Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document.

BACKGROUND: Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children. METHODS: A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three). RESULTS: Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery." CONCLUSIONS: The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.

The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature.

In anticipation of the "Chiari and Syringomyelia Consensus Conference" held in Milan in 2019, we performed a systematic literature review on the management of Chiari malformation type 1 (CM1) and syringomyelia (Syr) in children.We aimed to summarize the available evidence and identify areas where consensus has not been reached and further research is needed.In accordance with PRISMA guidelines, we formulated seven questions in Patients-Interventions-Comparators-Outcomes (PICO) format. Six PICOs concerned CM1 children with/without additional structural anomalies (Syr, craniosynostosis, hydrocephalus, tethered cord, and cranio-vertebral junction anomalies), and one PICO Syr without CM1. We searched Medline, Embase, Cochrane, and NICE databases from January 1, 1999, to May 29, 2019. Cohort studies, controlled and randomized clinical trials (CCTs, RCTs), and systematic reviews were included, all pertinent only to patients ≤ 18 years of age.For CM1, 3787 records were found, 460 full texts were assessed and 49 studies (46 cohort studies, one RCT, and two systematic reviews) were finally included. For Syr, 376 records were found, 59 full texts were assessed, and five studies (one RCT and four cohort studies) were included. Data on each PICO were synthetized narratively due to heterogeneity in the inclusion criteria, outcome measures, and length of follow-up of the included studies.Despite decades of experience on CM1 and Syr management in children, the available evidence remains limited. Specifically, there is an urgent need for collaborative initiatives focusing on the adoption of shared inclusion criteria and outcome measures, as well as rigorous prospective designs, particularly RCTs.

Chiari I malformation and pregnancy: a comprehensive review of the literature to address common questions and to guide management.

BACKGROUND: The optimal management of Chiari I malformation during pregnancy remains uncertain. Labor contractions, which increase intracranial pressure, and neuraxial anesthesia both carry the theoretical risk of brainstem herniation given the altered CSF dynamics inherent to the condition. Mode of delivery and planned anesthesia, therefore, require forethought to avoid potentially life-threatening complications. Since the assumed potential risks are significant, we seek to systematically review published literature regarding Chiari I malformation in pregnancy and, therefore, to establish a best practice recommendation based on available evidence. METHODS: The English-language literature was systematically reviewed from 1991 to 2018 according to PRISMA guidelines to assess all pregnancies reported in patients with Chiari I malformation. After analysis, a total of 34 patients and 35 deliveries were included in this investigation. Additionally, a single case from our institutional experience is presented for illustrative purposes but not included in the statistical analysis. RESULTS: No instances of brain herniation during pregnancy in patients with Chiari I malformation were reported. Cesarean deliveries (51%) and vaginal deliveries (49%) under neuraxial blockade and general anesthesia were both reported as safe and suitable modes of delivery. Across all publications, only one patient experienced a worsening of neurologic symptoms, which was only later discovered to be the result of a previously undiagnosed Chiari I malformation. Several patients underwent decompressive suboccipital craniectomy to treat the Chiari I malformation during the preconception period (31%), during pregnancy (3%), and after birth (6%). Specific data regarding maternal management were not reported for a large number (21) of these patients (60%). Aside from one abortion in our own institutional experience, there was no report of any therapeutic abortion or of adverse fetal outcome. CONCLUSIONS: Although devastating maternal complications are frequently feared, very few adverse outcomes have ever been reported in pregnant patients with a Chiari I malformation. The available evidence is, however, rather limited. Based on our survey of available data, we recommend vaginal delivery under neuraxial blockade for truly asymptomatic patients. Furthermore, based on our own experience and physiological conceptual considerations, we recommend limiting maternal Valsalva efforts either via Cesarean delivery under regional or general anesthesia or by choosing assisted vaginal delivery under neuraxial blockade. There is no compelling reason to offer suboccipital decompression for Chiari I malformation during pregnancy. For patients with significant neurologic symptoms prior to conception, decompression prior to pregnancy should be considered.

Chiari 1 malformation management: the Red Cross War Memorial Hospital approach.

