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At least 253 records · Page 14Linked to original sources
PubMed1994

Basilar invagination and mid-line skeletal abnormalities due to in utero exposure to phenytoin.

A case of basilar invagination which is thought to have arisen from the patient's intrauterine exposure to phenytoin is presented. The patient was treated surgically by foramen magnum decompression and occipito-cervical fusion, and by transoral resection of the clivus. His symptoms of brain stem compression were alleviated and the role of phenytoin in the production of his craniocervical abnormality is discussed.

Adult↗
PubMed1982

Radiofrequency trigeminal rhizotomy in treatment of symptomatic non-neoplastic facial pain.

Twenty patients suffering from non-neoplastic symptomatic facial pain underwent percutaneous radiofrequency trigeminal thermo-rhizotomy. Fourteen patients had long-standing severe multiple sclerosis, two patients had intracranial aneurysms, three patients had basilar impression secondary to Paget's disease and developmental malformations of the skull, and one patient was suffering from chronic ocular pain that developed after retinal hemorrhage. Pain paroxysms similar to tic douloureux were present in patients with multiple sclerosis and in those with basilar impression, while continuous aching pain was present in the others. After thermo-rhizotomy, pain disappeared in all patients; however, at 1 to 4 years follow-up examination, a high recurrence rate (40%) was present in the multiple sclerosis group, and the percutaneous procedure was successfully repeated. In the patients with intracranial aneurysm not amenable to direct surgery, and in the other non-neoplastic diseases, complete pain relief was found at 4 years follow-up review.

Adult↗
PubMed1993

Neurogenic hypertension related to basilar impression. Case report.

The authors report the resolution of essential hypertension following transoral odontoidectomy and medullary decompression in a 39-year-old woman with basilar invagination. Current understanding of central regulation of the cardiovascular system is discussed and the pertinent neuroanatomy illustrated. Experimental and clinical evidence supporting the role of neurogenic mechanisms in the pathogenesis of hypertension is reviewed.

Adult↗
PubMed1997

Basilar invagination in osteogenesis imperfecta and related osteochondrodysplasias: medical and surgical management.

Osteogenesis imperfecta (OI) is a heritable disorder of bone development caused by defective collagen synthesis. Basilar invagination is an uncommon but devastating complication of this disease. The authors present a comprehensive strategy for management of craniovertebral anomalies associated with OI and related osteochondrodysplasias. Twenty-five patients with congenital osteochondrodysplasias (18 OI, four Hajdu-Cheney syndrome, and three spondyloepiphyseal dysplasia) and basilar invagination were evaluated between 1985 and 1995. The male/female ratio in this cohort was 1:1. The mean age at presentation was 11.9 years (range 13 months-20 years). Fourteen patients (56%) presented during adolescence (11-15 years of age). Symptoms and signs included headache (76%), lower cranial nerve dysfunction (68%), hyperreflexia (56%), quadriparesis (48%), ataxia (32%), nystagmus (28%), and scoliosis (20%). Four patients (16%) were asymptomatic. Seven (28%) had undergone previous posterior fossa decompression; one had also undergone ventral decompression. Imaging findings included basilar invagination (100%), ventral brainstem compression (84%), hydrocephalus (32%), hindbrain herniation (28%), and syringomyelia/syringobulbia (16%). Patients with hydrocephalus underwent ventricular shunt placement. Reducible basilar invagination (40%) was treated with posterior fossa decompression and occipitocervical fusion. Those with irreducible ventral compression (60%) underwent transoral-transpalatopharyngeal decompression followed by occipitocervical fusion. All patients improved initially. However, basilar invagination progressed radiographically in 80% (symptomatic in 24%) despite successful fusion. Prolonged external orthotic immobilization with the modified Minerva brace afforded symptomatic improvement and arrested progression of the deformity. The mean follow-up period was 5.9 years (range 1.1-10.5 years). Ventral brainstem compression in OI should be treated with ventral decompression, followed by occipitocervical fusion with contoured loop instrumentation to prevent further squamooccipital infolding. Despite fusion, however, basilar invagination tends to progress. Prolonged immobilization (particularly during adolescence) may stabilize symptoms and halt further invagination. This study represents the largest series to date addressing craniovertebral anomalies in OI and related congenital bone softening disorders.

