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Intervention Review

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Surgery for scoliosis in Duchenne muscular dystrophy

  1. Daniel KL Cheuk1,*,
  2. Virginia Wong1,
  3. Elizabeth Wraige2,
  4. Peter Baxter3,
  5. Ashley Cole4

Editorial Group: Cochrane Neuromuscular Disease Group

Published Online: 28 FEB 2013

Assessed as up-to-date: 31 JUL 2012

DOI: 10.1002/14651858.CD005375.pub3


How to Cite

Cheuk DKL, Wong V, Wraige E, Baxter P, Cole A. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database of Systematic Reviews 2013, Issue 2. Art. No.: CD005375. DOI: 10.1002/14651858.CD005375.pub3.

Author Information

  1. 1

    The University of Hong Kong, Queen Mary Hospital, Department of Pediatrics and Adolescent Medicine, Hong Kong, China

  2. 2

    Evelina Children's Hospital, St Thomas' Hospital, Department of Paediatric Neurology, London, UK

  3. 3

    Sheffield Children's Hospital, Ryegate Children's Centre, Sheffield, South Yorkshire, UK

  4. 4

    Sheffield Children's Hospital, Orthopaedics Department, Sheffield, UK

*Daniel KL Cheuk, Department of Pediatrics and Adolescent Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam Road, Hong Kong, China. cheukkld@hkucc.hku.hk.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 28 FEB 2013

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This is not the most recent version of the article. View current version (01 OCT 2015)

 
Characteristics of excluded studies [ordered by study ID]

StudyReason for exclusion

Alman 1999Retrospective case series, not clinical trial.

Arun 2010Retrospective case series, not clinical trial.

Bellen 1993Retrospective case series, not clinical trial.

Bentley 2001Retrospective case series, not clinical trial.

Bridwell 1999Retrospective case series, not clinical trial.

Brook 1996Retrospective case series, not clinical trial.

Cambridge 1987Retrospective case series, not clinical trial.

Cervellati 2004Retrospective case series, not clinical trial.

Chataigner 1998Retrospective case series, not clinical trial.

Dubousset 1983Retrospective case series, not clinical trial.

Eagle 2007Retrospective case series, not clinical trial.

Gaine 2004Retrospective case series, not clinical trial.

Galasko 1992Retrospective case series, not clinical trial.

Galasko 1995Retrospective case series, not clinical trial.

Gayet 1999Retrospective case series, not clinical trial.

Granata 1996Retrospective case series, not clinical trial.

Hahn 2008Retrospective case series, not clinical trial

Harper 2004Prospective case series, not clinical trial.

Heller 2001Prospective case series, not clinical trial.

Hopf 1994Retrospective case series, not clinical trial.

Kennedy 1995Retrospective case series, not clinical trial.

Kinali 2006Retrospective case series, not clinical trial.

LaPrade 1992Retrospective case series, not clinical trial.

Marchesi 1997Retrospective case series, not clinical trial.

Marsh 2003Retrospective case series, not clinical trial.

Matsumura 1997Retrospective case series, not clinical trial.

Mehdian 1989Retrospective case series, not clinical trial.

Miller 1988Retrospective case series, not clinical trial.

Miller 1991Retrospective case series, not clinical trial.

Miller 1992Retrospective case series, not clinical trial.

Modi 2008aRetrospective case series, not clinical trial.

Modi 2008bRetrospective case series, not clinical trial.

Modi 2009Retrospective case series, not clinical trial.

Modi 2010Retrospective case series, not clinical trial.

Mubarak 1993Retrospective case series, not clinical trial.

Nakazawa 2010Prospective case series, not clinical trial.

Rice 1998Retrospective case series, not clinical trial.

Rideau 1984Retrospective case series, not clinical trial.

Sakai 1977Retrospective case series, not clinical trial.

Sengupta 2002Retrospective case series, not clinical trial.

Shapiro 1992Retrospective case series, not clinical trial.

Stricker 1996Retrospective case series, not clinical trial.

Sussman 1984Retrospective case series, not clinical trial.

Takaso 2010Prospective case series, not clinical trial.

Thacker 2002Retrospective case series, not clinical trial.

