Thoracovertebral malformations Cardiopulmonary consequences and treatment 15 17 June 2016 SwissTech Convention Center Lausanne Daniel Trachsel Pulmonology & Intensive Care University Children s Hospital Basel
Scoliosis in childhood Categorization of childhood scoliosis by Pathogenesis < 1% congenital 10% neuromuscular 90% idiopathic (infantile, juvenile, adolescent) Age at onset Curves > 70% Outcome earyl onset EOS (< 5 years) 86% High mortality juvenile (5 10 years) 69% survival adolescent (> 10 years) 46% 100% survival Koop SE. Orthop Clin North am 1988;19:331. Karol SE. Clin Orthop Relat Res 2011;469:1323
Outcome of untreated childhood scoliosis Excessive death rates in old cohorts: mean age at death 54 years Causes of death: - restrictive lung disease alveolar hypoventilation PHT cor pulmonale - infection Pehrsson K. Spine 1992;17:1091
Thoracic Insufficiency Syndrome (TIS) TIS I Unilateral missing ribs with thoracic instability (flail chest) TIS IIIa Bilaterally fused ribs vertebrale malformation stiff hypoplastic chest TIS II Unilaterally fused ribs with stiff and hypoplastic chest TIS IIIb Hypoplastic stiff chest normal spine Trachsel D. In: v. Mutius. Pädiatrische Pneumologie. Springer Heidelberg 2014 Campbell RM. Spine 2009;34:1815
Lung asymmetry in EOS n= =39, age 1-14yrs, 25 congenital (64% w/fused ribs) Redding G. Spine J 2008;8:639
Lung function in early onset scoliosis Long term outcome of spinal fusion for EOS Age @ F/U FVC%pred FEV1%pred TLC%pred RV%pred Spinal fusion before age 10 years (n=10) 20.5 ± 5% 41 ± 20% 41 ± 20% 50 ± 15% 88 ± 25% Spinal fusion after age 10 years (n=6) 20 ± 4% 68 ± 16% 72 ± 24% 72 ± 16% 84 ± 24% Goldberg CJ et al. Spine 2003;28:2397
Spine and chest: disynaptic growth cm Axial Growth Chest growth Sitting hight Lower limb 29 18.5 29 25.5 13 10 0 5 13 17 yrs Campbell RM. J Bone Joint Surg 2007;89:S108
300 Mio. alveoli for all of us? Estimates of alveolar #: historical data Narayanan M. AJCCM 2012;185:186
Severe congenital scoliosis, fused ribs, unilateral bar, hemivertebra, high insertion diaphragm V ertical E xpandable P rosthetic T itanium R ib geplante Durchtrennung der Rippen E.G., w. 1+11 präop. adapt. CC Hasler, UKBB postop.
CT-evidence of pulmonary growth after VEPTR Age preop, Dx LV init time LV F/U LV 4.8 yrs, absent ribs 380 cc 3 35 mo 635 cc 3 +255cc 3 (63%) 5.2yrs Jeune 573 cc 3 36 mo 702 cc 3 +129cc 3 (23%) 7 mo, EOS fused ribs 216 cc 3 33 mo 409 cc 3 +192cc 3 (89%) 3 yrs, Jarco-Levine 553 cc 3 36 mo 903 cc 3 +350cc 3 (63%) 3.3 yrs, myelodysplasia 422 cc 3 35 mo 531 cc 3 +109cc 3 (26%) 249 cc 3 3 yrs 636cc 3 +207cc 3 (53%) Gollogly S J Pediatr Orthop 2004;24:323 +50-60% Gollogly S Spine 2004;29:2061
Lung function after serial VEPTR expansion Average lung growth following percentiles Motoyama EK et al. Spine 2006; 31, 284-290
Lung function after VEPTR expansion: older kids n = 53; 33 congenital constrictive chest wall syndrome (CCCW) 20 progressive spinal disorder Age @ VEPTR implant 9.1 2.5 yrs (4.8 15.2), F/U after 8 5 mo pre-veptr post-veptr p FVC 62 4 % 54 3 % < 0.01 FEV1 59 4 % 52 2 % < 0.01 TLC* 66 5 % 66 4% NS RV/TLC* 35 3 % 42 4% 0.01 * n = 10 Mayer OH et al. Spine 2006; 31, 284-290 cf. Gadepalli SK et al. J Pediatr Surg 2011;46:77
