Fernández V José Pablo, Chica H Gabriela, Goycoolea R Andrés
Instituto de Neurocirugía Asenjo, Santiago, Chile.
Universidad de Chile, Santiago, Chile.
Rev Chil Pediatr. 2020 Dec;91(6):953-960. doi: 10.32641/rchped.vi91i6.1470. Epub 2020 Oct 8.
Craniosynostosis is defined as the premature fusion of one or more skull sutures, characterized by an abnormal shape of the head. It is a rare condition but should be recognized and timely referred to Neurosurgery in order to prevent complications. The objective of this review is to describe the most frequent clinical and genetic characteristics of this pathology, its classification according to the shape of the skull, and the most characteristic signs to achieve timely recognition. A search for scientific articles in Pubmed, Scielo, and EMBASE databases was performed using the terms craniosynostosis, plagiocephaly, scaphocephaly, and brachycephaly. We selected articles in Spanish and English that described the characteristics of the pathology and about its management, choosing systematic reviews or recommendations from scientific societies when available. Craniosynostosis may occur in isola tion or associated with other deformities. Its classification depends on the affected suture(s), leading to the characteristic shape of the skull and the presence of other malformations. This condition is usually diagnosed and referred late, which is associated with complications such as intracranial hy pertension and impaired brain development. Early surgery has less comorbidity and better esthetic results. In conclusion, the abnormal shape of the skull must raise the suspicion of craniosynostosis, even if it occurs in isolation. Surgical management before one year of life is associated with a better prognosis.
颅缝早闭被定义为一条或多条颅骨缝过早融合,其特征是头部形状异常。这是一种罕见的病症,但应予以识别并及时转诊至神经外科,以预防并发症。本综述的目的是描述这种疾病最常见的临床和遗传特征、根据颅骨形状进行的分类以及实现及时识别的最典型体征。我们使用颅缝早闭、斜头畸形、舟状头畸形和短头畸形等术语在PubMed、Scielo和EMBASE数据库中检索科学文章。我们选择了用西班牙语和英语描述该疾病特征及其治疗的文章,如有可用的系统评价或科学协会的建议则优先选择。颅缝早闭可能单独发生或与其他畸形相关。其分类取决于受累的颅骨缝,导致颅骨呈现特征性形状以及其他畸形的存在。这种情况通常诊断和转诊较晚,这与颅内高压和脑发育受损等并发症相关。早期手术的合并症较少且美容效果更好。总之,即使颅骨异常形状单独出现,也必须怀疑颅缝早闭。一岁前进行手术治疗预后更好。