SPINAL PROBLEMS IN THE PEDIATRIC PATIENT

SPINAL PROBLEMS IN THE PEDIATRIC PATIENT Michael C. Albert, MD Orthopaedic Center for Spinal and Pediatric Care, Inc. The Children s Medical Center, Dayton, Ohio Hobart Klaaren 1926-2002 Objectives Review Spinal Problems in pediatric patient Adult sequelae of untreated spinal deformity Non-Operative Treatment of Scoliosis Decision for Surgery Risks of Surgery Pre-Operative Planning Surgical Treatment of Scoliosis Advances in Spinal Instrumentation Case Studies 1

SPINAL PROBLEMS Scoliosis Definition Scoliosis Kyphosis Infection Tumor Spondylolisthesisp y Fracture Greater than 10º lateral deviation from the central line Lordosis: Abnormal posterior bend to spine Kyphosis: Abnormal forward bend of spine Prevalence Depending on definition overall prevalence of Scoliosis is from.5% to 2.0 % Curves Requiring Treatment = 03% 0.3% EVALUATION OF PATIENT History Onset Diagnosis Pain Progression Weakness, Numbness 2

EVALUATION OF PATIENT Growth History Menses Breast Development Pubic Hair Family History Spinal Deformity Neurologic Disease EVALUATION OF PATIENT Past Medical History Surgery(Open Heart) Radiation/Chemotherapy R.O.S. PHYSICAL EXAMINATION OF SPINE Rib Hump (Scoliometer) Waist Line Asym. Abnormal Skin Marking Leg Lengths Major right thoracic curve 3

Head center over midpelvis Shoulders level Scapulae symmetric Waistline symmetric (not here!) Iliac crest even RADIOGRAPHIC EVALUATION Standing PA/Lat Spine Cobb Technique Breast Shields Red Flag PHYSICAL EXAMINATION CENSORED Left Sided Curve Neurologic Anomalies Abnormal Skin Markings (hariy patch, Café Au Lait spots) Pain 20-30 % of patients with Scoliosis will have pain with 9% having other pathology (TRSH Study) Stiffness and poor flexibility SCOLIOSIS NON-STRUCTURAL Limb Length Inequality Hip Flexion Contracture Muscle Imbalance 4

SCOLIOSIS STRUCTURAL Idiopathic Congenital Neuromuscular Trauma Scoliosis Age and Gender AIS 80-85% of Scoliosis : ratio >10 1.4: 2 >20 5.4:1 >30 10:1 JBJS (Br) 1968 Feb;50(1):24-30. IDIOPATHIC SCOLIOSIS Most Common Curvature of Spine Adolescent l t Type Most Common Females Greater Than Males (overall) IDIOPATHIC SCOLIOSIS Theories Calmodulin Calcium induced muscle contraction disorder Elastic fiber system defect bt but not universal Disorganized skeletal growth-asymmetric mechanical forces Melatonin deficiency Genetics-chromosomes 6, 10, and 18 IDIOPATHIC SCOLIOSIS - CLASSIFICATION Infantile Birth 3 yrs. Juvenile 3 10 yrs. Adolescent >10 yrs. INFANTILE IDIOPATHIC SCOLIOSIS Age Birth to 3 years More Common Males Left Thoracolumbar Most Common 15% Severe Progression 5

INFANTILE TYPE TREATMENT JUVENILE IDIOPATHIC SCOLIOSIS Benign Progressive Brace Malignant Progressive Growing rod Techniques Age 3 10 years Treatment Brace Document Progression Curves >20 ADOLESCENT IDIOPATHIC SCOLIOSIS Most Common Cause Spinal Deformity Onset After Puberty (>10 yrs) Curve Pattern Rt. Thoracic Rt. Thoracic, Left Lumbar Thoracolumbar Double Thoracic ALL of these girls have greater than 60 degree curves (The one on the right has two 60 degree curves) INCIDENCE OF PROGRESSSION UNTREATED Adolescent Idiopathic Scoliosis Curve Magnitude Risser Sign 0-1 <19 20-29 22% 68% 2-4 2% 23% Lonstien JBJ 5 66A 1984 6

TREATMENT First attempt to treat scoliosis with bracing Walter Blount The Milwaukee Brace 1948 Options Observation Bracing Surgery Low Profile Brace Diagrams showing how the lateral pad works. The Boston System was developed as a modular system in which 95% of patients could be fit in a series of pre-fab fbmodules dl which could be cut to fit, thus freeing up Orthotists time Low Profile Braces Originally found to be best used when the curve apex was at T-10 or below. Above this level, published results have varied TLSO BRACING 7

