遗传性球形红细胞增多症副脾的临床特点及术后残留的预后分析  

Clinical features of accessory spleen and prognostic analysis of residual accessory spleen in hereditary spherocytosis

作　　者：严佳虞 刘亚坤 闫俊[1] 彭春辉[1] 庞文博[1] 陈亚军[1] Yan Jiayu;Liu Yakun;Yan Jun;Peng Chunhui;Pang Wenbo;Chen Yajun(Department of General 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

出　　处：《中华小儿外科杂志》2023年第12期1098-1103,共6页Chinese Journal of Pediatric Surgery

摘　　要：目的分析遗传性球形红细胞增多症(hereditary spherocytosis, HS)副脾的临床特点及脾切除术后副脾残留患儿的预后, 完善HS的诊疗流程。方法回顾性收集2017年1月至2021年12月于首都医科大学附属北京儿童医院行脾切除术的54例HS患儿的临床资料, 其中男30例, 女24例, 手术年龄为(8.2±3.5)岁。根据手术记录、术后病理及超声结果筛选存在副脾的患儿, 分析影像学资料和副脾情况。通过电话或微信随访预后, 包括有无严重感染和溶血性贫血复发, 分析副脾残留患儿的预后。结果 26例(26/54, 48.1%)患儿存在副脾, 4例(4/26, 15.4%)术前检查提示存在副脾。分别有16例、6例和3例患儿存在1个、2个和3个副脾。明确具体位置的副脾共37个, 主要位于脾门(30/37, 81.1%)和脾结肠韧带(5/37, 13.5%)。共50例(50/54, 92.6%)患儿获得随访, 平均随访时间为2.8(1.5, 3.6)年, 随访年龄为(11.1±3.4)岁。1例(1/50, 2.0%)术后出现2次严重感染。7例(7/54, 13.0%)脾切除术后残留副脾, 4例为术中未发现副脾, 均获得随访, 随访时间为(3.7±0.9)年。1例术中发现副脾, 但未切除, 术后3年内间接胆红素持续升高, 残留副脾体积逐渐增大, 且术后3年复查超声检查提示存在2个副脾;之后随访2年, 胆红素和副脾性质未再变化。其余副脾残留患儿随访未出现严重感染和溶血性贫血复发症状。结论 HS患儿存在副脾比例高, 主要位于脾门, 术前诊断副脾困难, 需术中仔细探查予以切除。HS脾切除术后出现黄疸需注意有无副脾残留。副脾残留患儿术后存在溶血情况但短期内可不出现贫血症状, 能否长期稳定需规律随访。Objective To explore the clinical features of accessory spleen in hereditary spherocytosis(HS)and track the prognosis of children with residual accessory spleen after splenectomy to improve the diagnosis and treatment of HS.Methods The relevant clinical data were retrospectively reviewed for 54 HS children undergoing splenectomy from January 2017 to December 2021.There were 30 boys and 24 girls with an average operative age of(8.2±3.5)years.The imaging data and clinical characteristics of accessory spleen were recorded.The prognosis was evaluated by either telephone or WeChat,including postoperative severe infection and recurrence of hemolytic anemia.The followed-up results of children with residual accessory spleen were also analyzed.Results Accessory spleen was detected by preoperative examination in 26(26/54,48.1%)children,including 4(4/26,15.4%)in splenic hilum.There were 16,6 and 3 cases with 1,2 and 3 accessory spleens respectively.A total of 37 accessory spleens were identified,predominantly in splenic hilum(30/37,81.1%)and ienocolic ligament(5/37,13.5%).Fifty children(50/54,92.6%)were followed up with an average time of 2.8(1.5,3.6)years and an average age of(11.1±3.4)years.One child(1/50,2.0%)developed severe infections twice post-splenectomy.Seven cases(7/54,13.0%)had residual accessory spleen post-splenectomy and were followed up with an average time of(3.7±0.9)years.No accessory spleen was found in 4 children during splenectomy.Accessory spleen was found in 1 child during operation but was not removed.Indirect bilirubin continued rising within 3 years post-splenectomy with an expanding volume of residual accessory spleen and another accessory spleen was found by ultrasound at 3 years post-splenectomy.However,bilirubin and accessory spleens stabilized after 3 to 5 years of follow-up.The remainders with residual accessory spleen were followed up without severe infection or recurrence of hemolytic anemia.Conclusions Accessory spleen is prevalent in HS and it predominates in splenic hilum.A preoperativ

关 键 词：遗传性球形红细胞增多症 副脾 残留 预后 

分 类 号：R726.5[医药卫生—儿科]