Atlas Hypoplasia Manifesting as Myelopathy in a Child. Case Report.

Urasaki, Eiichirou; Yasukouchi, Hideoki; Yokota, Akira

doi:10.2176/nmc.41.160

Cited by 29 publications

(19 citation statements)

References 10 publications

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“…Symptomatic developmental cervical stenosis at the level of the atlas and surgical outcome have been reported in 12 pediatric patients 2,9,19,20 (Table 1). This abnormality is considered rare because congenital cervical canal stenosis usually occurs below the level of C-2.…”

Section: Discussionmentioning

confidence: 99%

“…Finally, our 2 cases of atlantal hypoplasia associated with Williams syndrome support the supposition of a genetic basis for the malformation of the atlas. 17,20 A microdeletion of 1.5-1.8 million base pairs on the long arm of chromosome 7, known as the Williams-Beuren syndrome chromosome region, underlies the genetic basis of the syndrome. 16 Interestingly, the short arm of chromosome 7 also contains the HOXA1 gene, which in conjunction with other Hox genes, is responsible for development of the craniovertebral junction.…”

Section: Discussionmentioning

confidence: 99%

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

Desai

Vadivelu

Patel

et al. 2013

SPI

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Desai

Vadivelu

Patel

et al. 2013

SPI

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“…Procedures may be indicated in the following cases: (1) myelopathy due to compression of surrounding soft tissue or nomadic posterior tubercle or hypoplastic spinal canal stenosis [3,11,12]. (2) Atlantoaxial instability [7,8,13,14] due to injuries or other associating deformities.…”

Section: Discussionmentioning

confidence: 99%

Post atlantoaxial fusion for unilateral cleft of atlas posterior arch associated with os odontoideum: case report and technique note

Yang

Yan

et al. 2011

Eur Spine J

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A case of a 34-year-old female with unilateral cleft of atlas posterior arch associated with os odontoideum is reported. The patient had experienced neck pain for 6 months. Five days earlier to admission the pain aggravated as a result of mild head trauma from behind. Imaging examinations revealed C1-2 subluxation as well as the deformity. After 3 days of skull traction, a sound C1-2 reduction was achieved. Post atlantoaxial fusion using bilateral transarticular screws combined with C1 laminar hook on the intact side and autogenous bone graft was performed. On the sixth month of postoperative follow-up, CT revealed solid fusion was achieved. No related complications were detected within 3 years of follow-up. The clinical manifestations and imaging findings were presented. The incidence and etiopathogenesis of hypoplastic posterior arch of the atlas were concisely introduced. Techniques of post atlantoaxial fusion under circumstances of unilateral C1 posterior elements defects were discussed. The authors believe bilateral transarticular screws combined with C1 laminar hook on the intact side and autogenous bone graft can be applicable to atlantoaxial fusion on the premise of preoperative C1-2 reduction and C1 posterior arch remaining [1/2 of its full length.

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“…This type of acquired retroodontoid mass lesion is one of the reasons for the late appearance of clinical symptoms, because the congenital hypoplastic atlas usually does not result in serious neurological signs. 3,8,[13][14][15] The normal sagittal canal diameter at the level of the atlas is 16 to 20 mm in Asians. 8,10,12) If the canal is less than 10 mm, clinical manifestations usually occur.…”

Section: Discussionmentioning

confidence: 99%

“…6,8) Only 12 cases of atlas hypoplasia have been reported. [3][4][5]8,10,[12][13][14][15] On the other hand, various types of non-traumatic retro-odontoid extradural mass lesions may cause compression of the spinal cord, including rheumatoid pannus, 18) calcium pyrophosphate dihydrate (CPPD) deposition disease, 1,17) ossification of the transverse ligament, 7,13) herniated disc, 9) and synovial cyst. 2) Only three cases combining atlas hypoplasia and non-traumatic retro-odontoid soft tissue mass have been reported.…”

Section: Introductionmentioning

confidence: 99%

Atlas Hypoplasia Associated With Non-traumatic Retro-odontoid Mass

Sato

Kubota

Takeuchi

et al. 2006

Neurol. Med. Chir.(Tokyo)

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A 38-year-old man presented with progressive cervical myelopathy due to atlas hypoplasia associated with non-traumatic retro-odontoid mass. The neuroimaging findings suggested hypertrophy of the transverse ligament of the atlas. No histological confirmation of the retro-odontoid mass was obtained. Clinical manifestations improved after posterior decompression. Decompressive laminectomy of the atlas with or without fusion can achieve a good outcome in such cases.

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Atlas Hypoplasia Manifesting as Myelopathy in a Child. Case Report.

Cited by 29 publications

References 10 publications

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

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

Post atlantoaxial fusion for unilateral cleft of atlas posterior arch associated with os odontoideum: case report and technique note

Atlas Hypoplasia Associated With Non-traumatic Retro-odontoid Mass

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