We determined the frequency, rate and extent of development of scoliosis (coronal plane deformity) in wheelchair-dependent patients with Duchenne muscular dystrophy (DMD) who were not receiving steroid treatment. We also assessed kyphosis and lordosis (sagittal plane deformity). The extent of scoliosis was assessed on sitting anteroposterior (AP) spinal radiographs in 88 consecutive non-ambulatory patients with DMD. Radiographs were studied from the time the patients became wheelchair-dependent until the time of spinal fusion, or the latest assessment if surgery was not undertaken. Progression was estimated using a longitudinal mixed-model regression analysis to handle repeated measurements. Scoliosis ≥ 10° occurred in 85 of 88 patients (97%), ≥ 20° in 78 of 88 (89%) and ≥ 30° in 66 of 88 patients (75%). The fitted longitudinal model revealed that time in a wheelchair was a highly significant predictor of the magnitude of the curve, independent of the age of the patient (p <  0.001). Scoliosis developed in virtually all DMD patients not receiving steroids once they became wheelchair-dependent, and the degree of deformity deteriorated over time. In general, scoliosis increased at a constant rate, beginning at the time of wheelchair-dependency (p < 0.001). In some there was no scoliosis for as long as three years after dependency, but scoliosis then developed and increased at a constant rate. Some patients showed a rapid increase in the rate of progression of the curve after a few years – the clinical phenomenon of a rapidly collapsing curve over a few months. A sagittal plane kyphotic deformity was seen in 37 of 60 patients (62%) with appropriate radiographs, with 23 (38%) showing lumbar lordosis (16 (27%) abnormal and seven (11%) normal). This study provides a baseline to assess the effects of steroids and other forms of treatment on the natural history of scoliosis in patients with DMD, and an approach to assessing spinal deformity in the coronal and sagittal planes in wheelchair-dependent patients with other neuromuscular disorders. Cite this article: Bone Joint J 2014;96-B:100–5

Spinal fusion, ending caudally at L5 rather than at the sacrum, is recommended for selected patients with scoliosis due to Duchenne muscular dystrophy. We present a retrospective review of 48 patients operated on for this condition. Patients having spinal curvature with a Cobb angle of less than 40° and with less than 10° between a line tangential to the superior margins of both iliac crests and a line perpendicular to the spinous processes of L4 and L5, were fused to L5 (38 patients); patients not meeting these criteria were fused to the sacrum (10 patients). Spinal and sitting obliquity increased in patients fused to L5, rather than to the sacrum, but the severity of the worsening obliquity was significantly greater in patients in whom the apex of the curve was below L1. Two of the ten latter patients required revision procedures for worsening obliquity when their pulmonary function deteriorated to less than 25% of predicted values. We recommend fusion to the sacrum for scoliosis in Duchenne muscular dystrophy, especially for patients with an apex to their curve below L1

Of 55 patients with Duchenne muscular dystrophy offered surgical stabilisation of the spine, 32 accepted and 23 refused. We compared both groups pre-operatively and at six-month intervals in respect of survival, forced vital capacity, peak expiratory flow rate and severity of scoliosis. In the nonoperated patients, the forced vital capacity deteriorated by a mean of 8% per annum; in the operated group it remained static for 36 months and diminished slightly thereafter. Spinal stabilisation resulted in an improvement in the peak expiratory flow rate which was maintained for up to five years. In the nonoperated patients the scoliosis progressed from a mean of 37 degrees to a mean of 89 degrees at five years; in the stabilised spines it was improved from a mean of 47 degrees to a mean 34 degrees at five years. There was significantly improved survival in the patients who had undergone spinal stabilisation

Equinus deformity of the ankle is one of the serious orthopaedic problems associated with Duchenne muscular dystrophy. Sixty-nine patients (age range 4 to 17 years) were treated, 43 conservatively and 26 operatively. They were followed up at six-monthly intervals for a minimum of two years and a maximum of six years. The patients were divided into three groups: independently mobile, mobile in calipers, and wheelchair-bound. It was found that conservative treatment could at best only minimise progression of the deformity. The indications for surgery, the operative procedure and the postoperative management are described; all varied according to the stage of the disease. The postoperative follow-up suggests that, though the deformity recurs, the patients have several years of benefit from the procedure

