Orthopedics - Oleksa A.P. 2006

Chest Deformities
Congenital High Scapula (Sprengel's Deformity)

Congenital elevation of the scapula was first described in 1883 by Willet and Walscham. Later, in 1891, Sprengel published four personal cases and provided a detailed Description of the Clinical Features of the condition, identifying it as a congenital malformation.

In congenital elevation of the scapula, its normal intrauterine development is impaired. This condition is occasionally accompanied by defects in chest formation and spine ossification, such as Spina bifida occulta and wedge vertebrae.

While bilateral scapular elevation does occur, unilateral left-sided elevation is more common (85%). In this condition, one scapula is positioned higher than the other and displays an abnormal shape (Fig. 248). The superior medial border of the scapula is elongated and prominent, and its width often significantly exceeds its length. In some cases, the scapula is positioned so high that, in Sprengel's words, "it rests on the patient's occiput."

Sometimes the scapula is fixed to the spine, and radiographs reveal a synostosis between the scapula and the VI or VII cervical spinous process, or a fibrous fusion or cartilaginous bridge (V.D. Chaklin) connecting the superior medial angle of the scapula to the specified spinous process.

Studying The Development of the scapula during the Embryonic period, Wolf and Levis concluded that the scapular bud appears at the 5th week of intrauterine development, at which point it is located at the level between the IV cervical and II thoracic vertebrae. Gradually, the scapula descends, and by the end of the 3rd month of gestation, it lies between the VI cervical and IV thoracic vertebrae. Throughout development, The ratio of the scapula's width to its length changes.

The elevated position and altered shape of the scapula in Sprengel's deformity indicate delayed intrauterine development.

The scapula is displaced not only upward but also medially due to tension in the trapezius Muscle, in which congenital dystrophy and defects in its lower portion have been observed (Potel). Occasionally, the rhomboid, deltoid, or sternocleidomastoid Muscles may be absent.

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Fig. 248. Elevation of the left scapula.

Upon Palpation, the affected scapula is shorter and broader than the healthy one, protrudes from the chest wall, and is rotated around its axis such that its inferior angle approaches the spine. Scapular elevation is primarily a cosmetic defect, with patients only developing Complaints of difficulty raising the arm above 90° as older children.

Treatment. Congenital elevation of the scapula does not respond to conservative treatment, so one should not rely on it as a primary cure; however, it should be utilized prior to surgery to improve local muscle condition, Blood AND Lymph Circulation, and innervation. To this end, Therapeutic Exercises are prescribed to stretch the trapezius and rhomboid muscles and strengthen the entire muscle group of the back and shoulder girdle.

Surgical Procedures are performed on children who have reached 3 to 4 years of age. However, Bojchev, Conforti, and Chokanov believe that in preschool age, this surgery should only be performed if the deformity is unilateral, the scapular elevation is modest, and radiographic imaging reveals a bony bridge between the superior medial angle of the scapula and the spinous process, or between the scapula and a rib.

These authors consider bilateral scapular elevation a contraindication for surgery, as the muscles are severely contracted, making it extremely difficult—if not impossible—to lower the scapulae.

The primary goal of surgical intervention is to lower the scapula and secure it in its new position. Several surgical techniques have been proposed, ranging from relatively simple to complex, depending on the height of the scapular elevation, the degree of its fixation, and the patient's age.

König's operation is performed under endotracheal anesthesia with blood transfusion. A vertical incision is made along the entire length of the scapular body, which is osteotomized close to the vertebral border (2 cm from the edge). After mobilizing the lateral portion of the scapula, it is lowered to the normal level and sutured to the previously displaced upper vertebral border. The scapula is joined using two to three wire sutures, after which its inferior angle is anchored to the surrounding muscles using additional sutures.

Putti's operation involves a transverse incision of the trapezius muscle via dissection along the medial border of the scapula. Using an osteotome or resection knife, the bony or fibrous fusion between the superior medial angle of the scapula and the spinous process is divided or broken. The scapula is then lowered and fixed to the VII rib using a silk loop passed through its inferior angle.

Hick performs the same Procedure as Putti but supplements it with a Z-shaped osteotomy and lengthening of the clavicle, as the clavicle acts as the main obstacle to lowering even a mobilized scapula. Only after lengthening the clavicle does he lower the scapula and anchor its inferior angle with a loop to the seventh rib. Sometimes Hick also resects the upper portion of the scapula situated above its spine, which enhances the cosmetic outcome. Such operations yield good results in mild deformities; however, in severe cases, fully lowering the scapula (by more than 3 cm) is rarely achieved, and significant challenges arise in older children with pronounced scapular elevation. Therefore, V.D. Chaklin performed a resection of the superior and vertebral borders of the scapula, which facilitates its mobilization and improves the cosmetic result.

F.R. Bogdanov (1948) performed an osteotomy of the coracoid process of the scapula (*processus coracoideus*) combined with an oblique osteotomy of the clavicle, making it easier to separate the anterior and posterior muscle groups and thereby lower the scapula.

S.D. Ternovsky, while performing Bogdanov's operations, concluded that a clavicular osteotomy is not strictly necessary and proposed the following technique. Through a Skin incision curving superiorly and medially around the scapula, the broad Muscles of the back—the trapezius and rhomboids—are divided. Using periosteal elevators, the medial border of the scapula is freed from adjacent muscles. Next, the superior angle of the scapula and the Base of the coracoid process are exposed, and any adhesions between the scapula and the spinous process or chest wall are excised. An osteotomy of the coracoid process is performed near its base. The scapula is then lowered, and its inferior angle is fixed (via a prepared hole) to the seventh or eighth rib using a silk loop to align it with the healthy scapula. The wound is closed, an aseptic dressing is applied, and the arm is immobilized in a plaster cast with the shoulder abducted up to 90°. After one month, the cast is removed, and physical therapy and muscle massage are initiated.

Nowadays, instead of a silk suture, a Dacron vascular prosthesis or a Dacron tape (10 mm) is used to anchor the scapula. It is secured in a similar loop-and-sling fashion, as well as subperiosteally to the seventh rib.

Tylman D., Stępień K., and Marczyński W. (1998) performed an oblique osteotomy of the acromion in all cases, re-apposing it with braided suture material, followed by resection of the superior-medial angle of the scapula and repositioning it to an approximately normal level. Postoperatively, a Dessault plaster cast was applied for 4–6 weeks, followed by physical therapy.

Among the described techniques, V.D. Chaklin's procedure is the technically simplest; however, it is unsuitable for patients with severe scapular elevation.

Medical literature contains reports of Brachial Plexus palsies occurring after simple lowering of the scapula via Putti's method, caused by compression from the clavicle.

Because Sprengel's deformity is a rare congenital pathology, Surgical treatment should be performed by qualified specialists.