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PubMed1982

Management of odontoid fractures.

Fifty-one adults with odontoid fractures were treated between 1966 and 1979. Approximately 50% had additional trauma; over half of the patients were younger than 40 years old. Three patients presented with myelopathy: 1 was complete at C-2 and the others recovered. Cervical roentgenograms were initially considered normal in 4 patients. The fracture was through the base of the odontoid process (Type 2) in 49 patients and was into the body of C-2 (Type 3) in 2 patients. Thirty-four patients underwent early posterior cervical fusion, whereas the others were treated initially with external immobilization. Only the 2 patients with Type 3 fractures healed in Minerva casts. All 15 Type 2 fractures initially treated conservatively failed to heal and were fused. There were no age, sex, or radiological characteristics (other than fracture location) peculiar to the nonunion group. Two patients died after operation, and there was 1 minor infection in an iliac crest donor site. All patients who were operated upon demonstrated evidence of fusion within 6 weeks after operation; 16 demonstrated healing of the fracture at 6 months. After follow-up ranging from 16 months to 15 years, 7 patients complain of neck pain, of whom 6 do not require regular analgesics. Of 40 patients tested, 7 have evidence of decreased range of motion in the neck, of whom 6 have loss of less than 15% of normal motion. Traction followed by posterior cervical fusion is effective treatment for Type 2 odontoid fractures. The surgical and long term morbidity is low, and satisfactory fusion can be expected.

Adolescent↗
PubMed1978

Atlantoaxial malformation in a 46,XY, 17q+ child.

A child with 46,XY, 17q+ chromosome aberration and unusual skeletal abnormalities had the presenting symptom of respiratory arrest after a fall and minor head trauma. He exhibited proportionate short stature, peculiar facies with antimongoloid slant, simian creases, postaxial hexadactyly, metatarsus adductus deformity, and anomalous penoscrotal configuration. Delayed speech and mild mental retardation were also present. Atlantoaxial dislocation secondary to odontoid process hypoplasia was demonstrated. Additional skeletal abnormalities included dysplastic changes in the first and fourth metacarpals, the middle phalanx of the second finger, and hypoplasia of the first metatarsal, with medial insertion of the great toe. Treatment consisted of halo jacket cast and fusion of the occiput to C1, C2, and C3. Early detection and preventive surgery for atlantoaxial dislocation is necessary to prevent neurological deficit and possibly death by respiratory failure.

Axis, Cervical Vertebra↗
PubMed1976

Myelopathy in infancy complicating congenital atlantoaxial dislocation.

Two infants had congenital atlantoaxial dislocation associated with myelopathy. One of them, a 3-month-old girl, showed anterior dislocation of the atlas on the axis, and had extreme weakness of the extremities. Another patient, an 8-month-old boy, showed tetraplegia, and posterior dislocation with absent odontoid process was demonstrated radiologically. These cases add another cause to the varied etiologies of floppiness in early infancy.

Axis, Cervical Vertebra↗
PubMed1997

Another "new" form, the palagonia type of acrofacial dysostosis in a Sicilian family.

We described another previously apparently unreported form of acrofacial dysostosis (AFD) from Sicily, residing, coincidentally in the same small village as that with the recently delineated Catania AFD. In contra-distinction to the latter, the 4 patients with the Palagonia form of AFD are of normal intelligence, and instead of extensive caries have oligodontia (4), short stature (3), frizzy hair (pili torti) with aplasia cutis verticis (1), mild cutaneous syndactyly of digits 2-5 (4), attenuation of the 4th metacarpals (3/3), unilaterally cleft lip (1), and some vertebral anomalies such as a large atlas (1), mild scoliosis (1), small odontoid process, spina bifida occulta at S1 (1). Casually, this would appear to be an iceberg dominant disorder, with the proposita most severely affected. This could be an X-linked dominant, but more likely an autosomal dominant trait.

Abnormalities, Multiple↗
PubMed1990

A "new" skeletal dysplasia in two unrelated boys.

