Progressive scoliosis develops in most patients with
Duchenne muscular dystrophy (DMD) due to paralysis of
the extensor muscles . The progression can be rapid lead-
ing to serious deterioration of unsupported sitting ability
and deterioration of the respiratory function already com-
promised by the disease [2,3]. Spinal stabilization is
required to prevent these disabilities. Spinal instrumentation
in DMD has seen a sea of change over the last five decades
The trend in the 1980s was to extend the fixation to the
pelvis or sacrum. However, the prolonged operating time
and increased blood loss persuaded many to stop the distal
instrumentation at L5 level [10,15-17]. Most current litera-
ture deals with the instrumentation of the spine using hooks
and wires or hybrid constructs with pedicle screws in lum-
Received Sep 9, 2010; 1st Revised Nov 23, 2010; 2nd Revised Dec 3, 2010; Accepted Dec 6, 2010
Corresponding author: Ujjwal Kanti Debnath, FRCS (T&O), DM (Orth)
The Centre for Spinal Studies and Surgery, Queens Medical Centre, Nottingham NG7 2UH, UK
23, Barons Court Road, Cardiff CF23 9DF, UK
Tel: +44-7811165794, Fax: +44-2920456355, E-mail: email@example.com
Vol. 5, No. 1, pp 43~50, 2011
Copyright � 2011 by Korean Society of Spine Surgery
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0)
which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Asian Spine Journal�pISSN 1976-1902 eISSN 1976-7846
Spinal Deformity Correction in Duchenne Muscular
Dystrophy (DMD): Comparing the Outcome of
Two Instrumentation Techniques
Ujjwal Kanti Debnath, Syed M. Hossein Mehdian, John K. Webb
The Centre for Spinal Studies and Surgery, Queens Medical Centre, Nottingham NG7 2UH, UK
S St tu ud dy y D De es si ig gn n:: A retrospective matched cohort study.
P Pu ur rp po os se e:: To compare the results of combined Luque rod-sublaminar wiring (thoracic) and pedicle (lower lumbar) instrumen-
tation (SLW) versus those with pedicle screw fixation (PS) for scoliosis correction in Duchenne muscular dystrophy (DMD).
O Ov ve er rv vi ie ew w o of f L Li it te er ra at tu ur re e:: PS fixation is gaining popularity. Two instrumentation systems were not compared before in a
matched cohort of patients.
M Me et th ho od ds s:: Two groups of patients with DMD were matched according to the age at surgery, magnitude of deformity and
vital capacity. Indications for surgery included loss of sitting balance, rapid decline of vital capacity and curve progression.
In group 1 (22 patients) SLW fixation was used from T2/3 to pelvis or sacrum. In group 2 (18 patients) PS fixation was used
from T2/3 to L5. Five patients had all level segmental PS fixations. Minimum follow-up was 2 years (range, 2 to 13 years).
Radiographs, lung function tests and subjective/objective assessment were performed at standardized intervals.
R Re es su ul lt ts s:: Mean Cobb angle in group 1 improved from 45.3�(range, 26 to 75� ) to 17.7�(range, 0 to 37� ) and mean pelvic
obliquity improved from 14.5�(range, 8 to 28� ) to 5.6�(range, 0 to 15� ). Mean Cobb angle in group 2 improved from 42.8�
(range, 28 to 80� ) to 7.3�(range, 0 to 20� ) and mean pelvic obliquity improved from 11.2�(range, 7 to 30� ) to 2.0�(range, 0
to 5� ) (p < 0.05). Mean operating time and blood loss were less in group 2 (p < 0.05). In group 1, the infection rate and instru-
mentation failure was higher, and subjective/objective outcomes showed no significant difference between the groups.
C Co on nc cl lu us si io on ns s:: PS fixation had superior correction and controlled pelvic obliquity without the need for pelvic fixation.
