Pierre-Robin syndrome associated with Chiari type I malformation

Jangbo Lee, Kazutoshi Hida, Toshitaka Seki, Jun Kitamura, Yosinubo Iwasaki

    Research output: Contribution to journalArticlepeer-review

    19 Citations (Scopus)


    Case Report: Pierre-Robin syndrome (PRS) is a rare congenital malformation that shows severe micrognathia and cleft soft palate. A 15-year-old boy who was admitted with occipital headache and gait disturbance was diagnosed with PRS. Radiological evaluation revealed severe herniation of the cerebellar tonsil and multiple craniovertebral osseous anomalies. We carried out foramen magnum decompression (FMD) with duroplasty. Postoperative MRI showed the cerebellar tonsil was freed from strangulation. Result and Conclusion: This patient presented with the common finding between PRS and Chiari type I on the embryological aspect by illustrating pathophysiology of the Chiari I malformation. To our knowledge, this is the first reported case of PRS associated with Chiari malformation type I.

    Original languageEnglish
    Pages (from-to)380-383
    Number of pages4
    JournalChild's Nervous System
    Issue number5-6
    Publication statusPublished - 2003 Jun 1


    • Chiari malformation
    • Embryogenesis
    • Foramen magnum decompression
    • MRI
    • PRS
    • Pierre-Robin syndrome

    ASJC Scopus subject areas

    • Pediatrics, Perinatology, and Child Health
    • Clinical Neurology


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