Keith Bachmann, MD UVA Department of Orthopaedic Surgery

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1 Keith Bachmann, MD UVA Department of Orthopaedic Surgery

4 Definition Spinal deformity secondary to either neurologic or muscle pathology.

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

6 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

7 Onset Incidence Diagnosis Progression

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

9 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

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

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

12 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

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

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

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

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

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

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

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

20 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

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

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

23 Scoliosis Considerations Sitting ability Pain Pulmonary Function GI function Skin managment

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

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

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

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

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

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

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

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

32 Non-operative Tx Benign neglect Too ill for intervention

33 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

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

35 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

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

37 Technique

38 Operative Tx Luque wire Risk of PJK

39 Operative Tx Galveston Technique Risk of loosening

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

41 Technique

42 Operative Tx Anterior Spinal Fusion Problem scoliosis progression

43 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

44 Operative Tx Anterior release Multi-level discectomy vertebrectomy

45 Operative Tx

46 Operative Tx Anterior release/instrumentation Option of delayed stage

47 Operative Tx

48 Early Onset Scoliosis Bracing Growing Rod FUSION VEPTR Shilla

49 Growing Rods

50 VEPTR

51 Shilla Technique

52 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

54 References 1. Rosenthal et al. Dev Med Child Neurol 1974, 16: Mubarak et al. Spinal fusion in Duchenne muscular dystrophy--fixation and fusion to the sacropelvis? J Pediatr Orthop, (6): p 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, (13): p Bohtz, C et al., Changes in Health Related Quality of Life After Spinal Fusion and Scoliosis Correction in Patients with Cerebral Palsy. JPO, (6):

Spinal Deformity Pathologies and Treatments

Spinal Deformity Pathologies and Treatments Scoliosis Spinal Deformity 3-dimensional deformity affecting all 3 planes Can be difficult to visualize with 2-dimensional radiographs Kyphosis Deformity affecting