Early onset scoliosis

Early onset scoliosis

  1. 1. H Mehdian MD , FRCS Consultant Spinal Surgeon The Centre for Spinal Studies and Surgery, Queen’s Medical Centre, Nottingham Management of Paediatric Spinal Deformity
  2. 2. Paediatric Spinal Deformity 1) Early Onset Scoliosis 2) Late Onset Scoliosis a) Idiopathic Scoliosis b) Neuromuscular Scoliosis c) Congenital Scoliosis d) Syndromic Scoliosis
  3. 3. Classification Idiopathic Scoliosis  Old Classification  Infantile Onset < 3 yrs Age  Juvenile Onset 3-10 yrs Age  Adolescent Onset > 10 yrs Age  New Classification Early onset Onset < 8 yrs Age Late onset Onset > 8 yrs Age Approximately 80% of patients with scoliosis have idiopathic scoliosis Classification
  4. 4. Early Onset Scoliosis Definition Curves greater than 10 degrees in a child under the age of five years are defined as early onset scoliosis, this umbrella term includes: 1) Idiopathic Scoliosis 2) Neuromuscular Scoliosis 3) Congenital Scoliosis 1) Syndromic Scoliosis
  5. 5.  The first five years of life are crucial as the lungs are still growing dramatically  Constriction of the chest cavity as a result of a spinal deformity significantly restricts lung growth and may contribute to serious pulmonary complications Early Onset Scoliosis
  6. 6. Early Onset Scoliosis Scoliosis  New classification reflects the importance of lung development up to age 7  At birth 20 million alveoli  Age 5 300 million alveoli Early Onset
  7. 7. Lung development 4 8 300 250×10⁶200 100 Age(year)Birth No.ofalveoli(x10⁶) 300×10⁶ 20×10⁶ Early growth disturbance compromises thoracic volume Early Onset Scoliosis
  8. 8. Idiopathic Scoliosis  75% of the curves , resolve spontaneously  25% of the curves, progress very rapidly (malignant) • Identify the malignant curves, prevent development of respiratory compromise  Features • Male • Left sided Thoracic curve Early Onset Scoliosis Early Onset
  9. 9. Early onset Idiopathic Scoliosis Predicting Progression Early Onset Scoliosis  Rib vertebral angle measurement and its significance was brought in by Min Mehta  Measurement of this angle has an important implication in infantile Idiopathic scoliosis as it differentiates between progressive and resolving type of scoliosis  A curve with an initial RVAD of 20° or more is considered progressive RVAD > 20° Mehta 1972
  10. 10. Predicting Progression RVAD > 20° Mehta 1972 Convex RVA < 68° Kristmundsdottir 1980 80% 85%
  11. 11.  Observation • Differentiate between Resolving Curves, progressive  Bracing, serial casting • Is an attempt to stop progression of the curve but does not improve the curve. Early onset Idiopathic Scoliosis Treatment Early Onset Scoliosis
  12. 12. Operative Treatment • Convex Stapling Smith 1954 • Posterior Fusion Scott 1956 • Unilateral Growth Arrest Roaf 1963 • Concave Costoplasty Piggott 1971 • Convex Epiphysiodesis Roaf 1977 • Segmental Instrumentation Luque 1980
  13. 13. Convex Epiphysiodesis
  14. 14. Early Onset Scoliosis Convex Epiphysiodesis & Luque trolley
  15. 15. 46.5mm 27.3mm Age at 3 yrs Age at 9 yrsAge 3 Spinal Growth 2 cm
  16. 16. 2 cm growth for 6 years 27.3mm 46.5mm Growth per year 3 mm Growth per level 0.25 mm
  17. 17. Conclusion • It appears that convex epiphysiodesis has a tethering effect on growth phenomenon and therefore should be avoided when growing constructs are used.
