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Spontaneous resolution of a Chiari I malformation and cervicothoracic syrinx in a 9 years old girl with a 47° scoliosis responding favourably to bracing

Background

Pediatric case reports support the spontaneous resolution of Chiari I and syrinx. We present a case of a 9 years old girl with a spontaneous regression of a cervico-thoracic syrinx and a complete spontaneous resolution of the Chiari I malformation after one year of bracing to treat her scoliosis.

Case report

An 8 years old girl was first diagnosed with JIS. She presented a right thoracic single curve (Apex at T10) of 36° Cobb and ± 10° rotation. Normal sagittal configuration. MRI demonstrated a Chiari I malformation and a cervicothoracic syrinx (C4 to T9-10). She attended our clinic later showing a rapid progression of the Cobb angle to 47° Cobb. Absent superficial abdominal reflexes was the only neurological sign. Bracing with a RSC was indicated. After one year of treatment (in brace correction 47%) the scoliometer value reduced from a total value of 9.5° to 6°. Back asymmetry has dramatically improved. A second MRI showed a reduced syrinx and no Chiari I malformation.

Discussion and conclusion

This is a first report of a case showing a temporary improvement of a conservatively treated scoliosis coinciding with a spontaneous resolution of a Chiari I malformation and a related cervico-thoracic syrinx. The mechanism of such a resolution is not clear. However this case supports the idea that the resolution of the Chiari I and its related syrinx would improve the prognosis of the associated scoliosis.

References

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Author information

Correspondence to Manuel Rigo.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Keywords

  • Public Health
  • Rapid Progression
  • Cobb Angle
  • Neurological Sign
  • Pediatric Case