Active clinical trials for "Craniosynostoses"

Oral iron is commonly used in conjunction with EPO preoperatively for hemorrhagic surgeries in children and especially in the surgery of craniosynostosis. The bioavailability of oral iron is low and compliance with treatment is inconsistent. The aim of this study is to evaluate whether the use of ferric carboxymaltose by injection, which has a much better bioavailability, would make it possible to increase the preoperative hemoglobin level more effectively and thus reduce the risk of perioperative blood transfusion .

Endoscopic strip craniectomy (ESC) with post-operative helmeting is the gold-standard treatment for isolated, non-syndromic sagittal craniosynostosis in children under 6 months of age as it is has been demonstrated to reduce perioperative morbidity when compared to more invasive procedures such as cranial vault remodeling. ESC is frequently performed with or without the use of lateral osteotomies with technical selection being largely based on surgeon preference. Previous studies have shown that there are no statistically significant differences in cranial expansion or complications between the two procedure variants; however, these studies are retrospective in nature and do not account for aesthetic outcomes. The purpose of this study is to compare the efficacy of ESC with or without the use of lateral osteotomies in regard to cranial expansion and aesthetic outcomes for children treated with isolated, non-syndromic sagittal craniosynostosis. In addition, we seek to investigate if there are any observable changes in perioperative morbidity between the two procedures.

This study will examine how effective caregiver's repositioning strategies are in correcting an infant's deformational cranial shape, as well as the effectiveness of the use of a custom cranial remolding orthosis for treatment of deformational head shapes. Infants with torticollis will be concurrently enrolled in physical therapy treatment until the torticollis is resolved. A normal, unaffected population will be studied to compare typical growth to the growth of infants undergoing active treatment.

Context: Craniosynostosis is a common craniofacial abnormality which can be associated with various clinical syndromes. Though it has been established that children with craniosynostosis score lower on certain developmental tests, the effect of craniosynostosis and cranioplasty surgery on the neural circuitry and brain development is less well known or understood. Objectives: The purpose of this study is to describe the effect of cranial vault remodeling in children with craniosynostosis on white matter tracts with tractography and Diffusion tensor imaging (DTI), functional MRI, and neurodevelopmental tests, before and after surgery as compared to age-matched controls. Study Design: This will be a prospective study of patients diagnosed with craniosynostosis and who are going to have open or endoscopic cranial vault remodeling (CVR). Study Measures: The study will measure MRI sequences before and after surgery and at set time intervals to quantify the effect of white matter tract maturity. Parallel to this, neurodevelopmental tests will be administered at these same intervals.

The purpose of this study is to determine whether Amicar (ε-aminocaproic acid) is effective in reducing blood loss in children undergoing craniofacial reconstruction surgery. The investigators hypothesize that Amicar will decrease intraoperative blood loss and decrease the need for perioperative blood product administration in children undergoing craniofacial surgery.

The purpose of this study is to develop and test the effectiveness and diagnostic quality of Zero Echo Time Magnetic Resonance Imaging (ZTE MRI) in comparison to CT.

RESEARCH QUESTION Is the effectiveness of conservative policy comparable to that of surgery in children with trigonocephaly, with regard to clinical outcomes and psychosocial functioning, stress for parents, and costs? DESIGN Observational cohort study in children, aged 0 to 8 years old, with trigonocephaly, excluding metopic ridging (physiologic early closure of metopic suture), treated conservatively or surgically. OUTCOME MEASURES Primary: Head growth decline, indicating raised intracranial pressure Secondary: fundoscopy, cognition, behavior, refraction and vision, forehead shape, quality of life, posttraumatic stress, decisional conflict, costs.

FGF23 is the cornerstone of phosphate / calcium / vitamin D metabolism: it is synthesized mainly by osteocytes and acts as a phosphaturizing agent, inhibitor of dihydroxyvitamin D, and inhibitor of synthesis and secretion of PTH in most tissues. The specific role of FGF23 on bone has yet to be demonstrated. In osteoblasts, overexpression of FGF23 in vitro suppresses not only osteoblastic differentiation but also the synthesis of the mineralized matrix independently of its systemic action on phosphate metabolism. In osteoblasts, FGF23 also regulates the secretion of osteopontin by directly suppressing transcription of alkaline phosphatase. In some diseases such as hypophosphatemic rickets (HR), the direct role of FGF23 on bone has not yet been studied to our knowledge, whereas these genetic hypophosphatemias are secondary to overexpression of FGF23, whether an activating mutation of FGF23 or inhibitory mutations of its inhibitors (DMP1 and PHEX). However, patients with X-linked hypophosphatemic rickets (XLH) have higher circulating FGF23 levels than healthy controls and these levels are higher in treated patients. Management of XLH consists primarily of correcting the native vitamin D defect by prescribing active vitamin D analogs as well as phosphate supplementation to improve bone mineralization and decrease dental complications, growth, and bone deformities. Recently, a new therapeutic option has been developed for XLH, burosumab, a human monoclonal antibody that binds and inhibits FGF23 activity. The use of burosumab is currently authorized in France in some pediatric patients with severe forms of XLH. Independently of the indirect bone effects of phosphate correction and vitamin D levels, the direct role of burosumab on bone cells has never been studied. The objective of this project is to study the osteoblastic biology of patients with RH compared to control patients, and to evaluate the direct impact of the treatments used in this pathology on human osteoblasts.

Most children diagnosed with craniosynostosis undergo a relatively extensive cranial vault remodeling procedure. The decision of performing surgical cranial shape correction for patients with craniosynostosis typically rests on a subjective visual assessment of the severity of the cranial malformation and the main goal of this procedure is to reduce the risk of elevated intracranial pressure and to provide a more normal cranial shape and volume. Personalized surgical planning systems to optimize intervention and leverage surgical expertise in the reconstruction of the cranial vault do not exist. Thus, the expertise of the surgeon is paramount for the success of the surgical correction of craniosynostosis. The goal of our project is to evaluate the feasibility and utility of a surgical plan derived from software developed at Children's National, iCSPlan.

Aim of study: To evaluate outcome of cases( cosmoses and cognition) after Craniosynostosis surgery - To ascertain intra- and postoperative complications