For clinical, psychological and social reasons the diagnosis of Duchenne muscular dystrophy should be established as early as possible. In a survey of 83 families with 93 affected boys, the diagnosis was missed in every case referred to an orthopaedic surgeon (37 patients). In the whole group there was a mean delay of 2.0 years (0 to 6 years) during which time inappropriate treatment, difficulties in communication with parents, much parental anxiety and further pregnancies occurred. A serum creatine kinase estimation is a simple outpatient test which should be carried out on any boy with clumsy or abnormal gait, with flat feet or with an unexplained equinus deformity

Objective: To assess the effect of spinal surgery and nocturnal ventilation on lung function and survival in patients with scoliosis secondary to Duchenne Muscular Dystrophy. Study design: Prospective, observational study by a single observer (Research Physiotherapist). Subjects: 80 patients with Duchenne Muscular Dystrophy were treated between 1986 and 2002. During this period 40 patients underwent a spinal fusion at a mean age of 14.05y (95 % CI 13.6 – 14.6). Nocturnal ventilation was commenced when symptoms and signs of respiratory failure were evident. The mean FVC at commencement of nocturnal ventilation was 0.41 litres. The patients were divided into 2 groups based on whether they received nocturnal ventilation. A total of twenty eight patients received nocturnal ventilation and 52 did not. The groups were further sub-divided based on whether they had spinal fusion. There were fourteen patients in each sub-group of the ventilated group and 26 patients in each sub-group of those that were not ventilated. Outcomes: Serial forced vital capacity (FVC) measurements and survival measured by Kaplan Meir survival analysis. Results: The mean vital capacity dropped from 1.41 l (95 % CI 1.21 – 1.61) to 1.13 (95 % CI 0.893 – 1.37), a year post-operatively. This was not associated with the development of respiratory compromise. The vital capacity improved gradually, reaching the pre-operative level before it declined again. The shortest survival was seen in patients who received neither surgery nor ventilation (median survival 19.7y). The patients who received surgery but no ventilatory support were not as good as the patients that were ventilated but did not have surgery (median survival 24.3y). The best results were seen in the patients who had both surgery and ventilation (median survival 26.4y). The worst prognosis is in patients with early onset symptomatic cardiomyopathy (6 patients, with a median survival of 16.3y). Conclusion: Nocturnal ventilation is the most important factor in the improvement in survival of patients with Duchennes muscular dystrophy. Spinal surgery is also beneficial and the best results are in those patients who have both

Prior to the introduction of steroid management in Duchenne Muscular Dystrophy (DMD), the prevalence of scoliosis approached 100%, concomitant with progressive decreases in pulmonary function. As such, early scoliosis correction (at 20-25°) was advocated, prior to substantial pulmonary function decline. With improved pulmonary function and delayed curve progression with steroid treatment, the role of early surgery has been questioned. The purpose of this study was to compare the post-operative outcomes of early versus late scoliosis correction in DMD. We hypothesize that performing later surgery with larger curves would not lead to worse post-operative complications. Retrospective cohort study. Patients with DMD who underwent posterior scoliosis correction, had pre-operative pulmonary function testing, and at least 1-year post-operative follow-up, were included; divided into 2 Groups by pre-operative curve angle – 1: ≤45°, 2: >45°. Primary outcome was post-operative complications by Clavien-Dindo (CD) grading. Secondary outcomes included: age at surgery, forced vital capacity (FVC), steroid utilization, fractional shortening (FS) by echocardiogram, surgery duration, blood transfusion requirements, ICU length of stay (LOS), days intubated post-operatively, hospital LOS, infection, curve correction. Two-tailed t-test and chi-square testing were used for analysis of patient factors and CD complication grade, respectively. 31 patients met the inclusion criteria, with a mean total follow-up of 8.3±3.2 years. Steroid treatment (prednisone, deflazacort) was utilized for 21 (67.7%) patients, for a mean duration of 8.2±4.0 years. Groups were comparable for steroid use, FVC, echo FS, and age at surgery (p>0.05). Primary curve angle was 31.7±10.4° and 58.3±11.1° for Groups 1 and 2, respectively (p 0.05). Surgery duration, ICU LOS, days intubated, hospital LOS, were also not different between Groups. For the entire cohort, however, the overall complication rate was higher for patients with steroid treatment [61.9% vs 10%, respectively (p=0.008)], the majority being CDII. Neither FVC nor echo FS were different between Groups at final follow-up (p=0.6; p=0.4, respectively). Post-operative complication rates were not different for early and late scoliosis correction in DMD. In general, however, patients undergoing steroid treatment were at higher risk of blood transfusion and deep infection. Delaying scoliosis correction in DMD while PF is favourable is reasonable, but patients with prior steroid treatment should be counseled regarding the higher risk of complications

