Orthopedics

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PEDIATRIC ORTHOPEDICS: Congenital Elevation of the Scapula (Sprengel's Deformity)

Robert D Galpin, MD; John G Birch, MD

Abstract

History

A 6- year-old girl presented for evaluation of shoulder asymmetry and possible scoliosis. This had been noted several years earlier but no treatment had been sought. She was completely asymptomatic. She was the product of an uncomplicated, term pregnancy. At birth however, she was noted to have a left congenital diaphragmatic hernia necessitating a left thoracotomy at eight hours of age. Her subsequent development had been normal, with no significant medical or surgical illnesses.

Physical examination revealed an obvious asymmetry of the shoulder girdles with superior migration of the left scapula (Fig. 1, 2). There was a limitation of left shoulder abduction (combined glenohumeral and scapulothoracic) to 120° compared to 180° at the right shoulder. A firm, nontender mass was easily palpable at the superomedial corner of the left scapula. A minimal rotational deformity was seen in the upper thoracic spine. The previous left thoracotomy scar was well healed. The remainder of the physical and neurological examination was unremarkable.

Roentgenograms revealed an elevated position of the left scapula articulating with an omovertebral bone. Multiple anomalies of the lower cervical and upper thoracic vertebrae were noted, including spina bifida occulta of C3 and C5, congenital fusion of C5-6 (Klippel-Feil), with an associated minimal right convex scoliosis in the upper thoracic spine. There were multiple rib anomalies on the left side as well (Fig. 3).

The diagnosis of congenital elevation of the scapula (Sprengel's deformity) was made. Because of the congenital anomalies of the vertebrae an intravenous pyelogram was performed which was normal. Because of the significant cosmetic deformity, surgical correction was performed using the modified Woodward procedure. The patient was placed initially in the right lateral decubitus position with the entire left arm and shoulder girdle included in the operative field. A subperiosteal exposure of the clavicle was developed through a transverse incision above the middle third. A segment measuring 1.5 cm was removed at the midpoint of the clavicle, morselized and then replaced in the periosteal tube which was carefully repaired and the skin was closed. The patient was then rolled forward into the prone position without changing the draping.

A midline posterior incision was made, extending from the spinous process of C3 to T12. Extreme care was necessary in the cervical and upper thoracic exposure due to the presence of several bifid spinous processes. Ine omovertebral bone was palpated from the upper medial border of the scapula to the spinous processes and lamina from C3 to C5. This structure was extraperiosteally dissected from the surrounding soft tissues. The articulation at the superomedial corner of the scapula was then opened and the omovertebral bone was removed.

The trapezius and rhomboid muscular origins were then mobilized as a continuous sheet from the midline spinous processes. The trapezius was transected in the upper cervical region and the levator scapulae was released from the upper medial corner of the scapula. The medial edge of the scapula could then be elevated to free any adhesions between the scapula and the ribs. The superomedial corner of the scapula was further exposed extraperiosteally and the anteriorly-curving, supraspinous portion was resected.

Wilkinson performed a vertical osteotomy of the scapula 1 cm lateral to the vertebral border. 10 After excision of the omovertebral bone the lateral portion of the scapula is rotated and displaced caudally in relation to the vertebral border which remains unchanged in position. This effectively removes the fullness in the web of the neck and changes the direction of the glenoid fossa which improves shoulder range of motion.

Woodward described his procedure with a preliminary report of nine cases in 1961. u This technique…

History

A 6- year-old girl presented for evaluation of shoulder asymmetry and possible scoliosis. This had been noted several years earlier but no treatment had been sought. She was completely asymptomatic. She was the product of an uncomplicated, term pregnancy. At birth however, she was noted to have a left congenital diaphragmatic hernia necessitating a left thoracotomy at eight hours of age. Her subsequent development had been normal, with no significant medical or surgical illnesses.

Physical examination revealed an obvious asymmetry of the shoulder girdles with superior migration of the left scapula (Fig. 1, 2). There was a limitation of left shoulder abduction (combined glenohumeral and scapulothoracic) to 120° compared to 180° at the right shoulder. A firm, nontender mass was easily palpable at the superomedial corner of the left scapula. A minimal rotational deformity was seen in the upper thoracic spine. The previous left thoracotomy scar was well healed. The remainder of the physical and neurological examination was unremarkable.

