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Lower Extremity Contractures and Hip
Dysplasia in SMA
David A. Spiegel, M.D. Children’s Hospital of Philadelphia
Associate Professor of Orthopaedic Surgery University of Pennsylvania School of Medicine
Context
• Flaccid, progressive, proximal, LE • Phenotype varies • Promising strategies, alter the natural history – Gene therapy (AAV9) – SMN2 splicing modulation
• Challenging convention
Haaker J, Fujak A. Applic Clin Gen 2013;6:113-120.
• Positioning, comfort, ease of care • Limited upright mobility (stander)
Maximize ambulation
World Health Organization. International classification of functioning, disability, and health. Geneva, 2001
(http://www.who.int/classifications/icf/en/)
“The Boy with a Clubfoot”
Jusepe de Ribera (1642)
“The Clubfoot” Jusepe de Ribera (1623)
• What we see versus what pt/family sees
• Establish realistic, functional goals
• Proactive versus reactive
Contractures
• Why? Denervation/weakness, immobilization/positioning • What? Contractile Tissue ( connective tissue) Connective tissue (lose extensibility) Joint (fibrofatty connective tissue) Farmer SE, James M. Disability and Rehabilitation 2001;23:549.
• 143 pt, Type II, 5 yrs fu
• Hip: 45° FC, rotation, add, abd
• Knee: 37° FC • Ankle: Equinus (13° in 31%)
Fujak A, et al. Ortopedia, Traumatologia, Rehabilitacja; 1 3:27–36.
• 44 pt • Type II
– Flexion contracture • 48% hip, 89% knee, 52% ankle
• Type III – Flexion contracture
• 18% hip (45°) • 29% knee (27°) • 53% ankle (15°) Wang HY, et al. Arch Phys Med Rehab,
2004;85:1689–1693.
• Positioning • Physical therapy • Serial casting/wedging • Orthoses Static, Dynamic When and how long
• Practicality and sustainability • Shared responsibility
Nonoperative: Proactive vs. Reactive
Reactive:
• Operative – Soft tissue
• Muscle lengthening or release – Osteotomy
• Early mobilization • Risk of losing function • Deterioration/recurrence over time
• Stretch casting • Soft tissue lemgthening/release – Hamstrings – Posterior capsule
• “Guided growth” • Extension osteotomy
Knee Flexion Contracture
• Subluxation and dislocation very common, II > III
• Pain/functional consequences rare • Pathologic anatomy? • Reactive rather than proactive
Haaker J, Fujak A. Applic Clin Gen 2013;6:113-120. Sporer SM, Smith BG. J Pediatr Ortho 23:10-14. Thompson CE, Larsen LJ. J Pediatr Ortho 1990;10:638-641. Evans Zenios M, et al. JBJS [Br] 2005;87:1541-1544
Hip: What we think we know
• Age • Unilateral versus Bilateral?
• Subluxation or Dislocation
• Ambulatory? • Symptoms?
Treatment
What is the pain generator?
• Soft tissue contracture • Bony (impingement or arthritis) • Local pressure from asymmetric seating position