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In a previous study, the authors reported the clinical and radiological results of Duchenne muscular dystrophy (DMD) scoliosis surgery in 14 patients with a low FVC of <30%. The purpose of this study was to determine if surgery improved function and QOL in these patients. Furthermore, the authors assessed the patients’ and parents’ satisfaction. %FVC increased in all patients after preoperative inspiratory muscle training. Scoliosis surgery in this group of patients presented no increased risk of major complications. All-screw constructions and fusion offered the ability to correct spinal deformity in the coronal and pelvic obliquity initially, intermediate and long-term. All patients were encouraged to continue inspiratory muscle training after surgery. The mean rate of %FVC decline after surgery was 3.6% per year. Most patients and parents believed scoliosis surgery improved their function, sitting balance and quality of life even though patients were at high risk for major complications. Their satisfaction was also high. [ABSTRACT FROM AUTHOR]

Severe spinal collapse and pulmonary function deterioration are so common in patients with Duchenne muscular dystrophy (DMD). The limit for scoliosis surgery has been a minimum forced vital capacity (FVC) of about 30% of predicted value. DMD patients with low %FVC who might benefit from scoliosis surgery have not been being offered surgery. Between 2005 and 2007, a total of 17 DMD patients with FVC of <30% at admission underwent scoliosis surgery. %FVC at admission was 22% (range 16–27%). After admission, they were trained with inspiratory muscle training, using a pulmonary trainer (threshold IMT) for 6 weeks prior to surgery and encouraged to continue the training even after surgery and discharge. %FVC increased in all patients and noted 26% (range 22–31%) the day before surgery. The preoperative scoliosis was 97° (range 81°–130°). All patients had posterior spinal fusion and were extubated on the operative day. No patients developed any respiratory complications. No ventilatory support was needed. The mean ICU stay was 0.5 days (range 0–1 day). The postoperative scoliosis was 31° (range 18°–40°). DMD patients with severe scoliosis and FVC considered too low to permit reasonable surgical risk could undergo surgery after inspiratory muscle training, with no major complications. [ABSTRACT FROM AUTHOR]

Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of scoliosis in patients with Duchenne muscular dystrophy since the development of the intrailiac post. It is recommended for correcting pelvic obliquity. However, caudal extent of instrumentation and fusion has remained a matter of considerable debate. This study was performed to determine the efficacy and safety of stopping segmental pedicle screw constructs at L5 during surgical treatment of scoliosis associated with Duchenne muscular dystrophy (DMD). From May 2005 to June 2007, a total of 20 consecutive patients underwent posterior spinal fusion and segmental pedicle screw instrumentation only to L5 for scoliosis secondary to DMD. All patients had progressive scoliosis, difficulty sitting, and back pain before surgery. A minimum 2-year follow-up was required for inclusion in this study. Assessment was performed clinically and with radiological measurements. The Cobb angles of the curves and spinal pelvic obliquity were measured on the coronal plane. Thoracic kyphosis and lumbar lordosis were measured on the sagittal plane. These radiographic assessments were performed before surgery, immediately after surgery, and at a 3-month interval thereafter. The operating time, blood loss, and complications were evaluated. Patients were questioned about whether they had difficulty sitting and felt back pain before surgery and at 6 weeks, 1 year, and 2 years after surgery. A total of 20 patients, aged 11–17 years, were enrolled. The average follow-up period was 37 months. Preoperative coronal curves averaged 70° (range 51°–85°), with a postoperative mean of 15° (range 8°–25°) and a mean of 17° (range 9°–27°) at the last follow-up. Pelvic obliquity improved from 13° (range 7°–15°) preoperatively to 5° degrees (range 3°–8°) postoperatively and 6° (range 3°–9°) at the last follow-up. Good sagittal plane alignment was recreated and maintained. Only a small loss of correction of scoliosis and pelvic obliquity was noted. The mean operating time was 271 min (range 232–308 min). The mean intraoperative blood loss was 890 ml (range 660–1260 ml). The mean total blood loss was 2100 ml (range 1250–2880 ml). There was no major complication. All patients reported that difficulty sitting and back pain were alleviated after surgery. Segmental pedicle screw instrumentation and fusion only to L5 is safe and effective in patients with DMD scoliosis of <85° and pelvic obliquity of <15°. Good sagittal plane alignment was achieved and maintained. All patients benefited from surgery in terms of improved quality of life. There was no major complication. [ABSTRACT FROM AUTHOR]