Erdheim-Chester病手术治疗的近期疗效  

Preliminary study on surgical treatment of Erdheim-Chester disease

作　　者：张岩[1] 张彬[1] 李甲振[1] 卢新昌[1] 张翼[1] ZHANG Yan;ZHANG Bin;LI Jiazhen;LU Xinchang;ZHANG Yi(Department of Orthopaedics,the First Affiliated Hospital of Zhengzhou University,Zhengzhou Henan,450052,P.R.Chin)

机构地区：[1]郑州大学第一附属医院骨科,郑州450052

出　　处：《中国修复重建外科杂志》2018年第8期1026-1031,共6页Chinese Journal of Reparative and Reconstructive Surgery

摘　　要：目的总结Erdheim-Chester病(Erdheim-Chester disease,ECD)手术治疗的近期疗效,提高骨科医师对该病的认识。方法回顾性分析2012年12月-2017年10月收治的9例ECD患者,男6例,女3例;年龄8~61岁,中位年龄42岁。病程4~59个月,平均39个月。多发病变2例,其中1例累及臀部软组织、双侧胫骨,1例累及鼻窦、颅底、右肱骨近端;单发病变7例,其中累及右股骨颈3例,右胫骨近端1例,右肱骨干1例,肋骨2例。9例均依据临床表现、影像学检查及病理学诊断确诊,其中4例术前行穿刺活检术,5例术后病理确诊。5例行病变刮除植骨内固定术,1例行病变刮除植骨术,3例行病变切除术;其中1例多发病变术后采用干扰素、激素等综合治疗。结果 9例患者均安全完成手术,术后均无发热、伤口渗液、感染等发生,切口均Ⅰ期愈合。患者均获随访,随访时间4~59个月,平均31.4个月。1例双侧胫骨及臀部软组织受累及的患者仍继续接受内科治疗,肿瘤得到控制,未见明显增大;余8例患者术后3、6、12个月复查病变部位X线片示,植骨已融合,钢板、髓内钉均牢固固定、未见松动,肿瘤无复发。术后1年患者疼痛症状均改善,恢复正常生活;其中3例右股骨颈累及患者行走自如,生活质量明显提升。结论 ECD患者经过手术治疗均可消除病变、解除疼痛,术后近期未见复发;且手术治疗相较于内科治疗有快速解除疼痛、提高生活质量、副作用小等优点。Objective To summarize the preliminary effectiveness of surgical treatment of Erdheim-Chester disease（ECD）, so as to improve the understanding of the disease by orthopedic surgeons. Methods The clinical data of9 patients with ECD between December 2012 and October 2017 were retrospectively analysed. There were 6 males and3 females with an median age of 42 years（range, 8-61 years）. The disease duration was 4-59 months（mean, 39 months）.There were 2 cases of multiple lesions, including 1 case involving soft tissue of the buttocks and bilateral tibia, 1 case involving the sinus, skull base, and proximal right tibia; 7 cases with single lesion, including 3 cases of right femoral neck,1 case of proximal right tibia, 1 case of right humerus, and 2 cases of ribs. Nine patients were diagnosed according to clinical manifestations, imaging examination, and pathological diagnosis. Four patients underwent needle biopsy before operation and 5 patients were diagnosed by postoperative pathology examination. Five cases underwent lesional scraping and internal fixation, 1 case underwent bone scraping and bone grafting, and 3 cases underwent lesion resection. One of the multiple lesions was treated with interferon and hormone. Results Nine patients underwent the surgery safely.There was no fever, wound exudation, infection, etc., and the incisions healed by first intention. All the patients were followed up 4-59 months with an average of 31.4 months. One patient with bilateral tibia and hip soft tissue involvement continued to receive medical treatment, and the tumor was controlled without significant increasing. The remaining8 patients were examined for X-ray films at 3, 6, and 12 months after surgery, the bone has been fused and the steel plate and intramedullary nail were firmly fixed, and no tumor recurrence was observed. At 1 year after surgery, the pain symptoms of the patients improved and returned to normal life; 3 of them who involving the right femoral neck walked freely, and the quality of life improved signif

关 键 词：ERDHEIM-CHESTER病 脂性肉芽肿病 手术治疗 朗格汉斯组织细胞增生症 

分 类 号：R687.3[医药卫生—骨科学]