先天性皮肤缺损三例报告并文献复习

Congenital skin defect with a literature review： a report of 3 cases

目的探讨先天性皮肤缺损的病因、分类、治疗及预后，提高该病的诊治水平。方法回顾性分析我院治疗的3例先天性皮肤缺损患儿发病特点、治疗过程、方法，并复习国内外文献中报道的皮肤缺损305例，总结先天性皮肤缺损的临床特点及诊治经验。结果3例患儿缺损皮肤均由羊皮纸样瘢痕组织愈合，无组织挛缩，无活动障碍。305例患儿中死亡8例；保守治疗234例；手术治疗63例，其中因瘢痕挛缩影响肢体功能者5例。该病致死率、致残率低，预后良好。结论先天性皮肤缺损是一种病因尚不明确的疾病，创面表浅者多可经保守治疗后痊愈，骨外露、重要脏器外露、深度缺损宜适时采用手术治疗，该疾病若能给予正确处理，预后良好，对患儿日常生活影响小。

Objective To explore the etiology, classification, treatment and prognosis of congenital absence of skin so as to improve its management. Methods Retrospective analyses were performed for the clinical characteristics and treatment in 3 cases of congenital absence of skin at our hospital. And 305 cases of congenital absence of skin were collected from the literature. Results Three cases of skin defect healed by parchment-like scar tissue. And there was neither contracture nor dysfunction. Among 305 cases, there were 8 deaths and 234 cases received conservative treatments. And 5/63 surgical cases were affected by scar contracture. The mortality and disability rates were low. Conclusions The etiology of congenital skin defect has remained elusive. Superficial wound may be managed conservatively. Cases of bone exposure, vital organ exposure and deep defect should be operated. If treated properly, the prognosis is excellent without any significant impact of daily life.