Sagittal synostosis, the premature closure of the sagittal suture, accounts for more than 50% of all nonsyndromic single-suture synostoses. Although no detrimental neurologic effects can be directly attributed to the synostosis, a number of patients will have relatively increased intracranial pressure. Surgical correction for sagittal synostosis has evolved from simple removal of bone strips to extensive cranial remodeling, all in a attempt to achieve a normal head shape. The lack of outcome measures has limited the surgeon's ability to choose one surgical procedure over another. The use of a cranial helmet for passive molding of the head after surgery is effective when used with limited endoscopic surgery. We present the results of using an extended strip craniectomy combined with long-duration molding helmet therapy and compare it with other reported methods. The results suggest that passive molding with the helmet may have a greater effect on cranial shape than surgery.
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http://dx.doi.org/10.1016/j.spen.2004.11.006 | DOI Listing |
Bone Res
January 2025
Université de Paris Cité, Imagine Institute, Laboratory of Molecular and Physiopathological Bases of Osteochondrodysplasia, INSERM UMR 1163, Paris, France.
Gain-of-function mutations in fibroblast growth factor receptor (FGFR) genes lead to chondrodysplasia and craniosynostoses. FGFR signaling has a key role in the formation and repair of the craniofacial skeleton. Here, we analyzed the impact of Fgfr2- and Fgfr3-activating mutations on mandibular bone formation and endochondral bone repair after non-stabilized mandibular fractures in mouse models of Crouzon syndrome (Crz) and hypochondroplasia (Hch).
View Article and Find Full Text PDFCleft Palate Craniofac J
January 2025
Division of Plastic and Reconstructive Surgery, The Warren Alpert Medical School of Brown University, Providence, RI, USA.
Craniosynostosis, a condition marked by the premature fusion of one or more cranial sutures, exhibits diverse phenotypes. This study aims to advance the understanding of these phenotypes beyond the conventional 2-dimensional analysis by focusing on identifying indicators of increased intracranial pressure (ICP) such as bony thinning or irregularities in skull morphology. A retrospective review was conducted for all pediatric patients with midline craniosynostosis who presented to our tertiary academic center for evaluation.
View Article and Find Full Text PDFJ Craniofac Surg
January 2025
Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Objective: There is a lack of comprehensive comparative evidence regarding the effectiveness, intraoperative management, and safety of different surgical procedures for treating nonsyndromic sagittal synostosis. This study aims to evaluate existing clinical studies to provide evidence-based guidance for clinical practice.
Methods: The authors performed a comprehensive search of 5 databases up to August 2024.
Taiwan J Obstet Gynecol
January 2025
Gynecology and Obstetrics Clinic "Narodni front", Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
Objective: Prenatal detection of complex chromosomal rearrangements (CCR) is extremely rare, but is of great clinical importance, since CCR can be causative of different congenital disorders. We present an exceptionally rare case of prenatally diagnosed Saethre-Chotzen syndrome (SCS) rising as a consequence of chromothripsis involving chromosomes 5, 7 and 11 and deletion of TWIST1 gene.
Case Report: Brachycephaly, hypertelorism, flat face, micrognathia, relative macroglossia and small posterior fossa were noted on ultrasound examination at 28th gestational week.
J Craniofac Surg
January 2025
Department of Biomedical Engineering, University of Illinois, Chicago, IL.
Bioabsorbable internal fixation is a well-accepted modality that is especially suitable for application in craniosynostosis. When first introduced, high rates of adverse tissue reactions were observed that have since been ameliorated with more biocompatible polymer formulations. However, the phenomenon has not entirely disappeared, and such reactions remain vexing.
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