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"Sandwich Deformity" in Klippel-Feil syndrome: A "Full-Spectrum" presentation of associated craniovertebral junction abnormalities.
J Clin Neurosci 2018; 53:247-249JC

Abstract

Klippel-Feil syndrome (KFS) is defined as congenital fusion of two or more cervical vertebrae resulting from a segmentation failure in the developing spine. According to Samartzis et al., the most commonly fused segments are found at C2/3 (74.1%) and C6/7 (70.4%). In patients with C2/3 fusion, especially when there is additional C1 occipitalization, several secondary anomalies including atlantoaxial dislocation (AAD), basilar invagination (BI), Chiari malformation, and syringomyelia can be identified. In this report, we present a case of a 12-year-old patient with C2/3 and occipitalization and a "Full-Spectrum" presentation of associated CVJ abnormalities including C0/1 fusion, AAD, BI, Chiari malformation, syringomyelia, myelopathy and cranial neuropathy received neurological decompression of the cervico-medullary junction by posterior reduction of the AAD and reconstruction of her CVJ using an unconventional hybrid construct due to a high-riding right vertebral artery in C2. To our knowledge, her "Full-Spectrum" presentation may include the most categories of concomitant abnormalities in the literature. In addition, She received neurological decompression of the cervico-medullary junction using an unconventional hybrid construct due to a high-riding vertebral artery in C2. Three months after the surgery, all of her symptoms recovered significantly. Neither Chiari malformation nor syringomyelia could be identified by MRI two years after the surgery. At the last follow-up (4 years), the patient became completely asymptomatic.

Authors+Show Affiliations

Orthopaedic Department, Peking University Third Hospital, Beijing, China.Orthopaedic Department, Peking University Third Hospital, Beijing, China.Orthopaedic Department, Peking University Third Hospital, Beijing, China.Orthopaedic Department, Peking University Third Hospital, Beijing, China. Electronic address: pkuwsl@126.com.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

29731280

Citation

Tian, Yinglun, et al. ""Sandwich Deformity" in Klippel-Feil Syndrome: a "Full-Spectrum" Presentation of Associated Craniovertebral Junction Abnormalities." Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia, vol. 53, 2018, pp. 247-249.
Tian Y, Fan D, Xu N, et al. "Sandwich Deformity" in Klippel-Feil syndrome: A "Full-Spectrum" presentation of associated craniovertebral junction abnormalities. J Clin Neurosci. 2018;53:247-249.
Tian, Y., Fan, D., Xu, N., & Wang, S. (2018). "Sandwich Deformity" in Klippel-Feil syndrome: A "Full-Spectrum" presentation of associated craniovertebral junction abnormalities. Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia, 53, pp. 247-249. doi:10.1016/j.jocn.2018.04.047.
Tian Y, et al. "Sandwich Deformity" in Klippel-Feil Syndrome: a "Full-Spectrum" Presentation of Associated Craniovertebral Junction Abnormalities. J Clin Neurosci. 2018;53:247-249. PubMed PMID: 29731280.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - "Sandwich Deformity" in Klippel-Feil syndrome: A "Full-Spectrum" presentation of associated craniovertebral junction abnormalities. AU - Tian,Yinglun, AU - Fan,Dongwei, AU - Xu,Nanfang, AU - Wang,Shenglin, Y1 - 2018/05/03/ PY - 2018/02/27/received PY - 2018/04/22/accepted PY - 2018/5/8/pubmed PY - 2018/9/6/medline PY - 2018/5/8/entrez KW - Atlantoaxial dislocation (AAD) KW - Basilar invagination (BI) KW - Klippel-Feil syndrome (KFS) KW - Syringomyelia KW - Tonsillar herniation SP - 247 EP - 249 JF - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JO - J Clin Neurosci VL - 53 N2 - Klippel-Feil syndrome (KFS) is defined as congenital fusion of two or more cervical vertebrae resulting from a segmentation failure in the developing spine. According to Samartzis et al., the most commonly fused segments are found at C2/3 (74.1%) and C6/7 (70.4%). In patients with C2/3 fusion, especially when there is additional C1 occipitalization, several secondary anomalies including atlantoaxial dislocation (AAD), basilar invagination (BI), Chiari malformation, and syringomyelia can be identified. In this report, we present a case of a 12-year-old patient with C2/3 and occipitalization and a "Full-Spectrum" presentation of associated CVJ abnormalities including C0/1 fusion, AAD, BI, Chiari malformation, syringomyelia, myelopathy and cranial neuropathy received neurological decompression of the cervico-medullary junction by posterior reduction of the AAD and reconstruction of her CVJ using an unconventional hybrid construct due to a high-riding right vertebral artery in C2. To our knowledge, her "Full-Spectrum" presentation may include the most categories of concomitant abnormalities in the literature. In addition, She received neurological decompression of the cervico-medullary junction using an unconventional hybrid construct due to a high-riding vertebral artery in C2. Three months after the surgery, all of her symptoms recovered significantly. Neither Chiari malformation nor syringomyelia could be identified by MRI two years after the surgery. At the last follow-up (4 years), the patient became completely asymptomatic. SN - 1532-2653 UR - https://www.unboundmedicine.com/medline/citation/29731280/"Sandwich_Deformity"_in_Klippel_Feil_syndrome:_A_"Full_Spectrum"_presentation_of_associated_craniovertebral_junction_abnormalities_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0967-5868(18)30363-1 DB - PRIME DP - Unbound Medicine ER -