AIS. Objectives. Early onset scoliosis (0-9) Scoliosis 9/12/2018. Scoliosis Nigel Price, MD John T. Anderson, MD. Purpose/Objectives:

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1 Scoliosis Nigel Price, MD John T. Anderson, MD Children s Mercy Kansas City University of Missouri-Kansas City School of Medicine Department of Orthopaedic Surgery Section of Spine Surgery Opening Disclosure CAPS 2018 Disclosure: Drs. Price and Anderson have no actual or potential conflict of interest in relation to this program. Purpose/Objectives: Objectives AIS Defined as lateral deviation of the spine 10. In reality, a 3 dimensional deformity 1. Discuss different types of scoliosis that affect children 2. Discuss the assessment of scoliosis in children 2. Discuss the management of adolescent idiopathic scoliosis Scoliosis Idiopathic (infantile, juvenile, adolescent) Neuromuscular (CP, SB, DMD, NF-1) Congenital (due to vertebral malformations) Syndromic (Prader-Willi, Marfan) Thoracogenic (previous thoracotomy) Skeletal dysplasias Tumors Sciatic Early onset scoliosis (0-9) 14 month old 1

2 Early onset scoliosis Early onset scoliosis Thoracic height T1-12 is about 12cm at birth, 18cm at age 5 and 27cm at maturity Thoracic volume is 6% of final size at birth, 30% by age 5 and 50% by age 10 A T1-12 length of 18-22cm needed to avoid severe respiratory insufficiency Need MRI if curve > 25 deg and under age of % of infantile cases will resolve spontaneously EDF (Mehta) casting can cure if started before 2 and curve less than 50 deg Early onset scoliosis What kind of scoliosis is this? Congenital scoliosis Neuromuscular 5-7 weeks gestational age Can be rapidly progressive Renal ultrasound DMD CP 2

3 Thoracogenic Diagnosis? Chest wall injury from tornado 2 years later NF1 Syndromic scoliosis Rib dislocation Neurofibromatic scoliosis Can be idiopathic like or dystrophic Dystrophic type can be rapidly progressive Infantile Marfan syndrome What is this? Skeletal dysplasia Short stature Limb and foot deformities Cervical spine instability -MPS -Diastophic dysplasia Joint contractures 3

4 What s atypical about this curve? Convex left thoracic curve - MRI That s not her heart silhouette Tumors Tumors Osteoid osteoma most common -Night pain -Relieved with NSAIDs Osteoblastoma Neuroblastic tumors Osteoid osteoma Osteoblastoma 16 yo baseball player 16 yo baseball player Diagnosed with scoliosis Having pain in left leg No injury States he did not have scoliosis 6 months ago Note left knee flexion 4

5 16 yo baseball player What s the diagnosis? Atypical curve pattern in healthy kid No lumbar lordosis Sciatic scoliosis Leaning away from the side of nerve impingement. + Straight leg raise AIS - Prevalence 2% - 3% for curves >10 0.3% for curves >20 0.1% for curves >40 Female to male ratio: curves (1.4:1) Curves > 20 (5:1) Males are much less likely to progress Etiology Genetic component but most likely multifactorial Many other factors implicated but not proven - Melatonin deficiency - Growth hormone - Asymmetric spinal growth - Increased platelet calmodulin - Vestibulo-ocular system dysfunction Thoracic hypokyphosis Etiology Adolescent idiopathic scoliosis. Monograph Series 28. Anterior spinal growth outpaces posterior spinal growth 5

6 Not typical of AIS Screening Angle of Trunk Rotation (ATR) Identify truncal asymmetry Forward bend test -Value is often debated Referral rate: % Thoracic hyperkyphosis Scoliometer 7 is a reasonable referral point Cuts down on unnecessary referrals and missed curves > 30 Angle of Trunk Rotation Not the same as Cobb angle History Pain? Localize and quantify 23% will have pain* * 9% with identifiable cause Neurologic Symptoms Growth history (Very important) Onset of menses (girls) Axillary and facial hair (boys) Peak height velocity occurs approx 6-12 months prior to menses or axillary/facial hair in boys Family history: Idiopathic scoliosis, syndromes, dwarfing conditions Clinical Evaluation Neck and trunk motion Cutaneous lesions Gait Neuro exam, reflexes Limb length inequality *Ramirez et al. JBJS 1997; 79: 364 6

7 Leg length discrepancy Physical examination Pelvic obliquity is usually caused by LLD, not scoliosis, in an ambulatory child. Unilateral cavus foot Clinical evaluation Clinical Evaluation Diastematomyelia Clawed toes and high arch (cavus) Left thoracic curve Charcot-Marie-Tooth Clinical Evaluation Physical Examination Shoulder height Alignment Plumb line Pelvic tilt and waist Rotation Rib or scapular prominence Sagittal deformity Lower extremities - Asymmetry - Foot deformities 7

