The metopic-sagittal craniosynostosis—report of 35 operative cases

• Published in: Child's nervous system Vol. 33; no. 8; pp. 1335 - 1348
• Main Authors: Shimoji, Takeyoshi, Kimura, Takaoki, Shimoji, Kazuaki, Miyajima, Masakazu
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.08.2017
• Subjects: Age Distribution; Child; Child, Preschool; Craniosynostoses - diagnostic imaging; Craniosynostoses - physiopathology; Craniosynostoses - surgery; Female; Humans; Imaging, Three-Dimensional; Infant; Intracranial Pressure - physiology; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Neurosciences; Neurosurgery; Neurosurgical Procedures - methods; Original Paper; Sex Distribution; Sphenoid Bone - surgery; Treatment Outcome; Sphenoid ridge; Mild trigonocephaly; Metopic-sagittal synostosis; Developmental delays
• ISSN: 0256-7040; 1433-0350; 1433-0350
• DOI: 10.1007/s00381-017-3430-1

Purpose 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. Methods 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. Results Surgical intervention improved clinical symptoms in nearly all 35 patients; cosmetic problems in patients with scaphocephaly were also corrected. Conclusions 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.