P R O C E D U R E 1

Modified Woodward Procedure for Sprengel’s Deformity

Charles A. Goldfarb and Jakub S. Langer

P i t f a l l s

The physician should also evaluate for associated congenital anomalies such as fused ribs, cervical ribs, chest wall asymmetry, Klippel-Feil deformity, and scoliosis, which may affect the aesthetic outcome.
If the family is considering surgical intervention for aesthetic improvement, counsel the patient and family on the risk of a prominent scar.
Beware of severe shoulder girdle muscle atrophy or fibrosis, which will impair operative correction and affect functional improvement after surgery.
Patients over the age of 8 or 9 may have a less impressive aesthetic outcome and less improvement in shoulder range of motion.
Patients with scapular winging preoperatively may have worsened winging postoperatively. Therefore, some authors consider winging a relative contraindication to surgery.

Sprengel’s Deformity

INDICATIONS

Operative intervention is considered in children with functional limitations, including those with decreased shoulder forward flexion and/or abduction. Children with a range of motion of less than 120° in either plane have the greatest potential for improvement after surgery.
Operative intervention is also considered for aesthetic reasons due to the fact that the undescended scapula is quite noticeable.
The Cavendish classification of Sprengel’s deformity (Cavendish, 1972) may be helpful in assessing the need for operative intervention based on appearance. A limited intervention such as excision of superomedial scapular prominence may be utilized for grades 1 and 2 whereas a more significant procedure to relocate the scapula, such as the Woodward procedure, may be more appropriate for grades 3 and 4.
- Grade 1 (very mild): shoulder joints level and deformity invisible when the patient is dressed
- Grade 2 (mild): shoulder joints level but deformity visible even when patient is dressed (as a prominence in the neck web)
- Grade 3 (moderate): shoulder joint elevated 2–5 cm with deformity easily visible
- Grade 4 (severe): shoulder greater than 5 cm elevated with scapula near occiput
  
  Controversies
  - Cosmesis versus function: While the Woodward procedure offers potential for both functional and aesthetic improvement, those patients with functional limitations have the greatest potential for improvement.
  - Other procedures, such as a resection of the superior-medial angle of the scapula, are less invasive and nearly as effective as the Woodward procedure for aesthetic improvement for lesser deformities.
  - Clavicular osteotomy: A minimally invasive osteotomy of the clavicle may be performed to minimize tethering (and thus injury) to the brachial plexus as the scapula is mobilized inferiorly.
Age of intervention: The trend in treatment is for earlier intervention as it offers a better chance at functional improvement. Intervention between 3 and 6 years of age is favored, while others advocate intervention as early as 6–9 months. Nonetheless, some surgeons still offer the procedure in later childhood (5–9 years) or adolescence.

EXAMINATION/IMAGING
The general appearance of the patient is assessed, with specific attention to the symmetry of the shoulders. The patient is evaluated with the arms at the sides and in various positions of function. When both scapulae are undescended, there is less asymmetry but still a noticeable abnormality. Figure 1 demonstrates the typical appearance of a right-sided unilateral deformity as seen in the anterior clinical view of a 3-year-old patient.
Shoulder range of motion is measured, with particular attention to active forward flexion and abduction. Figure 2 shows posterior clinical views of a 4-year-old patient with deficient right-sided abduction secondary to Sprengel’s deformity preoperatively (Fig. 2A) and postoperatively (Fig. 2B). Passive glenohumeral abduction and rotation are typically normal, whereas scapulothoracic motion is most severely affected. Additionally, upper extremity strength may be decreased.
An anteroposterior radiograph to include both shoulders and centered on the clavicles is recommended to allow side-to-side comparison of the bony anatomy. The bilateral shoulder radiograph in Figure 3 demonstrates a typical hypoplastic, undescended, and rotated right scapula in the 3-year-old patient with right-sided deformity seen in Figure 1.
Computed tomography of both shoulders, including a three dimensional assessment, may be considered as it will provide a more detailed understanding of the anatomy.

