Introduction

Sprengel shoulder, which is characterized by congenital high riding scapula, is a rare complex deformity of shoulder griddle [1]. It is diagnosed as upward elevation of scapula in relation to thoracic cage or abnormally high placed scapula. Eulenberg, first described this condition of undescended scapula in 1863, and claimed its cause as traumatic dislocation of the scapula [2]. Later, it was Otto Sprengel who described the pathology and proposed a theory of its existence in 1891. The scapular embryonic primordium appears in 5^th intrauterine week at the level of 4^th to 5^th cervical vertebrae & acquires its final morphology by 8^th week of gestation & descends till 12^th week over the upper 5 ribs to reach the correct anatomical position. This failure to descend leads to sprengel deformity [3].

The exact cause of the interruption of the normal caudal migration of the scapula during fetal development is unknown, but different theories have been postulated, like cerebrospinal fluid leak through the membranes of the roof of the fourth ventricle into the adjacent tissues of the neck, increased intrauterine pressure, and abnormal articulation of the scapula to the cervical vertebrae with abnormal muscle formation (fibrous, cartilaginous, or osseous omovertebral) [4].

Females are more affected and it is bilateral in about 10-30% with a predilection to left side. It can be part of other congenital anomaly. It is associated with severe cosmetic deformity and functional leads to limitation of shoulder movements [5]. We report such a rare case of isolated congenital sprengel shoulder with was treated by Green’s procedure successfully.

Case Report

A 6 year old male child visited to us in outdoor patient department with complains of visible deformity of left shoulder. The child was delivered by full term normal vaginal delivery. He had full immunization completed. Parent and child denied any other complain present, other than the deformity.

Familial history was unremarkable for any congenital disorder. On detailed history, the deformity and asymmetry of shoulder folds was observed by the parents at the age of one year of the child but they did not take any treatment. Gradually, the deformity is increasing and has grown to its current size which is not cosmetically accepted to the parents as well as the child.

There is also difficulty in dressing up himself as well as difficulty in carrying his school bag. Child would complain of pain in left shoulder after returning from school. On clinical examination, there was asymmetrical elevation of the left shoulder fold compared to the right side, which was visible both from front and back.

Since the deformity was severe, initial inspection gave impression of congenital short neck with torticollis as well, but careful examination after proper exposure showed no torticollis although slight deviation of head towards left side was noted. His neck and its movements were normal.

Figure 2: Intraoperative photographs (a to d) of modified Green’s procedure

Figure 3: Post-operative AP (a) X ray of shoulder gridle & clinical photograph (b) showing good cosmetic correction & range of motion

Scapula was displaced distally into the pocket in latissimus dorsi along the attached muscles and holding scapula in this position trapezius, rhomboideus major and minor are reattached to spine of scapula and medial border of scapula respectively using non absorbable sutures (fig 2). Immediate post-operative radiograph of patient showed descended scapula with its superior angle at C7 level. Post-operatively, patient was given a shoulder immobiliser for 2 weeks, following which suture removal was done and shoulder range of motion was started. Patient showed good recovery with a 2 month follow up showing both the inferior angle of scapula at same level as well as significantly increased shoulder range of motion (fig 3).

Discussion

Sprengel shoulder occurs due to inadequate caudal movement of scapula during development resulting in an abnormally high placed scapula, which can be unilateral or bilateral [1-4]. This high position of the scapula in the process of skeletal development leads to changes in axial rotation, shape, and size and other musculoskeletal defects including hypoplasia, medialization, and adduction of the scapula, prominence of its upper angle, distal rotation and lateral angulation of the glenoid cavity, changes in the position of the clavicle, omovertral bar (ossified fibrous band between cervical spine with the undescended scapula), anomalies of the cervicothoracic vertebrae and ribs, and muscular hypoplasia or atrophy of the shoulder musculature [6]. Thus although, the deformity commonly occurs sporadically, but it can occur in combination with other congenital anomalies, such as congenital scoliosis, spina bifida, fusion of cervical vertebrae, like Klippel-Feil syndrome, kidney abnormalities and cleft palate [7,8]. But we did not find any associated anomaly with our patient.

Surgical complications described include scar-related complications, brachial plexus palsy, brachial neuritis, winging of the scapula, re-growth of the resected omovertebral bar, recurrence of the deformity and prominence of the sternoclavicular joint [12-16]. To prevent the complications of brachial plexus palsy, either osteotomy of clavicle or avoiding excessive correction is advised. Our patients at the last follow-up had got excellent cosmetic correction and increased range of motion without any complications.

Conclusion

Sprengel shoulder is a rare congenital deformity, which occurs due to failure of the scapula to descend during intrauterine development. It can lead to cosmetic problems and limitation of movements, but is accurately diagnose by plain x rays. Milder forms can be treated conservatively, whereas surgical correction is needed for severe variety.

References