  18. 18.  Surgery progressive curves (Growing construct) Spinal fusion is not an option, because the thoracic height would cease to develop and lung development would be restricted Early onset Idiopathic Scoliosis Treatment Early Onset Scoliosis
  19. 19. Consequences of Premature Spinal Fusion in a Case of Early Onset Scoliosis Centre for Spinal Studies and Surgery Nottingham 15 year old female  4 months Serial Casting  3 years Uninstrumented Convex Epiphysiodesis  7 years Post Fusion T3-L3
  20. 20.  Residual spinal deformity  Back pain & costo-pelvic impingement  Breathlessness & difficulty of ambulation  Disproportionately small thorax  FVC 17% of predicted value Centre for Spinal Studies and Surgery Nottingham Main Complaints & Clinical Examination
  21. 21. Centre for Spinal Studies and Surgery Nottingham
  22. 22.  Casting Failed to control progression of the deformity  Premature fusion Resulted in thoracic & respiratory insufficiency  Surgical options now Too late for surgery to effect respiratory function? Will respiratory function permit further surgery?  Outcome is premature death • A • SSSurgi Centre for Spinal Studies and Surgery Nottingham What to do next, if anything?
  23. 23. Early Onset Scoliosis Double Growing Rod Construct VEPTER Thoracic insufficiency syndrome , cog chest Wall deformities Shilla Construct Hybrid Construct H Bar Construct Growing Rod Constructs Vertical Expandable Prosthetic Titanium Rib Distraction Growth Guided
  24. 24. Early Onset Idiopathic Scoliosis
  25. 25. Early Onset Idiopathic Scoliosis
  26. 26. Early Onset Idiopathic Scoliosis
  27. 27. Early Onset Idiopathic Scoliosis
  28. 28. Early Onset Neuromuscular Scoliosis Neuromuscular Spinal deformity develops in majority of the Patients with neuromuscular disease  Deformity Early in life  Progression Common
  29. 29. Classification (SRS) EONMS Upper Motor Neuron  Cerebral Palsy  Syringomyelia  Spinal Cord Trauma  Spinal Cord Tumour  Spinocerebellar Degeneration Neuropathic CP
  30. 30. Classification (SRS) EONMS Neuropathic Lower Motor Neuron  Spinal Muscular Atrophy  Poliomyelitis  Traumatic  Dysautonomia SMA
  31. 31. Classification (SRS) EONMS Myopathic  Muscular Dystrophy  Congenital Hypotonia  Myotonia Dystrophica  Arthrogryposis  Fibre Type Disproportion CMD
  32. 32. Classification Brown & Swank Location of the lesion in the neuromuscular system Abnormal Muscle Activity (Scoliosis) Spastic Brain, Cerebellum, Upper motor neuron Flaccid Anterior horn cell, Motor neuron , Primary muscle diseases EONMS
  33. 33. General Principles Neuromuscular  Progression  Pulmonary dysfunction  Cardiomyopathy  Urinary tract disease  Pressure sore  Hip dislocation Patients with neuromuscular scoliosis present with significantly different and more complicated problems than those with idiopathic scoliosis
  34. 34. Neuromuscular  Osteoporosis  External support  Post-operative ventilatory support  Post-operative management  Blood loss The management of these patients by surgical means may be associated with much greater risk Surgical Risks
  35. 35. Neuromuscular  Correction of the spinal deformity  Maintain the correction during the growth period  Allow spinal growth and lung development  Prevent progressive deterioration of P.F.  Avoid the need for definitive fusion at an early age Goals of Surgery
  36. 36. Neuromuscular  Observation  Orthosis  Operation The modalities of treatment in neuromuscular scoliosis are similar to those of idiopathic scoliosis Treatment
  37. 37. Neuromuscular  Attempts at prophylactic or early bracing have not prevented curve development and progression.  An immediate effect of this will be to cause difficulty breathing and restrict growth of the chest wall in the longer term. Orthosis & Seating Systems
  38. 38.  Arterial and central lines  Urinary catheter  Temperature probe  Blood warmer  Heating blanket  Controlled hypotension  Cell saver  Spinal cord monitoring Experienced anaesthetic input is essential in major spinal surgery Surgery
  39. 39. Neuromuscular Pre-op Assessment  Chest Physician  Neurologist  Anaesthetist  Physiotherapist  Spinal Surgeon Multidisciplinary review Early Onset Neuromuscular Scoliosis
  40. 40. Spinal Muscular Atrophy  Autosomal recessive  Degeneration of anterior horn cells  Single gene responsible (chromosome 5) Scoliosis is present in more than 70% of patients with SMA. Those with type 2 and 3 are the most common group presenting to spinal surgeons and often present at a young age.
