ArticlePediatric neurology2020
Prevalence and Impact of Underlying Diagnosis and Comorbidities on Chiari 1 Malformation.
Article in Pediatric neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 66 citations in OpenAlex.
- Chiari I malformation.Nature reviews. Disease primers · 2026Review
- Chiari malformation type 1 is associated with a smaller fourth ventricle volume - a multi-cohort replication study.Scientific reports · 2026Article
- Central hypoventilation and choking episodes revealing Chiari malformation type I.BMJ case reports · 2026Article
- Bilateral Papilledema without Evident Intracranial Hypertension in Pediatric Chiari Malformation Type I: A Case Report.NMC case report journal · 2026Article
- Diagnostic utility of additional spine imaging for syrinx detection in the pre-operative assessment of pediatric Chiari malformation type 1.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Posterior fossa decompression in syndromic children with chiari-like posterior fossa crowding: a nationwide US-based study.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Article
- Clinical phenotypes among patients with familial forms of Chiari malformation type 1.Journal of neurosurgery. Pediatrics · 2025Article
- Hyponatremia Presents With Seizure in a Patient With Arnold-Chiari Malformation: A Case Report.Cureus · 2025Article
- A review of cerebrospinal fluid circulation with respect to Chiari-like malformation and syringomyelia in brachycephalic dogs.Fluids and barriers of the CNS · 2025Review
- A test of the ArchaicEvolution, medicine, and public health · 2025Article
- Neuraxial anaesthesia for the parturient with intracranial pathology.BJA education · 2025Review
- Spinal cord-spinal canal disproportion following treatment for syringomyelia associated with Chiari malformation type I: illustrative case.Journal of neurosurgery. Case lessons · 2024Article
- Long-term outcomes following posterior fossa decompression in pediatric patients with Chiari malformation type 1, a population-based cohort study.Acta neurochirurgica · 2024Article
- The Genetics of Chiari 1 Malformation.Journal of clinical medicine · 2024Review
- Prenatal diagnosis of non-typical Chiari malformation type I associated with de novo Nuclear Factor I A gene mutation: a case report.Journal of medical case reports · 2024Article
- Complication Avoidance in Chiari Malformation Surgery.Advances and technical standards in neurosurgery · 2024Review
- The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories.Cureus · 2023Article
- Article
- Hiccups due to Chiari type I malformation.CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienne · 2023Article
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
Abstract
backgroundChiari malformation type 1 affects approximately one in 1,000 people symptomatically, although one in 100 meet radiological criteria, making it a common neurological disorder. The diagnosis of underlying conditions has become more sophisticated, and new radiological markers of disease have been described. We sought to determine the prevalence and impact of additional comorbidities and underlying diagnoses in patients with Chiari malformation type 1 on symptomatology and surgical treatment.
methodsA retrospective review of 612 pediatric patients with a Chiari malformation type 1 diagnosis and imaging data evaluated between 2008 and 2018 was performed. Because of extensive clinical heterogeneity, patients were separated into four categories based on their primary comorbidities (nonsyndromic, central nervous system, skeletal, and multiple congenital anomalies) to identify associations with age of onset, radiographic measurements, syringomyelia, and surgical treatment.
resultsThe largest group had nonsyndromic Chiari malformation type 1 (70%) and the latest age at diagnosis of any group. In the syndromic group, 6% were diagnosed with a known genetic abnormality, with overgrowth syndromes being the most common. Patients with multiple congenital anomalies had the earliest Chiari malformation type 1 onset, the most severe tonsillar ectopia and obex position, and were overrepresented among surgical cases. Although there were no statistically significant differences between groups and rates of syrinx, we observed differences in individual diagnoses.
conclusionThe underlying diagnoses and presence of comorbidities in patients with Chiari malformation type 1 impacts rates of syringomyelia and surgery. Although most Chiari malformation type 1 cases are nonsyndromic, clinical evaluation of growth parameters, scoliosis, and joint hypermobility should be routine for all patients as they are useful to determine syringomyelia risk and may impact treatment.
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