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Radiological evaluation of the craniocervical junction in bone dysplasias and other related syndromes

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      Abstract

      The craniocervical junction, a vital but anatomically complex region, presents additional interpretative challenges in patients with skeletal dysplasias and other syndromes involving the musculoskeletal apparatus. Our objective was to review the radiological evaluation used in such conditions; to do so, we identified retrospectively all of the occipitocervical studies done during the last 3 years at our institution on patients with skeletal dysplasias and related syndromes. The 28 cases thus found were reviewed and the different diagnostic modalities identified; the diagnostic findings were summarized and classified into osseous and soft tissue abnormalities. The standard criteria for cervical instability and cord compression were compared with measurements reported in our selected cases. The lateral radiograph of the cervical spine was the first diagnostic test, followed by additional flexion-extension views and sagittal spin-echo magnetic resonance imaging; no conclusive diagnostic criteria for occipitoatlantal instability in children with bone dysplasias were identified.

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      References

        • LeDoux MS
        • Naftalis RC
        • Aronin PA
        Stabilization of the cervical spine in spondyloepiphyseal dysplasia congenita.
        Neurosurgery. 1991; 28: 580-583
        • Ojea JCG
        • Grana JM
        • Claderoni AM
        Subluxación occipitoatloidea en síndrome de Down.
        Radiologia. 1993; 6: 346-347
        • Harkey HL
        • Crockard HA
        • Stevens JM
        • Smith R
        • Ransford AO
        The operative management of basilar impression in osteogenesis imperfecta.
        Neurosurgery. 1990; 27: 782-786
        • Skeletal Dysplasia Group
        Instability of the upper cervical spine.
        Arch Dis Child. 1989; 64: 283-288
        • White KS
        • Ball WS
        • Prenger EC
        • Patterson BJ
        • Kirks DR
        Evaluation of the craniocervical junction in Down syndrome: correlation of measurements obtained with radiography and MR imaging.
        Radiology. 1993; 186: 377-824
        • Thomas JT
        • Frias JL
        • Williams JL
        • Friedman WA
        Magnetic resonance imaging in the assessment of medullary compression in achondroplasia.
        American Journal of Diseases of Children. 1988; 142: 989-992
        • Carson B
        • Winfield J
        • Wang H
        • Reid C
        • McPherson R
        • Kopits S
        • Uematsu S
        Surgical management of cervicomedullary compression in achondroplastic patients.
        Basic Life Sci. 1988; 48: 207-214
        • Hecht JT
        • Butler IJ
        Neurologic morbidity associated with achondroplasia.
        J Child Neurol. 1990; 5: 84-99
        • Moskowitz N
        • Carson B
        • Kopits S
        • Levitt R
        • Hart G
        Foramen magnum decompression in an infant with homozygous achondroplasia.
        J Neurosurg. 1989; 70: 126-128
        • Brooke DC
        • et al.
        Asymptomatic occipito-atlantal instability in Down syndrome (trisomy 21).
        J Bone Joint Surg [Am]. 1987; 69: 293
        • Stevens JM
        • Kendall BE
        • Crockard HA
        • Ransford A
        The odontoid process in Morquio-Brailsford's disease. The effects of occipitocervical fusion.
        J Bone Joint Surg [Br]. 1991; 73: 851-858
      1. Taybi H, Ladiman (eds). Radiology of Syndromes, Metabolic Disorders and Skeletal Dysplasias, 3rd ed. Year Book Medical

        • Greenberg AD
        Atlanto-axial dislocations.
        Brain. 1968; 91: 655-684
        • Roach JW
        • Duncan D
        • Wenger DR
        • Maravilla A
        • Maravilla K
        Atlanto-axial instability and spinal cord compression in children — diagnosis by computerized tomography.
        J Bone Joint Surg [Am]. 1984; 66: 708-714
        • Juhl JM
        4th ed. Essentials of Roentgen Interpretation. 78. Harper & Row, 1981
        • Chamberlain WE
        Basilar impression (platybasia).
        Yale J Biol Med. 1939; 11: 487
        • Wiesel SW
        • Rothman RH
        Occipitoatlantal hypermobility.
        Spine. 1979; 4: 187-191
        • Rosenbaum DM
        • Blumhagen JD
        • King HA
        Atlantooccipital instability in Down syndrome.
        AJR. 1986; 146: 1269-1272
        • El-Khoury GY
        • Clark CR
        • Dietz FR
        • Harre RG
        • Tozzi JE
        • Kathol MH
        Posterior atlantooccipital subluxation in Down syndrome.
        Radiology. 1986; 159: 507-509
        • Stein SM
        • Kirchner SG
        • Horev G
        • Hernanz-Schulman M
        Atlantooccipital subluxation in Down syndrome.
        Pediatr Radiol. 1991; 21: 121-124
        • Kaufman RA
        • Carroll CD
        • Buncher CR
        Atlantooccipital junction: standards for measurement in normal children.
        AJNR. 1987; 8: 995-999