P.227Secondary clinical outcomes of spinal surgery and satisfaction in patients with spinal muscular atrophy (SMA) II and non-ambulant III

Abstract

Scoliosis is a highly prevalent feature of SMA and surgery is frequently required. Studies in small cohorts have reported a positive impact of spinal surgery on respiratory function, while limited data are available on motor function, weight gain, post-surgical skeletal pain and satisfaction. We retrospectively reviewed the notes of 33 patients (26 SMA II, 7 non-ambulant SMA III) who successfully underwent spinal fusion (25), magnetic (4) or traditional (4) growth rods followed by final growth spinal fusion (3) at Great Ormond Street Hospital-London. Median age at surgery was 10.9 years (4.28–16.72), median pre-operative Cobb angle 70 degrees (35–89), median follow up before and after surgery 3.9 (0.9–12.3) and 3.7 (0.4–10.5) years respectively. Mean annual rate of Forced Vital Capacity % decline improved after surgery in SMA II: -2.8 versus -7.4 (p<0.001). Similar trajectories were observed in SMA III. At first post-surgery assessment a median decline of 4.5 points (0-14) was observed on the Hammersmith Functional Motor Scale while the Revised Upper Limb Module's scores showed a less progressive deterioration. A temporary negative deviation from previous weight curve was observed in 17 patients requiring food supplements (5); one/4 type II with significant weight loss (>5% of total weight) needed gastrostomy. Hip pain was the most common skeletal pain documented (13/33) requiring painkillers (8), intra-articular steroids (1) or surgery (1). Ten patients/parents participated in a phone interview developed by the neuromuscular team on satisfaction. Nine/10 reported major improvements in posture, physical appearance, self-image and all rated the surgical procedure as very successful. However, 7/10 did not report significant improvements in quality of life due to reduced mobility and increased unmet care needs. This ongoing study expands the knowledge on outcomes after spinal surgery in SMA and highlights the importance of a careful post-operative multidisciplinary approach.