Sprengel Deformity (Pediatric)

Synonyms
Sprengel's deformity

INTRODUCTION

Definition

Congenital anomaly with undescended scapula. 

Epidemiology

  • Most common congenital anomaly of the shoulder in children.
  • The scapula is attached to the cervical spine (in 30% of patients) by an omovertebral bone, cartilage, or fibrous tissue.

Etiology

Arises from interruption of normal caudal migration of the scapula.

CLINICAL FEATURES

Symptoms

The deformity is painless and many patients are not diagnosed until adolescence.

Signs

Inspection:
  • Often winging of the scapula, hypoplasia, and omovertebral connections (30% of cases) that can severely limit the scapulothoracic motion.
  • The affected scapulae are usually small, relatively wide, medially rotated and more cephalad than normal.
  • There is elevation and medial rotation of inferior scapula.
  • Due to scapular asymmetry, some patients are mistakenly thought to have scoliosis.
Movement/ROM: Examine the shoulder movement and look for loss of shoulder abduction and foward flexion. If an omovertebral bone is present, abduction of the shoulder is usually limited to less than 90°.
Associated disorders:
  • Klippel-Feil syndrome (one third have Sprengel deformity)
  • Cervical ribs
  • Kidney disease/renal abnormalities
  • Scoliosis
  • Diastematomyelia
  • Torticollis
  • Muscular hypoplasia (especially the trapezius)

DIAGNOSIS

X-ray

Will show elevation of the scapula. 

TREATMENT

Overview

If the patient needs treatment, it is primarily surgical.

Nonoperative Treatment

Passive stretching exercises that was advocated in the past are not successful.

Operative Treatment

Indication: Children between 3-8 years of age with significant deformities, both functional (decreased abduction) and cosmetic. Patients older than 8 years of age are not good candidates for scapular displacement procedures.
Method
  • Woodward procedure: Distal advancement of associated muscles and scapula.
    • The procedure has 80% satisfactory functional and cosmetic results.
    • Can improve abduction ollowing surgery by 40-50° (from 34-60°).
    • The child’s age at operation and differing methods of measurement play largest role in accounting for these differences.
    • Younger patients obtain better motion and postoperative correction.
    • Caudad displacement of scapula is reported to be 1.9 vertebra body heights in one series and 4 cm in another.
  • Schrock and Green procedures: Detachment and movement of scapula. Can improve abduction by 40-50°.
  • Clavicular osteotomy: Often needed to avoid brachial plexus injury caused by stretch och compression agains first rib.

Surgical Technique

Woodward procedure:
  • Resection of omovertebral bone and division of vertebral attachments of trapezius, rhomboids, and levator scapula.
  • Scapula is subsequently rotated and translated caudally.
  • The detached muscle origins are then sutured to more inferior vertebral spinous processes.
  • Clavicular osteotomy is often needed to avoid brachial plexus injury caused by stretch and to prevent compression of neurovascular structures against first rib.
  • Three weeks of postoperative immobilization is required. Postoperative improvement in shoulder abduction is maintained, although some loss of scapular translation can occur in first four months postoperatively.
  • One third of patients will have widening of their surgical scars, which can be cosmetically disturbing.
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