Adolescent↗
PubMed1998

Basilar invagination: a study based on 190 surgically treated patients.

OBJECT: The authors analyzed the cases of 190 patients with basilar invagination that was diagnosed on the basis of criteria laid down in 1939 by Chamberlain to assess the appropriate surgical procedure. METHODS: Depending on the association with Chiari malformation, the anomaly of basilar invagination was classified into two groups. Eighty-eight patients who had basilar invagination but no associated Chiari malformation were assigned to Group I; the remainder of the patients, who had both basilar invagination and Chiari malformation, were assigned to Group II. The principal pathological characteristic was observed to be direct brainstem compression due to odontoid process indentation in Group I and a reduction in posterior cranial fossa volume in Group II. CONCLUSIONS: Despite the anterior concavity of the brainstem in both groups, transoral surgery was the most suitable procedure for those patients in Group I and decompression of the foramen magnum was found to be appropriate for patients in Group II. After surgical decompression, a fixation procedure was found to be necessary in most Group I cases, but only in a small minority of Group II cases.

Adolescent↗
PubMed2003

A prospective controlled study of sleep respiratory events in patients with craniovertebral junction malformation.

OBJECT: Craniovertebral junction malformation (CVJM) or Chiari malformation in adults, with or without syringomyelia and basilar invagination, produces neuronal dysfunction of the brainstem, cerebellum, cranial nerves, and upper spinal cord. The respiratory center and some of its afferent and efferent components can be altered in these diseases. The authors studied patients with and without CVJM to determine whether this physical feature contributed to sleep disturbances. METHODS: Respiratory manifestations during sleep were studied prospectively, by using whole-night polysomnography, in 32 symptomatic patients (CVJM group) and 16 healthy volunteers (control group). Patients with CVJM presented with more sleep disturbances (reports of snoring and apnea) than those in the control group. The apnea/hypopnea index values were higher in patients with CVJMs than in the control group (13 +/- 15 compared with 3 +/- 6; p = 0.007) and the rate of central sleep apneas was higher in the CVJM than in the control group (22 +/- 30 compared with 4 +/- 8%; p = 0.009). The apnea/hypopnea index was highest in the subgroup with basilar invagination than in the other subgroups. The central apneic episodes were more frequent in the patients with basilar invagination (35 +/- 40%; p = 0.001) and in those with syringomyelia (17.6 +/- 24.6%; p = 0.003) than in the control group (4 +/- 8%). Patients with symptomatic CVJM, especially those with basilar invagination, presented with more sleep respiratory compromise than did those in the control group. CONCLUSIONS: The incidence of sleep apnea/hypopnea syndrome is significantly higher in patients with CVJM.

Adult↗
PubMed2002

Extreme lateral-transatlas approach for resection of the dens of the axis.

OBJECT: Various approaches have been described for resection of the dens of the axis, each of which has potential advantages and disadvantages. Anterior approaches such as the transoral route or its modifications are the most commonly used for resection of this structure. The transcondylar approach, however, which allows the surgeon to view the craniovertebral junction (CVJ) from a lateral perspective, has been introduced by Al-Mefty, et al., as an alternative approach. In this report, the authors describe the surgical technique of the extreme lateral-transatlas approach and their clinical experiences. METHODS: The authors first examined the surgical approach to the dens from a lateral perspective in five cadaveric heads. They found that removal of the lateral mass of the atlas provided adequate exposure for resection of the dens. Following this cadaveric study, the extreme lateral-transatlas approach was successfully performed at the authors' institution over a 1-year period (September 1998-August 1999) in five patients with basilar invagination due to congenital anomaly of the CVJ and rheumatoid arthritis. Furthermore, during the same procedure, unilateral occipitocervical fusion was performed following resection of the dens. In all cases complete resection of the dens was achieved using the extreme-lateral transatlas approach. This procedure provides a sterile operative field and the ability to perform occipitocervical fusion immediately following the resection. No postoperative complications or craniocervical instability were observed. The mean follow-up period was 17.2 months (range 13-24 months). CONCLUSIONS: The extreme lateral-transatlas approach for resection of the dens was found to be safe and effective. Knowledge of the anatomy of this region, especially of the V3 segment of the vertebral artery, is essential for the success of this procedure.

Adult↗