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• W2897595547 abstract "A 5-year-old boy was referred for evaluation of dysmorphic facial features. Examination revealed a small forehead, bilateral proptosis, hypertelorism, flat nasal bridge, hypoplasia of the maxilla, and overcrowding of teeth. Near similar facial features were seen in his mother and younger brother. Genetic evaluation of the brothers and mother revealed FGFR2 10q26.13 gene mutation. Crouzon syndrome was diagnosed. This syndrome is characterized by premature craniosynostosis and inherited as an autosomal-dominant trait. His skull radiograph showed prominent convolutional markings described as a copper beaten skull appearance (Figure 1, A and B). A computed tomography scan of the head with 3-dimensional reconstruction of the skull revealed fusion of all sutures and a diffuse prominent gyral impressions on the inner table of skull vault (Figure 2; available at www.jpeds.com). In chronic, raised intracranial pressure (ICP) owing to any etiology, the growing brain exerts a continuous pulsatile pressure on the malleable cranium and produces a gyral pattern on the inner table of the calvarium, evident on plain skull radiographs. The resultant copper beaten skull pattern was once presumed to be a marker of raised ICP, but currently it is widely considered to be a reflection of normal brain growth during periods of rapid brain growth between ages 2-3 and 5-7 years.1Tuite G.F. Evanson J. Chong W.K. Thompson D.N. Harkness W.F. Jones B.M. et al.The beaten copper cranium: a correlation between intracranial pressure, cranial radiographs, and computed tomographic scans in children with craniosynostosis.Neurosurgery. 1996; 39: 691-699Crossref PubMed Scopus (106) Google Scholar, 2Van der Meulen J. van der Vlugt J. Okkerse J. Hofman B. Early beaten-copper pattern: its long-term effect on intelligence quotients in 95 children with craniosynostosis.J Neurosurg Pediatr. 2008; 1: 25-30Crossref PubMed Scopus (18) Google Scholar This pattern is not specific for either Crouzon syndrome or raised ICP. When clinical suspicion of raised ICP is high and only skull radiography is available, other specific features including skull demineralization, splitting of the sutures, and erosion or enlargement of the sella turcica should be examined for more timely intervention. Our patient had no symptoms of raised ICP. His fundus examination was normal and there was no developmental delay. He was advised for close follow-up." @default.
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• W2897595547 date "2019-03-01" @default.
• W2897595547 modified "2023-09-30" @default.
• W2897595547 title "Copper Beaten Skull" @default.
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• W2897595547 doi "https://doi.org/10.1016/j.jpeds.2018.09.041" @default.
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