Modified Woodward Repair of Sprengel Deformity

 

 

 

 

DEFINITION

Sprengel deformity is a congenital anomaly of the shoulder characterized by a high elevation of a hypoplastic scapula and medial rotation of its inferior pole.6, 7, 22 The exact cause of the deformity is unknown.

Associated anomalies include Klippel-Feil syndrome, rib deformities, omovertebral bone formation, muscleanomalies, clavicle hypoplasia, tracheoesophageal fistula, anal stenosis, kidney anomalies, diastematomyelia, and scoliosis.1, 2, 5, 14, 17, 25

Eulenberg6, 7 first described three cases of congenital “high dislocation of the scapula” in 1863, and in

1880, Willet and Walsham25 were the first to describe the omovertebral bone—a broad osseous band of bone connecting the scapula with the spinous process of C6.

 

 

ANATOMY

 

The normal scapula forms in the fifth week of fetal development adjacent to the level of C5 and then descends to the dorsal thoracic area at a level between T2 and T8.

 

The scapula in Sprengel deformity is abnormally high, has a decreased vertical diameter, and is deformed in shape.

 

 

 

The supraspinous region is rotated anteriorly in a convexity near the shape of the dorsal thorax. The inferior aspect of the scapula is rotated medially.

 

The scapula in Sprengel deformity may be attached to the lower cervical vertebrae (usually C6) by an abnormal band of tissue, which may be fibrous, cartilage, or bone (ie, omovertebral bone).25

 

The musculature of the shoulder girdle may be hypoplastic, absent, or weak.

 

 

The trapezius muscle, the levator scapulae muscle, and the rhomboid muscles often are hypoplastic.

 

The trapezius is the most commonly affected muscle. Other muscle groups that attach to the scapula occasionally are affected.

 

Associated bony congenital anomalies include Klippel-Feil syndrome, fused ribs, cervical ribs, congenital scoliosis, cervical spina bifida, hypoplastic clavicle, and short humerus.2, 10

PATHOGENESIS

 

The normal scapula develops in the cervical region and then descends to the upper posterior area of the thorax by the end of the third month of fetal development.

 

Sprengel deformity occurs as a result of interruption of the normal caudal migration of the scapula during fetal development.10

 

The etiology of Sprengel deformity is unknown, but the following theories have been proposed5, 21, 24:

 

 

Cerebrospinal fluid escapes through a “bleb” in the membrane of the roof of the fourth ventricle into the adjacent tissue of the neck to cause malformations.

 

 

Heredity (there have been several reports of familial occurrence) Increased intrauterine pressure

 

Abnormal articulation of the scapula to the cervical vertebrae and defective musculature formation

 

NATURAL HISTORY

 

The Sprengel deformity is present at birth, and the location of the scapula in relation to the neck and thorax remains constant as the child grows.

 

The abnormal scapula appears to grow proportionally to the growth of the child.

 

Associated congenital anomalies such as congenital scoliosis may progress, thereby changing the appearance of the deformity.

 

PATIENT HISTORY AND PHYSICAL FINDINGS

 

At birth, the shoulder with a Sprengel deformity appears to be displaced upward and forward.

 

 

In unilateral cases, shoulder asymmetry is evident.

 

 

The left scapula is involved more commonly than the right (FIG 1A).

 

In bilateral cases, both shoulders appear to be high, and the neck may appear thick and short.

 

The scapula may be tilted upward.

 

 

 

Motion of the shoulder is reduced in abduction and elevation (FIG 1B). Muscle weakness or hypoplasia can be observed in the shoulder area. Torticollis may be present.

 

Scoliosis and kyphosis as well as deformities of the chest from rib anomalies may be observed.

 

IMAGING AND OTHER DIAGNOSTIC STUDIES

 

Radiographs of the shoulder and neck show the bone deformities (FIG 2).

 

Sonography of the spinal cord is helpful in infants younger than about 4 months of age who have congenital spine anomalies.

 

 

Sonography can be performed through the cartilage of the lamina and spinous process, but after about 4 to 5 months of age, ossification blocks the views.

