Takeyoshi Shimoji1, Takaoki Kimura2, Kazuaki Shimoji2, Masakazu Miyajima2. 1. Department of Neurosurgery, Amekudai Hospital, 1123, Ameku, Naha, Okinawa, 900-0005, Japan. trigono.research@gmail.com. 2. Department of Neurosurgery, Juntendo University School of Medicine, 2-1-1, Hongou Bunkyou-ku, Tokyo, 113-8421, Japan.
Abstract
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.
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 childpatients 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.
Authors: Daniel G Morritt; Fang-Jui J Yeh; Steve A Wall; Peter G Richards; Jayaratnam Jayamohan; David Johnson Journal: Plast Reconstr Surg Date: 2010-08 Impact factor: 4.730