Impact of Intermittent Positive Pressure Breathing on Thoracic Growth in Children With Type 1 Spinal Muscular Atrophy

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INTRODUCTION: The emergence of new disease-modifying treatments for type I spinal muscular atrophy (SMA I) has led to a paradigm shift in the respiratory management of these patients. Accompanying pulmonary and thoracic growth appears to be a key factor in their morbidity and mortality. The role of Intermittent Positive Pressure Breathing (IPPB) on thoracic growth has been described in the literature, but there are few studies available to objectively assess its impact. STUDY OBJECTIVE: To assess the impact of using IPPB on thoracic growth in children with SMA I. MATERIAL/METHOD: In this bicentric retrospective study, the thoracic circumference-to-head circumference ratio (TC/HC) was compared at three time points: 4 months before the initiation of IPPB, at the start of IPPB, and 4 months after initiation. RESULTS: Seven children (3 girls and 4 boys) with SMA I with a median (min-max) age of 4.8 (2.6-8.2) years old were included in this study. A statistically significant increase was observed in the mean change in TC/HC between the pre-IPPB and post-IPPB periods (-0.05 ± 0.03 vs. +0.06 ± 0.07, p = 0.04). CONCLUSION: In this study, IPPB was associated with a significant increase in TC/HC in children with SMA I. Further large-scale studies are warranted to better characterize the extent and clinical significance of this effect.

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