PURPOSE: In this paper, we aimed to review our institutional opinions and experience with Chiari 1 malformation management to determine physician practice and outcomes. METHODS: Discussion between 3 clinicians about practice preferences and the management of Chiari 1 worldwide. Retrospective review of clinical cases over a 10-year period (2009-2018). RESULTS: Although there are some minor differences between clinicians in our practice, our approach is broadly similar. We treat incidental Chiari 1 malformations conservatively, with clinical and radiological surveillance, reserving intervention for patients who develop clinical signs or radiological deterioration. We prefer surgical intervention for patients with typical symptoms or a Chiari 1 malformation with radiological progression. If symptoms are atypical, we prefer surveillance. Our preferred operation is a conservative suboccipital craniectomy with expansion duraplasty and adhesiolysis. Our operative complication rate was low and there was no mortality or major morbidity in our series. Surveillance for incidentally discovered Chiari 1 malformations has been a safe practice in our experience. CONCLUSION: Clinical practice among three clinicians in our institution is broadly consistent. We have a conservative approach to Chiari 1 malformation management and our approach appears to have a low morbidity.

Scoliosis in patients with Chiari malformation type I.

The literature about the association between Chiari malformations (CMs) and scoliosis has been growing over the last three decades; yet, no consensus on the optimal management approach in this patient population has been reached. Spinal anomalies such as isolated syrinxes, isolated CM, and CM with a syrinx are relatively common among patients with presumed idiopathic scoliosis (IS), a rule that also applies to scoliosis among CM patients as well. In CM patients, scoliosis presents with atypical features such as early onset, left apical or kyphotic curvature, and neurological deficits. While spinal X-rays are essential to confirm the diagnosis of scoliosis among CM patients, a magnetic resonance imaging (MRI) is also recommended in IS patients with atypical presentations. Hypotheses attempting to explain the occurrence of scoliosis in CM patients include cerebellar tonsillar compression of the cervicomedullary junction and uneven expansion of a syrinx in the horizontal plane of the spinal cord. Early detection of scoliosis on routine spinal examination and close follow-up on curve stability and progression are essential initial steps in the management of scoliosis, especially in patients with CM, who may require full spine MRI to screen for associated neuro-axial anomalies; bracing and spinal fusion may be subsequently pursued in high-risk patients.

International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions.

INTRODUCTION: In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M. MATERIALS AND METHODS: Pediatric neurosurgeons worldwide were surveyed, using an e-mail list provided by the ISPN communication committee chairperson. Respondents were given scenarios similar to the 2003 C1M survey in order to determine opinions regarding whether to surgically intervene, and if so, with which operations. RESULTS: Of 300 surveys electronically distributed, 122 responses were received (40.6% response rate)-an improvement over the 30.8% response rate in 2003. Pediatric neurosurgeons from 34 different countries responded. There was broad consensus that non-operative management is appropriate in asymptomatic C1M (> 90%) as well as asymptomatic C1M with a small syrinx (> 65%). With a large syrinx, a majority (almost 80%) recommended surgical intervention. Scoliotic patients with CIM were generally offered surgery only when there was a large syrinx. There has been a shift in the surgical management over the past decade, with a bone-only decompression now being offered more commonly. There remains, however, great variability in the operation offered. CONCLUSION: This survey, with a relatively strong response rate, and with broad geographic representation, summarizes current worldwide expert opinion regarding management of pediatric C1M. Asymptomatic C1M and C1M with a small syrinx are generally managed non-operatively. When an operation is indicated, there has been a shift towards less invasive surgical approaches.

Chiari malformation type 1-related headache: the importance of a multidisciplinary study.

