Drolet B A, Clowry L, McTigue M K, Esterly N B
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, USA.
Pediatrics. 1995 Aug;96(2 Pt 1):309-13.
To call attention to a cutaneous marker for neural tube closure defects of the scalp, the "hair collar" sign. This finding consists of a ring of long, dark, coarse hair surrounding a midline scalp nodule.
Four children with small congenital scalp nodules and the hair collar sign were studied from the standpoint of clinical findings, radiologic scans, and histology of the excised nodules. All four had an overlying vascular stain in addition to the hair collar. Patients 1 and 2 were found to have encephaloceles, and one had heterotopic brain tissue. The fourth family refused surgery, but the clinical and radiologic findings were consistent with a diagnosis of atretic encephaloceles. One infant had agenesis of the corpus callosum and a Dandy-Walker malformation as associated findings.
The "hair collar" sign should alert the pediatrician to the possibility of ectopic neural tissue in the scalp and/or underlying central nervous system malformations.
引起对头皮神经管闭合缺陷的一种皮肤标志物——“发圈”征的关注。这一发现表现为围绕头皮中线结节的一圈长而黑且粗糙的毛发。
从临床发现、放射学扫描以及切除结节的组织学角度，对4例患有先天性小头皮结节及发圈征的儿童进行了研究。所有4例除发圈外均有覆盖其上的血管痣。发现患者1和患者2患有脑膨出，其中1例有异位脑组织。第4个家庭拒绝手术，但临床和放射学检查结果与闭锁性脑膨出的诊断相符。1例婴儿伴有胼胝体发育不全和Dandy-Walker畸形。
“发圈”征应提醒儿科医生注意头皮存在异位神经组织和/或潜在中枢神经系统畸形的可能性。