  41. 41. Neuromuscular Type 1 : Acute Infantile (WH) Onset 6 months Type 2 : Chronic Infantile (WH) Onset 4 years Type 3 : Juvenile form (K W) Onset 2-15 years Type 4 : Distal SMA Onset 7-15 years Spinal Muscular Atrophy
  42. 42. Various surgical techniques have been employed to treat EONMS Limitations :  Lack of segmental control  Loss of sagittal balance  Multiple surgeries  High rate of complications EARLY ONSET NEUROMUSCULAR SCOLIOSIS
  43. 43.  I have been using two different definitive growing rod constructs based on sublaminar wiring system  These constructs have enabled me to achieve and maintain the correction during spinal growth. Sliding H Bar Construct Hybrid Construct Definitive Self Growing Rod Constructs
  44. 44. Screw + Wire Construct Sliding H Bar Construct Self Growing Rod Constructs
  45. 45. Neuromuscular Segmental Spinal Instrumentation using short closed wire loops H Mehdian, Clinical Orthopaedics, 1989, 247
  46. 46. Sliding H-Bar Construct Self Growing Rod Constructs (SMA)
  47. 47. 1994 Spinal Muscular Atrophy 1996 1997 30°10° Age 7
  48. 48. 19981997 1999 2000 Age 7 Age 9Age 8 Age 10 Spinal Muscular Atrophy
  49. 49. 2001 2002 2010 2010 Age 11 Age 20Age 12 Age 20 Post- op: 13 yrs Spinal G: 12 cm PJK: – Preserved: TK, LL Surgery: 1 Spinal Muscular Atrophy
  50. 50. 19981997 1999 2000 2001 2002 2010 2010 Spinal growth ( 12cm) Age 7 Age 8 Age 9 Age 10 Age 11 Age 12 Age 20 Age 20 Spinal Muscular Atrophy
  51. 51. Screw + Wire Construct Spinal Muscular Atrophy
  52. 52. AD Spinal Muscular Atrophy
  53. 53. 90˚ 80˚ A D Spinal Muscular Atrophy
  54. 54. Spinal Muscular Atrophy
  55. 55. AR Spinal Muscular Atrophy
  56. 56. 65˚ Type 2 A R Spinal Muscular Atrophy
  57. 57. 07.10.2009 18.3 mm 19.03.2012 44.7 mm 26.4 mm  2½ years Spinal Growth
  58. 58. TC Congenital Hypotonia
  59. 59. T C Congenital Hypotonia
  60. 60. 50° 60° 80° T C Congenital Hypotonia
  61. 61. A & S B Congenital Muscular dystrophy
  62. 62. AB Congenital Muscular dystrophy
  63. 63. AB 70˚ 60˚ Congenital Muscular dystrophy
  64. 64. SB Congenital Muscular dystrophy
  65. 65. 56⁰ 70⁰ Congenital Muscular Dystrophy
  66. 66. 07.10.2009 19.03.2012Spinal Growth 30 mm3 years
  67. 67. 07.10.2009 15.09.2012 Spinal Growth 30 mm3 years 22mm 52mm
  68. 68. 4 Years 4 cm Growth
  69. 69. 29.09.2008 20.01.2014 4 Years and 8 months 5 cm Growth
  70. 70. Definitive growing construct appears to be more advantageous over other systems in patients with early onset neuromuscular scoliosis, it eliminates the need for further surgeries  The ideal design of implants for the treatment of patients with EOS should have the following characteristics:  Eliminates the need for recurrent lengthening  Provides good fixation  Maintains sagittal curvature of the spine Conclusion
  71. 71. 6th International Congress on Early Onset Scoliosis & Growing Spine November 15-16, 2012- Dublin, Ireland Best Paper
  72. 72. Early Onset Congenital Scoliosis The spinal column develop at the same time as several other major organ systems such as the bladder, kidneys and heart.