 

Congenital spine anomalies have a high association with intraspinal abnormalities.

 

 

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FIG 1 • A. Sprengel deformity of the right shoulder. B. Appearance of Sprengel deformity when the right arm is held in maximum abduction.

 

 

Sonography of the kidneys is helpful in cases associated with congenital spine anomalies.

 

Magnetic resonance imaging (MRI) is extremely helpful for evaluating muscle and soft tissue development.

 

Computed tomography (CT) (with three-dimensional [3-D] reconstruction) is helpful to define the extent of bone deformity. CT provides excellent visualization of the omoverte-bral structure.

 

Both still and video photography are helpful to record pre- and postoperative appearance and to document function.

 

DIFFERENTIAL DIAGNOSIS

No other abnormality resembles Sprengel deformity.

 

 

FIG 2 • Anteroposterior (AP) radiograph of the right shoulder of a child with Sprengel deformity.

 

 

 

NONOPERATIVE MANAGEMENT

 

In infants and young children, passive and active stretching exercises may be performed daily to maintain motion of the shoulder.

 

SURGICAL MANAGEMENT

 

Operative procedures are designed to improve the appearance of the elevated shoulder and, to a limited extent, improve its function.3, 8, 9, 11, 12, 13, 15, 18, 19, 20, 23

 

Operative treatment can be considered in cases in which the deformity is disfiguring and shoulder function is impaired.

 

In children with mild deformities in which the appearance of the shoulder is acceptable, operative treatment probably is not indicated.

 

The recommended age for surgery is 3 to 8 years.

 

Preoperative Planning

 

Preoperative evaluation of the appearance of the deformity with photographs is advised.

 

 

The author prefers full-profile photographs taken from the frontal, posterior, and both side views.

 

Motion can be documented by a series of photographs taken with the arms extended, elevated, and abducted.

 

Videos of the patient performing motion activities of the shoulder are helpful to determine the degree of deformity and whether or not the appearance is acceptable.

 

 

“Gait laboratory” analysis of the upper extremities is helpful; it quantifies kinetic and kinematic functions, electromyography, postural equilibrium, and energy expenditure.

 

The Cavendish grading scale is helpful in evaluating appearance4:

 

 

 

Grade I (very mild): Shoulder joints are level, and the deformity is not obvious when the patient is dressed. Grade II (mild): Shoulder joints are level, but the deformity is visible when the patient is dressed.

 

Grade III (moderate): The involved shoulder is elevated 2 to 5 cm, and the deformity is obvious.

 

Grade IV (severe): The involved shoulder is greatly elevated, and the superior angle of the scapula is near the occiput.

 

Preoperative evaluation of shoulder motion

 

 

Occupational therapy measurement of combined abduction of both shoulders (combined glenohumeral and scapulothoracic movement) as well as other shoulder motion is useful.

 

Shoulder functional testing also may be useful.

 

The author uses radiographs at the extremes of motion and gait laboratory analysis to verify the degree of measurements.

 

 

The anomalies of the shoulder, spine, and rib cage need to be evaluated radiographically. CT scanning and MRI are helpful to determine both bone and soft tissue abnormalities.

 

Currently, the author uses somatosensory evoked potentials and transcranial electrical motor evoked potentials to evaluate the brachial plexus nerve function during surgery.

 

 

Baseline values are obtained after the induction of anesthesia, and monitoring is continued during the procedure.

 

Positioning

 

The patient is placed in the prone position with the head positioned as if facing forward. (Patients with congenital cervical spinal anomalies may require individualized head positioning to prevent undue stress on the neck.)

 

 

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The entire arm, the shoulder, and the posterior thorax back area (ie, superiorly from the high cervical area, inferiorly to the lumbar area, and laterally to the contralateral scapular area) are prepared and draped.

 

 

The arm and scapular girdle are left free for manipulation during the operation.

 

Leads for the somatosensory evoked potentials and transcranial electrical motor evoked potentials are positioned on the skin and muscles in a sterile fashion.

