Paper Congenital Malformation by mikeholy

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									Abstracts from:
The 1st International Congress on Early Onset Scoliosis and Growing Spine
November 2-3, 2007
Madrid, Spain
Chairman: Behrooz A. Akbarnia, M.D.

Free Papers

Paper #1

The Effect of Early Anterior Fusion on Spinal Canal Size: An Immature Porcine Model

Muharrem Yazici, M.D., Guney Yilmaz, M.D., Murat Pekmezci, M.D., Kenan Daglioglu,
M.D., FC Oner, M.D.

Introduction
Neurocentral cartilage (NCC) is located at the posterior 2/3 of vertebrae and responsible
for the growth of the pedicles and posterior vertebral body. The aim of this study is to
evaluate the effect of anterior spinal instrumentation and fusion on the development of
vertebral body in porcine model.
Materials & Methods
Twelve 8 week-old domestic pigs had CT scans preoperatively and underwent anterior
circumferential discectomy of the L3-L4 and L4-L5 discs. Anterior spinal
instrumentation was performed between L3 and L5 (L4 skipped). After 6 months their
vertebrae were evaluated with CT scan for the presence of anterior fusion and vertebral
canal size. The area of vertebral segments adjacent to proximal and distal instrumented
segments was used as control level area (AC), average area of L3 and L5 was used as
area change in instrumented levels (AI), and area of L4 was used as area change at
arthrodesis level (AA). The percent increases in the canal area and total canal area were
compared.
Results
All subjects had documented anterior fusion. There was no difference in the canal
diameters of three groups preoperatively(AA: 0.70cm2, AI: 0.70cm2, AC: 0.68cm2;
p≥0.05). At the end of 6 months average canal diameter was significantly lower than
control group in the arthrodesis and instrumentation groups (AA: 1.20cm2, AI:1.24cm2 ,
AC:1.41cm2; p≤0.001). The average canal diameters of the arthrodesis and
instrumentation groups were similar (AA: 1.20 cm2, AI: 1.24 cm2; p≥0.05). The average
percent increase in the canal area was significantly lower than the control levels in the
arthrodesis and the instrumented segments (AA: 72.6%, AI: 77.1%, AC: 110.1%;
p≤0.001). The percent increase in the canal area between the arthodesis and instrumented
segment was similar (AA: 72.6%, AI: 77.1%; p≥0.05).
Conclusion
This study demonstrated that anterior spinal arthrodesis or instrumentation in the
immature spine may result in iatrogenic spinal stenosis.
Paper #2

Growing Rod Instrumentation and Vertebral Body Growth: A Radiological Investigation
in Immature Pigs

Guney Yilmaz, M.D., Muharrem Yazici, M.D., Gokhan Demirkiran, M.D., Kenan
Daglioglu, M.D., Cenk Ozkan, M.D.

Introduction
Distraction forces applied on growth plate of appendicular skeleton stimulate longitudinal
growth. However the effect of distraction forces on axial skeletal growth has not been
fully investigated yet. The aim of this study is to evaluate the vertebral body growth
under distraction forces in immature pigs treated with growing rod technique.
Materials & Methods
Eight 8-week-old domestic pigs were used in this experimental model to simulate
growing rod instrumentation technique. Cranially T12-L1 and caudally L4-L5 vertebrae
were instrumented by pedicle screws bilaterally, while L2 and L3 were skipped.
Distraction between pedicle screws was applied at index surgery. The rods were then
lengthened twice in a month interval. All subjects were evaluated with lateral spinal X-
ray preoperatively, postoperatively and at the final follow-up. The vertebral body heights
of distracted segments (HD= L2 and L3) and control segments (HC= T9, T10 and T11)
were measured. Average vertebral body heights and the percent increase in the vertebral
body heights were compared among control segments (n=11) and distracted segments
(n=8).
Results
Four subjects were lost during the immediate postoperative period. The preoperative
vertebral body height was similar in two groups (HC:10.97mm, HD:11.27mm, p≥0.05).
At the final follow-up, the average vertebral body height in distraction group was
significantly higher than the control group (HC:16.92mm HD:18.56mm, p≤0.05). The
percent increase in vertebral body height was higher in distracted segments but there was
no statistically significant difference between the two groups (HC:54.1%, HD:64.4%,
p≥0.05). Postoperative average vertebral body height in distraction group was
significantly longer than preoperative measurements (HDpreop: 10.55mm, HDpostop:
17.49mm, p≤0.05).
Conclusion
The vertebral growth continues during growing rod instrumentation. Distraction forces
might stimulate also apophyseal growth of axial skeleton.
Paper #3

Results of Three Classes of Surgical Treatment for Congenital Scoliosis due to
Hemivertebrae: A Multicenter Retrospective Review

Michael O'Brien, M.D., Peter Newton, M.D., Randy Betz, M.D., Harry Shufflebarger,
M.D., Angel Macagno, M.D., Baron Lonner, M.D., Lynn Letko, M.D., Jurgen Harms,
M.D., Alvin Crawford, M.D., Suken Shah, M.D., Paul Sponseller, M.D., Michelle Marks,
PT MA

Summary
42 patients with hemivertebrae (HV) and congenital scoliosis were compared based on
one of three surgical treatments. HV resections with posterior instrumentation results in
reduced surgical time, shorter fusions, less blood loss, and improved % correction but
slightly higher rates of instrumentation and neurologic complications.
Introduction
We compare the outcomes of 3 surgical treatments for congenital deformities due to a
focal hemivertbra (HV).
Methods
A retrospective multi-center database was compiled from 8 centers to evaluate patients
treated surgically for congenital spinal deformity due to 1 or 2 level HV. The surgical
treatment were: Group 1, fusion without correction (hemi-epiphysiodesis or in-situ
fusion), Group 2, correction without HV resection (with or without anterior or posterior
release) and posterior instrumentation, and Group 3, correction with HV resection
(anterior and/or posterior) and posterior instrumentation.
Results
Forty-two patients, with two-year follow-up, were treated between 1991 and 2004. The
congenital anomalies were: fully segmented, non incarcerated HV (n=32, 76.2%),
incarcerated HV (n=1, 2.4%), and semi-segmented HV (n=9, 21.4%). The distribution of
surgical treatments were: Group 1: n=10(24%), Group 2: n=9 (21%), Group 3: n=23
(55%). Pre-operative curve sizes were statistically different: Group 1, 37° and Group 3,
34° were significantly smaller than Group 2, 55° (p=0.04 and p<0.01, respectively). The
mean age of the patients was 8 years (range 1-18). The complication rate was 25%:
Group 1, 20%, Group 2, 11%, Group 3, 35%. The % coronal correction at 2 years post-
op was better for Group 3 (74%) compared to Group 1 (30%) and Group 2 (45%) (both
p<0.01). A sub-analysis of Group 3 revealed shorter fusions in those treated with
posterior only resection compared to the anterior/posterior techniques (p=0.05).
Conclusion
HV resection gave the best % correction 2 years post-op. It had a slightly higher
complication rate than the other two techniques. HV resection in younger patients results
in better correction with fewer levels fused than either of the other two techniques.
Paper #4

A Retrospective Cohort Study of Pulmonary Function, Radiographic Measures and
Quality of Life in Children with Congenital Scoliosis: An Evaluation of Patient
Outcomes after Early Spinal Fusion.

Michael Vitale, M.D., MPH, Hiroko Matsumoto, M.A., Jaime A. Gomez, M.D., Michael
R. Bye, M.D., Joshua E. Hyman, M.D., Whitney A. Booker, B.S., David P. Roye, Jr.,
M.D.

Introduction
This study evaluates the pulmonary function and quality of life (QOL) of children after a
posterior spinal fusion for progressive congenital scoliosis and compares them to those of
healthy children. The relationships between radiographic measures, pulmonary function,
and QOL will also be examined.
Methods
Twenty-one patients 12.6 +/- 3.4 years old with diagnosis of congenital scoliosis who
were treated with posterior spinal fusion were evaluated using radiographs, pulmonary
function testing (PFT) and QOL surveys using Child Health Questionnaire Parent Form
(CHQ). Some of the patients were treated with growing rods before the fusion and they
were, on average, 6.9 years post definitive spinal fusion. Average age at initial surgery
was 4.9 (1-10) years.
Results
Forced vital capacity (FVC) (p<0.001), forced expiratory volume in one second (FEV1)
(p<0.0001), total lung capacity (p=0.001), and vital capacity (p<0.001) were significantly
lower than those in healthy children. The CHQ scores in our study patients were
significantly lower than healthy children in physical function (p=0.001), general health
(p=0.001), and physical summary (p<0.001) and significantly higher in emotional impact
on parental time (p=0.036). Patients with larger thoracic curves had lower FVC (r = -
.532, p= 0.075) and FEV1 (r = -.590, p=0.04). Patients with larger kyphotic angles had
lower self esteem (r = -.560, p=0.008). Patients with larger thoracic curves have lower
scores on family activities (r = -679, p = .011) and those with larger thoracolumbar
curves have lower scores on physical domains (r = -.701, p = .008). Patients who had
lower FEV1 (r = .526. p=.021) and FVC (r = .545, p=.016) had lower global behavior and
family activities scores.
Conclusion
Compared with healthy children, patients with congenital scoliosis, treated with posterior
spinal fusion at an early age, have significantly worse pulmonary function and quality of
life scores when assessed at average 7 years following initial surgery. These data will add
to growing literature which supports alternatives to early spinal fusion, such as fusion-
less surgery, growing rods and the Vertical Expandable Prosthetic Titanium Rib
(VEPTR) device.
Paper #5

How Does VEPTR Affect Pulmonary Function?

Hemal Mehta, M.S., Brian D. Snyder, M.D., Ph.D, Andrew C. Jackson, Ph.D, Stephen R.
Baldassarri, B.S., Melissa J. Hayward, M.D., Michael J. Giuffrida, M.D., Vahid Entezari,
M.D., Jay M. Wilson, M.D.

Summary
Thoracostomy in conjunction with VEPTR implantation partly reversed the constricted
hemithorax created in young rabbits thereby partially improving lung growth and alveolar
Introduction
Campbell demonstrated that thoracostomy in conjunction with VEPTR implantation to
expand the constricted hemithorax of children with acquired or congenital anomalies of
the thorax and spine improves respiratory function and controls spine deformity. The
mechanisms for the success of this treatment are unknown.
Hypothesis
Thoracostomy in conjunction with VEPTR implantation reverses impaired respiration by
increasing the volume of the constricted hemithorax to allow partial resumption of lung
growth.
Methods
A constricted left hemithorax was induced in seven 5-week old New Zealand rabbits by
asymmetrically tethering ribs 4-8. Thoracic and spinal deformities developed in the
Disease group (n=3). Thoracostomy and VEPTR implantation were performed at 10-
weeks in the VEPTR group (n=4). Two rabbits served as Normal Control. At serial time
points during growth, FRC and respiratory compliance were measured using whole body
plethysmography. Lung volume, spine and chest wall deformity were measured using
CT. Right, left and total lung volumes were calculated from sequential transaxial CT
images of the thorax. Histological analysis was performed on the dissected lungs.
Measures of respiratory function, spine and thoracic deformity were compared using
ANOVA and Fisher’s least significant difference.
Results
The constricted hemithorax created in the Disease group was partly reversed in the
VEPTR group. This allowed slight improvement in left lung volumes. However, due to
the small number of rabbits, this result was not significant. A compensatory increase in
right lung volumes resulted in normalization of total lung volume among the Disease and
VEPTR groups. Alveolar morphology was significantly improved in the VEPTR group.
Conclusions
Preliminary results indicate that VEPTR implantation partly reversed the constricted
hemithorax created by tethering ribs in a growing rabbit model. There was slight
improvement in the growth of the affected lung and partial normalization of alveolar
morphology histologically.
Paper #6

The Treatment of Secondary Thoracic Insufficiency Syndrome of Myelominingocele by a
Hybrid VEPTR "Eiffel Tower" Construct with S-Hook Iliac Crest Pedestal Fixation

Robert M. Campbell, M.D., M. Smith, M.D., W. Allen, M.D., JW Simmons, M.D., S.
Inscore, M.D., B. Cofer, M.D., J. Doski, M.D., C. Grohman, M.D.

Secondary thoracic insufficiency syndrome in myelomeningocele is associated with
lumbar kyphosis, lordosis, or pelvic obliquity. Fusion or kyphectomy may correct
angular deformity, but at the cost of growth of the spine with a high rate of
complications. A modification of the VEPTR construct for congenital scoliosis has
promise for treatment of this disorder.
Methods
0 pts. Avg f/u 5.75 yrs ( 2-11.5 yrs ) Unilat hybrids 8 pts., bilat 2 pts.
Results
Six had a marionette sign preop, none at follow-up. Ratio of diaphragm depth/abdominal
height (nl <1) improved from 2.17 preop to 1.56 at f/u. Lumbar kyphosis improved from
43˚ to 26˚. T/L spine ht increased 5.8mm/yr. Pelvic obliquity improved from 34˚ to 11˚.
Coronal moment arm of iliac crest s-hook fixation was 2.4 times longer than a theoretical
pedicle screw at the same level. Asymptomatic hook migration through avg 39 mm “safe
zone” of iliac crest was 24 mm (6.3 mm/yr, or 16% “safe zone”/yr ) Unilateral hooks
migrated at 8.4mm/yr , bilateral at 7.4 mm/yr . No slow migrations required reseating.
One s-hook incorrectly placed in the SI joint did require acute reseating.. Scoliosis
improved from 73˚ to 46˚ . Average f/u VC (n = 7) was 39% normal. Average implant
EBL 84 cc. Complications: 3 s-hook fractures, 2 rib cradle migrations, 1 skin slough, 4
wound infections. There were no spinal infections. One patient died of respiratory failure
unrelated to surgery.
Conclusions
VEPTR expansion thoracoplasty with a hybrid VEPTR “Eiffel Tower” construct with s-
hook iliac crest pedestal fixation can span the lumbar spine laterally with effective lateral
torso/pelvis distraction elevating the diaphragm away from the pelvis with correct of
spine deformity and pelvic obliquity without affecting growth potential of the spine.
Significance
S-Hook iliac crest pedestal fixation VEPTR modification is a powerful means to correct
pelvic obliquity because of inherent biomechanical advantage of an extended lateral
moment arm compared to central spinal fixation, and spine infection appears be avoided
because the central skin scarring in myelomeningocele is not violated.
Paper #7

VEPTR in Non - Ambulatory Myelodysplasia Patients

John M. Flynn, M.D., Norman Ramirez, M.D., Randal Betz, M.D., John Smith, M.D.,
Robert Campbell, M.D.

Non-ambulatory children with spina bifida are most likely to develop scoliosis. The
dysplastic anatomy of the chest wall in a paralytic child secondarily affects other organ
systems. Thoracic insufficiency may go unnoticed due to the child's limited physical
activities is and is due to increased sagittal plane deformity as the diaphragm invades the
pulmonary cavity.
The purpose of this report is to evaluate the patients with spina bifida and spinal
deformity treated with the Vertical Expandable Prosthetic Titanium Rib (VEPTR) and
included in the FDA Request for Approval of Humanitarian Device Exemption for the
Vertical Expandable Prosthetic Titanium Rib.
The FDA report includes 247 patients with surgeries performed by members of the Chest
Wall Deformity Study Group. Twenty patients were myelodysplastic none ambulatory.
Data from remaining 16 patients with adequate follow up was extracted from the FDA
report and analyzed.
The average age at the first surgery was 5 years; average follow up was 47.3 months. In
nine patients the Cobb angle was decreased an average of 14.4°; in five patients increased
12.6°. Thoracic spinal height was increased in twelve patients an average of 3.2 cm. and
lost in two patients 0.8 cm. Twelve patients improved and two patients deteriorated
ventilatory function. Implant lengthening was done on the average of every sixth months
and device exchange every 30 months. Complications in five patients were due to the
severity of the decreased pulmonary capacity. Implant failure and skin breakdown
occurred in six patients.
The rate of complications reported in this group of patients lies within the range reported
for spinal fusion using standard approaches. The number of surgical procedures increases
due to the need for expanding the implants as the child grows. The advantages of the
VEPTR are: surgical incisions are away from the midline avoiding the midline scarred
tissue and allowing growth of the spinal column with improved space available for the
lung.
Paper #8

Measurement of Forces Generated During Distraction of Growing Rods

Marco Teli, M.D., Giuseppe Grava, M.D., Alessio Lovi, M.D., Marco Brayda-Bruno,
M.D.

