Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document

Ciaramitaro, P.; Massimi, Luca; Bertuccio, Alessandro; Solari, Alessandra; Farinotti, Mariangela; Peretta, Paola; Saletti, Veronica; Chiapparini, Luisa; Barbanera, Andrea; Garbossa, Diego; Bolognese, Paolo A.; Brodbelt, Andrew; Celada, Carlo; Cocito, Dario; Curone, Marcella; Devigili, Grazia; Erbetta, Alessandra; Ferraris, Marilena; Furlanetto, Marika; Gilanton, Mado; Jallo, George I.; Karadjova, Marieta; Klekamp, Jörg; Massaro, Fulvio; Morar, Sylvia; Parker, Fabrice; Perrini, Paolo; Poca, María; Sahuquillo, Juan; Stoodley, Marcus A.; Talamonti, Giuseppe; Triulzi, Fabio; Valentini, Maria Consuelo; Visocchi, Massimiliano; Valentini, Laura

doi:10.1007/s10072-021-05347-3

Cited by 56 publications

(92 citation statements)

References 68 publications

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“…Therefore, the latest edition of the international consensus document on diagnosis and treatment of CM1 in children includes progressive spinal cord cavities as an indication for surgery. 3 In addition, the analysis of prognostic risk factors in this study showed that postoperative complications were not associated with age in patients. However, another retrospective study, also from the Park-Reeves Syringomyelia Research database, found that younger patients were associated with postoperative syrinx improvement.…”

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confidence: 58%

Letter to the Editor. Recovery of physical signs in Chiari malformation type I and syringomyelia after surgery

Cai

Liu

2022

Journal of Neurosurgery: Pediatrics

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TO THE EDITOR: We read with interest the article by Akbari et al. 1 (Akbari SHA, Yahanda AT, Ackerman LL, et al. Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium. J Neurosurg Pediatr. 2022;30[1]:39-51), which reports better outcomes but more postoperative complications with posterior fossa decompression with duraplasty compared to posterior fossa decompression. As the first large-scale, multi-institutional study of pediatric patients undergoing these procedures, although the article did not have surprising results, it has important implications for the choice of surgical procedures to manage Chiari malformation type I (CM1) and syringomyelia (SM) in our clinical practice. In addition, we found some new insights worth sharing with the readers from the study's analysis of prognostic assessment.As we can see from the results of the outcome evaluation of this study, the rate resolution or improvement of symptoms (e.g., headache and back pain) after surgery was more than 80%, but the results for the associated signs were not satisfactory. Why is there a worse recovery of signs after CM1-SM surgery? A careful observation of the clinical outcomes shows that these signs are mainly associated with SM. These signs are usually due to prolonged and continuous compression of the spinal cord and brainstem by SM, and therefore they are difficult to resolve after surgery even after the compression has been removed. 2 We can also observe in previous studies that the prognosis of patients with CM1-SM is significantly worse than in patients with CM1 alone. Therefore, the latest edition of the international consensus document on diagnosis and treatment of CM1 in children includes progressive spinal cord cavities as an indication for surgery. 3 In addition, the analysis of prognostic risk factors in this study showed that postoperative complications were not associated with age in patients. However, another retrospective study, also from the Park-Reeves Syringomyelia Research database, found that younger patients were associated with postoperative syrinx improvement. 4 Therefore, we speculate whether the corresponding signs and symptoms are more likely to resolve postoperatively because of the shorter duration of spinal cord compression by the syrinx in younger patients with CM1-SM. If this is indeed the case, it will have a significant impact on the choice for the timing of surgery for patients with CM1-SM. Of course, it is necessary to validate this by further studying the natural history of CM1-SM and the relationship between the disease duration and prognosis.

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confidence: 58%

Letter to the Editor. Recovery of physical signs in Chiari malformation type I and syringomyelia after surgery

Cai

Liu

2022

Journal of Neurosurgery: Pediatrics

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“…Due to CM-I often associated with other CVJ malformations, there are inevitably some confounding factors that make the evaluation criteria of CM-I inconsistent. 25 Moreover, children and adults share different clinical courses, not only for the natural history but also the postoperative prognosis. Our study focused on the preoperative clinical and radiologic parameters in simple CM-I patients with syringomyelia to find out the relationship between the clinical status of the patients and the morphometrical characteristics of the syrinx with surgical outcome.…”

Section: Discussionmentioning

confidence: 99%

Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration

Yuan

et al. 2022

Neurospine

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Objective: The specific association between morphometric characteristics of the syrinx and the prognosis of Chiari malformation type I (CM-I) with syringomyelia following surgical procedure seems to have not been fully elaborated. This study focused on the preoperative clinical and radiologic parameters in CM-I patients with syringomyelia to find out the relationship between the patients’ clinical status and the phenotypes of the syrinx with surgical outcome.Methods: A continuous series of pediatric and adult patients with CM-I and syringomyelia from a prospectively maintained database in a single center were included, and we explored the related factors affecting the prognosis following decompression surgery through retrospective analysis of clinical presentations, imaging characteristics, and the morphological features of syringomyelia, to provide a clinical reference for the treatment of syringomyelia.Results: There were 28 pediatric patients (13.8%), and 174 adults (86.2%) included in our study. The average Chicago Chiari Outcome Scale score was 14.56 ± 1.78. The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration, ventral subarachnoid space at the foramen magnum, and with/without straightened cervical physio-curve.Conclusion: Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis.

