Abstract
Background
Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended. When radiographic indicators of craniovertebral junction (CVJ) instability or symptoms related to ventral brainstem compression are present, CVJ fixation should also be considered.
Case description
We report the case of a 13-year-old girl who presented with severe tetraparesis after posterior decompression for Chiari malformation type 1.5, followed 5 days later by partial C2 laminectomy. Several months after the initial surgery, she underwent two fixations, first without and then with intraoperative cervical traction, leading to significant neurological improvement.
Discussion and conclusion
This case report underscores the importance of meticulous radiological analysis before CM surgery. For CM 1.5 patients with basilar invagination, CVJ fixation is recommended, and C2 laminectomy should be avoided. In the event of significant clinical deterioration due to nonadherence to these guidelines, our findings highlight the importance of traction with increased extension before fixation, even years after initial destabilizing surgery.
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References
Sangwanloy P, Vaniyapong T, Norasetthada T, Jetjumnong C (2022) Influence of clivo-axial angle on outcome after foramen magnum decompression in adult symptomatic Chiari type 1 malformation. Clin Neurol Neurosurg 216:107214
Chiari H (2008) Concerning alterations in the cerebellum resulting from cerebral hydrocephalus. Pediatr Neurosurg 13(1):3–8
Cools MJ, Wellons JC, Iskandar BJ (2023) The nomenclature of Chiari malformations. Neurosurg Clin N Am 34(1):1–7
Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ (2004) A critical analysis of the Chiari 1.5 malformation. J Neurosurg 101(2 Suppl):179–183
Del Gaudio N, Vaz G, Duprez T, Raftopoulos C (2018) Comparison of dural peeling versus duraplasty for surgical treatment of Chiari type I malformation: results and complications in a monocentric patients’ cohort. World Neurosurg 117:e595–602
Massimi L, Frassanito P, Bianchi F, Tamburrini G, Caldarelli M (2019) Bony decompression vs duraplasty for Chiari I malformation: does the eternal dilemma matter? Childs Nerv Syst 35(10):1827–1838
Ciaramitaro P, Massimi L, Bertuccio A, Solari A, Farinotti M, Peretta P et al (2022) Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document. Neurol Sci 43(2):1327–1342
Kim LJ, Rekate HL, Klopfenstein JD, Sonntag VKH (2004) Treatment of basilar invagination associated with Chiari I malformations in the pediatric population: cervical reduction and posterior occipitocervical fusion. J Neurosurg Pediatr 101(2):189–195
Bollo RJ, Riva-Cambrin J, Brockmeyer MM, Brockmeyer DL (2012) Complex Chiari malformations in children: an analysis of preoperative risk factors for occipitocervical fusion: Clinical article. J Neurosurg Pediatr 10(2):134–141
Grabb PA, Mapstone TB, Oakes WJ (1999) Ventral brain stem compression in pediatric and young adult patients with Chiari I malformations. Neurosurgery 44(3):520
Felbaum D, Spitz S, Sandhu FA (2015) Correction of clivoaxial angle deformity in the setting of suboccipital craniectomy: technical note. J Neurosurg Spine 23(1):8–15
Henderson FC, Henderson FC, Wilson WA, Mark AS, Koby M (2018) Utility of the clivo-axial angle in assessing brainstem deformity: pilot study and literature review. Neurosurg Rev 41(1):149–163
Klekamp J (2015) Chiari I malformation with and without basilar invagination: a comparative study. Neurosurg Focus 38(4):E12
Goel A (2015) Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine 22(2):116–127
Loe ML, Vivas-Buitrago T, Domingo RA, Heemskerk J, Tripathi S, Bendok BR et al (2020) Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale. J Neurosurg Spine 34(2):171–177
Koenigsberg RA, Vakil N, Hong TA, Htaik T, Faerber E, Maiorano T et al (2005) Evaluation of platybasia with MR imaging. AJNR Am J Neuroradiol 26(1):89–92
Massimi L, Peretta P, Erbetta A, Solari A, Farinotti M, Ciaramitaro P et al (2022) Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document. Neurol Sci 43(2):1311–1326
Goel A, Bhatjiwale M, Desai K (1998) Basilar invagination: a study based on 190 surgically treated patients. J Neurosurg 88(6):962–968
Goel A (2004) Treatment of basilar invagination by atlantoaxial joint distraction and direct lateral mass fixation. J Neurosurg Spine 1(3):281–286
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EV wrote the paper, reviewed the litterature and followed the patient; NdG and LdN reviewed the paper, followed the patient and assisted the senior surgeon during the surgery; CR defined the strategy, operated on the patient and reviewed the paper.
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Vigneul, E., Del Gaudio, N., de Nijs, L. et al. Neurological outcome following delayed traction and fixation in severe tetraparesis consecutive to posterior decompression for Chiari malformation: a case report. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06327-6
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DOI: https://doi.org/10.1007/s00381-024-06327-6