This is a retrospective study describing four patients who developed symptomatic subluxation of the hip after stabilisation to the pelvis for myopathic scoliosis in Duchenne Muscular Dystrophy (DMD). Fusion to the pelvis is recommended for treatment of scoliosis in DMD. Non-spinal extra-pulmonary complications following this have not been described. 4 patients (average age: 14 years) out of a cohort of patients who have undergone spinal stabilisation for DMD between 1991 and 1998 developed symptomatic subluxation of the hip at an average of three months after fusion from the upper thoracic spine to the pelvis. All four had pain and three noticed clicking in the hip. X-rays revealed subluxation of the hip in all patients, and conservative treatment by adjustment of seating position in the wheelchair was successful in reducing the symptoms in all patients. Flexion-abduction contractures of the hip, which are a feature of DMD, are known to cause uncovering of the contralateral hip. We postulate that the spine compensates for this uncovering to a large degree, and that spinopelvic fusion for scoliosis in patients with pre-existent abduction contractures negates the capacity of the spine to provide compensation. This leads to uncovering of the hip with the lesser degree of contracture, and the resultant symptoms. We recommend screening for, and treatment of, flexion-abduction contractures of the hip in all patients undergoing spinal fusion for DMD, to avoid the possibility of development of symptomatic subluxation of the hip

Aprotinin has been shown to reduce blood loss in a number of surgical specialities. Patients with Duchenne Muscular Dystrophy (DMD) bleed more during surgical procedures than patients without this condition. The aim of this study was to evaluate the effect of aprotinin in reducing blood loss in scoliosis correction surgery in patients with DMD. A retrospective analysis of case notes was performed. Thirty two patients diagnosed with DMD who underwent surgical correction for scoliosis over the last 25 years were included. All patients underwent posterior spinal fusion and instrumentation, between the levels T3 and L3. All procedures were carried out by the same lead surgeon. Patient age, body weight, length of procedure, and estimated blood loss were recorded. Blood loss as a percentage of total circulating volume was calculated and compared between patients who had not received aprotinin (seven patients), and those who did (25 patients). Blood loss as a percentage of total circulating volume in the group of patients with aprotinin (range 37% – 107% mean 67%) was significantly lower (P< 0.05) than the group without aprotinin (range 67% – 157% mean 111%). There was found to be no statistically significant relationship between blood loss and length of procedure. There was no statistically significant difference in the duration of the procedure between the two groups of patients. Despite the small number of patients this study shows a beneficial effect for aprotinin in reducing blood loss during scoliosis correction surgery in patients with DMD

Background: There are conflicting reports regarding the effect of scoliosis surgery on respiratory function in Duchenne Muscular Dystrophy (DMD). 1. ,. 2. Galasko et al. 2. found that the Percentage Predicted Forced Vital Capacity (%PFVC), remained static for thirty six months following surgery, in patients with DMD that underwent spinal stabilisation for scoliosis. The aim of the current study was to support or refute the above finding in a large series of patients with DMD. Methods: A retrospective analysis of data on 55 consecutive patients with DMD that underwent single stage posterior surgical correction for scoliosis. We analysed the data of 55 boys with DMD who underwent scoliosis surgery between 1990 and 2002. Age at surgery, pre-operative Cobb angles, pre-operative %PFVC, and post-operative %PFVC at 6 months, 12-18 months and 2–3 years were collected. We documented the pre-operative Cobb angle ± SD to assess the difficulty level of our surgical cases. Percentage PFVC was used as our outcome measure to assess respiratory function. The mean pre-operative %PFVC was compared to the post –operative mean %PFVC at three different time intervals; at 6 months, 12 to 18 months and at 2 to 3 years. Results: The mean age was 14.6 years (range 11.2–18yrs). The mean pre-operative Cobb angle was 65.4 degrees ± 14.8. The mean %PFVC pre-operatively was 33.9 ± 10.4. The mean post-operative %PFVC’s were: 6 months (29.1 ± 10.4), 12 to 18 months (27.6 ± 12.1) and 2 to 3 years (25.4 ± 8.7). Therefore the mean % PFVC following surgery at 6 months, 12 to 18 months and 2 to 3 years decreased from the mean pre-operative % PFVC by 4.8%, 6.3% and 8.5% respectively. Conclusion: The natural history of patients with DMD is a gradual decline in respiratory function. In the current study the mean post –operative %PFVC was less than the mean pre-operative %PFVC at 6 months, 12 to 18 months and at 2 to 3 years post surgery. Our series would suggest that respiratory function declines post-operatively, even in the short term, in patients with DMD that undergo spinal stabilisation. The decline in respiratory function in our study was progressive over the 3 year follow up period

