Surgery for scoliosis in Duchenne muscular dystrophy

Abstract

Background Scoliosis in people with Duchenne muscular dystrophy is usually progressive and treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most people with Duchenne muscular dystrophy and scoliosis. This is an updated review and an updated search was undertaken in which no new studies were found. Objectives To determine the effectiveness and safety of spinal surgery in people with Duchenne muscular dystrophy with scoliosis. We intended to test whether spinal surgery is effective in increasing survival, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects.

Functional Outcomes in Duchenne Muscular Dystrophy Scoliosis

“…Although several previous studies 10, 11 have had different objectives and focused on different outcomes, most studies 1,12,15,22,[26][27][28] have aimed to investigate whether spinal surgery improves the radiographic parameters. A few studies 12,29,30 have described patient-oriented subjective outcomes such as quality of life, selfimage, pain, and patient satisfaction.…”

“…See Instructions for Authors for a complete description of levels of evidence. S coliosis is a progressive and major problem of Duchenne muscular dystrophy 1 . Deformity progression may be very rapid and compromise respiratory and cardiac function 2 .…”

“…Generally, patients are more concerned with how surgery will help them walk, sit, and perform daily tasks in both the short and long term, a need which most functional outcome measurement tools fail to evaluate [4][5][6][7][8][9][10] . Thus, there is much to be investigated in terms of patient functionality after surgery 1,13 .…”

Successful Surgery for Scoliosis Supported by Pulmonary Rehabilitation in a Duchenne Muscular Dystrophy Patient With Forced Vital Capacity Below 10%

“…Severe scoliosis commonly induces many problems, such as imbalance of the sitting position, decreased cardiopulmonary function, musculoskeletal pain, pressure ulcers, and so on [5]. Also, high pressure on the thoracic cage and abdomen makes respiration and expectoration difficult, and mechanical ventilatory support and assistive coughing methods are needed.…”

Spinal management of Duchenne muscular dystrophy in children

“…30 They propose that ''randomized controlled trials are needed to investigate the effectiveness of scoliosis surgery, in terms of patients' satisfaction, quality of life, functional status, respiratory function (FVC, forced expiratory volume in one second, peak expiratory flow) and survival.'' 30 They propose that ''randomized controlled trials are needed to investigate the effectiveness of scoliosis surgery, in terms of patients' satisfaction, quality of life, functional status, respiratory function (FVC, forced expiratory volume in one second, peak expiratory flow) and survival.''…”