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Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty

Background: Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, indepen...

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Autores principales: Ciaramitaro, Palma, Migliaretti, Giuseppe, Ferraris, Marilena, Garnero, Andrea, Morana, Giovanni, Carucci, Paolo, Stura, Ilaria, Massaro, Fulvio, Garbossa, Diego
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10143011/
https://www.ncbi.nlm.nih.gov/pubmed/37109355
http://dx.doi.org/10.3390/jcm12083019
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author Ciaramitaro, Palma
Migliaretti, Giuseppe
Ferraris, Marilena
Garnero, Andrea
Morana, Giovanni
Carucci, Paolo
Stura, Ilaria
Massaro, Fulvio
Garbossa, Diego
author_facet Ciaramitaro, Palma
Migliaretti, Giuseppe
Ferraris, Marilena
Garnero, Andrea
Morana, Giovanni
Carucci, Paolo
Stura, Ilaria
Massaro, Fulvio
Garbossa, Diego
author_sort Ciaramitaro, Palma
collection PubMed
description Background: Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. In this study, we analyzed presurgery hydrodynamic markers to evaluate if their changes could be associated with clinical and radiological improvement after posterior fossa decompression and duraplasty (PFDD). As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes. Methods: In total, in this study, we enrolled 36 consecutive adults with Syr and CM1 who were followed by a multidisciplinary team. All the patients were prospectively evaluated with clinical scales and neuroimaging, including CSF flow, the fourth ventricle area, and the Vaquero Index by using a phase-contrast MRI before (T0) and after surgical treatment (T1-Tlast, with a range of 12–108 months). The CSF flow at the craniocervical junction (CCJ), the fourth ventricle area, and the Vaquero Index changes were statistically analyzed and compared to the clinical and quality of life improvement after surgery. The good outcome prediction ability of presurgical radiological variables was tested. Results: Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. The fourth ventricle area significantly reduced after surgery (T0-Tlast, p = 0.0093), but no significant associations with clinical improvement were found. The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50–0.87 and LH+ = 2.1, IC 95% 1.16–3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144). Conclusions: Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. Measurements of the fourth ventricle area could be useful additional information for evaluating surgical long-term follow-up; further experience on larger cohorts is required to better define the prognostic yield of this radiological parameter.
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spelling pubmed-101430112023-04-29 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty Ciaramitaro, Palma Migliaretti, Giuseppe Ferraris, Marilena Garnero, Andrea Morana, Giovanni Carucci, Paolo Stura, Ilaria Massaro, Fulvio Garbossa, Diego J Clin Med Article Background: Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. In this study, we analyzed presurgery hydrodynamic markers to evaluate if their changes could be associated with clinical and radiological improvement after posterior fossa decompression and duraplasty (PFDD). As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes. Methods: In total, in this study, we enrolled 36 consecutive adults with Syr and CM1 who were followed by a multidisciplinary team. All the patients were prospectively evaluated with clinical scales and neuroimaging, including CSF flow, the fourth ventricle area, and the Vaquero Index by using a phase-contrast MRI before (T0) and after surgical treatment (T1-Tlast, with a range of 12–108 months). The CSF flow at the craniocervical junction (CCJ), the fourth ventricle area, and the Vaquero Index changes were statistically analyzed and compared to the clinical and quality of life improvement after surgery. The good outcome prediction ability of presurgical radiological variables was tested. Results: Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. The fourth ventricle area significantly reduced after surgery (T0-Tlast, p = 0.0093), but no significant associations with clinical improvement were found. The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50–0.87 and LH+ = 2.1, IC 95% 1.16–3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144). Conclusions: Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. Measurements of the fourth ventricle area could be useful additional information for evaluating surgical long-term follow-up; further experience on larger cohorts is required to better define the prognostic yield of this radiological parameter. MDPI 2023-04-21 /pmc/articles/PMC10143011/ /pubmed/37109355 http://dx.doi.org/10.3390/jcm12083019 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Ciaramitaro, Palma
Migliaretti, Giuseppe
Ferraris, Marilena
Garnero, Andrea
Morana, Giovanni
Carucci, Paolo
Stura, Ilaria
Massaro, Fulvio
Garbossa, Diego
Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
title Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
title_full Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
title_fullStr Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
title_full_unstemmed Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
title_short Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty
title_sort syringomyelia associated with chiari 1 malformation in adults: positive outcome predictors after posterior fossa decompression with duraplasty
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10143011/
https://www.ncbi.nlm.nih.gov/pubmed/37109355
http://dx.doi.org/10.3390/jcm12083019
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