  73. 73. Cong Scoliosis Classification Centre for Spinal Studies and Surgery Nottingham Based on the embryological development of the spine  Defects of Formation  Defects of Segmentation  Mixed
  74. 74. • MRI shows abnormalities in 26% • Syrinx • Chiari Malformation • Tether cord • Diastomatomyelia • Single kidny Imaging / Associations
  75. 75. Neuromuscular Congenital Scoliosis Neuraxial Anomalies
  76. 76. Bracing • Proven little value for congenital curves Indication for Surgery • Unacceptable deformity • Bar + contralateral hemivertebra • Progression >10° in one year
  77. 77. Surgical Options • Posterior fusion • Anterior and posterior fusion • Convex hemiepiphyseodesis • Posterior vertebral resection
  78. 78. Classification  Failure of formation: Means one or more vertebrae become partially or fully triangular  Failure of segmentation: Means one or more vertebrae are abnormally connected together
  79. 79. Failure of formation Fully Segmented IncarceratedNonsegmented Semi Segmented Wedge Vertebra
  80. 80. Failure of Segmentation Bilateral Failure of Segmentation Unilateral Failure of Segmentation,unsegmented bar Unilateral Failure of Segmentation, unsegmented bar + contralateral hemi vertebra
  81. 81. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation A lateral defect of vertebral formation can vary from mild wedging to the complete absence of half of the vertebra (Hemivertebra) A hemivertebra is one of the most common causes of congenital scoliosis Hemivetebra consists of half of the vertebral body, a single pedicle, and hemi-lamina
  82. 82. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Hemivertebra Four different types of hemivertebrae  Fully Segmented Most common  Semi segmented Less common  Non-segmented Least common  Incarcerated Least common
  83. 83. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Fully Segmented Hemivertebra  At the thoracolumbar region the deformity can exceed 45⁰ at skeletal maturity  Cosmetic deformity is moderate  At the lumbosacral junction, cosmetic deformity is major
  84. 84. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Fully Segmented Hemivertebra  Two fully segmented hemivertebrae on the same side of the spine are less common  All exceed 50⁰ by 10 years of age  Without treatment these could reach 70⁰ by skeletal maturity  Two opposing fully segmented hemivertebrae are more common
  85. 85. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Semi-Segmented Hemivertebra  Is synostosed to its neighbouring vertebra  Two growth plates are obliterated on this convexity  Can induce a slowly progressive scoliosis  Curves usually do not exceed 40⁰ at skeletal maturity  Treatment is required if hemivertebra occurs at the lumbosacral junction
  86. 86. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Non-Segmented Hemivertebra  Is synostosed to both of its adjacent vertebrae  Has no growth potential  There is no cosmetic deformity  No treatment is required
  87. 87. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Incarcerated Hemivertebra  Ovoid in shape  Smaller than a fully segmented hemivertebra  The vertebrae above and below tend to compensate for the hemivertebra  There is minimal scoliosis  Scoliosis rarely exceeds 20⁰ at skeletal maturity
  88. 88. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Formation Wedge-vertebra  Rare cause of congenital scoliosis  Partial failure of a vertebra to form on one side  Scoliosis deteriorates relatively slowly  Surgical treatment may occasionally be required
  89. 89. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Associated Deforming Features Upper thoracic curve: Significant cosmetic deformity A 30⁰ curve upper limit of acceptability
  90. 90. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Segmentation unilateral Unsegmented bar
  91. 91. Cong Scoliosis Defects of Segmentation Centre for Spinal Studies and Surgery Nottingham  The unsegmented bar does not contain growth plates  Some degree of growth continues on the opposite side  Rib fusions are often seen adjacent to the unsegmented bar Unilateral unsegmented bar
  92. 92. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Segmentation Unilateral unsegmented bar  On average, these curves deteriorate at a rate of 5⁰ a year  The great majority will exceed 50⁰ by 10 years of age
  93. 93. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Segmentation Unilateral unsegmented bar with contralateral hemivertebrae  Is seen most clearly in the first few years of life  This type of anomaly produces the most severe and rapidly progressive of all types of congenital scoliosis
  94. 94. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Segmentation Unilateral unsegmented bar with contralateral Hemivertebrae On average, these curves deteriorate 6⁰ or more a year All exceed 50⁰ by 4 years of age If untreated, at an early age can lead to cor pulmonale
