誤嚥防止手術として声門下喉頭閉鎖術を選択したRett症候群の一例

Various types of aspiration prevention surgeries have been developed, and this surgery is considered to be indicated in children with recurrent aspiration pneumonia. We herein report a pediatric case of Rett syndrome in which subglottic laryngeal closure was performed to prevent aspiration. A 15-year-old girl had been diagnosed with Rett syndrome at 4 years old and had undergone multiple previous surgeries for scoliosis. She had been suffering from recurrent aspiration pneumonia since 12 years old and undergone ventilator management 3 times. The patient was re-hospitalized for aspiration pneumonia, and tracheostomy was considered due to long-term intubation management. Since her family desired her to have safe oral intake even at the expense of her speech function, surgical treatment to prevent aspiration was decided. After the induction of general anesthesia, we checked the glottis and found bilateral vocal cord inflammation and granulation of the posterior glottis due to prolonged intubation, suggesting that glottis closure was not indicated. In addition, because of thoracic deformity, a high permanent tracheostomy hole was recommended in order to reduce the risk of brachiocephalic artery fistula due to cannula retention. Therefore, subglottic laryngeal closure was performed at the level of the cricoid cartilage. A postoperative videofluorographic swallowing study showed no anastomotic leakage, and she regained her oral intake ability. At six months after the surgery, the patient has continued oral intake and shown no deterioration in her respiratory condition.