Key Key W Wordsords: Muscular dystrophy, Duchenne, Spinal deformity, Scoliosis, Pedicle Instrumentation, Sub laminar wiring
bar region. Although, the use of pedicle screw constructs in
thoracic spine for idiopathic scoliosis was established in late
90s [18-20], it was not until recently, Hahn et al. 
reported for the first time the outcome of patients following
use of only pedicle instrumentation in DMD.
The present study compares the clinical and radiographic
results of combined Luque rod-sublaminar wiring (thoracic)
and pedicle screws (lower lumbar) (SLW) versus only pedi-
cle screw fixation (PS) in two consecutive patient cohorts
with scoliosis in DMD.
Materials and Methods
The data for two-matched consecutive cohorts were col-
lected retrospectively at a single institution (University
Hospital, Nottingham). These patients were treated within a
span of 17 years from 1990-2007. Group 1 consisted of the
historic cohort of 22 patients treated between 1990 and
2000 with Luque rod-sublaminar wiring with pelvic fixation
with either Galveston or L-rod technique (SLW group).
Group 2 consisted of the cohort of 18 patients who were
treated between 2000 and 2007 with pedicle screw con-
structs (PS group). The senior authors (SHM and JKW)
were involved in treating patients from both groups.
The groups were matched according to three criteria: sim-
ilar age at surgery, similar magnitude of spinal curvatures
(all type 1 and 2) , and vital capacity. Indications for
surgery were loss of sitting balance, presence of scoliosis
with or without evidence of curve progression and dimin-
ishing vital capacity. Both groups had similar operative
exposures and upper thoracic fixation levels.
In group 1, the correction was achieved using Luque rods
fixed with sublaminar wires in the thoracic and lumbar
spine (Fig. 1). The Galveston technique was used in 6 cases
and the L-rod configuration in 16 cases for pelvic fixation.
Pedicle screws are first placed in the lumbar spine. Poste-
rior superior iliac spine is identified and the outer wall of
ilium is dissected in a subperiosteal plane to reach the
greater sciatic notch. This position acts as a reference for
insertion of the rod on either side. The rods are contoured
and prebent to realign the deformed spine. The basic princi-
44 / ASJ: Vol. 5, No. 1, 2011
Fig. 1. A 11-year-old boy, wheelchair bound for 8 months with Duchenne muscular dystrophy. (A) Preoperative
antero-posterior radiograph right sided showing 60。curve. (B, C) Two year post-operative antero-posterior and lat-
eral radiographs showing sublaminar wiring instrumentation with Luque rods and distal fixation to pelvis with L-rod
ple determining the sequence depended on where the initial
correcting load needed to be applied. Once the rods are
fixed to the lumbar pedicle screws (as the foundation) a
cantilever corrective load (that is, one that has support at
one end) to the rod or rods was applied, their unattached
ends are brought to the anchorage at the opposite end. A
strong foundation is defined as strong enough to accept cor-
rective loads and to resist deforming loads without dislodg-
ment of the anchors or plastic deformation of the rods. The
thoracic foundations consisted of instrumentation spanning
at least three consecutive vertebrae. The instrumentation
sequence was initiated on the convex side of the thora-
columbar curve and then added on the concave side, with
the cephalad rod connected side to side to the intrailiac post
(Galveston) anchorage or L rod configuration. Two short
closed loop sublaminar wires were used to fix the rods to
In group 2 fixation of the spine was carried out with pedi-
cle screws in the thoracic as well as in the lumbar spine
(Fig. 2) and to L5 vertebrae in all the patients. Pedicle
screws with a diameter of 5mm and 6mm were used in the
thoracic and the lumbar spine respectively. Anatomical
landmarks were used to insert the screws. In 5/18 patients
all level segmental pedicle screw construct was used and
the rest had alternate segmental pedicle screw constructs in
which every other vertebra in the main lumbar region and
every second or third vertebra in the upper thoracic region
were instrumented. The average number of pedicle screws
used was 24 (range, 18 to 34) per patient. Following instru-
mentation all had autologous bone graft and supplemental
femoral head allograft for fusion following standard facet
excision and decortication. All patients had intra-operative
neurophysiologic monitoring (SSEPs and SMEPs). Post-
operatively, all the patients were managed in a pediatric
intensive care unit until they no longer required any ventila-
The following clinical data were recorded: forced vital
capacity (FVC) and forced expiratory volume in the 1st sec-
ond estimated immediately preoperatively and at final fol-
low-up, electrocardiogram findings, echocardiogram
results, weight, age at surgery, estimated blood loss, opera-
tion (“skin to skin”) time, complications and the duration of
hospitalization following surgery.
