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World Neurosurgery Aug 2017Chiari malformation type 1 (CM-1) is a variation of hindbrain development that can sometimes occur in asymptomatic individuals. Conventional treatment is surgical... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Chiari malformation type 1 (CM-1) is a variation of hindbrain development that can sometimes occur in asymptomatic individuals. Conventional treatment is surgical decompression, but little is known about the natural history of patients who do not undergo surgical management. This information is critical to determine how these patients should be managed. We conducted a systematic literature review to determine the natural history of CM-1, particularly in patients who did not undergo surgery and in asymptomatic individuals, to help patients and physicians determine when surgery is likely to be beneficial.
METHODS
The literature search was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using the electronic databases PubMed, Scopus, Cochrane Library, and Web of Science. Inclusion and exclusion criteria were predefined.
RESULTS
In symptomatic patients who did not undergo surgery, headaches and nausea often improved, whereas ataxia and sensory disturbance tended not to improve spontaneously. Of patients, 27%-47% had an improvement in symptoms after 15 months, and 37%-40% with cough headache and 89% with nausea who were managed nonoperatively improved at follow-up. Most asymptomatic individuals with CM-1 remained asymptomatic (93.3%) even in the presence of syringomyelia.
CONCLUSIONS
The natural history of mild symptomatic and asymptomatic CM-1 in adults is relatively benign and nonprogressive; the decision to perform surgical decompression should be based on severity and duration of a patient's symptoms at presentation. It is reasonable to observe a patient with mild or asymptomatic symptoms even in the presence of significant tonsillar descent or syringomyelia.
Topics: Adult; Arnold-Chiari Malformation; Asymptomatic Diseases; Ataxia; Causality; Comorbidity; Disease Progression; Evidence-Based Medicine; Female; Headache; Humans; Incidence; Longitudinal Studies; Male; Nausea; Risk Factors; Sensation Disorders; Treatment Outcome
PubMed: 28435116
DOI: 10.1016/j.wneu.2017.04.082 -
EFORT Open Reviews Jan 2023The purpose of this study was to collect and evaluate clinical and radiological evidence on shoulder neuroarthropathy (NA) in syringomyelia (SM) that may support the... (Review)
Review
PURPOSE
The purpose of this study was to collect and evaluate clinical and radiological evidence on shoulder neuroarthropathy (NA) in syringomyelia (SM) that may support the management and treatment of patients with this condition.
MATERIALS AND METHODS
This systematic review is based on the analysis of reports available in PubMed, Embase, Cochrane Database of Systematic Reviews and Cochrane Central Register of Controlled Trials using the following keywords: syringomyelia, neuroarthropathy, Charcot joint and shoulder degeneration. Thirty-nine publications were found presenting case reports or case series meeting our criteria. Pooled data included a group of 65 patients and 71 shoulders with NA secondary to SM.
RESULTS
The most commonly reported symptoms were range of motion (ROM) limitation, weakness, swelling, pain and dissociated sensory loss. NA is usually monolateral and concerns only the shoulder. The average active shoulder ROM was flexion -59.2° (s.d. 37.9), internal rotation -29.8° (s.d. 22.6) and external rotation -21.1° (s.d. 23.6). Most of the patients (75%) presented with complete or nearly complete proximal humerus degeneration, while the degree of glenoid preservation varied. Fifty-two neuroarthropathic shoulders were treated conservatively with physiotherapy, anti-inflammatory medication and splinting. Eighteen patients were treated by surgical intervention.
CONCLUSION
Shoulder NA due to SM is a devastating and progressive condition, and its course is often unpredictable. Patients with unexplained shoulder degeneration should be evaluated for SM, especially if there are additional neurological symptoms. Conservative treatment usually reduces shoulder pain without improving ROM. For select patients, shoulder arthroplasty may be a better option for restoring function.
PubMed: 36705617
DOI: 10.1530/EOR-22-0008 -
Journal of Clinical Medicine Oct 2023Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial... (Review)
Review
Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.
PubMed: 37892831
DOI: 10.3390/jcm12206694 -
Acta Neurochirurgica Oct 2020Following spinal cord injury (SCI), the routine use of magnetic resonance imaging (MRI) resulted in an incremental diagnosis of posttraumatic syringomyelia (PTS)....
BACKGROUND
Following spinal cord injury (SCI), the routine use of magnetic resonance imaging (MRI) resulted in an incremental diagnosis of posttraumatic syringomyelia (PTS). However, facing four decades of preferred surgical treatment of PTS, no clear consensus on the recommended treatment exists. We review the literature on PTS regarding therapeutic strategies, outcomes, and complications.
