“Sandwich Deformity” in Klippel-Feil syndrome: A “Full-Spectrum” presentation of related craniovertebral junction abnormalities.
J Clin Neurosci. 2018 Jul;53:247-249
Authors: Tian Y, Fan D, Xu N, Wang S
Klippel-Feil syndrome (KFS) is outlined as congenital fusion of two or extra cervical vertebrae ensuing from a segmentation failure within the growing backbone. Based on Samartzis et al., probably the most generally fused segments are discovered at C2/three (74.1%) and C6/7 (70.four%). In sufferers with C2/three fusion, particularly when there may be further C1 occipitalization, a number of secondary anomalies together with atlantoaxial dislocation (AAD), basilar invagination (BI), Chiari malformation, and syringomyelia could be recognized. On this report, we current a case of a 12-year-old affected person with C2/three and occipitalization and a “Full-Spectrum” presentation of related CVJ abnormalities together with C0/1 fusion, AAD, BI, Chiari malformation, syringomyelia, myelopathy and cranial neuropathy acquired neurological decompression of the cervico-medullary junction by posterior discount of the AAD and reconstruction of her CVJ utilizing an unconventional hybrid assemble on account of a high-riding proper vertebral artery in C2. To our data, her “Full-Spectrum” presentation might embody probably the most classes of concomitant abnormalities within the literature. As well as, She acquired neurological decompression of the cervico-medullary junction utilizing an unconventional hybrid assemble on account of a high-riding vertebral artery in C2. Three months after the surgical procedure, all of her signs recovered considerably. Neither Chiari malformation nor syringomyelia could possibly be recognized by MRI two years after the surgical procedure. On the final follow-up (four?years), the affected person grew to become fully asymptomatic.
PMID: 29731280 [PubMed – indexed for MEDLINE]