Persistent torticollis, facial asymmetry, grooved tongue, and dolicho-odontoid process in connection with atlas malformation complex in three family subjects.
(41/133)
Congenital clefts and other malformations of the atlas are incidental findings identified while investigating the cervical spine following trauma. A persistent bifid anterior and posterior arch of the atlas beyond the age of 3-4 years is observed in skeletal dysplasias, Goldenhar syndrome, Conradi syndrome, and Down's syndrome. There is a high incidence of both anterior and posterior spina bifida of the atlas in patients with metabolic disorders, such as Morquio's syndrome [Baraitser and Winter in London dysmorphology database, Oxford University Press, 2005; Torriani, Lourenco in Rev Hosp Clin Fac Med Sao Paulo 53: 73-76, 2002]. We report two siblings and their mother, with congenital, persistent torticollis, plagiocephaly, facial asymmetry, grooved tongues, and asymptomatic "dolicho-odontoid process". All are of normal intelligence. No associated Neurological dysfunction, paresis, apnoea, or failures to thrive were encountered. Radiographs of the cervical spine were non-contributory, but 3D CT scanning of this area allowed further visualisation of the cervico-cranial malformation complex in this family and might possibly explain the sudden early juvenile mortality. Agenesis of the posterior arch of the atlas and bifidity/clefting of anterior arch of the atlas associated with asymptomatic "dolicho-odontoid process" were the hallmark in the proband and his female sibling. Some of the features were present in the mother. All the family subjects were investigated. To the best of our knowledge the constellation of malformation complex in this family has not been previously reported. (+info)
Management of odontoid fractures with percutaneous anterior odontoid screw fixation.
(42/133)
Minimally invasive techniques have revolutionized the management of a variety of spinal disorders. The authors of this study describe a new instrument and a percutaneous technique for anterior odontoid screw fixation, and evaluate its safety and efficacy in the treatment of patients with odontoid fractures. Ten patients (6 males and 4 females) with odontoid fractures were treated by percutaneous anterior odontoid screw fixation under fluoroscopic guidance from March 2000 to May 2002. Their mean age at presentation was 37.2 years (with a range from 21 to 55 years). Six cases were Type II and four were Type III classified by the Anderson and D'Alonzo system. The operation was successfully completed without technical difficulties, and without any soft tissue complications such as esophageal injury. No neurological deterioration occurred. Satisfactory results were achieved in all patients and all of the screws were in good placement. After a mean follow-up of 15.7 months (range 10-25 months), radiographic fusion was documented for 9 of 10 patients (90%). Neither clinical symptoms nor screw loosening or breakage occurred. Our preliminary clinical results suggest that the percutaneous anterior odontoid screw fixation procedure using a new instrument and fluoroscopy is technically feasible, safe, useful, and minimally invasive. (+info)
Osteoid osteoma of the dens axis.
(43/133)
An osteoid osteoma of the dens axis was diagnosed by computed tomography and bone scintigraphy in a 14-year-old girl with a 1.5-year history of pain. No case of an osteoid osteoma of the dens axis has been published in the literature yet. Regarding its clinical and radiological appearance, this osteoid osteoma was essentially similar to osteoid osteomas in other locations, i.e., it had an osteolytic nidus surrounded by sclerosis, associated with nocturnal pain and a positive aspirin test. (+info)
Bradycardia in minor trauma: don't be slow on the uptake!
(44/133)
We report the case of a 13-year-old boy presenting with profound bradycardia following minor trauma. Our patient had gastroschisis at birth and has moderate learning difficulties but is otherwise fit and well. Whilst playing at home he fell sustaining a minor cervical hyperextension injury. He immediately complained of tetraplegia and hyposensibility. The ambulance crew noted profound bradycardia with normotension and he was transported to hospital with full spinal immobilisation precautions. Over the subsequent 2 hours he made a full neurological recovery. Bradycardia persisted with a beat-to-beat variation of 30-60 bpm. ECG showed sinus bradycardia with atrial ectopics and he remained haemodynamically normal. Neuroimaging studies revealed hypoplasia of the odontoid peg with a relative narrowing of the spinal canal at this level. There was no evidence of spinal cord contusion or compression. His bradycardia resolved over 36 hours without further intervention. On discharge the patient was advised to wear a cervical hard collar when mobilising. This association of a craniocervical abnormality with learning difficulties, and gastroschisis has not been previously described. We discuss several other causes of odontoid peg instability, which may lead to severe autonomic effects with relatively insignificant trauma. (+info)
Timing of cervical spine stabilisation and outcome in patients with rheumatoid arthritis.
