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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 -
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 -
Revista Da Associacao Medica Brasileira... Mar 2020Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe...
BACKGROUND
Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology.
METHODS
A systematic literature review in the Pubmed Database was performed using the following key-terms: "cerebellar atrophy Chiari", and "cerebellar degeneration Chiari". Articles reporting the presence of cerebellar degeneration/atrophy associated with CM were included.
RESULTS
We found only six studies directly discussing the association of cerebellar atrophy with CM, with a total of seven cases. We added one case of our own practice for additional discussion. Only speculative causes were described to justify cerebellar atrophy. The potential causes of cerebellar atrophy were diffuse cerebellar ischemia from chronic compression of small vessels (the most mentioned speculative cause), chronic raised intracranial pressure due to CSF block, chronic venous hypertension, and association with platybasia with ventral compression of the brainstem resulting in injury of the inferior olivary nuclei leading to mutual trophic effects in the cerebellum. Additionally, it is not impossible to rule out a degenerative cause for cerebellar atrophy without a causative reason.
CONCLUSIONS
Severe cerebellar atrophy is found in some patients with CM. Although chronic ischemia due to compression is the most presumed cause, other etiologies were proposed. The real reasons for cerebellar degeneration are not known. Further studies are necessary.
Topics: Arnold-Chiari Malformation; Atrophy; Cerebellar Diseases; Cerebellum; Decompression, Surgical; Female; Humans; Magnetic Resonance Imaging; Male
PubMed: 32520161
DOI: 10.1590/1806-9282.66.3.375 -
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 -
World Neurosurgery Aug 2021Diffusion tensor imaging (DTI) application in Chiari malformation type I (CMI) is still poorly defined. This study aimed to systematically review the literature and...
BACKGROUND
Diffusion tensor imaging (DTI) application in Chiari malformation type I (CMI) is still poorly defined. This study aimed to systematically review the literature and propose perspectives toward the clinical application of DTI in CMI.
METHODS
PubMed and Embase were searched for English-language articles published until October 20, 2020. Clinical studies and case series, evaluating fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), or radial diffusivity values in patients with CMI, were included.
RESULTS
Eight articles were included. Lower FA values were found at the syrinx level, which decreased with syrinx extent and intensity of symptoms, reflecting myelopathy severity. Decreased AD and MD in the middle cerebellar peduncles in symptomatic patients with CMI might explain the presence of cerebellar signs. Increased FA in various supratentorial structures positively correlated with pain severity. Worse performance in neuropsychological tests correlated with decreased FA, increased MD, and radial diffusivity, reflecting axonal degeneration. Postoperative FA decrease in the brainstem compression area reflects successful decompression. A positive correlation was found between the extent of tonsillar ectopia and increased FA, MD, and AD values, which could act as an early indicator of acute brainstem compression.
CONCLUSIONS
DTI might provide a valuable insight into the neurobiological foundation of symptomatic CMI presentation. The severity of white matter injury evident on DTI could serve as a reliable predictor of postoperative outcomes, therefore facilitating selection of appropriate surgical candidates. Postinterventional DTI reassessment might enable differentiation between unsuccessful surgical technique and irreversible myelopathy. The extent of tonsillar ectopia reflects the severity of microstructural brainstem injury.
Topics: Arnold-Chiari Malformation; Diffusion Tensor Imaging; Humans; Neuroimaging
PubMed: 34147690
DOI: 10.1016/j.wneu.2021.06.052 -
Journal of Neurosurgery Feb 2015Chiari malformation Type I (CM-I) is a common and often debilitating neurological disease. Efforts to improve treatment of CM-I are impeded by inconsistent and limited... (Review)
Review
OBJECT
Chiari malformation Type I (CM-I) is a common and often debilitating neurological disease. Efforts to improve treatment of CM-I are impeded by inconsistent and limited methods of evaluating clinical outcomes. To understand current approaches and lay a foundation for future research, the authors conducted a systematic review of the methods used in original published research articles to evaluate clinical outcomes in patients treated for CM-I.
METHODS
The authors searched PubMed, Embase, the Cumulative Index to Nursing and Allied Health Literature, ClinicalTrials.gov , and Cochrane databases to identify publications between January 2003 and August 2013 that met the following criteria: 1) reported clinical outcomes in patients treated for CM-I; 2) were original research articles; 3) included at least 10 patients or, if a comparative study, at least 5 patients per group; and 4) were restricted to patients with CM-I.
RESULTS
Among the 74 papers meeting inclusion criteria, there was wide variation in the outcome methods used. However, all approaches were broadly grouped into 3 categories: 1) "gestalt" impression of overall symptomatic improvement (n=45 papers); 2) postoperative change in specific signs or symptoms (n=20); or 3) results of various standardized assessment scales (n=22). Among standardized scales, 11 general function measures were used, compared with 6 disease-specific tools. Only 3 papers used scales validated in patients with CM-I. To facilitate a uniform comparison of these heterogeneous approaches, the authors appraised articles in multiple domains defined a priori as integral to reporting clinical outcomes in CM-I. Notably, only 7 articles incorporated patient-response instruments when reporting outcome, and only 22 articles explicitly assessed quality of life.
