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Craniocervical arachnoid cyst in a patient with Klippel-Feil syndrome: a unique case: case report.
J Neurosurg Spine 2013; 18(2):161-4JN

Abstract

Klippel-Feil syndrome, or brevicollis, is a complex congenital disorder caused by the improper segmentation of the cervical vertebrae. The authors present the very rare case of a patient with Klippel-Feil syndrome who presented with an intradural arachnoid cyst at the craniocervical junction. They also examine possible factors contributing to this association. A 46-year-old woman presented with complaints of progressively worsening headaches and dizziness of 18 months' duration. She also demonstrated mild bilateral upper-extremity weakness. Magnetic resonance imaging revealed fused cervical vertebrae and a dorsal intradural arachnoid cyst at the craniocervical junction, extending down to the fourth cervical level. Because of worsening myelopathy and the presence of brainstem compression, the patient underwent surgical excision of the arachnoid cyst, which was approached via a midline posterior suboccipital/upper cervical route. An endoscope was introduced through a gap between the occiput and fused upper cervical vertebrae, and the arachnoid cyst was widely fenestrated. Postoperatively, the patient has remained symptom free for more than 2 years with evidence of good radiological decompression. The authors report a unique association between craniocervical arachnoid cyst and Klippel-Feil syndrome. To their knowledge, no other cases of this association have been reported in the literature. Arachnoid cysts should be part of the differential diagnosis in the presence of worsening myelopathic symptoms or pain in patients with Klippel-Feil syndrome.

Authors+Show Affiliations

Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, Tennessee, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

23231357

Citation

Khan, Imad Saeed, et al. "Craniocervical Arachnoid Cyst in a Patient With Klippel-Feil Syndrome: a Unique Case: Case Report." Journal of Neurosurgery. Spine, vol. 18, no. 2, 2013, pp. 161-4.
Khan IS, Ahmed O, Thakur JD, et al. Craniocervical arachnoid cyst in a patient with Klippel-Feil syndrome: a unique case: case report. J Neurosurg Spine. 2013;18(2):161-4.
Khan, I. S., Ahmed, O., Thakur, J. D., Shorter, C. D., & Guthikonda, B. (2013). Craniocervical arachnoid cyst in a patient with Klippel-Feil syndrome: a unique case: case report. Journal of Neurosurgery. Spine, 18(2), pp. 161-4. doi:10.3171/2012.11.SPINE12463.
Khan IS, et al. Craniocervical Arachnoid Cyst in a Patient With Klippel-Feil Syndrome: a Unique Case: Case Report. J Neurosurg Spine. 2013;18(2):161-4. PubMed PMID: 23231357.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Craniocervical arachnoid cyst in a patient with Klippel-Feil syndrome: a unique case: case report. AU - Khan,Imad Saeed, AU - Ahmed,Osama, AU - Thakur,Jai Deep, AU - Shorter,Cedric D, AU - Guthikonda,Bharat, Y1 - 2012/12/11/ PY - 2012/12/13/entrez PY - 2012/12/13/pubmed PY - 2013/5/17/medline SP - 161 EP - 4 JF - Journal of neurosurgery. Spine JO - J Neurosurg Spine VL - 18 IS - 2 N2 - Klippel-Feil syndrome, or brevicollis, is a complex congenital disorder caused by the improper segmentation of the cervical vertebrae. The authors present the very rare case of a patient with Klippel-Feil syndrome who presented with an intradural arachnoid cyst at the craniocervical junction. They also examine possible factors contributing to this association. A 46-year-old woman presented with complaints of progressively worsening headaches and dizziness of 18 months' duration. She also demonstrated mild bilateral upper-extremity weakness. Magnetic resonance imaging revealed fused cervical vertebrae and a dorsal intradural arachnoid cyst at the craniocervical junction, extending down to the fourth cervical level. Because of worsening myelopathy and the presence of brainstem compression, the patient underwent surgical excision of the arachnoid cyst, which was approached via a midline posterior suboccipital/upper cervical route. An endoscope was introduced through a gap between the occiput and fused upper cervical vertebrae, and the arachnoid cyst was widely fenestrated. Postoperatively, the patient has remained symptom free for more than 2 years with evidence of good radiological decompression. The authors report a unique association between craniocervical arachnoid cyst and Klippel-Feil syndrome. To their knowledge, no other cases of this association have been reported in the literature. Arachnoid cysts should be part of the differential diagnosis in the presence of worsening myelopathic symptoms or pain in patients with Klippel-Feil syndrome. SN - 1547-5646 UR - https://www.unboundmedicine.com/medline/citation/23231357/Craniocervical_arachnoid_cyst_in_a_patient_with_Klippel_Feil_syndrome:_a_unique_case:_case_report_ L2 - https://thejns.org/doi/10.3171/2012.11.SPINE12463 DB - PRIME DP - Unbound Medicine ER -