The physician should clinically evaluate for an accessory omovertebral bone connecting the superomedial aspect of the scapula to the mid- to lower cervical spinous process (Fig. 4); oblique shoulder radiographs and/or computed tomography scan may also be obtained.

Sprengel’s Deformity

FIGURE 1

FIGURE 2 A B

Cervical spine

Omovertebral bone

High scapula

FIGURE 3 FIGURE 4

Treatment Options

Sprengel’s Deformity

Minimal deformity (Cavendish grade 1) may be treated with conservative measures, including therapy focusing on range of motion, stretching, and strengthening.
Patients with a lesser degree of deformity (Cavendish grade 2) may be treated with resection of the prominent superior-medial scapular angle without muscle detachment and without scapular relocation.
The modified Green procedure involves detachment of all scapular stabilizers at their insertion on the scapula and creation of an inferior pocket in the latissimus to place the scapula in a more inferior, anatomic position.
An alternative option is a vertical scapular osteotomy with resection of the superior-medial angle and distal translation of the lateral scapula (modified Konig procedure).
The patient is evaluated for the most common associated abnormalities, including cervical vertebral deformities, such as Klippel-Feil syndrome (30%), congenital scoliosis (25%), chest wall defects/hypoplasia (25%), and renal/genitourinary disorders (10%). Other associated anomalies include diastematomyelia, long bone deficits, and ray abnormalities of the hand or foot. Renal ultrasound and cervical spine computed tomography or magnetic resonance imaging should be considered (Ross and Cruess, 1977).

Consultations from spine and/or plastic surgeons are obtained prior to intervention to allow treatment of associated spine or chest wall deformities as needed.

SURGICAL ANATOMY

The trapezius, one of the primary muscles released during the Woodward procedure, has a very broad origin from the occiput to the T12 spinous processes (Fig. 5).
Thirty percent of patients will have an osseous or cartilaginous omovertebral bone linking the superior-medial angle of the scapula to the cervical spine (see Fig. 4). This is resected to allow for correction.
Neurovascular structures to be protected:

The spinal accessory nerve provides innervation to the trapezius muscle and courses longitudinally along its anterior surface with the descending branch of the superficial cervical artery (deep surface when approached from posterior).

P e a r l s

Include the contralateral side in the anterior and, especially, the posterior operative fields, for ease of comparison and palpation.
A radiolucent operating table will allow, if necessary, intraoperative fluoroscopy to assure satisfactory mobilization of the scapula.
Branches of the dorsal scapular nerve innervate the rhomboid muscles.
The transverse cervical artery lies just anterior to the insertion of the levator scapulae and is at risk while releasing this muscle from the superomedial aspect of the scapula (Fig. 6).
Lateral dissection at the proximal scapula should be limited to avoid injury to the suprascapular neurovascular structures, located at the suprascapular notch.

The subclavian vessels are at risk during the clavicular osteotomy; curved Hohmann retractors placed subperiosteally will provide protection.

POSITIONING

Controversies

Some authors advocate semilateral or “floppy lateral” positioning to allow for access to the clavicle and posterior structures in one operative field, eliminating the need for redraping. We find this makes the posterior procedure awkward and prefer to simply redrape.
Initially, for the clavicular osteotomy, the patient is placed in the supine or beach chair position with a bump in the midline between the scapulae. A wide sterile field is created to include the entire upper extremity (to allow control of the clavicle) and the contralateral medial clavicle and sternum (Fig. 7).
Following clavicular osteotomy and closure of the wound, a sterile dressing is applied and the patient is placed prone.
- The head and neck are carefully supported with the head in a neutral to slightly flexed position. Longitudinal bolsters avoid undue pressure on the abdomen.

Again, the entire arm and shoulder girdle past the midline, including the opposite scapula, are prepped into the field to allow for adequate mobilization of the scapula and easy visualization and palpation of contralateral structures (Fig. 8).

