My Congenital Shoulder - Page #4
 

Working Diagnosis:
A small supraspinatus tear with a Sprengel deformity of the right shoulder

Treatment:
The athlete was initially treated with occupational therapy with a focus on pectoralis stretches, postural cueing, and isometric scapular stabilization. He was advanced to rotator cuff strengthening but plateaued because of ongoing pain. He received a corticosteroid subacromial space injection with improved pain control and has continued to progress.

Outcome:
The athlete is back to work lifting boxes and resumed his hiking. He continues to work on a home exercise program that focuses on improving his posture and stretching his pectoralis and trapezius. He now understands why his right shoulder is different from his left and that he may continue to have some limitation in his forward flexion and abduction motions of his shoulder.

Author's Comments:
Although the Sprengel deformity is the most common congenital shoulder girdle malformation, it is rare with a female to male ratio of 3:1. The Sprengel deformity is associated with hypoplasia, medialization, and adduction of the scapula, prominence of the superior angle, and distal rotation and lateral angulation of the glenoid cavity. A fibrous bridge may connect the cervical spine with the scapula which creates the omovertebral bone when ossified. This results in limited abduction of the shoulder as well as limitation in range of motion of the cervical spine.
The severity of the Sprengel deformity is classified radiographically by the Rigault classification. Grade 1 is defined by the superomedial scapular angle lying between the transverse processes of the second and fourth thoracic vertebrae. Grade 2 has the superomedial angle of the scapula between the transverse processes of the fifth cervical vertebrae and the second thoracic vertebrae. Grade 3 has the superomedial angle higher than the transverse process of the fifth cervical vertebrae. Treatment should focus on improving range of motion and function of the shoulder and managing expectations of the individual in regards to the deformity. More severe grades of deformity are likely to fail conservative treatment and need surgical intervention to resect the omovertebral attachments and transfer the origin of the trapezius and rhomboids more distally onto the spinous processes.

Editor's Comments:
This excellent case demonstrates how adult patient may sometimes present with an acute injury, but an overlooked congenital malformation is sometimes not even considered due to the patient's age. The medical team caring for this patient, took a detailed history and physical exam, which helped identify that there was more than a small rotator cuff tear as the pain generator. Pearl for this case, remember Sprengel deformity in the differential diagnosis for scapular asymmetry.

References:
Bindoudi A, Kariki EP, Vasiliadis K, Tsitouridis. The Rare Sprengel Deformity: Our Experience with Three Cases. J Clin Imaging Sci. 2014;4(4):1-5.
Thacker MM (2020, Jul 31). Sprengel Deformity. eMedicine. Accessed on April 18, 2022 at https://emedicine.medscape.com/article/1242896-overview.
Trajcevska E, Bell D (2021, Nov 24). Sprengel Deformity. Reference article, Radiopaedia.org. Accessed on April 18, 2022 at https://radiopaedia.org/articles/sprengel-deformity?lang=us.

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