Chiari type 1 Malformation (CM 1) is a structural defect consisting of a displacement of the cerebellar tonsils through the foramen magnum causing obstruction of cerebrospinal fluid (CSF) outflow. CM 1 has a variety of presentation with headache being the most common symptom. The evaluation and treatment of headache related to CM 1 are often difficult, because the pain in the occipital-suboccipital region or headache that is of cough-type suggests symptomatic CM 1, but patients suffering from CM 1 can also report migraine or tension-type headache. In 2015 we started a collaborative project in which our group of neurologists, neurosurgeons and neuroradiologists contribute to create a Chiari Special Outpatient Service; this was set up to provide a multidisciplinary evaluation, treatment and follow-up of patient suffering from CM 1. 201 patients (58 males, 143 females) suffering from CM 1 were multidisciplinary evaluated. Headache characteristics, clinical features, and treatment of patients are discussed. Further progress in multidisciplinary care of headache and CM 1 should be performed to define guidelines.

Asymptomatic Chiari Type I malformation: should patients be advised against participation in contact sports?

BACKGROUND: Chiari type I malformation (CM-I) is characterised by caudal displacement of the cerebellar tonsils through the foramen magnum, crowding the craniocervical junction. It is being increasingly diagnosed in asymptomatic patients due to the widespread availability of MRI, and there are case reports of these patients suffering sudden death or neurological injury following head or neck trauma, raising the issue of whether they should be prohibited from contact sport participation, given the likelihood of frequent trauma. General neurosurgical opinion is that patients who are symptomatic and those with an associated spinal cord syrinx should be offered foramen magnum decompression, however asymptomatic patients without syringomyelia are not offered this in the majority of cases. METHODS: The authors performed a full review of the published literature, including all case reports, case series, studies and literature reviews regarding CM-I and either contact sports or trauma, excluding patients that had undergone surgical intervention and those becoming symptomatic in circumstances other than head or neck trauma. RESULTS: 21 case reports of CM-I patients deteriorating following trauma were identified, including four cases of sudden death following head or neck injury. However, studies of large samples of CM-I patients are yet to capture an incident of sudden death or acute neurological deterioration, suggesting that the risk is very low. CM-I patients may have an increased risk of concussion and post-concussion syndrome compared to the background population however. CONCLUSION: Overall, the authors feel that there should be no restriction of sports participation for CM-I patients, but a discussion to make them and their families aware of the possible increased risks is important.

Chiari Type 1 Deformity in Children: Pathogenetic, Clinical, Neuroimaging, and Management Aspects.

Our understanding of cerebellar tonsillar herniation evolved over time and nowadays various pathomechanisms have been proposed. Causes of tonsillar herniation share a discrepancy between content (fore- and hindbrain) and container (supratentorial cranial vault, posterior fossa), may be associated with abnormalities of the craniocervical junction, and may have a developmental or acquired nature. In tonsillar herniation, the hindbrain is not malformed but deformed. Accordingly, "Chiari type 1 deformity," not "Chiari type 1 malformation" is the correct term to characterize primary tonsillar herniation. Chiari type 1 deformity is commonly seen in pediatric neurology, neuroradiology, and neurosurgery and may have various clinical presentations depending on patient age. In addition, Chiari type 1 deformity is increasingly found by neuroimaging studies as an incidental finding in asymptomatic children. An accurate and reliable selection of patients based on clinical and neuroimaging findings is paramount for the success of neurosurgical treatment. Future studies are needed to provide selection criteria with a higher sensitivity and specificity.

Chiari III malformation: a comprehensive review of this enigmatic anomaly.

INTRODUCTION: Chiari III is one of the rarest of the Chiari malformations and is characterized by a high cervical or low occipital encephalocele and osseous defect with or without spinal cord involvement and may include many of the anatomical characteristics seen in the Chiari II malformation. Herein, we provide a comprehensive review of this rare anomaly as well as a translation of Chiari's original description. METHODS: We review all reported cases of Chiari III malformation found in the extant literature. CONCLUSIONS: Out of 57 reported cases of Chiari III malformation, encephaloceles were in a high cervical/low occipital position in 23, 8 were in a high cervical position, 17 were in low occipital position, and the position in 9 cases was not reported. The pathogenesis of Chiari III malformation remains unclear. The majority of patients have concomitant hydrocephalus. Brain parts occurring in the sac from the most to least common include the following: cerebellum, occipital lobe, and parietal lobe. The severity of symptoms is correlated with the amount of brain structures within the encephalocele. Neurologic functional outcomes have been varied and depend on the neurological status of the patient before surgery.