We report on 2 unrelated boys with similar physical and radiographic findings that may represent a "new" skeletal dysplasia. Findings in common include early speech delay, short stature, frontal bossing with a depression over the metopic suture, a narrow nasal root with beaked nose, midfacial hypoplasia with relatively prominent eyes, and brachydactyly with blunt fingers. Radiographic findings include mild irregularities of the vertebral bodies, hypoplasia of the odontoid process, short phalanges with increased distal width, coning and sclerosis of several epiphyses, and overtubulation of the long bones. Although these patients share some manifestations with the floating-harbor syndrome (Robinson et al.: J Pediatr 113:703-706, 1988), their radiographic changes are distinctive and are not suggestive of a recognized skeletal dysplasia syndrome.

Bone Diseases, Developmental↗
PubMed1994

New autosomal dominant form of spondyloepiphyseal dysplasia presenting with atlanto-axial instability.

We present a family with a radiologically distinct new form of autosomal dominant spondyloepiphyseal dysplasia, presenting with cervical instability and attendant neurological compromise and emphasise the radiological characteristics which delineate this condition. Cervical vertebral abnormalities, including malformation of the odontoid process, have been observed in some forms of spondyloepiphyseal dysplasia, but rarely lead to neurological sequelae, in contrast to the pedigree we describe.

Atlanto-Axial Joint↗
PubMed1978

The cervical myelopathy associated with rheumatoid arthritis: analysis of patients, with 2 postmortem cases.

The clinical findings in a group of 32 rheumatoid patients with symptomatic myelopathy are described. The myelopathy appeared in late middle age after many years of rheumatoid disease (average duration, 18 years). The most common radiological abnormality was anterior subluxation of C1. Other patterns occurred in about half the cases. The most common neurological findings were those of a mild to moderate spastic paraplegia, often with atrophy of muscles of the hands and sensory changes in the hands. Symptoms originating above the foramen magnum (nystagmus, diplopia, slurred speech) appeared to have a vascular cause and were sometimes associated with upward as well as posterior movement of the odontoid process. Root pain into the arms was not present, and its absence helped to distinguish these disorders from cervical spondylosis. Root pain into the territory of the C2 root was common. Medical or conservative therapy was not effective, and the preferred approach was use of a halo traction device followed by posterior fusion, with or without laminectomy. Pathologically, in 2 autopsy cases, the maximal change had occurred in the central gray matter and adjacent posterior and lateral columns. We postulate that direct pressure caused intermittent compression and narrowing of distal transverse branches of the anterior spinal artery.

Arthritis, Rheumatoid↗
PubMed1990

Pseudotumor of the craniocervical junction during long-term hemodialysis.

A systematic study of the upper cervical spine was performed using magnetic resonance imaging in 25 patients (15 men and 10 women) who had been undergoing hemodialysis for more than 10 years. Seven pseudotumors of the periodontoid soft tissue were disclosed, which were similar to the pannus recently described in rheumatoid arthritis. Bone cystic radiolucencies were observed in association with these pseudotumors in 5 patients. The radiolucencies were located in the atlas (1 in the lateral mass and 1 in the anterior branch) and in the axis (3 in the odontoid process and 1 in the vertebral body). No horizontal or vertical atlantoaxial subluxation was demonstrated. These features were observed only in patients who had amyloid arthropathy. They could be a frequent, yet thus far little-recognized, feature of beta 2-microglobulin amyloidosis.

Adolescent↗
PubMed2005

Unusual finding of the craniocervical junction.

The authors report a rare anomaly of the upper cervical spine. After an automobile accident, an 8-year-old child underwent CT of the head. Imaging showed a bony anomaly of the neck that was further imaged with thin cuts through the atlas and axis. This demonstrated an unfused anterior arch of the atlas and fusion of the odontoid process to the anterior arch of the atlas. Neurologically, the child is normal with no decreased range of motion about the cervical spine. After a review of the literature, this seems to be only the sixth reported case.

Cervical Vertebrae↗
PubMed1988

Biomechanics of the craniocervical region: the alar and transverse ligaments.

