OBJECTIVE: Imaging parameters of Chiari malformation type I (CMI) development are not well established. This study aimed to collect evidence of general or specific imaging measurements in patients with CMI, analyze indicators that may assist in determining the severity of CMI, and guide its diagnosis and treatment.
METHODS: A comprehensive search was conducted across various databases including the Cochrane Library, PubMed, MEDLINE, Scopus, and Embase, covering the period from January 2002 to October 2023, following predefined inclusion criteria. Meta-analyses were performed using RevMan (ver. 5.4). We performed a quantitative summary and systematic analysis of the included studies. This study was registered in the PROSPERO (International Prospective Register of Systematic Reviews) prior to initiation (CRD42023415454).
RESULTS: Thirty-three studies met our inclusion criteria. The findings indicated that out of the 14 parameters examined, 6 (clivus length, basal angle, Boogard\'s angle, supraocciput lengths, posterior cranial fossa [PCF] height, and volume) exhibited significant differences between the CMI group and the control group. Furthermore, apart from certain anatomical parameters that hold prognostic value for CMI, functional parameters like tonsillar movement, obex displacement, and cerebrospinal fluid dynamics serve as valuable indicators for guiding the clinical management of the disease.
CONCLUSIONS: We collated and established a set of linear, angular, and area measurements deemed essential for diagnosing CMI. However, more indicators can only be analyzed descriptively for various reasons, particularly in prognostic prediction. We posit that the systematic assessment of patients\' PCF morphology, volume, and other parameters at a 3-dimensional level holds promising clinical application prospects.

There is an absent systematic analysis or review that has been conducted to clarify the topic of nomenclature history and terms misuse about Chiari malformations (CMs). We reviewed all reports on terms coined for CMs for rational use and provided their etymology and future development. All literature on the nomenclature of CMs was retrieved and extracted into core terms. Subsequently, keyword analysis, preceding and predicting (2023-2025) compound annual growth rate (CAGR) of each core term, was calculated using a mathematical formula and autoregressive integrated moving average model in Python. Totally 64,527 CM term usage was identified. Of these, 57 original terms were collected and then extracted into 24 core-terms. Seventeen terms have their own featured author keywords, while seven terms are homologous. The preceding CAGR of 24 terms showed significant growth in use for 18 terms, while 13, three, three, and five terms may show sustained growth, remain stable, decline, and rare in usage, respectively, in the future. Previously, owing to intricate nomenclature, Chiari terms were frequently misused, and numerous seemingly novel but worthless even improper terms have emerged. For a very basic neuropathological phenomenon tonsillar herniation by multiple etiology, a mechanism-based nosology seems to be more conducive to future communication than an umbrella eponym. However, a good nomenclature also should encapsulate all characteristics of this condition, but this is lacking in current CM research, as the pathophysiological mechanisms are not elucidated for the majority of CMs.

Patients with Chiari malformation (CM) associated with atlantoaxial dislocation (AAD) and basilar invagination (BI) may present with a small posterior cranial fossa, but data on the volumetric analysis are lacking. Additionally, whether additional foramen magnum decompression (FMD) is needed together with atlantoaxial fusion remains controversial. This study evaluated the volumetric alterations of the posterior cranial fossa in these patients and analyzed the radiological and clinical outcomes after posterior C1-C2 reduction and fixation plus C1 posterior arch resection.
Thirty-two adult CM patients with AAD and BI (CM-AAD/BI group) and 21 AAD and BI patients without CM (AAD/BI-only group) who received posterior atlantoaxial fusion plus C1 posterior arch resection were retrospectively studied. The clinical and radiological outcomes and volumetric measurements of the posterior cranial fossa were evaluated.
The majority of CM-AAD/BI patients (94%) improved clinically and radiologically at 12 mo postoperatively, and none required additional FMD. Morphological analysis revealed a significant reduction in the bony posterior cranial fossa volumes of the CM-AAD/BI group (P < 0.01) and the AAD/BI-only group (P < 0.01) relative to those of the CM group. No significant differences were observed between the CM-AAD/BI and AAD/BI groups.
Compared with patients with simple CM, patients with AAD/BI with or without CM demonstrated a considerably and equally reduced bony posterior cranial fossa volume. No additional FMD is needed in the treatment of CM-AAD/BI patients after posterior reduction and fusion plus C1 posterior arch resection.

Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.

A meta-analysis was conducted to analyze the incidence of typical and atypical headaches and outcomes following various treatments in patients with Chiari I malformation.
Headache is the most common symptom of Chiari malformation, which can be divided into typical and atypical subgroups to facilitate management. Much controversy surrounds the etiology, prevalence and optimal therapeutic approach for both types of headaches.
We identified relevant studies published before 30 July 2022, with an electronic search of numerous literature databases. The results of this study were reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.
A total of 1913 Chiari malformation type I CIM patients were identified, 78% of whom presented with headache, within this group cephalgia was typical in 48% and atypical in 29% of patients, and migraine was the most common type of atypical headache. The ratio of typical/atypical headaches with international classification of headache disorders diagnosis was 1.53, and without international classification of headache disorders diagnosis was 1.56, respectively. The pooled improvement rates of typical headaches following conservative treatment, extradural decompression and intradural decompression were 69%, 88%, and 92%, respectively. The corresponding improvement rates for atypical headaches were 70%, 57.47%, and 69%, respectively. The complication rate in extradural decompression group was significantly lower than in intradural decompression group (RR, 0.31; 95% CI: 0.06-1.59, I2 = 50%, P = 0.14). Low reoperation rates were observed for refractory headaches in extradural decompression and intradural decompression groups (1%).
The International Classification of Headache Disorders can assist in screening atypical headaches. extradural decompression is preferred for typical headaches, while conservative therapy is optimal for atypical headaches. A definite correlation exists between atypical headaches and Chiari Malformation Type I patients with higher prevalence than in the general population. Importantly, decompression is effective in relieving headaches in this particular patient population.

