SPORC 2017 EOS/Congenital Talk

SPORC 2017 EOS/Congenital Talk

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

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

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

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

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 1941-2008

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

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

Well established that thoracic fusion < age 5-8 is associated with TIS Goldberg et al Spine 2003 11 patients < 8 yr (1.4-7.8) PFT s @ 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%)

% 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) 100 90 80 70 60 50 40 30 20 10 0 20 40 60 80 100 % thoracic fusion

Goal of RX: T1-12 length > 18 cm

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

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

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

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

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

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

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

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

Congenital classify Benign BAD WORST McMaster

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

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

Expansion thoracoplasty + growing devices 1 yr po 5 yr po

Cast Treatment IIS +? Growth modulation Delay vs Cure Tactic

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

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

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

Cast x 1 yr, then brace

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

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

7/02 can t cast 100 o

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

Windswept Age: 15 mos Age: 4+4 yrs penetration Cobb: 100 0 T1-T12: 10.3 cm Cobb: 100 0 T1-T12: 12.1 cm

Non-op Operative 2+9 4+9 T1-12= 11.7 7+6 T1-12 = 11.3 cm R 379 L 415 = 794 T1-12= 15.4 R 679 L 801 = 1480

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

Rod fx 12/07 - revised

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

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

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

Axial plane = PFT?

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