11/59. A case of basilar impression complicated with left frontal meningioma. Case report: A 75-year-old baikarian woman was admitted to our hospital for treatment of seizures. From the results of neurological and radiological examination, a left frontal meningioma was suspected and the patient was referred to our department for neurosurgical intervention. At admission, the MRI showed a basilar impression accompanied by klippel-feil syndrome of C4/5/6/7, but neurological symptoms of basilar impression were absent. Subsequently, the tumor was resected via the left frontal approach using microsurgical technique. Histological examination disclosed fibroblastic meningioma. DISCUSSION: The coincidence of basilar impression with a brain tumor is a relatively rare occurrence. There are a few reports about craniovertebral junction anomaly including basilar impression associated with spinal or cerebral tumor. This time, we present an interesting combined case of BI and klippel-feil syndrome associated with left frontal meningioma. ( info) |
12/59. Syringobulbia: a surgical appraisal. Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation or at necropsy caused by cutting outwards of the CSF under pressure from the fourth ventricle into the medulla. These should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a previously established syringomyelia. Clinical analysis of 54 patients suggests that bulbar features are most often found with neither of the above mechanisms but are due to the effects of pressure differences acting downward upon the hind-brain with consequent distortion of the cerebellum and brainstem, traction on cranial nerves or indentation of the brain-stem by vascular loops. The commonest symptoms in the 54 patients were headache (35), vertigo (27), dysphonia or dysarthria (21), trigeminal paraesthesiae (27), dysphagia (24), diplopia (16), tinnitus (11), palatal palsy (11) and hypoglossal involvement (11). Careful attention to hydrocephalus is advisable before craniovertebral surgery, but the decompression of the hindbrain and the correction of craniospinal pressure dissociation remains the mainstay of surgical treatment. The results of careful surgery are good, 45 of the 54 cases reported improvement. Most of the reported deterioration occurred in a few patients who did conspicuously badly. ( info) |
13/59. Congenital basilar impression: correlated neurological syndromes. A series of 8 cases operated on for symptomatic basilar impression associated with occipitalization of the atlas is reported (with or without atlantoaxial dislocation). Symptoms of onset (such as the frequent association between nuchal pain and vertigo) are emphasized and analyzed in relation to the pathogenetic mechanism that underlies the multiform symptomatology of the basilar impression. The diagnostic workup for basilar impression foresees x-rays, magnetic resonance imaging and computed tomography. The most important diagnostic problem is that of considering the possible existence of such a pathology in the presence of very common symptoms such as nuchal pain and vertigo. The surgical treatment has certainly been useful both to improve and to stabilize the symptomatology mainly when there is atlantoaxial dislocation. In fact in these cases the symptomatology is more severe and progressive for the alteration of the transverse ligament of the atlas secondary to abnormal mechanical stimuli. ( info) |
14/59. Circumferential decompression of the foramen magnum for the treatment of syringomyelia associated with basilar invagination. Posterior fossa decompression utilizing suboccipital craniectomy and duraplasty remains the standard surgical treatment for Chiari-associated syringomyelia. In the presence of basilar invagination, anterior decompression, typically transoral odontoidectomy, or posterior decompression may be performed. We report two cases in which anterior and posterior (circumferential) decompression of the foramen magnum was used to treat cervical syringomyelia successfully. These cases demonstrate that circumferential decompression of the foramen magnum may be necessary in some cases of cervical syringomyelia associated with basilar invagination and Chiari malformation. ( info) |
15/59. Surgical management of high cervical disc prolapse associated with basilar invagination--two case reports. C3-4 cervical disc prolapse was associated with basilar invagination and short neck in a 21-year-old man and additionally with an extensive Klippel-Feil abnormality and fusion of multiple cervical vertebrae in a 32-year-old man. The transoral surgical route was adopted for cervical discectomy in the latter case and an additional odontoidectomy in the former case. Interbody plate and screw fixation was carried out in the patient with Klippel-Feil abnormality. Both the patients were relieved of symptoms and remained asymptomatic at follow up. Simultaneous fixation procedure is not mandatory after transoral surgery in patients with basilar invagination. ( info) |
