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

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 c...

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Autores principales: Morris, Cade A., Forrester, D. Alex, Zanabrie, Rosemarie, Puffinbarger, William, Borden, Neil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10586849/
https://www.ncbi.nlm.nih.gov/pubmed/37603712
http://dx.doi.org/10.5435/JAAOSGlobal-D-22-00111
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author Morris, Cade A.
Forrester, D. Alex
Zanabrie, Rosemarie
Puffinbarger, William
Borden, Neil
author_facet Morris, Cade A.
Forrester, D. Alex
Zanabrie, Rosemarie
Puffinbarger, William
Borden, Neil
author_sort Morris, Cade A.
collection PubMed
description 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.
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spelling pubmed-105868492023-10-20 Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis Morris, Cade A. Forrester, D. Alex Zanabrie, Rosemarie Puffinbarger, William Borden, Neil J Am Acad Orthop Surg Glob Res Rev Case Report 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. Wolters Kluwer 2023-08-21 /pmc/articles/PMC10586849/ /pubmed/37603712 http://dx.doi.org/10.5435/JAAOSGlobal-D-22-00111 Text en Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Orthopaedic Surgeons. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Morris, Cade A.
Forrester, D. Alex
Zanabrie, Rosemarie
Puffinbarger, William
Borden, Neil
Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis
title Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis
title_full Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis
title_fullStr Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis
title_full_unstemmed Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis
title_short Spontaneous Unilateral Chiari I Secondary to Acquired Tonsillar Hypertrophy/Pseudomass With Syringomyelia in a Juvenile With Progressive Scoliosis
title_sort spontaneous unilateral chiari i secondary to acquired tonsillar hypertrophy/pseudomass with syringomyelia in a juvenile with progressive scoliosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10586849/
https://www.ncbi.nlm.nih.gov/pubmed/37603712
http://dx.doi.org/10.5435/JAAOSGlobal-D-22-00111
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