Results
We enrolled 23 SMA children. Their age range between 6 months and 18 years. Eight of the 23 (35%) were affected by type 2 SMA and the other 15 (65%) by type 1. In agreement with the Dubowitz decimal classification [12], 2 (13%) of the 15 type 1 SMA patients were classified as 1.1, 8 (53%) as 1.5 and 5 (33%) as 1.9.
Three of the 23 (13%) had no scoliosis (Cobb angle <20°), 12/23 (52%) were classified as mild or moderate (Cobb angle between 20° and 50°), while 8/23 (35%) showed severe scoliosis ( Cobb angle > 50°) complicated by different degree of kyphosis or rotoscoliosis (Tab. 1).
Five of the 23 (22%) were on spontaneous breathing, 12/23 (52%) used non-invasive ventilation less than 16 hours/daily, none of them used non-invasive ventilation more than 16 hours/daily and 6/23 (26%) were mechanically ventilated through tracheostomy (Tab. 1).