Antero-posterior and lateral radiographs of the whole
spine in sitting were taken in all patients preoperatively and
during the follow-up. The Cobb angle of the scoliosis and
the pelvic obliquity were measured from the radiographs
preoperatively, 6 months postoperatively and at the final
follow-up. An independent assessor assed the radiographs
and measurements were made independently of the sur-
geons involved. Functional assessment was performed
using the EK scale . The EK scale comprises of ten cat-
egories (EK1-10) which was developed to determine the
need for and impact of intervention in the non-ambulatory
stages of DMD. The questionnaire incorporates 10 items
which deal with the patients ability to use wheelchair, abili-
ty to transfer from wheelchair, ability to stand, ability to
balance in the wheelchair, ability to move the arms, ability
Spinal Deformity Correction in Duchenne Muscular Dystrophy / 45
Fig. 2. A 13 year-old-boy wheelchair bound for 2 years with Duchenne muscular dystrophy. (A, B) Preoperative
antero-posterior and lateral radiographs showing left sided scoliosis with 40。 curvature. (C, D) Two year post-opera-
tive antero-posterior and lateral radiographs showing instrumentation with pedicle screws.
to use the hands and arms for eating, ability to turn in bed,
ability to cough, ability to speak and physical well being. A
range of scores can be possible from 0-30. Lower the score
better the function.
2. Statistical analysis
Pre and post-operative and final follow-up radiographs
were used to analyse the Cobb angle and pelvic tilt. Post-
operative corrections were analysed using paired t-test.
Similarly correction percentage at initial post-operative
radiographs and final follow-up radiographs was compared
using paired t-test (p < 0.05 was considered to identify sig-
nificant differences). We determined the duration of opera-
tion, mean blood loss and mean post-operative hospital stay
to see if these factors were associated with better outcome
with chi square test.
1. Group 1
DMD was diagnosed at a mean age of 4.5 years (range,
1.5 to 9 years). The patients became wheelchair bound at a
mean age of 9.6 years (range, 6 to 12.5 years) and operated
at a mean age of 12.5 years (range, 9 to 16 years). At the
time of surgery, the FVC was 57% (range, 34 to 72%). The
scoliosis was thoracic in 2 cases, thoracolumbar in 14 cases
and lumbar in 6 cases. The mean body mass index (BMI)
was 22.5 kg/m2. The mean follow-up in this group was 4.5
years (range, 3 to 9 years). The mean Cobb angle was 45.3�
(range, 26 to 75� ) and the mean pelvic obliquity was 14.5�
(range, 8 to 28� ) at the time of surgery. Immediately after
surgery, the mean Cobb angle measured 16�(range, 0 to
35� ) and at final follow-up was 17.7� (range, 0 to 37� ) (Fig.