Roentgenograms revealed an elevated position of the left scapula articulating with an omovertebral bone. Multiple anomalies of the lower cervical and upper thoracic vertebrae were noted, including spina bifida occulta of C3 and C5, congenital fusion of C5-6 (Klippel-Feil), with an associated minimal right convex scoliosis in the upper thoracic spine. There were multiple rib anomalies on the left side as well (Fig. 3).

The diagnosis of congenital elevation of the scapula (Sprengel's deformity) was made. Because of the congenital anomalies of the vertebrae an intravenous pyelogram was performed which was normal. Because of the significant cosmetic deformity, surgical correction was performed using the modified Woodward procedure. The patient was placed initially in the right lateral decubitus position with the entire left arm and shoulder girdle included in the operative field. A subperiosteal exposure of the clavicle was developed through a transverse incision above the middle third. A segment measuring 1.5 cm was removed at the midpoint of the clavicle, morselized and then replaced in the periosteal tube which was carefully repaired and the skin was closed. The patient was then rolled forward into the prone position without changing the draping.

A midline posterior incision was made, extending from the spinous process of C3 to T12. Extreme care was necessary in the cervical and upper thoracic exposure due to the presence of several bifid spinous processes. Ine omovertebral bone was palpated from the upper medial border of the scapula to the spinous processes and lamina from C3 to C5. This structure was extraperiosteally dissected from the surrounding soft tissues. The articulation at the superomedial corner of the scapula was then opened and the omovertebral bone was removed.

The trapezius and rhomboid muscular origins were then mobilized as a continuous sheet from the midline spinous processes. The trapezius was transected in the upper cervical region and the levator scapulae was released from the upper medial corner of the scapula. The medial edge of the scapula could then be elevated to free any adhesions between the scapula and the ribs. The superomedial corner of the scapula was further exposed extraperiosteally and the anteriorly-curving, supraspinous portion was resected.

Fig. 1: Anterior view at presentation, age 6.

Fig. 1: Anterior view at presentation, age 6.

Fig. 2: Posterior view at presentation.

Fig. 2: Posterior view at presentation.

With the scapula now fully mobile it was moved distally on the chest wall and anchored in its new position by caudal advancement and suture of the muscular sheet to the contralateral deep fascia and spinous processes. Any redundant musculofascial sheet was trimmed and the wounds closed. An early postoperative x-ray demonstrated good correction and healing of the clavicular osteotomy (Fig. 4).

Postoperative care consisted of a sling for 2 weeks followed by gentle active assisted range of motion exercises, progressing to active and eventually resisted exercises by 6 weeks after surgery. Six weeks postoperative this patient had improved motion at the left shoulder and had returned to full activities. At the 4 year follow up her cosmetic result was excellent. She had completely normal function and full abduction at the left shoulder (Figs. 5, 6, 7).

Discussion

The condition of congenital elevation of the scapula was first described by Eulenberg in 1863, ' but has carried its commonly-recognized eponym since Sprengel reported on a series of four patients with this condition in 1891. 2 Since that time a number of methods of correction have appeared in the orthopedic literature.

Fig. 3: Preoperative x-ray.

Fig. 3: Preoperative x-ray.

Fig. 4: X-ray, 6 weeks postoperative.

Fig. 4: X-ray, 6 weeks postoperative.

Sprengel's deformity is felt to be an arrest of the caudal descent of the scapula which normally occurs between the ninth and twelfth weeks gestation.3 Heredity may be a factor in at least some cases as suggested by Engel.4

Several authors have suggested Sprengel's deformity is not just a scapular abnormality but rather a developmental anomaly of the entire shoulder girdle. This is evidenced by the association with other congenital anomalies.4·5 Most frequently there is abnormal development of one or more ribs, vertebral bodies in the cervical and upper thoracic region (hemivertebra, unilateral bars), and hypoplasia of some or all of the trapezius, rhomboids, levator scapulae, and pectoralis major muscle bellies. Other associated abnormalities are Klippel-Feil syndrome, shortness of the humerus, and malformation of the clavicle. The classic omovertebral bone is an abnormal connection between the superomedial comer of the scapula and the lower cervical spine and is found in 30% of reported cases. The spinal connection may be to the transverse process, lamina or spinous process. Laterally, the connection to the scapula may be fibrous or bony.

