2018 Volume 54 Issue 2 Pages 231-235
Purpose: We evaluated the benefit of early infantile laryngotracheal separation in spinal muscular atrophy (SMA) type I following diagnosis.
Methods: We examined 5 patients (3 males, 2 females) with SMA type I, who underwent laryngotracheal separation between April 2008 and April 2015, with all operations performed using a modified Lindeman technique in infancy. We retrospectively examined the background, status at time of surgery, and postoperative developments.
Results: The mean monthly incidence rate was 1.6 ± 1.0 months. Four patients were diagnosed as having SMA type I by genetic analysis, and 1 patient was diagnosed on the basis of family history and clinical developments. All the patients were admitted with the visualization of atelectasis upon imaging before surgery. The mean age was 5.6 ± 1.0 months and the mean weight was 6.4 ± 1.4 kg at the time of surgery. All the patients had no postoperative complications, and the frequency of their hospitalization due to respiratory infection was 0.77 times/year on average. Currently, all the patients are continuing with home management. In addition, 2 patients use a communication device in order to interact with their surroundings.
Conclusions: We were able to safely perform laryngotracheal separation in infancy for 5 patients with SMA type I, as well as prevent lung degradation due to repeated respiratory infections. It is considered beneficial for patients and families living at home to undergo laryngotracheal separation in infancy for SMA type I.
