Journal article
Pierre-Robin syndrome associated with Chiari type I malformation
Child's nervous system, Vol.19(5-6), pp.380-383
06/01/2003
DOI: 10.1007/s00381-003-0740-2
PMID: 12748800
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.
Details
- Title: Subtitle
- Pierre-Robin syndrome associated with Chiari type I malformation
- Creators
- Jangbo Lee - Hokkaido UniversityKazutoshi Hida - Hokkaido UniversityToshitaka Seki - Department of Neurosurgery, Graduate School of Medicine, University of Hokkaido, N-15 W-7, Kita-Ku, 060-638 Sapporo, JapanJun Kitamura - Hokkaido UniversityYosinobu Iwasaki - Hokkaido University
- Resource Type
- Journal article
- Publication Details
- Child's nervous system, Vol.19(5-6), pp.380-383
- Publisher
- Springer
- DOI
- 10.1007/s00381-003-0740-2
- PMID
- 12748800
- ISSN
- 0256-7040
- eISSN
- 1433-0350
- Language
- English
- Date published
- 06/01/2003
- Academic Unit
- Neurosurgery
- Record Identifier
- 9984460335502771
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