[A clinical study of laryngotracheal separation in spinal muscular atrophy type I patients].

Abstract

Artificial respiration by tracheostomy is necessary for long-term survival in spinal muscular atrophy (SMA) Type I patients. There are two types of tracheostomy: i )simple tracheostomy and ii) tracheostomy plus aspiration prevention surgery, including laryngotracheal separation. We experienced three cases of SMA type I patients that had good outcomes after undergoing laryngotracheal separation. The patients' ages at onset were 14 days, two months and one and a half months. Laryngotracheal separation was performed at five months, seven months, and 15 years and five months respectively, and the times from diagnosis to surgery were a month, three months and 15 years. The aspiration pneumonia disappeared in all three cases. This study suggests that laryngotracheal separation is an effective surgery to prevent aspiration in SMA type I cases, and also contributes to the improved quality of life of patients and their families.