Velasco 2007Retrospective case series, not clinical trial.

Weimann 1983Retrospective case series, not clinical trial.

 
Table 1. Characterisitcs of excluded studies

Study referenceNumber of patientsTreatmentsOutcome measuresFindingsRemarks

Arun 201043Sublaminar instrumentation (19) or hybrid sublaminar and pedicle screw (13) or pedical screw (11)Cobb angle, flexibility index, blood loss, operating time, complicationsPercentage correction of Cobb angle was 72.5 +/- 14.5% (Group A), 82 +/- 6% (Group B) and 82 +/- 8% (Group C). Flexibility indices were 60 +/- 6.33% (Group A), 70 +/- 4.65% (Group B) and 67 +/- 6.79% (Group C). Mean blood loss was 4.1 L (Group A), 3.2 L (Group B) and 2.5 L (Group C). Mean operating times were 300 min (Group A), 274 min (Group B) and 234 min (Group C). Complications: 3 wound infections and 2 implant failure (Group A), 1 implant failure (Group B), 1 wound infection and 1 partial screw pull out (Group C).Concluded that pedicle screw system might be favored because of the lesser blood loss and surgical time.

Alman 199948Spinal fusion to L5 (38) or spinal fusion to sacrum (10) using multiple level sublaminar wires with either a modified unit rod with Galveston extensions to the pelvis cut-off, a modified rod with a cross-link placed at the caudal end, or 2 Luque rods.Cobb angle, torso decompensation, sitting obliquity, spinal obliquity, need for revision surgery, mortality.Sitting obliquity and spinal obliquity increased in patients fused to L5. 2 patients had fracture of L5 lamina. 2 patients required revision surgery.

Bellen 199347Segmental spinal instrumentation according to Luque's technique.Mortality, complications.Many patients have general and pulmonary and mechanical complications.Concluded that a total spinal arthrodesis could probably be avoided in these patients, which often demonstrate a satisfying spontaneous fusion after instrumentation.

Bentley 2001101 (included 33 patients with SMA and 4 patients with congenital muscular dystrophy)Modified Luque (87), Harrington-Luque (14)Cobb angle, pelvic obliquity, mortality, complications, patient satisfactionCobb angle decreased from 70 to 37º, pelvic obliquity decreased from 20 to 13º. Early severe complications in 10 patients, late complications in 24 patients. No peri-operative mortality. Excellent satisfaction in 89.6% of patients.Incidence of minor or temporary complications was high, but chiefly occurred in patients with very severe curves and considerable pre-existing immobility.

Bridwell 199933 (included 21 patients with SMA)Posterior segmental spinal instrumentation applied from the upper thoracic spine (T2, T3, T4, T5) down to L5 or the sacrum and pelvis. Early in the series, patients with DMD with smaller curves (< 40º) were fixed to L5. All had bilateral segmental fixation with Wisconsin or sublaminar wires at each level and at times with hook supplementation. All patients fused to the sacrum had Galveston or Galveston-like fixation.Questionnaires to evaluate function, self-image, cosmesis, pain, pulmonary status, patient care, quality of life, satisfaction,
radiographic data.
All patients seemed to have benefited from the surgery. Cosmesis, quality of life, and overall satisfaction rated the highest.

Brook 199617L-rod instrumentation (10), distal instrumentation with Galveston construct and rigid cross-linking (7)Cobb angle and pelvic obliquity, %FVC, mortality, complicationsCorrection of Cobb angle better in the Galveston group (63% versus 51%). No pseudoarthroses or instrument failures in the Galveston group. Totally 4 patients had FVC < 25%, 2 required ventilation postoperatively. No other respiratory complications. No peri-operative mortality.The effect of surgery on respiratory function remains uncertain.

Cambridge 198714Segmental spinal instrumentation (13), Harrington distraction rods (1).Mortality, complications, sitting tolerance.No peri-operative mortality, 1 required repeated re-intubation. All achieved excellent long-term sitting tolerance.Recommended posterior spinal fusion with segmental instrumentation when scoliosis > 30º. Spinal fusion did not increase life expectancy or pulmonary function.

