Klippel-Feil syndrome

Case contributed by Dr Karwan T. Khoshnaw

Presentation

Chronic neck pain and backache

MRI

Evidence of cervical vertebral scoliosis with apex to the right, there are a fusion of C2-C3 and C6-C7 cervical vertebra with reduced anteroposterior diameter (wasp-waist sign), at C3-C4 and C5-C6 disc spaces: moderate degenerative dehydration with decreased height, there is a circumferential disc bulge indenting adjacent ventral theca, compressing on left nerve roots and causing mild canal stenosis at these levels, normal other intervertebral discs with mild degenerative dehydration.

the above findings in favor of a Klippel-Feil syndrome type III.

MRI

Evidence of lumbar vertebral scoliosis with apex to the right, there is an abnormal shape and partially fused vertebra (hemivertebra) at the level of L3-L5. At L3-L4, L4-L5 and L5-S1 disc spaces: Moderate degenerative dehydration, mildly decreased height and there is a circumferential disc bulge mildly indenting adjacent ventral theca at these levels, normal other intervertebral discs with mild degenerative dehydration.

there is a tethered cord and type II Diastematomyelia at L4-L5 vertebral body.

Case Discussion

Klippel-Feil syndrome is a complex heterogeneous entity that results in cervical vertebral fusion. Two or more non-segmented cervical vertebrae are usually sufficient for diagnosis.

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Case information

rID: 66688
Published: 1st Mar 2019
Last edited: 4th Mar 2019
System: Spine
Inclusion in quiz mode: Included

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