Most common type of congenital deformities which result from vertebral malformation during the gestational period. Family history is rare: 13/1,250 Patients positive F-Hx (Winter, 1983). They are grouped as:
- Isolated Anomalies: No Genetic Risk (Wynne Davies, 1975)
- Complex Anomalies: 5-10% Risk
Two main types of malformation are described and include formation and segmentation defects.
- Formation defects: Missing parts of vertebral (Hemivertebra most common)
- Segmentation defects: Failure of part if the vertebral to separate Unsegmented bars).
Depends on type and site of anomaly with increasing risk of progression as follows:
- Block vertebrae
- Wedge vertebrae
- Single Hemivertebra
- 2 Unilateral Hemivertebrae
- Unilateral Unsegmented Bar
- Unilateral Segmented Bar with Contralateral Hemivertebra
True incidence unknown but in general 25% of curves do not progress, 25% of curves slowly progress and 50% are very progressive.
Evaluation of the congenital patient should include:
- Physical Examination
- Radiographic and special studies such as CT, MRI and Ultrasound
Associated Anomalies are present in 60% of patients and can include organ systems such as: genitourinary (15-25%), cardiopulmonary (10%), neurologic (20-40%), followed by lesser occurrences of musculoskeletal, EENT and gastrointestinal tract.
Attention to managing the associated anomalies sometime is critical for patient’s health than the spine deformity.
Non operative treatment and braces are rarely indicated for most congenital scoliosis. Surgery is Mainstay of Treatment and options include:
- Posterior Fusion,
- Anterior/Posterior Fusion,
- Hemivertebra/Wedge Excision and
- Vertebra column resection (VCR)
Growth arrest procedures are unpredictable and are reserved for single and fully segmented hemivertebra without kyphosis: Results show 30% result in In-Situ fusion, 30% obtain correction and 30 % fail or are worse. Due to the need to wait till end of growth to assess the full effect of the procedure most surgeons resort to the current method of hemivertebral resection and short fusion to achieve instant correction. Hemivertebra resection is preferred for Fully segmented hemivertebral of the lumbosacral and lumbar spine.
Posterior Vertebral Column Resection is indicated for:
- Complex multiplanar deformity
- Previously fused spine
Patients with Poor Pulmonary function
CEO, FOCOS Orthopedic Hospital
Orthopedic Surgeon, FOCOS Orthopedic Hospital