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).
CONCLUSIONS: 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.

Treatment of Chiari malformation (CM) is controversial, especially when it coexists with \"stable\" or Type II basilar invagination (CM + II-BI). Precise evaluation of craniovertebral junction (CVJ) stability is crucial in such patients; however, this has never been validated. This study aimed to dynamically evaluate atlanto-condyle and atlantoaxial stability by kinematic computed tomography (CT) and report its surgical treatment.
The study recruited 101 patients (control, CM, and CM + II-BI groups: 48, 34, and 19 patients, respectively). During kinematic CT, the CVJ stability-related parameters were measured and compared between the 3 groups. The surgical strategy for treating CM + II-BI was based on these results. Preoperative and postoperative images were acquired, and functional scores were used to assess the outcome.
Among the 3 groups, the length of the clivus and the height of the condyle were the shortest in the CM + II-BI group, which was accompanied by the greatest rotation of the atlas and atlanto-condyle facet movement on cervical flexion and extension. Moreover, in such patients, increased Chamberlain\'s baseline violation indicated the aggregate invagination of the odontoid in the flexed position, and asymmetric displacement of atlantoaxial facets was observed. Seventeen CM + II-BI patients underwent surgical treatment with atlantoaxial distraction and occipitocervical fusion. The syringomyelia width and tonsillar herniation decreased significantly, and functional scores indicated symptom relief and good outcomes.
CVJ instability, especially the ultramovement of atlanto-condyle facets, commonly exists in II-BI as evaluated using kinematic CT. The surgical strategy of atlantoaxial distraction and occipitocervical fusion should be considered to treat such patients.

Diagnosis of Chirai malformation type I (CM-I) is based on magnetic resonance imaging of the brain or cervical spinal cord. The main goal of surgery is to relieve the blockage to the free pulsatile flow of cerebrospinal fluid beyond the foramen magnum and to stop the progression of a syringomyelia. Despite recent advances in imaging and surgery, even today, there is no consensus on optimal management of CM-I. Ongoing focus is devoted to a better consideration of the pathophysiology of CM-I and the development of more effective medical and surgical treatments. It is hoped that proposed algorithm helps the neurosurgeon to provide a precise management for patients with CM-I in advance.

OBJECTIVE: To evaluate the effectiveness of brace treatment in patients with Chiari malformation type 1 (CM-1) or syringomyelia associated scoliosis without neurosurgical intervention.
METHODS: This was a retrospective case-control study. 34 CM-1 or syringomyelia (CMS) patients who received brace treatment without neurosurgical intervention were recruited. Another 68 matched patients with idiopathic scoliosis who received bracing served as the control group. The matching criteria included gender, age (± 1 years), Risser sign (± 1 grade), initial curve magnitude (± 5°), curve patterns and follow-up time (± 6 months). Patients who encountered curve progression and scoliosis surgery were compared between different groups.
RESULTS: Until the last visit, 16 (47%) patients in CMS group and 18 (26%) patients in IS group occurred curve progression; 9 (26%) patients and 15 (22%) patients underwent scoliosis surgery, respectively. Compared to idiopathic scoliosis, patients with CMS-associated scoliosis had a significantly higher rate of curve progression (P = 0.038). However, no significant difference was observed between two groups regarding to the rate of surgery (P = 0.867). Patients with combined CM-1 and syringomyelia had a higher rate of surgery than patients with isolated CM-1 or syringomyelia (P = 0.049). The double major curve pattern was identified as the risk factor for curve progression.
CONCLUSIONS: Brace treatment is effective for CMS-associated scoliosis without neurosurgical intervention. Compared to idiopathic scoliosis, brace can provide similar prevention for scoliosis surgery in CMS patients, but slight or moderate curve progression may occur. Specifically, patients with combined CM-1 and syringomyelia should be followed closely with a higher expectation of curve progression.

Chiari type I malformation (CM I) is a congenital defect of the skull base and brain. Posterior fossa decompression alone or with duraplasty and partial resection of cerebellar tonsils is a standard approach. The effectiveness of other approaches such as the dural splitting technique is still under the study.
To study and compare the outcome following a dural splitting surgery with the duraplasty group.
184 patients that received dural splitting surgery and 39 patients that received duraplasty surgery were identified. The outcome of the individual procedure in terms of symptoms, pre- and postoperative presence of syrinx, operation time, hospital admission period, and Chicago Chiari Outcome Scale (CCOS) were analyzed and compared.
The observed values in terms of symptomatic relief between the two groups were similar Pearson\'s chi-square test was 0.677 with p=0.411. Independent samples Mann-Whitney U test to analyze CCOS score showed p = 0.249 at Z = 1.152. The average postoperative period in days was less in PFDDS group 9.25 days, the average admission period in days for the group was less in PFDDS group 14.23 days with p < 0.0005. Fisher\'s exact test showed the Syrinx disappearance rate was better in the PFDDS group. The average duration for surgery was less on the PFDDS group.
Our study showed that more optimal results were obtained in terms of syrinx resolution as well as shorter operation time and postoperative stay among the PFDDS group. Both procedures are equally effective in terms of CCOS score.