Brace treatment versus observation alone for scoliosis associated with Chiari I malformation following posterior fossa decompression: a cohort study of 54 patients.

PURPOSE: To be deemed effective in preventing curve progression, brace treatment should show alteration of the expected natural history. Most of the reported studies on the effect of bracing on the evolution of Chiari malformation-associated scoliosis (CMS) following posterior fossa decompression (PFD) were small series with inconclusive results. The goal of this study was to investigate whether post-PFD brace treatment for CMS produces better outcomes than observation alone. METHODS: The clinical and radiographic data of 21 patients treated with observation alone (Ob group) and 33 patients receiving brace treatment (Br group) were retrospectively reviewed. Evolution of scoliosis was defined as progression of Cobb angle of the primary curve of >5°, as compared with that at brace initiation. RESULTS: At the latest follow-up, curve progression occurred in 10 (30 %) of Br group and 13 (62 %) of Ob group (P = .022). Overall, 8 (24 %) patients in the Br group and 9 (43 %) in the Ob group underwent further scoliosis surgery (P = .151). When analyses were restricted to the 27 braced and 17 observed patients who had reached skeletal maturity or undergone spinal fusion surgery, decreased curve progression and surgical rates were also observed in the Br group though not reaching statistical significance. Results of the survival analysis, however, demonstrated a significantly increased survival proportion in the Br group (0.63 versus 0.35, P = .014). CONCLUSIONS: Compared with observation alone, bracing treatment following PFD in patients with CMS was observed to reduce the rates of curve progression and scoliosis surgery. However, patients and their parents should be informed that almost a third of patients still go on to surgery.

The Chiari Malformations: A Bibliometric Analysis of the 100 Most Cited Articles.

OBJECTIVE: The Chiari malformations present heterogeneous entities, raising many questions regarding their natural history, pathophysiology, treatment options, and prognosis. Bibliometric analyses have emerged as a method to assess the impact of articles in current clinical practice. METHODS: The most cited articles were identified via Scopus Library by using the keywords "Chiari," "Arnold-Chiari," and "Chiari malformation." The 100 most cited articles were then assembled and analyzed in detail. RESULTS: The top 100 articles yielded a mean of 155,28 citations per article, ranging from 87 to 896 citations, and from 1.63 to 38.96 per year. Years of publications ranged from 1950 to 2015. Oakes was the most cited author (n = 7), followed by Tubbs and Milhorat. The country with the highest cited articles was the United States (n = 63), followed by the United Kingdom (n = 6), Italy (n = 5), Spain (n = 5), Japan (n = 4), and Germany (n = 3). Neurosurgery is the journal with the most highly cited articles (n = 21), followed by Journal of Neurosurgery (n = 19). Most articles focused on Chiari malformation type I (n = 83). The topic discussed most often was imaging (n = 63), followed by the evaluation of treatment outcome (n = 58), clinical signs and symptoms (n = 57), and the role of surgery (n = 56). CONCLUSIONS: The present bibliometric analysis provides a succinct appraisal of the most cited articles concerning Chiari malformation, allowing a deeper insight in this area and its main influential articles with their impact on current clinical practice and future research.

A Comprehensive Approach to the Diagnosis and Management of Klippel Feil Syndrome.

