Background: Nasal chondromesenchymal hamartomas are benign, rare nasal tumors associated with DICER1 pathogenic germline variation. They can be locally destructive and recurrent if not completely resected.
Methodology: In this single-center, case-control study, otorhinolaryngology evaluations and review of systems questionnaires of DICER1-carriers and controls enrolled in the DICER1 Natural History Study at the National Cancer Institute were collected. Review of these medical records were analyzed to determine if DICER1-carriers experienced different sinonasal clinical manifestations compared to controls. Additionally, the number of diagnoses of nasal chondromesenchymal hamartoma cases in the NCI DICER1 study was compared against the total person years of observation of DICER1-carriers in the study to determine the total number of cases per person-years of observation. Lastly, both the NCI DICER1 study and the International Pleuropulmonary Blastoma/DICER1 Registry were queried for unpublished cases of nasal chondromesenchymal hamartomas.
Results: There were no clinical differences in sinonasal symptomatology between DICER1-carriers and control patients seen in the ENT clinic. We observed of two cases of nasal chondromesenchymal hamartoma in a total of 555 person-years of monitoring DICER1-carriers. We include six unpublished nasal chondromesenchymal hamartoma cases. When combined with a comprehensive literature review, 38% of nasal chondromesenchymal hamartoma cases had at least one additional DICER1-associated tumor and 24% of the NCMH were found in the ethmoid sinus, the most commonly involved paranasal sinus.
Conclusions: We quantify the risk of developing nasal chondromesenchymal hamartomas in our cohort of 236 DICER1-carriers, report six unpublished cases, and provide an updated review of the literature.
Introduction: Intranasal corticosteroids sprays (INCS) are first line treatment for allergic rhinitis and are frequently used for chronic rhinosinusitis. Improperly aiming INCS increases the risk of epistaxis and may decrease the efficacy of the medication. The goal of this study was to determine how patients position INCS for drug delivery and if verbal or written instructions improve their positioning.
Methods: Patients in rhinology clinics were photographed while administering a generic spray bottle. The angle of the spray bottle relative to the patients' head and a fixed background was determined.
Results: A total of 46 participants were included. The average spray angle for the right naris was 10.1° towards the septum and 67.2° below the Frankfurt Horizontal plane. The average spray angle for the left naris was 4.5° towards the septum and 62.2° below the Frankfurt horizontal plane. The angle of the spray bottle ranged from 50° toward the septum to 43° away from the septum. Only 8 patients aimed away from the septum for both nares. Patients who recalled receiving verbal and written instructions aimed the INCS bottle at the lateral wall and inferior turbinate in contrast to patients who only received one form of instruction or no instructions.
Conclusions: Most patients (83%) incorrectly aim INCS when compared to current guidelines. There was statistically significant improvement in the positioning of patients who reported receiving both verbal and written instruction; however, this study highlights a greater need for patient education.