Sprengel deformity
Overview
Sprengel's deformity is a condition where the scapula (shoulder blade) on one or both sides are underdeveloped (hypoplastic) and abnormally high. It is due to failure of descent of the scapula during embryonic development from its position in the neck to its normal position in the posterior thorax.. It happens usually in girls, and may have a genetic basis in some instances.
Symptoms
The obvious problem is cosmesis. But there is often also a functional problem. The hypoplastic scapula is usually tethered to the spine and posterior ribs by tight bands and even an omohyoid bone, which restricts scapular movements, and therefore abduction of the arm. There are also frequent associated problems, like absence or hypoplastic trapezius, deltoid and rhomboid muscles on the affected side, spine and neck problems like torticollis, scoliosis and Klippel-Feil syndrome. Limb length discrepancy is not uncommon, and visceral abnormalities like absent kidney, atrial septal defect and situs inversus can be present.
Treatment
X-rays to determine the severity of the Sprengel's deformity are necessary, as well as to rule out any neck or spinal abnormalities. Tests to rule out visceral abnormalites are also important. The child should also be checked for limb length discrepancies and muscle weakness. In mild cases, where cosmesis is not a major problem, and there is no restriction of shoulder abduction or muscle weakness or associated problems, regular follow-up is all that is necessary. In more severe ases, surgery can be performed to release the tight band and remove the omohyoid bone that tether the scapula in its abnormal position. The trapezius and rhomboid muscles are detached from their origins in the spinous processes and reattached at a lower level to keep the scapula in a more normal position.