METHODS
We performed a systematic bibliographic search on ("spinal cord injuries" [Mesh] AND "syringomyelia" [Mesh]). English language literature published between 1980 and 2020 was gathered, and case reports and articles examining syrinx due to other causes were excluded. The type of study, interval injury to symptoms, severity and level of injury, therapeutic procedure, duration of follow-up, complications, and outcome were recorded.
RESULTS
Forty-three observational studies including 1803 individuals met the eligibility criteria. The time interval from SCI to the diagnosis of PTS varied between 42 and 264 months. Eighty-nine percent of patients were treated surgically (n = 1605) with a complication rate of 26%. Symptoms improved in 43% of patients postoperatively and in 2% treated conservatively. Stable disease was documented in 50% of patients postoperatively and in 88% treated conservatively. The percentage of deterioration was similar (surgery 16%, 0.8% dead; conservative 10%). Detailed analysis of surgical outcome with regard to symptoms revealed that pain, motor, and sensory function could be improved in 43 to 55% of patients while motor function deteriorated in around 25%. The preferred methods of surgery were arachnoid lysis (48%) and syrinx drainage (31%).
CONCLUSION
Even diagnosing PTS early in its evolution with MRI, to date, no satisfactory standard treatment exists, and the present literature review shows similar outcomes, regardless of the treatment modality. Therefore, PTS remains a neurosurgical challenge. Additional research is required using appropriate study designs for improving treatment options.
Topics: Adult; Decompression, Surgical; Drainage; Female; Humans; Male; Middle Aged; Postoperative Complications; Reoperation; Sensation; Spinal Cord Injuries; Syringomyelia
PubMed: 32820376
DOI: 10.1007/s00701-020-04529-w -
PloS One 2022This patient and public-involved systematic review originally focused on arachnoiditis, a supposedly rare "iatrogenic chronic meningitis" causing permanent neurologic... (Review)
Review
BACKGROUND & IMPORTANCE
This patient and public-involved systematic review originally focused on arachnoiditis, a supposedly rare "iatrogenic chronic meningitis" causing permanent neurologic damage and intractable pain. We sought to prove disease existence, causation, symptoms, and inform future directions. After 63 terms for the same pathology were found, the study was renamed Diseases of the Leptomeninges (DLMs). We present results that nullify traditional clinical thinking about DLMs, answer study questions, and create a unified path forward.
METHODS
The prospective PRISMA protocol is published at Arcsology.org. We used four platforms, 10 sources, extraction software, and critical review with ≥2 researchers at each phase. All human sources to 12/6/2020 were eligible for qualitative synthesis utilizing R. Weekly updates since cutoff strengthen conclusions.
RESULTS
Included were 887/14286 sources containing 12721 DLMs patients. Pathology involves the subarachnoid space (SAS) and pia. DLMs occurred in all countries as a contributor to the top 10 causes of disability-adjusted life years lost, with communicable diseases (CDs) predominating. In the USA, the ratio of CDs to iatrogenic causes is 2.4:1, contradicting arachnoiditis literature. Spinal fusion surgery comprised 54.7% of the iatrogenic category, with rhBMP-2 resulting in 2.4x more DLMs than no use (p<0.0001). Spinal injections and neuraxial anesthesia procedures cause 1.1%, and 0.2% permanent DLMs, respectively. Syringomyelia, hydrocephalus, and arachnoid cysts are complications caused by blocked CSF flow. CNS neuron death occurs due to insufficient arterial supply from compromised vasculature and nerves traversing the SAS. Contrast MRI is currently the diagnostic test of choice. Lack of radiologist recognition is problematic.
DISCUSSION & CONCLUSION
DLMs are common. The LM clinically functions as an organ with critical CNS-sustaining roles involving the SAS-pia structure, enclosed cells, lymphatics, and biologic pathways. Cases involve all specialties. Causes are numerous, symptoms predictable, and outcomes dependent on time to treatment and extent of residual SAS damage. An international disease classification and possible treatment trials are proposed.
