Myelodysplasia 

Background 

• Common group of congenital disorders by various chromosomal anomalies that lead to failurre of closure of the spinal cord 
  • Anatomic anomalies 
  • Neurologic impairment 
• Chromosomal abnormalities 
  • 10% T13, T18, triploidy, various single gene 
• AFP 2nd trimester 

Risk Factors 

• Folate deficiency 
• Maternal hyperthermia 
• Maternal diabetes 
• Valproic acid 

Associated Conditions 

• Ortho 
  • Path fractures (osteopenia) 
  • Spine 
    • Scoliosis –  – PSIF to pelvis, often anterior required, high infection (poor soft tissue) 
    • Kyphosis – gibbus deformity 
  • Hip dysplasia 
    • Hip dislocations  
      • L3 – NON OP always 
        • Adducted and flexed hip (unopposed hip flexion and adduction) 
    • Contractures  
      • L1 – ER and flexed hips – HKAFO 
      • Add – proximal TFL division, distal IT band release – prevents pelvic obliquity if >40 
      • Flex – anterioro hip release with tentoomy  
  • Knee deformities 
    • Tibial torsion – distal tibial derotation osteotomy (age >5) 
    • Contractures – hamstring lengthening, SC extension osteotomy if it failtst