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At least 199 records · Page 11Linked to original sources
PubMed2002

Treatment of craniocervical spine lesion with osteogenesis imperfecta: a case report.

STUDY DESIGN: A case report of craniocervical spine lesions including basilar impression, atlantoaxial dislocation, and syringomyelia, with osteogenesis imperfecta is presented, and the literature is reviewed. OBJECTIVE: To discuss the problems involved in the surgical management of craniocervical spine lesion with osteogenesis imperfecta. SUMMARY OF BACKGROUND DATA: Osteogenesis imperfecta is known to have various spine lesions as complications. However, few reports have described craniocervical lesions associated with osteogenesis imperfecta. METHODS: A 14-year-old girl with osteogenesis imperfecta, Silence classification IVB, experienced difficulty walking, with marked motor disturbance and muscle weakness in the extremities. Deep tendon reflexes were exaggerated bilaterally in the upper and lower extremities, and positive Babinski reflex and ankle clonus were observed bilaterally. Basilar impression, atlantoaxial dislocation, and syringomyelia were shown by plain radiography, tomography, three-dimensional computed tomography, and magnetic resonance imaging. RESULTS: In the reported patient, posterior fossa decompression and atlantoaxial posterior fusion could not be performed because the foramen magnum and upper cervical spine invaginated to the base of the skull. Therefore, occipitocervical spine fusion using titanium loop and wires was performed at the reduced position of the atlantoaxial dislocation, resulting in improvement of neurologic deficits. CONCLUSIONS: For patients with atlantoaxial dislocation, syringomyelia, and basilar impression without clinical symptoms or signs of brain stem compression, occipitocervical spine fusion alone at the reduction of the atlantoaxial dislocation may be indicated because these procedures improve neurologic deficits and prevent postoperative development of basilar impression and enlargement of syringomyelia.

Adolescent↗
PubMed2002

Combined anterior and posterior approach for managing basilar invagination associated with type I Chiari malformation.

Ventral brainstem compression is common in patients with basilar invagination associated with type I Chiari malformation. The aim of this study was to investigate the reliability of a combined one-session anterior-posterior surgical approach for these patients. Nine patients underwent transoral odontoidectomy, posterior foramen magnum decompression, occipitocervical fixation, and bone grafting. All but two patients had ventral and dorsal operations in one surgical sitting. One of these two individuals had previously undergone posterior decompressive surgery at another hospital, but his condition had deteriorated rapidly afterward. The mean follow-up time was 19 months (range, 14-30 months). There was no mortality and no significant morbidity in this series. All but one of the patients showed significant improvement in their symptoms. Chiari malformations have a surprisingly high association with basilar invagination, and patients may have symptoms in both conditions. If there is a marked anterior compression, anterior transoral and posterior decompression, fusion, and instrumentation is an optimal strategy for treating patients with basilar invagination associated with type I Chiari malformation.

Adolescent↗
PubMed2005

Progressive basilar invagination after transoral odontoidectomy: treatment by atlantoaxial facet distraction and craniovertebral realignment.

STUDY DESIGN: The issue of progression of congenital basilar invagination is evaluated on the basis of 3 clinical cases. The rationale of treatment of basilar invagination in general and, particularly, in the complex clinical situation encountered in the presented cases, by the authors' technique of atlantoaxial joint distraction, reduction of basilar invagination, and direct lateral mass plate and screw fixation is discussed. OBJECTIVE: The subject of progression of congenital basilar invagination and recurrent craniocervical cord compression after an initial transoral decompression is analyzed, and an alternative surgical treatment involving craniovertebral bone realignment is suggested. SUMMARY OF BACKGROUND DATA: This is a review of 3 cases of basilar invagination treated earlier by transoral decompression. No fixation procedure was performed. The patients presented with recurrent symptoms of high cervical cord compression. Investigations revealed a progression of basilar invagination and cord compression. The treatment of such a complex clinical situation by atlantoaxial joint distraction is discussed. METHODS: Between September 2001 and January 2004, we treated 3 patients with congenital basilar invagination who underwent transoral odontoidectomy but did not undergo any posterior atlantoaxial or occipitoaxial fixation. The patients had postoperative improvement but worsening of their neurologic condition after an average duration of 26 months after transoral surgery. Repeat investigations revealed that basilar invagination and the craniovertebral alignments worsened during the period, and there was recurrent cervicomedullary cord compression. These patients were treated by atlantoaxial facet distraction, and attempts were made toward reduction of the basilar invagination and craniovertebral junction bony realignment, and atlantoaxial fixation by the technique recently described by us. RESULTS: During the average follow-up of 25 months, all 3 patients have had a neurologic recovery. CONCLUSIONS: The probable cause of basilar invagination and its progression is a congenital malformation ofalignment of the facets of the atlantoaxial joint. Distraction of the facets and direct interarticular atlantoaxial fixation presents a unique opportunity of reduction of the basilar invagination and fixation of the region.

Adolescent↗
PubMed2006

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.

Aged, 80 and over↗
PubMed1998

A case of Arnold-Chiari Type I malformation presenting with dysrhythmic breathing during sleep.