Clinically this deformity presents with the asymmetry in the neck region. The elevated location of the scapula combined with the omovertebral extension create a fullness at the base of the neck with loss of the normal contour. The hypoplastic scapula tethered at its superomedial corner is rotated such that the inferior pole is medially displaced toward the midline, which directs the glenoid fossa and shoulder joint inferiorly. Scapulothoracic motion is restricted by this tethering effect, leading to a reduction of total abduction of the shoulder. The amount of abduction at the involved shoulder varies from 60° in severe cases to almost normal range. Flexion, rotation and passive glenohumeral abduction are normal.

The indications for treatment of Sprengel's deformity include a limitation of mobility at the shoulder such that function is impaired, and significant cosmetic deformity. Motion is limited in the majority of patients with Sprengel's deformity but frequently this is not functionally significant. The cosmetic aspects of this deformity may be quite marked with webbing of the neck on the involved side and often an unsightly appearance. This is often the reason medical attention is sought by the parents.

The treatment of congenital elevation of the scapula, when necessary, is surgical. As with other structural deformities, conservative measures of treatment are of little benefit. There are two general types of operations described for correction of this condition, including 1) procedures which release the tether of the scapula, including the anteriorly-curved supraspinous portion of the scapula and the omovertebral bone6·7; and 2) procedures designed to reposition the scapula to a more normal position on the chest wall, combined with resection of the tether.811

The first group of procedures removes the prominent structures superiorly, thereby decreasing the fullness in the web of the neck and improving appearance. Mobility is usually increased due to release of the tethering effect at the superomedial corner of the scapula. The amount of improvement of abduction is often less than with the repositioning operations which result in more complete mobilization of the bone.12 There is also a higher rate of recurrence with resection alone.12

The second group of procedures release the tethering effect at the superomedial corner of the scapula as well as mobilize the bone, moving it caudally to a more normal position, and then fixing it there by reattachment of muscles or fascia. Schrock presented the first corrective procedure for this condition to the American Orthopaedic Association in 1928, and reported his technique of near complete subperiosteal release of the scapula in 1949. 3 Essentially all muscles which have attachment to the scapula were elevated. The omovertebral bone and much of the supraspinous portion of the scapula were then excised. If mobility was not adequate to relocate the scapula he then recommended osteotomizing the base of the acromion. The scapula was then relocated and sutured at its inferior pole to the "lowest rib possible." Adhesive strapping was then used to support the scapula in the early phase of healing. Schrock mentioned several complications including four cases of postoperative brachial plexus palsy, two cases of scapular winging, and "several cases" of recurrent bone formation noted on follow up radiographs but in no case was this latter finding clinically significant.

Fig. 5: Anterior view 4 years postoperative.

Fig. 5: Anterior view 4 years postoperative.

Fig. 6: Posterior view 4 years postoperative.

Fig. 6: Posterior view 4 years postoperative.

Green described a method of correction of this deformity consisting of extraperiosteal release of the muscles from the vertebral border of the scapula and excision of the omovertebral bone and supraclavicular portion of the bone.8 Ine scapula was then displaced caudally and the muscles sutured in the new position. To support the repair a piano wire connected the inferior pole of the scapula to a body cast with 3 lb traction through a spring. This was continued for 3 weeks. Green reported 16 cases with excellent results in terms of function and cosmesis. The major disadvantage was the postoperative routine with percutaneous traction and body jacket.

Fig. 7: X-ray, 4 years postoperative.

Fig. 7: X-ray, 4 years postoperative.

Wilkinson performed a vertical osteotomy of the scapula 1 cm lateral to the vertebral border. 10 After excision of the omovertebral bone the lateral portion of the scapula is rotated and displaced caudally in relation to the vertebral border which remains unchanged in position. This effectively removes the fullness in the web of the neck and changes the direction of the glenoid fossa which improves shoulder range of motion.

Woodward described his procedure with a preliminary report of nine cases in 1961. u This technique detaches the trapezius and rhomboid muscles from their spinous process origins and reattaches them further distally after resecting the omovertebral bone and any anterior-curving supraspinous extension of the scapula. This procedure has the advantage of less dissection and theoretically less periscapular scarring, with immobilization being unnecessary. Several points relating to the surgical technique merit emphasis: 1) careful analysis of the x-rays preoperati vely to identify any defects in the posterior elements of the spine which might make the surgical exposure hazardous; 2) maintenance of the continuity of the rhomboid and trapezius muscle origin during exposure, to provide a strong reattachment to the spine when the scapula is displaced caudally; 3) transfer of the scapula distally should only be attempted to the point that the scapular spines are at the same level. The congenitally elevated scapula is also hypoplastic, and an attempt to get the inferior poles to the same level, produces over-correction and an increased risk of neurovascular complications; 4) a tight subcuticular skin closure is recommended, as widening of these scars postoperatively is a common complication.