Cervellati 200420Modified Luque technique (19) or Cotrel-Dubousset instrumentation (1).Cobb angle, vital capacity, mortality.Mean correction at follow-up was 28º. Mean loss of correction was 6º. Vital capacity showed a slow progression, slightly inferior to its natural evolution in untreated patients. Death in 1 patient.

Chataigner 199827Sublaminar wiring with Luque rods (5) or Hartshill rectangle (22)
Sacral fixation with ilio-sacral screws linked to the rectangle by Cotrel-Dubousset rods and dominos (15).
Cobb angle, pelvic obliquity, coronal imbalance, sagittal imbalance, vital capacity, mortality, complications.Scoliosis reduced to 10º after surgery and 13º after 30 months' follow-up. Pelvic obliquity was reduced to 4º after surgery and 7º after 30 months. A good spinal balance was present in 20 patients after surgery. A coronal or sagittal imbalance averaging 40 mm was observed in 22 patients at follow-up. Vital capacity had annual decrease of 6.4%. 17 patients were alive with a 50 months follow-up. No operative mortality. 1 patient required tracheostomy post-operatively.Concluded that surgery did not result in respiratory improvement nor in life duration lengthening.

Dubousset 198337Luque rods, Harrington rods, segmental instrumentation.Cobb angle, vital capacity,
mortality.
Scoliosis reduced from 80 to 24º. No effect on decline of vital capacity. No clear benefit in length of survival.

Eagle 200775Surgery and nocturnal ventilation (27), nocturnal ventilation only (13), no surgery or ventilation (35)Survival, complications, FVCNo peri-operative deaths. Complications: GIB (2), postoperative ileus (1), spinal infection requiring removal of surgical rods (1), pressure sores (1), chronic pain due to prominence of metal prosthesis (2). Mean FVC reduced significantly (mean 1.4 L to 1.13 L) after 1 year. Median survival longer in surgery with ventilation group compared to ventilation alone (30 versus 22.2 years). Survival at 24 years higher in surgery with ventilation group compared to ventilation or no intervention (84% versus 34.6% versus 10.7%)Spinal surgery does not improve FVC. Combined surgery and nocturnal ventilation improves survival

Gaine 200474Luque rod (55), Isola pedicle screw (19).Cobb angle, pelvic obliquity, mortality, complicationsFusion to S1 did not offer benefit over fusion to more proximal level.
Isola system appears to maintain a slightly better Cobb angle. 1 perioperative mortality due to cardiorespiratory failure. Complications: Failure of implants (3), wound infection (2), pseudarthrosis (2), metal implant prominence requiring removal (1)

Galasko 199255Surgery (32),
refused surgery (23).
Mortality, complications, FVC, PEFR, Cobb angle.In surgery group, FVC static for 3 years then slightly decreased. Improved PEFR maintained for up to 5 years. Cobb angle improved from 47 to 34º at 5 years. Slightly improved survival with surgery. Complications: respiratory failure requiring tracheostomy (1), pneumonia (1), heart block (1), superficial wound infection (1)

Galasko 199576Surgery (48), refused surgery (28)Mortality, complications, FVC, PEFR, Cobb angle.No pseudarthrosis or post-operative failures. Annual decrease of FVC lower in surgery group (0.07 versus. 0.15). PEFR increased annually by 7.6 L/min in surgery group but decreased annually by 7.6 L/min in non-surgery group. Cobb angle after 3 years better in surgery group (34 versus 93 degrees). At 5 years, survival higher in surgery group (61% versus 23%). Complications: respiratory failure requiring tracheostomy (1)Patients with surgery have better lung function and improved survival.

Gayet 199937Pedicular screwing system in the lumbo-sacral area and transversal attachments with steel threads at the thoracic level. A sub-laminar fastening was placed at L1.Vital capacity, mortality, complications, Cobb angle, pelvic obliquity.Cobb angle decreased from 19 to 5.2º , and 9.5% at the latest measurement. Pelvic balancing was corrected and results have held over time. Vital capacity was reduced by 3.6% per year. Complications: stem rupture (1), superficial infection (4)Cardiorespiratory function and life expectancy were not improved, but most patients and families were very satisfied by the comfort brought about by the surgical operation.