Charleston Bracing Providence Nighttime BRACING The Providence Brace was designed to treat Idiopathic Scoliosis in growing children with the desired outcome to prevent progression and need for surgery. Recumbant Brace worn only at night Computer Designed (CAD/CAM) Natural History Studies Lonstein & Carlson JBJS 1984 Skeletally immature patients with curves 20-29º, 68% risk progression Nachemson & Peterson JBJS 1995 66% risk progression with curves between 25-35º Effectiveness of Treatment with a Brace in Girls with AIS: Nachemson & Peterson JBJS June 1995 Prospective Multicenter study over 10 years Success Rate-Brace 74% Success Rate-Observation only 33% Not Randomized Professional Opinion Concerning the Effectiveness of Bracing Relative to Observation in AIS Weinstein et al. Journal of Pediatric Orthopaedics. April/May 2007 Majority of fbracing Studies Level liv Case Studies Only one level II prospective cohort study 15 Studies have surgery rates after Bracing Treatment Range 5% to 46% High degree of variability in opinion among clinicians concerning effectiveness of bracing Suggest randomized trial of bracing Clinical Equipoise 8

Surgical Rates After Observation and Bracing for AIS: An Evidence Based Review Dolan and Weinstein et al. Spine 2007. 18 Studies Reviewed Pooled estimates for surgical rates extremely variable No clear advantage of bracing vs. observation Compliance Most studies show if braces are worn, the patients do better Edmonson labeled three groups of Milwaukee Brace wearers: Cooperative 65% Did best Cheaters 20% Not so good Non-Cooperative 15% Worse yet Compliance We all are cynical about how much braces are actually worn. Most studies with any brace claim @ 2/3rds compliance Sensors placed in braces reveal less wear than self reported by patients How often do you see kids at the ball game or the mall with their brace on? Accuracy in the Prediction and Estimation of Adherence to Bracewear Before and During Treatment of Adolescent Idiopathic Scoliosis os s Birch, et al. Journal of Pediatric Orthopaedics. Vol 28:3. April/May 2008. Methods 124 patients with AIS fitted with temporary sensor in Boston Brace Brace-Belief Belief questionnaire to measure adherence Patient, Parent, Doctor, Orthotist Results Patients wore brace 47 % of the time Estimations are much higher Conclusions It is difficult to predict adherence before and during treatment 9

Standardization of Criteria for AIS Brace Studies: SRS Inclusion Criteria Adolescent Idiopathic scoliosis (Age 10 or older) Immaturity (Risser sign 0,1,2) Primary curve between 25-40 No previous treatment If female, premenarche Intent to treat not just compliant patients SRS Orthotic Effectiveness Criteria 5 or less/6 or more of Curve Progression at maturity % patients progression over 45 % patients who had surgery or surgery reccomended Minimum of 2 year follow-up Providence Brace Studies Nighttime Bracing with Providence Brace in Adolescent Girls with Idiopathic Scoliosis D Amato et al. Spine. 2001. A Comparison of the TLSO and Providence Orthosis in Treatment of Adolescent Idiopathic Scoliosis Thompson et al. Journal of Pediatric Orthopaedics. June 2007. Providence Brace Study: Preliminary Results- Dayton, OH Providence Brace D Amato et al 102 females with AIS treated in Providence Brace. Compared to natural history data 29% progressed over 5º Risser 0-1 (versus 68 %) and 17 % over 5º Risser 2-4 (versus 23%). Providence Brace D Amato et al Percent Success of the Different Curve Types Only 24% of all patients with an apex T8 to L1 progressed, and 94% of lumbar curves were successful. Thompson et al. JPO. June 2007. A comparison of the Thorocolumbosacral orthoses and Providence Orthosis in Treatment of AIS Use of SRS Inclusion and Assessment Criteria for Bracing Studies 83 of 160 pts. Met SRS Criteria TLSO Group 15 % did not progress Providence Group 31% did not progress Initial curves 25-35 Providence Brace more effective Question Effectiveness of Bracing overall (need multicenter randomized study) 10

Providence Brace Treatment for Adolescent Idiopathic Scoliosis: Intermediate Results in a Community Setting Jim Lehner, M.D. Mike Albert, M.D. Cyndi King, C.P.O. Dayton Children s Overall Failure Rate as defined by SRS Effectiveness Criteria 38% Cooperative 8/62 13% Cheaters 10/17 60% Nogoodnicks 20/22 91% *Included lost to follow-up as failure Overall Failure Rate Immature Compliant Patients 1/29 (3.4%) TREATMENT ADOLESCENT IDIOPATHIC SCOLIOSIS Observation Under 20 Brace If Curve in 30 s and Immature Brace If Document Progression Surgery Curves >50 GUIDE TO REFERRAL TO ORTHOPAEDIC SPECIALIST Age 0-10 10-20 Over 20 Pre-Pubertal Re-Check 6 mos. Re-Check 4 mos. Refer to Orthopaedics Pubertal Re-Check 1 yr. Refer if Progression >5 Congenital Scoliosis Hemivertebrae Failure of Formation Bar Failure of Segmentation Post-Pubertal No Follow-up Follow-up 1 yr. 11