  95. 95. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Treatment Defects of Segmentation Unilateral unsegemented bar with or without contralateral hemivertebrae Fusion should be performed as soon as the anomaly is recognized The best results are achieved when surgery is performed before the age of 2 years
  96. 96. Cong Scoliosis Centre for Spinal Studies and Surgery Nottingham Defects of Segmentation Bilateral Block Vertebra
  97. 97. Congenital Kyphosis • Type I: Failure of formation (hemivertebra) • Rapidly progressive • May produce paraplegia • Type II: Failure of segmentation (bar) • Less progressive • Does not produce paraplegia
  98. 98. Congenital EO Scoliosis
  99. 99. Congenital Early Onset Scoliosis
  100. 100. Congenital EO Scoliosis
  101. 101. Congenital Kyphosis Prognosis • Most patients have slight short stature • 70% risk of progression • Progression greatest after 10 years of age • May cause cor pulmonale • Congenital kyphosis may cause paralysis
  102. 102. Congenital Kyphosis
  103. 103. Neuromuscular Management of Kyphosis (Kyphectomy)  Kyphectomy
  104. 104. Congenital Early onset Scoliosis
  105. 105. Early onset Congenital Scoliosis
  106. 106. Spinal Growth • Averages 0.07 cm/year per segment • Anomalous segments will never have this degree of growth • Spinal growth is two thirds complete by age 6
  107. 107. Early onset Congenital Scoliosis
  108. 108. Early onset Congenital Scoliosis
  109. 109. Rhys Hart, 12 y/o M K1738918 10 cm T11 PVCR T2–Ileum segmental instrumented fusion
  110. 110. 120º 72º
  111. 111.  The Segmental Self Growing Rod Construct is a powerful, definitive technique for the management of early onset scoliosis Construct Advantages:  Excellent correction  Maintain the correction during growth period  PJK is prevented  Sagittal contour of the spine is well preserved  Maximum spinal growth and thoracic development is achieved just with one surgery Conclusion
  112. 112. Adolescent Idiopathic Scoliosis Natural History • Progression related to maturity and curve size • Risk of progression increases strongly at in an immature patients • Curves> 45° should be considered for surgery • Pulmonary compromise > 75° to 100°
  113. 113. Initial Evaluation Whole Spine X-rays (curve measurement) Regular outpatient review
  114. 114. Progression more than 5° in curves between 20°-25° in patients with ( Risser 0-3) Brace treatment for curves > 40° to 45° has very lower success rate Brace Indications
  115. 115. Brace Types • Thoracolumbosacral (TLSO) TLC • Milwaukee Brace high thoracic curves • Charleston Night Brace Single curves 25°
  116. 116. Preoperative assessment MRI indicated if: Left Lower thoracic curve Significant back or neck pain Neurological abnormality Less than 10 years of age
  117. 117. Late Onset Scoliosis Lenke Classification  The Lenke Classification is a complex, accurate and reproducible . It relies on measurements taken from standard x-rays  X-rays of the patient from the AP, LAT, and in bending positions are measured and evaluated Each scoliosis curve is then classified in three ways
  118. 118. Late Onset Scoliosis Lenke Classification Curve type (1-6) : proximal thoracic, main thoracic and thoracolumbar/lumbar Lumbar modifier (A, B, C) lumbar modifier based on the distance of the center of the lumbar spine to the midline
  119. 119. Late Onset Scoliosis Lenke Classification Sagittal thoracic modifier (-, N, +) Sagittal thoracic modifier based on the amount of thoracic kyphosis The most common type is a 1AN curve
  120. 120. Type 1 B,N ( Main Thoracic) Seg Screw Fixation 68º
  121. 121. Type 1 B,N (Main Thoracic) Seg Screw Fixation 85º
  122. 122. Type 1C,N (Main Thoracic ) Seg Screw Fixation 90º 60º
  123. 123. Type 3C,N ( Double Major ) Seg Screw Fixation 78º 72º
  124. 124. Type 3 C,N (Double Major) 3C Seg Screw Fixation 78º 75º
  125. 125. Type 6C,N (Thoracolumbar) Seg Screw Fixation 85º 75º
  126. 126. Image Free technique Type 6C,N (Thoracolumbar)
  127. 127. Seg Screw Fixation Type 3C,N ( Double Major ) 80º 78º
  128. 128. Seg Screw Fixation Type 1 C,N (Main Thoracic)
  129. 129. Seg Screw Fixation
  130. 130. Seg Screw Fixation
  131. 131. Seg Screw Fixation
  132. 132. Paediatric spinal deformity surgery should be performed in a specialist centre where a high volume of procedures are performed  Good medical support staff, including experienced paediatric anaesthetists, are an essential part of the team dealing with these children with deformity Conclusion
  133. 133. Centre for Spinal Studies and Surgery Nottingham
  134. 134. EOS TO REMAIN NORMAL • Weight: 40 kg • T1-T12= 22 cm • VC: more than 50%
  135. 135. Early Onset Syndromic Scoliosis Prader willi Syndrome
  136. 136. Early Onset Syndromic Scoliosis
  137. 137. Early Onset Syndromic Scoliosis

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