Robinson et al stressed the importance of the clavicular component of the Sprengel's deformity and suggested osteotomy of the clavicle in conjunction with Woodward's procedure.13 This was prompted by their experience of several cases of brachial plexus palsy, which were felt to be secondary to compression of the nerve roots by the clavicle after mobilization of the scapula. This modification was used in the case reported here, and is supported in the recent literature.13·14 Carson et al reported 11 patients with 13 shoulders treated with the Woodward procedure. 14 They found excellent or good cosmetic improvement in 9 of the 11, and patient satisfaction in the same number. The major complaint at follow up was the appearance of the scar in 7 of the 11 patients. They also found equally good results in the older child but empirically felt that the operation should be done by 7 or 8 years for best results.

After reviewing the literature it appears that the results are generally better following the procedures which relocate the scapula3·8·11 than after simple resections.6·7 Ross and Cruess reviewed 77 cases of surgically treated Sprengel's deformity gathered from 19 Shriner's Hospitals in the United States and Mexico from 1935 to 1970. 12 They found better abduction at the shoulder with the procedures which relocate the scapula and noted a higher recurrence rate with the simple resections. Neurovascular complications should be prevented by prophylactic clavicular osteotomy and avoiding over-correction.

The results of the Woodward procedure, in terms of function and cosmesis, are generally quite satisfactory, if these complications are avoided. The case presented has had an excellent result and although perhaps better than the average, is quite illustrative of the improvement which can be obtained in properly selected patients with significant deformity from congenital elevation of the scapula.

References

1. Eulenberg: Beitrag zur Dislocation der Scapula. Amlicht Ber Deutscher Naturforsch Aerzte Karlsbad 1863; 37:291-294.

2. Sprengel: Die angeborene Verschiebung des Schulterblattes nach Oben. Arch KUn Chir 1891; 42:545.

3. Schrock RD: Congenital anomalies at the cervicothoracic level. American Academy of Orthopaedic Surgeons Instructional Course Lectures. 1949; 6.

4. Engel D: The etiology of undescended scapula and related syndromes. J Bone Joint Surg 1943; 25A:613-625.

5. Jeannopoulos CL: Congenital elevation of the scapula. J Bone Joint Surg 1952; 34A:883-892.

6. Cavendish ME: Congenital elevation of the scapula. J Bone Joint Surg 1972; 54B:395-408.

7. McFarland B: Congenital deformities of the spine and limbs, in Piatt H (ed): Modern Trends in Orthopaedics. London. Butterworths. 1950, pp 107-137.

8. Green WT: The surgical correction of congenital elevation of the scapula (Sprengel's deformity): Proceedings of the American Orthopaedic Association. J Bone Joint Surg 1957; 39A:1439.

9. Schrock RD: Congenital elevation of the scapula. J Bone Joint Surg 1926; 8A:207-2I5.

10. Wilkinson JA. Campbell D: Scapular osteotomy for Sprengel's shoulder. J Bone Joint Surg 1980; 62B:486^90.

11. Woodward JW: Congenital elevation of the scapula. Correction by release and transplantation of muscle origins. A preliminary report. J Bone Joint Surg 1961; 43A:219-228.

12. Ross DM, Cruess RL: The surgical correction of congenital elevation of the scapula. A review of seventy-seven cases. Clin Orthop 1977; 125:17-23.

13. Robinson RA. Braun RM, Mack P, et al: The surgical importance of the clavicular component of Sprengel *s deformity. Proceedings of the American Orthopaedic Association. J Bone Joint Surg 1967; 49A:1981.

14. Carson WG, Lovell WW, Whitesides TE: Congenital elevation of the scapula - Surgical correction by the Woodward procedure../ Bone Joint Surg 1981; 63A: 1199-1207.

Section Editor: Charles E. Johnston U, MD

10.3928/0147-7447-19870601-16

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