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      Relationship Between Syrinx Resolution and Cervical Sagittal Realignment Following Decompression Surgery for Chiari I Malformation Related Syringomyelia Based on Configuration Phenotypes

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          Abstract

          Objective

          Combined with different configuration types of syringomyelia, to analyze the correlation between syrinx resolution and changes in cervical sagittal alignment following Foramen magnum and Magendie dredging (FMMD) for syringomyelia associated with Chiari I malformation (CM-I), and to further explore the respective relationship with clinical outcome.

          Methods

          A consecutive series of 127 patients with CM-I and syringomyelia who underwent FMMD in our center met the inclusion criteria of this study. Their clinical records and radiologic data were retrospectively reviewed. The Japanese Orthopedic Association (JOA) scoring system and the Chicago Chiari Outcome Scale (CCOS) were used to evaluate the surgical efficacy. The phenotypes of syringomyelia and the clinical characteristics of the patients were analyzed according to grouping by cervical curvature at baseline.

          Results

          The preoperative straight or kyphotic cervical alignment is more common in the moniliform syrinx. After surgery, the syrinx resolution and cervical sagittal realignment in the moniliform group are more obvious, and the corresponding prognosis is relatively better. Spearman correlation analysis showed that the ΔS/C ratio (the change ratio of syrinx/cord) was positively correlated with the CCOS (p = 0.001, r = 0.897) and ΔC2–7A (the change of lower cervical angle) (p = 0.002, r = 0.560). There was also a correlation between the ΔJOA score (the change rate of the JOA score) and ΔC2–7A (p = 0.012, r = 0.467).

          Conclusion

          After decompression surgery, syrinx resolution may coexist with the changes in the subaxial lordosis angle, especially for syrinx in moniliform type, and the relationship between syrinx resolution and cervical sagittal realignment might be valuable for evaluating the surgical outcome.

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          Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients.

          C Wolpert, R Kula, Juan Trinidad … (1999)
          Chiari malformations are regarded as a pathological continuum of hindbrain maldevelopments characterized by downward herniation of the cerebellar tonsils. The Chiari I malformation (CMI) is defined as tonsillar herniation of at least 3 to 5 mm below the foramen magnum. Increased detection of CMI has emphasized the need for more information regarding the clinical features of the disorder. We examined a prospective cohort of 364 symptomatic patients. All patients underwent magnetic resonance imaging of the head and spine, and some were evaluated using CINE-magnetic resonance imaging and other neurodiagnostic tests. For 50 patients and 50 age- and gender-matched control subjects, the volume of the posterior cranial fossa was calculated by the Cavalieri method. The families of 21 patients participated in a study of familial aggregation. There were 275 female and 89 male patients. The age of onset was 24.9+/-15.8 years (mean +/- standard deviation), and 89 patients (24%) cited trauma as the precipitating event. Common associated problems included syringomyelia (65%), scoliosis (42%), and basilar invagination (12%). Forty-three patients (12%) reported positive family histories of CMI or syringomyelia. Pedigrees for 21 families showed patterns consistent with autosomal dominant or recessive inheritance. The clinical syndrome of CMI was found to consist of the following: 1) headaches, 2) pseudotumor-like episodes, 3) a Meniere's disease-like syndrome, 4) lower cranial nerve signs, and 5) spinal cord disturbances in the absence of syringomyelia. The most consistent magnetic resonance imaging findings were obliteration of the retrocerebellar cerebrospinal fluid spaces (364 patients), tonsillar herniation of at least 5 mm (332 patients), and varying degrees of cranial base dysplasia. Volumetric calculations for the posterior cranial fossa revealed a significant reduction of total volume (mean, 13.4 ml) and a 40% reduction of cerebrospinal fluid volume (mean, 10.8 ml), with normal brain volume. These data support accumulating evidence that CMI is a disorder of the para-axial mesoderm that is characterized by underdevelopment of the posterior cranial fossa and overcrowding of the normally developed hindbrain. Tonsillar herniation of less than 5 mm does not exclude the diagnosis. Clinical manifestations of CMI seem to be related to cerebrospinal fluid disturbances (which are responsible for headaches, pseudotumor-like episodes, endolymphatic hydrops, syringomyelia, and hydrocephalus) and direct compression of nervous tissue. The demonstration of familial aggregation suggests a genetic component of transmission.
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            Asymptomatic Chiari Type I malformations identified on magnetic resonance imaging.

