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.

A Case of Spontaneous Hematomyelia

This is a report of 23 year old soldier who developed sudden onset of flaccid paraplegia and loss of all sensory modalited below the level of T4. The clinical entities of hematomyelia were sudden onset of pain, repidly developing pyramidal signs and paraplegia. The mainly involving sites of this disease were cervical and thoracic regions. The causative factors were hemorrhagic diasthesis, vascular malformation and inflammatory process as well as trauma and neoplasms affecting the spinal cord. We experienced a case of hematomyelia which did not combine with vascular anomaly, inflammatory process, hemophilia or trauma history.

A Case of Posterior Medullary Hemangioblastoma

The source of hemangioblastoma in the hindbrain was considered to be avascular analoge in or adjacent to the posterior medullary velum. In hemangioblastoma of the brain stem, the area postrema has been known as the most common site. Operative and autopsy findings were confirmed by Okawara that the solid lesions are more likely to involve the brain stem. The solid hemangioblastoma is much more difficult to treat and the mortality is higher than the cystic type due to involvement of the brain stem, massive postoperative edema, and intraoperative or postoperative bleeding from the tumor mass. Operative and histopathologic findings of our case are confirmed with solid hemangioblastoma which involves the posterior medullary oblongata and was regarded as arising at the postrema.