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“…One hundred patients (75 females; average age: 51 ± 13.08 SD; range: 18–76) affected by Chiari syndrome and/or syringomyelia without dyspnea were selected for the study. The diagnosis was based on the patient’s history, clinical examination and magnetic resonance imaging, according to the standardized diagnostic criteria [ 3 , 4 ]. All the patients presented typical symptoms, i.e., “cough”, headache and neck pain, associated with signs of brainstem compression, cerebellar syndrome and myelopathy.…”

Section: Methodsmentioning

confidence: 99%

“…Since the advent of MRI, the estimated prevalence of Syr has ranged from 1.9 to 5.9 per 100.000; that of symptomatic Syr has been 3.9 per 100.000; and the incidence of Syr has ranged from 1.06 to 10 per 100.000. A diagnosis of Syr (“clinically defined”) is made in the presence of syrinx/syringobulbia at MRI in addition to spinal/bulbar signs related to the syrinx level [ 3 ]. The estimated prevalence of CM1 in adults is 8.7 per 100.00; that of Chiari syndrome is 3.5 per 100.00.…”

Section: Introductionmentioning

confidence: 99%

“…The clinical diagnostic criteria include “headache caused by CM1” and neurological symptoms and signs related to the brainstem (i.e., nystagmus, dysphagia and sleep apnea), cerebellar (ataxia) and spinal cord dysfunction (i.e., muscles hypotrophy, sensory and motor deficits and autonomic disorders), as well as otoneurological deficits (e.g., dizziness, disequilibrium, sensations of alteration in ear pressure, hypoacusia or hyperacusia, nystagmus and oscillopsia). The presence of at least two of the clinical criteria is enough to judge symptomatic Chiari [ 3 ]. Brainstem syndromes have frequently been reported in Chiari syndrome and in syringobulbia; central sleep apnea syndromes (CSASs) were evidenced in 5–8% of a cohort of 600 CM1 adults by polysomnography [ 4 ], and the same frequency was reported in a large pediatric study [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Neurophysiological Correlates in Patients with Syringomyelia and Chiari Malformation: The Cortico-Diaphragmatic Involvement

Cocito

Peci

Garbossa

et al. 2022

JCM

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Purpose. Brainstem syndromes have frequently been reported in Chiari syndrome and in syringobulbia; previous studies have shown that determining the central motor conduction time (CMCT) along the circuit of the phrenic nerve makes the assessment of the voluntary control of the respiratory pathway possible. In our study, we evaluated the transcranial magnetic stimulation (TMS) of the phrenic nerve in patients affected by Chiari syndrome and/or syringomyelia (Syr) with the aim of identifying subclinical neurophysiological alterations. Methods. One hundred patients (75 females; average age: 51 ± 13.08 SD; range: 18–76) affected by Chiari syndrome and/or Syr without dyspnea were selected. The magnetic stimulation of the second motor neuron correlating with the phrenic nerve was performed using cervical magnetic stimulation (C5-MEP); the cortical MEP after magnetic stimulation (Cz-MEP) was recorded by magnetic stimulation of the motor cortex (areas corresponding to the diaphragm). The CMCT was calculated. The differences between the patients and controls were calculated (Student’s t test). Results. In 13% of the patients, the Cz-MEP were absent bilaterally, and the CMCT was not evaluable. In all these cases, bulbar/cervical Syr was present at MRI; in 10 of them, this was associated with CM1. A bilateral response was obtained in all the other patients (87%), and the CMCTs were normal. All the patients with alterations/absence of Cz-MEP presented bulbar/cervical Syr at MRI. The C5-MEP latency was prolonged or absent in 48%; of these, 84% presented bulbar/cervical Syr associated with CM1 at MRI. The C5-MEP latency values were significantly higher in the group of patients. Conclusions. Neurophysiological alterations of the diaphragmatic pathway were recorded in a group of Chiari syndrome and, particularly, in bulbar/cervical Syr. Future studies with larger cohorts of patients are needed to further assess the specific role of the TMS of the phrenic nerve in CM/Syr patients.

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Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document

Cited by 56 publications

References 68 publications

Letter to the Editor. Recovery of physical signs in Chiari malformation type I and syringomyelia after surgery

Letter to the Editor. Recovery of physical signs in Chiari malformation type I and syringomyelia after surgery

Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration

Neurophysiological Correlates in Patients with Syringomyelia and Chiari Malformation: The Cortico-Diaphragmatic Involvement

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