Santos de Oliveira Ricardo, Barros Jucá Carlos Eduardo, Lopes Lins-Neto Antônio, Aparecida do Carmo Rego Maria, Farina Jaime, Machado Helio Rubens
Division of Pediatric Neurosurgery, Ribeirão Preto School of Medicine, University of São Paulo, Campus Universitário, 14049-900 Ribeirão Preto, Brazil.
Childs Nerv Syst. 2006 Sep;22(9):1072-9. doi: 10.1007/s00381-006-0074-y. Epub 2006 Apr 26.
Aplasia cutis congenita (ACC) is a rare disease of unknown etiology, involving any site of the body. The scalp is the most frequent location, followed by the forearms, knees, both sides of the trunk, and neck, in decreasing order of frequency. Superficial lesions may heal spontaneously and seldom result in morbidity or mortality. However, in patients with large scalp and skull defects, there are risks of infection and bleeding. Conservative treatment has been described and advocated, but some authors have highlighted the disadvantages of this treatment modality. On the other hand, several authors claim that aggressive surgical treatment has an important role for large defects.
In this paper, we report three newborns with ACC of the scalp, two large defects and a medium one, respectively, treated conservatively and surgically.
The management of ACC of the scalp is still controversial. Our series suggests that conservative treatment should be performed for initial management in newborns.
先天性皮肤发育不全（ACC）是一种病因不明的罕见疾病，可累及身体的任何部位。头皮是最常受累部位，其次是前臂、膝盖、躯干两侧和颈部，按频率递减顺序排列。浅表病变可能会自愈，很少导致发病或死亡。然而，对于有大面积头皮和颅骨缺损的患者，存在感染和出血风险。已有保守治疗的描述和倡导，但一些作者强调了这种治疗方式的缺点。另一方面，一些作者声称积极的手术治疗对大面积缺损具有重要作用。
在本文中，我们报告了3例头皮ACC新生儿，分别有2例大面积缺损和1例中等面积缺损，分别接受了保守治疗和手术治疗。
头皮ACC的治疗仍存在争议。我们的系列研究表明，新生儿初始治疗应采用保守治疗。
