Laumonier F, Varlet F, Favier T, Berthelot J, Pouplard F
Service de Chirurgie Pédiatrique, Centre Hospitalier Régional Universitaire, Angers.
Chir Pediatr. 1990;31(2):106-12.
Report of four cases of congenital defects of the scalp. In two of these cases, the skin defect was associated with a skull defect and in one, there were other cranial deformities. In each case, the lesion seemed isolated in otherwise normal children. This study contains a review of congenital scalp defects. The different aspects of this rare disease are presented and the treatment is discussed with reference to natural evolution. The authors differentiate between cases with isolated lesion and cases with associated malformations or abnormalities, malformation syndromes, as has been previously defined. In the case of skull defects, it is thought that immediate reparatory surgery must be performed, to prevent infection and hemorrhage of sagittal venous sinus, as it was the case in one of the four children. The covering of the defect can be realized with rotated skin flaps, which seem preferable to any form of plastic surgery, for cosmetic reasons, and for the secondary treatment of the skull defect, if this is necessary. When spontaneous closure of the bone defect, is not noted, which occurs generally in the case of large defects, it seems reasonable to close the latter by cranio-plasty, to protect the brain. In one of the four cases reported, this technical approach of the lesion was used, with a good result being reported, four years later.
四例先天性头皮缺损报告。其中两例皮肤缺损伴有颅骨缺损，一例存在其他颅骨畸形。在每例中，病变在其他方面正常的儿童中似乎是孤立存在的。本研究包含对先天性头皮缺损的综述。介绍了这种罕见疾病的不同方面，并参照自然病程讨论了治疗方法。作者区分了孤立性病变病例与伴有畸形或异常、畸形综合征的病例，如先前所定义。对于颅骨缺损病例，认为必须立即进行修复手术，以防止矢状窦感染和出血，四例患儿中的一例就是如此。缺损的覆盖可通过旋转皮瓣实现，出于美容原因以及必要时对颅骨缺损进行二期治疗的考虑，旋转皮瓣似乎比任何形式的整形手术更可取。当未观察到骨缺损自然闭合时（这种情况通常发生在大的缺损病例中），通过颅骨成形术闭合骨缺损以保护大脑似乎是合理的。在所报告的四例病例中的一例，采用了这种针对病变的技术方法，四年后报告效果良好。
