Shimoji Takeyoshi, Kimura Takaoki, Shimoji Kazuaki, Miyajima Masakazu
Department of Neurosurgery, Amekudai Hospital, 1123, Ameku, Naha, Okinawa, 900-0005, Japan.
Department of Neurosurgery, Juntendo University School of Medicine, 2-1-1, Hongou Bunkyou-ku, Tokyo, 113-8421, Japan.
Childs Nerv Syst. 2017 Aug;33(8):1335-1348. doi: 10.1007/s00381-017-3430-1. Epub 2017 May 3.
We have diagnosed 35 cases of the supposedly rare condition metopic-sagittal synostosis in the past 20 years. Here, we introduce their clinical symptoms, neuroradiological findings, and surgical treatment methods, as well as discuss the relevant literature.
Subjects included 35 patients (33 boys and 2 girls; mean age 4.2 years; range 1-8 years). Magnetic resonance imaging (MRI) confirmed that there were no abnormal findings in the brain. Thirty patients presented with symptoms including speech delay, hyperactivity, autistic tendency, motor impairment, self-mutilation, and panic/temper tantrum behaviors. No other congenital malformation was observed, and all cases were considered to be the non-syndromic type. The final diagnosis was made using three-dimensional computed tomography (3D-CT) scans. The surgery was done the fronto-orbital advancement in addition to remove the large parts of sphenoid bones including sphenoid ridges at the skull base and trimmed the calvarium as necessary to reduce pressure.
Surgical intervention improved clinical symptoms in nearly all 35 patients; cosmetic problems in patients with scaphocephaly were also corrected.
In the cases of child patients with metopic-sagittal synostosis who had clinical symptoms, surgical intervention improved such symptoms, suggesting its potential utility for metopic-sagittal synostosis with clinical symptoms. A surgical procedure focusing on the skull base was important for our successes. Based on the fact that metopic-sagittal synostosis was diagnosed in 35 patients at one institution over a relatively short period of time, this pathological condition may not be as rare as is currently believed.
在过去20年里，我们诊断出35例疑似罕见的额缝矢状缝早闭病例。在此，我们介绍其临床症状、神经放射学表现及手术治疗方法，并讨论相关文献。
研究对象包括35例患者（33例男性，2例女性；平均年龄4.2岁；年龄范围1 - 8岁）。磁共振成像（MRI）证实脑部无异常发现。30例患者出现言语发育迟缓、多动、自闭症倾向、运动障碍、自残以及惊恐/发脾气行为等症状。未观察到其他先天性畸形，所有病例均被认为是非综合征型。最终诊断采用三维计算机断层扫描（3D - CT）。手术采用额眶前移术，同时切除包括颅底蝶骨嵴在内的大部分蝶骨，并根据需要修整颅骨以减轻压力。
手术干预使几乎所有35例患者的临床症状得到改善；舟状头畸形患者的美容问题也得到了纠正。
对于有临床症状的儿童额缝矢状缝早闭病例，手术干预改善了这些症状，表明其对有临床症状的额缝矢状缝早闭具有潜在应用价值。针对颅底的手术操作对我们的成功至关重要。基于在一个机构相对较短时间内诊断出35例额缝矢状缝早闭患者这一事实，这种病理状况可能不像目前认为的那样罕见。
