A Review of Surgical Indications for Scoliosis in Duchenne Muscular Dystrophy

Truong, Phong; Kuklova, Kristina; Brush, Natalie

doi:10.31080/asor.2020.03.0214

Act Scie Ortho

2020

DOI: 10.31080/asor.2020.03.0214

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A Review of Surgical Indications for Scoliosis in Duchenne Muscular Dystrophy

Phong Truong¹,

Kristina Kuklova²,

Natalie Brush³

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2023

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Duchenne muscular dystrophy (DMD) is an X-linked inherited disorder affecting 1 in 3600 to 6000 live male births. 1,2 Although initially expected to carry a dismal prognosis, life expectancy has increased over the last few decades because of recent medical advancements and improved patient care. 3 Children with DMD frequently develop spinal deformities in the form of scoliosis or kyphoscoliosis, especially after losing their ability to walk.

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Survival of patients with Duchenne muscular dystrophy who underwent spinal deformity correction

Arshad

2023

Develop Med Child Neuro

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AimWe investigated survival rates after scoliosis correction in individuals with Duchenne muscular dystrophy (DMD) and evaluated factors that can affect them.MethodThis was a retrospective cohort study from 2000 to 2022 with a minimum 2‐year postoperative follow‐up. We reviewed the hospital records/spinal radiographs and analysed data with XLSTAT. Kaplan–Meier and multivariate Cox regression survival analysis was performed.ResultsForty‐three patients had a mean age at surgery of 14 years 5 months. Mean postoperative follow‐up was 10 years 10 months. There was no operative or 30‐day postoperative mortality in this group. Twenty‐four patients died because of cardiorespiratory failure. Median survivorship was 14 years 2 months, with the longest observed survival being 22 years 6 months given the limitation of the length of postoperative follow‐up. The degree of preoperative coronal imbalance and pelvic obliquity, as well as intraoperative blood loss, were factors that significantly affected survival. The impact of preoperative sagittal imbalance and extension of the fusion to the sacrum/pelvis trended towards significance. In contrast, age at surgery, preoperative/postoperative scoliosis, thoracic kyphosis, lumbar lordosis, scoliosis and pelvic obliquity flexibility or correction indices, postoperative coronal/sagittal balance, need of preoperative non‐invasive ventilation, preoperative feeding disorders, development of surgical complications, and length of hospital and intensive care unit stay were exposure variables that did not affect postsurgical survival in patients with DMD.InterpretationSurvival of up to two decades or more was possible among young patients with DMD after scoliosis correction. This was affected by factors that related to disease and deformity severity, as well as surgical morbidity.

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confidence: 99%

“…Duchenne muscular dystrophy (DMD) is an X‐linked inherited disorder affecting 1 in 3600 to 6000 live male births 1,2 . Although initially expected to carry a dismal prognosis, life expectancy has increased over the last few decades because of recent medical advancements and improved patient care 3 .…”

mentioning

confidence: 99%

Survival of patients with Duchenne muscular dystrophy who underwent spinal deformity correction

Arshad

2023

Develop Med Child Neuro

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A Review of Surgical Indications for Scoliosis in Duchenne Muscular Dystrophy

Cited by 1 publication

References 0 publications

Survival of patients with Duchenne muscular dystrophy who underwent spinal deformity correction

Survival of patients with Duchenne muscular dystrophy who underwent spinal deformity correction

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