1. 4th International Congress on Early Onset Scoliosis & Growing Spine November 19-20, 2010
Treatment of Kyphoscoliosis and TIS Associated with Myelodysplasia Using the VEPTR in an Eiffel Tower Construct
Ajeya Joshi, MD
Robert Campbell, MD
Davin Cordell, MD
Vishwas Patil, MD
James W. Simmons, DO, PhD
William Koeck, MD
Kent Reinker, MD
University of Texas Health Science Center at San Antonio (UTHSCSA) San Antonio, Texas
Children’s Hospital of Philadelphia Philadelphia, PA

2. Disclosures
A. Joshi, MD Celling Technologies R. Campbell, MD Synthes D. Cordell, MD none V. Patil, MD none J. Simmons, DO, PhD none W. Koeck, MD none K. Reinker, MD none

3. Introduction
Thoracic Insufficiency Syndrome (TIS) is a source of morbidity and mortality in children with spinal and thoracic deformities
VEPTR has been a successful treatment for TIS associated with spinal and thoracic deformities
We analyzed the effect of VEPTR treatment on spinal, thoracic, and pelvic deformities and TIS in children with myelomeningocele

4. Background Orthopedic management of myelomeningocele
Correct spinal, thoracic, and pelvic deformities
Improve respiratory function
Maintain spinal growth
Surgical management of spinal deformities in myelomeningocele is challenging
High infection rates
Poor soft tissue coverage
High hardware failure rates
Myelomeningocele can result in both primary and secondary TIS
With improved neurosurgical and urological management of myelomeningocele, orthopedics has become more involved in management of children with myelomeningocele.
Number of different strategies have been developed: kyphectomy (Sherrard), fusion (posterior and anterior/posterior) with multiple techniques, growing rods.
Kyphosis/scoliosis: relentlessly progressive; to halt progression and correct coronal and sagittal balance
Pelvic obliquity: to improve sitting balance, decrease pressure, improve function
Complications:
Most common: hardware failure, infection, pseudarthroses, shunt insufficiency
Niall et al (2004) [aka Goldberg study]; 20 of 24 experienced post-op complications, complicated primary wound healing most frequent; 18 required further surgeries for protruding hardware.

5. Secondary TIS Thoracic Insufficiency Syndrome Secondary TIS
The inability of the thorax to support normal respiration or lung growth
Secondary TIS
upward pressure of the abdominal contents against the diaphragm, interfering with normal respiration and lung growth
It occurs in myelomeningocele due to:
Lack of active lumbar extension
Severe pelvic obliquity
The marionette sign is a clinical manifestation of this phenomenon
Primary TIS in myelomeningocele: volume depleting deformities due to fused/absent ribs, thoracic kyhposcoliosis, windswept deformities, and thoracic hypoplasia/shortening
Lack of lumbar extension: diminished or absent posterior spinal elements with anterior migration of paravertebral muscles; thoracic or upper lumbar neurological level.
Severe pelvic obliquity causes approximation of pelvis to thorax, causing upward pressure against diaphragm.

6. Objectives
To describe an operative technique using VEPTR for children with myelomeningocele
To assess the effects of VEPTR on the spinal, thoracic, and pelvic deformities
To measure changes in respiratory status
To measure response of TIS to VEPTR treatment
To identify complications associated with the use of VEPTR in children with myelomeningocele

7. Materials & Methods Retrospective study Study Criteria
Inclusion:
Diagnosis of myelomeningocele with kyphoscoliosis
Selection for treatment using VEPTR instrumentation
Minimum 2 years follow-up
Exclusion: prior spinal or thoracic surgeries
Patient selection
10 patients met the inclusion criteria; none were excluded
6 males; 4 females
Age at first surgery: 7.7 years (range, )
Length of follow-up: 6.2 years (range, )
Average age at final f/u =
18 patients with myelomeningocele and kyphoscoliosis
8 had less than 2 years follow-up
3 deceased; 3 received follow-up elsewhere; 2 <?>

8. Materials & Methods Chart review Radiographic Assessment
Demographic data
Operative technique and frequency
Respiratory status: Assisted Ventilatory Rating (AVR), respiratory rate, capillary blood gases, marionette sign
Complications
Radiographic Assessment
Cobb angle
Lumbar kyphosis
Pelvic obliquity
Space Available for Lungs (SAL)
Data analyzed using paired student t-test and Mann-Whitney U test

