Shivakumar S K, Dwarakanath S, Swaroop Gopal, Venkataramana N K
Department of Neurosurgery, Manipal Institute for Neurological Disorders, Bangalore, 560 017, Karnataka, India.
Neurol India. 2006 Sep;54(3):312-3. doi: 10.4103/0028-3886.27165.
Agenesis of scalp is an uncommon but well-recognized clinical entity. Congenital scalp and skull defects can be either obvious or occult; over 300 cases have been reported in literature. Aplasia cutis congenita (ACC) is recognized as a heterogeneous disorder, all characterized by focal absence of the epidermis, dermis and sometimes the calvarium and/or dura. We present a case of ACC in an infant whose mother was exposed to a teratogenic drug (Methimazole--an antithyroid drug) during pregnancy. This case report is presented to highlight the steps to successful management. Definitive full thickness scalp cover at the earliest avoids secondary infection, eschar formation and exsanguination.
头皮发育不全是一种罕见但已被充分认识的临床病症。先天性头皮和颅骨缺损可明显或隐匿;文献中已报道300多例。先天性皮肤发育不全(ACC)被认为是一种异质性疾病,其特征均为表皮、真皮局灶性缺失,有时颅骨和/或硬脑膜也缺失。我们报告一例婴儿患ACC的病例,其母亲在孕期接触了致畸药物(甲巯咪唑——一种抗甲状腺药物)。本病例报告旨在强调成功治疗的步骤。尽早进行确定性全层头皮覆盖可避免继发感染、焦痂形成和失血。