A 43-year-old woman presented with dull headache, left tinnitus and dizziness. Neurological examination revealed down-beat gaze nystagmus, left tinnitus, positive Romberg sign, poor standing on the left foot, poor tandem gait, left spastic gait and positive pathological reflexes in the bilateral upper and lower extremities. Plain X-ray of the skull and cervical vertebrae demonstrated basilar impression and atlantoaxial fusion. Magnetic resonance imaging of the brain and cervical spine showed cerebellar tonsil descent and syringomyelia located in the left side of the spine at the II-III vertebral level which communicated with the fourth ventricle. The patient was diagnosed as having cervical syringomyelia. Arnold-Chiari type I malformation and basilar impression. Preoperative polysomnography showed dysrhythmic breathing and bradypnea during sleep. Abnormal breathing improved after suboccipital decompression craniotomy and upper cervical laminectomy. It was suggested that dysrhythmic breathing was caused by a disorder of the medullary respiratory center. Herniation of the cerebellar tonsil and syringomyelia might have compressed the medulla.

Adult↗
PubMed1972

Basilar impression of the skull in patients with adult coeliac disease and after gastric surgery.

Chamberlain's, McGregor's and Bull's angle measurements for basilar impression of the skull were made on 22 adult patients with idiopathic steatorrhoea (probable gluten enteropathy), 24 patients who had had previous gastric surgery, and 48 control subjects. For each of the three measurements a value greater than the mean plus two standard deviations was taken as the upper limit of normal. In seven patients with adult steatorrhoea all three measurements were abnormal suggesting basilar impression, while basilar impression was probable in only one patient who had gastric surgery. The trend towards abnormal measurements was significant in the steatorrhoea patients but not in those who had gastric surgery. Basilar impression also was present in patients who did not have rickets or present evidence of osteomalacia. It was argued that this study could support a hypothesis that some cases of primary basilar impression of the skull are secondary to bone softening associated with malabsorption in early life, the evidence of which may have disappeared in adult life.

Adult↗
PubMed1976

'Sneeze syncope', basilar invagination and Arnold-Chiari type I malformation.

Syncope precipitated by sneezing in an adult male associated with an Arnold-Chiari type I malformation and basilar invagination presents a clinical problem in the differential diagnosis and pathological anatomy of Valsalva-related syncope. An abnormally acute clivoaxial angle, small foramen magnum, and type I Arnold-Chiari malformation appear to be a combination of features intolerant of Valsalva-induced changes in cerebral volume, brain-stem position, CSF fluid dynamics, or blood vessel relationships. Proposed mechanisms of pressure transmission to the area of intracranial pathology are discussed.

Adult↗
PubMed1992

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.

Adolescent↗
PubMed1992

Somatosensory and brainstem auditory evoked potential in congenital craniovertebral anomaly; effect of surgical management.

Clinical features and evoked potential recordings were analysed in 32 patients with congenital atlantoaxial dislocation before and after surgery. Seven patients (group 1) had atlantoaxial dislocation, while 22 patients had associated basilar invagination (group 2). In both groups, pyramidal tract signs, posterior column signs, wasting of the upper limbs, and abnormality of somatosensory evoked potentials (SSEP) were similar. Conversely, lower cranial nerve involvement and abnormal brainstem auditory evoked potentials (BAEP) were significantly more in patients with basilar invagination (p less than 0.05). All seven patients in group 1 and 17 patients in group 2 were operated upon. Clinical and electrophysiological deterioration were significant in patients with basilar invagination (group 2), following posterior fixation compared with group 1. Among the patients in group 2, who clinically deteriorated following posterior fixation, seven had transoral excision of odontoid and six of them improved both clinically and electrophysiologically. Two patients in group 2 had odontoid excision before posterior fixation, and in both the evoked potentials improved postoperatively. In group 1 the patient's BAEP remained unaffected following posterior fixation, however, in group 2, eight patients over 53% showed improvement in brainstem function following posterior fixation. This study shows the value of evoked potentials in congenital atlantoaxial dislocation, and rationalizes the surgical procedure in these patients. In patients with basilar invagination, odontoid excision is the preferred first stage procedure.

Atlanto-Axial Joint↗
PubMed1999

Basilar impression complicating osteogenesis imperfecta type IV: the clinical and neuroradiological findings in four cases.

OBJECTIVES: To describe the clinical and neuroradiological features of basilar impression in patients with osteogenesis imperfecta type IV. METHODS: Four patients with basilar impression were ascertained in a population study of osteogenesis imperfecta. All four had detailed clinical and neuroradiological examination with both CT and MRI of the craniocervical junction and posterior fossa structures. RESULTS: All four showed significant compression of the posterior fossa structures and surgical decompression was performed with relief of symptoms. CONCLUSION: Symptoms of cough headache and trigeminal neuralgia occurring in patients with osteogenesis imperfecta are indications for detailed clinical and neuroradiological investigation to document basilar impression.

Adolescent↗
PubMed1987

Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis.

Previous work confirmed the abnormal potential difference between the undersurface of the inferior nasal turbinate and a reference electrode in cystic fibrosis, but the technique is difficult and the results show overlap between the cystic fibrosis and the control populations. In the present study the potential difference from the floor of the nose has therefore been assessed in normal subjects, as well as in adult patients with cystic fibrosis, bronchiectasis and Young's syndrome. Voltages existing along the floor of the nasal cavity were recorded. The mean potential difference was similar in controls (-18 (SD 5) mv) and in patients with bronchiectasis (-17 (6) mv) and Young's syndrome (-20 (6) mv). The potential difference in cystic fibrosis (-45 (8) mv) was significantly different from controls (p less than 0.002) and there was no overlap between the cystic fibrosis values and values obtained in normal and diseased controls. This simple technique therefore discriminates well between patients with cystic fibrosis and other populations, raising the possibility of its use to assist in diagnosis.

Adolescent↗