Choi Young-Ah, Suh Dong In, Chae Jong-Hee, Shin Hyung-Ik
Department of Rehabilitation Medicine, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Republic of Korea.
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Republic of Korea.
Int J Pediatr Otorhinolaryngol. 2020 Mar;130:109818. doi: 10.1016/j.ijporl.2019.109818. Epub 2019 Dec 18.
This study aimed to elucidate the change in progressive swallowing dysfunction from birth up to 2 years of age to provide clinical insights into the management of swallowing difficulty in patients with spinal muscular atrophy (SMA) type I.
Data of 11 patients with SMA type I were retrospectively reviewed. The Neuromuscular Disease Swallowing Status Scale (NdSSS) scores and videofluoroscopic swallowing study (VFSS) were used.
Swallowing function deteriorated in patients with SMA type I at an approximate age of 6 months. Tube feeding was initiated at the median age of 6 months (interquartile range, 3-7 months). The transition period for switching the feeding route from totally oral to tube feeding varied widely among patients (5-12 months). In four patients, aspiration was observed in VFSS, even when nutrition was provided orally. In two patients, the evidence of laryngeal aspiration was obtained via the VFSS during the very early stages of the disease at 3 and 4 months. Conversely, in one patient, total oral feeding was maintained for up to 12 months, and evidence of aspiration was not observed in the VFSS.
An individualized approach is essential, as the timeline of deterioration of swallowing function varies widely in patients with SMA type I.
本研究旨在阐明从出生到2岁进行性吞咽功能障碍的变化，为I型脊髓性肌萎缩症（SMA）患者吞咽困难的管理提供临床见解。
回顾性分析11例I型SMA患者的数据。使用神经肌肉疾病吞咽状态量表（NdSSS）评分和电视荧光吞咽造影检查（VFSS）。
I型SMA患者在大约6个月大时吞咽功能恶化。管饲在6个月的中位年龄开始（四分位间距，3 - 7个月）。从完全经口喂养转换到管饲的过渡期在患者之间差异很大（5 - 12个月）。在4例患者中，即使经口提供营养，VFSS检查中也观察到误吸。在2例患者中，在疾病早期3个月和4个月时通过VFSS获得了喉误吸的证据。相反，在1例患者中，完全经口喂养维持了长达12个月，VFSS检查中未观察到误吸证据。
由于I型SMA患者吞咽功能恶化的时间线差异很大，个体化方法至关重要。