CONCLUSIONS
The methods used to evaluate clinical outcomes in CM-I are inconsistent and frequently not comparable, complicating efforts to analyze results across studies. Development, validation, and incorporation of a small number of disease-specific patient-based instruments will improve the quality of research and care of CM-I patients.
Topics: Age Factors; Arnold-Chiari Malformation; Clinical Trials as Topic; Humans; Outcome Assessment, Health Care; Reproducibility of Results; Research Design
PubMed: 25380104
DOI: 10.3171/2014.9.JNS14406 -
BioMed Research International 2019This study aimed to summarize the clinical features, diagnosis, and treatment of Chiari malformation type I- (CM-1-) associated syringobulbia. We performed a literature...
This study aimed to summarize the clinical features, diagnosis, and treatment of Chiari malformation type I- (CM-1-) associated syringobulbia. We performed a literature review of CM-1-associated syringobulbia in PubMed, Ovid MEDLINE, and Web of Science databases. Our concerns were the clinical features, radiologic presentations, treatment therapies, and prognoses of CM-1-associated syringobulbia. This review identified 23 articles with 53 cases. Symptoms included headache, neck pain, cranial nerve palsy, limb weakness/dysesthesia, Horner syndrome, ataxia, and respiratory disorders. The most frequently involved area was the medulla. Most of the patients also had syringomyelia. Surgical procedures performed included posterior fossa decompression, foramen magnum decompression, cervical laminectomy, duraplasty, and syringobulbic cavity shunt. Most patients experienced symptom alleviation or resolution postoperatively. A syringobulbic cavity shunt provided good results in refractory cases. Physicians should be aware of the possibility of syringobulbia in CM-1 patients, especially those with symptoms of sudden-onset brain-stem involvement. The diagnosis relies on the disorder's specific symptomatology and magnetic resonance imaging. Our review suggests that the initial therapy should be posterior fossa decomposition with or without duraplasty. In refractory cases, additional syringobulbic cavity shunt is the preferred option.
Topics: Adolescent; Adult; Arnold-Chiari Malformation; Child; Child, Preschool; Female; Headache; Humans; Infant; Magnetic Resonance Imaging; Male; Medulla Oblongata; Middle Aged; Neurosurgical Procedures; Syringomyelia; Treatment Outcome; Young Adult
PubMed: 31016190
DOI: 10.1155/2019/4829102 -
Hormone Research in Paediatrics 2023The link between the effects of recombinant human growth hormone (rhGH) therapy in patients with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is...
BACKGROUND
The link between the effects of recombinant human growth hormone (rhGH) therapy in patients with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is controversial.
SUMMARY
We report the case of a patient with an unusual association of GHD due to ectopic posterior pituitary and CM-1. Our patient developed a headache and worsening of CM-1 after the initiation of rhGH therapy. Following an atlo-occipital decompression surgery, the patient was able to resume therapy with a marked growth improvement. Based on this observation, we provide a systematic review of the current literature about these two pathologies.
KEY MESSAGES
A careful follow-up of all patients with CM-1 treated with GH is mandatory, paying particular attention to the appearance of any neurological signs and symptoms.
Topics: Humans; Arnold-Chiari Malformation; Human Growth Hormone; Dwarfism, Pituitary; Recombinant Proteins
PubMed: 36001954
DOI: 10.1159/000526617 -
Neurosurgical Review Apr 2024Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior...
BACKGROUND
Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior fossa-related conditions, such as Chiari malformation, trigeminal neuralgia, post-operative pediatric cerebellar mutism syndrome, and Crouzon syndrome. Herein, we present a summary of the current literature on segmentation of the posterior fossa. The review highlights the various segmentation techniques, and their respective strengths and weaknesses, employed along with objectives and outcomes of the various studies reported in the literature.
METHODS
A literature search was conducted in PubMed, Embase, Cochrane, and Web of Science up to November 2023 for articles on segmentation techniques of posterior fossa. The two senior authors searched through databases based on the keywords of the article separately and then enrolled joint articles that met the inclusion and exclusion criteria.
RESULTS
The initial search identified 2205 articles. After applying inclusion and exclusion criteria, 77 articles were selected for full-text review after screening of titles/abstracts. 52 articles were ultimately included in the review. Segmentation techniques included manual, semi-automated, and fully automated (atlas-based, convolutional neural networks). The most common pathology investigated was Chiari malformation.
CONCLUSIONS
Various forms of segmentation techniques have been used to assess posterior fossa volumes/pathologies and each has its advantages and disadvantages. We discuss these nuances and summarize the current state of literature in the context of posterior fossa-associated pathologies.
Topics: Humans; Arnold-Chiari Malformation; Cranial Fossa, Posterior; Magnetic Resonance Imaging
PubMed: 38637466
DOI: 10.1007/s10143-024-02366-4 -
Asian Journal of Surgery Apr 2024
Topics: Humans; Decompression, Surgical; Treatment Outcome; Arnold-Chiari Malformation; Retrospective Studies
PubMed: 38218646
DOI: 10.1016/j.asjsur.2023.12.141