Craniosinostoza

Craniosinostoza term refers to premature fusion of cranial sutures.It is normally present at birth but is not always obvious, especially if it's easy. It manifests as a clinical strain in the first few months of life. In addition to cosmetic facial and skull deformities obvious early suture closure may cause and affect the development of intracranial hypertension in some patients. Because the brain does not extend in the direction of fused sutures is forced to grow in the direction of forcing open sutures frequently abnormal shape of the head and face.
It is estimated that craniosinostoza every 2,000 births. Syndrome may be a result of hereditary or sporadic. The sporadic form is not known why. The skull of a child is not a solid bone, and bony plates that form it are separated by fibrous sutures. Child's skull is composed of metopes suture, coronal, sagittal and lambdoida.These sutures allow the skull to expand as the brain develops.While these sutures fuse together and form a solid skull.
Craniosinostoza usually appears as the only manifestation, and in 20% of cases are associated with different syndromes. A syndrome is diagnosed by the presence of a variety of signs and symptoms. Genetic testing may be available to confirm the diagnosis of a specific syndrome. A family history of the presence of an abnormally shaped head is suggestive of the other members of a genetic syndrome, although multiple syndromes are caused by mutations in November and no family history. The cranisinostoza encountered syndromes associated with Crouzon syndrome and Aperta.
In the past 30 years has developed a better understanding of the pathophysiology and therapeutics craniosinostozei. Today surgery is a cosmetic procedure especially in children of 1-2 suture fusion.For children with microcephaly is not required surgery.Surgery is used to separate the fused sutures of the skull and reshape the skull. To address the issues of cosmetic surgeons use a combination of orthodontics and orthognatic to improve some deficiencies of the face.
Patients with primary craniosinostoza require monitoring after surgery. Most patients with simple synostosis not show postsurgical morbidity. The prognosis is dependent secondary craniosinostoza etiology of substance. Craniosinostoza syndromic patients may present a more complicated evolution due to the increased severity and related problems sinostozelor: airway obstruction and hydrocephalus.
Pathogenesis
Cranial vault ossification begins in the central region of each cranial bone and extending to the periphery of the cranial sutures.Skull bones: frontal, parietal, temporal and occipital are well developed in the fifth month of gestation. Membranous skull bones are joined by connective tissue in the sagittal sutures, coronal, lambdoida, metopes and scaly. Anterior fontanel bone junction is the intersection of frontal and parietal sutures and metopes, sagittal and coronal. It normally closes at the age of 20 months after birth. Posterior fontanel is located at the junction lambdoida and sagittal sutures and closes at the age of 3 months after birth.Suture closure appears normal at the age of 12, but complete fusion continues until the third decade of life.
Cranial sutures include:-Coronal suture separates the two parietal bones, frontal boneFrontal bone-suture separating metopes-Sagittal suture separates the two parietal bonesLambdoida suture separates the occipital bone, the two parietal bones.The main factor that maintains open shots is continuous growth of the brain. Normal development of the skull should be perpendicular to each suture.
Craniosinostoza primary clinical forms:Scafocefalia-fusion-type of the sagittal sutureAnterior-fusion-plagicefalia an early coronary suturesBack-close-plagicefalia lambdoide types of sutureEarly-fusion-brachycephalism bilateral coronal suturesTrigonocefalia-early-suture fusion metopes.
Causes
Craniosinostozei etiology is not clear at present, but the genetic defects are the main suspects. Autosomal dominant heredity was clearly identified in syndromic forms of craniosinostozei, although a number of patients have spontaneous mutations in November.Familial cases are frequently constituted 46% of the total. Full Penetrantra was observed in all inherited cases. No identifiable pattern of inheritance for forms nonsindromice craniosinostoza. In familial cases was observed orinzontala penetrance and variable vertical.Using immunohistochemistry techniques described abnormal osteoblast activity in cultures of bone synostosis. Histopathology of resected sutures show different stages of new bone formation along the suture zone. These stages range from interdigitatii trabecular fibrous tissue along the bone until fusion with the training and over-bridges at the affected suture, palpable on clinical examination and observed the operation.
Cases craniosinostozei:-Hyperthyroidism, hypophosphatemia, vitamin D deficiency, kidney osteodistrophy-Hypercalcaemia, rickets, sickle cell anemia, thalassemiaMicrocephaly, hidrocefalus are.