Craniosinostoza - Signs and symptoms

Craniosinostoza consists of premature fusion of one or more cranial sutures cause abnormal head shape. Primary defect may result printrun-craniosinostoza ossification or more frequently by primary failure of brain development, secondary craniosinostoza.Craniosinostoza simple is the term used when only one suture fuses prematurely. Craniosinostoza complex or compound refers to premature fusion of several sutures. When children craniosinostoza shows and other deformations of the body they are called craniosinostoza syndrome.
Craniosinostoza PrimaryWhere two or more sutures fuse prematurely, skull growth is restricted perpendicular to the suture. If multiple sutures fuse while the brain are still developing and growing in size is increased intracranial pressure.Craniosinostoza may be evident at birth or in childhood with cranial anomalies. It may become evident when the child manifests late in neurodevelopmental delay.Scafocefalia:Premature fusion of the sagittal suture is most common craniosinostoza, constituting over half of cases. Commonly occurs in premature babies. The head is elongated in the anterior-posterior diameter and biparietal diameter shortened. Sagittal suture is prominent and palpable.
Previous Plagiocefalia seprezinta premature fusion of coronal sutures.
Brachycephalism:Premature fusion of both coronal sutures biparietal diameter increases. This anomaly is frequently syndrome. The skull is short in the anterior-posterior diameter. Since the coronal suture is developed in conjunction with suture of the skull base, middle and lower facial hypoplasia occurs. Orbits can be elliptical and supraorbitala crest may not be formed properly.
Plagicefalia back:The two main causes of hind plagicefaliei craniosinostoza lambdoide suture flattened head syndrome. Torticollis is often associated. Previous position on the ear is flattening. Note the flattening boselarea ipsilateral side and contralateral head in lambdoidiana synostosis.
Trigonocefalia:Mergers often result in premature suture metopes triangular prominent forehead. The anomaly is usually mild and do not require surgery. Surgery is performed when the fault is persistent and severe.Craniosinostoza is frequently associated with craniofacial syndromes such as Crouzon sporadic, Aperta, Carpenter. In these cases, facial characteristics, tangible and closing shots of fontanels types suggest the diagnosis.
Crouzon syndrome:Syndrome is an autosomal dominant inheritance pattern in 75% of patients. The remaining 25% of cases are sporadic. Skull shape varies depending on the order of fusion, but brachycephalism is most common in basal and coronal sutures closing. Items associated proptoza include eye, maxillary hypoplasia, parrot beak nose and ocular hypertension. Hidrocefalusul Crouzon syndrome is more common than in other syndromes and herinerea tonsils is common in these patients.
Aperta syndrome:It represents an autosomal dominant disease characterized by cranial synostosis with malformed and short cranial base. It is associated with associated sindactilia fingers two, three and four fingers wide with radial deviation, sindactilia hallux and visual impairment. Aperta syndrome, have a high risk of mental retardation. 50% of patients had IQs below 70. Cervical fusion occurs in 68% of patients.
Craniosinostoza secondary:Much more commonly found than the primary form, secondary craniosinostoza may result from early fusion of sutures through the primary failure of brain development. Once brain growth progressing bone plates distances of shots, so a lack of brain growth allows the fusion of all sutures.Intracranial pressure is usually normal and surgery is rarely indicated. Typical lack of brain growth cause microcephaly.Premature closure of sutures does not compromise growth and does not require brain surgery to open shots.Reduced intrauterine space may play a role in premature fusion of skull sutures in the fetus. This phenomenon was demonstrated in coronary craniosinostoza. Other causes of craniosinostozei include systemic diseases affecting bone metabolism such as rickets and hypercalcemia.
Besides the obvious clinical deformation affecting the face and head, the children especially those with respiratory problems as a craniosinostozei sidnromica. Maxillary hypoplasia Because these children have difficulty in breathing through the nose and mouth.Night shows sleep apnea. These phenomena affect growth, behavior and language spoken. Children with increased intracranial pressure may develop chronic headaches, decreased school performance and visual disturbances. As the child grows aspectl abnormal face has a negative effect on social integration and individual development.A minority of patients have a delayed onset. They have a slight deformation of the face is not recognized early in life and shows in 4-8 years age intracranial hypertension.
Disease progression:For most patients that involve a single suture craniosinostoza main problem is cosmetic. Early diagnosis and surgical therapy are essential to prevent craniofacial deformation irreversible. Patients with diffuse craniosinostoza are at risk of developing increased intracranial pressure. They may have breathing problems because of eye problems associated with maxillary hypoplasia and orbital deformities. Aperta syndrome, Pfeiffer Crapenter shows problems or anomalies due to finger surgery.