Boys affected by Duchenne Muscular Dystrophy (DMD) often develop significant scoliosis in the second decade of life and require scoliosis surgery. Our aim was to establish whether cardiac MRI (CMR) improves the preoperative risk assessment in DMD patients and evaluate the current risk of surgery. Case records were retrospectively reviewed for 62 consecutive DMD boys who underwent pre-surgical evaluation at a single tertiary neuromuscular centre between 2008–2013. 62 DMD patients aged 7–18 years underwent pre-operative assessment for a total of 70 procedures (45 spinal, 19 foot, 6 gastrostomy). Echocardiography data were available for 68 procedures. Echo revealed a median left ventricular (LV) shortening fraction (SF) of 29% (range: 7–44). 34% of boys (23/68) had abnormal SF <25%, 48% (31/65) showed dyskinesia and 22% (14/64) had LV dilatation. CMR was routinely performed on 35 patients. Of those who underwent CMR, median left ventricular ejection fraction (LVEF) was 52% (range: 27–67%), 71% of boys (25/35) had dyskinesia. Echocardiography shortening fraction (SF) correlated significantly with CMR LVEF (r. s. = 0.67; p<0.001). Increasing severity of dyskinesia on CMR correlated with reduced CMR LVEF (r. s. = −0.64; p<0.001) and reduced echo SF (r. s. = −0.47; p = 0.004). Although functional echocardiography and CMR data tended to correlate in 35 DMD boys who underwent both imaging modalities nine (26%) had discrepant results. Seven (20%) had evidence of dysfunction on CMR (LVEF < 55%) not detected on echocardiography (SF ≥ 27%); in two cases echocardiogram measured worse function than CMR. Based on multi-disciplinary risk assessment, surgery was considered too high risk in 23 out of 67 (34%) cases. In 21 cases (91%) this was due to underlying cardiomyopathy. The highest risk among older boys assessed for spinal surgery; 21 out of 43 (49%). Of 19 boys undergoing spinal surgery, six (32%) experienced complications: two wound infections; three patients required readmission to intensive care; one patient died in the post-operative period with acute heart failure

Objective: This study investigates whether fixation down to lumbar spine only can prevent pelvic tilt compared to pelvic fixation, in the surgical treatment of Duchenne Muscular Dystrophy (DMD). Design: Retrospective and prospective clinical outcome study, with long-term follow up. Materials and Methods: Nineteen cases of DMD with scoliosis had early stabilisation (mean age 11.5 years, range 9–16) with sublaminar wires and rods, and pedicle screws up to the lumbar spine. This cohort was followed up for a mean 4.2 years (3–10 years). 31 cases in another centre had late stabilisation (mean age 14.5 years, range 10–17), with Luque rod and sublaminar wire fixation, and pelvic fixation using L-rod (22 cases) configuration or Galveston technique (9 cases) and were followed up for 4.6 years (0.5–11.5 years). Post-op morbidity, Cobb angle correction and pelvic obliquity data were collected retrospectively and prospectively for comparison. Results: In the lumbar fixation group FVC was 58%, the mean Cobb angle and pelvic obliquity were 19.8° and 9° preoperative, 3.2° and 2.2° direct postoperative, and 5.2° and 2.9° at final follow up respectively. The mean estimated blood loss was 3.3 litres and average hospital stay 7.7 days. In the pelvic fixation group FVC was 44%, the mean Cobb angle and pelvic obliquity were 48° and 19.8° preoperative, 16.7° and 7.2° direct postoperative, and 22° and 11.6° at final follow up respectively. The mean blood loss (4.1 litres) and the average hospital stay (17 days) were significantly higher (p< 0.05) compared to the lumbar fixation group. The pelvic fixation group had higher complication rate at the lower end of fixation. No progression of the pelvic obliquity was noted in the lumbar fixation group during follow up. Conclusion: Lumbar fixation may be adequate for scoliosis in DMD, if the stabilisation is performed early, before the pelvis becomes tilted, and scoliosis becomes significant. The caudal pedicular fixation in the lumbar spine stops rotation of the spine around the rods, and prevent pelvic tilt to occur. Pelvic fixation may be necessary in presence of established pelvic obliquity and larger scoliosis, but is associated with higher morbidity and complications

