|Title : Case 641|
Age / Sex : 48 / M
Chief complaint: numbness of both upper and lower extremities
Answer: Klippel-Feil syndrome (type II)
Findings: C-spine plain radiographs of AP, Lateral projection, Chest PA and coronal and sagittal reformatted CT images. Segmentation failure of C2-C3-C4 vertebrae. And bony fusion of atlas (anterior arch and lateral masses) with occipital bone. Abnormal elongated bone lesion in the posterior neck, at from C5-6 level to upper back on lateral projection, indicating omovertebral bone. This extends to, and articulates with upper portion of left scapular bone, which is abnormally high in position (Splengel deformity). Also seen is spinal bifida at C2~C6 (not shown here).
Complex disorder characterized by congenital fusion of the cervical vertebrae with a short neck, limitation of the movement of the head or neck and a low posterior hairline.
Etiology: failure of the normal segmentation of the cervical somites during the 3rd to 8th weeks of gestation.
Incidence: 1:40,000- 42,000
Multiple system anomalies: scoliosis(60%), renal anomalies(35%), Sprengel’s deformity(30%), deafness(30%), congenital heart disease(14%)
May develop neurological symptoms that are secondary to the degenerative disc disease of the adjacent mobile segments, spinal instability from the hypermobility or from trauma, and spinal stenosis in later decades of life
Congenital high scapula, a characteristic high dysmorphic scapula; most common congenital abnormality of the shoulder
Caused by arrested caudal migration of the scapula during embryogenesis
Dysplastic scapula: hypoplastic, decreased height to width ratio and occasionally with an anteriorly curved spinous process. At imaging, elevation of the affected scapula with inferior angle rotated medially pointing to the spine
M = F, left>right, bilateral in 10%
|Total applicants||30||Correct answers||27|
|윤유성||순천향대 부천병원, 전문의|
|장민영||국민건강보험공단 일산병원, 전문의|