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CC BY 4.0 · Indian Journal of Neurosurgery 2024; 13(03): 279-281
DOI: 10.1055/s-0044-1788906
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An Unusual Case of Cerebellar Tonsillar Descent till C7 Vertebrae in a Case of Chiari Malformation with Meningomyelocele

Abhijit Acharya 
1   Department of Neurosurgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
,
A. K. Mahapatra
1   Department of Neurosurgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
,
Soubhagya Tripathy
1   Department of Neurosurgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
,
Souvagya Panigrahi
1   Department of Neurosurgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
,
Rama Chandra Deo 
1   Department of Neurosurgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
,
Satya Bhusan Senapati
1   Department of Neurosurgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
,
Mehnaj Begum
2   Department of Surgery, IMS and SUM Hospital, SOA University, Bhubaneswar, Odisha, India
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Abstract

Arnold–Chiari or Chiari malformations are a group of posterior fossa malformations that include the pons, medulla, and cerebellum. This group of disorders may consist of associated abnormalities like myelomeningocele and encephalocele. Chiari malformation has been classified majorly into four categories. Chiari I malformation has been defined as tonsillar descent more than 5 mm below the level of the foramen magnum. The maximum descent of tonsillar herniation reported is 27 mm below the foramen magnum around the C2 vertebrae. In this study, we report the case of a 4-month-old infant with swelling over the lumbosacral (LS) spine since birth with a gradually progressive increase in head size since the age of 2 months. The infant had occasional stridor with respiratory distress for 2 months. Magnetic resonance imaging (MRI) of the brain and spine revealed gross hydrocephalous with myelomeningocele at the L1–L5 level with associated Chiari malformation. The tonsillar descent was almost at the level of C7, which was unusual in his patient. The infant underwent a right ventriculoperitoneal shunt placement and myelomeningocele repair. It was decided that the malformation would be managed in the second sitting. The postoperative period was uneventful and the respiratory distress improved. Cases of tonsillar descent (Chiari I malformation) to the C7 vertebral level are very rare.

Keywords

Chiari malformation - myelomeningocele - cerebellar tonsillar herniation


Publication History

Article published online:
16 September 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Tubbs RS, Yan H, Demerdash A. et al. Sagittal MRI often overestimates the degree of cerebellar tonsillar ectopia: a potential for misdiagnosis of the Chiari I malformation. Childs Nerv Syst 2016; 32 (07) 1245-1248
    CrossrefPubMedSearch in Google Scholar
  • 2 de Arruda JA, Figueiredo E, Monteiro JL, Barbosa LM, Rodrigues C, Vasconcelos B. Orofacial clinical features in Arnold Chiari type I malformation: a case series. J Clin Exp Dent 2018; 10 (04) e378-e382
    PubMedSearch in Google Scholar
  • 3 Abd-El-Barr MM, Strong CI, Groff MW. Chiari malformations: diagnosis, treatments and failures. J Neurosurg Sci 2014; 58 (04) 215-221
    PubMedSearch in Google Scholar
  • 4 Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ. The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 1998; 89 (02) 212-216
    CrossrefPubMedSearch in Google Scholar
  • 5 Arnautovic A, Splavski B, Boop FA, Arnautovic KI. Pediatric and adult Chiari malformation type I surgical series 1965-2013: a review of demographics, operative treatment, and outcomes. J Neurosurg Pediatr 2015; 15 (02) 161-177
    CrossrefPubMedSearch in Google Scholar
  • 6 Giammattei L, Borsotti F, Parker F, Messerer M. Chiari I malformation: surgical technique, indications and limits. Acta Neurochir (Wien) 2018; 160 (01) 213-217
    CrossrefPubMedSearch in Google Scholar
  • 7 Markunas CA, Enterline DS, Dunlap K. et al. Genetic evaluation and application of posterior cranial fossa traits as endophenotypes for Chiari type I malformation. Ann Hum Genet 2014; 78 (01) 1-12
    CrossrefPubMedSearch in Google Scholar
  • 8 Dlouhy BJ, Dawson JD, Menezes AH. Intradural pathology and pathophysiology associated with Chiari I malformation in children and adults with and without syringomyelia. J Neurosurg Pediatr 2017; 20 (06) 526-541
    CrossrefPubMedSearch in Google Scholar
  • 9 Rocque BG, Oakes WJ. Surgical treatment of Chiari I malformation. Neurosurg Clin N Am 2015; 26 (04) 527-531
    CrossrefPubMedSearch in Google Scholar