Sprengel Deformity Treatment & Management

Nonoperative treatment for the Sprengel deformity consists of physical therapy. Exercises are used to maintain an individual's range of motion and to strengthen the weak periscapular muscles.

Many patients with the Sprengel deformity do not require operative intervention. For those who do require surgery, the aims in the correction of this condition are twofold. First, the cause of the scapular binding must be released. Second, the scapula must be relocated.

The main objectives in performing surgery are to improve the cosmetic appearance and contour of the neck and to improve the scapular function when it is severely impaired. The optimal age for operative intervention is controversial; however, most authors would recommend surgery be performed when patients are younger than age 8 years in order to obtain the best surgical result.

Surgical options include subperiosteal resection of part of the scapula, extraperiosteal release, transplantation of the muscular origins of the scapula, excision of the superomedial portion of the scapula, and vertical scapular osteotomy.^{[32, 33, 34, 35]} Clavicle resection and excision of the omovertebral bone have also been described. Many of these procedures leave unsightly scars; therefore, the cosmetic improvement needs to be considered carefully. The ability to increase shoulder abduction with surgery is also limited.

It is extremely important to explain the expected outcome of the surgery to the patient's parents and to ensure that they have realistic expectations of the surgery. Parents must be told that while cosmesis may be improved, the improvement in the patient's range of motion may be limited.

Before surgery, certain factors should be considered, including the cosmetic severity, the functional impairment, the age of the patient, other congenital anomalies, and the medical fitness of the patient to undergo the surgery. These factors are important because they ultimately determine the postoperative outcome.

Preoperatively, radiographs of both shoulders, including the cervical and thoracic spine, should be obtained to determine the presence of congenital scoliosis, Klippel-Feil syndrome, or an omovertebral bone. Furthermore, a CT scan or magnetic resonance image (MRI) may be useful to delineate the attachments of the omovertebral bone or to determine the presence of spina bifida occulta or an intraspinous lesion.

Although multiple surgical procedures are described in the literature, the Green and the Woodward procedures remain the criterion standards for correction of the Sprengel deformity. These 2 surgical procedures are described below.

Modified Green scapuloplasty

The modified Green scapuloplasty procedure usually is performed for a moderate or severe deformity. The patient is placed in the supine position on a radiolucent table. A supraclavicular incision is made 2 cm cephalad to the mid portion of the clavicle, in line with the skin creases. The deep fascia is incised, and the periosteum of the clavicle is divided longitudinally. The underlying subclavian vessels and brachial plexus must be protected carefully. The anterior cortex of the middle third of the clavicle is sectioned with an oscillating saw. Gentle force is then used to produce a greenstick fracture. The periosteum is then closed.

Next, the patient is turned to the prone position. A midline incision is made from C4 to T10. A plane is developed between the subcutaneous tissues and the fascia underlying the trapezius muscle. Dissection then proceeds laterally to expose the spine of the scapula. The insertion of the entire trapezius muscle onto the scapular spine is sectioned and tagged. The trapezius is reflected medially. Care must be taken to avoid injury to the spinal accessory nerve. The supraspinatus muscle is then detached extraperiosteally to the greater scapular notch. The transverse scapular artery and the suprascapular neurovascular bundle must be protected.

The omovertebral bar is then excised. The scapular attachment is sectioned first. The omovertebral bar is then gently detached from its insertion to the cervical spine. The insertions of the levator scapulae and rhomboid muscles are dissected extraperiosteally, divided, and tagged. Starting medially, the subscapularis muscle is elevated extraperiosteally. The suprascapular neurovascular bundle is protected, and the supraspinous portion of the scapula, along with its periosteum, is excised.

The scapular attachments of the latissimus dorsi muscle are divided extraperiosteally. Blunt dissection is used to create a large pocket in the superior part of the latissimus dorsi muscle. Fibrous bands may connect the scapula to the chest wall; these should be divided in order to mobilize the scapula. The scapula is then displaced distally. To prevent migration, the inferior pole of the scapula is fixed to the adjacent ribs. If winging is present, the scapula can be fixed to the rib cage in a lower and more laterally rotated position.

The muscles are reattached in the following order: supraspinatus muscle to the base of the scapular spine, subscapularis muscle to the vertebral border of the scapula, serratus anterior muscle to the vertebral border, levator scapulae muscles to the superior border, rhomboid muscles to the medial border, trapezius muscle to the scapular spine, and the superior edge of the latissimus dorsi muscle to the inferolateral edge of the trapezius. The wound is then closed in layers.

Woodward procedure

The Woodward procedure is also performed for a moderate or severe Sprengel deformity. The patient is placed in the prone position. A midline incision is made from C1 to T9. The wound is dissected laterally to the medial border of the scapula. The lateral border of the trapezius is identified. The lower portion of the trapezius is dissected from the latissimus dorsi muscle. The origin of the trapezius is detached from the scapular spine, and the edges are tagged. The origins of the rhomboid muscles are divided and tagged. The entire muscle sheet is retracted laterally, exposing the omovertebral bar, which is excised extraperiosteally. The levator scapula muscle is sectioned at its attachment to the scapula. Fibrous bands may attach the scapula to the chest wall; these should be sectioned. The serratus anterior muscle must be detached from its insertion in the vertebral border of the scapula. The supraspinatus and the subscapularis muscles are elevated extraperiosteally.

The supraspinous portion of the scapula is resected with its periosteum. Care is taken to avoid injury to the suprascapular nerve and vessels. The scapula is then lowered to the desired position. The subscapularis muscle is reattached to the vertebral border, and the supraspinatus is sutured to the scapular spine. The serratus anterior is reattached to the vertebral border, and the latissimus dorsi is reattached to the scapula. The trapezius and the rhomboid muscles are then resutured to the spinous processes at a more distal level. The wound is then closed in layers.

Postoperatively, the arm is supported with a sling (for 3 weeks), and gentle range of motion (active and passive) and strengthening exercises are gradually started. Physical therapy is continued for up to 6 months.

The patient is seen monthly for the first 3 months, every 3 months subsequently for the first year, and yearly thereafter. Scapular appearance, function, and motion, and general patient satisfaction are assessed at each visit. The appearance of the surgical scar, scapular symmetry, presence and degree of winging, scapular range of motion, muscle bulk, and strength are measured. Radiographs and clinical pictures are obtained for comparison.

Postoperative complications include the following:

• Winging of the scapula that may result from incomplete reattachment of the serratus anterior muscle
• Brachial plexus injury
• Keloid formation, which may complicate wound healing

Prognostic factors include the following:

• Severity of the deformity
• Age at surgery: Generally, results of surgery in children older than age 6 years are not as good.
• Type of procedure: Relocation surgeries have better functional outcomes.
• Associated anomalies: Anomalies such as Klippel-Feil syndrome compromise the eventual result.

Future

In a report by Mears, the author described a novel approach that included an oblique plane osteotomy of the scapular body, along with release of the long head of triceps from the scapula.^[36] He reported a significant improvement in function following this procedure.

Controversies

Surgical correction in older patients (>8 y) is controversial, and the results of surgery are not as good. However, in a study by Doita et al, the authors had good results after surgical correction in 2 adults, and they recommended surgery even in older patients.^[17]