Effect of spinal surgery on lung function in Duchenne muscular dystrophy.

Abstract

Background -The effect on subsequent respiratory function of spinal stabilisation for scoliosis in Duchenne muscular dystrophy is unclear. In order to clarify this clinical problem, changes in the forced vital capacity of a group of children with Duchenne muscular dystrophy who had undergone spinal surgery were measured and compared with a group of children with Duchenne muscular dystrophy who had not had surgery. Methods -In this retrospective study 17 boys with Duchenne muscular dystrophy who underwent spinal stabilisation at a mean age of 14-9 years (surgical group) were compared with 21 boys with Duchenne muscular dystrophy who had not had surgery (non-surgical group). The mean (SD) Cobb angle of the surgical group at 14-9 years was 57 (16.4)0, and of the non-surgical group at 15 years was 45 (29.9)°. Forced vital capacity expressed as percentage predicted (% FVC) was measured in total over a seven year period in the surgical group and over 6 5 years in the non-surgical group, and regression equations were calculated. Survival curves for both groups were also constructed. Results -No difference was found between spinal stabilisation (surgical group) and the non-surgical group in the rate of deterioration of % FVC which was 3-5% per year. There was no difference in survival in either group. Conclusions -Spinal stabilisation in Duchenne muscular dystrophy does not alter the decline in pulmonary function, nor does it improve survival.

Scoliosis in Duchenne’s muscular dystrophy: a changing trend in surgical management

“…Surgical treatment of scoliosis in DMD is mainly performed to restore the balance of the spinal column in both coronal and sagittal planes and to improve table top activities. Surgery has also been advocated to improve lung compliance; however this is controversial [18][19][20]. Surgical treatment can halt progression of deformity in patients with DMD, however, their compromised general condition, quality and requirements of life, and associated medical comorbidities should be taken into account prior to embarking on such major surgical procedures.…”

Spinal fusion in patients with Duchenne's muscular dystrophy and a low forced vital capacity

“…It is the most common of the dystrophies (1 in 5000 live male births) and is characterised by a progressive muscle wasting and weakness leading to a loss of ambulation by the early teens. Muscle weakness is due to lack of the protein dystrophin in skeletal muscle cell membranes, and this can be tested for by immunohistochemical techniques or by western blotting electrophoresis on a muscle biopsy specimen [3,7,9,10,13,15,16,17].…”

“…Most patients with DMD develop a progressive scoliosis starting at the time of loss of ambulatory status [1,3,5,7,9,12,13,15,18,19,20,22,23]. This occurs in 50-95% of patients, and is associated with a deteriorating vital capacity (4% for every 10°Cobb angle).…”

Can the caudal extent of fusion in the surgical treatment of scoliosis in Duchenne muscular dystrophy be stopped at lumbar 5?

“…Spinal deformity is almost universal and progressive in patients with DMD [7,11,19,29,33]. The posterior spinal fusion for neuromuscular scoliosis has been highly effective in stabilizing the spine and maintaining an upright and comfortable sitting balance [5,9,13].…”

“…Duchenne muscular dystrophy (DMD) is the most common and severe form of muscular dystrophy and is a flaccid neuromuscular disorder in which gradual deterioration is the hallmark [7,11,19,22,27,29,33]. Spinal deformity such as scoliosis is almost universal in patients with DMD [7,11,19,22,27].…”