Sprengel's Deformity

Abstract

Sprengel's deformity, a striking abnormality involving one or both scapulæ, was first described by Eulenburg in 1863 (1). It received its present name after Sprengel's description in 1891 (2). The affected scapula, more often the left (3), is rotated on its sagittal axis, so that instead of being parallel to the spine, the superior vertebral border is closer to the medial line and the inferior angle closer to the axilla. While this condition is present at birth, it is not always recognized before adult life. This congenital elevation of the scapula is usually associated with other abnormalities, such as defects of the ribs, vertebrae, or muscles. Among these are fusion or malformation of the ribs or vertebrae. Defects of the cervical vertebræ; are especially prone to cause the Klippel-Feil (4) syndrome of short neck, limitation of head movements, and growth of the hair low down on the neck. Scoliosis is found in less than half of the cases and is compensatory because of the attempt to straighten the back. As Sprengel's deformity is usually unilateral, the patient has an asymmetrical appearance, the neck being thicker and shorter on the affected side. The mobility of the scapula is lessened and the movements of the arm are restricted. There are weakness and poor development of the muscles which normally hold the scapula, especially the trapezius (5). The maldevelopment with consequent altering of the shape of the scapula is due to improper muscular tension, as the traction of the muscles aids in shaping the bone during the growth process. Often there are defects in, or even absence of, certain muscles (6). The muscles attached are fourteen in number, namely rhomboideus major and minor, teres major and minor, pectoralis minor, coracobrachialis, deltoid, triceps, biceps, trapezius, omohyoideus, supraspinatus, levator scapulae, infraspinatus, subscapularis, serratus anterior (7). The high position of the scapula is explained by failure of its normal descent in the embryo. The scapulae appear about the fifth week of embryonic development, at the level of the fifth cervical and first dorsal vertebrae. They gradually lengthen and descend to the normal position at the level of the second to the seventh dorsal vertebrae. The migration of the scapula is due to muscular actions which may be insufficient where the intra-uterine pressure is excessive or where the musculature is defective (8). The cause of the deformity, according to Sprengel, is abnormal intra-uterine pressure, although the frequent occurrence of bone and muscular defects in the shoulder and elsewhere indicate that the condition is frequently an anomaly of development. Changes in the size and shape of the scapula are due to improper or defective muscular tension. Normally there is a definite ratio between the length and breadth of the scapula.