 

Approach

 

The Woodward procedure consists of detaching the origins of the trapezius and rhomboid muscles from the spinous process and moving them downward after resection of the omovertebral bone and any fibrous bands from the scapula.21, 26

 

The procedure described by Green8 involves division of the muscles connecting the scapula to the trunk, excision of the omovertebral bone, excision of the supraspinous portion of the scapula, and reattachment of the muscles to hold the scapula reduced.

 

The modification described by the author2 is performed as originally described by Woodward,26 with the

addition of excision of the medial border of the scapula and resection of the supraspinous portion of the scapula.

 

 

The muscles attached on the medial and superior borders of the scapula are reflected extraperiosteally to facilitate bony resection.

 

Bone resection superiorly is medial to the suprascapular notch, and about 1 cm of the medial border of the scapula is excised.

 

The author does not usually recommend routine osteotomy of the clavicle, but it is indicated if neurologic issues arise during surgery. The procedure may be performed at the discretion of the surgeon to diminish the risk of neurologic problems.

 

TECHNIQUES

• Modified Woodward Procedure2

Incision and Dissection

 

A midline incision is made that extends from the spinous process of the C4 distally to the spinous process of T9 (TECH FIG 1A).

 

The skin and subcutaneous tissue are undermined on the involved side laterally to the medial border of the scapula and the lateral border of the trapezius muscle.

 

The trapezius muscle is bluntly dissected from the underlying latissimus dorsi muscle.

 

To achieve this, bluntly dissect the lateral border of the trapezius muscle in the inferior aspect of the operative area from the latissimus dorsi muscle.

 

Continue the dissection medially to the origin of the trapezius muscle at the spinous process of T9. The fibers of the trapezius muscle blend into the fibers of the other muscles that originate from the spinous processes.

 

Detach the trapezius muscle distally and proceed superiorly by detaching the remainder of the trapezius and then the rhomboid muscles to the level of the spinous process of C4 (TECH FIG 1B).

 

Retract the trapezius and rhomboid muscles laterally.

 

The levator scapulae muscle is identified as it originates from the superior medial aspect of the scapula and courses toward the spinous process of the cervical vertebra.

 

Occasionally, the muscles are fibrotic, which makes identification and dissection more difficult.

 

The omovertebral structure (which may be fibrotic, cartilage, or bone) is under the levator scapulae muscle.

 

The omovertebral structure is excised extraperiosteally by sharp dissection.

 

Any fibrotic bands in the area that may limit inferior mobility of the scapula are incised.

 

During the dissection, the spinal accessory nerve and the nerve to the rhomboids must be protected as they course beneath the trapezius muscle.

 

The spinal accessory nerve is in line with the vertebral border of the scapula.

 

In cases involving significant fibrosis of muscles, the nerves may be difficult to identify, and the use of spontaneous or electrical triggered electromyography may be helpful.

 

 

 

TECH FIG 1 • A. Location of the incision. B. Dissection of the trapezius and rhomboid muscles from the spinous processes of the vertebrae.

 

 

The levator scapulae muscle is divided at the superior medial corner of the scapula.

 

The transverse cervical artery, which is deep to the levator scapulae muscle, needs to be protected at the superomedial area of the scapula because bleeding occasionally can be problematic.

Scapular Resection and Reduction

 

Superiorly, the scapula is excised medially to the suprascapular notch, after which approximately 1 cm of the medial border of the scapular is excised (TECH FIG 2).

 

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TECH FIG 2 • Areas of resection of the scapula.

 

 

The scapula can be lifted, and any fibrotic bands between the undersurface of the scapula and chest wall are incised.

 

The scapula can now be drawn inferiorly and reduced to a more normal anatomic level.

 

Any fibrotic bands that prevent the reduction may be incised.

 

As the scapula is reduced, the somatosensory evoked potentials and the transcranial electrical motor evoked potentials may be used to verify the function of the nerves to the arm.

 

During reduction, the nerves of the brachial plexus may become entrapped between the clavicle and the chest wall.