Klippel-Feil Syndrome (KFS) is a rare genetic disorder characterized by the abnormal development of the cervical spine, leading to the fusion of two or more cervical vertebrae. The syndrome presents diverse symptoms, including limited neck movement, chronic pain, and neurological manifestations such as limb numbness or weakness. The severity of KFS can vary significantly, and treatment primarily focuses on symptom management and preventing complications such as scoliosis or spinal cord compression. Surgical interventions are often necessary for patients with complex forms of the syndrome. Interestingly, Chiari 1 malformation, a cranial anomaly affecting the brainstem, can coincide anatomically with KFS. In this case report, we present the case of a 9-year-old patient who sought medical attention due to persistent, unchanging neck pain. The patient's medical history was notable for developmental delays and cervical restraint observed during physical examination. Magnetic resonance imaging (MRI) findings revealed hydrocephalus and brainstem descent, indicating the presence of Chiari 1 malformation. Comprehensive MRI and CT scans were performed, and a management plan was formulated, primarily involving cranial surgery and physiotherapy. Implementation of the treatment approach resulted in significant improvement in the patient's symptoms. This case highlights the significance of considering Chiari 1 malformation as a potential comorbidity in patients diagnosed with KFS who present with persistent neck pain. Early detection and appropriate management of both conditions are crucial for achieving favorable outcomes and enhancing the quality of life for affected individuals. Understanding the complex interplay between KFS and Chiari 1 malformation is essential for providing comprehensive care and tailored treatment strategies. Further research is warranted to elucidate the underlying mechanisms linking these two conditions and to explore optimal management approaches for patients with dual pathology. By reporting this case, we contribute to the existing literature and increase awareness among healthcare professionals regarding the potential coexistence of KFS and Chiari 1 malformation. Continued efforts in identifying associated anomalies and optimizing therapeutic interventions will aid in improving patient outcomes and ensuring optimal care for individuals affected by these conditions.

Conservative Treatment of Chiari Malformation Type I Based on the Phase-Contrast Magnetic Resonance Imaging: A Retrospective Study.

BACKGROUND: The use of phase-contrast magnetic resonance imaging is interestingly increased in the diagnosis and follow up of patients with Chiari type I malformation (CM1). The current study aimed to elaborate the benefits of conservative treatment by evaluating consecutively treated adult patients with CM1 who were selected on basis of phase-contrast magnetic resonance imaging. METHODS: Medical records of patients diagnosed with CM1 were retrospectively reviewed at 2 neurosurgical centers spanning 8 years (2010-2017). Adult patients with CM1, who were treated conservatively and met study criteria, were selected to be the core sample for this study. Between-group (benefited vs. nonbenefited) comparisons were performed to understand the factors that may affect the outcomes. RESULTS: Ninety adult patients (68 female and 22 male) received conservative treatment for CM1. The mean age was 40.6 years. Headaches and pinprick loss were the most commonly recorded symptoms and clinical findings, which were recorded in 58 (64.4%) and 31 (34.4%) patients, respectively. Eleven patients were presented with a syrinx. The mean aqueductal stroke volume (ASV) was 16.5 µL. Conservative treatment was ineffective in treating 5 (5.6%) patients, who underwent surgical intervention. The means of ASV in the benefited and nonbenefited groups were 16.7 and 13.2 µL, respectively (P = 0.004). CONCLUSIONS: Conservative approaches (prescriptive medications, physical therapy, Pilates, and swimming) can improve the life quality of nonsurgical candidate adult patients with CM1. Conservative treatment can be useful in selected patients with variably CM1 (ASV = 16.7 µL). Heavy sleep apnea or/and functional symptoms were prognostic factors that affected the conservative treatment negatively.

Diagnosis, Management, and Return to Sport of a 16-Year-Old Patient With a Chiari I Malformation: A Case Report and Literature Review.

This case report discusses a 16-year-old female volleyball, basketball, and track and field athlete who was diagnosed with a Chiari I malformation after a concussion. Surgical decompression was recommended and performed 3 months after her initial diagnosis. This patient presented unique challenges due to her age, desire to return to sport, and lack of access to medical care due to living in a rural area. Few evidence-based best-practice recommendations are available for the management and return to sport of patients with Chiari I malformation, particularly for those who have undergone surgical decompression. In this case study, we address the treatment and return-to-sport process for the patient and provide a comprehensive review of the published literature on patients attempting to return to sport after a diagnosis of Chiari I malformation. In addition, we explore the value of an athletic trainer in reconciling various barriers in management and return to sport evident in this case and the reviewed literature.

The factors affecting the outcomes of conservative and surgical treatment of chiari i adult patients: a comparative retrospective study.

