Rosalinda Calandrelli1, Fabio Pilato2, Antonio Marrazzo3, Luca Massimi4,5, Marco Panfili3, Concezio Di Rocco6, Cesare Colosimo3,7. 1. Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma-UOC Radiologia e Neuroradiologia, Polo Diagnostica per immagini, radioterapia, oncologia ed ematologia, Area diagnostica per immagini, Largo Francesco Vito 1, -00168, Rome, Italy. rosalinda.calandrelli@policlinicogemelli.it. 2. Unit of Neurology, Neurophysiology, Department of Medicine, University Campus Bio-Medico, via Álvaro del Portillo 21, 00128, Rome, Italy. 3. Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma-UOC Radiologia e Neuroradiologia, Polo Diagnostica per immagini, radioterapia, oncologia ed ematologia, Area diagnostica per immagini, Largo Francesco Vito 1, -00168, Rome, Italy. 4. Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma -UOC Neurochirurgia Infantile - Polo scienze dell'invecchiamento, neurologiche, ortopediche e della testa-collo, Area Neuroscienze, Rome, Italy. 5. Università Cattolica del Sacro Cuore, Istituto di Neurochirurgia, Rome, Italy. 6. International Neuroscience Institute, Hannover, Germany. 7. Università Cattolica del Sacro Cuore, Istituto di Radiologia, Rome, Italy.
Abstract
PURPOSE: Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation. METHODS: High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the "major" and "minor" sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index. RESULTS: Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2 infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p<0.05). CONCLUSIONS: Our data show that FGFR2 mutation together with posterior coronal branch synostotic involvement has a synergic effect in causing a more severe degree of orbital proptosis.
PURPOSE: Evaluation of orbital proptosis and sutural synostosis pattern along the coronal ring in craniofaciosynostosis patients with or without fibroblastic growth factor receptor 2 (FGFR2) mutation. METHODS: High-resolution computer tomography was used to assess, in children with or without FGFR2 mutation, the early synostotic involvement of the "major" and "minor" sutures/synchondroses of the coronal arch along with the following orbital parameters: interorbital angle, bone orbital cavity volume, globe volume, ventral globe volume, ventral globe index. RESULTS:Infants with FGFR2 mutation showed an increased number of closed minor sutures/synchondroses along the posterior coronal branch while both groups showed a comparable synostotic involvement of the minor sutures of the anterior coronal branch. FGFR2infants with posterior coronal branch synostotic involvement showed a higher degree of proptosis due to both reduced bony cavity volume and increased globe volume (p<0.05). CONCLUSIONS: Our data show that FGFR2 mutation together with posterior coronal branch synostotic involvement has a synergic effect in causing a more severe degree of orbital proptosis.
Authors: Antonio Jorge Forte; Derek M Steinbacher; John A Persing; Eric D Brooks; Tom W Andrew; Nivaldo Alonso Journal: Plast Reconstr Surg Date: 2015-11 Impact factor: 4.730