先天性营养不良型大疱表皮松解症的手功能重建

The Hands Functional Reconstruction of the Dystrophic Congenital Epidermolysis Bullosa Patients

目的探讨改善先天性营养不良型大疱表皮松解症患者手部挛缩畸形并重建手功能的方法。方法在传统治疗原则基础上,对8例大疱表皮松解症患者进行系统治疗,通过手术、ABC法包扎和换药等方法,改善和重建手功能。结果本组8例患者均取得满意疗效,术后第8周手部创面愈合,表皮生长并覆盖双手,手指能主动背伸和屈曲,手功能及外观均较满意。结论手术方法重建先天性营养不良型大疱表皮松解症患者手部功能,疗效显著,改变了对大疱表皮松解症双手畸形治疗的认识,具有广泛的临床推广价值。

Objective To introduce and investigate the operation, which can improve the hand contracture deformity of dystrophic epidermolysis bullosa patients, reconstruct the function of the hands. Methods On the base of traditional way, operating for eight dystrophic epidermolysis bullosa patients , wrapping their hands by ABC way, changing their dressing in time and others postoperative treatment, their function of hands are restored and improved. Results Eight patients are all satisfied with operation results, especially, the appearances and functions. The wounds of hands are heal at the eighth week, the epidermis of hands are growth, and the fingers can flex and extend actively. Conclusion This method for dystrophic epidermolysis bullosa patients, the curative effect is perfect without special complications, changing the recognition of the treatment of the hand contracture deformity of epidermolysis bullosa patients, which should spread in clinic.