Ⅱ型Abernethy畸形4例诊治体会  

Clinical managements of type Ⅱ Abernethy malformation: a report of 4 cases

作　　者：彭春辉[1] 王增萌[1] 吴东阳[1] 黄心洁[1] 王凯[1] 严佳虞 陈亚军[1] Peng Chunhui;Wang Zengmeng;Wu Dongyang;Huang Xinjie;Wang Kai;Yan Jiayu;Chen Yajun(Department of General Surgery,Affiliated 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

出　　处：《中华小儿外科杂志》2023年第10期915-921,共7页Chinese Journal of Pediatric Surgery

摘　　要：目的评估Ⅱ型Abernethy畸形术前合并症、术后并发症,探讨最佳治疗方法,降低手术风险。方法回顾性分析2021年1月至2021年12月首都医科大学附属北京儿童医院收治的术前伴有严重合并症或术后出现严重并发症的4例Ⅱ型Abernethy畸形患儿的临床资料。其中男1例,女3例;主要因血便、发绀、劳力性呼吸困难、转氨酶升高、肝脏肿瘤就诊。评估患儿术前合并症、术后并发症情况,并对其进行随访。结果4例患儿中,3例有术前合并症,其中肝脏肿瘤2例、肺动脉高压(术前均为中度)2例、肝性脑病2例、肝肺综合征(重度)1例。2例肝脏肿瘤患儿甲胎蛋白均在正常水平范围,其中1例直径小未活检,而另1例术前穿刺活检证实良性。4例患儿均行开腹分流关闭手术,术中行阻断实验。分流阻断后,3例患儿肠管无淤血,门静脉压力在25 mmHg(1 mmHg=0.133 kPa)以下,一期关闭分流;另1例患儿肠管淤血严重,门静脉压力达36 mmHg,分期关闭分流。术后常规给予肝素、低分子肝素序贯抗凝,超声监测有无血栓。1例患儿术后第3天出现门静脉广泛血栓,有轻微腹痛、腹胀,给予全身溶栓、抗凝治疗,血栓大部分消退且无症状。术后血便、发绀、转氨酶升高缓解;大的肝脏肿瘤较前缩小,小的边界较前不清;肺动脉高压术后有波动,需药物治疗,但均降至轻度;肝性脑病、肝肺综合征缓解。结论大部分症状或合并症在分流关闭手术后可缓解或减轻;肠管有无淤血是决定分流能否一期关闭的重要条件;术前需确定肝脏肿瘤良恶性;而术前肺动脉压力控制在中度及以下,围手术期相对安全;术后超声监测血栓,及时的溶栓、抗凝治疗可缓解门静脉血栓。Objective To propose an optimal management of typeⅡAbernethy malformation through evaluating preoperative and postoperative complications.Methods From January 2021 to December 2021,the relevant clinical data were retrospective reviewed for four children with typeⅡAbernethy malformation with severe perioperative complications.There were 3 girls and 1 boy.Major complaints were melena,cyanosis,dyspnea,elevated transaminases and liver tumor.The perioperative complications were evaluated.Results Among them,three cases presented with preoperative complications,including liver tumor(n=2),moderate pulmonary arterial hypertension(PaHT,n=2),hepatic encephalopathy(HE,n=2)and severe hepatopulmonary syndrome(HPS,n=1).Alpha feto-protein(AFP)was normal in two liver tumor patients.Benign lesion was confirmed by needle biopsy confirmed in one case while another child did not undergo biopsy since the lesion was small.Shunt closure and intraoperative occlusion test were performed.Three children underwent successful one-stage shunt closure with portal pressure<25 mmHg and there was no sign of bowel ischemia on occlusion test.One child underwent staged shunt closure due to markedly elevated portal pressure(36 mmHg)on occlusion test and signs of bowel ischemia.Sequential anticoagulation therapy(heparin and low molecular heparin)was offered and routine ultrasonic screening of thrombosis performed after closure.One child developed mild abdominal pain and distension due to portal vein thrombosis at Day 3 postoperatively.And discomfort and thrombus regressed after systematic thrombolytic and anticoagulation therapy.During follow-ups,melena,cyanosis and elevated transaminases were improved.Liver tumor decreased in size.PaHT fluctuated and yet eventually remained mild.Both HE and HPS improved.Conclusions Most symptoms and associated complications may be relieved or alleviated after shunt closure.One-stage closure is largely dependent upon no signs of bowel ischemia on occlusion test.The exact nature of liver tumor should be confirmed be

关 键 词：血管畸形 ABERNETHY畸形 合并症 并发症 

分 类 号：R726.5[医药卫生—儿科]