Trapezius muscle

T12

Levator Transverse scapulae cervical muscle artery

Trapezius muscle reflected

Rhomboideus major and minor reflected

Sprengel’s Deformity

FIGURE 5

FIGURE 6

FIGURE 7 FIGURE 8

P e a r l s

Controversies

Sprengel’s Deformity

PORTALS/EXPOSURES

Use blunt subperiosteal dissection for the clavicular osteotomy to protect the underlying neurovascular structures.
If bilateral scapulae are affected, or if an approach to the cervical or thoracic spine will be necessary, a midline posterior incision may be utilized.
Some authors do not routinely perform a clavicular osteotomy, citing good results without this step especially in younger patients and those with a lesser degree of deformity. This is further discussed below.
Anterior approach
- A minimal, 2-cm incision is made over the midportion of the clavicle, with dissection carried to the periosteum (Fig. 9A). The superficial sensory nerves are protected and the platysma is detached as necessary.
- The periosteum is incised in line with the clavicle and blunt, circumferential subperiosteal elevation is performed.
- Subperiosteal mini-Hohmann retractors protect the subclavian vein and artery beneath the clavicle (Fig. 9B).
Posterior approach
- A 10- to 15-cm incision slightly lateral to midline is used from the level of the C5 spinous process to the T8 spinous process (Fig. 10).

Sharp dissection is carried out through the skin and subcutaneous tissue to the level of the dorsal fascia. The overlying tissues are elevated laterally to create a flap allowing visualization of the scapula and supporting musculature (see Fig. 10).

Sprengel’s Deformity

FIGURE 9

Incision

Trapezius muscle

FIGURE 10

P i t f a l l s

Controversies

Sprengel’s Deformity

PROCEDURE

steP 1

The subclavian vessels are directly deep to the clavicle and should be carefully protected with curved Hohmann retractors.
Some authors recommend clavicular osteotomy only in older patients (>6 years) or in cases of severe deformity, citing low risk of brachial plexus palsy and adequate correction without this osteotomy.
In adolescents, it has been described that the coracoid should also be osteotomized to prevent compression of neurovascular structures against a rib.
We routinely perform a clavicular osteotomy to minimize the risk of a brachial plexus traction injury that may result from mobilization of the scapula.
A bone biter or rongeur is used to create an osteotomy through the midaspect of the clavicle (Fig. 11).
A simple osteotomy or morcellization of a small segment of the clavicle is performed.
The wound is irrigated and the skin is closed with subcutaneous 4-0 or 5-0 absorbable sutures and a 5-0 running, absorbable subcuticular stitch.
The wound is dressed, and patient is turned prone for posterior

exposure.

steP 2
The lateral aspect of the distal trapezius is identified and dissected free from the underlying latissimus dorsi and paraspinal musculature (Fig. 12). This step helps identify the key surgical plane. Adhesions or hypoplastic musculature can make identification of this surgical plane difficult.

P e a r l s
- Preserve tendinous muscle origins during dissection to aid in stabilization of the reduced scapula.
- Protect the spinal accessory nerve and descending branch of the superficial cervical artery running longitudinally on the deep surface of the trapezius.
- Tagging sutures are placed at regular intervals to assist with mobilization of the muscle.
- During the release of the superomedial attachment of the levator scapulae onto the scapula, placing a finger behind the attachment will protect the transverse cervical artery (see Fig. 14).
The trapezius is sharply dissected from the thoracic and cervical spinous processes from caudad to cephalad. As the dissection progresses proximally, the origins of the rhomboid major and minor are reflected as well. Figure 13 shows the trapezius and rhomboids detached at their origins and elevated, with the levator scapulae exposed at the superomedial aspect of the scapula. The tendinous insertion of these muscles is preserved to allow later, more caudal, reattachment. At the level of the C4 spinous process, the trapezius muscle is divided transversely to allow adequate release.
In the proximal aspect of the incision, the levator scapulae and, in 30% of cases, the omovertebral bone are evident. The spinal accessory nerve innervating the trapezius and branches of the dorsal scapular nerve innervating the rhomboids are carefully preserved.
The surgeon must avoid injuring the descending branch of the superficial cervical artery or the transverse cervical artery.
The levator scapulae is released at its superomedial insertion on the scapula. The transverse cervical artery travels directly beneath the levator scapulae and is protected during the release (Fig. 14).
The omovertebral bone or fibrous bands in its location connecting the scapula to the cervical spine must be released with heavy scissors or bone biters. The omovertebral bone is removed extraperiosteally.
Finally, the undersurface of the scapula is swept bluntly free of the underlying chest wall and any remaining fibrous adhesions

