The Association between Congenital Elevation of the Scapula and Diastematomyelia

Congenital elevation of the scapula was first described by Eulenburg in 1 863 and there have been many descriptions and reviews of the literature since then (Horwitz 1908; Cavendish i972; Ross and Cruess 1 977). The condition is usually known as Sprengel’ s shoulder (Sprengel i891), and because it is clinically recognisable and partiafly impairs function it is more familiar to orthopaedic surgeons than is diastematomyelia. Diastematomyelia consists of complete separation of the spinal cord into two, not necessarily equal, parts which are often contained in separate dural sheaths and lie together in an expanded spinal canal over a distance of several segments. A septum, which is frequently bony, separates the two parts in an approximately sagittal plane, and is attached ventrally to the rear of a vertebral body and dorsally to the often malformed lamina. In most cases the onset of progressive neurological deficiency in the lower limbs or disturbance of control of the bladder lead to the discovery of the spinal abnormality. Several authors have stressed the danger to the spinal cord during corrective operations for congenital scoliosis that can arise from diastematomyelia (Faithfull 1973; Gillespie et al. i973; Keim and Greene 1973; Winter 1973; Winter et al. 1974; Leonard 1975; Banniza von Bazan et a!. 1976). Recently we have reported several patients with diastematomyelia and abnormal caudal fixation of the spinal cord in whom there was no neurological deficiency (Banniza von Bazan, Krastel and Lohkamp i978). We suggested that diastematomyelia is not as rare as had previously been thought and that the abnormal anatomical findings frequently give rise to no symptoms and remain undetected, so that in many instances the abnormality itself may have no orthopaedic or neurological significance. Neither of these uncommon deformities has yet been fully explained, but we have seen them occurring together in the same individual on several occasions and believe they may have a common aetiology. This is the basis of the present paper.