In the treatment of spine fractures and fracture-dislocations, stability of the spine is one of the major objectives. In the craniocervical joint, the alar and transverse ligaments provide much of the stability of the healthy spine. Because the anatomy appears well described, the contribution of each of these structures so far has received little attention. The alar ligament restrains rotation of the upper cervical spine, whereas the transverse ligament restricts flexion as well as anterior displacement of the atlas. A lesion in one or both structures can produce damage to the neural structures and/or cause pain. To investigate the possible role of each of these ligaments, a mechanical and histologic study of the upper cervical spine was made. The bone-ligament-bone complex of the alar and transverse ligaments was subjected to uniaxial mechanical testing in seven specimens. The alar ligaments had an in vitro strength of 200 N, and the transverse ligaments had an in vitro strength of 350 N. Histologic analysis revealed a mainly collagenous nature of these ligaments. Clinical evidence (broken odontoid processes) suggests that the transverse ligament is strong enough to withstand physiologic loads. The alar ligament, on the other hand, due to its lower strength and its axial direction of loading, might be prone to injury and therefore require stabilization of the appropriate vertebra more often than normally is assumed.

Aged↗
PubMed1980

Craniosynostosis with associated cranial base anomalies: a morphologic and histologic study of affected like-sexed twins.

A set of like-sexed male twins with the same malformation syndrome and exhibiting identical anomalous alterations of the craniofacial complex were studied in detail. The right and left coronal and left lamboidal sutures were prematurely synostosed in both specimens. The cranial base exhibited a mediolateral asymmetry, with the right half larger than the left. The occipital bone demonstrated the greatest abnormality, including bilateral clefting of the basioccipital component and alterations in the size and shape of the exoccipital bone. In lateral view, the cranial base was flattened and the ventral flexure occurred between the two components of the basioccipital. The odontoid process of the second cervical vertebra was rotated anteroinferiorly and formed part of the cranial floor. Histologically, the bilateral clefts of the basioccipital bone were united by fibrous connective tissue, and the central area of union contained chondroid-like bone. The cartilaginous spheno-occipital synchondrosis and the epiphyseal cartilage of the finger demonstrated similar dystrophic alterations. The brain was normal but exhibited external cerebral deformation secondary to aberrant skull shape.

Abnormalities, Multiple↗
PubMed1986

The orientation of the cervical vertebral column in unrestrained awake animals. I. Resting position.

The orientation of the cervical vertebral column was studied by X-ray photography of the region containing the head and the neck in nine unrestrained species of vertebrates (man, monkey, cat, rabbit, guinea pig, rat, chicken, frog, lizard). In addition, the orientation of the horizontal semicircular canals was measured in four species using landmarks on the skull. In all vertebrates studied, with the exception of frog and lizard, the general orientation of the cervical vertebral column was vertical when animals were at rest, and not horizontal or oblique as suggested by the macroscopic appearance of the neck. The posture of the animal, whether lying, sitting or standing, had little effect on this general vertical orientation, although some variability was noticed depending on the species. This finding prompted the definition of a resting zone, where the cervical column can take any orientation within a narrow range around a mean position. The cervical vertebral column composes part of the S-shaped structure of the entire vertebral column, with one inflection around the cervico-thoracic (C7/Th1) junction. This feature is already noticable in the lizard. The vertical orientation of the cervical vertebral column is interpreted to provide a stable and energy saving balance of the head. Furthermore, when the head is lowered or raised, the atlanto-occipital and cervico-thoracic junctions are predominantly involved, while the entire cervical column largely preserves its intrinsic configuration. The curved configuration of the cervico-thoracic vertebral column embedded in long spring-like muscles is interpreted to function as a shock absorber. At rest, animals did not hold their heads with the horizontal canals oriented earth horizontally all the time, but often maintained them pitched up by ca. 5 deg, as has been reported for man. At other times, presumably when the vigilance level increased, the horizontal canals were brought into the earth horizontal plane. The vertical orientation of the cervical column results in a vertical positioning of the odontoid process of the axis (second cervical vertebra, C2), which thus provides the axis of rotation for yaw movements of the head. This axis corresponds to that of the horizontal semicircular canals. The vertical organization of the cervical vertebral column in birds and mammals, whether the animal is quadrupedal or bipedal, points to a common organizational principle for eye and head movement systems.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1989

Marked canal stenosis at the level of the atlas.

