Sinus Development and Pneumatization in a Primary Ciliary Dyskinesia Cohort.

BACKGROUND: Primary ciliary dyskinesia (PCD) is a genetically diverse disease which causes impaired mucociliary clearance, and results in pulmonary, otologic, and rhinologic disease in affected patients. Genetic mutations in multiple genes impair the ability of patients to clear mucous from the lungs, middle ear, and sinonasal cavity and lead to chronic pulmonary and sinonasal symptoms. METHODS: We identified 17 PCD patients who had available CT scans. Volumes for bilateral maxillary, sphenoid, and frontal sinuses were calculated. A control population of patients who had preoperative CT scans for endoscopic endonasal resection of skull base pathology without sinonasal cavity involvement was also identified. RESULTS: The mean age of PCD was 33 and ranged from 13 to 54 years. Patients were age- and gender-matched to a control group that underwent resection of anterior skull-base tumors and had a mean age of 35 that ranged between 17-53 years old. The volumes for all thee sinus cavities were significantly smaller (p < 0.007) compared to the control population. The average Lund-Mackay score was 10.6 in the PCD cohort (range 6-16) in comparison to an average of 0.7 in the control cohort (range 0-2). CONCLUSIONS: Overall sinus volumes were smaller in patients with PCD compared to our control population. Future studies will be aimed at understanding defects in sinus development as a function of specific genetic mutations in PCD patients. Ultimately, a better understanding of the underlying pathophysiology of PCD will allow us to identify the optimal treatment practices for this unique patient group.

A Pilot Comparison between Caregiver's and Patient's Perceived Quality of Life in Chronic Rhinosinusitis.

Introduction Chronic rhinosinusitis negatively impacts a patient's quality of life, but current studies only address the patient's perception of their disease. Caregivers living with the patient may have an alternative perception of the severity of the quality of life disturbance that patient's experience with chronic rhinosinusitis. Methods This was a prospective cohort study that enrolled patients with a confirmed chronic rhinosinusitis diagnosis who presented to clinic with a caregiver. At the initial visit, patients completed a Rhinosinusitis Disability Index. The caregiver completed a Rhinosinusitis Disability Index based on their perception of the patient's symptoms in addition to a Modified Caregiver Strain Index and a Short Form-36 to assess caregiving strain and overall health, respectively. Statistical analyses were performed with significance defined as P < 0.05 a priori. Results A total of 44 total subjects (22 patient and caregiver pairs) were enrolled. Patients reported a total Rhinosinusitis Disability Index of 36.8 (confidence interval: 26.9, 46.6), and caregivers reported a total Rhinosinusitis Disability Index of 50.4 [confidence interval: 38.8, 61.9] ( P = 0.02). The principal differences between patient and caregiver scores were noted in the emotional and physical domains ( P = 0.01 and P = 0.05, respectively). Only the functional domain was not statistically different ( P = 0.20). The patient's total Rhinosinusitis Disability Index is positively correlated with the caregiver's total Modified Caregiver Strain Index with a spearman coefficient of .60 (p ≤ 0.005). Conclusions Caregivers experience greater strain as the patient's quality of life declines. Caregivers perceive patients to have worse quality of life than patients report. Based on these data, caregivers may provide additional insight to the quality of life disturbance of chronic rhinosinusitis. In addition, the societal impact of chronic rhinosinusitis may be underestimated.

Stricture location predicts swallowing outcomes following endoscopic rendezvous procedures.

OBJECTIVES/HYPOTHESIS: Complete pharyngoesophageal strictures may be encountered by the otolaryngologist as a consequence of radiation/chemoradiotherapy therapies for head and neck cancer. A combined anterograde and retrograde dilation procedure (rendezvous procedure) has proven to be a useful surgical intervention in these cases. We assess the long-term swallowing outcomes of this patient cohort including gastrostomy tube (G-tube) reliance, swallowing quality of life, and variables that contribute to improved swallowing outcomes. STUDY DESIGN: Retrospective chart review. METHODS: A retrospective chart review of 18 consecutive patients treated with rendezvous procedures between April 2007 and May 2015 was carried out. Data were collected from chart review and follow-up telephone calls including demographics, surgical/postoperative course details, and Eating Assessment Tool (EAT-10) (swallowing quality of life) scores. RESULTS: The completion rate of the procedure was 83% (15 completed/3 procedures aborted). Average follow-up was 22 months. Thirteen of 15 (86.7%) achieved an oral diet, and 7/15 (46.7%) had their G-tube removed. G-tube-independent (GTI) patients had an average stricture length of 2.33 cm and an average distance from the incisors of 17.4 cm compared to G-tube dependent-(GTD) patients who had an average stricture length of 2.63 cm and 14.6 cm mean distance from the incisors (P = .66 and .0343, respectively). Final EAT-10 scores averaged 20.1 in GTI patients and 33.8 in GTD patients (P = .022). Stricture/incisor distance and EAT-10 scores demonstrated a moderate to strong negative correlation (r = -0.67). CONCLUSIONS: Following the endoscopic rendezvous procedure, swallowing outcomes and G-tube status is related to the distance of the stricture from the incisors. LEVEL OF EVIDENCE: 2b Laryngoscope, 127:1388-1391, 2017.