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

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

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

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

We have treated 101 patients with scoliosis secondary to muscular dystrophy over a 13-year period; 64 had Duchenne’s muscular dystrophy, 33 spinal muscular atrophy and four congenital muscular dystrophy. The patients underwent a modified Luque (87) or Harrington-Luque instrumentation (14) combined with a limited Moe fusion in all except 27 cases. A mean of 13 levels was instrumented. The mean preoperative sitting Cobb angle was 84° (10 to 150) and the mean postoperative angle 40° (52% correction). Most patients (96%) were able to discard their braces and there was a high level of patient satisfaction (89.6%). Less correction was seen for severe curves, and there was a greater recurrence of postoperative pelvic tilt in those patients not instrumented to the sacrum. Although the incidence of minor or temporary complications was high, these occurred chiefly in the early high-risk patients with very severe curves and considerable pre-existing immobility

Aims

Historically, patients undergoing surgery for adolescent idiopathic scoliosis (AIS) have been nursed postoperatively in a critical care (CC) setting because of the challenges posed by prone positioning, extensive exposures, prolonged operating times, significant blood loss, major intraoperative fluid shifts, cardiopulmonary complications, and difficulty in postoperative pain management. The primary aim of this paper was to determine whether a scoring system, which uses Cobb angle, forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and number of levels to be fused, is a valid method of predicting the need for postoperative critical care in AIS patients who are to undergo scoliosis correction with posterior spinal fusion (PSF).

We report the incidence of and risk factors for complications after scoliosis surgery in patients with Duchenne muscular dystrophy (DMD) and compare them with those of other neuromuscular conditions.

We identified 110 (64 males, 46 females) consecutive patients with a neuromuscular disorder who underwent correction of the scoliosis at a mean age of 14 years (7 to 19) and had a minimum two-year follow-up. We recorded demographic and peri-operative data, including complications and re-operations.

There were 60 patients with cerebral palsy (54.5%) and 26 with DMD (23.6%). The overall complication rate was 22% (24 patients), the most common of which were deep wound infection (9, 8.1%), gastrointestinal complications (5, 4.5%) and hepatotoxicity (4, 3.6%). The complication rate was higher in patients with DMD (10/26, 38.5%) than in those with other neuromuscular conditions (14/84, 16.7% (p = 0.019). All hepatotoxicity occurred in patients with DMD (p = 0.003), who also had an increased rate of deep wound infection (19% vs 5%) (p = 0.033). In the DMD group, no peri-operative factors were significantly associated with the rate of overall complications or deep wound infection. Increased intra-operative blood loss was associated with hepatotoxicity (p = 0.036).

In our series, correction of a neuromuscular scoliosis had an acceptable rate of complications: patients with DMD had an increased overall rate compared with those with other neuromuscular conditions. These included deep wound infection and hepatotoxicity. Hepatotoxicity was unique to DMD patients, and we recommend peri-operative vigilance after correction of a scoliosis in this group.

Cite this article: Bone Joint J 2014; 96-B:943–9.

Aims

Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

We compared the clinical, radiological and quality-of-life outcomes between hybrid and total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis. A matched comparison using prospectively collected data was undertaken. A total of 66 patients underwent posterior or anteroposterior correction and fusion with hybrid (n = 33, mean age at surgery 15.8 years (9.10 to 19.6)) or total pedicle screw instrumentation (n = 33, mean age 14.7 years (7.0 to 20.7)) with a minimum follow-up of two years. The major curve pre-operatively was a mean of 87° (sd 29, 25° to 141°) and 81° (sd 18, 47° to 116°) in the hybrid and total pedicle screw groups, respectively (p = 0.29) and at a minimum of two years it was 33° (sd 20; 2° to 87°) and 20° (sd 12; 1° to 55°), respectively (p = 0.0016). The mean correction of the major curve was 59% (41% to 88%) in the hybrid and 75% (43% to 99%) in the total pedicle screw groups at two-year follow-up (p = 0.0011). The mean operating time was 7.45 hours (sd 2.18) and 6.04 hours (sd 1.71) in the hybrid and total pedicle screw groups, respectively (p = 0.001), and the mean intra-operative blood loss was 3760 ml (sd 2790) and 1785 ml (sd 1110), respectively (p = 0.001).

Total pedicle screw instrumentation provided shorter operating times, less blood loss and better correction of the major curve compared with hybrid constructs in patients undergoing surgery for neuromuscular scoliosis.