Sprengel's Deformity Case Study

Sprengel’s deformity is a rare congenital deformity of the shoulder girdle due to failure of descent of the scapula. It causes cosmetic concern and sometime functional disability. This study describes a grade I congenital deformity of the left shoulder joint (Sprengel’s deformity) in a 14-year-old female, associated with an undescended scapula, the presence of hemi-vertebrae of C7,some degree of limited shoulder abduction and scoliosis of upper dorsal spine with convexity towards left. The diagnosis of SD should be differentiated from osteomalacia, rickets, malunited scapular fracture, cervical tuberculosis, and paralysis. As deformity was very less and patient had minimal shoulder restriction, the patient was advised for conservative management

The SD has been firstly described by Eulenberg in 1863.[9] In 1891, Sprengel described four cases of upward dislocation of the scapula.[10] Further, in the same year, Kolliker described four cases of upward displacement of the scapula and named the condition Sprengel’s deformity.[11] The aetiology of SD is unknown and few cases of familial SD described.[8] Most authors agree that the incidence of the undescended scapula affects women three times more often than men, although Kadavkolan and others reported the incidence of this entity equally in both sexes.[12],[13] We also reported a case of a female

In most of the patients with a Sprengel’s deformity, active abduction is limited, which was also seen in the present study.[4],[5],[12],[14] The severity of the condition has been classified by Cavendish into four clinical grades of increasing severity, from very slight malformation (grade I) to severe malformation (grade IV), depending on the size of the swelling and how far the scapula is raised [Table 1].[15] The presence of high scapula seen on X-ray confirms the diagnosis. Rigault radiographic classification used to grade the severity of the condition [Table 2].[16] Similar to the present study, the left side is usually more commonly affected than the right