Impact of scoliosis surgery on pulmonary function in patients with muscular dystrophies and spinal muscular atrophy.

BACKGROUND: Scoliosis is a common complication of severe neuromuscular diseases. The aim of this study is to determine the impact of posterior spinal fusion on pulmonary function parameters in patients with severe neuromuscular disease at our medical center. METHODS: Retrospective chart review of all patients with severe neuromuscular disease who had posterior spinal fusion between 2012 and 2017 at Texas Children's Hospital. Patients with growing rods, brain injury or malformation, and/or spina bifida were excluded. Pulmonary function measures before and after spinal surgery were determined. RESULTS: A total of 20 eligible patients were identified, 7 with Duchenne muscular dystrophy, 6 with spinal muscular atrophy, 3 with merosin deficient muscular dystrophy, 2 with Charcot-Marie-Tooth, 1 with central core disease, and 1 with dystroglycanopathy. The mean change in vital capacity from pre- to postspine surgery was a loss of 0.63 L for the spinal muscular atrophy patients, a loss of 0.36 L for the Duchenne muscular dystrophy patients, and a gain of 0.23 L for the merosin deficient patients. The difference between spinal muscular atrophy and merosin deficient patients was statistically significant (P = .02) CONCLUSION: In this single-center retrospective study, we found that after spine surgery for scoliosis, all patients with spinal muscular atrophy and most patients with Duchenne muscular dystrophy lost vital capacity, while the patients with merosin deficient muscular dystrophy gained vital capacity. These differences were not associated with differences is respiratory strength, body mass index, or surgical outcomes.

Changes in body composition after lung transplantation in children.

BACKGROUND: The evaluation of nutritional status, including body composition measurements, in pediatric patients before and after lung transplant (LTx) can aid in adapting nutrition support and physical rehabilitation programs to meet individual patient needs. The purpose of this retrospective study was to determine the changes in weight, lean body mass (LBM), and body fat (BF) before and after LTx and their association with lung function in pediatric patients. METHODS: Included were 41 LTx patients, aged 3 months to 20.7 years, who had at least 2 body composition measurements determined by dual-energy X-ray absorptiometry (GE Lunar Prodigy, Waukesha, WI) in the first 2 years after LTx were measured pre-LTX and at 12 or 24 months post-LTX, for weight, LBM, and BF. RESULTS: Pre-LTx, 29% of patients had moderate and 12% had severe chronic malnutrition (growth stunting). This compares with 21% of patients being moderately LBM-depleted and 23% being BF-depleted. The weight change at 12 and 24 months was +9.3% (interquartile range, 5.6%-23%) and +4.7% (0.9%-11.6%), respectively; whereas the LBM change at 12 and 24 months was +15.2% (6.8%-17.1%) and +4.2% (-0.6% to 7.7%), respectively. LBM percentiles correlated with pulmonary function tests ( % predicted forced vital capacity [ρ = 0.36, p = 0.001] and forced expiratory volume in 1 second [ρ = 0.265, p = 0.015). CONCLUSIONS: Maximum weight and LBM gain occur at 12 months after LTx, with smaller gains noted at 24 months. Clinicians must look beyond height and weight and evaluate LBM and fat mass in pediatric patients after LTx.