Kasabach-Merritt综合征治疗中自体原位植皮术的应用

目的 对Kasabach-Merritt综合征手术治疗过程中使用自体原位植皮术,评价其治疗效果,提出一种新的手术治疗方式.方法 回顾性分析2010年至2015年间自体原位植皮手术治疗的Kasabach-Merritt综合征患儿23例,病例纳入标准:均为瘤体分布弥散,皮肤大面积(＞5 cm×5 cm)异常,手术切除瘤体后创面缝合困难;非手术治疗方法无效;血小板值在2×109/L～31×109/L.23例Kasabach-Merritt综合征患儿瘤体均位于四肢,上肢6例,下肢17例;男9例,女14例;年龄7 d～9个月.总结该手术治疗方法的手术要点及术中、术后处理,分析治愈率及术后并发症.术后均经病理学检查证实为卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)20例,丛状血管瘤(tufted angioma,TA)3例.结果 23例患儿均在术后1周内血小板值恢复正常,随访1～5年,治愈率为100％;18例患儿自体皮片原位移植全部成活,有3例成活面积约90％,经后续换药处理后愈合,2例原位皮片移植成活约60％,行二期皮肤移植修补手术后治愈,术后并发症少.结论 手术治疗Kasabach-Merritt综合征可作为有效的治疗方法,本组资料显示,自体原位植皮术作为一种新的手术方式,治愈率100％,术后并发症少,其对符合适应证者是一种可选择治疗手段.

Application of autologous skin grafting for Kasabach-Merritt syndrome

Objective To introduce one surgical approach for Kasabach-Merritt syndrome by autologous skin grafting and evaluated the effect of surgical treatment.Methods Retrospective analysis of autologous skin grafting was performed for 23 cases of Kasabach-Merritt syndrome.The area of tumor dispersion was ＞5 cm × 5 cm.Non-operative treatment was ineffective.Platelet count was under 2-31 × 109/L.All patients were confirmed postoperatively by pathological examination.The pathological diagnoses were KHE (kaposiform hemangioendothelioma,n =20) and TA (tufted angioma,n =3).Results All platelet counts recovered to normal within at 1 week post-operation.During a follow-up period of 1-5 years,the curative rate was 100％.And autologous skin grafts survived 100％ (n =18),90％ & healed after dressing (n =3) and 60％ & healed after repairing (n =2).Conclusions Autologous skin grafting is efficacious and has few complications for KasabachMerritt syndrome,especially tumor involvement of large skin area.