Harold J Farber1, William A Phillips2, Kenneth L Kocab3, Darrell S Hanson4, John A Heydemann2, Benny T Dahl2, Elizabeth T Spoede5, Larry S Jefferson1. 1. Section of Pulmonology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. 2. Department of Orthopedic Surgery, Texas Children's Hospital Orthopedic Surgery Service, Baylor College of Medicine, Houston, Texas. 3. Outcomes and Impact Service, Texas Children's Hospital, Houston, Texas. 4. Department of Orthopedic Surgery, Houston Methodist Hospital, Texas Children's Hospital Orthopedic Surgery Service, Houston, Texas. 5. Food and Nutrition Services, Texas Children's Hospital, Houston, Texas.
Abstract
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.
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 atrophypatients, a loss of 0.36 L for the Duchenne muscular dystrophypatients, and a gain of 0.23 L for the merosin deficientpatients. The difference between spinal muscular atrophy and merosin deficientpatients 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.
Authors: Federica Trucco; Deborah Ridout; Mariacristina Scoto; Giorgia Coratti; Marion L Main; Robert Muni Lofra; Anna G Mayhew; Jacqueline Montes; Marika Pane; Valeria Sansone; Emilio Albamonte; Adele D'Amico; Enrico Bertini; Sonia Messina; Claudio Bruno; Deepak Parasuraman; Anne-Marie Childs; Vasantha Gowda; Tracey Willis; Min Ong; Chiara Marini-Bettolo; Darryl C De Vivo; Basil T Darras; John Day; Elizabeth A Kichula; Oscar H Mayer; Aledie A Navas Nazario; Richard S Finkel; Eugenio Mercuri; Francesco Muntoni Journal: Neurology Date: 2020-10-16 Impact factor: 9.910
Authors: Esther S Veldhoen; Anneloes de Vries; Tom P C Schlosser; Moyo C Kruyt; Ruben P A van Eijk; Joyce M Tersmette; Erik H Hulzebos; Ludo W van der Pol; Roelie M Wösten-van Asperen; Cornelis K van der Ent Journal: Pediatr Pulmonol Date: 2022-02-24