Granata 199630Segmental spinal instrumentation and fusion.Cobb angle, mortality, complications, vital capacity, quality of life, sitting position, aesthetic improvement.29 had a mean 59% correction of scoliosis.
Very limited loss of correction over time.
One died after cardiac arrest. Complications: pressure sore (1), metal prominence requiring trimming (1). Mean vital capacity decreased from 57 +/- 17% to 34 +/- 13% at 3.9 +/- 2 years after surgery. The sitting position, aesthetic improvement and quality of life were positively evaluated by majority of the patients and their parents.

Hahn 200820Spinal fixation with pedicle-screw-alone constructs%FVC Cobb angle, degree of pelvic tilt, lumbar lordosis and thoracic kyphosis, mortality, complicationsCobb angle improved from 44 to 10º, pelvic tilt improved from 14 to 3º . Lumbar lordosis improved from 20 to 49º, thoracic kyphosis remained unchanged. No problems related to iliac fixation, no pseudarthrosis or implant failures. No pulmonary complications %FVC decreased from 55% preoperatively to 44% at the last follow-up. One patient died intraoperatively due to a sudden cardiac arrest.The rigid primary stability with pedicle screws allowed early mobilisation of the patients, which helped to avoid pulmonary complications.

Harper 200445AO Universal Spinal System inserted through a posterior approach.Mortality, complications, hospital stay.No significant difference in operative and postoperative outcomes between patients with pre-operative forced vital capacity > 30% and ≤ 30%. Complications in 9 patients: pneumonia, respiratory failure requiring tracheostomy, ARDS, pleural effusion, cardiac arrhythmiaConcluded that routine postoperative use of mask ventilation to facilitate early tracheal extubation was vital.

Heller 200131Isola system.Cobb angle, pelvic obliquity, mortality, complications.Cobb angle decreased from 48.6 to 12.5º, pelvic obliquity decreased from 18.2 to 3.8º. 1 post-operative death due to cardia failure. Complications: pneumonia (1), respiratory arrest (1), pneumothorax (1), respiratory failure requiring tracheostomy (1), dislocation of hook (2), infection requiring revision surgery (5), iliac vein thrombosis (1), massive bleeding (1).

Hopf 199420Multi-segmental instrumentation.Mortality, complications, Cobb angle.Mean Cobb angle decreased from 70.6 to 31.2º (mean correction 39.4º or 55.8%). Lordosis of the lumbar spine corrected from 4.1 to 17.8º. No perioperative mortality. Complication: bladder dysfunction in 1 patient.Recommended using multi-segmental instrumentation methods to enable rapid mobilization and a postoperative care without brace or cast.

Kennedy 199538Surgery (17), no surgery (21).Cobb angle, forced vital capacity (FVC), mortality.Mean Cobb angle of the surgical group at 14.9 years was 57 +/- 16.4º, and of the non-surgical group at 15 years was 45 +/- 9.9º. No difference in the rate of deterioration of % FVC which was 3 to 5% per year. No difference in survival in either group.Spinal stabilization in DMD did not alter the decline in pulmonary function, nor did it improve survival.

Kinali 2006123Surgery (43), no surgery (80)Survival, (FVC, sitting comfortNo difference in survival, respiratory impairment, or sitting comfort among patients managed conservatively or with surgery.

Laprade 19929Sublaminar wiring (4), intraspinous segmental wiring (5).Mortality, complications, operative time, blood loss, Cobb angle.Operative time and blood loss lower in sublaminar compared to intraspinous wiring.
Allogeneic bone grafts to supplement the autogenous bone graft allowed for extensive fusion.
Cobb angle decreased by a mean of 32º.
Complications: dural leak (1), transient numbness of left foot (1), dislodgement of sacral alar hooks (2).
Recommended segmental fusion and allogeneic bone grafts.

Marchesi 199725Modified Luque: sacral screws in each S-1 pedicle and a device for transverse traction between the caudal right-angle bends of the L-rods.Cobb angle, pelvic obliquity, mortality, instrumental failure, sitting balance.Cobb angle decreased from 68 to 18º and pelvic obliquity decreased from 21 to <15º with mean correction of 75%. No instrumentation failure or loss of correction >3º. In every patient, a good sitting balance could be restored. No peri-operative mortality.