Introduction: Historically segmental sublaminar wiring (SLW) fixation has been used for the correction of spinal deformity in neuromuscular scoliosis, however pedicle screw (PS) fixation is gaining popularity. We compared the results of both techniques in patients with Duchenne Muscular Dystrophy (DMD). Methods: 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 to S1 with the Galveston technique. In Group 2 (18 patients) PS fixation was used from T2 to L5. Minimum follow-up was 2 years (range 2–13 years). Radiographs, SRS-22 and lung function tests were performed at standardised intervals. Results: Mean Cobb angle in Group 1 improved from 47° (range 26°–75°) to 23.5° (range10°–36°) and mean pelvic obliquity improved from 15° (range8°–25°) to 2.4° (range0°–8°). Mean Cobb angle in Group 2 improved from 46° (range28°–82°) to 8.5° (range 0°–18°) and mean pelvic obliquity improved from 15° (range7°–30°) to 1.1° (range 0°–6°) [p< 0.05]. Mean operating time and blood loss were less in Group 2 [p< 0.05]. In Group 1, the infection rate and instrumentation failure was higher, and SRS-22 outcomes showed no significant difference between the groups. Interestingly the mean Body Mass Index (BMI) in Group 2 was much higher than group 1. Conclusions: PS fixation resulted in superior correction and controlled pelvic obliquity to a large extent without the need for pelvic fixation. Lower rates of infection and failure of instrumentation were noted with PS fixation, despite high BMI of patients presumably due to steroid therapy. We recommend the use of PS instrumentation for the correction of spinal deformity in DMD

Aim:. Our aim was to report the rate and risk factors for post-operative complications in Duchenne Muscular Dystrophy (DMD) patients undergoing spinal arthrodesis for scoliosis, with a comparison to neuromuscular scoliosis of other aetiology. Methods:. From a prospective single surgeon spinal deformity database, we identified all patients with neuromuscular disorders who underwent surgical correction for progressive scoliosis. We recorded demographic and peri-operative data, including complications and subsequent procedures. The rate and risk factors for complications was determined, with a sub-analysis of the DMD group to determine any peri-operative factors predictive of overall complication rates. Results:. There were 98 patients, 59 (60%) were male with mean age at surgery 14 yrs (7–19 yrs). Forty-eight patients had cerebral palsy (n=48, 49%) and 26 DMD (27%). The overall complication rate was 18.4% (n=18), with deep wound infection (DWI; n=9, 9.2%) and acute liver injury (ALI; n=4, 4.0%) most frequent. The complication rate was significantly higher in DMD patients (35%) compared to other neuromuscular disorders (13%; p=0.013). All ALIs occurred in DMD patients (p=0.004), with an increased DWI rate (19% vs 6%; p=0.053). On subanalysis, no peri-operative factors correlated with overall complication rate or DWI rate. Increased intraoperative blood loss was the only factor associated with ALI (p=0.036). Discussion:. Scoliosis correction has an acceptable complication rate in patients with neuromuscular diagnoses. DMD patients have an increased complication rate when compared to other neuromuscular disorders. ALI in our series was unique to DMD patients and we would recommend peri-operative vigilance for hepatotoxicity in these patients. Conflict Of Interest Statement: No conflict of interest

