Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis

Abstract

Upper airway obstruction is common in children with mucopolysaccharidosis. The acoustic reflection method is a noninvasive technique that can analyze the caliber of the upper airways. The aim of the study was to evaluate the feasibility of the acoustic reflection method in children with mucopolysaccharidosis, and to compare the characteristics of the upper airways evaluated by the acoustic reflection method in patients with mucopolysaccharidosis to matched healthy counterparts. Open, single center, prospective, study. Accurate acoustic reflection measurements could be obtained in 7 of 10 patients (mean age: 10.4 ± 3.9 years; mucopolysaccharidosis type II (n = 3); type IV (n = 2), type VI (n = 1), and fucosidosis (n = 1)). The mean minimum cross-sectional area was lower in mucopolysaccharidosis patients (1.6 ± 0.3 cm(2) ) as compared to 14 healthy counterparts (1.8 ± 0.3 cm(2); P = 0.03). The mean resistance of the airways was significantly higher in the MPS group (7.9 ± 1.8 cmH(2) O l(-1) sec) as compared to the controls (5.5 ± 1.2 cmH(2) O l(-1) sec; P = 0.006). This study is the first to analyze the upper airways by the noninvasive acoustic reflection method in children with mucopolysaccharidosis. Due to a lack of cooperation, reliable measurements could only be obtained in 70% of a selected group of patients. Children with mucopolysaccharidosis have significant upper airway obstruction as assessed by the reduction of the minimal cross-sectional area of the upper airways and the increase in airway resistance.