ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2022
Long-term imaging course of Chiari malformation type I due to fibrous dysplasia/McCune-Albright syndrome.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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- Pediatric triad of craniofacial fibrous dysplasia, Chiari malformation type I and syringomyelia: a case report.Acta neurochirurgica · 2026Article
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Abstract
introductionMcCune-Albright syndrome (MAS) and fibrous dysplasia (FD) have been reported to cause Chiari type I malformation (CM1) and skull base invagination (BI). CASE: A 6-year-old girl was diagnosed with MAS and FD. She was diagnosed with CM1 at age 8 years, and the syringomyelia had gradually increased by age 20 years. We performed foramen magnum decompression and C1 laminectomy, and the syringomyelia stopped spreading after surgery. DISCUSSION: This patient underwent long-term radiological observation and morphological evaluations, which revealed that the skull thickening was progressing, while the posterior cranial fossa volume (PCFV) remained unchanged for 14 years. Therefore, although PCFV did not decrease, it was considered to be relatively inadequate due to the increase in brain volume with growth, resulting in posterior fossa overcrowding, causing CM1.
conclusionIn patients with FD/MAS, long-term evaluation of bone thickening, odontoid position, and PCFV is necessary.
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