Tratamiento de la disfagia en la distrofia muscular oculofaríngea. Revisión sistemática

Abstract

Introduction and objective: Dysphagia has been reported to be a concern in adults with oculopharyngeal muscular dystrophy (OPMD). This problem can result in several complications including aspiration pneumonia, reduced quality of life and an increase in mortality rate. The objective was to evaluation and comparison of the different current treatments available. Method: We performed a systematic review in the databases PubMed, EMBASE, Cochrane Library and Scielo published in the period 1990-2020, selecting those articles in wich the dysphagia in OPMD is treated. Results: We include 9 articles with a total of 164 with this disease, in which surgical myotomy of the upper esophageal sphincter was the most frequently performed procedure in 6 series with 117 patients, followed by endoscopic dilatation and botulinum toxin. The improvement in the 9 series is around 77, 3% and if we consider surgical myotomies is 84, 66%. The incidence of complications is low, we do not find associated mortality in any of the series. Discussion and conclusions: The priority of patient’s treatment is determining the degree and severity of the dysphagia that is the main factor determining prognosis.  Upper esophageal sphincter myotomy is the most effective and widely used of all the possibilities, with high rate of success and low rate of complications.