Marsh 200330Posterior spinal fusion.Cobb angle, mortality, complications, hospital stay.Mean correction of Cobb angle 36º. Two subgroups of patients were compared: those with more than 30% pre-operative FVC (17 patients) and those with less than 30% pre-operative FVC (13 patients). One patient in each group required a temporary tracheotomy and there were nine complications in total. The post-operative stay for patients in each group was similar (24 days in the >30% group, 20 days in the <30% group) and the complication rate was comparable with other published series. No peri-operative mortality.Concluded that spinal fusion could be offered to patients with DMD even in the presence of a low FVC.

Matsumura 19978Luque rod (2), Cotrel-Dubousset rod (6).Cobb angle, FVC, quality of life, mortality, complications, sitting balance.Cobb angle corrected from 58.8 to 28.6º with the mean corrective rate of 51.3%. FVC increased in 3 patients with moderate scoliosis (Cobb angle: 50 to 80º). Two cases with low % FVC (16.9% and 30.4%, respectively) had poor prognosis in respiratory status. One died of pneumonia at 17 months after the surgery and the other required mechanical ventilation. Sitting balance improved in all patients.Recommended spinal fusion for patients with Cobb angle more than 30º and with % FVC more than 35%. Although the impact of spinal fusion upon the life expectancy remained unclear, favorable effect on respiratory function and quality of life could be expected for carefully selected patients with DMD.

Mehdian 198917Luque rods secured by conventional sublaminar wires (9), Luque rods secured by sublaminar nylon straps (4), 2 L-shaped rods connected by H-bars secured by closed wire loops (3), Hartshill rectangle and sublaminar wires (1).Cobb angle, respiratory function.Significant loss of correction in Luque rods secured by sublaminar nylon straps and Hartshill system.
Strong correlation between advance of scoliosis and respiratory function.

Miller 198867Surgery (21), no surgery (46).FVC.No difference was found in the rate of deterioration of the percentage of normal FVC.

Miller 199139Surgery (17), no surgery (22).Respiratory function, sitting comfort, sitting appearance.No significant differences in terms of declining respiratory function. All operated patients reported either improved sitting comfort, appearance, or both.Concluded distinct benefits from segmental spine fusion; however, no salutary effect upon respiratory function either in the short term or after up to 5 years follow-up.

Miller 1992183Surgery (68), no surgery (115).Survival, patient comfort, ease of care, respiratory function, quality of life.Patients with surgery were more comfortable in the later years of life and easier to care for, but deteriorating pulmonary function was not affected by spinal fusion. Age at death for the 29 boys who underwent spinal fusion was 18.3 years, similar to that of the 58 boys without surgery. Factors that improved the patients' quality of life included segmental instrumentation, fusion from T2 to the pelvis, correcting or balancing scoliosis, creating normal sagittal plane alignment and correcting pelvic obliquity.

Modi 2008a26 (including 7 cerebral palsy, 5 SMA, 4 others)posterior pelvic screw fixationCobb angle, pelvic obliquity, complicationsMean Cobb angle: 78.53º (before surgery), 30.7º (after surgery), 33.06º (final follow-up). There was no difference in the percentage correction between the groups with >90º or <90º. Complications: 1 transient loss of lower limb power, 1 deep wound infection.

Modi 2008b24 patients (including 6 cerebral palsy, 5 SMA, 4 others) and 12 controls (adolescent idiopathic scoliosis)Posteriod pedicle screwCobb angle, pelvic obliquity, apical rotationMean Cobb angle decreased from 74 to 32º. Mean pelvic obliquity decreased from 14 to 6º. Mean apical rotation decreased from 42 to 33º. There was no significant difference between different patient groups or between patients and controls.

