SPORC 2017 EOS/Congenital Talk

Transcription

1 SPORC 2017 EOS/Congenital Talk

2 Early Onset / Congenital Spine Deformity What Do We Know? What Are We Doing? Charles E Johnston MD TSRHC Dallas Tx Disclosures : Medtronic, Elsevier

3 EOSD / Congenital Untreated (nat l hx) -> early mortality Inappropriate treatment -> same Few conditions in orthopedics that result in death Death by Scoliosis

4 What we know. Natural Hx untreated EOS not good increased mortality Pehrsson Spine 92 Branthwaite Br J Dis Chest 86

5 Not Enough Alveoli Post-mortem studies Intrinsic problem of EOS Apparent RX > enlarge thorax early (<age 2?) Normal TIS Olsen et al 2011

6 Respiratory Death 2 o Scoliosis J Bone Joint Surg 3/03 The inability of the thorax to support normal respiration & lung growth Melvin Smith, MD

7 What we know. >70 o Natural hx large curves Increased mortality (Pehrsson) PFT s < 45% maturity

8 The basics - Fusion prior age 4-5 Goldberg ( 03).early surgery, even with anterior growth arrest did not halt the deformation of scoliosis and did not reliably preserve respiratory function in this group whose scoliosis presented before age 4. Emans ( 04) Karol ( 08) Vitale ( 08) Typical PFT s 20-50% pred. when tested 10 yr later Early Spinal Fusion

9 Well established that thoracic fusion < age 5-8 is associated with TIS Goldberg et al Spine patients < 8 yr ( ) PFT 20.5 yr. (15-30) FEV1 = 41% (14-72) FVC = 41% (12-67) If fusion delayed to age 10 PFT s = 70% mean (45-100%)

10 % FVC TSRHC study (Karol et al, JBJS 6/08) Fusion age 3.3 yr, f/u 11 yr FVC 58% (27-99) FEV1 55% (23-91) % thoracic fusion

11 Goal of RX: T1-12 length > 18 cm

12 Fusion w/o deformity control or correction = double jeopardy 1. Shortens T spine -> alveolar growth effects 2. Fails to alter chest wall dysfunction rib mobility, intercostal, diaphragm In situ fusion -> not a good idea

13 Why control deformity? Prevent spinal penetration into convexity extrinsic chest wall deformity restrictive/obstructive respiratory disease Fusion w/o correction

14 EOSD < 10 years (< 4 yr) 100 o IIS CS w/ CW Syndr/n-m

15 Hypoplastic Thorax -> T.I.S. the inability of the thorax to support normal respiration and lung growth

16 Chest Wall Deformity Congenital scoliosis w/ fused or absent ribs 1 o chest wall syndromes Jarcho-Levin IIIb Costo- IIIa cerebro-mandibular

17 Questions to ask Spine deformity vs chest wall deformity or both Natural Hx Treat now or can we delay / how bad IS it? Is there growth possible, can we harness it or do WE have to grow it? 15 mo 100 o

18 Spine deformity vs chest wall deformity or both Natural Hx Treat now or can we delay / how bad IS it? Is there growth possible, can we harness it or do WE have to grow it? 5 mo

19 Spine deformity vs chest wall deformity or both Natural Hx Treat now or can we delay / how bad IS it? Is there growth possible, can we harness it or do WE have to grow it? 21 mo

20 Congenital classify Benign BAD WORST McMaster

21 Single hemivertebra short segment fusion, curative in 1 procedure Age 2 ipo 6 yr po

22 Cong Scoli w/ fused ribs Primary chest wall deformity Thoracic fusion too extensive

23 Expansion thoracoplasty + growing devices 1 yr po 5 yr po

24 Cast Treatment IIS +? Growth modulation Delay vs Cure Tactic

25 De-rotation of thorax, not 3 point lateral pressure Morel/Mehta

26 Correction is Rotational and Not Lateral Avoid straight lateral pressure on convex ribs

27 11 mo old IIS Treat / Delay? no MRI Do both at same time

28 Cast x 1 yr, then brace

29 Curve pattern change age 2 age 5 age 6, brace d/c d

30 16 Months: wt 5.2 kg (<5%) - MRI, -CT non-cong.

31 7/02 can t cast 100 o

32 Halo-gravity Traction Treatment option + Delaying Tactic Halo Traction Applied 65 0

33 Windswept Age: 15 mos Age: 4+4 yrs penetration Cobb: T1-T12: 10.3 cm Cobb: T1-T12: 12.1 cm

34 Non-op Operative T1-12= T1-12 = 11.3 cm R 379 L 415 = 794 T1-12= 15.4 R 679 L 801 = 1480

35 Growing Rods - Indications Deformity no longer controllable by delaying tactics Significant growth remaining 2/2 age Current + predicted spine length inadequate for PFT Magic?

36 Rod fx 12/07 - revised

37 Jan 11 age 10 Broken rod #3 (+ 9 scheduled lengthenings) Decision for final traction + fusion Final improvement accepted Open TRC

38 Rod removal, final HGT -> ASF/PSF Best corrections still by HGT

39 ASF (vats)/psf with extensive posterior facet ankylosis T1-12 = 21.0 cm T1-S1 = 32.3 cm T4-L1 48 o 3 ¾ yr delay 5 yr surgical rx PFT s : FVC 46% FEV1 50% 6 mo po 4/11 final fusion 5 yr surgical rx

40 Axial plane = PFT?

41 EOS/Congenital - Summary Natural hx and early extensive fusion -> unfavorable long-term prognosis Key numbers : T1-12 > 22 cm, 18 minimum PFT < 45% pred -> mortality Chest circumference doubles > age 10 EOS includes all etiologies presenting < age 10 (definition) < age 4 = potential fatal scoliosis

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