Crouzan Syndrome - A Case Report.

Genetic disorders account for a significant amount of morbidity and mortality in children and are of primary interest to the dentist. Crouzan syndrome is one of a rare group of syndromes characterized by craniosynostosis or premature closing of the cranial sutures. The major features are Brachycephaly, ocular proptosis, under developed maxilla, midface hypoplasia, rare cleft lip, palate. Early Craniectomy is often needed to alleviate the raised intracranial pressure. This paper discusses a case report of five year old girl with the features of crouzan syndrome and a multidisciplinary approach to be followed in managing the situation.

Hydrocephalus In Achondroplasia: Case Report

Major neurological features of achondroplasia are attributed to premature synostoses of the pedicles of the vertebrae and of the base of the skull producing obstruction of the ventricular fluid circulation or compression of the medulla and spinal cord. Significant hydrocephalus is due to the obstruction of the cerebrosspinal fluid pathways at the level of the foramen magnum or the disturbance of CSF absorption caused by increased superior sagittal sinus pressure. In generally, the hydrocephalus in achondroplasia is of the communicating type in the pattern, so the CSF diversionary shunt may not be indicate. But, shunting procedures may be needed in patients who showed signs of severe IICP such as bulging fontanels and enlarged lateral ventricle. In this case, we experienced that the hydrocephalus in achondroplasia is prosorption following the stenosis of the foramen magnum and increased superior sagittal sinus pressure.