are released.

Sprengel’s Deformity

11

FIGURE 11

Lateral inferior

border of trapezius muscle

FIGURE 12

Levator scapulae muscle

Levator scapulae muscle

Rhomboid muscles

Trapezius muscle

FIGURE 14

FIGURE 13

12

P e a r l s

Controversies

Sprengel’s Deformity

steP 3

Bone wax may be placed at the site of the bone resection to minimize bleeding.

P i t f a l l s

Periosteal stripping may result in bone growth and deformity recurrence.
Some authors advocate greenstick fracture transversely through the superomedial portion of the scapula rather than excision for correction of its prominent curvature.
The prominent superomedial aspect of the scapula is resected using bone biters in a medial to lateral direction (Fig. 15). The suprascapular neurovascular structures are protected.
This resection is performed extraperiosteally to minimize bone regrowth.

steP 4
The scapula should be fully mobile and is reduced to a more caudal position.
- The spine of the scapula is placed at the level of the contralateral scapular spine.
- The previously elevated aponeurosis of trapezius and rhomboid muscles is sutured in place with multiple heavy nonabsorbable sutures in a more caudal position (Fig. 16). The redundant distal trapezius may be folded over or trimmed.

A pocket may be created in the latisimuss dorsi muscle to accommodate the lowered inferior angle of the scapula.

P e a r l s

Controversies

Sprengel’s Deformity

Superomedial angle of scapula

Levator scapulae muscle

FIGURE 15

Latissimus dorsi muscle

Trapezius muscle

FIGURE 16

P i T f a L L s

Sprengel’s Deformity

sTEP 5: CLOsURE

Use the scapular spines as landmarks to assess for adequacy of reduction.
Inferior mobilization of the scapula may be limited by the proximal, supraspinous aspect of the scapula if it is curved anteriorly over the chest wall. This curvature is resected (with care given to protect the neurovascular structures) to allow mobilization of the scapula.

P i t f a l l s
The scapula is hypoplastic, and aligning its inferior angle with the inferior angle of the contralateral normal scapula may result in an overcorrec-tion, which increases the risk of a traction brachial plexopathy.
Some authors recommend release of the subscapularis and serratus anterior to allow mobilization of the scapula caudally. If released, those muscles may be repaired after the scapula is stabilized in the inferior position. We have not found this step to be necessary.
When nylon or wire suture is used for closure, a disfiguring scar may result in up to 60% of cases.
The wound is closed with a 4-0 absorbable subcutaneous and a 5-0 absorbable subcuticular suture.
Bleeding is typically minimal, and a drain is rarely required.

Figure 17 shows the aesthetic (Fig. 17A) and functional (Fig. 17B) outcome from the modified Woodward procedure.

POSTOPERATIVE CARE AND EXPECTED OUTCOMES

P E a R L s

A simple sling will suffice for the older child while a sling and swathe are helpful for the younger patient.

