Dutra Leandro Brum, Pereira Max Domingues, Kreniski Tessie Maria, Zanon Nelci, Cavalheiro Sérgio, Ferreira Lydia Masako
Division of Plastic Surgery, Federal University of São Paulo (UNIFESP), São Paulo, Brazil.
J Craniofac Surg. 2009 Jul;20(4):1288-92. doi: 10.1097/SCS.0b013e3181ae2108.
Aplasia cutis congenita is a rare disorder characterized by absence of skin. Lesions typically occur on the vertex and are sometimes small, but they can affect deep tissues such as the skull bone and dura. Mortality is related to the depth and size of the lesion and can amount to a rate of more than 50% when full thickness is involved. The treatment remains controversial -- both surgical and conservative managements are described. Minor lesions can be controlled with nonsurgical treatment, but large defects require early surgery. We report the case of a female newborn with acrania and scalp aplasia cutis congenita, which was treated with a bipedicle scalp flap based on the temporal vessels. Full- and partial-thickness skin grafts were used to cover the donor site on the temporo-occipital region. Postoperatively, the patient developed a liquorice cyst, which was treated with a shunt, and she has been followed up for evaluation of the bony defect closure and skull morphology. Her neuropsychomotor development is normal.
先天性皮肤发育不全是一种以皮肤缺失为特征的罕见疾病。病变通常发生在头顶，有时较小，但可累及深部组织，如颅骨和硬脑膜。死亡率与病变的深度和大小有关，当全层受累时，死亡率可达50%以上。治疗仍存在争议——手术治疗和保守治疗均有描述。较小的病变可用非手术治疗控制，但较大的缺损需要早期手术。我们报告了一例患有无头盖畸形和先天性头皮皮肤发育不全的女婴病例，采用基于颞血管的双蒂头皮瓣进行治疗。全厚和部分厚皮片用于覆盖颞枕部的供区。术后，患者出现了甘草囊肿，采用分流术进行治疗，并对其进行随访以评估骨缺损闭合情况和颅骨形态。她的神经心理运动发育正常。
