卡波西样血管内皮瘤伴慢性淋巴水肿的临床特征、风险评估与治疗  

Clinical analysis and risk management of chronic lymphedema in children with kaposiform hemangioendothelioma

作　　者：邱桐 周江元 杨开颖 代诗懿 张学鹏 陈思源[2] 吉毅[1] Qiu Tong;Zhou Jiangyuan;Yang Kaiying;Dai Shiyi;Zhang Xuepeng;Chen Siyuan;Ji Yi(Department of Pediatric Surgery,West China Hospital,Sichuan University,Chengdu 610041,China;Department of Critical Care Medicine,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院小儿外科,成都610041 [2]四川大学华西医院重症医学科,成都610041

出　　处：《中华小儿外科杂志》2022年第9期780-784,共5页Chinese Journal of Pediatric Surgery

基　　金：国家自然科学基金(81401606、81400862);四川省科技厅重点研发项目(2019YFS0322);四川大学优秀青年学者基金(2015SU04A15);四川大学华西医院学科卓越发展1·3·5工程临床研究孵化项目(2019HXFH056、2020HXFH048、ZYJC21060)。

摘　　要：目的探讨卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)患儿慢性淋巴水肿的发生率、临床特征、危险因素和治疗。方法收集2010年1月至2018年6月于四川大学华西医院小儿外科就诊的KHE患儿共159例,其中男88例,女71例,KHE发病时的平均年龄为10.5个月,确诊时的平均年龄为15.1个月。根据患儿的病情进行个体化治疗并进行至少3年的随访。回顾临床特征以分析慢性淋巴水肿的可能原因、淋巴水肿的程度、危险因素和治疗策略。结果61.6%(98/159)的患儿发生卡梅现象(Kasabach-Merritt phenomenon,KMP),病变累及四肢占58.5%(93/159)。159例患儿平均随访时间为4.6年,18例患儿在KHE发病1年后通过淋巴显像诊断为慢性淋巴水肿,且均为单侧肢体受累,并伴有同侧KHE。淋巴水肿在病变累及四肢、病灶较大(≥10 cm)和混合型病变的患儿中更为常见(P<0.01)。14例患儿在转诊后接受了西罗莫司治疗,其中10例转诊时发现肢体肿胀,其余4例在转诊后西罗莫司治疗期间出现肿胀。随访期间有12例患儿接受了加压治疗。末次随访时,患儿淋巴水肿均未完全消退。结论慢性淋巴水肿是KHE较为严重的后遗症,其发生率为11.3%,病变累及四肢、病灶较大(≥10 cm)和混合型病变为淋巴水肿的危险因素,应当密切鉴别并检测慢性淋巴水肿的发生,予以患儿个体化治疗。Objective To explore the incidence,clinical features,risk factors and managements of chronic lymphedema in children with kaposiform hemangioendothelioma(KHE).Methods Retrospective analysis was performed for 159 KHE children.There were 88 boys and 71 girls with a follow-up time for at least 3 years.Clinical characteristics were analyzed for examining the possible causes,degrees of lymphedema,risk factors and managements of chronic lymphedema.Results Kasabach-Merritt phenomenon(KMP)occurred in 61.6%(98/159)and 58.5%(93/159)had an involvement of extremities.The average follow-up period was 4.6 years.In 18 cases,chronic lymphedema was diagnosed by lymphoscintigraphy at 1 year after an onset of KHE.Chronic lymphedema with unilateral extremity involvement was accompanied by ipsilateral KHE.Lymphedema was more common in children involving extremities with larger lesions(≥10 cm)and mixed lesions(P<0.01).Upon referral,extremity swelling was present(n=10)and absent(n=4).All 14 cases(77.8%)received sirolimus.Twelve cases(66.7%)received compression therapy.At the last follow-up,lymphedema had not completely resolved.Conclusions Chronic lymphedema is a serious sequela of KHE with an incidence of 11.3%.Lesions involving extremities,larger lesions(≥10 cm)and mixed lesions are risk factors for lymphedema.The occurrence of chronic lymphedema should be closely differentiated and individually treated.

关 键 词：血管肿瘤 卡波西血管内皮瘤 淋巴水肿 卡梅现象 

分 类 号：R732.2[医药卫生—肿瘤]