Cloverleaf skull in a 5-month toddler (Apert syndrome). A: characteristic cloverleaf skull. B: MRI showing cranial conformation. Severe compromise of the parieto-occipital area (arrow). Brain herniation through the anterior region of the skull. Hydrocephalus is present. C: MRI after parieto-occipital remodelling: the anterior aspect of the skull has improved its appearance. D: postoperative aspect of the patient after parietal-occipital remodelling and expanding posterior fossa craniectomy performed in a second stage. 

Cloverleaf skull in a 5-month toddler (Apert syndrome). A: characteristic cloverleaf skull. B: MRI showing cranial conformation. Severe compromise of the parieto-occipital area (arrow). Brain herniation through the anterior region of the skull. Hydrocephalus is present. C: MRI after parieto-occipital remodelling: the anterior aspect of the skull has improved its appearance. D: postoperative aspect of the patient after parietal-occipital remodelling and expanding posterior fossa craniectomy performed in a second stage. 

Source publication
Article
Full-text available
Objective. To review the results and complications of the surgical treatment of craniosynostosis in 283 consecutive patients treated between 1999 and 2007. Patients and methods. Our series consisted of 330 procedures performed in 283 patients diagnosed with scaphocephaly (n=155), trigonocephaly (n=50), anterior plagiocephaly (n=28), occipital plagi...

Similar publications

Article
Full-text available
INTRODUCAO: A craniossinostose coronal unilateral (CCU) e a ossificacao prematura da sutura coronal unilateralmente e provoca uma deformidade em 3 dimensoes, que pode afetar o crânio e as orbitas. O objetivo do presente trabalho foi aferir e comparar a assimetria do crânio e face dos pacientes portadores de CCU no periodo pre e pos-operatorio. METO...
Article
Full-text available
A primeira revisão das ossificações prematuras das suturas da calvária e seus efeitos na forma e aparência do crânio data de 1858, tendo sido publicada por Virchow, que as denominou de craniossinostoses. Quando uma configuração anormal da calvária é detectada, a avaliação radiológica é necessária para caracterizar a deformidade e para guiar o proce...
Article
Full-text available
Deformation of the cranium in infancy represents a spectrum of deformity, ranging from severe asymmetric yet proportional distortion of the skull in plagiocephaly, to nearly symmetric yet disproportional distortion in brachycephaly. As such, the condition is best described as deformational plagiocephaly-brachycephaly with isolated plagiocephaly and...
Article
Full-text available
Objective: The aim of the study was to determine the intra-and inter-reliability for measuring infants with plagiocephaly and brachycephaly with a craniometer when using a marked headband as landmarks. Subjects: Six physiotherapists and eight infants participated in the study. Methods: The physiotherapists measured all infants twice; each infant wa...
Article
Full-text available
Introduction Le nombre de plagiocéphalies sans synostose (PSS) a fortement augmenté depuis une dizaine d'années. Son impact sur la mandibule reste mal compris d'autant qu'il existe différentes formes de PSS. Cette étude a pour but d'analyser la déformation mandibulaire en fonction de la déformation des PSS. Matériel et méthodes L'étude a été réali...

Citations

Article
Full-text available
The incidence of craniosynostosis is one in every 1,800-2500 births. The gene-environment model proposes that if a genetic predisposition is coupled with environmental exposures, the effects can be multiplicative resulting in severely abnormal phenotypes. At present, very little is known about the role of gene-environment interactions in modulating craniosynostosis phenotypes, but prior evidence suggests a role for endocrine factors. Here we provide a report of the effects of thyroid hormone exposure on murine calvaria cells. Murine derived calvaria cells were exposed to critical doses of pharmaceutical thyroxine and analyzed after 3 and 7 days of treatment. Endpoint assays were designed to determine the effects of the hormone exposure on markers of osteogenesis and included, proliferation assay, quantitative ALP activity assay, targeted qPCR for mRNA expression of Runx2, Alp, Ocn, and Twist1, genechip array for 28,853 targets, and targeted osteogenic microarray with qPCR confirmations. Exposure to thyroxine stimulated the cells to express ALP in a dose dependent manner. There were no patterns of difference observed for proliferation. Targeted RNA expression data confirmed expression increases for Alp and Ocn at 7 days in culture. The genechip array suggests substantive expression differences for 46 gene targets and the targeted osteogenesis microarray indicated 23 targets with substantive differences. 11 gene targets were chosen for qPCR confirmation because of their known association with bone or craniosynostosis (Col2a1, Dmp1, Fgf1, 2, Igf1, Mmp9, Phex, Tnf, Htra1, Por, and Dcn). We confirmed substantive increases in mRNA for Phex, FGF1, 2, Tnf, Dmp1, Htra1, Por, Igf1 and Mmp9, and substantive decreases for Dcn. It appears thyroid hormone may exert its effects through increasing osteogenesis. Targets isolated suggest a possible interaction for those gene products associated with calvarial suture growth and homeostasis as well as craniosynostosis.