Modi 200950 (including 18 patients with cerebral palsy, 8 patients with SMA and 6 others)Posterior spinal fusion with segmental spinal instrumentation using pedicle screw fixationMortality, complications, Cobb angle, pelvic obliquityCobb angle decreased from 79.3+/-30.3º to 31.3+/-21.6º. Pelvic obliquity decreased from 14.6+/-9.4º to 6.8+/-6.3º. 2 deaths (1 due to cardiac arrest, 1 due to hypovolemic shock. 34 patients had at least 1 perioperative complication (16 pulmonary, 14 abdominal, 3 wound related, 2 neurological, 1 cardiovascular). Post-operative complications: 7 coccygodynia, 3 screw head prominence, 2 bed sore, 1 implant loosening.DMD patients had higher risk of post-operative coccygodynia.

Modi 201055 (including 28 patients with cerebral palsy and 10 patients with SMA)Spinal fixation from T2/T3/T4 to L4/L5 with or without pelvic fixation. Group 1: pelvic obliquity>15º with pelvic fixation; group 2: pelvic obliquity >15º without pelvic fixation; group 3: pelvic obliquity <15º without pelvic fixationCobb angle, pelvic obliquity, complicationsMean correction of Cobb angle after operation: group 1: 43.8º; group 2: 40º; group 3: 48.7º. Mean loss of correction of Cobb angle at last follow-up: group 1: 0.6º; group 2: 2.3º; group 3: 3º. Mean correction of pelvic obliquity: group 1: 14.4º; group 2: 10.7º; group 3: 5º. Mean loss of correction of pelvic obliquity at last follow-up: group 1: -0.6º; group 2: 6.5º; group 3: 0.8º. Group 2 showed significant loss of pelvic obliquity compared to group 1. Complications: 3 patients had sacral sores in group 1.Patients who have pelvic obliquity >15 degrees require pelvic fixation to maintain correction.

Mubarak 199322Luque segmental instrumentation and fusion
instrumented to the sacropelvis (12), instrumented to L5 (10).
Cobb angle, pelvic obliquity.Outcomes similar between the 2 groups.Concluded that if treatment is initiated early, Luque instrumentation and fusion from high thoracic (T2 or T3) to the fifth lumbar vertebra should be sufficient.

Nakazawa 201036Autogenous bone graft (20), allogeneic bone graft (16)Cobb angle, operating time, blood lossNo difference in Cobb angle between the 2 groups. Mean operating time longer in autogenous group (253 min) compared to allogenous group (233 min). Mean blood loss higher in autogenous group (850 ml) compared to allogenous group (775 ml).90% and 50% of patients in autogenous group reported donor site pain after 1 week and 3 months respectively. Concluded against autogenous bone graft for scoliosis surgery in DMD patients.

Rice 199819Long spinal fusion to L5 and ongoing wheelchair seating attention.Sitting position.At long-term follow-up 15 patients continued to sit in a well-balanced position.Concluded that surgical fusion of the spine to L5 combined with ongoing attention to seating was associated with good long-term functional results in these patients.

Rideau 19845Luque segmental spinal stabilization without bone fusion.Cobb angle, vital capacity, mortality, complications, hospital stay, pelvic obliquity, patient comfort.Cobb angle decreased from 27 to 11º. Pelvic obliquity partially reduced. Static vital capacity after 2 years. No peri-operative mortality, 1 bronchopneumonia. All patients more comfortable during wheelchair activities.Concluded that surgical intervention should be prophylactically undertaken when there is high risk of a rapidly evolving curve with a severe restrictive lung syndrome.

Sakai 197741Surgery (10), no surgery (31).Sitting stability, mortality, complications.Pulmonary complications were minimized by performing preoperative tracheostomy on all patients who had vital capacities less than 40% and or non-functional coughs. No peri-operative mortality. Spinal fusion permitted long-term sitting stability despite the progression of the disease.

