Keith Bachmann, MD UVA Department of Orthopaedic Surgery

Keith Bachmann, MD UVA Department of Orthopaedic Surgery

Definition Spinal deformity secondary to either neurologic or muscle pathology.

Etiology Imbalance of muscle forces Lack of truncal support Similar to all scoliosis negatively affected by growth

Diagnosis Primary Neuropathies CP Friedreich s Ataxia CMT disease Spinal Cord Injury Syringomyelia Transverse myelitis Polio Spinal Muscle Atrophy Myelomeningocele Primary Myopathies Muscular Dystrophies Arthrogryposis Congenital hypotonia Prader-Willi Syndrome

Onset Incidence Diagnosis Progression

Co-morbidities Gastrointestinal problems Pulmonary Seizures Cardiac Immunodeficient Musculoskeletal Hematologic GU

CP Largest population of NM scoliosis Severity of CP Hemiplegia vs diplegia vs quadriplegia GMFCS (I V) IV and V most severe Ambulators 2% (> 40 0 ) 1 Scoliosis Kyphosis vs lordosis

CP Co-morbidities Gastrointestinal Mal-nourished anemic, immunosuppressed G tube Post-op ileus Seizure Meds osteoporosis Instrumentation failure Musculoskeletal Osteoporosis contractures

CP Surgical challenges Baclofen pump Improves spasticity May increase scoliosis Osteoporosis Uncontrolled seizures Poor soft tissue coverage

Myelodysplasia Scoliosis Age of onset - variable Dependent on spinal level Secondary to paralysis and/or tethering At birth surgical closure Kyphosis Lack of posterior tether Lordosis secondary to post-surgical tethering

Myelodysplasia Cognitive function Normal mental function Poor mental function Hydrocephalus Tx with VP shunting

Co-morbidities Neurologic Paralysis Insensate skin breakdown GU incontinent Self catherization Frequent UTI s GI - incontinent Contractures

Surgical challenges Lack posterior elements Non-union Infection Insensate skin UTI s Bowel incontinence

DMD Affects only males Dystrophin gene Scoliosis nearly 100% Onset loss of ambulation Recommend fusion 20 0 Progressive Kyphosis Loss of posterior muscle strength

DMD Steroid Tx Pros Delays loss of ambulation May delay onset of scoliosis Increased survival Cons Doesn t prevent scoliosis Osteoporosis Immunosuppressive

Co-morbidities Pulmonary Secondary to muscle weakness Monitor PFT s Cardiac Requires pre-op echo Hematologic

DMD Surgical Challenges Pulmonary Prolonged post-op intubation Obesity Wound complications Bleeding Platelet dysfunction Decreased vascular smooth muscle fxn

Spinal Muscle Atrophy Anterior horn cells Most common lethal NM disease of infancy Three types onset Scoliosis type dependent Early Onset risk of scoliosis Kyphosis Loss of posterior muscle strength

Co-morbidities Pulmonary progressive decline Secondary to muscle weakness Monitor PFT s GI G-tubes Musculoskeletal contractures Osteoporosis

SMA Surgical Challenges Pulmonary Prolonged post-op intubation Early Onset Lung maturation crankshaft Emaciation Nusinersin delaying scoliosis?

Scoliosis Considerations Sitting ability Pain Pulmonary Function GI function Skin managment

Scoliosis Considerations Sitting ability Sit upright in chair Ability to be in chair Pain Pulmonary Function GI function Skin management

Scoliosis Considerations Sitting ability Pain Ribs on pelvis Spasm pain Pulmonary Function GI function Skin management

Scoliosis Considerations Sitting ability Pain Pulmonary Function Decline in PFTs associated with increased scoliosis GI function Skin management

Scoliosis Considerations Sitting ability Pain Pulmonary Function GI function Space for GI system Reflux Small food volumes Skin management

Scoliosis Considerations Sitting ability Pain Pulmonary Function GI function Skin management Cleaning Prevent breakdown

NM Scoliosis Long C curve Pelvic obliquity Hip dislocations Sagittal imbalance Global kyphosis Lumbar lordosis

NM Scoliosis Treatment Goals Halt curve progression Attain coronal/sagittal balance sitting balance

Non-operative Tx Bracing Does not prevent scoliosis but may help with positioning May slow progression Young patient Not definitive treatment Recommend soft TLSO

Non-operative Tx Benign neglect Too ill for intervention

Operative Tx Curve Magnitude Coronal 50 degrees most NM pts 20 degrees DMD: older dogma in general want to get curve treated before heart function deteriorates Sagittal Lordosis Kyphosis

Operative Tx Posterior Spinal Fusion Gold standard Proximal Extension T2/T3 Distal Extension Pelvis Rule breaker - DMD

Operative Tx DMD Pelvis vs L5 Muburak et al 2 L5 if pelvic obliquity < 15 degrees L5 Decreased surgical time Decreased EBL Pelvis Decreased decompensation

Operative Tx Instrumentation - Traditional Luque wires Segmental Cheap Risk of neurologic injury Unit rod Cheap Galveston Pelvic fixation

Technique

Operative Tx Luque wire Risk of PJK

Operative Tx Galveston Technique Risk of loosening

Operative Tx Anterior Spinal Fusion Myelodysplasia Lumbar scoliosis Normal thoracic tone Young patients Lumbar curves Salvage thoracic growth

Technique

Operative Tx Anterior Spinal Fusion Problem scoliosis progression

Operative Tx Anterior/Posterior Spinal Fusion Large curves Stiff curves Severe sagittal deformity Risk of non-union myelodysplasia Risk of crankshaft? need with pedicle screws

Operative Tx Anterior release Multi-level discectomy vertebrectomy

Operative Tx

Operative Tx Anterior release/instrumentation Option of delayed stage

Operative Tx

Early Onset Scoliosis Bracing Growing Rod FUSION VEPTR Shilla

Growing Rods

VEPTR

Shilla Technique

Surgical Outcome Bridwell et al. 3 Questionnaire, retrospective Improved cosmesis and QOL Patients families satisfied with surgery CP Child outcome measure: Caregiver Priorities and Child Health Index of Life with Disabilities Swiss study 2011: 50 consecutive patients: 91.7% satisfaction, statistical significant improvement in CP-Child score, 8 complications with 6 requiring repeat surgery

References 1. Rosenthal et al. Dev Med Child Neurol 1974, 16:664 2. Mubarak et al. Spinal fusion in Duchenne muscular dystrophy--fixation and fusion to the sacropelvis? J Pediatr Orthop, 1993. 13(6): p. 752-7. 3. Bridwell, K.H., et al., Process measures and patient/parent evaluation of surgical management of spinal deformities in patients with progressive flaccid neuromuscular scoliosis (Duchenne's muscular dystrophy and spinal muscular atrophy). Spine, 1999. 24(13): p. 1300-9. 4. Bohtz, C et al., Changes in Health Related Quality of Life After Spinal Fusion and Scoliosis Correction in Patients with Cerebral Palsy. JPO, 2011. 31(6): 668-673