Topics: Arachnoiditis; Biological Products; Humans; Iatrogenic Disease; Meningitis; Prospective Studies
PubMed: 36178925
DOI: 10.1371/journal.pone.0274634 -
Evidence-based Spine-care Journal Oct 2013Study Design Systematic review. Study Rationale One of the most consistent indications for a Chiari decompression is tonsillar descent meeting the radiographic... (Review)
Review
Study Design Systematic review. Study Rationale One of the most consistent indications for a Chiari decompression is tonsillar descent meeting the radiographic criteria and an associated syrinx in a symptomatic patient. In counseling patients about surgery, it would be advantageous to have information regarding the expected outcome with regard to the syrinx and other possible treatments available if the result is suboptimal. Clinical Questions The clinical questions include: (1) What is the average rate of recurrent or residual syringomyelia following posterior fossa decompression as a result of Chiari malformation with associated syringomyelia? (2) What treatment methods have been reported in the literature for managing recurrent or residual syringomyelia after initial posterior fossa decompression? Materials and Methods Available search engines were utilized to identify publications dealing with recurrent or residual syrinx after Chiari decompression and/or management of the syrinx. Rates of residual or recurrent syrinx were extracted and management strategies were recorded. Overall strength of evidence was quantified. Results Of the 72 citations, 11 citations met inclusion criteria. Rates of recurrent/residual syringomyelia after decompression in adults range from 0 to 22% with an average of 6.7%. There were no studies that discussed specifically management of the remaining syrinx. Conclusion Rates of recurrent/residual syringomyelia after Chiari decompression in adults range from 0 to 22% (average 6.7%). Although no studies describing the optimal management of residual syrinx were found, there is general agreement that the aim of the initial surgery is to restore relatively unimpeded flow of cerebrospinal across the craniocervical junction. Large holocord syrinx may induce a component of spinal cord injury even with adequate decompression and reduction in the caliber of the syrinx, resulting in permanent symptoms of injury.
PubMed: 24436709
DOI: 10.1055/s-0033-1357362 -
Cureus Oct 2023We report a case of a 42-year-old female presenting with left axillary pain radiating down the arm and weakness in the ipsilateral hand. Specialist examinations of...
We report a case of a 42-year-old female presenting with left axillary pain radiating down the arm and weakness in the ipsilateral hand. Specialist examinations of neurological and musculoskeletal systems were insignificant. Magnetic resonance imaging (MRI) of the whole spine and brain revealed cerebellar tonsillar herniation of 9-10mm indicating a Chiari type 1 malformation and a large tubular T2 high-intensity lesion in the cervical cord, extending from the C2/3-disc level down to C6/C7 as well as a similar but smaller lesion behind the bodies of C7 and T1. Both lesions were consistent with syringomyelia. Surgical intervention was deemed inappropriate, and she was treated with three months of physiotherapy. Regular follow-up for two years showed gradual symptom resolution, syrinx shrinkage, and no further complications arising secondary to Chiari type 1 malformation. Chiari malformation is an anatomical anomaly of the cranio-cervical junction. It is often incidentally found on MRI, but although asymptomatic in the population, complications associated with the condition such as syringomyelia are a common initial presentation. The relationship between Chiari malformation, particularly Chiari type 1 malformation, and syringomyelia is close with the majority of patients often presenting with idiopathic syringomyelia also found to have a Chiari type 1 malformation. Considerable discussion about the pathogenic mechanisms for syringomyelia development in Chiari malformation is recognized and advancing continually.
PubMed: 38022024
DOI: 10.7759/cureus.47301 -
World Neurosurgery Aug 2021This study aimed to compare the effectiveness of posterior fossa decompression (PFD), posterior fossa decompression with duraplasty (PFDD), and posterior fossa... (Comparative Study)
Comparative Study Meta-Analysis
Comparative Assessment of Three Posterior Fossa Decompression Techniques and Evaluation of the Evidence Supporting the Efficacy of Syrinx Shunting and Filum Terminale Sectioning in Chiari Malformation Type I. A Systematic Review and Network Meta-Analysis.
BACKGROUND
This study aimed to compare the effectiveness of posterior fossa decompression (PFD), posterior fossa decompression with duraplasty (PFDD), and posterior fossa decompression with resection of tonsils (PFDRT) in Chiari malformation type I (CMI). Furthermore, we aimed to evaluate the evidence supporting the efficacy of filum terminale sectioning (FTS), syringosubarachnoid shunting (SSS), and syringoperitoneal shunting (SPS) in CMI.
METHODS
PubMed, Cochrane, and Embase databases were screened for English-language studies published from inception until August 11, 2020. A total of 3593 studies were identified through the searching process. Fifteen cohort studies were included in the systematic review and network meta-analysis.