9. Operative Technique: Eiffel Tower Construct

10. Operative Technique: Eiffel Tower Construct

11. Results Operative Technique Implantation
8 unilateral rib-to-pelvis device
2 staged bilateral rib-to-pelvis devices
All received lateral rib-to-rib devices (6 bilateral; 4 unilateral)
Replacements
Mean of 2.5 surgeries (25 total)
2 for migration; 3 for DM hook fracture; 2 for infection
Expansions
Mean of 7.8 surgeries (range, 3-15)
Unilateral devices: 4 right, 3 left (4)
Time between staged implants: 16mo, 5.5mo (24mo, 8yr7mo)
Migrations: 2 superior hooks, 2 D-M hooks, 2 inferior hooks
Mean number of expansions: 11.9 (range, 6-15)
Mean interval betw expansions: 10 months

12. Results Spinal, Thoracic, & Pelvic Alignment Pre-implant Final F/u
Cobb angle: (p = 0.004)
Lumbar kyphosis: (p = 0.07)
Pelvic obliquity: (p = 0.009)
SAL: (p = 0.002) n
Pre-Implant
Final F/u
p-value*
Cobb Angle 10
70.8° ±18.4°
46.9° ±17.2°
0.004
Lumbar Kyphosis 6
42.5° ±13.7°
22.3° ±18.9°
0.07
Pelvic Obliquity
24.7° ±11.6°
8.6° ±6.6°
0.009
SAL
0.659 ±0.100
0.880 ±0.057
0.002
* paired student t-test
Cobb angle correction (%) = 62.6%
Pelvic obliquity correction (%) = 65.2%
Criteria for normal lumbar curvature came from Propst et al, Radiographic determination of lordosis and kyphosis in normal and scoliotic children, J Pediatr Orthop, (1983) 3: Normal children had mean 40° (range, 31°-49.5°) lordosis.
All children with a normal lumbar lordosis (n=4) maintained curves. Pre = 37.8°, post = 36.0° (p=0.54).
If definition of lumbar kyphosis expanded to less than 30deg lordosis: 19.8 (pre), 3 (post), p=0.05

13. Results Respiratory Status
AVR: (4=full-time mechanical ventilation; 0=no assistance)
8 unchanged (AVR 0)
1 improved (AVR 1 to AVR 0); 1 declined (AVR 0 to AVR 1)
p > 0.05 using Mann-Whitney U test
Respiratory rate: 29 (pre) 24 (post)
CBG:
pO (pre) 68.4 (post) (p = 0.15)
pCO (pre) 38.8 (post) (p = 0.014)
Marionette sign: 6 positive (pre); 0 positive (post) n
Pre-Implant
Final F/u
p-value
AVR 10
8 unchanged (AVR 0)
1 improved (AVR 1 to AVR 0)
1 declined (AVR 0 to AVR 1)
>0.05*
Respiratory Rate
29.3 ±5.4
24.0 ±1.7
n/a
CBG: pCO2
34.5 ±2.9
38.8 ±2.1
0.01**
Marionette Sign
6 positive
0 positive
AVR: (0=no assistance; 4=full-time ventilator dependence)
* Mann-Whitney U test
** paired student t-test

14. Results Complications Device migrations: 6 (in 4 patients)
2 superior cradles
2 D-M hooks
2 inferior cradles
Hardware fatigue fractures: 3 (in 3 patients)
all D-M hook fractures
Wound Infections: 4 (in 4 patients)
3 superficial
1 deep
all successfully treated
Device migrations: all from hybrid device except inferior cradles
Device fractures: 1 wheelchair track racing and weight-lifting with severe flexion, the other 2 occurred with histories of falls from wheelchair
Wound infections: 3 device replacements occurred; otherwise treated with I&D, abx,

15. Conclusions
VEPTR treatment is a useful technique for addressing primary and secondary TIS in myelomeningocele
Spinal, thoracic and pelvic deformity correction was observed
The ‘Eiffel Tower’ construct using bilateral or unilateral rib-to-pelvis hybrid devices with wide base and narrow apex might be most effective for correcting lumbar kyphosis and pelvic obliquity
Infections were surgically treatable with implant salvage and acceptable morbidity

16. Thank you