The management of Chiari 1 malformation (CM1) and Syringomyelia (Syr) has shown many changes in surgical indications and techniques over time. The dedicated neurosurgical and neurological community recently planned to analyze the state of the art and find conduct uniformity. This led to international consensus documents on diagnostic criteria and therapeutic strategies. We aimed to evaluate, in a large, monocentric surgical series of adult and children CM1 patients, if the daily clinical practice reflects the consensus documents. Our series comprises 190 pediatric and 220 adult Chiari patients submitted to surgery from 2000 to 2021. The main indications for the treatment were the presence of Syr and symptoms related to CM1. While there is great correspondence with the statements derived from the consensus documents about what to do for Syr and symptomatic CM1, the accordance is less evident in CM1 associated with craniosynostosis or hydrocephalus, especially when considering the early part of the series. However, we think that performing such studies could increase the homogeneity of surgical series, find a common way to evaluate long-term outcomes, and reinforce the comparability of different strategies adopted in different referral centers.

The diagnosis of Chiari malformation type 1 (CM1) and Syringomyelia (Syr) has become increasingly common during the past few years. Contemporarily, the body of literature on these topics is growing, although randomized controlled studies on significant case series to drive guidelines are missing in the pediatric and adult populations. As a result of the different opinions about surgical indications and techniques raised by CM1-Syr, an increasing number of well-informed but disoriented patients is emerging. To bridge this gap, an International Consensus Conference on CM1-Syr held in Milan in November 2019 aimed to find a consensus among international experts, to suggest some recommendations that, in the near future, could lead to guidelines. Here, we comment on the most relevant recommendations about the definition, diagnosis, surgical management, failures and re-intervention, and outcome. We also focus on some \"wrong\" indications or techniques that, although widely disapproved by the experts, and negatively experienced by many patients, are still largely in use.

Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions.
To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution.
A systematic review was performed using the National Library of Medicine/PubMed and Embase databases for studies on CIM in children and adults. The most appropriate surgical interventions, the use of neuromonitoring, and clinical improvement during follow-up were reviewed for studies published between 1946 and January 23, 2021.
A total of 80 studies met inclusion criteria. Posterior fossa decompression with or without duraplasty or cerebellar tonsil reduction all appeared to show some benefit for symptom relief and syrinx reduction. There was insufficient evidence to determine whether duraplasty or cerebellar tonsil reduction was needed for specific patient groups. There was no strong correlation between symptom relief and syringomyelia resolution. Many surgeons follow patients for 6-12 months before considering reoperation for persistent syringomyelia. No benefit or harm was seen with the use of neuromonitoring.
This evidence-based clinical guidelines for the treatment of CIM provide 1 Class II and 4 Class III recommendations. In patients with CIM with or without syringomyelia, treatment options include bone decompression with or without duraplasty or cerebellar tonsil reduction. Improved syrinx resolution may potentially be seen with dural patch grafting. Symptom resolution and syrinx resolution did not correlate directly. Reoperation for a persistent syrinx was potentially beneficial if the syrinx had not improved 6 to 12 months after the initial operation. The full guidelines can be seen online at https://www.cns.org/guidelines/browse-guidelines-detail/3-surgical-interventions .

BACKGROUND: Syringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing.
OBJECTIVE: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults.
METHODS: A multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement <75% were revised and entered round 2. Round 3 was face-to-face, during the Chiari Consensus Conference (Milan, November 2019).
RESULTS: Thirty-one out of 32 Jury members (6 neurologists, 4 neuroradiologists, 19 neurosurgeons, and 2 patient association representatives) participated in the consensus. After round 2, a consensus was reached on 57/63 statements (90.5%). The six difficult statements were revised and voted in round 3, and the whole set of statements was further discussed and approved.
CONCLUSIONS: The consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.

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.

暂无摘要。

Several toy breed dogs are predisposed to syringomyelia (SM), a spinal cord disorder, characterised by fluid-filled cavitation. SM is a complex trait with a moderately high heritability. Selective breeding against SM is confounded by its complex inheritance, its late onset nature and high prevalence in some breeds. This study investigated the early outcome of existing SM breeding guidelines. Six hundred and forty-three dogs, 550 Cavalier King Charles spaniels (CKCS) and 93 Griffon Bruxellois (GB), were identified as having either one (454 dogs) or both parents (189 dogs) with MRI-determined SM status. Offspring without SM were more common when the parents were both clear of SM (SM-free; CKCS 70 per cent, GB 73 per cent). Conversely, offspring with SM were more likely when both parents had SM (SM-affected; CKCS 92 per cent, GB 100 per cent). A mating of one SM-free parent with an SM-affected parent was risky for SM affectedness with 77 per cent of CKCS and 46 per cent of GB offspring being SM-affected. It is recommended that all breeding dogs from breeds susceptible to SM be MRI screened; that the SM status at five years old is established; and all results submitted to a central database that can be used by dog breeders to better enable mate selection based on estimated breeding values.