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. 2024 Nov;70(5):1000-1009.
doi: 10.1002/mus.28238. Epub 2024 Sep 4.

Secondary outcomes of scoliosis surgery in disease-modifying treatment-naïve patients with spinal muscular atrophy type 2 and nonambulant type 3

Chiara Brusa  1 , Giovanni Baranello  1 , Deborah Ridout  2 , Julie de Graaf  1 , Adnan Y Manzur  3 , Pinki Munot  3 , Anna Sarkozy  1 , Marion Main  3 , Evelin Milev  1 , Mario Iodice  1 , Danielle Ramsey  1 , Stewart Tucker  4 , Tom Ember  4 , Ramesh Nadarajah  4 , Francesco Muntoni  1 , Mariacristina Scoto  1
Affiliations

Secondary outcomes of scoliosis surgery in disease-modifying treatment-naïve patients with spinal muscular atrophy type 2 and nonambulant type 3

Chiara Brusa et al. Muscle Nerve. 2024 Nov.

Abstract

Introduction/aims: Available studies on scoliosis surgery in spinal muscular atrophy (SMA) have focused on the primary outcome of the procedure-the correction of the curve-whereas research focusing on secondary outcomes is scarce. We aimed to investigate postsurgical changes in respiratory function, motor function, weight, pain, and satisfaction.

Methods: We retrospectively reviewed the clinical notes of 32 disease-modifying treatment-naïve patients (26 SMA2, 6 nonambulant SMA3). We also performed investigator-developed phone interviews and conducted a focus group with families on postsurgical satisfaction.

Results: Mean annual rate of forced vital capacity percent decline improved in SMA2: -3.2% postsurgery versus -6.9% presurgery (p < .001), with similar trajectories in SMA3. Gross motor functional scores (Hammersmith Functional Motor Scale) available in 12/32 dropped immediately after surgery: median loss of 6.5 points, with relatively spared upper limb function. Weight z-scores postsurgery dropped in 16/32, requiring food supplements (5/16); one/16 lost >5% of total weight requiring gastrostomy. Postsurgical pain was frequently reported, especially hip pain (13/32). Overall, 10/10 patients/parents participating in the phone interview rated the procedure as very successful for posture and physical appearance. Nonetheless, 7/10 reported postsurgical pain, reduced mobility, and unmet care needs. The seven patients/parents attending the focus group highlighted lack of intensive physiotherapy programs, occupational therapy assistance, and psychological support as postsurgical unmet care needs.

Discussion: This study reports a positive impact of scoliosis surgery on respiratory function and overall satisfaction with posture and physical appearance. The observed negative impact on the other outcomes highlights the importance of multidisciplinary approaches to improve postoperative management.

Keywords: postsurgical motor function; postsurgical pain; postsurgical respiratory function; postsurgical weight gain; scoliosis surgery; spinal muscular atrophy.

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References

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