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Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review

Raquel Rodríguez-Blanque1,2,3, Cristina Almazán-Soto4, Beatriz Piqueras-Sola1,5

  • 1Research Group CTS1068, Andalusia Research Plan, Junta de Andalucía, 18071 Granada, Spain.

Journal of Clinical Medicine
|October 28, 2023
PubMed

Insights

Arnold Chiari syndrome, a congenital posterior cranial malformation, presents diverse symptoms and diagnostic challenges. Research suggests a potential genetic origin and posterior cerebral fossa decompression as the primary treatment.

Area of Science:

  • Neurology
  • Genetics
  • Developmental Biology

Background:

  • Arnold Chiari syndrome is a rare congenital posterior cranial malformation.
  • Its etiology remains under investigation, with a wide spectrum of symptoms from severe to asymptomatic.
  • Diagnosis is often delayed until adolescence due to varied clinical presentations.

Purpose of the Study:

  • To provide an updated comprehensive review of Arnold Chiari syndrome.
  • To analyze scientific literature published within the last 5 years.
  • To adhere to the PRISMA model for systematic reviews.

Main Methods:

  • Exhaustive literature search of scientific publications from the past 5 years.
  • Systematic review conducted following PRISMA guidelines.
  • Study registered in PROSPERO (CRD42023394490).

Main Results:

  • The review synthesizes current knowledge on Arnold Chiari syndrome.
  • Identified challenges in early diagnosis and treatment efficacy.
  • Highlighted the potential genetic underpinnings of the condition.

Conclusions:

  • Arnold Chiari syndrome may have a genetic basis.
  • Posterior cerebral fossa decompression is the recommended surgical intervention.
  • Further research is needed to fully elucidate the syndrome's origins and optimize treatment outcomes.

Keywords:
Arnold Chiari SyndromeArnold Chiari malformationhydrocephalusposterior cranial fossascoliosissyringomyelia

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