What are the differences in treatment approaches for congenital scoliosis versus early onset scoliosis?

Treatment Approaches for Congenital Scoliosis vs Early Onset Scoliosis

Congenital scoliosis requires MRI of the complete spine before any treatment decision due to the 21-43% prevalence of intraspinal anomalies, while early onset idiopathic scoliosis follows a growth-friendly surgical approach when conservative management fails, with both conditions demanding fundamentally different treatment algorithms based on their distinct etiologies. 1, 2

Key Diagnostic Distinctions

Congenital Scoliosis

• Results from vertebral formation or segmentation failures present at birth, accounting for 10% of surgical scoliosis patients 1
• Initial imaging must include both PA/lateral radiographs AND MRI complete spine as complementary procedures 2
• Intraspinal anomalies occur in 21-43% of cases including tethered cord, filar lipoma, syringohydromyelia, and diastematomyelia 1
• Critical finding: Normal neurological examination does NOT predict normal MRI - physical exam accuracy is only 62% for detecting intraspinal anomalies 1

Early Onset Scoliosis

• Appears before age 10 in the absence of vertebral anomalies or associated syndromes 2
• Natural history is dismal with poor pulmonary function and increased mortality if untreated 3
• Requires both radiographs and MRI for initial imaging per American College of Radiology guidelines 2
• Must avoid extended posterior fusion to prevent crankshaft phenomenon and restrictive lung disease 4

Treatment Algorithm for Congenital Scoliosis

Step 1: Mandatory Pre-Treatment Evaluation

• Obtain MRI complete spine in ALL cases before any treatment decision - this is non-negotiable 1, 2
• Obtain CT spine for surgical planning if surgery is being considered, as it reduces screw misplacement from 15.3% to 6.5% 1
• Serial PA radiographs every 6 months maximum to monitor progression 1

Step 2: Risk Stratification by Curve Type

• Unilateral bar with contralateral hemivertebra: >10° progression per year - highest risk pattern requiring early intervention 1
• Isolated hemivertebra: 28% intraspinal anomaly rate 1
• Complex vertebral anomalies: 21% intraspinal anomaly rate 1

Step 3: Treatment Selection

• Conservative management first - advanced bracing technology may allow many patients to avoid surgery 5, 6
• Early surgical intervention (before age 3) is traditionally recommended but lacks prospective or randomized controlled trial evidence 5, 6
• Surgical options when indicated:
  • In situ fusion for limited deformities 7
  • Hemivertebra resection with segmental fusion (contradictory safety data exists) 5, 6
  • Growth-friendly techniques for progressive curves 7

Treatment Algorithm for Early Onset Scoliosis

Step 1: Initial Conservative Management

• Conservative treatment is first-line for most cases due to fewer complications than surgery 4
• Bracing for curves 20-40° in skeletally immature patients with >70% progression likelihood 8
• Serial radiographs: every 12 months for Risser 0-3, every 18 months for Risser 4-5 2, 8

Step 2: Growth-Friendly Surgery When Conservative Fails

The fundamental principle: Allow curve control while maintaining spine and thorax growth 3

Three surgical strategy categories:

1. Distraction-based approaches:

   • Growing rods (gold standard since mid-1980s) - lengthened at regular intervals 4
   • Magnetically controlled growing rods - distracted outpatient without anesthesia 4
   • VEPTR (vertical expandable prosthetic titanium rib) - attaches to ribs for chest wall dysplasia, but has high complication rates 5, 6, 4

2. Guided-growth approaches:

   • Luque trolley technique 3
   • Shilla technique 3
   • Rods guide spinal growth without repeated procedures 4

3. Compression-based approaches:

   • Tethers and staples to slow convexity growth 3, 4

Step 3: Timing of Definitive Fusion

• Delay final fusion until patient is older to allow adequate thoracic and spinal growth 4
• Curves >50° typically require surgical intervention in adolescents and young adults 8

Critical Differences in Management Philosophy

Congenital Scoliosis

• Focus on identifying and addressing underlying structural anomalies 1
• MRI is mandatory - cannot proceed without ruling out intraspinal pathology 1, 2
• 25% do not progress, 25% progress mildly, 50% need treatment based on age, curve characteristics, and anomaly type 7
• Associated anomalies common: genitourinary, musculoskeletal, cardiac, rib anomalies 7

Early Onset Scoliosis

• Focus on preserving growth while controlling deformity 3, 4
• Pulmonary development is the primary concern - restrictive lung disease significantly impacts mortality 3, 4
• Growth-friendly surgery is a "delaying" treatment until definitive fusion can be performed safely 4
• Extended fusion is contraindicated in young children 4

Common Pitfalls to Avoid

• Never assume normal neurological exam rules out intraspinal anomalies in congenital scoliosis - exam accuracy is only 62% 1
• Never perform extended posterior fusion in early onset scoliosis - causes crankshaft phenomenon and restrictive lung disease 4
• Do not skip MRI in congenital scoliosis - 21-43% have intraspinal anomalies that alter surgical planning 1
• Recognize VEPTR has high complication rates despite its utility for chest wall dysplasia 5, 6
• Do not rush to surgery in congenital scoliosis - conservative management with advanced bracing should be attempted first given lack of high-quality evidence for early surgical intervention 5, 6

Pre-Surgical Requirements for Both Conditions

When surgery becomes necessary:

• MRI complete spine is mandatory to rule out neural axis abnormalities 2, 9, 8
• CT spine for surgical planning in congenital cases to visualize bony malformations 1
• Pulmonology evaluation to assess baseline lung function and screen for sleep hypoventilation 9
• Cardiology evaluation for cardiomyopathy or arrhythmia risk 9
• Nutritional optimization to support healing 9