Isolated cervical spinal canal stenosis at C-1 in the pediatric population and in Williams syndrome

Clinical article

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  • 1 The Paul Randall Harrington Neuro-Spine Program and
  • | 2 Division of Pediatric Neurosurgery, Texas Children's Hospital, Department of Neurosurgery, Baylor College of Medicine, Houston; and
  • | 3 Division of Neurosurgery, University of Texas Medical Branch, Galveston, Texas
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Object

Isolated cervical canal stenosis at the level of the atlas (C-1) is a rare cause of cervical myelopathy in the pediatric population. It has been associated with several genetic disorders including spondyloepiphysial dysplasia congenita, Down syndrome, and Klippel-Feil syndrome. The purpose of this study is to highlight the authors' experience with 4 additional pediatric cases, review the literature, and report a new association of this disease with Williams syndrome.

Methods

The medical records and radiological imaging studies of 4 patients treated at Texas Children's Hospital for symptomatic hypoplasia of the atlas were retrospectively reviewed. Pertinent patient demographic data, clinical presentation, imaging findings, and outcomes after surgery were recorded. A thorough literature review was performed, allowing the authors to compare and contrast their 4 cases to surgical cases already published in the literature.

Results

There were 11 boys and 1 girl in the aggregate series. The average age of the patients was 7 years (range 13 months–14 years), and the duration of symptoms prior to presentation was 6 months (range 0–36 months). The mean sagittal diameter of the spinal canal at the level of the atlas measured from the posterior aspect of the dens to the anterior aspect of the arch of C-1 was 11.9 mm (range 8.3–16 mm) in the aggregate series. In 2 new pediatric patients with hypoplasia of the atlas the disease was associated with Williams syndrome, which has not been previously described. Patients in the aggregate series were followed for an average of 18 months (range 3–50 months). Laminectomy of C-1 provided neurological improvement in all patients who presented.

Conclusions

Isolated cervical spinal canal stenosis at the level of the atlas is a rare cause of cervical myelopathy. The authors hope that this report will prompt clinicians to consider it when searching for the origin of signs and symptoms of cervical myelopathy, especially in children.

Abbreviation used in this paper:

AP = anteroposterior.

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  • 1

    Currarino G, , Rollins N, & Diehl JT: Congenital defects of the posterior arch of the atlas: a report of seven cases including an affected mother and son. AJNR Am J Neuroradiol 15:249254, 1994. (Erratum in AJNR Am J Neuroradiol 15:A9, 1994)

    • Search Google Scholar
    • Export Citation
  • 2

    Devi BI, , Shenoy SN, , Panigrahi MK, , Chandramouli BA, , Das BS, & Jayakumar PN: Anomaly of arch of atlas—a rare cause of symptomatic canal stenosis in children. Pediatr Neurosurg 26:214218, 1997

    • Search Google Scholar
    • Export Citation
  • 3

    Ebara S, , Yonenobu K, , Fujiwara K, , Yamashita K, & Ono K: Myelopathy hand characterized by muscle wasting. A different type of myelopathy hand in patients with cervical spondylosis. Spine (Phila Pa 1976) 13:785791, 1988

    • Search Google Scholar
    • Export Citation
  • 4

    Greenberg AD: Atlanto-axial dislocations. Brain 91:655684, 1968

  • 5

    Hinck VC, , Gordy PD, & Storino HE: Developmental stenosis of the cervical spinal canal. radiological considerations. Neurology 14:864868, 1964

    • Search Google Scholar
    • Export Citation
  • 6

    Hinck VC, , Hopkins CE, & Savara BS: Sagittal diameter of the cervical spinal canal in children. Radiology 79:97108, 1962

  • 7

    Hinck VC, & Sachdev NS: Developmental stenosis of the cervical spinal canal. Brain 89:2736, 1966

  • 8

    Komatsu Y, , Shibata T, , Yasuda S, , Ono Y, & Nose T: Atlas hypoplasia as a cause of high cervical myelopathy. Case report. J Neurosurg 79:917919, 1993

    • Search Google Scholar
    • Export Citation
  • 9

    Liliang PC, , Lui CC, , Cheng MH, & Shih TY: Atlantal stenosis: a rare cause of quadriparesis in a child. Case report. J Neurosurg 92:2 Suppl 211213, 2000

    • Search Google Scholar
    • Export Citation
  • 10

    Martich V, , Ben-Ami T, , Yousefzadeh DK, & Roizen NJ: Hypoplastic posterior arch of C-1 in children with Down syndrome: a double jeopardy. Radiology 183:125128, 1992

    • Search Google Scholar
    • Export Citation
  • 11

    Matsunaga S, , Imakiire T, , Koga H, , Ishidou Y, , Sasaki H, & Taketomi E, et al.: Occult spinal canal stenosis due to C-1 hypoplasia in children with Down syndrome. J Neurosurg 107:6 Suppl 457459, 2007

    • Search Google Scholar
    • Export Citation
  • 12

    Nakamura K, , Miyoshi K, , Haga N, & Kurokawa T: Risk factors of myelopathy at the atlantoaxial level in spondyloepiphyseal dysplasia congenita. Arch Orthop Trauma Surg 117:468470, 1998

    • Search Google Scholar
    • Export Citation
  • 13

    Ogden JA: Radiology of postnatal skeletal development. XI. The first cervical vertebra. Skeletal Radiol 12:1220, 1984

  • 14

    Pang D, & Thompson DNP: Embryology and bony malformations of the craniovertebral junction. Childs Nerv Syst 27:523564, 2011

  • 15

    Phan N, , Marras C, , Midha R, & Rowed D: Cervical myelopathy caused by hypoplasia of the atlas: two case reports and review of the literature. Neurosurgery 43:629633, 1998

    • Search Google Scholar
    • Export Citation
  • 16

    Pober BR: Williams-Beuren syndrome. N Engl J Med 362:239252, 2010

  • 17

    Pober BR, & Filiano JJ: Association of Chiari I malformation and Williams syndrome. Pediatr Neurol 12:8488, 1995

  • 18

    Sabuncuoglu H, , Ozdogan S, , Karadag D, & Kaynak ET: Congenital hypoplasia of the posterior arch of the atlas: case report and extensive review of the literature. Turk Neurosurg 21:97103, 2011

    • Search Google Scholar
    • Export Citation
  • 19

    Tubbs RS, , Oakes WJ, & Blount JP: Isolated atlantal stenosis in a patient with idiopathic growth hormone deficiency, and Klippel-Feil and Duane's syndromes. Childs Nerv Syst 21:421424, 2005

    • Search Google Scholar
    • Export Citation
  • 20

    Urasaki E, , Yasukouchi H, & Yokota A: Atlas hypoplasia manifesting as myelopathy in a child—case report. Neurol Med Chir (Tokyo) 41:160162, 2001

    • Search Google Scholar
    • Export Citation
  • 21

    Vangilder JC, & Menezes AH: Craniovertebral junction abnormalities. Clin Neurosurg 30:514530, 1983

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