Sengupta 200250Galveston technique (9), L-rod (22), pedicle screw + sublaminar wires (19).Cobb angle, pelvic obliquity, mortality, complications, hospital stay.In the pelvic fixation group, the mean Cobb angle and pelvic obliquity were 48º and 19.8º at the time of surgery, 16.7º and 7.2º immediately after surgery, and 22º and 11.6º at the final follow-up (mean 4.6 years). The mean hospital stay was 17 days. 5 major complications: deep wound infection (1), revision of instrumentation prominence at the proximal end (2), loosening of pelvic fixation (2). In the lumbar fixation group, the mean Cobb angle and pelvic obliquity were 19.8º and 9º at the time of surgery, 3.2º and 2.2º immediately after surgery, and 5.2º and 2.9º at the final follow-up (mean 3.5 years). The mean hospital stay (7.7 days) was much less compared with the pelvic fixation group. Pelvic obliquity was corrected and maintained below 10º in all but two cases, who had an initial pelvic obliquity exceeding 20º. 2 complications: instrumentation failure at the proximal end (1), deep wound infection (1). No peri-operative mortality.

Shapiro 199227Harrington rod (2), Harrington rod with sublaminar wires (7), Harrington rod, Luque rod and 2 double sublaminar wires at each level (17).Cobb angle, FVC, mortality, complications.1 sudden cardiac arrest and died intra-operatively. 3 intra-operative complications reversed without sequelae. Mean post-operative correction 13.1 +/- 11.9º, with mean loss of correction 5.1 +/- 3.1º at 2.4 +/- 1.8 years. Mean FVC preoperatively was 45.3 +/- 15.9% with continuing diminution to 28.7 +/- 14.9% at 3.3 +/- 2.2 years after surgery.Concluded that the main benefit of surgical stabilization was the relative ease and comfort of wheelchair seating compared with those non-operated patients who develop progressive deformity. No lasting improvement or stabilization in FVC following surgery as decreasing function was related primarily to muscle weakness.

Stricker 199646 (included other neuromuscular diseases)Modified Luque technique.Cobb angle, complications.Cobb angle decreased from 63 to 24º (correction of about 62%). Failure of implants, pseudarthroses and major losses of correction in purely neuromuscular scolioses could be avoided by using rigid segmental fixation and a dorsolateral fusion with a mixture of autologous and allogenous bone.Recommended that in DMD the best method of treatment was surgery performed as early as possible, i.e. at the time of loss of walking capacity in the case of a scoliosis exceeding 20º and with two consecutive X-rays proving curve progression.

Sussman 198411Harrington instrumentation (group I) (3), Luque instrumentation (group II) (3), segmental spinal instrumentation with fusion (group III) (5).Complications, Cobb angle, hospital stay.Mean Cobb angle correction: 40% (I), 35% (II), 60% (III). When surgery to stabilize spinal deformity is done in younger patients in whom pulmonary function is better and curves are milder, complication rate and length of hospital stay are diminished, correction and balance are improved, and patients rapidly return to their normal life-style.Concluded that segmental spinal instrumentation had advantage of allowing rapid mobilization without need of a cast or body jacket. Recommended stabilization of the collapsing spine surgically with segmental instrumentation and fusion when scoliosis reached 30 to 40º.

Takaso 201020Segmental pedicle screws instrumentation and fusion to L5.Cobb angle, pelvic obliquity, operating time, blood loss, complications.Mean Cobb angle decreased from 70º to 15º. Mean pelvic obliquity decreased from 13º to 6º . The mean intraoperative blood loss was 890 ml (range: 660 to 1260 ml). The mean total blood loss was 2100 ml (range: 1250 to 2880 ml). There was no major complication.

Thacker 20025Not detailed in DMD patients.FEV1, FVC, mortality, complications.FVC and FEV1 maintained, pseudarthrosis in 1 patient, no peri-operative mortality.Included 7 SMA, 6 spastic cerebral palsy, 3 congenital myopathy, 2 spina bifida, 1 paraspinal neuroblastoma in the series.

Velasco 200756Posterior spinal fusionPercent normal FVCThe rates of FVC decline were 4% per year presurgery, which decreased to 1.75% per year postsurgery

Weimann 198324Long Harrington instrumentations and spinal fusions from S1 up to the upper thoracic spine (T4, 5, or 6).Mortality, complications.One patient died 2 years after his operation from dystrophic cardiomyopathy.Concluded that prophylactic spinal fusion deserved consideration in the care planned for these patients.

 ARDS: adult respiratory distress syndrome;DMD: Duchenne muscular dystrophy; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 second; PEFR: peak expiratory flow rate; SMA: spinal muscular atrophy