3A). The immediate postoperative pelvic obliquity mea-
sured 5.7� (range, 0 to 15� ) and at final follow-up was 5.6�
(range, 0 to 15� ). The mean operative time was 260 minutes
(range, 180 to 380 minutes), mean estimated blood loss was
3.4 l (range, 2 to 10.0 l), mean intensive care stay was 56
hours (range, 48 to 80 hours) and mean hospital stay was
8.5 days (range, 6 to 16 days). Two patients had excessive
intra-operative bleeding treated with blood transfusion. Five
patients had infection (four superficial and one deep). Three
patients required prolonged ventilatory support but didn’ t
require any tracheotomy. Two patients had implant failure
46 / ASJ: Vol. 5, No. 1, 2011
Fig. 3. (A) The distribution of Cobb angle at the time of surgery, immediately after surgery, and at final follow-up in
individual cases in group 1 (SLW). (B) The distribution of Cobb angle at the time of surgery, immediately after
surgery, and at final follow-up in individual cases in group 2 (PS). SLW: Luque rod-sublaminar wiring (thoracic) and
pedicle (lower lumbar) instrumentation, PS: Pedicle instrumentation, Preop: Pre-operative, Postop: Post-operative,
(one had loosening of the implant and the other had proxi-
mal junctional kyphosis). Pelvic obliquity was maintained
at or below 10� in all but two patients.
2. Group 2
The mean age of diagnosis in this group was 5 years
(range, 4 to 12 years). The patients became wheelchair
bound at a mean age of 9.8 years (range, 6 to 13 years) and
operated at a mean age of 11.8 years (range, 9 to 15 years).
At the time of surgery, the FVC was 53% (range, 34 to
78%). Fifteen patients had C shaped curve with equal distri-
bution of sidedness. The curves were mainly thoracic in 3,
thoracolumbar in 13 and lumbar in 2 patients. The mean
follow up in this group was 2.3 years (range, 2 to 6 years).
The mean BMI was 25.4 kg/m2. The mean Cobb angle was
42.8�(range, 28 to 80� ) and the mean pelvic obliquity was
11.6�(range, 0 to 20� ) at the time of surgery. Immediately
after surgery, the mean Cobb angle measured 4.5� (range, 0
to 15� ) and at the final follow-up was 7.3� (range, 0 to 20� )
(Fig. 3B). The immediate postoperative pelvic obliquity
measured 2.6�(range, 0 to 6� ) and at final follow-up was
2�(range, 0 to 5� ). The mean operative time was 216 min-
utes (range, 180 to 300 minutes), mean estimated blood loss
was 2.0 l (range, 1 to 3.7 l), mean intensive care stay was 44
hours (range, 36 to 72 hours) and mean hospital stay was 7
days (range, 6 to 14 days). Two patients had infection treat-
ed with drainage, irrigation and antibiotics. One patient had
broken rod and loose screw, which was revised.
Comparing the two groups it was observed that although
both groups had surgery for similar curve magnitudes, the
mean curve magnitude at final follow up was significantly
higher in group 1 (p < 0.01) (Table 1). The percentage of
curve correction and pelvic obliquity correction was signifi-
cantly higher in the group 2 (p < 0.01). Intra-operative
instrumentation was more time consuming in group 1 (p <
0.01). Intra-operative blood loss was significantly higher in
group 1 (p < 0.01). The functional outcome as measured by
EK scale was similar in both groups. The mean EK score
was 8.95 (range, 2 to 23) in group 1 as compared to 8.72
(range, 3 to 19) in group 2 (p = 0.8).
The scoliosis correction in DMD is challenging because
of the primary muscular pathology and the frequently asso-
ciated reduced pulmonary reserve. Several instrumentation
techniques has evolved over the last five decades since Har-
rington  first reported the use of rods and hooks in spinal
fusion for DMD. This was discarded in early 70s due to
multiple complications encountered in various series
[6,9,22]. Luque  introduced sublaminar wiring in early
80s for spinal stabilization in deformities which was under-
taken in many centres with variable success. The authors
Spinal Deformity Correction in Duchenne Muscular Dystrophy / 47
Table 1. A comparison of the key mean data from the two groups
Data Group 1 (SLW)Group 2 (PS)
No. of patients
Mean age at diagnosis (yr)
Mean age at surgery (yr)
Mean preoperative FVC (% pred. value)
Mean preoperative Cobb angle (range, � )
Mean preoperative Pelvic tilt angle (range, � )
Flexibility Index (range)
Mean body mass index (kg/m2)
Mean final Cobb angle (range, � )
Mean final pelvic tilt angle (range, � )
Mean % Cobb angle correction (range)
Mean % pelvic tilt correction (range)
Mean operative time in min (range)
Mean blood loss in l (range)
Mean intensive care stay (hr)
Mean hospital stay in days (range)
SLW: Luque rod-sublaminar wiring (thoracic) and pedicle (lower lumbar) instrumentation, PS: Pedicle screw fixation, FVC: Forced
2test, b)Paired t-test.