Chest development infants Openshaw P et al. Thorax 1984; 9,624 adults
Lung function after VEPTR: summary Growth of chest volume along percentiles (LuFu, 3D-CT) Motoyama EK et al. Spine 2006; 31, 284-290 Gollogly S J Pediatr Orthop 2004;24:323 Catch up growth of the lungs has not been documented Stimulation of spinal growth likely Flynn JM. J Pediatr Orthop 2013;33:679 Emans JB. Spine 2005;30:S58 Olgun ZD. J Pediatr Orthop 2012:32:184 Beneficial w/regard to QOL, sitting stability, thrive Hell AK et al. J Pediatr Orthop B 2005; 14, 287
Idiopathic scolioses Definition: Spinal angulation >10 = scoliosis Scoliosis research society www.srs.org Thoracic Thoracolumbar Lumbar Apex =maximal translation adapt. CC Hasler, UKBB
Chest growth Openshaw P et al. Thorax 1984; 9,624 Openshaw P et al. Thorax 1984; 9,624 Krahl VE, in Fenn WO & Rahn H eds. Williams &Wilkins 1964
Lung asymmetry in scoliosis Rib distorsion 0% 11% Closkey RF. J Biomechanics 1992;25:529 47%
Risk of AIS progression in adulthood Mortality not increased cor pulmonale in only 1/33 Back pain not increased Weinstein SL. J Bone Joint Surg 1981;63-A:702
Risk of scoliosis progression Premenarchal 100 % 0-2yrs post menarche > 2 yrs post menarche Surgery Surgery 80 Brace 60 40 Observe 20 10 20 40 60 Cobb adapt. CC Hasler, UKBB
Corrective moments in scoliosis 1 0.9 axial load 0.8 0.7 relative correktive moment 0.6 0.5 0.4 0.3 0.2 0.1 0 20 40 60 80 100 120 140 transverse load Cobb-angle Braces work by transverse forces, not effective >40-50 Cobb angle adapt. CC Hasler, UKBB
Pulmonary function impairment in AIS Hallmark: Thoracic restriction with elevated RV/TLC Donath J. Semin Respir Crit Care Med 2009;30:275
Pulmonary function impairment in AIS n = 858 (682 f), age 14.7 yrs (range 8.5 22.5), pre-op PFT % % % % % % Mild impairment possible with all Cobb angles Increasing impairment with Cobb angles > 70-80 Johnston CE. Spine 2011;36:1102
What influences pulmonary function impairment Kyphosis Rotation n = 41, age 13 yrs (8 25) Barrios C. Spine 2005;30:1610 McMaster MJ. J Spinal Disord Tech 2007;20:203
Effect of surgery on pulmonary function Slight reduction in spirometry post spinal fusion FVC (%) FEV1% (L/s) TLC (%) Preoperative 86 ± 17 81 ± 16 87 ± 14 Postoperative 80 ± 16 77 ± 14 87 ± 15 p-value < 0.001 < 0.001 NS n = 254, 2 yrs post surgery Newton PO. Spine 2007;32:1875
Cardiorespiratory fitness in AIS Barrios C. Spine 2005;30:1610
Muscle dysfunction in AIS n=60, age 20±3 yrs, thoracic Cobb 56±10, lumbar Cobb±16 Martininez-Llornes J. ERJ 2010:36:393
Muscle dysfunction in AIS n=60, age 20±3 yrs, thoracic Cobb 56±10, lumbar Cobb±16 Handgrip forces Quardiceps muscle isovolumetric contraction Martininez-Llornes J. ERJ 2010:36:393
Exercise capacity after surgery of AIS FVC 88 ± 14% 81 ± 15% pred. VO 2max. 94 ± 20% 85 ± 18% pred. Lenke LG. Spine 2002;27:2014
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