View clinical trials related to Craniosynostoses.
Filter by:The purposes of this study are: - to better understand the experience of the announcement for the diagnostic of craniosynostosis to patients and their families to improve the understanding of it and it modes of appropriation - to compare the announcement process concerning "simple" and "complex" forms. - to identify the intra-family issues at the announcement of a genetic mutation. - to reconstruct the care course of patients by analyzing the time of the announcement and the post-operative period.
This research study is being performed to evaluate two different doses of Tranexamic acid (TXA) in children who have craniosynostosis and have been referred to Boston Children's Hospital for corrective surgery. This surgery is associated with significant blood loss and frequently requires the transfusion of blood. TXA is a medication that reduces the amount of bleeding during surgery by improving clotting of the blood at the surgical site. TXA is an FDA-approved drug that is routinely used in infants and children undergoing major surgery including heart surgery, craniofacial surgery and scoliosis surgery. It has been shown to decrease both the amount of bleeding and the amount of blood transfusion needed. We would like to compare the different doses of TXA to see if a lower dose has the same effect on blood loss as a higher dose. We are also interested to learn why TXA seems to work better in some patients than in others. In order to study the effect of this drug we would like to give this drug to your child and measure the blood loss and the volume of blood given to your child during his/her surgery. The research is being done at two sites; Boston Children's Hospital and Gaslini Children's Hospital in Genoa, Italy. The main study doctor from Boston Children's Hospital is Dr. Susan Goobie. The Department of Anesthesiology at Boston Children's Hospital is sponsoring this study. We are planning to study a total of 68 infants and children from age 3 months to 6 years old scheduled for open craniosynostosis surgery at Boston Children's Hospital or Gaslini Children's Hospital.
Massive bleeding is expected when performing distraction osteotomy for craniosynostosis patients. Since such operation is performed at very young age, many efforts are performed in order to reduce the total amount of bleeding and the transfusion during and after the surgery. Our study aims at correcting the coagulopathy from massive bleeding and transfusion during distraction osteotomy using continuous infusion of antifibrinolytic agent, tranexamic acid. Tranexamic acid infusion is determined according to the pharmacokinetic model, and the changes in coagulopathy will be defined using thromboelastography.
Craniosynostosis is a birth defect that causes the bones on a baby's head to fuse together earlier than normal. This causes the baby to have an abnormally shaped head. These children are operated on to prevent or treat increased pressure on the brain, allowing for normal development. There is not good evidence of which children with craniosynostosis have increased pressure on the brain. Up to twenty patients with craniofacial abnormalities will be enrolled in this pilot study. The investigators will use a magnetic resonance scanner to obtain several measures of brain metabolism. The investigators will also obtain data which are markers of developmental delay. The results will also be compared to age and gender matched data from children without craniofacial abnormalities. There study hypothesis is that patients with craniofacial abnormalities associated with intracranial pressure will have decreased metabolic activity compared to control patients.
Being the craniostenoses sutural basically a disease, the fact that the brain being trapped in an enclosure that does not have complacency required to accompany their growth constitutes the challenge of treatment, which aims to restore the complacency of the suture and correct the stenotic compensatory cranial deformity. This paper proposes the combination of a helicoid osteotomy distraction osteogenesis provided by the use of springs distracting.
The National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) was initiated in 2006 by the National Heart, Lung, and Blood Institute (NHLBI) and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). GenTAC established a registry of 3706 patients with genetic conditions that may be related to thoracic aortic aneurysms and collected medical data and biologic samples. The study ended in September 2016. Data and samples are available from NHLBI and requests should be made to BioLINCC. See the NHLBI website for more information: https://www.nhlbi.nih.gov/research/resources/gentac/.
Craniofacial reconstruction surgery involves a surgical approach to the craniofacial region to repair cranial vault and facial deformities. The surgery is extensive, often requiring wide scalp dissections and multiple osteotomies and has been associated with significant morbidity. Some of the most severe and commonly seen problems are associated with the rate and extent of blood loss. Efforts to minimize surgical bleeding may translate to reduced transfusion requirements and a lessening of associated risks Epsilon-aminocaproic acid (EACA), an inhibitor of fibrinolysis, reduces transfusion requirements in children undergoing procedures on cardiopulmonary bypass (CPB), as well as in older children undergoing spinal surgery for scoliosis (1-6). Before controlled studies to assess efficacy of EACA in a craniofacial surgical population can be done, appropriate pharmacokinetic (PK) data are needed to determine the optimal dosing strategy. PK data exist for EACA in children undergoing operations on CPB and hypothermia. The aim of this study is to determine the pharmacokinetics of EACA in infants and children undergoing craniofacial reconstruction procedures.
The primary objectives of this study are - to procure human temporalis muscle, subcutaneous adipose (fat), and bone tissue samples from children with craniosynostosis, - to grow cells from these tissues in vitro, - to evaluate the osteogenic potentials of these cell types.
The research purpose is to determine if thymus transplantation with immunosuppression is a safe and effective treatment for complete DiGeorge anomaly. The research includes studies to evaluate whether thymus transplantation results in complete DiGeorge anomaly subjects developing a normal immune system.
The study purpose is to determine if cultured thymus tissue implantation (CTTI) (previously described as transplantation) with tailored immunosuppression based on the recipient's pre-implantation T cell population is a safe and effective treatment for complete DiGeorge anomaly. This study will also evaluate whether cultured thymus tissue implantation and parathyroid transplantation with immunosuppression is a safe and effective treatment for complete DiGeorge anomaly and hypoparathyroidism.