ミトコンドリア脳筋症に伴う嚥下障害に対し喉頭気管分離術を施行した1例

Abstract

Surgical treatment is sometimes necessary for patients with degenerative neuron disease that experience repeated aspiration pneumonia. Mitochondrial encephalomyopathy with severe dysphagia has rarely been reported. We performed laryngotracheal separation for a patient with mitochondrial encephalomyopathy who had been suffering from severe dysphagia and repeated aspiration pneumonia. The patient (a 31-year-old woman) complained of repeated convulsive and stroke-like episodes since 11 years of age. Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) was diagnosed by clinical findings and muscle biopsy revealed ragged red fibers as well as mitochondrial DNA mutation (A3243G). A tracheostomy and a gastrostomy had been performed. but aspiration pneumonia could not be prevented due to the patient's continuous aspiration of saliva. She had severe complications such as cardiac and renal dysfunction. We evaluated her swallowing function using videofluorography and videoendoscopy. No laryngeal paralysis was found, but there was severe pooling of saliva above the hypolarynx, which indicated a significant degree of aspiration. In August of 2003, a laryngotracheal separation was performed. She no longer suffered from further episodes of aspiration pneumonia and experienced an overall satisfactory improvement of her general condition and an increase in her quality of life. We reached the conclusion that laryngotracheal separation is a highly beneficial procedure for patients with dysphagia due to progressive degenerative neuron diseases such as mitochondrial encephalomyopathy.