SPR unknown #37-- Final

Michael David Chang, BSc, MS III

Arie Franco, MD, PhD

Kristopher Neal Lewis, MD

Children's Medical Center

Medical College of Georgia

Keywords

SPR unknown 37, Sprengel


Publication Date: 2010-09-22

History

2 year old male with torticollis and restricted motion of the left shoulder and right arm.

Findings

Figure 1: Frontal radiograph of the neck demonstrates elevated left scapula, omovertebral bone, and partial fusion of vertebral bodies C5-C7.

Figure 2: Frontal radiograph of the right elbow demonstrates fusion between the proximal radius and the proximal ulna.

Diagnosis

Left Sprengel Deformity and right radioulnar synostosis

Discussion

Sprengel deformity, also termed congenital high scapula, is the result of failure of the scapula to descend in utero. The scapula is of cervical origin and normally migrates into the thorax within the first trimester [3]. Failure of descent manifests as a hypoplastic, elevated scapula, which affects the growth and development of other structures of the shoulder girdle [1, 4]. This disorder most often occurs unilaterally [2, 3].

Children with Sprengel deformity usually present with torticollis and restricted forward flexion and abduction of the affected shoulder. Other associated anomalies include scoliosis, cervical spina bifida, cervical ribs, and Klippel-Feil syndrome [1, 3, 4]. The finding of a proximal forearm deformity in this case underscores the multifaceted phenotypic nature of Sprengel. One-third of individuals with Sprengel deformity also have a bony, cartilagenous, or fibrous omovertebral connection between the superior pole of the scapula and the cervical spine [1, 2, 4]. Typical systemic congenital anomalies include those of the kidneys and the lungs [4].

Management is guided by the patient’s functional and cosmetic status, in addition to the extent of the anatomical deformity, classified by Cavendish grade.

Cavendish Grade I – level glenohumeral joints, no deformity visible with patient dressed

II – level glenohumeral joints, cervical lump observed with patient dressed

III – 2 to 5 cm scapular elevation

IV – scapula elevated to the vicinity of the occiput

Definitive treatment involves surgical excision of omovertebral connections and re-positioning of the scapula. Two common operations are the Woodward and Green procedures. The Woodward procedure moves the scapula inferiorly by detachment and reattachment of the parascapular muscles at their origins on the spinal process. The Green procedure involves extraperiosteal detachment of the scapular insertion of the paraspinal muscles, and reattachment after the scapula has been moved inferiorly with traction cables. Surgery will not correct hypoplasia of the scapula, but various re-positioning procedures have been shown to significantly alleviate the functional and cosmetic burden of the deformity [1, 4].

References

  1. Ahmad AA. Surgical Correction of Severe Sprengel Deformity to Allow Greater Postoperative Range of Shoulder Abduction. J Pediatr Orthop. 2010; 30: 575-581.
  2. Hamner DL, Hall JE. Sprengel’s Deformity Associated with Multidirectional Shoulder Instability. J Pediatr Orthop. 1995; 15(5): 641-3.
  3. Jeannopoulos CL. Observations on Congenital elevation of the scapula. Clinical Orthop. 1961; 20:132-9.
  4. Waters PM. Sprengel Deformity. Lovell Winter Pediatr Orthop. 2006; 2: 977-9.

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