Arnold Chiari Malformation With Sponastrime (Spondylar and Nasal Changes, With Striations of the Metaphyses) Dysplasia: A Case Report.

Medicine (Baltimore)

From the Department of Neurosurgery (JHJ, S-BI), Department of Radiology (ALL), Soonchunhyang University Bucheon Hospital, Bucheon, Korea; and Department of Pediatrics (SYC, DKJ), Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Published: May 2016

SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain.We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4863758PMC
http://dx.doi.org/10.1097/MD.0000000000003155DOI Listing

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