Study design
Consecutive series of measurements.
Objective
To measure the forces applied during distraction of growing-rods.
Summary of Background Data
Growing-rods have been used for decades to treat deformities of the immature spine, with
variable success. These rods are periodically lengthened by repeated surgeries, and
failure of growing instrumentation is often reported.
Methods
Distraction forces were measured in 10 pre-puberty patients (mean age, 8 years; range, 6
to 11) undergoing the first distraction of growing-rods, 6 months after implantation for
idiopathic scoliosis treatment. For each measurement, output from the transducer of a
dedicated pair of distraction calipers was recorded at zero load status and at every 1 mm
distraction, up to a maximum of 12 mm.
Results
10 measurements were obtained showing a mean peak force of 485N at 12 mm
distraction. In one case a single peak force reached 552N at 6 mm distraction.
Conclusions
At 500N no failure of instrumentation was recorded. This led to satisfactory elongation of
the rods. The above limit is therefore to be regarded as the uppermost level of force to be
applied during surgical distraction of growing rods.
Paper #9

Single Growing Rods (Review of 21 cases). Changing the Foundations: Does it Affect
the Results?

Hazem Elsebaie, FRCS, M.D.

Study Design
A retrospective case review of children treated with submuscular single growing rod
technique with proximal double claw and distal double level pedicle screws construct
done by the author.
Objectives
Evaluation of a newer version of single growing rod technique inserted sub muscularly.
Changing the foundations in the single rod may change the results.
Background Data
Single growing rod techniques used to include proximally a single claw and many other
constructs that have evolved over years, these reports were used commonly to be
compared with the double rods. To the authors’ knowledge there were no reports on sub
muscular single rod using consistently these foundations which are assumed to be and
more powerful in correction.
Methods
From 2002 to 2007, 21 patients average age 6 years ( 4 -9) underwent single growing rod
procedures using pediatric Isola instrumentation and tandem connectors, 8 had
annulotomy. They underwent an average of 4 lengthening ( 1- 9). Diagnoses included
infantile and juvenile idiopathic scoliosis (13), congenital (3), neurofibromatosis (3),
syndromic (1) and post hydrocephalus (1). Analysis included age at initial surgery,
number and frequency of lengthening, changes in scoliosis Cobb angle, length of T1-S1
and complications.
Results
The mean scoliosis improved from 85° (range, 45° -123°) to 31° (range, 11°-61°) after
initial surgery and was 36° (range, 13°-75°) at the last follow-up. T1-S1 length increased
on average of 1.32 cm per year. During the treatment period, complications occurred in 7
of the 21 patients, and they had a total of 11 complications all were implant related
including 4 proximal claw pull out, 5 rod breakage, one loose set screw of the tandem and
one pedicle screw distal migration, there were no infections, skin complications nor
neurological complications.
Conclusion
The sub muscular single growing rod technique with proximal double claw and distal
double pedicle screws seems to offer a valid alternative to the double rod techniques with
comparable degrees of correction and less incidence of skin complications and infections
especially in thin children with minimal subcutaneous fat.
Paper #10

Safety and Efficacy of Growing Rod Technique for Pediatric Congenital Spinal
Deformities

Hazem Elsebaie, FRCS, M.D., Muharrem Yazici, M.D., George H. Thompson, M.D.,
John B. Emans, M.D., David S. Marks, FRCS, David L. Skaggs, M.D., Alvin H.
Crawford, M.D., Lawrence I. Karlin, M.D., Richard E. McCarthy, M.D., Connie Poe-
Kochert, NP, Patricia Kostial, RN, BSN, Tina Chen, BS, Behrooz A. Akbarnia, M.D.

Study Design
A retrospective analysis of patients with congenital spinal deformities treated with single
or dual growing rods that had a minimum of 2 years follow-up.
Objectives
To determine the safety and efficacy of this technique.
Summary of Background Data
Growing rod technique has been used as a modern alternative treatment for young
children with spinal deformities. There is no comprehensive study focused mainly on the
use of growing rod technique in congenital spine deformities.
Methods
A total of 19 patients with the average age of 6 years and 10 months (3 to 10) with
progressive congenital spinal deformities that underwent growing rod procedures with a
minimum of 2 years follow up. The congenital anomalies included failure of
segmentation in 5, failure of formation in 4, mixed 5 and unclassified or not recorded in
5. The average of affected vertebrae per patient was 5.2 (2-9). The average follow up
period was 3 years 9 months (2 to 6).
Results
The mean scoliosis cobb angle improved from 65.3 (40-90) pre-initial to 44.9 (13-79)
post initial (31.2% correction) and 47.2 (18-78) at the last follow-up or post-final fusion.
T1-S1 length increased from 263.8mm (192-322) after initial surgery and to 310.5mm
(261-352) at last follow-up or post-final fusion with an average T1-S1 length increase
12mm per year. The space available for lungs (SAL) ratio increased from 0.81
preoperatively to 0.94 post latest follow up. Five patients reached final fusion. During the
treatment period, complications occurred in 8 of the 19 patients (42%), and there were a
total of 15 complications out of 100 procedures (15%): 2 pulmonary, 2 infections and 11
implant-related. There were no neurological complications in any of the patients during
the treatment period.
Conclusion
The growing rod technique is a safe and effective treatment for congenital spinal
deformities. There is less correction obtained at initial surgery compared with previous
reports for the same technique in other etiologies. However, there was minimal loss of
correction over the treatment period. The spinal growth and the SAL improved. The rate
of complication is acceptable.
Paper #11

Pelvic Fixation of Growing Rods
Paul D. Sponseller, M.D., Behrooz A. Akbarnia, M.D., George H. Thompson, M.D.,
Richard E. McCarthy, M.D., John B. Emans, M.D., Marc A. Asher, M.D., Muharrem
Yazici, M.D., David L. Skaggs, M.D., Connie Poe-Kochert, RN, CNP, Pat Kostial, RN,
BSN, Tina Chen, BS

Previous studies have not evaluated growing systems in which the distal anchor is the
pelvis. This project analyzed the outcomes and complications of this population. 22
patients were studied from 6 centers. Indications included severe pelvic obliquity, distal
deformity, or lack of satisfactory alternative anchor sites. All had a minimum of two
years treatment with growing rods fixed to the pelvis. Diagnoses included
myelomeningocele (4), cerebral palsy (3), congenital (2), arthrogryposis (1) SMA (1),
miscellaneous/syndromic (11). Mean age at surgery was 6.1 ± 3.1 years. Mean
preoperative curve was 86 ± 22°. Mean coronal imbalance was 9.7 ± 8.2 cm. Mean
follow-up was 50 months. Iliac screws or rods were used in 17, sacral hooks used in five.
Proximal fixation was with hooks in 12 patients and screws in 10. Dual rods were used in
18 patients; single rod in 4. Use of a distal crosslink improved construct stability. Patients
underwent a mean of 2.9 ± 1.8 lengthenings. Mean curve improved to 47± 19° at final
follow-up. Coronal imbalance improved to 4.4 cm postoperatively. Mean increase in T1-
S1 length was 7.3 ± 2.9 cm during distraction. Seven patients have undergone final fusion
at a mean of 10.8 ± 1.4 years. Complications: 6 patients developed deep wound
infections; 5 patients had distal fixation complications, but all were salvaged. There were
3 rod breakages; this rate did not differ statistically from the rate for dual growing rods as
a whole. Pelvic fixation may be used successfully as a distal foundation for growing rods.
Both screws and hooks provide satisfactory distal fixation. Pelvic fixation is a useful
adjunct to growing rods for many children who develop severe syndromic or
neuromuscular spinal deformity at an early age.
Paper #12

Iatrogenic Thoracic Outlet Syndrome as a Complication of VEPTR

Steven Mardjetko, M.D., Ahmad Nassr, M.D., Benjamin Crane, M.D., Kim
Hammerberg, M.D.

Introduction
An innovative treatment for Thoracic Insufficiency Syndrome (TIS) involves a vertical
expansion of the chest wall through a horizontal chest wall osteotomy and a distraction
device (VEPTR). However, 10% of these patients demonstrate upper extremity
neurovascualr dysfunstion following expansion. This study identifies potential etiologies
for compression of the brahial plexus following the VEPTR procedure, and suggests
strategies to reduce the incidence of this complication.
Materials and Methods
A cadaveric study with a simulated VEPTR procedure was performed. A thoracotomy
and sequential rib distraction was performed while manometric measurements were taken
in three anatomic regions of the thoracic outlet. Also, a barium-impregnated putty was
placd along the brachial plexus, and evaluated after expansion using fluoroscopy. A
midclavicular osteotomy was then performed and this process was repeated.
Results
Using the manometric technique described, a 20% increase in pressure was seen in the
mid-clavicular region of the thoracic outlet after thoracic expansion. Reapproximation of
the scapula on the chest wasll further increased the pressure within the thoracic outlet.
constriction of the mid-clavicular region of the thoracic outlet between the first rib and
clavicle was appatent using the putty technique. Mid-clavicular osteotomy alleviated this
region of compression. A second region of compression was noted in the infraclavicular
region of the thoracic outlet. This was secondary to a lateral displacement of the second
and third ribs during the expansion thoracoplasty. This constriction was worsened by
anatomic reapproximation of the scapula to the chest wall.
Discussion
Surgeons should avoid any attempt to pull the scapula distal or medial to its anatomic
position. The utilization of mid-clavicular osteotomy to alleviate thoracic outlet
narrowing after VEPTR procedure may be considered if compression is thought to be in
the mid-cavicular zone. For compression in the infra-clavicular region, lateral
second/third rib osteotomy or resection may be another strategy.
Paper #13

The Development of Spinal Deformities Following Open Heart Surgery

Jose Herrera-Soto, M.D., Kelly L. Vander Have, M.D.

Patients with congenital heart disease (CHD) are at an increased risk of developing
scoliosis. The purpose was to determine the prevalence of spinal anomalies in patients
with CHD post open heart surgery.
Methods
221 patients post open heart surgery for CHD without congenital anomalies were
evaluated. There were 132 males and 89 females with a mean follow-up of 13 years.
Results
59 patients presented scoliosis (27%), with similar incidence of scoliosis between groups.
18 presented moderate to severe scoliosis. 39% with moderate to severe scoliosis
presented with hyperkyphosis (>40 deg). All patients with severe scoliosis underwent
spinal fusion, 9 female and 4 male. Forty patients (18%) presented with hyperkyphosis.
Only 1/40 with hyperkyphosis had a thoracotomy (2.6% of thoracotomy patients). The
remaining 39 patients underwent a sternotomy or combined procedure. There was no
difference in those with cyanosis or not and the development of scoliosis or kyphosis. No
difference between those with and without scoliosis and the age at the first procedure was
found. Patients with multiple procedures were not at increased risk of deformity.
There is an increased incidence of scoliosis in CHD patients. Sternotomy may affect
coronal alignment as thoracotomies. But, it was shown to affect sagittal alignment. It is
important for continued monitoring of spinal deformities as 80% of our severe curves
developed before the age of 9.
Paper #14

Health Related Quality of Life in Children with Thoracic Insufficiency Syndrome

Michael Vitale, M.D., David P. Roye, Jr., M.D., Hiroko Matsumoto, MA, Jaime A.
Gomez, M.D., Randal R. Betz, M.D., John B Emans, M.D., David L. Skaggs, M.D.,
John T. Smith, M.D., Kit Song, M.D., Robert M Campbell, Jr., M.D.

Purpose
The purpose of this study was to compare quality of life (QOL) of children with TIS and
impact on their parents prior to and after the implantation of the VEPTR. We also
compared the QOL of children with TIS and the parental impact prior to implantation of
the VEPTR with previously published QOL of healthy children.
Methods
As part of the original multi-center evaluation of the VEPTR, Child Health Questionnaire
CHQ was collected preoperatively on 45 patients who were subsequently treated with
expansion thoracoplasty using the VEPTR. The average age was 8.2 +/-2.6 and parent
form of the CHQ was filled by the primary caretaker. Patients were divided into three
diagnostic categories: Rib Fusion (RF) N=15, Hypoplastic Thorax Syndromes (HT)
N=17 and Progressive Spinal Deformity (PS) N=13.
Results
There were significant differences between the study patients and healthy children in
physical domains. Compared with parents of healthy children, parents of children with
TIS experienced more limitations on their time and emotional lives due to their children's
health problems. In addition they had poor expectations for their children's health. There
were no significant differences in CHQ scores in these children before and after the
surgery except for a significant decrease in Self Esteem score among patients with HT.
None of the domains in the CHQ had moderate or large degree of responsiveness across
all three diagnostic categories.
Discussion
The children with TIS had lower physical domain scores and higher caregiver burden
scores than healthy children. However, the scores in psychosocial domains were similar
to those in healthy children. Since responsiveness of the CHQ was small, it can be
concluded that our study demonstrates the lack of ability in the questionnaire to reflect
clinically important minimal changes in response to the VEPTR instrumentation in this
population. Therefore, a Disease Specific Instrument (DSI) may be needed in order to
detect the minimal changes in this population. Current efforts are on their way to better
understand the clinical features that have the most profound effects on the life of these
children and to develop a DSI for this population.
Paper #15

Surgical Complications in Early Onset Scoliosis

Jonathan H. Phillips, M.D., D. Raymond Knapp, Jr., M.D., Jose Herrera-Soto, M.D.

Between March 2002 and July 2005 a total of 44 surgical procedures were performed in
20 young scoliosis patients involving the use of unfused instrumentation. Average age
was 8 years-1 month, range 2 + 3 to 12 + 7. Total number of procedures was 44, average
2.2. and only one patient has gone on to definitive fusion at this point. This indicates the
early stage of development of this program at our center. All patients received either
ISOLA growing rod instrumentation or VEPTR. There was a 100% complication rate in
the VEPTR patients and 64% in the growing rods. 20% of total complications occurred
in patients who moved to our area from out of state and were established complications,
sometimes after multiple surgeries done elsewhere. Our local complication rate was 45%
which is nearly identical to published literature (Akbarnia, BA, et al 2005). There was an
alarmingly high rate of deep infection which occurred early in the VEPTR group and late
after many lengthenings in the growing rod group. Of 9 infections, implants were
salvaged in only 2 cases. The others all need to be removed and definitive treatment of
the scoliosis is still pending. There was 1 perioperative death in the group. There was
one transient intraoperative neurological deficit with no permanent loss postoperatively.

A large variety of very rare syndromes comprised much of this group. As in other reports,
implant cut out and breakage constituted the majority of non infectious complications.
These patients represent a high risk group but seem to have a complication rate in excess
of other high risk patients. For example, our group reported an 8% infection rate in
definitive fusion for neuromuscular scoliosis recently (Phillips et al 2005). Our infection
rate for this early onset group was five times greater.

It is assumed that the multiple surgeries for rod lengthening lie at the heart of this high
complication rate and strategies to avoid repeated open procedures may reduce the rate of
infection at least. The fact that 20% of complications were inherited from other centers in
the USA underlies the need for a central data bank of this small group of patients to truly
delineate outcomes in Early Onset Scoliosis surgery.
Paper #16

Long-term Results of Congenital Scoliosis Treatment

Martin Repko, Martin Krbec, Jan Burda, Jan Pesek, Richard Chaloupka, Vladimir Tichy

Aim
The aim of our study is to evaluate clinical and X-ray long-term results of congenital
scoliosis treatment.
Material
The total number of 442 patients with congenital scoliosis treated in our department in
the period from 1976 to 2006 was retrospectively evaluated. There were 175 (40%)
patients treated by conservative manner, 64 (14%) pts. were treated by single bone
fusion, 141 (32%) pts. were treated by correction with instrumentation using the posterior
approach and 62 (14%) pts. were treated by combined anterior/posterior surgical
approach. An average follow up was 16 years and 3 month.
Results
1. conservative treatment - the magnitude of the curves was at time of detection on
average 44,1° according to Cobb angle and 39,8 at time of last control.
2. single bone fusion by posterior surgical approach - an average time of surgery was 6,6
years, follow up was 14,2 years. The magnitude of the curves was at time of detection on
average 44,1°, 44,2 preoperatively, 34,4 postoperatively and 38,4 at time of last control.
There were no neurological complication.
3. posterior surgical approach with instrumentation - the time of surgery was 8,6 years,
follow up was 12,6 years. The magnitude of the curves was at time of detection on
average 61,1°, 66,8 preoperatively, 40,9 postoperatively and 46,0 at time of last control.
There were 1,7% of neurological complications.
4. combined anterior/posterior surgical approach with hemivertebrectomy and
instrumentation - the time of surgery was 11,2 years, follow up was 17,9 years. The
magnitude of the curves was at time of detection on average 52,2°, 64,9 preoperatively,
38,3 postoperatively and 39,0 at time of last control. There were 1,2% of neurological
complications.
Discussion and Conclusions
Early detection, good timing and choosing of adequate surgical treatment type are the
main factors of quality treatment results. All methods of surgical treatment led to the
improvement in magnitude of the scoliotic curve. The best method seems combined
anterior/posterior surgical approach with hemivertebrectomy and instrumentation
stabilization.
Paper #17

VEPTR in Patients with a Previous Spinal Fusion

Peter Sturm, M.D., John M. Flynn, M.D., Randal R. Betz, M.D., John T. Smith, M.D.,
John B. Emans, M.D., Sohrab Gollogly, Robert M. Campbell, M.D., Melissa P. Smart,
BS, R.N.

Introduction
Spinal fusion had been the mainstay of treatment for children with complex congenital
scoliosis. This treatment had an unintended deleterious effect on spinal growth and
pulmonary development. The use of the VEPTR in these patients has been shown to
control curve progression while allowing for trunk growth and further lung maturation.
This study looks at the use of a VEPTR in patients who had undergone a spinal fusion.
Methods
Seventeen patients who had previous spinal fusions, and then subsequently underwent the
insertion of a VEPTR between September 1996 and February 2003, were identified
among patients entered prospectively into a database for an FDA IDE study. Pre and
postoperative Cobb angle, thoracic height and complications were recorded. Eight to
twelve month follow up data was available for 12 patients and 36 month follow up in 7.
Results
The indication for VEPTR in these patients was a progressive curve despite previous
fusion, and persistent thoracic insufficiency. Average age at VEPTR insertion was 6
years and 7 months. Average preoperative Cobb angle was 59° (range 10 - 95°).
Average postoperative curve measured 49° (range 5 - 80°). In the subgroup with one year
follow up the average preoperative Cobb angle was 58.3°, postop 41.6°, follow up 48.7°.
The average change in trunk height was 0.74 cm at index surgery. 7 patients had
complications. This included 3 patients with loss of fixation alone, 1 patient with a
postoperative infection, 2 patients with both an infection and loss of fixation, and 1
patient with postoperative Horner’s syndrome.
Conclusion
The use of a VEPTR has been shown to be beneficial in children with thoracic
insufficiency due to various etiologies. In patients who have already had a spinal fusion
the goals of VEPTR implantation are to improve truck deformity, expand the chest, and
modulate the spinal deformity when possible. The amount of correction of both the Cobb
angle and thoracic height is less than in chlidren who have not undergone a prior spinal
procedure. In addition, the complication rate is higher. It should be viewed as a salvage
procedure in this group of patients.
Paper #18

Short Anterior Instrumented Fusion of Lateral Hemivertebra

Enrique Garrido, MRCS, EBOT, F. Tome, SK Tucker, TR Morley, HNM Noordeen

Study Design
Retrospective study with clinical and radiological evaluation of 29 patients with
congenital scoliosis who underwent 31 short segment anterior instrumented fusions of
lateral hemivertebrae.
Objective
To evaluate the safety and efficacy of early surgical anterior instrumented fusion with
partial preservation of the body of the HV in the treatment of progressive congenital
scoliosis in children below the age of 6.
Summary of Background Data
A variety of treatments have been described in the literature for the treatment of HV. We
report the results of a novel technique.
Materials and Methods
Between 1996 and 2005, 29 consecutive patients with 31 lateral HV and progressive
scoliosis underwent short segment anterior instrumented fusion with partial preservation
of the body of HV. Additional posterior Moe fusion was performed on 29 HV. Mean age
at surgery was 2.9 years. Mean follow-up period was 6.2 years.
Results
Preoperative segmental Cobb angle averaging 39, was corrected to 15 after surgery, being
16° at the last follow up (56,4% of improvement). Compensatory cranial and caudal
curves corrected by 50% and did not change significantly on follow up. The angle of
segmental kyphosis averaged 14 before surgery, 13 after surgery, and 14° at follow up.
Coronal and sagital balance improved postoperatively and gradually during follow up to
normal values. 10 patients had a thoracic kyphosis and two patients a lumbar lordosis
below average for their age. There were 2 posterior wound infections requiring surgical
debridment, one intraoperative fracture of the vertebral body during screw insertion and
in one case there was loss of correction due to implant failure. All went on to stable bony
union. There were no neurologic complications and no additional operations.
Conclusions
Early diagnosis and early and aggressive surgical treatment are mandatory for a
successful treatment of congenital scoliosis and to prevent the development of secondary
compensatory deformities. Anterior instrumentation is a safe and effective technique
capable of transmitting a high amount of convex compression allowing short segment
fusion which is of great importance in the growing spine
Paper #19

Prior Instrumentation and Fusion Method at the Anchor Sites

Tomoaki Kitagawa, M.D., Hiroshi Taneichi, Daisaku Takeuchi, Takashi Namikawa,
Satoshi Inami, Tetsuro Kiya, Yutaka Nakamura, Takahiro Iida, Yutaka Nohara

Background
Growing rods system is usually used for early onset scoliosis that have small and fragile
posterior elements due to not only its young age but also congenital or genetic factors,
which make it difficult to apply strong corrective force at the time of initial surgery
and/or necessitate post-operative immobilization using cast or orthosis.
Method
To prevent this problem, we have developed a staged operation to apply growing rods. At
the initial surgery, only the proximal and distal anchor sites are exposed and the patients
undergo one- or two-level instrumentation and fusion at the both sites. Instrumentation
includes either hooks, pedicle screws, or their combination. Fusion procedure includes
decortication of the lamina and facet joint, and local bone grafting. The second surgery
will be planned after the fusion mass becomes mature and solid, usually about three
months after initial surgery. The anchor sites will be exposed again and previous screws
and hooks will be replaced with thicker ones if necessary. Four cases of this method were
investigated retrospectively. The age at the surgery ranged from three to eight years old.
The primary diagnosis includes Turner syndrome, neurofibromatosis, Ehlers-Danlos
syndrome, and scoliosis associated with CHARGE syndrome.
Results
Mean pre-operative Cobb angle of the major curve is 112°, ranging from 48 to 143°.
Mean post-operative Cobb angle after correction surgery using growing rods was 46°,
ranging from 27 to 63°. Mean operation time was 202 minutes at the first surgery and 173
minutes at the second surgery. The laminae were covered with solid fusion mass and
pedicle walls were thickened and expanded at the second surgery, so stronger anchors
could be obtained. Then growing rods were applied and strong corrective force could be
applied to achieve enough initial correction. Patients were allowed to walk within three
days after surgery without use of cast or orthosis. Correction loss did not happened until
next rods lengthening surgery.
Conclusion
Prior instrumentation and fusion method for growing rods is effective for strong initial
correction and can avoid failure of the posterior element of the spine.
Paper #20

Evaluation of Vertebral Anomalies and Vertebral Osteotomy

Noriaki Kawakami, M.D., Taichi Tsuji, Kazuyoshi Miyasaka, Tetsuya Ohara, Yasunori
Tatara, Kei Ando, Ayato Nohara

Posterior hemivertebrectomy has been reported as effective, safe, and less invasive for
the surgical treatment of hemivertebrae; although it is technically demanding. However,
the combined anterior and posterior operation is still useful and may be selected for some
types of congenital vertebral anomaly.
Purpose
The purpose of this study was to assess the type of congenital vertebral anomaly and the
operative strategy using vertebral osteotomy and to determine which types of vertebral
anomaly should be treated through an the combined anterior and posterior approach.
Materials and Method
Sixty patients who underwent vertebral osteotomy for congenital vertebral anomaly were
evaluated retrospectively. They consisted of 31 males and 29 females and the average age
at the time of operation was 13 years. One patient underwent two separate operations for
discrete, distinct anomalies. Of 61 operations in total, 36 used the posterior approach and
25 used the combined anterior and posterior approach. Of 25 combined anterior and
posterior operations, 7 were two-staged. The overall postoperative follow-up time was 55
months (17-156).
Results
The types of congenital vertebral anomaly were evaluated using 3DCT and were
classified into 16 cases of solitary simple, 24 of multiple simple, 16 of multiple complex,
and 4 of segmentation failure. The reasons for selection of the combined anterior and
posterior operation included the release and/or osteotomy of multiple levels in 11 cases,
leaning curve in 7, and additional anterior bone graft for a big gap in the osteotomy site in
6 cases. In 36 patients in whom the posterior approach was utilized, 29 (80%) were
included in solitary simple or multiple simple, which indicated anterior and posterior
unison type anomalies. Scoliosis was corrected from 43.2° to 16.1° using the posterior
approach only, and from 72.6° to 33.1° with the combined anterior and posterior
operation, indicating statistically significant differences for both preoperative and
postoperative curve magnitudes.
Conclusion
More severe curves and multiple congenital vertebral anomaly may be factors that
necessitate the combined anterior and posterior operation.
Paper #21

PRSS in Management of Adolescent and Juvenile Scoliosis

Qibin Ye

Objective
To study on the therapeutic mechanism of an innovated instrumentation-Plate-Rod
System for scoliosis (PRSS) and its effectiveness for the surgical management of
adolescent and juvenile scoliosis
Method
To avoid the necessity of repeated operative instrumentation lengthening, a new device-
PRSS was developed in our department. Since October 1998, a total of 183 scoliotic
children were treated using PRSS. Among them, 66 cases adolescent and 23 cases
juvenile scoliosis were evaluated prospectively. The mean age at the time of surgery was
12.15 years in adolescent group and 7.98 years in juvenile group. The experimental study
on type X collagen which reflect cartilage degeneration was studied in PRSS-
instrumented animal spine to express the therapeutic biomechanism .
Results
The preoperative scoliotic curve was 66.58° and post-operative curve was 22.70°
(68.86% ) in adolescent group and in juvenile group, the scoliotic curve was improved
from 80.7° to 30.5° (62.2%). An average of 11.13mm spinal lengthening of the
instrumented segments was achieved in adolescent group and 13.38mm in juvenile group.
In 10 cases, the implant was removed after bone maturing was reached. The spine keep
good maintained of correction and essential normal flexibility. When PRSS is placed in
place, compressive stress was found to exert on the convex side, while tensile stress on
the concave side of the curvature, and more type X collagen to be expressed on convex
side than concave side, it suggest that compressive stress leads to increase earlier
cartilage degeneration of end plate in convex side, so as to retard the growth of the end
plate of this side, resulting in maximum spinal realignment.
Conclusion
The PRSS, which dispenses with spinal fusion and allows extension along with the
children growth, is able to provide and maintain desirable correction of scoliosis in one
stage operation and it is helpful to prevent Crankshaft phenomenon, essential normal
spinal flexibility can be obtained after treatment. This new device is an effective
instrumentation for correcting scoliosis, especially in growing children.
Paper #22

Spinal Hemiepiphysiodesis Correlates with Structural Changes

Eric Wall, M.D., Donita I. Bylski-Austrow, Ph.D, David L. Glos, BSE, Edgar T. Ballard,
M.D., Andrea Montgomery, BS, Alvin H. Crawford, M.D.

Introduction
Some stapling methods have been shown to alter spine alignment in preclinical models.
In long bones, staples have been shown to alter growth plate structure particularly in the
hypertrophic zone. In the spine, however, stresses transmitted to the physes are likely
affected by the intervertebral disc. The purpose of this study was to determine if
structural changes to the vertebral growth plate accompanied increased spine curvatures
in a porcine model of spinal hemiepiphysiodesis. The hypotheses were that the height of
the hypertrophic zone and size of hypertrophic cells were lower at the stapled levels and
side compared to both an unoperated level and the contralateral side.
Methods
Anatomically-based spine staples were implanted endoscopically into the left side of 6
mid-thoracic vertebrae of five skeletally immature domestic pigs. Each staple was
centered over an intervertebral disc and two growth plates, with placement aided by a
guide-wire. After 8 weeks, spines were harvested. Mid-coronal sections were prepared
for histologic analysis. Hypertrophic zone height, height and width of hypertrophic cells,
and disc height were measured at discrete sampling locations across the plane at stapled
and unstapled levels.
Results
Zone height, cell height, and cell width were lowest on the stapled side of the stapled
level, with significant differences in the overall statistical model (p≤0.02). Disc heights
were reduced at the stapled levels across the coronal plane.
Conclusions
A staple-like implant and particular endoscopic insertion procedures were associated with
gradually decreased growth plate height and cell size, as well as decreased disc height.
Clinical Relevance
This is the first report of spinal hemiepiphysiodesis using clinically relevant procedures
that has been shown to affect the growth plate asymmetrically. The results indicate that
some devices may be capable of slowing progression of a developing scoliotic curve by
differential growth inhibition.
E-Posters

E-Poster #1

Efficacy of Growing Rods in Infantile Marfans Scoliosis

Paul D. Sponseller, M.D., George H. Thompson, M.D., Behrooz A. Akbarnia, M.D.,
Marc A. Asher, M.D., John B. Emans, M.D., Tina Chen, BS, Connie Poe-Kochert, RN,
CNP

This is a retrospective analysis of nine patients with Marfan Syndrome and scoliosis
(developing before age 3) treated with growing rod techniques with a minimum of 2
years follow-up. Radiographic and chart reviews included the following parameters: age,
lordosis, kyphosis, complications, coronal balance, T1-S1 length, and initial curve angle.
Three patients had single and 6 had dual growing rods. Age at initial surgery was 4.5
years (2-9). Mean scoliosis curve was 80° (54-105) and thoracolumbar kyphosis was 56°
(27-85) preoperatively. Patients on Coumadin were lengthened yearly or throughout
longer intervals of time. The mean follow up was 65 months. Four of 9 patients have
undergone final fusion. The common construct was T2/4 to L4 in 4 patients and or to the
pelvis in 4. Curve correction was 54% (41% with single rods, 61% with dual rods). Mean
coronal imbalance improved from 56 to 18mm and sagittal imbalance from 31 to 21mm.
Increase in T1-S1 length over the treatment period was 10.2cm for the whole group and
13.3cm for those who underwent final fusion. Complications included two rod breakages
and two intra-operative dural leaks. There was one anchor dislodgement, however no
postoperative dural leaks. None of the patients developed clinically significant junctional
kyphosis. One patient died of unrelated causes three months postoperatively. Growing
rod techniques can prevent large infantile curves from becoming severe in patients with
Marfan Syndrome. Dual rods were more corrective compared to single rods. Significant
spinal length can be obtained to minimize trunk disproportion.
E-Poster # 2

The VEPTR "Parasol" Expansion Thoracoplasty for Treatment of Transverse Volume
Depletion Deformity of the Convex Hemithorax Rib Hump in Early Onset Scoliosis

Robert M. Campbell, M.D., JT Woody Smith, JW Simmons, S. Inscore, BR Cofer, JJ
Doski, C. Grohman

Cobb angle correction and improved SAL is common with VEPTR treatment, but
windswept deformity narrows the room for the lung contained within the rib hump. The
structural basis of rib hump is usually not acute angulation of the rib, but a spine rotation-
driven deviation of the ribs. Each rib “bucket handle” folds downward, closing shut the
convex hemi-thorax, resembling the closure of a parasol. To reverse the mechanism of
this deformity, a VEPTR expansion thoracoplasty was developed, termed the “parasol”
procedure. Multiple levels of intercostal muscle are lysed to mobilize the depressed chest
wall. Rib segments are elevated upward and outward, then stabilized in the “open
parasol” position.
Methods
10 pts. Avg f/u of 6 yrs from initial concave surgery and 4 yrs from convex parasol
procedure. 7 pts 70 mm radius VEPTR, 4 pts hybrid/rib-rib VEPTR
Results
Cobb angle pre-op 79°, pre-convex proc 51°, 49° at f/u. SAL 1.5 pre-op, 0.92 at f/u.
Concave/convex hemithorax width ratio was 2.79 pre-concave implant, 3.07 pre-convex
implant, and 2.17 f/u. CT lung volumes (n = 6): pre-implant convex/concave l.v. ratio
was 0.87, 0.91 at f/u. Avg. vital capacity at f/u was 37.8% predicted (n=9). Pt
complications: (4) spinal hook migration, (3) superior migration rib cradles, (1) fractured
hybrid, (1) titanium sling migration, (3) infection, (3) skin slough, (2) pneumonia.
Conclusion
Expansion of the rib hump in early onset scoliosis by the VEPTR “parasol” procedure
increases the lateral width of the hemithorax on radiograph. Some improvement in the
convex/concave lung volume ratio is seen. Scoliosis is not increased by the convex
distraction. The complication rate is frequent, but treatable.
Significance
The loss of transverse volume in the convex hemithorax due to rib hump of the pt with
EOS contributes to TIS. The VEPTR “parasol” expansion thoracoplasty directly
addresses the anatomic deformity of rib hump, probably partially reversing the convex
volume depletion problem. If done early in life, this may help encourage convex lung
growth. For any growing pt with rib hump, especially if vital capacity is low, the
“parasol” procedure should be considered instead of a traditional orthopaedic
thoracoplasty.
E-Poster #3

Anterior Instrumented Correction of Congenital Scoliosis

Hazem Elsebaie, M.D., Yasser Elmaelligy, FRCS, M.D., Wael Koptan, M.D., Mootaz
Salaheldine, MSc, Hilali Noordeen, FRCS Orth

Study Design
Retrospective analysis.
Objectives
To determine the safety and efficacy of this technique in the management of progressive
congenital spinal deformities due to failure of formation analyzing: correction of spinal
deformity, fusion rate and incidence of complications.
Summary of Background Data
Several modalities have been reported for the treatment of young children with congenital
spinal deformities including the recent reports on hemivertebrectomy with
instrumentation. There has always been concerns regarding epidural bleeding,
neurological complications, pedicle screws placement, implant faillure and prominence
of posterior constructs in this very young age group. Anterior release, decancellation of
the hemivertebra without opening the spinal canal and anterior instrumentation offers a
new alternative which can avoid these concerns.
Methods
A total of 11 patients with progressive congenital spinal deformities due to failure of
formation who had single stage anterior instrumented fusion, decancellation of the
hemivertebra and posterior non instrumented fusion followed for a minimum of 2 years
were analyzed. All patients had single hemivertebra. The average age of the patients was
2 years 7 months (1y 9m to 3y 10 m). And the average follow up period was 3 years 1
months (2ys to 4ys 5m).
Results
The mean scoliosis angle improved from 48° (34-58) preoperative to 17 (11-25) at the
final follow up, a mean correction rate of 64.5% , the angle of kyphosis was 20° before
surgery and 11° at the final follow up a mean correction of 45%, all the patients having
radiological fusion. The average operative time was 120 mins (98-180 min). There was
one superficial infection.
Conclusion
Anterior instrumentation with decancellation of the hemivertebra a offers a safe and
effective alternative to hemivertebrectomy with less risks and less operative time and
blood loss with an equal amount of correction, making the need of the more aggressive
hemivertebrectomy questionable in this very young age group.
Significance
With anterior instrumentation decancellation of the hemivertebra is safer and simpler
than hemivertebrectomy with same effectiveness in the very young children.
E-Poster #4

Spinal Open-Wedge Osteotomy

Dezso J. Jeszenszky, M.D., Tamas Fulop Fekete, Martin Sutter, Friederike Lattig,
Andreas Eggspuhler, Frank S. Kleinstuck

Introduction
Correction of congenital scoliosis is usually achieved by in situ fusion or by shortening of
the spine. Intraoperative distraction may cause neurological damage and is therefore
usually avoided or is performed indirectly by slowly distracting the ribcage. A surgical
method to correct spinal deformity by osteotomy and distraction in congenital scoliosis
has not been reported before.
Methods
3 patients treated for mixed congenital scoliosis were studied. The surgical procedure
involved concave side exposure of the bar to the anterior aspect using a posterior
approach. The surgeon performed a near circumferential osteotomy around the dural sack
and then opened up the osteotomized segment to correct the curve. The correction was
augmented through distraction under continuous intraoperative spinal cord monitoring.
Stabilization was performed without fusion and on one side only, with pedicle screws,
rods and rib hooks. The Cobb angles of scoliosis were measured pre- and postoperatively.
Results
Age at surgery was 4.0 (2.4 – 5.25) years. Follow-up time was 1.52 (0.5 – 2.92) years.
The avg scoliotic curve was 60.7° (50 – 69) pre-implant, 29.3° (18 – 40) post-implant,
yielding a correction of 31.4° (51.8%). The pre- and postoperative compensatory curves
were 34° (31-56) and 17° (12-23), respectively. Multimodal monitoring of the spinal cord
during surgery revealed potential damage of the spinal cord in two cases immediately
following the correction. Therefore the final correction was delayed for one week in both
cases. No postop neurological complication was detected.
Conclusion
Spinal open-wedge osteotomy is an effective surgical technique for correction of
congenital scoliosis. Surgery is performed only at the congenitally affected region of the
spine. Goal of surgery is to achieve the greatest correction possible, at this site. All other
healthy regions (secondary curves, convex side) of the spine are left intact. The surgery
should be performed as early as possible, so all the intact spinal regions can grow
normally. The use of intraoperative spinal cord monitoring is essential.
E-Poster #5

The Influence of Fixation Rigidity on Intervertebral Joints

Charles-H Rivard, M.D., Christine Coillard, M.D., Gary L. Lowery, M.D., Souad
Rhalmi, M.Sc., Marco Berard, M.D.

Objectives
The study was to determine whether allowing intervertebral micromotion compared to
the effect of the rigidity of immobilization on the biological changes in the intervertebral
joints.. The OrthobiomTM was developed and used as instrumentation in comparison
with a rigid system and a control group.
Methods
Twenty growing Yucatan minipigs were divided into 4 groups. Six were fixed with a
rigid system. Eleven were fixed with a mobile unconstrained implant (OrthobiomTM)
that allowed intervertebral micromotion. Three minipigs were used as control. They were
euthanized and underwent the same necropsy. X-ray follow-ups were taken to provide
information about the fixation of the mobile and rigid system. In addition, a CT scan was
performed on a control, rigid and mobile minipigs.
Results
In the rigid group, despite the growth, the length of the instrumented segment remained
unchanged. Initial scoliosis of 31° was maintained at 27° (p= 0.37, paired t test). In the
mobile fixation group, the length of the instrumented segment grew from 25.3 cm to 30
cm (p=0.0004, paired t-test). The scoliotic curve of 19° was maintained at 17° (p=0.21,
paired t-test). CT scans of a minipig instrumented with the rigid system showed
spontaneous fusion within the instrumented section. The CT scans of the minipig with the
mobile fixation performed after the removal of the implant showed the posterior joints to
be well maintained with the preservation of the joints space, and a clearly visible
radiolucent line. The discs viability was maintained with the mobile fixation and
degenerated with the rigid one.
Conclusions
Intersegmental micromotion using flexible unconstrained internal system could preserve
the viability of the intervertebral joints incorporated within the instrumented section
while maintaining reasonably stable fixation in young pigs during growth. This new
approach may be a valuable option to fusions, though it needs to be proven effective in
humans.

E-Poster #6

Nutritional Improvement Following VEPTR Surgery in Children with Thoracic
Insufficiency Syndrome

David L. Skaggs, M.D., Josh Albrektson, Tishya Wren, Robert M. Campbell, M.D.

Introduction
Young children with thoracic insufficiency syndrome (TIS) secondary to spinal and/or
thoracic deformity are often characterized by a failure to thrive. Nutritional depletion
occurs in part as the work of breathing approaches the nutritional gain of eating.
Methods
A total of 79 patients at 7 different institutions underwent placement of VEPTR devices
for treatment or prevention of TIS. All patients underwent weight measurements pre-op
and post-op. Patients had a mean age at surgery of 44 months (+/- 33) with an average
follow up of 45 months (+/-16, minimum f/u 24 months). All weights were converted to
normative percentiles based on the patient’s age.
Results
There was a significant increase in the mean percentile of patients’ weights relative to
normative values (p=0.002). Of the 79 patients, 62 (78%) were <5 percentile in weight
pre-operatively. Of these most nutrionally depleted patients 35% (22/62) showed a
measureable increase in percentile weight following VEPTR surgery. Of patients not
showing an improvement, a change in percentile weight may have occurred, but would
not be recognized due to the basement effect of normative percentiles. Of the 17 patients
who were >5 percentile weight pre-operatively, 76% (13/17) showed measurable
improvement in weight percentiles.
Conclusion
Children with TIS undergoing VEPTR surgery have very poor nutritional status, with
78% of patients being <5 percentile in weight. This study documents an improvement in
the nutritional status of children with TIS following VEPTR surgery, which is a critically
important outcome measure in this population. Children who have better nutrition pre-
operatively (>5 percentile weight) demonstrate larger gains following VEPTR surgery
than those < 5 percentile.
E-Poster #7

Outcomes in the Early Treatment of Progressive Infantile Scoliosis

Ascani Elio, Ramieri Alessandro, M.D.

Infantile scoliosis, idiopathic, congenital or neurophatic, show common characteristics
for gravity and rapid worsening. Their treatment is still controversial, with different
choices among the spinal surgeons. We present the long-term results (mean F.U. 13 yrs;
range 16-30) of a retrospective series of 43 progressive infantile scoliosis with different
etiology, treated from 1975 to1990. Conservative treatment consisted in brace or cast
immobilization. Halo-traction was also applied. Initial surgical approach was achieved by
anterior hemiepiphysiodes and posterior hemiarthrodesis (n=4), posterior epiphysiodesis
(n=7), hemivertebra excision with or w/o instrumentation (n=12), Ascani-Zielke
subcutaneous rod (n=12), early posterior fusion with pediatric instrumentation (n=8).
Trying to define the best procedure in the management of severe spinal deformity in
young children, our results with conservative treatment were unsatisfactory due to curve
progression. Posterior-anterior hemiepiphysiodes achieved an acceptable stabilization of
the congenital curves before the final fusion (n=4), while no significant difference
between hemivertebra resection techniques was detected. Posterior epiphysiodes was
more effective for kyphoscoliosis. Ascani-Zielke rods generally obtained correction,
showing nevertheless mechanical (n=4) or general complications (n=3). Early
instrumented fusion obtained the improvement and stabilization of the deformity, except
for a case of initial crankshaft, re-operated by an anterior arthrodesis.
Our experience in the early treatment of infantile scoliosis recommends a surgical
approach. Hemiepiphysiodes, circumferential procedures or growing rod techniques may
be safe and effective if correctly applied. Nevertheless, also definitive instrumented
spinal fusion at a young age can be judged as valid unique solution for these complex
deformities.
E-Poster #8

Surgery Before Age 3 in Patients with Congenital and Syndromic Scoliosis Followed
Until the End of Growth

Charles D'Amato, M.D.

Purpose
Patients with progressive congenital and syndromic scoliosis have in the past been treated
with spinal fusion at an early age to prevent progression. The effects of spinal surgery on
pulmonary development and function are still under investigation. Recent reports have
shown diminished pulmonary function in patients who have undergone fusion of the
thoracic spine at an early age occasionally leading to pulmonary hypertension, cor
pulmonale and, eventually, death. The aim of this retrospective study is to investigate
how early spinal arthrodesis (before age 3 years) affects the pulmonary function and the
growth of the thorax in patients followed to skeletal maturity.
Material and Methods
Six patients with congenital scoliosis and three with early onset scoliosis were surgically
treated before age 3. The average age at initial surgery was 20 months (range: 12 to 39
months). Six patients were treated with a posterior spinal fusion and three had anterior
and posterior surgery. Instrumentation was used in only one patient. Six was the average
number of thoracic segments fused (range: 2 to 12). Final standing height (STH) and
trunk height (TRh) were measured in all patients. Using standard radiographs thoracic
spine height (Th), lumbar spine height (Lh), pelvic width (PW) and chest width (CW)
were measured in all patientsª. Vital Capacity (VC), Forced Expiratory Volume in 1 sec.
(FEV1), Max. Mid Exp. Flow rate (FEV25-75), Residual Volume (RV) and Total Lung
Capacity (TL) were available for four patients. The average follow up was over 14 years.
Results
STH, TRh, Th were significantly reduced in all patients but one who had fusion of only
two thoracic vertebrae. Lh was normal. The observed thoracic dimensionsª were
significantly reduced compared to the expected ones in all patients but one who had only
two thoracic spine levels fused. Average VC was 1.98 L and 67% of predicted values
(range: 1.27-2.96), average FEV1 was 1.69 L and 62% of predicted values (range: 1.04-
2.42), average FEV25-75 was 1.73 L/min. and 56% of predicted values (range: 1.04-
2.23), average TL was 0.85 L and 69% of predicted values (range: 0.73-0.97).
Conclusion
Patients undergoing thoracic fusion for spinal deformity scoliosis before age three have
significantly reduced TRh, Th and chest dimensions. Early thoracic spine surgery inhibits
the growth and function of the thorax. The number of thoracic vertebrae fused seems to
be related to the severity of the pulmonary impairment.
ªEmans JB and coll. Prediction of thoracic dimensions and spine length based on
individual pelvic dimensions in children and adolescents. Spine 2005;30(24):2824-2829
E-Poster #9

Unilateral Unsegmented Bars, Do They Grow with Distraction?

Hazem Elsebaie, M.D., David L. Skaggs, M.D., Behrooz A. Akbarnia, M.D.

Study Design
Retrospective analysis of 3 patients with congenital unilateral thoracic unsegmented bar
treated with distraction implants with a minimum of 2 years follow-up.
Objectives
To determine the ability of unilateral bars to grow, quantify this growth and the amount
of local coronal angle correction of the unilateral bar by concave side distraction.
Summary of Background Data
Unilateral bars are identified as having absolutely no growth potential, it has always been
assumed that the concave sides of the congenital curve do not grow. A recent study by
Campbell and Hell-Vocke showed that unilateral bars placed under tension by a rib
prosthesis showed an increase in length of the bar. This finding has been questioned and
not repeated to
Methods
Three patients with progressive congenital spinal deformities due to unilateral thoracic
bar treated by distraction implants were analyzed.
Results
First patient, a 7y 8 m old girl treated with single concave growing rod followed for 2+6
years and had 5 distractions. The cobb angle (measured at both ends of the bar) improved
from 48 post initial surgery to 38 at the latest follow up (correction of 20.8%), the bar
increased 5mm in length an increase of 16.6% of the original length of the bar The
second patient, a 9y 1m old girl treated with single concave growing rod followed for 2
years and had 5 distractions. The Cobb angle improved from 40.5 post initial to 29 at the
latest follow up (correction of 28.5%), the bar increased 4mm in length an increase of
(12.5% of the bar) The third patient, a 2y 3m old girl treated with VEPTR followed for 5
y 1m and had 7 distractions. The cobb angle remained almost unchanged and the bar
increased 6mm in length an increase of (28.5 % of the bar)
Conclusion
The unilateral bars seem to grow under concave vertebral distraction; their growth is
much slower than the normal vertebrae under the same circumstances (around 25%) The
coronal Cobb angle of the unsegmented bar was also improved significantly in patients
treated with concave growing rod.
E-Poster #10

Continued Spinal Growth in Early Onset (Juvenille) Idiopathic Scoliosis

Lynn Letko, M.D., Rubens Jensen, M.D.

Introduction
The treatment of early onset (juvenile) idiopathic scoliosis remains a challenge for spinal
deformity surgeons. The goal of treatment is to control an often rapidly progressing
deformity while continuing to allow for an increase in spinal growth. We present a
technique of single rod pedicle screw instrumentation without fusion as a treatment
alternative to allow for continued spinal growth until time of definitive fusion.
Operative Technique
The spine is prepared unilaterally using a scissor or scalpel staying above the periosteum.
The pedicle screws are placed in the area to be instrumented but not fused. A rod is then
placed and the correction is undertaken. The incision is closed. No brace treatment is
required. The patient is followed closely. Pre-operatively, it is discussed with the parents
that the patient will require multiple lengthenings and/or exchange to larger diameter
screw and rods with growth prior to a definitive fusion.
Case Study
4 year 7 mo. old patient with EOIS . At the time of the first surgery (4.05) , 106 cm tall.
The right thoracic curve measured 37° T6 toT11 ( bending correction to 14°). The left
lumbar curve measured 35° T12 to L3 (bending correction to 17°).The patient underwent
right dorsal instrumentation T11 to L3 with correction of the thoracic curve to 15° and
the lumbar curve to 15°. The correction was maintained until the rod broke in
December 2006. The patient was re-instrumented to T8 in 6.07. At the time of revision
surgery, the patient measured 124.5 cm . The curves measured 20° T5 to T11 and 23°
T12 to L3 pre-operatively The curves measured 20° and 15° respectively post-
operatively with 30° thoracic kyphosis and 40° lumbar lordosis.
Conclusions
Unilateral pedicle screw instrumentation without fusion allows for excellent correction of
the spinal deformity and allows for continued spinal growth. Normal thoracic kyphosis
and lumbar lordosis may be maintained. The patient is allowed full activity including
sports. Close follow-up is required as the instrumentation will need to be lengthened or
exchanged prior to definitive spinal fusion.
E-Poster #11

Assessment of Construct Variability Among Experienced Vertical Expandable Prosthetic
Titanium Rib (VEPTR) Users

Michale Vitale, M.D., Jaime Gomez, M.D., Hiroko Matsumoto, MS, Randal Betz, M.D.,
Robert Campbell, M.D., John Emans, M.D., Jack Flynn, M.D., John Flynn, M.D.,
Norman Ramirez, M.D., Brian Snyder, M.D., Sturm Peter, M.D., Kit Song, M.D. John
Smith, Jeffrey Shilt, M.D., David Roye, M.D.

Background
Several reports have demonstrated remarkable variability in construct patterns for
adolescent idiopathic scoliosis and adult deformity correction. To this date, the treatment
for patients with early onset scoliosis has no algorithm. New treatment options are
available such as the vertical expandable prosthetic titanium rib (VEPTR). It is important
to identify variability and trends in treatment to guide surgical decisions.
Methods
As part of the discussion of indications and choice of implant, 13 surgeons were given 12
cases with diagnosis of progressive congenital scoliosis and thoracic insufficiency
syndrome, with standard spine radiographs. The reviewers were asked to choose type of
treatment, type of construct, construct location and whether or not a thoracotomy should
be performed.
Results
In 8 of the 12 cases all surgeons chose to perform surgical treatments. For each of the
remaining 4 cases only one surgeon decided to treat conservatively with casting/bracing
(n=3) or observation (n=1) while the others chose surgical treatments. When the
reviewers chose surgery, 76.3% (range 40-100%) matched using the VEPTR. Of these
VEPTR users 61.2% (range 0-100%) coincided on using it bilaterally. Agreement on the
use of growing rods and fusions was 19.5% (range 0-60%) and 4.2% (range 0-25%)
respectively. Among all cases agreement on whether instrumentation should extend to the
pelvis or not was 70.8% (50-100%). In 5 of 12 cases all surgeons agreed not to perform a
thoracotomy. In 3 of the remaining 7 cases a thoracotomy was chosen by only one
surgeon, in another 2 cases only two surgeons did not agree with the rest of the group. In
the remaining 2 cases about half of the physicians agreed to perform a thoracotomy.
Conclusion
The study found wide variability in choice of construct type, number of constructs and
level of instrumentation, however there was a trend in this experienced group of surgeons
to a common solution. This was illustrated by the data on thoracotomy where there was
less variability and by the agreement that chest wall instrumentation is indicated
frequently.
E-Poster #12

The Development of Thoracic Hypokyphosis/ Lordosis After Dorsal Hemivertebrae

Lynn Letko, M.D., Rubens Jensen, M.D.

Since 1997, our standard treatment of complex congenital thoracic scoliosis is dorsal
hemivertebra resection with or without bar or rib synostosis resection and pedicle screw
instrumentation. We report 3 cases which developed thoracic hypokyphosis/ lordosis
after this procedure with improvement in the sagittal profile after rod removal.
Methods
Retrospective review of 3 patients (3 M) with complex thoracic congenital scoliosis
deformity who underwent dorsal hemivertebrae with or without bar resection with
pedicle screw instrumentation between December 1997 and February 2003. Mean age at
index surgery was 27 mos (range 19 to 33 mos ). Mean follow-up 74 mos (range 45 to116
mos). Mean number of vertebrae resected was 3 (range 3 to 4). Mean number of
segments instrumented was 8 (range 8 to 9) all between T2 and L1.
Results
Mean scoliosis pre-op 82° (range 70 to 102°). Mean thoracic kyphosis pre-op 37° (range
22 to 60°). Mean scoliosis post-op 6° (range 0 to13°). Mean thoracic kyphosis post-op
27° (range 20 to 30°). Development of thoracic hypokyphosis/lordosis surgically treated
with rod removal a mean of 56 mos after index procedure (range 37 to 92 mos) . Mean
kyphosis prior to rod removal was 5° (range minus 5 to10°). Mean kyphosis after rod
removal was 13° (range 0 to 20°) Mean kyphosis at last follow- up or before rod
reinsertion was 21° (range 10 to 32°). Rod reinsertion was indicated in 2 cases of
increasing scoliosis.
Conclusions
Dorsal hemivertabra resection and pedicle screw instrumentation is provides excellent
scoliosis correction in cases of complex thoracic congenital deformity. The posterior
pedicle screw instrumentation may act as a tether resulting in the development of
hypokyphosis /lordosis as the anterior column continues to grow especially in cases of
multilevel instrumentation. Rod removal has allowed for improvement of the thoracic
sagittal profile (plastic deformation versus growth). Rod reinsertion with the rods bent to
match the improved sagittal profile may be necessary in cases of scoliosis progression
E-Poster #13

Growing Rod for Syndromic Scoliosis

Koki Uno, Norihide Sha, Takuya Kimura, Hiroshi Miyamoto, Yoshiyuki Inui, Kou
Tadokoro, Naoatsu Megumi

Twenty-five syndromic scoliosis treated with growing rods were retrospectively
evaluated. There were 9 boys and 16 girls and the average age at first operation was 6.7,
ranged 1.5 to12 years old and average follow-up was 3.5 years. Pathology included
neuromusucular disease in 4, neurofibromatosis in 3, congenital in 3, bone metabolic
disorder in 6, congenital malformation syndromes with mental retardation in 7, thoracic
cage defect in 2. Three were 21 ambulators and 4 non-ambulators. Seven patients were
mentally retarded. The average magnitude of the curve was 95° and 55° after operation
and 60° at final follow-up. Proximal foundation was between T1 to T4 and lowest
foundation were between L1 to sacrum. One hundred and thirty operations were
performed including initial surgery. The average interval between operations for rod
lengthening was 7 months ranging from 5 months to 12 months. Rod lengthening was
performed periodically, based on curve progression of 10° for earlier cases. For recent
cases, lengthening was performed every 6 months routinely. There were 29
complications. Hook dislodgement in 13, deep infection in 3, superficial infection in 2,
rod breakage in 4, others in 2. Of the 29 complications, 18 complications out of 50
surgery occurred in boys and 11 out 80 occurred in girls. Fourteen complications
occurred in early 5 cases. Four patients were performed final fusion due to skeletal
maturity or due to failure of controlling the deformity. One of them suddenly died 2 years
after final fusion . Whether patients have mental retardation or not, and were ambulator
or not did not influence the result. Growing rod for syndromic scoliosis can be performed
with favorable results at this moment comparimg to those of growing rod for juvenile
idiopathic scoliosis.
E-Poster #14

Cell Signaling Pathways Growth of in Mouse Vertebral GP

Chitra Dahia, Eric Mahoney, Atiq Durrani, Christopher Wylie

Introduction
The objective of this study was to delineate signaling pathways present in postnatal
lumbar vertebral growth plate (LVGP), and their expression pattern during growth and
aging.
Material and Methods
Eight micrometer cryosections in the coronal plane were collected from decalcified
lumbar vertebrae of 1-12 weeks old male FVB mice. Histology was analyzed by H&E.
Immunolocalization of components of the TGFbeta1&2, BMP2,4&7, IHH & FGF2
pathways was carried out using confocal microscopy.
Results
Membrane localization of IHH & its receptor PTC was observed in proliferative zone
chondrocytes until 2-weeks of age. Between 2 and 12 weeks of age, active TGF1&2
signaling determined by downstream intermediate (p)Smad2/3 was present at the junction
of the proliferative and early hypertrophic zones which disappeared at 9 weeks. Active
BMP2, 4&7 signaling determined by their downstream intermediate (p)Smad1/5/8 was
found only in the hypertrophic zone chondrocytes at all ages.. FGF2 ligand and activated
receptor were found only in the hypertrophic zone chondrocytes of the LVGP of 2-week
old mice and decreased with age.
Conclusion
Our data suggest that IHH signaling is responsible for maintenance of the PZ while TGF
beta signaling is involved in the transitioning of the cells from PZ to HZ. TGF beta
signaling diminishes with age. Absence of TGF beta signaling marks the end of
proliferative zone and closure of vertebral growth plate despite the presence of IHH.
E-Poster #15

Three Dimensional Analysis of a New Porcine Model for Scoliosis

Virginie Lafage, Ph.D., Frank Schwab, M.D., Ashish Patel, M.D., Jean-Pierre Farcy,
M.D.

Introduction
Spinal fusion remains the mainstay for surgical treatment of severe/progressive
adolescent idiopathic scoliosis. However, there is marked interest in non-fusion
techniques that may spare mobility of the spine and induce correction through growth
modulation. Development of non-fusion techniques requires an animal model with all the
attributes of a scoliotic curve: global deformity but also true vertebral dysplasia and axial
rotation
Methods
This study included 7 Yorkshire pigs. Scoliosis was surgically initiated through a left
pedicle based spinal tether and a left-sided ribcage tethering. Animals were euthanized
once they reached severe deformity (≥50° Cobb) or stopped progressing (mean, 12 weeks
following procedure). Spines were harvested and CT-scans obtained. Axial CT slices
were analyzed to compute vertebral and inter-vertebral height (convex vs. concave) of the
apical functional unit as well as the axial rotation of apical/end vertebrae.
Results
Mean Cobb angle was 52° (SD 13°). Apical mean axial rotation was 18° (SD 9°) toward
the concavity of the curve. Highly significant correlation was found between apical axial
rotation and Cobb angle (r=0.959; p≤0.001). Mean axial rotation of vertebrae outside the
tether was 1.5° (SD 5°). In terms of vertebral and intervertebral heights of the apical
functional unit, no significant differences between anterior and posterior height were
found. However, the convex side was always taller than the concave side: 3.7mm
(vertebral), 1.2mm (Disc), differences p≤0.005.
Discussion & Conclusion
While several authors have reported animal studies related to spinal deformity, none have
conducted a true 3-D analysis to quantify the dysplasia induced by growth modulation.
To our knowledge, this is the first animal scoliosis model reporting a detailed 3-D
analysis of the deformity. Findings in the porcine scoliosis model are promising and
further analysis is in progress to quantify the vertebral deformity (pedicle, body, spinous
process). A greater understanding of scoliotic deformity will emerge as will a more
rigorous analysis of corrective techniques related to growth modulation (ex. fusionless)
E-Poster #16

A Porcine Model for Progressive Scoliotic Deformity

Frank Schwab, M.D., Virginie Lafage, Ph.D., Ashish Patel, M.D., Jean-Pierre Farcy,
M.D.

Introduction
Optimal development of non-fusion techniques requires a large animal scoliosis model.
Several authors have reported creation of spinal deformity in animals but few of these
relate to large animals. Braun et al. demonstrated scoliosis in a goat model. However
there were a number of drawbacks: cyclical breeding, a flattened ribcage, and very severe
deformities. The goal of this study was to develop a reliable porcine scoliosis model, with
moderate deformity, amenable to non-fusion device implantation.
Materials and Methods
This IACUC approved study included 14 Yorkshire pigs; 6 of them were part of a pilot
study to establish surgical technique. Scoliosis was induced in 8 animals through a left
midline ligamentous tethering of the spine, fixated superiorly and inferiorly with pedicle
screws, and left-sided ribcage tethering. Progressive deformity was documented with bi-
weekly x-rays. Frontal and sagittal curves were measured through the Cobb method, axial
rotation was estimated by spinous process deviation (with 0 = midline, -50% = concave
lateral border). Animals were observed until severe deformity (≥50°), and then sacrificed.
Results
Failure of progression was noted in 2 animals due to tether rupture. The 6 animals with
documented progression were observed during 12 weeks. The mean coronal Cobb angle
was 28° immediately post-op and 55° at final follow up. The mean lordosis increased
from 9° post op to 23° at final follow up. Apical axial rotation with the posterior vertebral
elements into the concavity of the coronal deformity increased from -1.35% post op to -
19.76% at last follow up
Conclusions
The study establishes a porcine scoliosis model. With placement of a unilateral
ligamentous tether technique to the spine, combined with concave ribcage ligament
tethering, during the rapid growth stage of Yorkshire pigs; a three dimensional spinal
deformity can be achieved. The speed of scoliotic deformity leaves significant remaining
growth to assess growth modulating therapies for correction. This work forms the basis
for a number of investigative efforts at developing new fusionless therapies for patients
suffering from Adolescent Scoliosis.
E-Poster #17

VEPTR a Two Year Follow Up

Cornelius Wimmer, Peter Wallnoeffer

Since 2004 the treatment with VEPTR is established in Europe. From 2005 to 2007 20
patients were treated with VEPTR. Diagnosis were congenital, neuropathic, and
idiopathic scoliosis.
Material and Method.
There were 3 congenital, 9 neuropathic, and 8 idiopathic scoliosis. The average age of the
15 female and 5 male patients were 7,5 range from 3 to 13 years. Correction of the
primary curve and after lengthening were measured according to Cobb angle before, after
and at the follow up. Complications were noted, a patient satisfaction score and lung
function were measured. 5 of the 20 patients had had previous surgery.
Results
The primary curve measured 65° range from 45 to 130 and improved to 32° range from
25 to 75 at index operation. No complications during surgery were noted. The time at
operation was 125 min. range from 65 to 185. In 15 cases a rib to lumbar spine hyprid
was used, and in 5 cases a combination from rib to rib and rib to lumbar spine were used.
The blood loos during surgery was in mean 125 ml range from 65 to 180. One patient
showed a wound healing, another patient had had a pneumonia, which resolved with
adequate therapy. All patient were braced after surgery. The average stay in the hospital
was 18 days range from 14 to 31. 15 out of 20 patients had 1 to 3 lengthening procedures.
The average correction of the lengthening procedures was 15° 19,8%. In 5 of the patients
the second curve must be instrumented. All patients and parents were satisfied with this
procedure and would do this procedure again.
Conclusion
The first results of the VEPTR instrumentation are encouraging. Remarkable is the low
complication rate and the high patient satisfaction.
E-Poster #18

A Cadaver Spine Study of the Effect of Positional Changes on the Accuracy of Manual
and Digital Radiographic Measurement of Spinal Landmarks

Robert M. Campbell, M.D., A. Reis, A. Gajjar, L. Cooper

It has been assumed that a six foot tube to x-ray plate distance minimizes beam
divergence and magnification error for radiographs of the spine, and that digital
radiographic measurements are more accurate for measurement than manual, but no study
to our knowledge has validated these assumptions. This was studied in a cadaver spine
model using 6 foot tube to plate distance with varied distances from posterior spine to
plate to simulate obesity, kyphosis, or lordosis. Radiographs were taken at standard
tube/plate distance, performed at 2.5cm, 4.5cm, 6.5cm and 8.5cm distances of spine to
the plate, measured manually by micrometer and digitally. For spinal lengths there was
an avg. 4.3% magnification error rate for manual measurements, and 5.7% for the
digitally measured radiographs (p<0.001). The avg. magnification error was 2.7% for the
2.5cm distance radiographs and 7.0% for the 8.5cm group (p<0.001). For the
interpedicular distances there was an avg. 3.3% magnification error rate for manual
micrometer measurement compared to 4.2% for the digitally measured radiographs
(p=0.002). The avg. magnification error was 1.6% for the 2.5cm distance radiographs
and 5.6% for the 8.5cm group (p<0.001).
Conclusion
Manual measurement of radiographic spinal lengths by micrometer is more accurate than
digital measurement, but there is still a magnification error of 4.3%. Increasing the
distance between spine and plate increases the magnification error. Interpedicular
distance measurements also have the same problem with magnification error.
Significance
Investigators should be aware that measurement of the spine on radiographs, even at 6 ft
tube to plate distances, has significant error rates and conclusions about significant
growth in length must be interpreted cautiously. While the manual method seems
superior, both it and the digital method measure only the virtual image of the spine, not
the true dimensions. Increasing the distance of the spine from the plate, either due to
obesity, spine kyphosis, or lordosis, probably increases the measurement error. When
high spine measurement accuracy is needed, perhaps CT scan analysis would be
preferable to radiographic measurement.
E-Poster #19

Posterior Surgery of Scoliosis by Hybrid Instrumentation

Costanzo Giuseppe, Ramieri Alessandro, M.D., Barci Vincenzo, M.D.

The effects of a dynamic fixation are due to an increase of the back stability, reduction of
disk stresses and the chance of restore the sagittal profile. In scoliotic deformities, the use
of shape memory staples in anterior procedures seems promising as also the association
of rigid fixation and “dampers” in the junctional areas. The aim of the present study was
to assess the effectiveness of hybrid constructs in surgical treatment scoliosis of different
etiology. We studied a series of 13 scoliosis of different etiology, both of the growing age
and of the adult, treated with mixed back fixation, that is composed by classical rigid
segmental instrumentation and by “shape memory” elastic interlaminar instrumentation
(in compression at the convex side) in nickel-titanium, implanted cephalad or caudad to
rigid instrumentation, at the apex of compensation curves (in some cases reducible), with
the aim to preserve transition areas. In 11 patients elastic fixation without arthrodesis
facilitated curve stabilization. In 2 patients curve progression less than 10° has been
shown. We did not find either instrumentation mobilization or intolerance. At 2 years
follow-up, no adjacent level degeneration was found. The advantages offered by rigid
segmental fixation in scoliosis treatment are well recognized, in relation both to the entity
of the obtainable correction and to stability, either primary or in a long term basis.
Similarly, adjacent level syndrome may occur above or below the area of arthrodesis.
There are interesting recent multicentric studies about dynamic or hybrid systems in the
treatment of degenerative conditions of the lumbar spine. Applying the principles of
“dynamic” vertebral fixation, it is possible to perform hybrid instrumentations by
positioning interlaminar “shape memory” staples, in order to preserve transitional areas.
Equally, these staples can be implanted on to “cover” compensation curves (often mild
and/or reducible in bending) at the convex side, which facilitate compression reduction.
E-Poster #20

Short-term Results of Dual Growing Rod Technique

Yutaka Nakamura, Takahiro Iida, Akihisa Atou, Kanta Tajima, Junya Katayanagi, Satoru
Ozeki, Yutaka Nohara

Introduction
Severe spinal deformity in young children often progresses rapidly. Recently we started
the dual growing rod technique that was instrumentation with limited arthrodesis only for
cranial and caudal anchor points. We report the short term results of six cases that treated
in this method.
Material and Methods
Between December 2002 and Jun 2006, dual growing rod technique was performed in six
patients. The posterior correction was performed with pediatric ISOLA system. Hooks
were used in the two cranial vertebra and pedicle screws were used in the caudal two
vertebra. Then limited bone graft was performed only for a cranial and caudal anchor
part. Rod extension was scheduled once in a half year. Hard brace was applied until bone
union was provided.
Results
The mean Cobb angle was 107° before surgery and 49° after initial correction. We
performed twenty times of rod extension to maintain spinal growth. There was one
complication with instrument breakage. Case 1: Nine-year-old girl (Sotos syndrome) had
severe and rapidly progressive scoliosis. Preoperative Cobb angle was 84° in Th4-11.
Growing rod was performed from Th4 to 11 with. Cobb angle decreased to 36° after
surgery (correction rate; 57.1%). Six months after the first surgery, the rod extension was
performed and spinal deformity was maintained to 46°. Case 2: A three-year-old male
(Ehlers-Danlos syndrome) had severe thoracic scoliosis of 143° in Th2-L1. Dual growing
rod technique from Th2 to L4 was performed. Postoperative curvature decreased to 82°
with correction rate of 42.7%. However, instrumentation failure occurred after surgery.
Discussion
The dual growing rod technique had good initial correction rate and ability to keep
growth spread. In addition, short hard brace periods reduce the mental and physical stress
of the patients. We consider this technique is good choice as time saving procedure until
final posterior correction and fusion. However, especially in younger patients with bone
fragility, additional technique or device is necessary.
E-Poster #21

Controversies in Jarcho- Levin Syndrome

Norman Ramirez, John M. Flynn, M.D., Alberto S. Cornier, M.D., Ph.D., Simon Carlo,
M.D., Nigel Price, M.D., Frams Pino, M.D.

Jarcho-Levin syndrome is an eponym that has been used to describe a variety of clinical
phenotypes with short trunk dwarfism associated with rib and vertebral anomalies.
Recently molecular, clinical and radiological data has allowed to further characterize
between Spondylothoracic and Spondylocostal dysplasia.
Introduction
This review article will focus on characterizing the differences between Spondylothoracic
and Spondylocostal dysplasia and provide a valuable tool for clinical diagnosis and
management.
Methods
This is a literature review of all Jarcho-Levin studies.
Results
Spondylothoracic Dysplasia (STD) is a rare pleiotropic genetic disorder with autosomal
recessive inheritance. Typical radiological findings include segmentation and formation
defects throughout cervical, thoracic and lumbar spine, such as hemi-vertebrae, block
vertebrae, and unsegmented bars, with fusion of all the ribs at the costo-vertebral junction
(Crab like or fan like configuration) The majority of STD cases previously described in
the literature had a poor prognosis due to respiratory complications such as pneumonia,
congestive heart failure and pulmonary hypertension( mortality rate 42%) . The gene
responsible for this disorder is in the MESP2 gene
Spondylocostal Dysostosis (SCD) constitutes a heterogeneous group of patients with
autosomal recessive and dominant inheritance. They have axial skeletal malformations,
including multiple vertebral segmentation and formation defects and unilateral rib
anomalies. These malformations are typically more prominent in one hemithorax and
frequently leading to a progressive scoliosis of the thoracic spine due to the tethering
effect secondary to the rib anomalies. The majority of cases reported in the medical
literature have a good prognosis, due in part to the asymmetry of the thoracic anomalies
resulting in a less restrictive thorax. The molecular mutations responsible for the majority
of cases of SCD rely on the Dll3 gene on chromosome 19q13.
Conclusion
Jarcho-Levin eponym consists of two distinct pathological conditions. It is essential that
this be recognized since the natural history and management of the spinal deformity is
completely different.
E-Poster #22

Spinal Fusion with Cotrel-Dubousset Instrumentation for Neuropathic Scoliosis

Marco Teli, M.D., Giuseppe Grave, M.D., Alessior Lovi, M.D., Marco Brayda-Bruno,
M.D.

Study Design
Retrospective.
Objective
To report on the treatment of patients with cerebral palsy and neuropathic scoliosis with
third-generation instrumented spinal fusion by Cotrel-Dubousset instrumentation.
Summary of Background Data
Evidence is needed to evaluate the increasing use of third-generation instrumented spinal
fusion in similar patients.
Methods
Patients with cerebral palsy and spinal deformity treated consecutively by 1 surgeon with
Cotrel-Dubousset instrumentation and minimum 2-year follow-up were reviewed. An
outcome questionnaire was administered at final follow-up.
Results
A total of 60 patients were included. Mean age was 15 years at surgery. Mean follow-up
was 79 months. There were 26 anteroposterior and 34 posterior-only procedures.
Correction of coronal deformity and pelvic obliquity averaged 60% and 40%,
respectively. Major complications affected 13.5% of patients (implant loosening, deep
infection, and pseudarthrosis). Outcome questionnaires showed marked improvements in
the areas of satisfaction, function, and quality of life after surgery.
Conclusions
Segmental, third-generation instrumented spinal fusion provides lasting correction of
spinal deformity and improved quality of life in patients with cerebral palsy and
neuropathic scoliosis, with a lower pseudarthrosis rate compared to reports on second-
generation instrumented spinal fusion.
E-Poster #23

Effect of Removal of NP Cells on the AF of Mouse IVD

Chitra Dahia, Eric Mahoney, Atiq Durrani, Christopher Wylie

Introduction
The study was designed to analyze the effect of removal of NP cells on the AF in mouse
lumbar intervertebral discs (IVD).
Methods
The nucleus pulposus cells were surgically aspirated from L2-3 and L3-4 discs of 2-week
old male mice using a 27-gauge syringe. L4-5 disc in the same mouse was sham-operated
as control. The effects on the IVD were assayed 2-8 weeks after the surgery. Eight
micrometer cryosections were collected in the coronal plane and histological analysis was
carried out using H&E staining. Cell death was determined by active caspase-3 staining.
Results
5-weeks following removal of the NP there was significant collapse of the IVD,
compared to the sham-operated controls. By 7-weeks, fibrocartilage cells derived from
the AF were invading the disc space, which became completely filled by 8 weeks.
Growth of the disc was reduced in both cranio-caudal and transverse diameters by 30%,
compared to control discs. At 2 weeks following removal of NP, there was no cell
apoptosis in the AF as determined by active caspase-3 staining. By 8 weeks following the
removal of NP, significant cell apoptosis is seen in the AF.
Conclusion
Removal of NP cells leads to invasion of the disc space by fibrous tissue which is very
similar to that observed in the human degenerated discs. There appears to be a window of
time in which the cells of the AF are alive despite removal of NP cells. A potential exists
for intervention during this time to prevent the AF from degeneration.
E-Poster #24

Thoracolumbar Kyphosis Associated to Vertebral Hypoplasia

Pedro Fernandes, Mauricio Daziano, Lori Dolan, Stuart Weinstein

Introduction
Thoracolumbar kyphosis with a hooked vertebra has been usually associated with bone
dysplasia where the natural history is known to be benign. These anomalies differ from a
true posterior hemivertebra as the anterior defect is frequently incomplete. Posterior
elements are intact which adds also doing the proper differential with the more serious
condition of congenital vertebral dislocation.
Material and Methods
We present 8 patients (6 males, 2 females) with “idiopathic infantile” thoracolumbar
kyphosis, with a mean follow up of 6 years (22 months -12 years). Mean age at diagnosis
was 5 months. Lumbar hypoplasia with a hooked vertebra was present at L1 in five
patients and L2 in three. Three patients were premature and inguinal and umbilical hernia
repairs were performed in four patients. Patients were ruled out for congenital anomalies
and bone dysplasia. All patients were followed without treatment.
Results
Mean Cobb angle at detection was 36D (31-51D). No defects were found in posterior
vertebral elements. Conus medularis ended between T12 and L2 and no relation was
found with level of affected vertebrae. Mean Cobb angle at latest follow up was 9D (0-
33D), with complete resolution of kyphosis and vertebral anatomy in four patients and all
exhibiting improvement. Resolution was time dependent and no patient had or developed
neurological complications.
Conclusion
Infantile thoracolumbar kyphosis due to vertebral hypoplasia can also affect apparently
normal infants. The normal reconstitution of vertebral anatomy with growth and
resolution of kyphosis raises the possibility of a combine etiology where mechanical
overload may impose vascular compromise to centrum formation. Integrity of posterior
elements should make less likely the diagnosis of congenital vertebral dislocation. In
theses cases observation is advised despite some possible overall kyphosis progression
while infants start to sit and stand as kyphosis tends to resolve spontaneously.
E-Poster #25

Instrumented Convex Hemiepiphysodesis in Treatment of Congenital Scoliosis

Muharrem Yazici, M.D., H.Gokhan Demirkiran, Houmen Ahmadi, Mehmet Ayvaz,
Ahmet Alanay, Emre Acaroglu

Anterior and posterior convex hemiepiphysodesis is a widely used surgical alternative in
the treatment of congenital scoliosis. This procedure has the advantage of solving the
problem in a single surgery and the disadvantage of the need anterior and posterior
surgery. Furthermore, outcome may be unpredictable. Posterior convex hemiepipysodesis
with pedicle screws at each segment on the convex side may obviate the need of anterior
surgery and make outcomes more predictible.
Patients who had posterior convex hemiepiphysodesis with convex pedicle screw
instrumentation for congenital scoliosis between April 2004 and April 2006 were
evaluated with preoperative, early postoperative and latest follow-up standing
anteroposterior and lateral radiograms.
Eleven patients with congenital scoliosis (5 male, 6 female) were inculuded. Average
follow-up was 18 months (min. 12 months) and average age at the time of operation was
5.6 years. Average curve magnitude was 48.9° (34-60) preoperatively, 40° (28-50) early
postoperatively, and 38.1° (22-54) at latest follow-up. There were no wound infections or
instrumentation failures during follow-up. In 10 patients curve control or correction was
obtained whereas additional surgery was needed in 1 patient with progression due to a
misplaced pedicle screw.
Our short term results imply that instrumented convex hemiepiphysodesis is a safe and
reliable procedure for the stabilization as well as possible correction of congenital
scoliosis.
E-Poster #26

Cell Signaling Pathways in the Growth of Mouse IVD

Chitra Dahia, Eric Mahoney, Atiq Durrani, Christopher Wylie

Introduction
The aim of the study is to understand the cellular and molecular mechanisms of
intervertebral disc (IVD) growth and development in mouse.
Material and Methods
Eight micrometer thick coronal and transverse cryosections, from lumbar vertebrae (LV)
of one to 48 weeks old male mice were collected. Histology was analyzed by H and E
and alkaline phosphatase staining. The number and thickness of the layers of the annulus
fibrosus (AF), was measured using DIC optics. Cell proliferation and death was
determined by phospho Histone H3 (PH3) and TUNEL staining, respectively.
Immunolocalization of components of the TGF beta, BMP, FGF and Shh pathways was
carried out using confocal microscopy.
Results
During the first week, the AF became divided into a fibrous and mineralized component
as determined by AP staining. Cells in the mineralized component of AF became
hypertrophic with age. Cell proliferation ceased in the AF and NP after 3 weeks of age.
Cells in the NP and fibrous AF secreted Shh which acted on the mineralized AF cells via
its patched (ptc) receptor. FGF signaling was ceased at 4 weeks of age at which time
BMP and Shh signaling significantly increased. PTHrP expression was observed only in
the mineralized AF cells which increased with age.
Conclusion
Analysis of active cell signaling pathways suggests that BMP signaling in the NP and
fibrous AF cells stimulates Shh signaling while FGF signaling inhibits this pathway. Shh
then acts on the mineralized AF leading to increased expression of PTHrP. These
signaling pathways seem to be actively involved in disc maintenance.
E-Poster #27

Surgical Treatment for Severe Cervical Kyphosis in Infants with Larsen Syndrome

Félix Tomé-Bermejo, Garrido E. Tucker, SK Thompson, D. Noordeen, HNN

Summary of Background Data
Larsen syndrome is known to produce severe cervical kyphosis with life-threatening
spinal cord compression. There has been one previous report in the literature treating this
condition with insitu posterior fusion with variable success. We show that correction of
the deformity is possible using preoperative halocervical traction and final correction
with ribstrut grafts and anterior plating.
Objective
To evaluate the safety and efficacy of halocervical traction followed by anterior
instrumented fusion with locking plate, for the treatment of severe cervical kyphosis in
children with Larsen syndrome.
Materials and Methods
Three infants with documented Larsen syndrome and severe cervical hyperkyphosis were
treated between 2004 and 2006 in our institution. All three presented with cord
compression. Two patients, both 1-year-old of age, were treated with preoperative
halocervical traction followed by final correction and stabilisation was with anterior
cervical locking plate and rib strut graft. One patient had undergone posterior
decompression elsewhere and suffered progressive neurological deficit despite attempted
reduction with halocervical traction.
Results
Preoperative kyphosis averaged 130° and was reduced on traction to 70°. Final correction
was obtained with anterior strut grafting and plating. Both patients treated operatively
made a good neurological recovery.
Conclusions
To our knowledge this is the first report describing the safety and effectiveness of
progressive reduction with halo traction followed by anterior strut grafting and internal
fixation with anterior locking plate in very young children with Larsen syndrome.
Correction of the deformity leads to decompression of the spinal cord and neurological
improvement.
E-Poster #28

Dual Growing Rod Instrumentation with a Polyaxial Pedicle Screw System in Early-
Onset Scoliosis

Dezso J. Jeszenszky, M.D., Friederike Lattig, Frank S. Kleinstuck, Tamas Fulop Fekete,
Thomas Forster

Introduction
There are mainly three different systems used currently for the surgical treatment of
severe early-onset scoliosis: single rod, dual rod and the vertical expandable titanium
prosthetic rib implant. Good correction and balance of the spine should be achieved as
early as possible. Therefore a strong primary fixation is necessary also in the thoracic
spine.
Methods
Three patients with early-onset double curve scoliosis were treated with a dual growing
rod system. There were polyaxial pedicle screws implanted also in the upper thoracic
spine in all three patients (T3, T4 in one patient, T4, T5 in two patients). The curves were
distracted under continuous spinal cord monitoring to achieve the maximal correction
possible. The Cobb angles of the scoliosis were measured pre- and postoperatively. The
frequency of lengthening procedures conforms to the curve progression.
Results
Age at surgery was 4.75 (range, 4.3 – 5.6) years. The average preoperative Cobb angle
was 57.7° (range, 55-60) in the thoracic curve and 65.7° (range, 60-75) in the lower
curve. With the first procedure the thoracic curve improved to 29° (range, 19-45) and the
lower curve to 24° (range, 16-39), yielding an over all correction of 57%. The correction
improved due to the lengthening procedures to 18.5° of all curves (70%) at the last
follow-up.
There were one screw loosening, one screw breakage. There were no neurological
complications.
Conclusion
The insertion of pedicle screws in the upper thoracic spine is also safe in young children.
A dual growing rod system with polyaxial pedicle screw fixation is a strong construct to
achieve maximal correction and balance of the spine in severe early-onset scoliosis,
already at the initial procedure. The curves will further be corrected with staged
lengthening manoeuvres.
E-Poster #29