RESULTS
No studies concerning FTS, SSS, and SPS were found eligible. Therefore, only PFD, PFDD, and PFDRT were compared. PFDD showed significantly higher incidence of complications (relative risk [RR], 3.79; 95% confidence interval [CI], 2.54-5.64) cerebrospinal fluid leak (RR, 9.74; 95% CI, 2.33-40.78) and neurologic deficit (RR, 8.76; 95% CI, 2.08-36.90) than did PFD. Both PFDD and PFDRT achieved higher syringomyelia improvement (RR, 1.23, 95% CI, 1.09-1.39 and RR, 1.32, 95% CI, 1.15-1.51, respectively) and greater clinical improvement (RR, 1.24, 95% CI, 1.10-1.39 and RR, 1.24, 95% CI, 1.08-1.44, respectively) than did PFD. No differences were found between PFDD and PFDRT.
CONCLUSIONS
PFDD and PFDRT are superior to PFD, especially in patients with syringomyelia-Chiari complex, because of greater syringomyelia reduction and better clinical improvement. However, PFDD and PFDRT can be considered equally efficient. There is no evidence pleading in favor of SFT, SSS, and SPS over any PFD technique.
Topics: Arnold-Chiari Malformation; Cauda Equina; Cerebrospinal Fluid Shunts; Decompression, Surgical; Humans; Neurosurgical Procedures
PubMed: 34098134
DOI: 10.1016/j.wneu.2021.05.124 -
Turkish Neurosurgery Jan 2022Some meta-analyses have focused on foramen magnum decompression with duraplasty (PFDD) and without duraplasty (PFD) in paediatric or mixed populations. Nevertheless, no...
AIM
Some meta-analyses have focused on foramen magnum decompression with duraplasty (PFDD) and without duraplasty (PFD) in paediatric or mixed populations. Nevertheless, no meta-analysis has evaluated adults only. This study aimed to include new relevant findings in a systematic review to provide the first comparison of PFDD and PFD in adult CM-I.
MATERIAL AND METHODS
We retrospectively searched Web of Science, PubMed, Embase and ClinicalTrials.gov to summarize all relevant published papers on adults. A systemic review was adopted to evaluate clinical or radiological improvement, surgical complications, and reoperation rates between the PFD and PFDD groups.
RESULTS
Nine papers containing information on 497 adult participants met the criteria. PFDD was related to a lower revision rate (RR=2.96, 95% CI: 1.34-6.51, P=0.007) but a higher complication rate (RR=0.35, 95% CI: 0.22-0.55, P 0.00001). No significant difference was noted between PFD and PFDD in terms of overall symptom improvement (RR=0.93, 95% CI: 0.84-1.03, P = 0.17) or syringomyelia reduction (RR=0.84, 95% CI: 0.63-1.12, P = 0.24). No significant difference in symptom improvement was observed between patients with syringomyelia (RR=0.86, 95% CI: 0.69-1.08, P = 0.20) and patients without syringomyelia (RR=0.94, 95% CI: 0.68-1.30, P = 0.73).
CONCLUSION
This systematic review of observational studies reveals that PFDD may provide lower revision rates but pose a higher risk than PFD in the management of CM-I in adults. However, PFD is similar to PFDD in clinical and radiological improvements.
PubMed: 35652180
DOI: 10.5137/1019-5149.JTN.35727-21.5 -
Journal of Rehabilitation Medicine Aug 2022This scoping review aimed to identify and synthesize existing research on active conservative management of primary spinal syringomyelia and associated symptoms and to...
OBJECTIVE
This scoping review aimed to identify and synthesize existing research on active conservative management of primary spinal syringomyelia and associated symptoms and to discuss perspectives for clinical application using an activity-based approach.
METHODS
PubMed, Embase, Scopus, and Web of Science were systematically searched for empirical studies of conservative management or therapies of adults with primary spinal syringomyelia from inception to April 2021. In addition, abstracts from relevant conferences were searched. Study characteristics and key findings were extracted, and findings descriptively synthesized.
RESULTS
Of 1,186 studies screened, 7 studies met the eligibility criteria (4 single case studies and 3 cohort studies, a total of 90 individuals). The interventions were primarily physiotherapeutic, mostly by posture correction and exercises, and effects were alleviation of pain, improved physical function, improved activities of daily living and quality of life. Analysis of factors triggering symptoms and rationale for choice of intervention based upon these was limited.
CONCLUSION
Evidence of active conservative management of primary spinal syringomyelia and associated symptoms is limited. Many variations and limitations in the existing research limit the conclusions. High-quality research is needed to enable healthcare professionals to apply evidencebased active conservative interventions.
Topics: Activities of Daily Living; Adult; Conservative Treatment; Exercise Therapy; Humans; Quality of Life; Syringomyelia
PubMed: 35924332
DOI: 10.2340/jrm.v54.2398