P i T f a L L s

Selection of patients with coexisting significant spinal or chest deformity may lead to poor aesthetic outcome because of residual deformity after scapular correction.
Postoperatively, the patient is placed in a prefitted sling and swath. The patient remains in the hospital for 1–2 days for pain control. Immediate gentle neck motion, symmetric posture training, isometric scapular depression, and elbow, wrist, and hand motion exercises are initiated as indicated based on patient age.
At 3 weeks postoperatively, a home therapy program is advanced to include Codman exercises and passive and active-assistive shoulder motion to 90° while stabilizing the clavicle.
At 5 weeks postoperatively, radiographs are taken and the patient is weaned of the sling and swath. Active and passive range of motion beyond 90° is begun and scapular depression exercises are emphasized.
At 3 months postoperatively, a more rigorous strengthening protocol is initiated.
Complications include disfiguring scar, transient brachial plexus palsy, recurrence of deformity secondary to bone regeneration or cephalad migration of the scapula, and inability to improve deformity because of other factors (scoliosis, cervical spine abnormality).

Sprengel’s Deformity

FIGURE 17

Sprengel’s Deformity

EVIDENCE

Borges JLP, Shah A, Torres BC, Bowen JR. Modified Woodward procedure for Sprengel deformity of the shoulder: long-term results. J Pediatr Orthop. 1996;16:508–13.

In this retrospective review over 3 to 15 years of follow-up, 15 patients had an 86% satisfaction rate and 14 of 15 had markedly improved appearance as per the Cavendish grading system. Average abduction improved by 35° and scapular lowering by 2.7 cm. The authors advocated removal of the medial border of the scapular, hypothesizing that it may prevent postoperative winging. One case of brachial plexus palsy was reported, which resolved after clavicular osteotomy. (Level IV evidence)

Carson WG, Lovell WW, Hitesides TE. Congenital elevation of the scapula: surgical correction by the Woodward procedure. J Bone Joint Surg [Am]. 1981;63:1199–1207.

This 11-patient case series of the Woodward procedure with clavicular osteotomy demonstrated 9 of 11 patients were satisfied with their outcome, with one of the unsatisfied patients having an unsightly scar and the other scapular winging. Better outcomes were achieved in patients with more severe limitations in preoperative range of motion. (Level IV evidence)

Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg [Br]. 1972;54:395–408.

This comprehensive review of 100 cases of Sprengel’s deformity focused on description of surgical techniques and a classification system aimed at tailoring an appropriate surgical approach based on degree of deformity. (Level IV evidence)

Greitemann B, Rondhuis J, Karbowski A. Treatment of congenital elevation of the scapula: 10 (2–18) year follow-up of 37 cases of Sprengel’s deformity. Acta Orthop Scand. 1993;64:365–8.

In this retrospective analysis of 23 operative procedures, including 6 Woodward procedures without clavicular osteotomy, the latter yielded the best results and satisfaction rates secondary to improved scar appearance and larger scapular mobilization. There was one transient brachial plexus palsy. (Level IV evidence)

Grogan DP, Stanley EA, Bobechko WP. The congenital undescended scapula: surgical correction by the Woodward procedure. J Bone Joint Surg [Br]. 1983;65:598–605.

This retrospective review of 21 Woodward procedures without clavicular osteotomy yielded 80% good to excellent results with average follow-up of nearly 9 years. One patient had transient brachial plexus palsy and one had worsening of preoperative scapular winging. One patient had recurrence of resected superomedial scapula. The authors indicated a trend toward improved scar appearance with a subcuticular stitch. (Level IV evidence)

Ross DM, Cruess RL. The surgical correction of congenital elevation of the scapula: a review of seventy-seven cases. Clin Orthop Relat Res. 1977;(125):17–23.

The authors presented a comprehensive review of all operatively treated Sprengel’s deformity cases at U.S. Shriners Hospitals between 1935 and 1970. The review included 17 Woodward procedures, 3 of which led to recurrence or scapular winging, and 1 case with transient brachial plexus palsy. (Level IV evidence)

Woodward JW. Congenital elevation of the scapula: correction by release and transplantation of muscle origins, a preliminary report. J Bone Joint Surg [Am]. 1961; 43:219–28.

In his original paper, Woodward described his technique, which did not include clavicular osteotomy, and his experience with nine patients. One patient experienced transient brachial plexus palsy and three patients had unsightly scars. (Level IV evidence)