Neuromuscular Scoliosis Cerebral Palsy Muscular Dystrophy/SMA Neurofibromatosis Myelomeningocele Connective Tissue Disorders Ehter s Danlos Marfan s Syndrome Anatomy-Think 3-D Vertebral Rotation Other Disorders Osteogenesis Imperfecta Klippel Feil Syndrome Miss Idaho 2009 ADULT SEQUELAE OF UNTREATED SPINAL DEFORMITY Review Natural History of Untreated Patients 12

Adult Sequelae Study Number Follow-up Mortality Nilsonne/ 113 50 yrs. 2x Lundgren (90%) Nachemson 130 35 yrs. 2x (90%) Collis/Ponseti 353 24 yrs. Adult Sequelae Study Never Disability Back Pain Married Nilsonne/ 76% 47% 90% Lundgren Nachemson - 30% 40% Collis/Ponseti - - 54% Spine,Chest and Rib Deformities add to decrease Pulmonary Functions Significant Curves get worse in adulthood Increased Mortality? Yes in severe curves: Neuromuscular Curves Curves starting in Infantile or Juvenile age group Congenital Curves Basically, in curves that have the ability to get over 100 degrees 76% of females with curves over 60 degrees never get married 13

ADULT SEQUELAE OF UNTREATED SPINAL DEFORMITY Thoracic Curve of 60 or more: Pulmonary Function Progression Lumbar Curves of 50 or more: Back Pain Progression DECISION FOR SURGERY Goals of Surgery Magnitude of curve Risk of Progression Spinal Imbalance 1. DO NO HARM 2. Obtain Solid Fusion 3. Improve Spinal Balance (coronal and sagital) 4. Fuse lowest number of segments possible RISKS OF SURGERY Neurological Bleeding Infection Implant Failure/Failure of Spine to Fuse G.I. Complications PRE-OPERATIVE PLANNING Pre-Op talk with Physician Pre-Op Blood Donation Special X-rays 14

PRE-OPERATIVE PLANNING Pre-Op Evoked Potentials Pre-Op Consultation Nutrition Pulmonary Cardiology INTRA-OPERATIVE Special Lines Evoked Potentials: Multi Modality Spinal cord monitoring Spinal Frame Patient Position Wake Up Test Casting for Post-Op brace Wake-Up Test 15

Surgical Techniques Laminar Hooks Surgical Techniques Rod and Clip Insertion Surgical Techniques Pedicle Screws Surgical Techniques Sublaminar wires, cables, universal clamp Surgical Techniques U-Clamp System Transverse Process Option Lamina Option 16

0 +15 60 10 Isola Hybrid Construct POST-OPERATIVE Pain Control ICU Overnight 5 7 Days in Hospital Out of Bed Once Brace is Ready POST-OPERATIVE ACTIVITY Out of School Minimum 2 Weeks Activity Sheet with Instructions GOAL: To Resume Previous Sporting Activities by Six Months to One Year Advances in Spinal Instrumentation VEPTR Vertical Expandable Prosthetic Titanium Rib Growing Rod Distractible Rod Pedicle Screw Instrumentation Universal Clamp Universal Clamp in Treatment of Pediatric Spinal Deformity Retrospective Review 22 Cases 196 Clamps 196 Clamps In 6-15 months 17

Diagnostic Categories Idiopathic Scoliosis (8 Cases/25 Clamps) Congenital Scoliosis (3 Cases/28 Clamps) N-M Deformity, including Scoliosis Kyphosis and Lordosis (8 Cases/114 Clamps) Spondylolyis/Spondylolisthesis (3 Cases/ 6 Clamps) Indications for the Universal Clamp Dysplastic Pedicles on the concave side of scoliotic curves Fractured pedicles Hypokyphosis in the Thoracic Spine Neuromuscular Lumbar Lordosis Osteoporotic Bone Back Up of Pedicle screws/hooks Fusionless surgery for congenital and early onset scoliosis Complications None in Idiopathic Group No neurologic complications One feeding tube (CP Patient) SMA Syndrome (congenital scoliosis) Two superficial wound dehiscences (SMA and Myotubular Myopathy) Summary: Universal Clamp Another Tool in the Box for Spinal Deformity Surgery Superb in Neuromuscular Scoliosis and Kyphosis correction New Technique for Fixation Spondylolysis Superior Sublaminar Implant Excellent choice in osteoporotic bone Spinal Muscular Atrophy 18

SMA: Post Op Case Presentations: Neuromuscular Scoliosis with Kyphosis BS is a 15 year old male with congenital hydrocephalus and progressive scoliosis/kyphosis Ambulatory but developmental delay MRI-No cord abnormalities Pre-Op Bending 23º 43º 82º 43º 40 º Prone Hyperextension 82 º 51º Pre-Op Planning Hyperextension Lateral to Access Flexibility Multilevel Osteotomies (ponte type) Posterior Approach (versus Ant/Post) 19

Intra-Op Photos Post-Op 35º (57%) 0º (100 %) Post-Op Photos Before & After 20

Kyphosis Video 21