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Short Note: Congenital and Idiopathic Scoliosis
(For MS Orthopaedics Theory)
Definition
Scoliosis is a lateral curvature of the spine greater than 10 degrees (Cobb angle). It is broadly classified as congenital or idiopathic, and together these two types account for the majority of all scoliosis presentations (idiopathic alone accounts for ~70%).
Part I: Congenital Scoliosis
Definition
A three-dimensional spinal curvature caused by vertebral anomalies resulting in an imbalance of longitudinal spinal growth. A vertebral anomaly must be radiographically visible to make this diagnosis.
Prevalence
~1 in 1000 live births.
Embryology
The critical embryological period is the 5th to 8th weeks of gestation, when vertebral segmentation and formation occur.
Classification (Winter-Moe-Eilers / MacEwen modification)
| Type | Examples |
|---|
| Failure of Formation | Partial (wedge vertebra); Complete (hemivertebra) |
| Failure of Segmentation | Unilateral (unilateral unsegmented bar); Bilateral (block vertebra) |
| Mixed | Combination of both |
Natural History and Progression Risk (worst to best)
- Unilateral unsegmented bar + contralateral hemivertebra - most severe; mean progression 6-7°/year; most exceed 50° by age 2 years
- Unilateral unsegmented bar (most common congenital anomaly) - ~5°/year; reaches 50° by age 10
- Double convex hemivertebrae - 3-4°/year
- Single fully segmented hemivertebra - slowest; 1-2°/year
- Block vertebra - best prognosis; least severe
Progression is fastest in the first 5 years of life and during the adolescent growth spurt (10-15 years).
Associated Anomalies
- Neural axis abnormalities (diastematomyelia, tethered cord): up to 35-43% - MRI is mandatory
- Congenital heart disease: 25-54% - echocardiogram required preoperatively
- Genitourinary anomalies: 20-40% - renal ultrasound for screening
- Other musculoskeletal anomalies: Klippel-Feil syndrome, Sprengel deformity, clubfoot
Clinical Evaluation
- Examine skin of back for hair tufts, lipomas, dimples (suggest underlying dysraphism)
- Full neurological examination (clubfoot, calf atrophy, asymmetric lower limb - signs of cord involvement)
- Imaging: X-ray AP + lateral; MRI for intraspinal anomalies; echocardiography; renal ultrasound
Treatment
Nonoperative:
- Observation with radiographs every 6 months during rapid growth phases (0-5 yrs, 10-15 yrs)
- Bracing: generally ineffective for the primary curve but can control secondary compensatory curves
- Serial derotational casting: useful time-buying strategy to delay surgery
Operative (required in ~75% of patients):
| Procedure | Indication |
|---|
| In situ posterior spinal fusion | Small progressive curves; early intervention |
| Combined anterior + posterior fusion | Young patients - to prevent crankshaft phenomenon |
| Convex hemiepiphysiodesis | Small curves with growth potential |
| Growth-friendly techniques (growing rods, VEPTR, Shilla) | Young patients (<8-10 yrs) with large progressive curves |
| Hemivertebra resection | Isolated hemivertebra; combined with A+P arthrodesis |
| Vertebral column resection / osteotomy | Severe rigid deformity |
Note: Congenital scoliosis is the condition in which paraplegia occurs most often after instrumentation. Intraoperative spinal cord monitoring (SSEP + MEP) is mandatory.
Part II: Idiopathic Scoliosis
Definition
Scoliosis of unknown cause; accounts for 70% of all presentations. Likely multifactorial in origin.
Classification by Age of Onset
| Type | Age | Key Features |
|---|
| Infantile | < 4 years | Left thoracic curve; more common in boys; plagiocephaly; ~90% resolve spontaneously; use Mehta RVAD to predict progression |
| Juvenile | 4-10 years | Right thoracic curve; 25% have spinal cord abnormality; 95% risk of progression; less responsive to bracing than adolescent type |
| Adolescent (AIS) | > 10 years | Most common; right thoracic; female predominance; associated with near-normal life expectancy |
Distinction between early onset (<8 yrs) and late onset (>8 yrs) is important because alveolar development ceases at age 8 - severe early-onset curves can lead to cor pulmonale and premature death.
Adolescent Idiopathic Scoliosis (AIS) - Key Points
- Prevalence: Cobb >10° in 0.5-3% of population; Cobb >30° in 1.5-3 per 1000
- Risk factors for progression: Female sex, remaining skeletal growth, thoracic location, larger initial curve magnitude
- Risser sign: Used to assess skeletal maturity and guide treatment
- Long-term studies show 68% experienced curve progression; thoracic curves of 50-75° progress at ~1°/year even after maturity
Infantile Idiopathic Scoliosis - Mehta Classification
- RVAD (Rib-Vertebra Angle Difference): measured between apical vertebra and its ribs
- Phase I (no rib overlap): RVAD <20° = 80% chance of resolution; RVAD >20° = 80% chance of progression
- Phase II (rib overlaps apical vertebra) = very high risk of progression
Treatment of Idiopathic Scoliosis
| Curve | Management |
|---|
| < 25° | Observation; clinical + radiographic follow-up |
| 20-45° (growing child) | Bracing (prevents progression; does not correct curve) - e.g., TLSO, Milwaukee brace |
| > 45° | Bracing ineffective |
| > 40° with progression, truncal imbalance, or unacceptable cosmesis | Surgery |
Surgical treatment:
- Corrective instrumentation and posterior spinal fusion
- Intraoperative monitoring: SSEP, MEP, free-run and stimulated EMG
- Risk of neurological injury: 0.4% (1 in 250)
- For young children with large curves: growing rods, VEPTR, magnetically controlled growing rods - followed by definitive fusion after age 10
Summary Comparison Table
| Feature | Congenital | Idiopathic |
|---|
| Cause | Vertebral anomaly (visible on X-ray) | Unknown, multifactorial |
| Key investigation | MRI (neural/intraspinal anomalies) | Clinical + X-ray; MRI if juvenile/infantile |
| Associated anomalies | Cardiac, renal, neural axis | Usually none |
| Bracing | Limited; for compensatory curves only | Effective for 20-45° in growing child |
| Surgery rate | ~75% | Indicated at >40° with progression |
| Most severe type | Unilateral bar + contralateral hemivertebra | Juvenile type (95% progression) |
Sources: Campbell's Operative Orthopaedics 15th Ed 2026; Miller's Review of Orthopaedics 9th Ed; Bailey and Love's Short Practice of Surgery 28th Ed