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Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis – Lumbar Spinal Stenosis

This article discusses a rare case of Chiari type 1 malformation (CM-1) with syringomyelia and scoliosis in a 9-year-old boy. CM-1 is a structural defect in which part of the cerebellar tonsils descend below the foramen magnum. Typically, CM-1 is believed to be congenital, but in this case, it occurred spontaneously without any known underlying cause. The patient had scoliosis that progressed rapidly, prompting a whole spine MRI that revealed the CM-1. Surgical decompression was performed, and postoperative MRI showed improvement. This case highlights the importance of routine MRI screenings for early detection of CM-1 in select patients with idiopathic scoliosis

Summarised by Mr Mo Akmal – Lead Spinal Surgeon
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Published article

Chiari type 1 malformation (CM-1) is a structural defect of the central nervous system in which part of the cerebellar tonsils descend below the level of the foramen magnum, sometimes with associated syringomyelia. Although Chiari malformations were traditionally believed to be congenital, several cases of acquired CM-1 with syringomyelia have been reported. Usually associated with repeat lumbar puncture, increased intracranial pressure, and craniocephalic disproportion, CM-1 in the absence of…

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J Am Acad Orthop Surg Glob Res Rev. 2023 Aug 21;7(8). doi: 10.5435/JAAOSGlobal-D-22-00111. eCollection 2023 Aug 1.ABSTRACTChiari type 1 malformation (CM-1) is a structural defect of the central nervous system in which part of the cerebellar tonsils descend below the level of the foramen magnum, sometimes with associated syringomyelia. Although Chiari malformations were traditionally believed,

J Am Acad Orthop Surg Glob Res Rev. 2023 Aug 21;7(8). doi: 10.5435/JAAOSGlobal-D-22-00111. eCollection 2023 Aug 1.

ABSTRACT

Chiari type 1 malformation (CM-1) is a structural defect of the central nervous system in which part of the cerebellar tonsils descend below the level of the foramen magnum, sometimes with associated syringomyelia. Although Chiari malformations were traditionally believed to be congenital, several cases of acquired CM-1 with syringomyelia have been reported. Usually associated with repeat lumbar puncture, increased intracranial pressure, and craniocephalic disproportion, CM-1 in the absence of an underlying etiology is rare. We report a rare case of spontaneous idiopathic tonsillar hypertrophy causing unilateral CM-1 with syringomyelia associated with progressive scoliosis in a juvenile with a previously normal neonatal MRI brain and no known underlying pathology. A 9-year-old boy was found to have scoliosis at a routine well-child visit with progression indicated on radiographs 4 months later. Whole spine MRI was performed and showed a new CM-1 with globular, mass-like configuration of the descended right tonsil with otherwise normal tonsillar characteristics. Surgical decompression via suboccipital craniectomy and C1 laminectomy with duraplasty was performed with improvement illustrated on repeat MRI 3 months postoperatively. This rare case emphasizes the importance of routine MRI spine early in select patients with idiopathic scoliosis and illustrates the favorable outcomes noted after decompressive craniectomy.

PMID:37603712 | DOI:10.5435/JAAOSGlobal-D-22-00111

The London Spine Unit : best recognised spine clinic in UK

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Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis

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J Am Acad Orthop Surg Glob Res Rev. 2023 Aug 21;7(8). doi: 10.5435/JAAOSGlobal-D-22-00111. eCollection 2023 Aug 1.ABSTRACTChiari type 1 malformation (CM-1) is a structural defect of the central nervous system in which part of the cerebellar tonsils descend below the level of the foramen magnum, sometimes with associated syringomyelia. Although Chiari malformations were traditionally believed
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