16/59. Cervical arachnoidal cyst with basilar impression and arnold-chiari malformation: a case report. A case of cervical spinal arachnoidal cyst is presented. The association with basilar impression and arnold-chiari malformation is a peculiarity seldom reported. The clinical aspects with remission and exacerbation are discussed. The importance of the neuroradiological findings and the usefulness of the neurophysiological examination (evoked potentials) are suggested. ( info) |
| Hadju-Cheney syndrome (HCS) is a rare autosomal-dominant disorder with variable expressivity. It is characterized by facial dysmorphism, premature tooth loss, osteolysis of distal phalanges, and skull abnormalities. In some cases, progressive platybasia can occur and can lead to Chiari malformation with an obstruction of cerebrospinal fluid flow. To the best of the authors' knowledge, only five cases of HCS-associated syringomyelia have been reported in the literature. Because of the rarity of this association, little is known about its natural history. The authors present the case of a 16-year-old boy affected by HCS. On initial magnetic resonance (MR) imaging, a severe basilar invagination with Chiari malformation and cervicothoracic syringomyelia was documented. The syringomyelia had no clinical manifestations. A repeated MR image demonstrated a spontaneous resolution of the syrinx with no changes in the tonsil or the platybasia. The regression of the syringomyelia was confirmed by a control MR imaging examination performed after a 2-year period. No changes in the patient's clinical conditions were found during the follow-up period. This is the first case of spontaneous regression of the syringomyelia despite a severe platybasia in HCS. It did not appear correlated to a modification of the tonsil's structure or position. This observation illustrates one possible evolution of syringomyelia in the natural history of HCS and raises the question of the potential mechanisms involved in the spontaneous drainage of the syringomyelic cavity. ( info) |
18/59. neuronavigation-assisted transoral-transpharyngeal approach for basilar invagination--two case reports. Two patients presented with congenital basilar invagination manifesting as progressive myelopathy. Both patients underwent surgery using a neuronavigation-assisted transoral-transpharyngeal approach. The Brain-LAB Vector Vision navigation system was used for image guidance. The registration accuracies were 0.9 and 1.3 mm. After decompression, posterior stabilization was performed. Both patients had an uneventful postoperative course. The transoral-transpharyngeal approach with the neuronavigation system provides safe exposure and decompression for basilar invagination. ( info) |
19/59. Melnick- needles osteodysplasty presenting with quadriparesis. Melnick-needles syndrome or osteodysplasty, a monogenic heritable bone dysplasia, is characterized by a typical facies and characteristic radiological findings. Less than 70 well-documented cases have been reported in literature; most of them were sporadic. We report the first case from Eastern india in an adolescent male, who had cranio-vertebral junction anomalies and presented with spastic quadriparesis at the age of 13 years. ( info) |
20/59. Myelopathy by lesions of the craniocervical junction in a patient with forestier disease. STUDY DESIGN: The authors report a case of a patient with diffuse idiopathic skeletal hyperostosis (DISH) associated with basilar impression resulting in tetraparesis. OBJECTIVE: To describe neurologic compromise associated with DISH. SUMMARY OF BACKGROUND DATA: Neurologic deficits due to DISH are very rare, and only 1 case of basilar impression associated with DISH has previously been reported in the literature. methods: diagnosis was confirmed by radiograph and MRI, which demonstrated basilar impression associated with a hyperintense signal in the spinal cord on T2-weighted sequences. Transoral resection of the dens associated with posterior occipitocervical fixation was performed during the same anesthesia. RESULTS: Postoperative outcome demonstrated regression of the pyramidal signs without recovery of unassisted walking. CONCLUSION: Early MRI should be performed in the evidence of spinal cord suffering in patients with DISH. Transoral approach allowed a good decompression of the spinal cord. ( info) |