reported a mean correction of deformity on the frontal plane
equal to 50%, and only the severest cases required postoper-
ative immobilization. The advantages of surgery that they
described include increased comfort and sitting tolerance
[1,4,5,8,10-12]. More recently, Cervellati et al.  recom-
mend the use of Luque rods and sublaminar wiring since it
ensures a satisfactory, stable and durable correction of the
deformity with good restoration of trunk balance.
In 1984, Allen and Ferguson  proposed a modified
system of the segmental spinal instrumentation, known as
the Galveston technique. This modification provided better
fixation and correction of pelvic obliquity. However, they
pointed out that pelvic fixation is a technically demanding
procedure that increases operative time and risk. The fixa-
tion to pelvis was the norm for better sitting stability. Suss-
man  suggested that instrumentation and fusion to L5
might be adequate in DMD. Fusion to the fifth lumbar ver-
tebra has been recommended  in cases in which pelvic
obliquity (measured as the angle between a line tangential
to the superior margins of both iliac crests and a line per-
pendicular to one through the spinous processes of L4 and
L5), is less than 10� , and the Cobb angle is less than 40� .
Sengupta et al.  recommended L5 fixation for young
patients with smaller curves and minimal pelvic obliquity.
Bony fusion to the pelvis is difficult to achieve because of
the severe osteopenia in patients with DMD. Increased
blood loss is another intra-operative complication which is
dreaded by surgeons and anesthetist. In the current series
i.e., SLW group had significantly increased operating time
as well as blood loss which confirm the observation of oth-
ers . This was due to bleeding from epidural vessels
during the subperiosteal dissection for passage of the sub-
Since the introduction of segmental pedicle fixation in
idiopathic scoliosis in mid 90s, many surgeons are using
pedicle fixation to good use in DMD patients [19,20,26].
Hahn et al.  reported the first series of twenty patients
treated with pedicle instrumentation from T3/T4 to the
pelvis. Pelvic fixation was done with iliac screws similar to
Galveston technique . The mean total blood loss was
3.7 l, intensive care unit stay was 77 hours and hospital stay
was 19 days. The mean Cobb angle correction was 77%
from 44�to 10� , pelvic obliquity improved 65% from 14�
to 3� . In our series, lower mean blood loss of 2 l was
recorded, intensive care unit stay was maximum of 48 hours
and mean hospital stay was 7 days in the PS group. The
radiographic results in this series of pedicle instrumentation
were comparable to the series presented by Hahn et al. .
This series had a mean Cobb correction of 83% and pelvic
obliquity correction of 83%. The pelvic obliquity correction
achieved was much higher as compared to others. It is an
interesting difference since the pedicle instrumentation
group didn’ t have any pelvic fixation as described for every
patient in the series presented by Hahn et al. . It is pre-
sumed that the reason for such a difference may be due to
the fact that their series had patients who had higher pelvic
obliquity (at least five patients had more than 20�pelvic
Alman and Kim  questioned the use of pelvic fixation
of rods since the pelvic obliquity progressed in 78% of their
patients despite pelvic fixation. But they also recommended
that if the apex of the curve is caudal to L1, one may use
pelvic or sacral fixation. They further suggested the use of
hooks or screws in the lumbar region to avoid the failures
seen in sublaminar wiring. Use of pedicle screw system for
DMD was started in this institute since the turn of the mil-
lennium with an idea to avoid the complications observed in
the sublaminar wiring. It was observed in our practice that
pedicle screw system had better intra-operative control of
the correction of the curve in sagittal and coronal plane as
compared to the sublaminar wiring. Gayet et al.  intro-
duced the flexible spine instrumentation with rods fixed to
the lumbar and sacral spine using screws. They achieved
good correction with improved spinal growth by 5 cm.
