Radiology Resident Case of the Week
Etiology/Pathophysiology
Original Klippel Feil syndrome consists of a triad of signs including; short neck; a low posterior hair line; limitation of movement. Current definition of the syndrome is congenital fusion of 2 or more cervical vertebrae. classic triad is seen in 50% of the patients.
Sprengel's deformity is unilateral or bilateral elevation of scapula and seen in 20-25% of the patients, especially in the cases with high and extensive cervical fusions. other associated malformations with Klippel Feil syndrome are cervical ribs, webbed neck(pterygium coli), hemivertebrae, spina bifida.
Pathology:
Miscellaneous
Imaging
AP view of the chest shows elevation of the left scapula. otherwise, unremarkable. lateral view of the cervical spine demonstrates congenital fusion of the c5-6 cervicall vertebral bodies.
DDX
nothing
Key references
Resnick; bone and joint imaging
ACR Code
Keywords: