International Congress on Early Onset Scoliosis and Growing Spine
November 2-3, 2007
Chairman: Behrooz A. Akbarnia, M.D.
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.
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
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).
This study demonstrated that anterior spinal arthrodesis or instrumentation in the
immature spine may result in iatrogenic spinal stenosis.
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.
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
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:
The vertebral growth continues during growing rod instrumentation. Distraction forces
might stimulate also apophyseal growth of axial skeleton.
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,
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.
We compare the outcomes of 3 surgical treatments for congenital deformities due to a
focal hemivertbra (HV).
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.
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).
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.
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.,
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.
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.
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.
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)
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.
Thoracostomy in conjunction with VEPTR implantation partly reversed the constricted
hemithorax created in young rabbits thereby partially improving lung growth and alveolar
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.
Thoracostomy in conjunction with VEPTR implantation reverses impaired respiration by
increasing the volume of the constricted hemithorax to allow partial resumption of lung
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.
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.
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
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.
0 pts. Avg f/u 5.75 yrs ( 2-11.5 yrs ) Unilat hybrids 8 pts., bilat 2 pts.
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.
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.
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.
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
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
Measurement of Forces Generated During Distraction of Growing Rods
Marco Teli, M.D., Giuseppe Grava, M.D., Alessio Lovi, M.D., Marco Brayda-Bruno,
Consecutive series of measurements.
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.
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.
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.
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.
Single Growing Rods (Review of 21 cases). Changing the Foundations: Does it Affect the
Hazem Elsebaie, FRCS, M.D.
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.
Evaluation of a newer version of single growing rod technique inserted sub muscularly.
Changing the foundations in the single rod may change the results.
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.
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
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
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.
Safety and Efficacy of Growing Rod Technique for Pediatric Congenital Spinal
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.
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.
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.
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).
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.
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.
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.
Iatrogenic Thoracic Outlet Syndrome as a Complication of VEPTR
Steven Mardjetko, M.D., Ahmad Nassr, M.D., Benjamin Crane, M.D., Kim Hammerberg,
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Long-term Results of Congenital Scoliosis Treatment
Martin Repko, Martin Krbec, Jan Burda, Jan Pesek, Richard Chaloupka, Vladimir Tichy
The aim of our study is to evaluate clinical and X-ray long-term results of congenital
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.
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
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
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,
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.
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.
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.
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.
Short Anterior Instrumented Fusion of Lateral Hemivertebra
Enrique Garrido, MRCS, EBOT, F. Tome, SK Tucker, TR Morley, HNM Noordeen
Retrospective study with clinical and radiological evaluation of 29 patients with
congenital scoliosis who underwent 31 short segment anterior instrumented fusions of
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.
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.
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
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
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.
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.
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.
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.
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.
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
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.
More severe curves and multiple congenital vertebral anomaly may be factors that
necessitate the combined anterior and posterior operation.
PRSS in Management of Adolescent and Juvenile Scoliosis
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
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 .
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.
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.
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.
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.
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
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.
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.
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.
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,
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
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
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.
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.
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
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
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.
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).
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.
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.
With anterior instrumentation decancellation of the hemivertebra is safer and simpler than
hemivertebrectomy with same effectiveness in the very young children.
Spinal Open-Wedge Osteotomy
Dezso J. Jeszenszky, M.D., Tamas Fulop Fekete, Martin Sutter, Friederike Lattig,
Andreas Eggspuhler, Frank S. Kleinstuck
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.
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.
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.
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.
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.
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.
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.
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.
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
Nutritional Improvement Following VEPTR Surgery in Children with Thoracic
David L. Skaggs, M.D., Josh Albrektson, Tishya Wren, Robert M. Campbell, M.D.
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.
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.
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.
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.
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
Surgery Before Age 3 in Patients with Congenital and Syndromic Scoliosis Followed
Until the End of Growth
Charles D'Amato, M.D.
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.
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).
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
Unilateral Unsegmented Bars, Do They Grow with Distraction?
Hazem Elsebaie, M.D., David L. Skaggs, M.D., Behrooz A. Akbarnia, M.D.
Retrospective analysis of 3 patients with congenital unilateral thoracic unsegmented bar
treated with distraction implants with a minimum of 2 years follow-up.
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
Three patients with progressive congenital spinal deformities due to unilateral thoracic
bar treated by distraction implants were analyzed.
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)
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.
Continued Spinal Growth in Early Onset (Juvenille) Idiopathic Scoliosis
Lynn Letko, M.D., Rubens Jensen, M.D.
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.
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.
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.
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.
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.
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.
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
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.
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
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.
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.
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
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
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
Cell Signaling Pathways Growth of in Mouse Vertebral GP
Chitra Dahia, Eric Mahoney, Atiq Durrani, Christopher Wylie
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
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.
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.
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.
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,
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
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.
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)
A Porcine Model for Progressive Scoliotic Deformity
Frank Schwab, M.D., Virginie Lafage, Ph.D., Ashish Patel, M.D., Jean-Pierre Farcy,
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.
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
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.
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
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.
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.
The first results of the VEPTR instrumentation are encouraging. Remarkable is the low
complication rate and the high patient satisfaction.
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).
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.
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.
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.
Short-term Results of Dual Growing Rod Technique
Yutaka Nakamura, Takahiro Iida, Akihisa Atou, Kanta Tajima, Junya Katayanagi, Satoru
Ozeki, Yutaka Nohara
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.
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.
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.
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.
This review article will focus on characterizing the differences between Spondylothoracic
and Spondylocostal dysplasia and provide a valuable tool for clinical diagnosis and
This is a literature review of all Jarcho-Levin studies.
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.
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
Spinal Fusion with Cotrel-Dubousset Instrumentation for Neuropathic Scoliosis
Marco Teli, M.D., Giuseppe Grave, M.D., Alessior Lovi, M.D., Marco Brayda-Bruno,
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.
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.
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.
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.
Effect of Removal of NP Cells on the AF of Mouse IVD
Chitra Dahia, Eric Mahoney, Atiq Durrani, Christopher Wylie
The study was designed to analyze the effect of removal of NP cells on the AF in mouse
lumbar intervertebral discs (IVD).
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.
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.
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.