(45/133)
One complication of rheumatoid arthritis (RA) is the involvement of the cervical spine (CS). Although prophylactic stabilisation is recommended, the timing at which this should occur is poorly defined. The aim of our study was to evaluate the course of neurological symptoms in terms of the timing of surgery. A total of 34 patients with RA and CS involvement were surgically stabilised. These patients were classified using the Ranawat (RW) score both preoperatively and at an average of 54 months post-operatively. For each patient, the presence of atlantoaxial and subaxial subluxation as well as vertical migration of the odontoid was recorded. The anterior atlantodental interval was also assessed pre- and post-operatively. Improvement was obtained in 20 patients, the clinical situation remained unchanged in three patients and three patients manifested disease progression. In terms of the RW score, the 16 patients with pre-operative RW grades I-II showed no deterioration at the post-operative follow-up, with 13 of these patients showing an improvement; the 12 patients with pre-operative RW grades IIIA-IIIB did not show any improvement of neurological symptoms at follow-up, although seven of these patients subjectively assessed the symptoms to be less severe after surgery; three other patients showed a worsening of symptoms. Our results suggest that preventive stabilisation of CS in RA leads to acceptable results, although the complications of the surgery are obvious. However, early operative treatment may delay the detrimental course of cervical myelopathy in RA. (+info)
Computed tomographic evaluation of the odontoid process for two-screw fixation in type-II fracture: a Malaysian perspective.
(46/133)
PURPOSES: To measure the diameter of the odontoid process in a Malaysian population using computed tomographic (CT) scan and determine the feasibility of treating type-II odontoid fractures using 2 cortical screws. METHODS: CT images of the odontoid process of 85 patients aged 18 to 80 years were analysed; 69 (81%) were male (mean age, 44 years) and 16 (19%) were female (mean age, 48 years). Both anteroposterior (AP) and transverse diameters of the odontoid process were measured via axial CT images at 3 different levels: the base of the odontoid process and 1.2 mm and 2.4 mm above the base. RESULTS: The mean AP and transverse diameters of the odontoid process in men were 11.3 (range, 10.0-12.6; standard deviation [SD], 0.7) mm and 10.2 (range, 8.5-12.3; SD, 0.8) mm respectively, whereas in women were 10.9 (range, 9.4-13.2; SD, 0.8) mm and 10.1 (range, 7.9-11.6; SD, 0.9) mm respectively. The difference in corresponding mean dimensions between men and women was not statistically significant. The mean AP diameter was significantly larger than the mean transverse diameter. At the base and 1.2 mm and 2.4 mm above the base, the respective transverse diameters of 4 (5%), 13 (15%), and 24 (28%) of the patients were <9.0 mm. None had an odontoid AP diameter of <9.0 mm at any level. CONCLUSION: Two 3.5-mm cortical screws appear too big for fixation in one third of our sample presenting with type-II odontoid fracture. Fixation by two 2.7-mm screws is recommended for Malaysians and other Asian populations. (+info)
Pure endoscopic endonasal odontoidectomy: anatomical study.
(47/133)
Different disorders may produce irreducible atlanto-axial dislocation with compression of the ventral spinal cord. Among the surgical approaches available for a such condition, the transoral resection of the odontoid process is the most often used. The aim of this anatomical study is to demonstrate the possibility of an anterior cervico-medullary decompression through an endoscopic endonasal approach. Three fresh cadaver heads were used. A modified endonasal endoscopic approach was made in all cases. Endoscopic dissections were performed using a rigid endoscope, 4 mm in diameter, 18 cm in length, with 0 degree lenses. Access to the cranio-vertebral junction was possible using a lower trajectory, when compared to that necessary for the sellar region. The choana is entered and the mucosa of the rhinopharynx is dissected and transposed in the oral cavity in order to expose the cranio-vertebral junction and to obtain a mucosal flap useful for the closure. The anterior arch of the atlas and the odontoid process of C2 are removed, thus exposing the dura mater. The endoscopic endonasal approach could be a valid alternative to the transoral approach for anterior odontoidectomy. (+info)
Posterior atlantoaxial subluxation due to os odontoideum combined with cervical spondylotic myelopathy: a case report.
(48/133)
In patients with os odontoideum and posterior atlantoaxial subluxation are extremely rare. No reports have described posterior atlantoaxial subluxation associated with os odontoideum combined with cervical spondylotic canal stenosis, both of which require surgical treatment. We report one case of a 75-year-old female who underwent arthrodesis between the occiput and C3 using a hook-and-rod system and also a double-door laminoplasty from levels C3 to C7. The claw mechanism was applied between the C2 lamina and the C3 inferior articular process. The posterior atlantoaxial subluxation was completely reduced by the method that the rod gradually pushed the posterior arch of C1 anteriorly during connection to the occiput. Twelve months after surgery, the patient showed improvement in preoperative clumsiness and gait disturbance, and the latest plain radiographs showed solid osseous fusion, with no loss of correction or instrumentation failure. (+info)