Thus, the quest for better instrumentation techniques contin-
ued until the pedicle screws were found to be biomechani-
cally superior since they are penetrating anchors as com-
pared to the gripping fixation obtained by wires and cables
Kim et al.  compared two techniques i.e., pedicle
instrumentation and hybrid system of sublaminar wiring
and lumbar pedicle screws in idiopathic scoliosis. They
found similar results in junctional changes, lowest instru-
mented vertebra, surgical time and clinical outcome.
Although, similar curve magnitudes were observed in both
surgical groups in this series, the curve correction (both in
coronal and sagittal plane) achieved was much higher in the
pedicle instrumentation group . These results obtained
in DMD patients points out that pedicle instrumentation has
a better deformity correction force and probably maintains
the correction even without pelvic or sacral fixation. Good
functional outcome was evident in five patients who had
high BMI and had pedicle instrumentation at all segments
from T2 to L5. Increased body weight owing to steroid
48 / ASJ: Vol. 5, No. 1, 2011
intake in these patients may be benefited by pedicle instru-
mentation at all levels since they may be at risk of implant
failure. This precludes the need for revision surgery in these
patients who has little longevity.
In this study, both the groups were matched for mean age
at surgery, mean spinal curvature and diminishing vital
capacity. The matching study cohorts make it easier to
interpret the results. In one series, three methods of instru-
mentation techniques were compared but none of the groups
were matched. The number of subjects in each group was
less and authors had to interpret the results of curve correc-
tion with caution .
The risks involved in pedicle screw instrumentation are
minimized by better understanding of the orientation of the
pedicles during surgery and intra-operative image guidance.
In both the groups early surgery results in less post-opera-
tive respiratory complications and therefore, reduced hospi-
tal stays. Early surgery in DMD also avoids pelvic instru-
mentation since pelvic obliquity was usually less than 10。 .
The percentage curve correction was significantly higher
(83% vs. 62%) in the PS group as compared to SLW group.
PS system appeared to retain the correction achieved at
Although the radiographic parameters show quite signifi-
cant change in the PS group as compared to SLW group, the
functional outcome remains similar in both groups. This is
owing to the fact that both the techniques provide adequate
support to the spine for a better sitting balance and may pre-
vent further deterioration of pulmonary function. We rec-
ommend that pedicle instrumentation has better inherent
capability of maintaining the corrected curve due to its bio-
The strengths of our study include single centre study,
matching cohort of patients, availability of pre and post
operative data, involvement of an independent assessor and
critical analysis of the data. There were few limitations.
First limitation, it’s comparing a historical surgical outcome
study from two different decades. Second limitation, the
SLW group had pelvic fixation while the PS group had no
pelvic fixation. Third limitation, since curve flexibility
index was a prerequisite to compare the percentage correc-
tion, immediate post-operative correction was subtracted
from the flexibility index in this series for interpreting the
Spinal instrumentation with pedicle screws for scoliosis
correction in patients with DMD results in a good correc-
tion of the spinal and pelvic deformity. Pedicle instrumenta-
tion gives a solid foundation, on which the rest of the spine
may be held upright, maintain the balance and rotation. Due
to the improvement of lumbar curve and pelvic obliquity by
pedicle instrumentation, a good sitting balance is restored
even without the pelvic or sacral fixation in patients with
pelvic obliquity less than 10。 .
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