BACKGROUND: The prognosis of the Chiari malformation type 1 (CM1) demonstrates a variant spectrum that varies from full recovery to complicated worse neurological disability. OBJECTIVE: To investigate the factors affecting the outcomes of conservative and surgical treatment for CM1 by evaluating adult patients consecutively managed at our institutions. MATERIALS AND METHODS: We retrospectively reviewed the medical records of patients diagnosed with CM1 at two reference neurosurgical centers for eight years (2010-2017). We selected all CM1 adult patients who managed conservatively or surgically as the core sample for this study. For clinical evaluation, we used a Chicago Chiari Outcome Scale (CCOS). For radiological assessment, we adopted both craniocervical and contrast-phase MRIs. We investigate factors such as age, sex, pretreatment symptoms, symptoms duration, and radiological findings in both groups. RESULTS: Ninety patients were treated conservatively. After a progression, five of them were treated surgically later and included in a total of 72 patients who underwent decompressive surgery. We successfully managed 85 patients (94.4%) of the conservative group and 64 patients (88.9%) of the surgical group. We found that patients with aqueductal stroke volume (ASV) of 12 µl are surgical candidates. We observed a strong positive correlation between clinical improvement and the increase in ASV values. CONCLUSIONS: ASV≤12 µl is a significant predictor for surgical intervention. The presence of heavy sleep apnea or/and functional symptoms, tonsillar herniation >13.4 mm on coronal images, low ASV, long symptom durations, and a syrinx are the independent prognostic factors that affected outcomes negatively.

Neck pain and Headache Complicated by Persistent Syringomyelia After Foramen Magnum Decompression for Chiari I Malformation: Improvement with Multimodal Chiropractic Therapies.

BACKGROUND Patients with Arnold-Chiari Malformation I (CM-I) treated with foramen magnum decompression (FMD) can have ongoing neck pain, headaches, and other symptoms complicated by persistent syringomyelia, yet there is little research regarding treatment of these symptoms. CASE REPORT A 62-year-old woman with a history of residual syringomyelia following FMD and ventriculoperitoneal shunt for CM-I presented to a chiropractor with progressively worsening neck pain, occipital headache, upper extremity numbness and weakness, and gait abnormality, with a World Health Organization Quality of Life score (WHO-QOL) of 52%. Symptoms were improved by FMD 16 years prior, then progressively worsened, and had resisted other forms of treatment, including exercises, acupuncture, and medications. Examination by the chiropractor revealed upper extremity neurologic deficits, including grip strength. The chiropractor ordered whole spine magnetic resonance imaging, which demonstrated a persistent cervico-thoracic syrinx and findings of cervical spondylosis, and treated the patient using a multimodal approach, with gentle cervical spine mobilization, soft tissue manipulation, and core and finger muscle rehabilitative exercises. The patient responded positively, and at the 6-month follow-up her WHO-QOL score was 80%, her grip strength and forward head position had improved, and she was now able to eat using chopsticks. CONCLUSIONS This case highlights a patient with neck pain, headaches, and persistent syringomyelia after FMD for CM-I who improved following multimodal chiropractic and rehabilitative therapies. Given the limited, low-level evidence for these interventions in patients with persistent symptoms and syringomyelia after FMD, these therapies cannot be broadly recommended, yet could be considered on a case-by-case basis.

Chiari and Syringomyelia Consortium: a model of multidisciplinary and sharing path for rare diseases.

Piemonte and Valle d'Aosta Interregional Network for Rare Diseases (RD) is a model of "diffuse" network; it involves all the health system specialists devoted to the diagnosis, the therapy and the follow-up of a RD. The Consortium is a multidisciplinary team operating throughout the Region composed of volunteer Physicians and Biologists that promotes periodical meetings to develop shared protocols. In 2008 the Specialist-Technical Committee for the Interregional RD Network approved the set up of the "Chiari and Syringomyelia Consortium" (CSC) with two specific objectives: to identify the shared diagnostic criteria and to develop the interdisciplinary diagnostic-therapeutic-assistance path (DTAP) to be used interregionally. Other objectives are: to define the prevalence of the pathologies (both symptomatic and asymptomatic, both in adults and children) and to improve collaboration between the patient Associations and the Physicians.