title: Klippel-Feil syndrome associated with a craniocervico-thoracic dermoid cyst.
abstract: BACKGROUND
  Uncommonly, Klippel-Feil syndrome (KFS) has been associated with intracranial or spinal tumors, most frequently dermoid or epidermoid cysts. Although the associated dermoid cyst (DC) is usually located in the posterior fossa, isolated upper cervical DC has been reported. Extension from the posterior fossa to the upper cervical spine (C2) has been reported once. We report a rare case of KFS in association with a posterior fossa DC that extended down to the upper thoracic spine and review the current literature.
  
  
  METHODS
  A 47-year-old female with presented cervical myelopathy related to a cranio-cervico-thoracic DC in association with KPS-related cervicothoracic fusion (C2-T6) and thoracic kyphosis. The patient underwent complete tumor resection following sub-occipital craniectomy and C1-C4 cervical laminectomy. The patient exhibited complete resolution of symptoms with no tumor recurrence and no deformity at 6-year follow-up.
  
  
  CONCLUSIONS
  DC should be added to the list of congenital central nervous system abnormalities, which should be sought in patients with KFS. Therefore, the presence of a cystic lesion in the posterior fossa, the craniocervical junction or the anterior cervical spine should suggest the possibility of a DC in patients with KFS. In cases of cranio-cervical DC, the tumor may extend quite far down the spinal column (reaching the thoracic spine), as demonstrated in the present case.
authors: Department of Surgery, Division of Neurosurgery, Spine Unit, Centre Hospitalier de l'Université de Montréal - Hôpital Notre-Dame, Canada.
authors: Nancy
authors: N
authors: McLaughlin
authors: Alexander G
authors: AG
authors: Weil
authors: Jacques
authors: J
authors: Demers
authors: Daniel
authors: D
authors: Shedid
keywords: Congenital spinal malformation; Klippel–Feil syndrome; craniocervical junction; dermal sinus; dermoid cyst
references: Neurol Res. 2002 Jul;24(5):501-4
references: 12117323
references: Childs Nerv Syst. 2001 Jan;17(1-2):97-100
references: 11219631
references: Neurosurgery. 2003 Nov;53(5):1230-3; discussion 1233-4
references: 14580293
references: Childs Nerv Syst. 1985;1(5):255-63
references: 3910229
references: Pediatr Neurol. 2011 Sep;45(3):197-9
references: 21824571
references: Clin Orthop Relat Res. 2004 Jul;(424):183-90
references: 15241163
references: Pediatr Neurosurg. 2001 Sep;35(3):158-61
references: 11641627
references: Va Med. 1982 Aug;109(8):520-1
references: 7136233
references: J Bone Joint Surg Am. 1974 Sep;56(6):1246-53
references: 4436358
references: Neurosurgery. 1989 Oct;25(4):652-5
references: 2677824