 

If the evoked potentials become abnormal, the scapula is elevated to relieve tension on the

nerves, and a clavicular osteotomy16 is recommended to alleviate compression on the nerves that may have developed between an intact clavicle and the chest wall.

Clavicular Osteotomy16

 

A 2-cm incision is made over the middle clavicle area.

 

Beneath the platysma muscle, the periosteum is incised longitudinally, and the clavicle is exposed by subperiosteal elevation.

 

The author incises the mid-area of the clavicle with a rongeur to make chips of cortical bone over a length of approximately 1 cm.

 

 

The incised bone chips are used as graft in the osteotomy. The periosteum and operative wound are closed in layers.

 

The scapula is reduced, and the rhomboid muscles (and fascia) and the trapezius muscle are reattached in a more caudal position at the midline to the ligaments between the spinous processes.

The latissimus dorsi muscle can be lifted to allow the inferior wing of the scapula to be positioned beneath it.

The inferior tip of the scapula wing can be sutured to the latissimus dorsi muscle.

The operative wound is closed in layers, and wound suction drainage may be used at the surgeon's discretion.

 

 

Somatosensory evoked potentials and transcranial electrical ▪ May indicate brachial plexus

motor evoked potentials are helpful to monitor the compromise and the need for a neurologic status of the arm. clavicular osteotomy

Resection of the prominent superior and the medial border

of the scapula

• Offers the opportunity to improve

both appearance and functional outcomes

Osteotomy of the clavicle

• May prevent brachial plexus

palsy

PEARLS AND PITFALLS

 

 

POSTOPERATIVE CARE

 

Postoperatively, the arm is maintained in a Velpeau bandage for about 4 weeks.

 

Physical therapy is initiated after removal of the Velpeau bandage, with emphasis on glenohumeral motion and muscle strengthening.

 

OUTCOMES

Operative treatment of Sprengel deformity generally improves the appearance of the elevated shoulder and, to a limited extent, improve its function.3, 8, 9, 11, 12, 13, 15, 18, 19, 20, 23

In the author's cases,2 at an average of 8 years postoperatively, the glenohumeral/scapulothoracic motion was 150 degrees (range, 100 to 180 degrees). This represents 45-degree improvement from preoperative measurements. All children improve their appearance by at least on Cavendish grade and most returned to grade I or II. One out of 14 case was grade III and that child had multiple spinal

deformities and scoliosis adjacent to the Sprengel deformity.2

 

 

COMPLICATIONS

Brachial plexus palsy Nerve palsy

 

Persistent scapular winging Incomplete correction Vascular problems

Wound infection

Operative scar appearance

 

 

 

REFERENCES

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17. Schrock RD. Congenital abnormalities at the cervicothoracic level. Instr Course Lect 1949;6:228.

     

     

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19. Shea KG, Apel PJ, Showalter LD, et al. Somatosensory evoked potential monitoring of the brachial plexus during a Woodward procedure for correction of Sprengel's deformity. Muscle Nerve 2010;41:262-264.

     

     

20. Siu KK, Ko JY, Huang CC, et al. Woodward procedure improves shoulder function in Sprengel deformity. Chang Gung Med J 2011;34:403-409.

     

     

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22. Sprengel O. Die angeborene Verschiebung des Schulterblattes nach oben. Arch Klin Chir 1891;42:545-549.

     

     

23. Walstra FE, Alta TD, van der Eijke JW, et al. Long-term follow-up of Sprengel's deformity treated with the Woodward procedure. J Shoulder Elbow Surg 2013;22:752-759.

     

     

24. Weed LH. The Development of the Cerebro-spinal Spaces in Pig and Man. Washington, DC: Carnegie Institute of Washington, 1916.

     

     

25. Willet A, Walsham WJ. An account of the dissection of the parts removed after death from the body of a woman the subject of congenital malformation of the spinal column, bony thorax, and left scapular arch; with remarks on the probable nature of the defects in development producing the deformities. Med Chir Trans 1880;63:257-302.

     

     

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