Guillaume Coll1, Laurent Sakka2, Céline Botella3, Nathalie Pham-Dang4, Corine Collet5, Michel Zerah6, Eric Arnaud6, Federico Di Rocco7. 1. Unité de Chirurgie Craniofaciale, Service de Neurochirurgie Pédiatrique, Centre de Référence National des Dysostoses Crâniofaciales, Hôpital Necker-Enfants Malades, APHP, Paris, France; Service de Neurochirurgie, Hôpital Gabriel Montpied, Clermont-Ferrand, France; Image-Guided Clinical Neurosciences and Connectomics (IGCNC), Axe thérapies guidées par l'image (TGI), CNRS, Sigma, Institut Pascal, Université Clermont Auvergne, Clermont-Ferrand, France. Electronic address: collguillaume@me.com. 2. Service de Neurochirurgie, Hôpital Gabriel Montpied, Clermont-Ferrand, France; Université Clermont Auvergne, Université d'Auvergne, Laboratoire d'Anatomie et d'Organogenèse, NeuroDol UMR INSERM 1107 CNRS, Université Clermont Auvergne, Clermont-Ferrand, France. 3. Service de Neurochirurgie, Hôpital Gabriel Montpied, Clermont-Ferrand, France. 4. Service de Chirurgie Maxillo-Faciale, Hôpital Estaing, Clermont-Ferrand, France. 5. Service de Biochimie et Biologie Moléculaire, Hôpital Lariboisière, APHP, Paris, France. 6. Unité de Chirurgie Craniofaciale, Service de Neurochirurgie Pédiatrique, Centre de Référence National des Dysostoses Crâniofaciales, Hôpital Necker-Enfants Malades, APHP, Paris, France. 7. Unité de Chirurgie Craniofaciale, Service de Neurochirurgie Pédiatrique, Centre de Référence National des Dysostoses Crâniofaciales, Hôpital Necker-Enfants Malades, APHP, Paris, France; Service de Neurochirurgie Pédiatrique, Hôpital Femme Mère Enfant, Lyon, France; Université Claude Bernard, Lyon, France.
Abstract
BACKGROUND: The age of closure of skull base synchondroses has never been analyzed in a homogenous population of children with Crouzon syndrome. METHODS: A retrospective case-control study was performed on 30 Crouzon children (17 male, 13 female) aged 1 month to 12.48 years with Fibroblast Growth Factor Receptor type 2 mutation. Eleven synchondroses were analyzed on millimetric computed tomodensitometric slices before surgery. Syndromic patients were compared with a series of 235 healthy children previously published. RESULTS: Synchondrosis closure follows a global pattern that occurs earlier in Crouzon syndrome than in controls (P ≤ 0.002). Synchondrosis fusion starts at 10 months of age with posterior intraoccipital synchondroses and lambdoid sutures, followed by occipitomastoid synchondroses between 1.85 (right) and 2.27 years (left) and anterior intraoccipital synchondroses at approximately 2.80 years. Time to complete fusion varies considerably according to the synchondroses. Spheno-occipital and petro-occipital synchondroses fuse last, at approximately 3 years old. CONCLUSIONS: In children with Crouzon syndrome, synchondrosis closure occurs prematurely, with a time course specific to each synchondrosis.
BACKGROUND: The age of closure of skull base synchondroses has never been analyzed in a homogenous population of children with Crouzon syndrome. METHODS: A retrospective case-control study was performed on 30 Crouzon children (17 male, 13 female) aged 1 month to 12.48 years with Fibroblast Growth Factor Receptor type 2 mutation. Eleven synchondroses were analyzed on millimetric computed tomodensitometric slices before surgery. Syndromic patients were compared with a series of 235 healthy children previously published. RESULTS:Synchondrosis closure follows a global pattern that occurs earlier in Crouzon syndrome than in controls (P ≤ 0.002). Synchondrosis fusion starts at 10 months of age with posterior intraoccipital synchondroses and lambdoid sutures, followed by occipitomastoid synchondroses between 1.85 (right) and 2.27 years (left) and anterior intraoccipital synchondroses at approximately 2.80 years. Time to complete fusion varies considerably according to the synchondroses. Spheno-occipital and petro-occipital synchondroses fuse last, at approximately 3 years old. CONCLUSIONS: In children with Crouzon syndrome, synchondrosis closure occurs prematurely, with a time course specific to each synchondrosis.
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