Thoracovertebral malformations

Transcription

1 Thoracovertebral malformations Cardiopulmonary consequences and treatment June 2016 SwissTech Convention Center Lausanne Daniel Trachsel Pulmonology & Intensive Care University Children s Hospital Basel

2 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

3 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

4 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

5 Lung asymmetry in EOS n= =39, age 1-14yrs, 25 congenital (64% w/fused ribs) Redding G. Spine J 2008;8:639

6 Lung function in early onset scoliosis Long term outcome of spinal fusion for EOS 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

7 Spine and chest: disynaptic growth cm Axial Growth Chest growth Sitting hight Lower limb yrs Campbell RM. J Bone Joint Surg 2007;89:S108

8 300 Mio. alveoli for all of us? Estimates of alveolar #: historical data Narayanan M. AJCCM 2012;185:186

9 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 präop. adapt. CC Hasler, UKBB postop.

10 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 cc 3 (63%) 5.2yrs Jeune 573 cc 3 36 mo 702 cc cc 3 (23%) 7 mo, EOS fused ribs 216 cc 3 33 mo 409 cc cc 3 (89%) 3 yrs, Jarco-Levine 553 cc 3 36 mo 903 cc cc 3 (63%) 3.3 yrs, myelodysplasia 422 cc 3 35 mo 531 cc cc 3 (26%) 249 cc 3 3 yrs 636cc cc 3 (53%) Gollogly S J Pediatr Orthop 2004;24: % Gollogly S Spine 2004;29:2061

11 Lung function after serial VEPTR expansion Average lung growth following percentiles Motoyama EK et al. Spine 2006; 31,

12 Lung function after VEPTR expansion: older kids n = 53; 33 congenital constrictive chest wall syndrome (CCCW) 20 progressive spinal disorder VEPTR implant yrs ( ), F/U after 8 5 mo pre-veptr post-veptr p FVC 62 4 % 54 3 % < 0.01 FEV % 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, cf. Gadepalli SK et al. J Pediatr Surg 2011;46:77

13 Chest development infants Openshaw P et al. Thorax 1984; 9,624 adults

14 Lung function after VEPTR: summary Growth of chest volume along percentiles (LuFu, 3D-CT) Motoyama EK et al. Spine 2006; 31, 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

15 Idiopathic scolioses Definition: Spinal angulation >10 = scoliosis Scoliosis research society Thoracic Thoracolumbar Lumbar Apex =maximal translation adapt. CC Hasler, UKBB

16 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

17 Lung asymmetry in scoliosis Rib distorsion 0% 11% Closkey RF. J Biomechanics 1992;25:529 47%

18 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

19 Risk of scoliosis progression Premenarchal 100 % 0-2yrs post menarche > 2 yrs post menarche Surgery Surgery 80 Brace Observe Cobb adapt. CC Hasler, UKBB

20 Corrective moments in scoliosis axial load relative correktive moment transverse load Cobb-angle Braces work by transverse forces, not effective >40-50 Cobb angle adapt. CC Hasler, UKBB

21 Pulmonary function impairment in AIS Hallmark: Thoracic restriction with elevated RV/TLC Donath J. Semin Respir Crit Care Med 2009;30:275

22 Pulmonary function impairment in AIS n = 858 (682 f), age 14.7 yrs (range ), pre-op PFT % % % % % % Mild impairment possible with all Cobb angles Increasing impairment with Cobb angles > Johnston CE. Spine 2011;36:1102

23 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

24 Effect of surgery on pulmonary function Slight reduction in spirometry post spinal fusion FVC (%) FEV1% (L/s) TLC (%) Preoperative 86 ± ± ± 14 Postoperative 80 ± ± ± 15 p-value < < NS n = 254, 2 yrs post surgery Newton PO. Spine 2007;32:1875

25 Cardiorespiratory fitness in AIS Barrios C. Spine 2005;30:1610

26 Muscle dysfunction in AIS n=60, age 20±3 yrs, thoracic Cobb 56±10, lumbar Cobb±16 Martininez-Llornes J. ERJ 2010:36:393

27 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

28 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

29 Thank you