长,症状无复发。文献检索到5例布‑加综合征新生儿,结合本例共6例患儿,患儿均存在一种或多种消化道症状(包括食欲差、腹胀、肝脾肿大、腹水、皮肤黄染),其中5例为男婴,5例为足月儿,5例患儿病变血管位置明确,1例患儿检测到凝血因子ⅤLeiden突变。文献检索的5例患儿均未接受手术治疗,其中4例患儿死亡,仅有1例患儿报道时存活。结论布‑加综合征一经确诊建议立即临床干预,血管内治疗(单纯球囊扩张成形)新生儿布‑加综合征的短期效果良好,术中应充分扩张闭塞段血管,术后应选择合适的药物如利伐沙班进行持续抗凝治疗。
Objective To preliminarily evaluate the short‑term efficacy of endovascular treatment for neonatal Budd‑Chiari syndrome(BCS),and to summarize clinical treatment experience.Methods A retrospective analysis was conducted on the clinical data of one neonate with BCS admitted to Fujian Maternity and Child Health Hospital in June 2022.A male infant at 5 days of age,was hospitalized after color Doppler ultrasound indicated“hyperechoic signals in the hepatic segment of inferior vena cava(possible thrombosis).”Following abdominal computed tomography venography(CTV),the patient was diagnosed with BCS.Endovascular treatment was performed with simple balloon dilation of the occluded segment of the inferior vena cava.A literature review was conducted by searching keywords including“neonate,”“Budd‑Chiari syndrome,”in both Chinese and English languages in the CNKI,WanFang Data,VIP,and PubMed databases up to May 2024.Results The patient in our center was successfully treated by surgical procedures,with an intraoperative heparin usage of 370 IU,contrast agent usage of 90 ml,X‑ray exposure time of 21 minutes,and radiation absorption dose of 15 mGy.Postoperatively,the patient continued receiving fluid replacement and anticoagulation therapy.The patient's appetite gradually improved,and liver function indices returned to the normal reference ranges during one‑week follow‑up.Color Doppler ultrasound one week after surgery showed patent blood flow in the inferior vena cava.After discharge,the patient received rivaroxaban for anticoagulation therapy.During 22 months of follow‑up,the patient's weight increased normally without a recurrence.Through literature review,5 neonatal BCS cases were reported.By analyzing clinical data of 6 neonatal cases of BCS(plus one case in our center),all patients presented with one or more gastrointestinal symptoms,including poor appetite,abdominal distension,hepatosplenomegaly,ascites,and skin jaundice.Among them,5 were male infants,5 were full‑term infants,and 5 reported the location of the affected vessels.Factor V Leiden mutation was detected in one patient.None of the 5 previously reported patients received surgical intervention.Among them,4 died,and only 1 was reported as surviving at the time of publication.Conclusions Immediate clinical intervention is recommended once BCS is diagnosed.Endovascular treatment(simple balloon angioplasty)for neonatal BCS has favorable short‑term outcomes.Intraoperatively,the occluded vascular segment should be fully dilated.Postoperatively,appropriate medications such as rivaroxaban should be promptly administered for continuous anticoagulation therapy.
作者
张荣
林云峰
何剑锋
蔡天鸿
詹腾辉
Zhang Rong;Lin Yunfeng;He Jianfeng;Cai Tianhong;Zhan Tenghui(Department of Vascular Surgery&Interventional Therapy,Fujian Maternity and Child Health Hospital,Fuzhou 350001,China;Neonatal Intensive Care Unit,Fujian Children's Hospital,Fuzhou 350001,China)
出处
《中华小儿外科杂志》
北大核心
2025年第7期598-603,共6页
Chinese Journal of Pediatric Surgery
基金
福建省科技创新联合资金项目(2021Y9159)。
关键词
布‑加综合征
血管内治疗
抗凝治疗
新生儿
Budd-Chiari syndrome
Endovascular treatment
Anticoagulation therapy
Neonate