A 38-year-old man with severe canal stenosis at the level of the atlas is reported. The clinical manifestations were muscular weakness and wasting of the upper limbs and spastic paresis of the lower limbs, which all progressed slowly. The atlas was hypoplastic and its retrodental space was narrow. The spinal cord was markedly compressed between the odontoid process and the posterior arch of the atlas. The clinical manifestations improved after a posterior laminectomy of the atlas.

Adult↗
PubMed1981

Atlantoaxial instability in Marfan's syndrome. Diagnosis and treatment. A case report.

A case of Marfan's syndrome is described in which progressive neurological signs and symptoms were produced by flexion of the head. Radiological examinations revealed hypermotility between the atlas and axis, as well as compression of the medulla oblongata by the odontoid process on flexion, since low positioned cerebellar tonsils prevented dorsal shift of the oblongata. The signs and symptoms disappeared after occipitocervical internal transfixation.

Adult↗
PubMed1991

Overdistraction: a hazard of skull traction in the management of acute injuries of the cervical spine.

In acute cervical spine trauma, skull traction is used to reduce a dislocation or fracture dislocation, to immobilize an unstable lesion until definitive treatment (operative or conservative) is possible or, more rarely, as a definitive treatment until healing occurs. This method may be dangerous when an unstable lesion is accidentally overdistracted. A few cases have been reported in the literature, some with neurological complications. We report five cases in which overdistraction was seen. Two hangman's fractures were overdistracted. One of the two patients developed a Cheyne-Stokes breathing pattern during traction which resolved after the weight was reduced. Furthermore, two hyperextension/distraction injuries (C4/5 and C6/7) and one bilateral C5/6 fracture dislocation were overdistracted without neurological deterioration. Occipitocervical dislocations, fractures of the odontoid process, hangman's fractures, hyperextension/distraction injuries and bilateral dislocations or fracture dislocations may present disruption of both the anterior and posterior elements. Therefore, these injuries are specially vulnerable to overdistraction when skull traction is used. To prevent accidental overdistraction during skull traction, we recommend the use of less weight than is generally proposed in the literature. To reduce a dislocation, we start traction weight at 2 kg and slowly increase it under continuous neurological and radiological monitoring until reduction is completed. Traction of 5-7 kg is usually sufficient; however, heavier traction may occasionally be necessary. After reduction is completed, traction is reduced to 2 kg. This weight is sufficient to immobilize a lesion until definitive treatment is possible. Inadvertent rotation may be prevented by placing sandbags on both sides of the head.

Adolescent↗
PubMed1991

Hajdu-Cheney syndrome: MR imaging.

Hajdu-Cheney syndrome is a rare congenital disease with acro-osteolysis, osteoporotic changes of the spine and long bones of extremities and marked basilar invagination with an unusually deformed skull. Magnetic resonance imaging of a 32-year-old male revealed the deformed skull and almost horizontal basal angle and the elongated and upwardly shifted brain stem caused by the tip of the odontoid process of the second cervical vertebra invaginating the base of the skull. In addition there were atrophic pituitary gland, widely open sella turcica and symmetrical fluid collections along the optic nerve sheath.

Adult↗
PubMed1993

Unusual mechanism of lethal cervical spinal cord injury in a case of atlanto-axial diastasis.

A case of traumatic cervical spinal cord injury due to congenital craniocervical abnormalities is described. The autopsy revealed evidence of hyperextension of the neck due to frontal impact, congenital occipitalization of the atlas accompanied by basilar invagination of the odontoid process and atlanto-axial diastasis. The subsequent narrowing of the spinal canal led to a predisposition for this unusual mechanism of spinal cord injury.

Accidents, Traffic↗