Progressive Cervical and Cervico-Thoracic Kyphosis in Children

Marco Carbone, Gianluca Piatelli, Gilberto Stella, Armando Cama

The Authors report the cases of 5 children with cervical or cervico-thoracic kyphosis that
were treated in their institute.
The objective of the study was to evaluate the effectiveness of the treatment in correcting
deformities and in improving the neurological deficit.
From 2000 to 2007, 5 patients of a mean age of 13 years (6,5-20) were treated. The
etiology of the kyphosis was the outcome of laminectomies for spinal cord tumors (3) and
malformative syndromes (2). In 4 cases there was a deformity of the cervico-thoracic
junction, in 1 patient a medio-cervical kyphosis. All patients had a neurological
involvement, with MRI or electrophysiology signs of spinal cord ischemia; all but one
manifested clinical deficits.
In every patient a Halo-vest or a Halo-plaster was applied, depending on the age and on
the severity of chest deformity. Surgery was performed through a posterior approach,
after a mean period of 3,5 months (1-10) of gradual distraction. A cervical hooks or
screws instrumentation was applied and connected to an analogous thoracic device.
Multiple discectomies and fusion with use of titanium meshes and bone graft were then
performed with an anterior latero-cervical approach. The Halo traction was subsequently
removed, and a Minerva brace was worn for several months. Partial neurological
recovery was observed after surgery. The mean follow-up 1s 3 years (0,5-7); in every
case, arthrodesis remains complete without loss of correction, the neurological status is
stable.
The strategy involving Halo traction, posterior instrumentation and fusion, and anterior
fusion with titanium meshes seems to be appropriate also in the treatment of pediatric
cervical and cervico-thoracic kyphosis. With the latero-cervical approach, the disc
between C7 and T1 can be reached without effort. A early diagnosis may lead to a mild
recovery of the neurological deficit.
E-Poster #30

Growing Rods in Neurofibromatosis Scoliosis

Yasser Elmelligy, Hazem Elsebaie, FRCS, M.D., Wael Koptan, M.D., Mootaz
Salaheldine, MSc

Study Design
Preliminary report on children with neurofibromatosis scoliosis treated with growing rod
technique. Patients included had neurofibromatosis scoliosis with no previous surgery
and a minimum of 6 months follow-up from initial surgery with at least 1 distraction.
Objectives
To determine the safety and effectiveness of the use of growing rod technique in
neurofibromatosis scoliosis.
Summary of Background Data
The growing rod techniques have been used in treating spinal deformities from different
pathologies in pediatric age group .Neurofibromatosis scoliosis in pediatric age group has
a rapidly progressive course, sharp angles and soft bones where gradual distractions
seems suitable. We do not know of any published study exclusively reporting on the
results of growing instrumentation in neurofibromatosis scoliosis.
Methods
From 2005 to 2007, 3 patients (5, 6 and 8 years old) with neurofibromatosis scoliosis
underwent single growing rod procedures using pediatric Isola instrumentation and
tandem connectors. All had curves more than 60° at index surgery 1 had annulotomy as a
first stage and they underwent an average of 3 lengthenings ( 1- 5). Analysis included age
at initial surgery , number and frequency of lengthenings, increase in T1S1 length and
complications.
Results
The mean scoliosis improved from an average of 81° to an average of 40° after initial
surgery and was maintained at 40° at the latest follow up . T1-S1 length increased at an
average of 1.1 cm per year. During the treatment period, 1 complication occurred a loose
set screw leading to loss of correction between lengthening .
Conclusion
The growing rod technique seems to be safe and effective in treating neurofibromatosis
scoliosis. It can offer the best treatment alternative for these resistant and rapidly
progressive curves in the very young age group.
E-Poster #31

Can We Predict Cord Anomaly in Congenital Scoliosis?

Hamid Behtash, M.D., Behrooz A.Akbarnia, M.D., Ganjavian, M.S., M.D., E. Ameri,
M.D., B. Mobini, M.D., S.H. Tari Vahid, M.D., M. Nojumi, M.D.

Background
In congenital scoliosis, we may see some other anomaly in other part of body so as
anomaly of cord, heart, genitourinary system, and . . . .In these patients cord anomaly
had important role in outcome and treatment options. In this paper we want to find
relative factors that correlate with cord anomaly.
Method
In 381 patients with congenital scoliosis, cord anomaly considered with MRI or
myelography and its relation with patients gender, type and site of vertebral anomaly,
curve direction, skin lesions and anomaly in other part of body was considered and
analyzed.
Result
Cord anomaly was seen in 83 patients (21.8 % ) that only 26 ones had neurologic
abnormality in physical examination but had significant correlation (P = 0.000) . Skin
lesions (P = 0.001 ) and type 3 vertebral anomaly (P = 0.01 ) had significant correlation
with cord anomaly .
Conclusion
More than neurologic abnormality in physical examination, in patients with skin lesions
or type 3 congenital anomaly, cord anomaly is more common and complete and careful
cord evaluation should be done.
E-Poster #32

Instrumentation Without Fusion in Early-Onset Scoliosis

Marco Carbone, Gilberto Stella, Francesca Vittoria, Stelvio Becchetti

The Authors review the cases of 25 children with progressive early-onset scoliosis, who
were treated at their institution with growing instrumentation. The objective of the study
was to evaluate the effectiveness of this method in controlling deformities and the
incidence of complications. Surgery was performed with a posterior approach, with
bilateral application of two-level hook claws proximally and distally to the curve. The
periosteum and laminae were exposed only in these sites, with application of bone graft.
Two subfascial rods were inserted on each side of the spine and then jointed with one or
two domino device and connected to the claws. Two transverse connectors completed the
frame. The concavity of the curve was then distracted with spinal cord monitoring. In
most cases, a plaster cast was applied for 3 months and then a Milwaukee brace for 12
months. The patients underwent a lengthening procedure every 12 months with a small
exposition of the domino connectors and a wake-up test. From 2000 to 2007, 25 children
of a mean age of 6.6 years (2-11) were treated with this technique. The etiology of the
scoliosis was infantile idiopathic (10), malformative syndromes (5), NF (3), congenital
(3), others (4). The instrumentation employed were CD Horizon pediatric (11), Isola (6),
Legacy (5), others (3). The mean pre-operative Cobb angle was 81° (60-108); the mean
post-operative was 43°. A total of 72 lengthening procedures were performed, 5 patients
underwent 5 or more lengthenings. The mean follow-up is 45 months (6-86), with a mean
Cobb angle of 48°. 4 patients have completed the lengthening period and are scheduled
for the final fusion, with a good curve control and a fair cosmetic appearence.
Complications were common: 5 breakages of the rods, 1 proximal bilateral hooks
displacement, 2 crankshaft phenomenons, 1 progressive thoracic hyperkyphosis with
bending of the rods. The present technique seems to allow a good control of the spinal
deformity in young children. A stable correction can be obtained also in the sagittal
plane. Mechanical complications are common, but usually easy to resolve. Possible
difficulties in performing the final spine fusion will have to be evaluated.
E-Poster #33

Concave Rib Osteotomy (CRO): A Modified Technique Revisited

Youssry El Hawary, M.D., Ablaa Saleh, Mohamed El Masry

Aim
To report on the efficacy of CRO in conjunction with posterior instrumented fusion in the
treatment of adolescent idiopathic scoliosis (AIS).
Summary of Background
The accepted treatment for large rigid curves is a combined anterior release with
posterior fusion which has been demonstrated to have 10.2 percent. pulmonary
complications. The concept of (CRO) was introduced by Flinchum in 1963. Cadaveric
studies tested flexibility before and after sectioning the ribs on the concave side, and
found an average increase in deflection of 53 percent.
However there are little published data reporting the efficacy of such technique.
Methods
A prospective cohort of 78 patients diagnosed as having AIS with a Cobb angle greater
than 70°. All patients underwent posterior instrumented spinal fusion, iliac crest bone
grafting and CRO. Our modifications of the technique included using a gauze swab
underneath the osteotomised ribs to protect the underlying pleura, and creating a sling to
lift the lateral ends of the ribs above the concave rod. We also filled the area with saline
together with the use of positive ventilation to check for air leaks. No chest tubes were
inserted routinely.
Pulmonary rehabilitation program (PRP) commenced one week postoperatively.
Results
The overall incidence of pulmonary complications was 11.5 percent (9 patients). Two
patients had air leaks from the pleural cavity intra-operatively. Post-operatively, four
patients developed pneumothorax; and three had a pleural effusion. There were no post
operative wound complications and no neurological complications. There was a mean of
68 percent curve correction.
At 2 years follow up, there was no evidence of pseudoarthrosis, but there was a mean loss
of correction of 3° (range 2-4).
Conclusions
The addition of CRO to posterior instrumented fusion in patients with AIS is a safe and
reliable method in the treatment of severe and rigid curves.
E-Poster #34

Role of P factor in AIS Initiation and Curve Progression

Alain Moreau, M.D., Bouziane Azeddine, M.Sc., Anita Franco, M.Sc., Isabelle Turgeon,
B.Sc., Mamadou Samba Boiro, B.Sc., Sacha Blain, B.Sc., Hugo Boulanger, B.Sc., Keith
M. Bagnall, Ph.D., Benoit Poitras, M.D., Hubert Labelle, M.D., Charles-Hilaire Rivard,
M.D., Guy Grimard, M.D., Jean Ouellet

Introduction
Adolescent idiopathic scoliosis (AIS) is the most common form of scoliosis and we
hypothesized that scoliosis development in AIS patients and different melatonin-deficient
animal models could be induced by a similar mechanism involving a common
downstream effector regulated by melatonin. In that context, we have identified a specific
molecule, that we named the P factor and tested the hypothesis that elevated plasma P
factor relates to AIS initiation and curve progression.
Methods
We studied 109 patients with AIS divided into two subgroups according to the severity of
their spinal curve (Cobb’s angle ≤ 45° vs. ≥ 45°) and 37 healthy control subjects. Plasma
concentrations of P factor and soluble P factor receptor (sFPR) were measured by
enzyme-linked immunosorbent assays. Unilateral (one-tail), unpaired Student’s T-tests
with equal variance were performed to compare the difference in plasma levels between
AIS and control groups. We also studied genetically modified bipedal C57Bl6/j mice
devoid of either P factor or its receptor to assess the mechanism whereby this secreted
factor triggers scoliosis.
Results
Plasma concentrations of P factor were significantly higher in patients with AIS having a
Cobb’s angle ≥ 45° than AIS patients with a Cobb’s angle ≤ 45° or in controls (1028.34
ng/mL, 733.18 ng/mL and 561.39 ng/mL respectively). Conversely, plasma sFPR
concentrations were significantly decreased in both AIS patients groups when compared
to controls (535.44 ng/mL, 547.64 ng/mL and 864.13 ng/mL respectively). Bipedal
C57Bl6j mice developed a scoliosis in a proportion of 45% while none of the genetically
modified bipedal mice developed a scoliosis.
Conclusions
Elevated plasma P factor and low sFPR levels were found to be associated with scoliosis
initiation and curve progression. Study of genetically modified C57Bl6/j mice further
confirmed that scoliosis induction by P factor was mediated through interactions with its
receptor while sFPR can act as a disease modifying factor by interfering with P factor
signaling. Moreover, these biochemical tests can be performed without any prior
knowledge of mutations in any defective genes causing AIS.
E-Poster #35

The Meaning of the Autonomic Nerve System in Etiology of AIS

Martin Repko, Drahomir Horky, Martin Krbec, Richard Chaloupka, Irena Lauschova

Introduction
The main purpose of this study is to search the possible causation of idiopathic scoliosis
in development changes of the autonomic nerve system (ANS). In our prospective study
we followed the changes of autonomic nerve structures, as well as discrepances between
concavity and convexity of the scoliotic curve.
Material and Methods
We evaluated 9 patients treated for idiopathic scoliotic deformities and control set of 3
patients without scoliotic deformity within the period March-November 2005. We took
the samples of peripherial autonomic nerves from convexity and concavity of the
scoliotic deformity during the surgical correction using the transthoracic approach. We
examined the samples using the electronmicroscopic method. Then we applied the
morphometric statistical evaluation for comparison with control samples which have been
taken during the surgical treatment of non-scoliotic patients.
Results
There were 23,71% of myelinised nerve fibres (MNF), 12,21% of unmyelinised nerve
fibres (UNF) and 5,0% of Schwanns cells (SC) in samples from scoliotic convexity
measured by morphometric method. There were 17,36% of MNF, 5,82% of UNF and
5,27% of SC in samples from scoliotic concavity . In control non-scoliotic samples there
were 29,9% of MNF, 19,9% of UNF and 16,7% of SC.
Discussion and Conclusion
Trunk and rib cage developmental asymmetry seems like one of possible etiology of
idiopathic scoliosis. These deformities can be induce by asymmetrical intercostal
vascular nutrition due to changes in ANS. There were the main morphological
abnormalities of ANS in scoliotic concavity expressed in the electronmicroscopic
evaluation as degenerated nerve fibers, massive lesion and separation of the myelin
sheath, vacualization of the Schwanns cells cytoplasma and condensation of the
cytoblast. All these changes had been not found in control set of patients without scoliotic
deformities.This findings can help us in the search for the scoliotic etiology.
E-Poster #36

The Changes of Scoliotic Sagittal Profile

Martin Repko, Martin Krbec, Richard Chaloupka, Milan Leznar, Vladimir Tichy, Tomas
Obtulovic

Aim
Surgical correction of the frontal as well as sagital planes impact the standing and sitting
stability. The aim of our prospective study is the measurement of the changes in sagittal
plane in correlation with types of segmental instrumentation for posterior approach.
Material and Methods
We evaluated 96 scoliotic patients surgically managed by posterior correction and
stabilisation by various segmental instrumentations (USS, SSE, ISOLA, Miami, TSRH).
There were 76 idiopathic and 20 neuromuscular scoliotic deformities. We evaluated x-ray
parameters on sagittal films. We assessed kyphosis and lordosis in degrees according to
Cobb. Sagital tilt a T9 tilt has been assessing according the degrees according to Duval-
Beaupere.
Results. There were mean preoperative values of the thoracic kyphosis +24,0° (+/-18,3)
and postop +24,3 (+/-15,6) in idiopathic scoliotic group. Lumbar lordosis were preop -
52,7 (+/-23,1) and postop -54,9 (+/-15,5). In group of neuromuscular scoliosis there were
correction of thoracic kyphosis from preop values +42 (+/-18) to postop +36 (+/-10).
Lumbar lordosis has been corrected from preop value -60 (+/-14) to postop -49 (+/-12).
In group of idiopathic scoliosis there were reduction of the T9 tilt from preop +7,7 (+/-
9,7) to postop +7,1 (+/-4,3), and sagittal tilt from preop +2,7 (+/-4,4) to postop +2,1 (+/-
4,4). In group od neuromuscular scoliosis there we reduction of the T9 tilt from +6,8 (+/-
4,9) to postop +3,1 (+/-5,9), and sagittal tilt from preop +3,1 (+/-5,9) to postop +1,6 (+/-
2,5).
Conclusion. Surgical technique of the segmental instrumentation in scoliotic deformities
allows good correction not only in the frontal plane, but also in sagittal plane. There were
statistically significant changes in the group of neuromuscular scoliosis. There were not
the significant statistical changes in types of segmental instrumentations.
E-Poster #37

Neck Muscle Asymmetry in a Case of Idiopathic Scoliosis

Asghar Rezasoltani, Heikki Kauhanen, Ph.D., Veikko Avikainen, M.D.

Background and Purpose
Different methods have been used in the evaluation of the patient with idiopathic
scoliosis. Ultrasonogeraphy is a non-invasive technique in the study of muscle function.
The purpose of this study was to evaluate the neck semispinalis capitis muscle (SECM)
size in a patient with idiopathic scoliosis.
Case Description
The subject was a fifteen-year old high school female student. The Cobb angles were 43°
for the thoracic curve and 25° for the lumbar curve.
Outcomes
The clinical diagnosis was idiopathic scoliosis with primary right thoracic and
compensatory left lumbar curves with no curvature in the cervical region. The cross-
sectional area (CSA) and linear dimensions of the SECM were measured by real-time
ultrasonography. The CSA was 25.3 % smaller on the right side than on the left side. The
muscle was rounder on the left side than on right side. The difference between the sides
was outside the normal range as determined in our earlier study in healthy females (0-16
%).
Discussion
Ultrasonography appeared be a useful method for screening muscle asymmetry in a
patient with idiopathic scoliosis.

