To date, no study precisely described ear, nose and throat (ENT) disease in adults with primary ciliary dyskinesia (PCD) and its relationship with ciliary function/ultrastructure. A retrospective study of standardized ENT data (exam, audiogram, sinus Computed tomography (CT), and bacteriology) was conducted in 64 adults with confirmed PCD who were followed in two ENT reference centers. Rhinorrhoea and hearing loss were the main symptoms. Symptom scores were higher in older patients. Nasal endoscopy was abnormal in all patients except one, showing nasal polyps in one-third of the patients and stagnant nasal mucus secretions in 87.5% of the patients. Sinus CT opacities were mainly incomplete and showed one-third of the patients with sinus hypoplasia and/or agenesis. Middle meatus mainly grew Haemophilus influenzae, Streptoccocus pneumoniae and Pseudomonas aeruginosa. Otitis media with effusion (OME), which is constant in childhood, was diagnosed in less than one-quarter of the patients. In two-thirds of the patients, audiogram showed hearing loss that was sensorineural in half of the patients. ENT disease severity was not correlated with ciliary function and ultrastructure, but the presence of OME was significantly associated with a forced expiratory volume (FEV1) < 70%. Rhinosinusitis is the most common clinical feature of PCD in adults, while OME is less frequent. The presence of active OME in adults with PCD could be a severity marker of lung function and lead to closer monitoring.
This study demonstrates the high recurrence rate of mucocele, particularly in multi-operated patients with chronic sinusitis. Long-term, regular, clinical and radiological follow-up is necessary to detect asymptomatic lesions prior to the onset of complications.
Chronic rhinosinusitis is the foremost manifestation in adult patients with primary ciliary dyskinesia (PCD). We present a retrospective series of 41 adult patients with a confirmed diagnosis of PCD followed in our reference centers. As part of the diagnostic work up in our centers, sinus computed tomography scans (CTs) are systematically performed. All patients also undergo a sampling of purulent secretions sampled from the middle meatus under endoscopic view for bacteriological analysis. In our series, CT opacities were consistent in all the patients, as well as mainly partial and located in ethmoid cells (100% of patients) and in maxillary sinuses (85.4% of patients), and stayed stable over time. In the 31 patients who had purulent secretions, bacteriological culture showed at least one bacterium in 83.9% (n = 26). There was no significant difference in positive cultures for Pseudomonas aeruginosa in patients >40 years old versus those <40 (p = 0.17; Fisher). Surgical management was performed in only 19% of patients in order to improve sinonasal mechanical drainage. Our data support the hypothesis that the sinuses can be considered as a bacterial reservoir. From this retrospective study, we have introduced several changes into our routine clinical practice in our reference centers. Based on our analyses, medical and surgical treatments benefit from incorporating bacteriological information and sinonasal symptoms much more than CT scan evaluation alone. All patients now undergo systematically an annual simultaneous bacteriological sampling of the middle meatus and sputum to follow the relationship between ENT and lung disease and to help to antibiotic therapy strategy.
Nasal valve collapse is a dynamic abnormality that is currently diagnosed purely on the basis of clinical features and thus subject to certain interpretation. The aim of this study was to develop a new and reliable functional test to objectively characterize nasal valve collapse. This was an observational prospective study including consecutive patients referred to our center for exploration of chronic nasal congestion. The patients were classified into two groups according to their symptoms and clinical abnormalities: the nasal valve collapse (NV+) group when nasal valve collapse was clinically detected during moderate forced inspiration and/or when the feeling of nasal congestion improved during passive nasal lateral cartilage abduction (n = 32); and the no-nasal valve collapse (NV-) group for the others (n = 23). All patients underwent nasal functional tests (posterior rhinomanometry and acoustic rhinometry) before and after topical nasal decongestion. We compared the difference between the pressure flow of the inspiratory and expiratory phases during posterior rhinomanometry [flow rate inspiratory-expiratory difference (FRIED) test] between the two groups. The difference between the absolute value of inspiratory and expiratory flow was significantly higher in the NV+ group than in the NV- group both before and after topical decongestion. The cutoff value for the FRIED test was -0.008 l/s with a good sensitivity (82%) and a specificity of 59%. We suggest that the FRIED test constitutes an objective and easy-to-apply technique to diagnose nasal valve collapse in daily practice.
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