            M. Guarnieri, Matthew B Carson, Jeffery J Meadows … (2000)
            Chiari Type I malformation (CMI) is a congenital disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Frequently, associated findings include abnormalities of nearby bony and neural elements as well as syringomyelia. Cerebellar tonsillar ectopia is generally considered pathological when greater than 5 mm below the foramen magnum. However, asymptomatic tonsillar ectopia is an increasingly recognized phenomenon, the significance of which is poorly understood. The authors retrospectively reviewed the records of all brain magnetic resonance (MR) images obtained at our hospital over a 43-month period in an attempt to ascertain the relative prevalence and MR imaging characteristics of asymptomatic CMIs. Of 22,591 patients who underwent MR imaging of the head and cervical spine, 175 were found to have CMIs with tonsillar herniation extending more than 5 mm below the foramen magnum. Of these, 25 (14%) were found to be clinically asymptomatic. The average extent of ectopia in this population was 11.4 +/- 4.86 mm, and was significantly associated with a smaller cisterna magna. Syringomyelia and osseous anomalies were found in only one asymptomatic patient. The authors suggest that the isolated finding of tonsillar herniation is of limited prognostic utility and must be considered in the context of all available clinical and radiographic data. Strategies for treating patients with asymptomatic CMIs are discussed.
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              Surgical treatment of Chiari I malformation--analysis of intraoperative findings, complications, and outcome for 371 foramen magnum decompressions.

              Jörg Klekamp (2012)
              Foramen magnum decompression is widely accepted as the treatment of choice for Chiari I malformation. However, important surgical details of the procedure are controversial.
              Article 0 comments Cited 32 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Neurospine
                Journal ID (iso-abbrev): Neurospine
                Journal ID (publisher-id): NS
                Title: Neurospine
                Publisher: Korean Spinal Neurosurgery Society
                ISSN (Print): 2586-6583
                ISSN (Electronic): 2586-6591
                Publication date (Print): December 2022
                Publication date (Electronic): 31 December 2022
                Volume: 19
                Issue: 4
                Pages: 1057-1070
                Affiliations
                [1 ]Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China
                [2 ]Neurospine Center, China International Neuroscience Institute (CHINA-INI), Beijing, China
                [3 ]Research Center of Spine and Spinal Cord, Beijing Institute of Brain Disorders, Capital Medical University, Beijing, China
                [4 ]National Center for Neurological Disorders, Beijing, China
                Author notes
                Corresponding Author Fengzeng Jian China International Neuroscience Institute (CHINA-INI), Xuanwu Hospital, Capital Medical University, No. 45 Changchun Street, Xicheng District, Beijing 100053, China Email: jianfengzeng@ 123456xwh.ccmu.edu.cn
                Author information
                http://orcid.org/0000-0001-7860-278X
                Article
                Publisher ID: ns-2244530-265
                DOI: 10.14245/ns.2244530.265
                PMC ID: 9816586
                PubMed ID: 36597642
                SO-VID: 65df3525-cee9-4dc6-b82c-81471e06054e
                Copyright © Copyright © 2022 by the Korean Spinal Neurosurgery Society
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 26 June 2022
                Date revision received : 23 August 2022
                Date accepted : 26 August 2022
                Categories
                Subject: Original Article

                Keywords: cervical sagittal alignment,syringomyelia,chiari malformation,decompression surgery,prognosis
                Data availability:
                Keywords: cervical sagittal alignment, syringomyelia, chiari malformation, decompression surgery, prognosis

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