Introduction: Duchenne’s Muscular Dystrophy (DMD) is a progresssive sex linked recessive disorder predominantly involving skeletal muscle. Scoliosis is almost universal in patients with DMD. Surgical stabilisation carries significant risks and complications with per-operative mortality of < 6%. Cardiopulmonary complications along with severe intraoperative blood loss requiring massive nlood transfusion are the major cause of morbidity. Aim: To evaluate the efficacy of single rod fusion technique in reducing the peroperative and post operative complications especially blood loss, duration of surgery and progression of curve. Materials and Methods: Retrospective review- 32 patients with scoliosis secondary to DMD with an average age of 14 years (range, 11–18) underwent either single rod fusion technique (19 patients) using Isola rod system or Hartshill rectangle/double rod fusion technique (13 patients). Blood loss was measured directly from the peroperative suction and postoperative drainage, indirectly by weighing swabs. Vapour free hypotensive anaesthesia was used in all cases. Progression of curve was followed up in the outpatients. Results: The mean operative time was 130 minutes (range, 80–180) for the single rod fusion technique in comparison to 250 minutes (range, 170–300) for the Hartshill/Double rod technique. The average blood loss for the single rod fusion technique was reduced, 2.2 L (range 0.4–4) versus 3.1L (0.8–4). The mean follow up was 35 months (range, 5–72). The inpatient stay was 12 days (range, 6–23). Seven patients developed complications: 3 ileus, 2 respiratory tract infections, one patient had loosening and migration of the rod, which required revision under LA, and one patient developed a superficial wound infection, which resolved with intravenous antibiotics. Conclusion: In our experience, single rod stabilisation is a safe and quick method of correcting the DMD scoliotic spine, with less blood loss and complications compared to traditional methods

Introduction: Duchenne’s Muscular Dystrophy (DMD) is a progressive sex linked recessive disease, predominantly involving skeletal muscle. Scoliosis is almost universal in patients with DMD. Surgical stabilization carries a significant risks and complications with peroperative mortality of < 6%. Cardiopulmonary complications along with severe intraoperative blood loss requiring massive blood transfusion are the major cause of morbidity. Aim: To evaluate the efficacy of single rod fusion technique in reducing the peroperative and post operative complications especially blood loss, duration of surgery and progression of curve. Material & Methods: Retrospective review – 14 patients with scoliosis secondary to DMD with an average age of 14.5 years (range, 11–17) underwent single rod fusion technique using Isola rod system and pelvic was not included in fixation. Blood loss was measured directly from the peroperative suction and post operative drainage, indirectly by weighing the swabs. Vapour free hypotensive anesthesia was used in all case. Progression of curve was monitored over a period of five years. Results: The mean operative time was 110 min (range, 80 – 180). The average blood loss was 1.6L (range, 0.7 – 5). The mean follow up was 32 months (range, 4 – 60). There was no progression noticed in the curve on follow up. Two patients develop complications, one had loosening & migration of the rod, required revision and superficial wound infection treated with intravenous antibiotics. Conclusion: In our experience, single rod stabilization is a safe and quick method of stabilizing the spine in DMD with less blood loss and complications compared to traditional methods

A consecutive series of 85 patients with Duchenne’s muscular dystrophy who underwent spinal fusion over a period of 16 years was followed up with regard to the progression of the scoliosis and pelvic obliquity. Of 74 patients with adequate radiographic follow-up, 55 were instrumented with the Luque single-unit rod system and 19 with the Isola pedicle screw system; seven were instrumented to L3/4, 42 to L5, 15 to S1 and 10 to the pelvis with intrailiac rods. The mean period of follow-up was 49 months (SD 22) before and 47 months (SD 24) after operation. There was one peri-operative death and three cases of failure of hardware. The mean improvement in the Cobb angle was 26° and in pelvic obliquity, 9.2°. Fusion to L3/4 achieved a poorer correction of both curves while intrapelvic rods, achieved and maintained the best correction of pelvic obliquity. Fusion to S1 did not provide any benefit over more proximal fusion excluding the sacrum, with regard to correction and maintenance of both angles. The Isola system appeared to provide and maintain a slightly better correction of the Cobb angle

