The physical examination revealed a nonobese man of short stature (157 cm, body mass index 22.3 kg/m2) with a deep, slighdy husky voice. His tongue, uvula, and soft palate were enlarged. Indirect laryngoscopic examination revealed thickening of the epiglottis and true vocal cords with good motility. The left false vocal cord was thickened and overhanging. The right false vocal cord appeared normal. His neck was short and there were noisy, coarse upper airway sounds although there was no stridor. His chest expansion was poor and the lung fields were clear to auscultation and percussion. Cardiovascular exam revealed a grade 2 systolic ejection murmur at the left sternal border. The abdominal exam was normal and there was no evidence of cyanosis, clubbing, or edema of the extremities. buy levaquin online
An MRI scan of the neck and upper mediastinum (Fig 1) showed marked compromise of the nasopharyngeal, oropalatal, oropharyngeal, and tracheal airway due to a combination of bony structural deformities as well as increased soft tissues due to glycosaminogly-can deposition and probable tracheomalacia. Pulmonary (unction tests (Table 1) revealed a mild obstructive defect with equivocal response to inhaled bronchodilators and a moderate restrictive defect. The MW was decreased and the flow-volume curves were consistent with an extrathoracic upper airway obstruction. The baseline arterial blood gas values showed pH, 7.37; Pco2, 51; and Po2, 80 on room air at rest and pH, 7.46; Pco2, 39; and Po2, 98 after maximum voluntary hyperventilation maneuver.
Figure 1. Sagittal T,-weighted MR scan of the neck showing marked narrowing of the upper airway including the trachea.
Table I—Pulmonary Function Tests