What is sprangles deformity?

Sprengel's Deformity

Sprengel's deformity is a rare congenital anomaly characterized by the abnormal elevation of the scapula, resulting from failure of the scapula to descend normally during embryonic development. 1 It is the most common congenital shoulder abnormality and can significantly impact both cosmesis and shoulder function.

Clinical Characteristics

• Anatomical features:

  • Elevated, dysplastic, and malrotated scapula
  • Often asymmetric (can be unilateral or bilateral)
  • Frequently associated with an omovertebral connection (bony or fibrous band between scapula and cervical spine)
  • Webbed neck appearance (pterygium colli)

• Functional limitations:

  • Restricted shoulder abduction (<70°) and flexion (<80°) 2
  • Decreased range of motion
  • Difficulty with overhead activities

Associated Conditions

Sprengel's deformity commonly occurs with other congenital anomalies:

• Klippel-Feil syndrome (fusion of cervical vertebrae) 1, 2
• Congenital scoliosis 1, 3
• Rib anomalies 1
• Spina bifida 3
• Tethered cord syndrome 3

Diagnostic Evaluation

• Physical examination: Reveals high-riding scapula, webbed neck, and limited shoulder range of motion
• Radiographic assessment: Shows elevated scapula and may reveal omovertebral connections
• MRI: Important to evaluate for associated spinal abnormalities, especially tethered cord syndrome 3

Important: Diagnostic confusion with limited scoliosis can delay proper treatment of other associated abnormalities, even in mild cases 1

Management

Treatment decisions are based on:

1. Severity of deformity
2. Functional limitations
3. Cosmetic concerns
4. Associated conditions

Non-surgical Management

• Appropriate for mild cases with minimal functional limitations 1
• Home-based exercise programs to strengthen periscapular and intrinsic muscles 3
• Physical therapy to maximize available range of motion

Surgical Management

Surgical intervention is typically warranted for more severe cases to improve both cosmesis and function 1:

1. Timing: Traditionally performed in childhood, but can be beneficial in adults with untreated deformity 4

2. Surgical techniques:

   • Modified Woodward procedure: Resection of omovertebral connection, scapular derotation and inferior migration 2
   • Scapular resection: Removal of the protruding superomedial border of the scapula 4
   • Green procedure: Detachment of muscle origins, scapular repositioning, and reattachment

3. Outcomes:

   • Improved cosmetic appearance
   • Reduction in neck pain
   • Variable improvement in shoulder motion (may depend on size, shape, and position of omovertebral bone) 4
   • Recent long-term studies indicate maintained improvements in function and appearance 1

Special Considerations

• Neurological assessment: Comprehensive neurological examination is essential as Sprengel's deformity can be associated with tethered cord and other neurological issues 3

• Genetic factors: Although typically sporadic, rare familial cases have been reported, suggesting possible genetic factors in some cases 2

• Adult treatment: Surgical intervention can still produce good results in adults who did not receive treatment as children 4

• Monitoring: Regular follow-up is recommended to assess for progression of associated conditions, especially scoliosis and neurological symptoms

Proper recognition and management of Sprengel's deformity and its associated conditions are essential to optimize functional outcomes and quality of life for affected individuals.