8 Radiologic Evaluation Cobb Angle Standard series 1) Standing PA C spine to hips 2) Standing Lateral Radiographic Evaluation Bone age/sander s Hand Score Iliac apophysis ossifies from lateral to medial over a year Risser 5 equates with skeletal maturity Closure of the triradiate cartilage signals the end of the peak growth spurt I generally do not brace after distal phalanx physis of middle phalanx has closed Olecranon apophysis Advanced Imaging CT - If you re worried about abnormal anatomy MRI* - Abnormal exam - Unusual curve pattern (left convex thoracic curve) - Rapid progression (> 1 /month) - Thoracic kyphosis > 20 Davids et al. JBJS 2004; 86:

9 Advanced Imaging Thoracic Kyphosis Hypokyphosis typical of AIS Ganglioneuroma Hyperkyphosis-not normal Question Answer Is it typical of AIS patients to have severe pain? NO It is not uncommon to have occasional pain but not pain that is disabling or wakes them up from sleep. Advanced Imaging 1280 patients, 274 with MRI 10% of the 274 w/ abnormal findings Loss of thoracic segment lordosis (more kyphosis) best single indicator (8/39) Optimal diagnostic yield when atypical curve pattern and neurologic findings were present (25%) 0/20 w/ pain as only indicator had + findings Davids et al. JBJS 2004; 86: 2187 Curves > 30 before maturity tend to progress Curves 50 at maturity tend to progress ~ 1 /year into adulthood Curves 80 : VC < 65% Level III Weinstein et al. JBJS 1981; 63: 702 Weinstein et al. JBJS 1983; 65: 447 Natural History 9

10 No Treatment Question Curves <25 Immature patients (Risser 0-2): Follow up in 4 to 6 months Mature patients (Risser 4-5): Follow up not needed Curves 25 to 45 in (Risser 4-5) girls: Re-evaluate in 1 year Does bracing scoliosis typically result in curve correction? Answer No It will hopefully prevent correction or at least prevent the need for surgery. Brace Indications No set rules Need to have growth remaining Not much data on bracing curves > 40 I don t typically brace after distal phalange physis have closed and/or the patient is Risser 4 Milwaukee brace Boston Brace Boston brace Introduced (1971) Low-profile brace Prefabricated Created from the full length x-rays Prevents progression; not a cure. Not effective: Curves w/ apex above T7 10

11 Charleston Brace (Night-time bending brace) Brace Regimen Well established dose dependent response Continue until maturity: 2 years post-menarche Risser 4 in females, Risser 5 in males No significant gain in height Best for thoracolumbar and lumbar curves Not that effective in males (Karol. Spine 2001; 26: 2001) Katz et al. Spine 1997; 22: 1302 Bracing Nachemson et al. JBJS 1995; 77: Multicenter, multinational, prospective study of 286 female patients - Brace group: 74% success - Observation: 34% success - Electric stimulation: 33% success Level II Bracing Katz et al. J Bone and Joint Surg 2010 Prospective evaluation of 126 subjects with AIS Used heat sensor in brace to monitor compliance 82% success with brace wear > 12 hrs/day 31% success with brace wear < 7 hrs/day Best to wear during daytime Level II Treatment 72% success with brace wear 48 % success without brace Dose dependent response *0-6 hrs = 41% *6-13 hrs = 72% *13-18 = 90% *>18 = 93% Surgical - Skeletally immature w/ curves > Skeletally mature w/ curves > 50 * Exception may be well balanced curves - Use clinical judgment as well. Level 1 11

12 Surgical Treatment Spinal Fusion Posterior spinal fusion and instrumentation - The work horse of AIS surgical treatment Goals 1) Balance head over pelvis 2) Maintain sagittal alignment 3) Do the least amount possible 4) Don t hurt the patient Long-term results Moskowitz et al. JBJS 1980; 62: year follow up of 61 patients - No difference in back pain compared to normal population Danielsson and Nachemson. Spine 2003; 28: E373 -Minimum 20 year f/u on 156 patients -Mild lumbar pain more common than in controls (65% vs. 47%) -No difference in back function and quality of life Tsutoma et al. Spine 2012; 37: year followup on 256 pts - Decreased function and self-image vs. controls - No difference in pain, mental health or low back pain Level III Natural History Weinstein et al. JAMA year follow up of untreated AIS patients with average curve 85 - Chronic back pain (61% vs 35%) - Trend towards increased SOA with activity * Curves > 80 with thoracic apex Level III New Horizons Schroth therapy -Scoliosis specific physical therapy -Developed in Barcelona -Data quality not great but showing some promise Vertebral body tethering -Fusionless treatment of AIS -Still experimental at this time Schroth New Horizons Vertebral body tethering 12

13 Thanks The Section of Spine Surgery 13