Klippel Feil syndrome

Case contributed by Dr Sinéad Culleton

Presentation

Neck pain.

Patient Data

Age: 25 years
Gender: Male
X-ray

Fusion of C2-C3 and C5-C6-C7.

CT

Fusion of C2-C3  and C5-C6-C7 again is noted, with a bone island posterior aspect C4.

MRI

MRI demonstrates the wasp-waist sign. The degree of canal stenosis at the level of C4-C5 actually reduces with flexion of the neck also during flexion.

X-ray

Cervical spine x-rays post op. The patient underwent anterior fusion and decompression C4-C5.

Case Discussion

Klippel-Feil syndrome is characterized by failure in segmentation of two or more cervical vertebrae. Congenital fusion can be seen occurring at any level in the cervical spine, In up to 75% of cases fusion is seen to effect the first three cervical vertebrae 1,2. In addition Klippel -Feil may be associated with anomalies of the aortic arch, subclavian, carotid and vertebral arteries, and a persistent trigeminal artery 3-7.

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Case information

rID: 40548
Published: 2nd Nov 2015
Last edited: 20th Dec 2019
Inclusion in quiz mode: Included

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