Aim: This study is to assess whether spinal fusion surgery can be performed safely in patients with Duchenne’s muscular dystrophy (DMD) and a low (less than 30%) predicted forced vital capacity (PFVC). Patients- Methods: Patients were identified with a diagnosis of scoliosis secondary to DMD who underwent spinal fusion procedures at the Royal National Orthopaedic Hospital, Stanmore between January 1990 and December 1999. Their notes and radiographs were reviewed and a standardised data collection form was completed. Results: Thirty patients with a mean age of 14 years 8 months at surgery underwent posterior spinal fusions. All were discharged from hospital alive and self-ventilating on average 22 days postoperatively (range 13–62 days). Thirteen patients had a PFVC less than 30%. The mean preoperative curve was 61 degrees (range 30 to 90) and the mean number of levels fused was 15 (i.e. T3 to sacrum). The mean correction was 36 degrees (range 16 to 61). Two patients required temporary tracheotomies, one with a PFVC of 34% and one with a PFVC of 20%. Both were removed successfully after 39 days and 27 days respectively. There was no association between PFVC and operative time, blood loss, length of time on ventilatory support, time intubated, incidence of complications or length of admission. Conslusion: Historically, only curves of greater than 20–350 have been considered suitable for surgery, as the progression of the curve is associated with a marked decline in respiratory function. Considering the currently used criteria for surgery, the group of 13 with low PFVCs normally would have been denied surgery. We conclude that spinal fusion surgery can be safely performed in DMD patients with a low PFVC

This study assessed whether spinal fusion surgery could be performed safely in patients with Duchenne’s muscular dystrophy (DMD) and a low (less than 30%) predicted forced vital capacity (PFVC). Patients were identified with a diagnosis of scoliosis secondary to DMD who underwent spinal fusion procedures at the Royal National Orthopaedic Hospital, Stanmore between January 1990 and December 1999. Their notes and radiographs were reviewed and a standardised data collection form was completed. Thirty patients with a mean age of 14 years 8 months at surgery underwent posterior spinal fusions. All were discharged from hospital alive and self-ventilating on average 22 days post-operatively (range 13–62 days). Thirteen patients had a PFVC less than 30%. The mean pre-operative curve was 61 degrees (range 30 to 90) and the mean number of levels fused was 15 (i.e. T3 to sacrum). The mean correction was 36 degrees (range 16 to 61). Two patients required temporary tracheotomies, one with a PFVC of 34% and one with a PFVC of 20%. Both were removed successfully after 39 days and 27 days respectively. There was no association between PFVC and operative time, blood loss, length of time on ventilatory support, time intubated, incidence of complications or length of admission. Historically, only curves of greater than 20–35 degrees have been considered suitable for surgery, as the progression of the curve is associated with a marked decline in respiratory function. Considering the currently used criteria for surgery, the group of 13 with low PFVCs normally would have been denied surgery. We conclude that spinal fusion surgery can be safely performed in DMD patients with a low PFVC

Introduction and Aims: This study is to assess whether spinal fusion surgery can be performed safely in patients with Duchenne’s muscular dystrophy (DMD) and a low (less than 30%) predicted forced vital capacity (PFVC). Method: Patients were identified with a diagnosis of scoliosis secondary to DMD who underwent spinal fusion procedures at the Royal National Orthopaedic Hospital, Stanmore between January 1990 and December 1999. Their notes and radiographs were reviewed and a standardised data collection form was completed. Results: Thirty patients with a mean age of 14 years 8 months at surgery underwent posterior spinal fusions. All were discharged from hospital alive and self-ventilating on average 22 days post-operatively (range 13–62 days). Thirteen patients had a PFVC less than 30%. The mean pre-operative curve was 61 degrees (range 30–90) and the mean number of levels fused was 15 (i.e. T3 to sacrum). The mean correction was 36 degrees (range 16–61). Two patients required temporary tracheotomies, one with a PFVC of 34% and one with a PFVC of 20%. Both were removed successfully after 39 days and 27 days respectively. There was no association between PFVC and operative time, blood loss, length of time on ventilatory support, time intubated, incidence of complications or length of admission. Conclusion: Historically, only curves of greater than 20–350 have been considered suitable for surgery, as the progression of the curve is associated with a marked decline in respiratory function. Considering the currently used criteria for surgery, the group of 13 with low PFVCs normally would have been denied surgery. We conclude that spinal fusion surgery can be safely performed in DMD patients with a low PFVC