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Child's Nervous System

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01-06-2003 | Case Report

Pierre-Robin syndrome associated with Chiari type I malformation

Authors: Jangbo Lee, Kazutoshi Hida, Toshitaka Seki, Jun Kitamura, Yosinobu Iwasaki

Published in: Child's Nervous System | Issue 5-6/2003

Abstract

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.

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Title: Pierre-Robin syndrome associated with Chiari type I malformation
Authors: Jangbo Lee
Kazutoshi Hida
Toshitaka Seki
Jun Kitamura
Yosinobu Iwasaki
Publication date: 01-06-2003
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 5-6/2003
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-003-0740-2

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Pierre-Robin syndrome associated with Chiari type I malformation

Abstract

Case report

Result and conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Case report

Result and conclusion

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