Klippel-Feil malformation was reported first as a clinical triad of short or absent neck, severe limitation of head movement and a low posterior hairline in 1912 and has an incidence of about 1 in 42,000 births. The main anomalies associated with this syndrome are Sprengel deformity, spina bifida, mostly of the cervical spine, deafness, scolio-sis or kyphoscoliosis and pterygium colli. Many other anomalies may be present. The cervical spine deformity is mostly due to a congenital numerical reduction of cervical vertebra due to a lack of embryological segmentation involving the bodies, which are often reduced in height and the vertebral arches.
展开▼