第1型脊髓性肌肉萎縮症兒童吞嚥障礙之管理：範疇綜述

// The Management of Swallowing Disorders in Children With Spinal Muscular Atrophy Type 1: A Scoping Review

納入38篇、1,784例SMA1患兒｜以FEES、VFSS與臨床檢查為主，僅有一項兒童SMA驗證工具：口與吞嚥能力量表｜常見吞嚥起始延遲、口部功能障礙、咽殘留與誤吸；重度常置PEG，急需標準化評估及復健／補償介入研究。

// This scoping review described assessment and management of swallowing disorders in children with spinal muscular atrophy Type 1 (SMA1). Following PRISMA-guided searches of four databases, 38 studies (1,784 patients) were included; swallowing was assessed mainly with instrumental tests (FEES, VFSS) and clinical exams, with only one validated pediatric SMA tool identified (Oral and Swallowing Abilities Tool). Common problems were delayed swallow initiation, oral impairment, pharyngeal residue, and aspiration; percutaneous endoscopic gastrostomy was used for severe dysphagia and malnutrition, while compensatory and rehabilitative treatments were rarely reported. The review concludes that standardized assessment approaches and studies evaluating rehabilitative and compensatory interventions are urgently needed.

Published 2026年9月23日