Approach Considerations
Indications for surgical intervention of a Sprengel deformity (congenital elevation of the scapula) include significant cosmetic concerns and significant restriction of shoulder abduction in children younger than age 6 years.
The presence of a mild deformity with minimal restriction of movement is a contraindication for surgery. Although treatment of the Sprengel deformity is essentially surgical, some factors exist that could compromise the results of surgery and thus may be considered contraindications. These factors include the presence of associated syndromes that affect the final functional outcome.
Mears described a novel approach that included an oblique plane osteotomy of the scapular body, along with release of the long head of the triceps from the scapula. [38] He reported a significant improvement in function following this procedure.
Surgical correction in older patients (>8 years) 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 two adults, and they recommended surgery even in older patients. [15]
Nonoperative Therapy
Nonoperative treatment for the Sprengel deformity consists of physical therapy. Exercises are used to maintain an individual's range of motion (ROM) and to strengthen the weak periscapular muscles.
Surgical Therapy
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 that surgery be performed when patients are younger than 8 years in order to obtain the best surgical result.
Surgical options
Surgical options include the following [39, 40, 41, 42] :
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Subperiosteal resection of part of the scapula
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Extraperiosteal release
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Transplantation of the muscular origins of the scapula
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Excision of the superomedial portion of the scapula
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Vertical scapular osteotomy
Clavicle resection and excision of the omovertebral bone have also been described. Many of these procedures leave unsightly scars; therefore, the cosmetic improvement must be considered carefully. The ability to increase shoulder abduction with surgery is also limited.
Preparation for surgery
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 for surgical treatment. Parents must be told that whereas cosmesis may be improved, the improvement in the patient's ROM 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, computed tomography (CT) or magnetic resonance imaging (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.
Operative details
Although multiple surgical procedures are described in the literature, the Green scapuloplasty and the Woodward procedure remain the criterion standards for correction of the Sprengel deformity.
Modified Green scapuloplasty
The modified Green scapuloplasty usually is performed for a moderate or severe deformity. [43, 44, 45]
The patient is placed in the supine position on a radiolucent table. A supraclavicular incision is made 2 cm cephalad to the midportion 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. Dissection proceeds laterally to expose the spine of the scapula. The insertion of the entire trapezius onto the scapular spine is sectioned and tagged. The trapezius is reflected medially. Care must be taken not to injure the spinal accessory nerve. The supraspinatus 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 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 are divided extraperiosteally. Blunt dissection is used to create a large pocket in the superior part of the latissimus dorsi. 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:
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Supraspinatus to the base of the scapular spine
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Subscapularis to the vertebral border of the scapula
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Serratus anterior to the vertebral border
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Levator scapulae to the superior border
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Rhomboid muscles to the medial border
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Trapezius to the scapular spine
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Superior edge of the latissimus dorsi 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. [46, 47, 48]
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. 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 scapulae 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 must be detached from its insertion in the vertebral border of the scapula. The supraspinatus and the subscapularis 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 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.
Modifications of this procedure have been developed. [49, 50] The Woodward procedure has also been performed endoscopically. [51]
Postoperative Care
Postoperatively, the arm is supported with a sling (for 3 weeks), and gentle ROM (active and passive) and strengthening exercises are gradually started. Physical therapy is continued for up to 6 months.
Complications
Postoperative complications include the following:
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Winging of the scapula that may result from incomplete reattachment of the serratus anterior
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Brachial plexus injury - The risk may be reduced by performing intraoperative neurophysiologic monitoring of the brachial plexus [52, 53]
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Keloid formation, which may complicate wound healing
Long-Term Monitoring
The patient is seen monthly for the first 3 months, every 3 months subsequently for the remainder of 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 ROM, muscle bulk, and strength are measured. Radiographs and clinical pictures are obtained for comparison.
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Clinical photograph of a child with Sprengel deformity and Klippel-Feil syndrome.
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Posteroanterior chest radiograph. This image depicts bilateral Sprengel deformities.
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Photo illustrating the Leibovic method for determining scapular rotation and position. ISA = inferior scapular angle; Line 1 = line drawn from the midpoint of the acromioclavicular joint to the midpoint of the sternoclavicular joint; Line 2 = line drawn from the midpoint of the acromioclavicular joint to the ISA; Line 3 = vertical line drawn along the spinous processes of the vertebrae; SSA = superior scapular angle.