References
1. Rezasoltani A, Kallinen M, Malkia E, Vihko V. Neck semispinalis capitis muscle size
in sitting and prone position measured by real-time ultrasonography. J Clin Rehabil.
1998; 12: 36-44
Cases

Case #1

Posterior Hemivertebra Resection without Fusion in a Two Year Old Child

Dezso J. Jeszenszky, M.D., Tamas Fulop Fekete, Friederike Lattig, Frank S. Kleinstuck

Introduction
Resection of a hemivertebra from a posterior approach is a widely accepted technique.
This technique requires a short segment fusion. Aiming for motion preservation at the
mobile lumbar area by avoiding fusion is worth considering. We present a case and
describe a fusionless surgical technique. A surgical method to correct spinal deformity by
fusionless hemivertebra resection in congenital scoliosis has not been reported before.
Surgical Technique
24 months old girl with a semi-incarcerated hemivertebra between the L2 and L3 on the
right side, resulting in progressive scoliosis, was operated. Preoperative radiographs and
CT scans showed that reconstruction of a facet joint between the right articular processes
of the L2 and L3 vertebras is technically feasible. The surgical technique consisted of
placing transpedicular screws in the right pedicles of L2 and L3, removal of the
hemivertebra, taking care to preserve the joint capsules and the cranial endplate of the
hemivertebra. After resection, a posterior tension band was applied by connecting the
screw heads with wire. The joint capsules were connected by sutures. A brace was
applied postoperatively for 3 months, the implants were removed subsequently.
Results
Preoperative the right convex lumbar scoliotic curve was 41° between L1 and L4, sagittal
kyphotic deformity was 12° between L2 and L3 with subluxation. 14 years later, the
scoliotic curve was 5° between L1 and L4. The kyphotic deformity has been corrected,
the lordosis was 12° between L2 and L3 at followup.
Conclusion
Correction surgery of congenital scoliosis should be performed early, before the
development of severe local deformities and secondary structural changes. This allows
normal growth in the unaffected parts of the spine. Posterior resection of the
hemivertebra with transpedicular instrumentation allows for excellent correction in the
frontal and sagittal planes.
When performing surgery for hemivertebra resection, careful anatomical analysis is
recommended to asses the applicability of above described technique, to preserve motion
in the affected segment.
Case #2

Infantile Idiopathic Scoliosis

Toru Maruyama, Katsushi Takeshita

At the first presentation, when the patient was 1 year old, he had right thoracic curve
from T5 to T11 with Cobb angle of 21° in the lying position. His curve gradually
progressed to 53° when he was referred to our clinic at the age of 5y 1m. Despite bracing,
his right thoracic curve from T5 to T11 and left lumbar curve from T11 to L4 progressed
to 81/55° at the age of 10y 8m. His 81° thoracic curve was corrected to 61° on the supine
bending radiograph and 55° lumbar curve was corrected to 24°. At the age of 11y 3m he
underwent definitive fusion surgery, anterior release from T8/9 to T10/11 using video
assisted thoracoscopy and posterior instrumentation from T4 to L1 using hooks,
sublamina cables, and pedicle screws. His curve was corrected to 25/20° and the
correction was maintained 6 months after surgery. How would be the results changed if
we could use the growing rod system?
Case #3

Congenital Myopathy

Toru Maruyama

Scoliosis with congenital myopathy, type 1 fiber predominance.
On newborn, the patient had asphyxia, dyspnea, dyaphagia, and eye and facial palsy. For
the first 2 months she had been respirator dependence. At the age of 2, she began
ambulation and oral intake. A magnitude of her left thoracolumbar curve from T8-L3 was
25° at the age of 1y 5m in the supine position, but deteriorated to 70° at 2y 6m in the
sitting position. Despite bracing, her curve gradually progressed to 112°, which was
corrected to 44° on the supine side-bending radiograph. At the age of 9y 9m she
underwent definitive fusion surgery, posterior instrumentation from T5 to L3 using
hooks, sublamina cables and pedicle screws. Her curve was corrected from 112° to 30°
by the surgery and the correction was maintained as Cobb angle of 31° 1 year after
surgery. How would be the results changed if we could use the growing rod system?
Case #4

Juvenile Idiopathic Scoliosis

Toru Maruyama, Katsushi Takeshita, Tomoaki Kitagawa

At the first presentation, when the patient was 4 year and 11 month old, she had right
thoracic curve from T5 to L1 with Cobb angle of 44°. Despite bracing, her curve from T5
to T11 progressed to 77° at the age of 9y 2m. At the age of 10y1m her 87° thoracic curve
was corrected to 79° on the supine bending radiograph. At the age of 10y 6m she
underwent definitive fusion surgery, anterior release from T5/6 to T10/11 using video
assisted thoracoscopy and posterior instrumentation from T2 to L2 using hooks,
sublamina wires and pedicle screws. Her curve was corrected from 113° to 52° by the
surgery and the correction was maintained as Cobb angle of 51° five years after surgery.
How would have been the results changed if we could use the growing rod system?
Case #5

Congenital Scoliosis Case Report

Sumon Bhattacharjee, M.D.

HF is 7 year-old female with a known congenital hemivertebrae with associated other
anomalous vertebral body formation. The patient at the age six (6+0 years) underwent a
convex growth arrest procedure with posterior convex arthodesis without
instrumentation. The patient continued to experience progression of her thoracolumbar
curve from 32 to 56° over a six-month period. There was overall loss of truncal balance
with an associated kyphotic deformity of 50° across the thoracolumbar junction. The
patient was also noted to have some gait difficulties, with MRI demonstrating the
presence of a syrinx at the level of the conus medullaris. The patient on the bending x-
rays showed an overall flexible curve but significant rigidity across the apex of the
deformity, which was associated with the hemivertebrae. The patient at age 7+1 years
underwent a posterior only approach for correction of her deformity. Multilevel Smith –
Petersen osteotomies were performed across the posterior fusion mass. Posterior based
hemivertebrae resection was achieved and pedicle screw instrumented fusion was
performed across the segments from T11to L3. The postoperative follow-up x-rays,
coronal curve was measured at 18°, with 10° thoracolumbar kyphosis on the sagittal
plane. Restoration of spinal balance was achieved and improvement in gait and mobility
was noticed. There were no adverse peri-operative complications. This case report
demonstrates that when convex growth arrest fails in congenital deformities, aggressive
posterior only correction methods with pedicle screws instrumented fusion in young
children can safely achieve spinal balance.
Case #6

L4-5 High Grade Spondolysthesis in a Skeletally Immature Patient

Sumon Bhattacharjee, M.D.

High grade spondolysthesis in the pediatric skeletally immature patient and its surgical
correction is often reported involving the L5-S1 segments. No particular surgical
correction strategy for high-grade L4-5 spondolysthesis could be identified in the
literature after an extensive Medline search.
SR was 11+11 year old pre-menarche female, presented with hamstring tightness and a
depression in the lumbar region that was noted by her mother. The patient was Risser 0,
and x-ray revealed the presence of Grade IV spondolysthesis. The patient on the coronal
plane was noted to have a reactive scoliosis, with a thoracolumbar deformity measuring
at 15°. Brief management with TLSO brace and leg extension was tried, with no
significant success. MRI scan, demonstrated prominent herniated disc with doming of
the L5 superior endplate. The sagittal x-rays demonstrated a prominent slip angle of 62°
at the level of the spondolysthesis.
A novel correction technique, with an entirely posterior based approach for the deformity
correction was applied. Pedicle screws were placed from L3 though S1. Long post
reduction screws were placed at L4, and short post at the other levels. Interbody fusion
with PEEK interbody cage was performed at the L5-S1 level. Wide decompression with
extended exposure of the both the L4 and L5 nerve was achieved bilaterally. A through
disckectomy with bilateral approach was performed, and complete exposure and
preparation of the bony endplates. Osteotomy of the L5 dome endplate was performed.
Using the L5-S1 as the base of the construct, the L4 segment was reduced on the superior
endplate of L5, with close neurophysiological monitoring. Bone-on bone fusion was
achieved between L4-L5, with significant correction of slip angle. Postoperative follow-
up shows posterolateral and interbody fusion and maintenance of slip angle correction
without any neurological sequale. There was resolution of the reactive thoracolumbar
scoliosis.
Case #7

Lumbar Agenesis: From Intrauterine Diagnosis to Spinal Reconstruction

Marco Teli, M.D., Giuseppe Grava, M.D., Alessio Lovi, M.D., Marco Brayda-Bruno,
M.D.

Study Design
Follow-up of a case of lumbar agenesis treated with spinal reconstruction, with review of
the pertinent literature.
Background
Congenital absence of isolated segments of the lumbar spine is rarely described.
Differential diagnosis includes congenital dislocation of the spine, lumbo-sacral agenesis,
spinal dysgenesis and spina bifida.
Methods
The absence of spinal segments was suspected on an ultrasound scan and confirmed by
X-rays at birth of a female showing thoracolumbar kyphosis and meningocele with
bilateral talipes and paraplegia. X-rays confirmed absence of T12, L1, L2 and partially
L3, with presence of spina bifida of the caudal segments. The spine was protected by
serial casting and bracing until the eight year of life, when progression of kyphosis
mandated spinal reconstruction. This was performed through a postero-lateral approach.
Results
Deformity improved to normal kyphosis after posterior spinal fusion. The girl 36 months
after surgery is enjoying pain-free activities, attending school and playing in sitting
position.
Conclusion
Lumbar agenesis has sporadic incidence. No etiologic factor is known. Its functional
prognosis is dependent on the level of neurologic impairment. Survivorship into
adolescence has been described. We showed the disease may be suspected by uterine
ultrasound before birth, and spinal reconstruction can be undertaken in the occurrence of
progressive deformity, with promising results.
Case #8

A Rare Case of Spinal Pediatric Osteoblastoma

Gianluca Piatelli, Marco Carbone, Carlo Gandolfo, Andrea Rossi, Paolo Nozza, Miriam
Tumolo, Armando Cama

Purpose
The aim of this study is to report a pediatric case of osteoblastoma associated with an
aneurysmal bone cyst (ABC) involving the lumbar spine.
Materials and Methods
A 2 year-old male with back pain, right lower limb tremor and progressive inability to
walk underwent clinical, MRI and CT examinations. They showed a pathological L2
vertebral body with a large amount of extra and intraspinal soft tissue containing large
flow voids, with a compression of the conus apex and of nerve roots. Owing to a
progressive neurological deficit, the child was operated on emergency. Profuse bleeding
occurred during surgery: only a partial spinal decompression and histological diagnosis
was possible. The patient then underwent catheter angiography, showing a highly
vascularized mass that was selectively embolized with cyanoacrylate glue trough lumbar
arteries in order to decrease the degree of vascular feeding and intra-operative
hemorrhage risk. Then a first posterior surgical approach was performed with
decompressive laminectomy and posterior arthrodesis instrumented with hooks from
T11-T12 to L2-L3. A second anterior approach was performed with right
hemicorpectomy and arthrodesis with a cage between L1-L3.
Results
Complete neurological recovery after surgery was obtained.
Conclusion
Osteoblastoma is a rare primary bone neoplasm derived from well-vascularized stroma of
connective tissue. Osteoblastoma may affect any bone; in the spine, it involves most
frequently the posterior arches. The diagnosis is based on a combination of plain
radiography, CT, MRI, and angiographic studies, but can be very difficult. A biopsy is
often necessary for a histological diagnosis. While osteoblastoma can be histologically
undistinguishable from osteoid osteoma, both clinical/neuroradiological features and
biological behavior can be significantly different. As many as 10% of patients with
osteoblastoma have associated ABC components that may potentially bleed, either
spontaneously or during surgery. Pre-operative selective embolization is useful to reduce
bleeding risk during surgery. The goal of the treatment is the complete removal of the
lesion and spinal stabilization is often required.
Case #9

A case of Rapidly Progressive Infantile Scoliosis

Kazuhiro Sato, Ken Yamazaki, Hirooki Endo, Satoshi Yoshida, Hideki Murakami,
Tadashi Shimamura

Case
Two year old infant, male
Past Medical History
The patient had not been treated although scoliosis was noticed by his mother since his
birth.He was diagnosed with scoliosis at the age of 1 year and was referred to our
hospital. Upon the first visit, a left convex thoraco-lumber curve (Apex Th11) was
observed. The Cobb angle was 32°. No congenital abnormality was seen in his spinal
column. The patient remained under observation as an outpatient, but the angle
aggravated to 54° at the age of 1 year and 9 months. At that time, treatment by an under
arm brace was initiated. At the age of 2 year and 1 month, his condition rapidly
aggravated to a Cobb angle of 75°, and was admitted for a surgical operation.
Physical Findings
Height 82.5 cm, Body weight 11.5 kg.
Operation Method
Minimal exposure was given. A pedicle screw (3.5mm in diameter, 20mm in length) was
to be inserted in the vertebrae at L3 and L4. However, the pedicle diameters at Th3 and
Th4 were found to be 2.2mm and 1.7mm, respectively, according to pre-op CT. No
screws compatible with these sizes were available. Therefore, pedicle screws(3.5mm in
diameter, 20mm in length)were inserted by the in-out-in method under the fluoroscopic
observation of the pedicles, and facet fusions at Th3/4 and L2/3 were performed.
Extension of the correction was made with a wedding band using a Titanium 4.75 mm
dual-rod. The Cobb angle improved to 21° post-operatively.
Discussion
Rapidly progressive infantile scoliosis must be treated at an early stage. However, no
effective treatment method has been established yet. In a rapidly progressive case, the
preparation of strong anchors for in-out-in method at an early stage seems to be indicated.
In this case, 3.5mm cervical pedicle screws were inserted by the in-out-in method and
used as anchors. Considering the current situation in which there is no suitable type or
size of pedicle screw system available, the decision to shorten the time required in order
to prepare a strong anchor by fixation seemed appropriate in this rapidly progressive case.
Conclusion
The pedicle screw system is a good method to shorten the time required to prepare a
strong anchor in a rapidly progressive case.
Case #10

Navigation Surgery in Juvenile Congenital Scoliosis

Katsushi Takashita, Toru Maruyama, Atsushi Seichi, Kozo Nakamura

Relatively small sizes of the spine have been hindering full adaptation of the pedicle
screw system in infantile/juvenile scoliosis surgery. Individual unique shapes are also
major concern in congenital cases. Though several surgeons reported the beneficial use of
navigation system in scoliosis surgery, its effectiveness in infantile scoliosis might be
obscured because of its tiny structure as well as a relatively large component of cartilage
which is not usually incorporated in constructing the virtual spine. We report of the
successful utilization of navigation technique in placing pedicle screws in small and
deformed congenital scoliosis. Eight-year-old presented with back deformity with
congenital scoliosis. The Cobb angle was 46° from T5 to T12. Computed tomography
revealed right unilateral unsegmented bar at T6 to T9, left hemilamina at T10 and right
fused laminae at T7 to T9. Posterior-only in situ fusion was planned. Preoperative CT
data was input into the navigation software, and the placement level, direction and depth
of screws of each pedicle screw placement were determined in accordance of individual
spine shape. Segmental pedicle screw instrumentation with ten screws was performed
with no sequelae. With three-year follow-up, there is no instrument failure, no Crankshaft
phenomenon, and no progress of the decompensation curve. Navigation system is useful
in placement of pedicles screws as well as in individualized preoperative planning.
Case #11

Use of VEPTR in Severe Infantile Scoliosis Below Age 6

Pierre Lascombes, T Haumont, P Journeau

The treatment of severe infantile scoliosis remains a challenge. When bracing is unable to
stop the progression of the curve, a surgical procedure can be considered. Subcutaneous
rods have some disadvantages including early ossification and the inherent difficulty of
the final surgical arthrodesis. Rib distraction is an alternative which could be more
efficient, as the corrective strength is lateral to the spine.
Material and Method
One girl, aged 6 years developed an upper thoracic 90° curve after a complex congenital
diaphragmatic hernia. Two concave VEPTR were inserted, one from rib 3 to 9 and one
between the rib 4 and L1.
One boy, three years old, had an idiopathic thoracic scoliosis of 90°. One distractor was
fixed on the left concave side between rib 3 and L3; the second distractor was placed on
the right convex side from rib 3 to the iliac crest. Lengthening was performed once per
year.
Results
One major difficulty resides in the size of the implants, including the vertebral implants,
in this age group. In case 1, it was impossible to insert a secure double laminar hooks on
L1. The case 2 was programmed to have a bilateral fixation from the iliac crest, but it
would have been totally impossible to close the surgical wound, as the iliac hook was too
big. In both cases, the proximal rib fixation dislodged when the distal part was anchored
on a lumbar vertebrae or the iliac crest, necessitating a change of proximal anchorage. A
new rib was spontaneously remodelling below the hook. However, the CTscan showed
clearly the inappropriate diameter of the rib hook compared to the rib size, which
probably led to a poor anchorage.
Discussion
Adaptation of implant size must be considered for younger children, including the rib
hooks, the vertebral and iliac implants.

								
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