Posterior fossa decompression without expansive duroplasty in an adult with Chiari malformation type 1-syringomyelia
Keywords:
Chiari malformation, syringomyelia, posterior fossa decompression, duroplasty.Abstract
Introduction: Chiari malformation type 1 includes a heterogeneous group of congenital malformations, characterized by caudal descent of the cerebellum through the foramen magnum. It has associated syringomyelia in 30-70% of cases. Controversies exist regarding the ideal surgical technique.
Objective: To present a case of MC-1 associated with syringomyelia in which an expansive duroplasty is not applied.
Case report: Female patient, 43 years old, with history of arterial hypertension. She went to the neurosurgical consultation for cervical pain radiating to the right upper limb. On neurological examination, she showed signs of first and second motor neuron involvement. MRI confirmed the diagnosis of MC-1. She underwent surgery by decompression of the posterior fossa without expansive duroplasty. The patient evolved without complications and she was discharged after 48 hours. During the follow-up, the paresthetic manifestations improved; however, the neurological examination remained the same. At six months, MRI indicated a marked decrease in syringomyelia and reshaping of the cisterna magna. Until 18 months after treatment, symptoms had not worsened and functional capacity was acceptable (Karnofsky 90/100).
Conclusions: Posterior fossa decompression without expansive duroplasty, followed by microsurgical re-permeabilization of Magendie's foramen, had favorable results in our case.
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References
Rosenblum JS, Pomeraniec IJ, Heiss JD. Chiari Malformation (Update on Diagnosis and Treatment). Neurol Clin. 2022;40(2):297-307. DOI: https://doi.org/10.1016/j.ncl.2021.11.007
Nishikawa M, Bolognese PA, Kula RW, Ikuno H, Ohata K. Pathogenesis and classification of Chiari malformation Type I based on the mechanism of ptosis of the brain stem and cerebellum: a morphometric study of the posterior cranial fossa and craniovertebral junction. J Neurol Surg Part B Skull Base. 2021;82(3):277-84. DOI: https://doi.org/10.1055/s-0039-1691832
Ciaramitaro P, Garbossa D, Peretta P, Piatelli G, Massimi L, Valentini LG, et al. Syringomyelia and Chiari Syndrome Registry: advances in epidemiology, clinical phenotypes and natural history based on a North Western Italy cohort. Ann DellIstituto Super Sanit? . 2020 [acceso 18/07/2022];56(1):48-58. DOI: https://annali.iss.it/index.php/anna/article/view/914
Lei Z, Wu S, Zhang Z, Han Y, Wang J, Li F, et al. Clinical characteristics, imaging findings and surgical outcomes of Chiari malformation Type I in pediatric and adult patients. Curr Med Sci. 2018;38(2):289-95. DOI: https://doi.org/10.1007/s11596-018-1877-2
Shane R, Beckman J, Naftel RP, Chern JJ, Wellons JC, Rozzelle CJ, et al. Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I: Clinical article. J Neurosurg Pediatr. 2011;7(3):248-56. DOI: https://doi.org/10.3171/2010.12.PEDS10379
Durham SR, Fjeld-Olenec K. Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis. J Neurosurg Pediatr. 2008;2(1):42-9. DOI: https://doi.org/10.3171/PED/2008/2/7/042
Lu VM, Phan K, Crowley SP, Daniels DJ. The addition of duraplasty to posterior fossa decompression in the surgical treatment of pediatric Chiari malformation Type I: a systematic review and meta-analysis of surgical and performance outcomes. J Neurosurg Pediatr. 2017;20(5):439-49. DOI: https://doi.org/10.3171/2017.6.PEDS16367
Varela A, Mosquera G, Medrano R, Corimayta J, Guevara RI. Descompresión de la fosa posterior en pacientes con malformación de Chiari I-siringomielia. Rev Cub Neurol Neurocir. 2014 [acceso 18/07/2022];4(1):43-8. Disponible en: http://www.revneuro.sld.cu/index.php/neu/article/view/121
O’Reilly EM, Torreggiani W. Incidence of asymptomatic Chiari malformation. Ir Med J. 2019 [acceso 18/07/2022];112(7):972. Disponible en: https://pubmed.ncbi.nlm.nih.gov/31642646/
Langridge B, Phillips E, Choi D. Chiari malformation type 1: a systematic review of natural history and conservative management. World Neurosurg. 2017;104:213-9. DOI: https://doi.org/10.1016/j.wneu.2017.04.082
Bordes S, Jenkins S, Tubbs RS. Defining, diagnosing, clarifying, and classifying the Chiari I malformations. Childs Nerv Syst. 2019;35(10):1785-92. DOI: https://doi.org/10.1007/s00381-019-04172-6
Wilkinson DA, Johnson K, Garton HJL, Muraszko KM, Maher CO. Trends in surgical treatment of Chiari malformation Type I in the United States. J Neurosurg Pediatr. 2017;19(2):208-16. DOI: https://doi.org/10.3171/2016.8.PEDS16273
Chai Z, Xue X, Fan H, Sun L, Cai H, Ma Y, et al. Efficacy of posterior fossa decompression with duraplasty for patients with Chiari malformation Type I: a systematic review and meta-analysis. World Neurosurg. 2018;113:357-365.e1. DOI: https://doi.org/10.1016/j.wneu.2018.02.092
Xu H, Chu L, He R, Ge C, Lei T. Posterior fossa decompression with and without duraplasty for the treatment of Chiari malformation Type I-a systematic review and meta-analysis. Neurosurg Rev. 2017;40(2):213-21. DOI: https://doi.org/10.1007/s10143-016-0731-x
Hankinson T, Tubbs RS, Wellons JC. Duraplasty or not? An evidence-based review of the pediatric Chiari I malformation. Childs Nerv Syst. 2011;27(1):35-40. DOI: https://doi.org/10.1007/s00381-010-1295-7
Massimi L, Frassanito P, Chieffo D, Tamburrini G, Caldarelli M. Bony decompression for Chiari malformation Type I: long-term follow-up. En: Visocchi M, editor. New Trends in Craniovertebral Junction Surgery: Experimental and Clinical Updates for a New State of Art. Cham: Springer International Publishing; 2019. p. 119-24. DOI: https://doi.org/10.1007/978-3-319-62515-7_17
Bond AE, Jane JA, Liu KC, Oldfield EH. Changes in cerebrospinal fluid flow assessed using intraoperative MRI during posterior fossa decompression for Chiari malformation. J Neurosurg. 2015;122(5):1068-75. DOI: https://doi.org/10.3171/2015.1.jns132712
Ma J, You C, Chen H, Huang S, Ieong C. Cerebellar tonsillectomy with suboccipital decompression and duraplasty by small Incision for chiari I malformation (with syringomyelia): Long term follow-up of 76 surgically treated cases. Turk Neurosurg. 2012;22(3):274-9. DOI: https://doi.org/10.5137/1019-5149.jtn.4634-11.1
Kennedy BC, Kelly KM, Phan MQ, Bruce SS, McDowell MM, Anderson RCE, et al. Outcomes after suboccipital decompression without dural opening in children with Chiari malformation Type I. J Neurosurg Pediatr. 2015;16(2):150-8. DOI: https://doi.org/10.3171%2F2014.12.PEDS14487
Chowdhury MM, Dagash H, Pierro A. A systematic review of the impact of volume of surgery and specialization on patient outcome. Br J Surg. 2007;94(2):145-61. DOI: https://doi.org/10.1002/bjs.5714
