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What’s New in the Management of Neuromuscular Scoliosis

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Background:

Patients with neuromuscular scoliosis (NMS) can pose treatment challenges related to medical comorbidities and altered spinopelvic anatomy. We reviewed the recent literature regarding evaluation and management of NMS patients and explored areas where further research is needed.

Methods:

We searched the PubMed database for all papers related to the treatment of NMS published from January 1, 2011 through July 31, 2014, yielding 70 papers.

Results:

A total of 39 papers contributed compelling new findings. Steroid treatment has been most promising in patients with Duchenne muscular dystrophy, leading to a significantly lower death rate, better pulmonary function, and longer independent ambulation. Growing rods in early-onset NMS were shown to result in significant improvements in major Cobb angles and pelvic obliquity, with low complication rates in patients with spinal muscular atrophy but high infection rates in those with cerebral palsy. Early reports of magnetic growing rods in NMS patients are favorable. Intraoperative neural monitoring is variable in this patient population; however, use of transcranial motor-evoked potentials in NMS patients seems to be safe. Blood loss is the highest in NMS patients when compared with all other diagnostic categories. However, tranexamic acid seems to significantly lower intraoperative blood loss. In a multicenter study, patients diagnosed with NMS had the highest surgical-site infection rate at 13.1%. Best-practice guidelines have been created regarding prevention of infection in NMS patients. Preoperative nutritional optimization and postoperative nutritional supplementation seem to help with lowering the infection rate in these patients.

Conclusions:

There have been major advances in the management of NMS patients, but many challenges remain. Further multicenter studies and randomized clinical trials are needed, particularly in the areas of infection prophylaxis, nutritional optimization, improvement in intraoperative neural monitoring, and prevention of proximal junctional kyphosis.

Level of Evidence:

Level 4—literature review.
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Keywords: Duchenne muscular dystrophy; Friedreich’s ataxia; Rett syndrome; cerebral palsy; myelomeningocele; neuromuscular; scoliosis; spinal cord injury; spinal muscular atrophy; syringomyelia

Document Type: Research Article

Affiliations: Department of Orthopaedic Surgery, The Johns Hopkins University/Johns Hopkins Bayview Medical Center, Baltimore, MD

Publication date: September 1, 2016

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