BACKGROUND: Spinal muscular atrophy (SMA) affects respiratory muscles, which in addition to progressive scoliosis leads to respiratory impairment. Children with developing scoliosis are usually treated with spinal bracing to delay the progress. AIMS: To assess the impact of body position and application of spinal bracing on lung function during tidal breathing in children with SMA. METHODS: Lung function was determined by tidal flow volume loops and passive respiratory mechanics (single breath occlusion technique) in all eight children in southern Norway with SMA type I and II, in both the sitting and supine position. Additional measurements were performed with and without bracing in five children. Muscle strength was assessed by the Brooks scale. RESULTS: Tidal expiratory volume (V(E)) and compliance of the respiratory system (CRS) tended to be higher in the sitting compared to the supine position, but this was not statistically significant. However, applying bracing in the sitting position significantly reduced V(E). The highest values of CRS and V(E) were found in the sitting position without bracing. CONCLUSION: Impairment of tidal respiration must be considered when applying spinal bracing in very young children developing scoliosis with SMA.
BACKGROUND:Spinal muscular atrophy (SMA) affects respiratory muscles, which in addition to progressive scoliosis leads to respiratory impairment. Children with developing scoliosis are usually treated with spinal bracing to delay the progress. AIMS: To assess the impact of body position and application of spinal bracing on lung function during tidal breathing in children with SMA. METHODS: Lung function was determined by tidal flow volume loops and passive respiratory mechanics (single breath occlusion technique) in all eight children in southern Norway with SMA type I and II, in both the sitting and supine position. Additional measurements were performed with and without bracing in five children. Muscle strength was assessed by the Brooks scale. RESULTS: Tidal expiratory volume (V(E)) and compliance of the respiratory system (CRS) tended to be higher in the sitting compared to the supine position, but this was not statistically significant. However, applying bracing in the sitting position significantly reduced V(E). The highest values of CRS and V(E) were found in the sitting position without bracing. CONCLUSION: Impairment of tidal respiration must be considered when applying spinal bracing in very young children developing scoliosis with SMA.
Authors: Rachel L Lenhart; Sylvester Youlo; Mary K Schroth; Kenneth J Noonan; James McCarthy; David Mann; Scott Hetzel; Sarah A Sund; Matthew A Halanski Journal: J Pediatr Orthop Date: 2017-12 Impact factor: 2.324
Authors: Sebastian Lippross; Antonia Grages; Katja A Lueders; Lena Braunschweig; Friederike Austein; Konstantinos Tsaknakis; Heiko M Lorenz; Anna K Hell Journal: Eur Spine J Date: 2021-02-22 Impact factor: 3.134
Authors: Mg Mullender; Na Blom; M De Kleuver; Jm Fock; Wmgc Hitters; Amc Horemans; Cj Kalkman; Jeh Pruijs; Rr Timmer; Pj Titarsolej; Nc Van Haasteren; Mj Van Tol-de Jager; Aj Van Vught; Bj Van Royen Journal: Scoliosis Date: 2008-09-26