Primary ciliary dyskinesia is a rare, autosomal recessive disease, with diminished ciliary motility resulting in chronic disease of the upper and lower airway. Due to its low prevalence, there are few studies with significant numbers of cases, and a lack of guidelines for the management of this group of patients. Most patients present chronic rhinosinusitis at diagnosis and while there is improvement with saline irrigations and medical treatment, its effects are increased after endoscopic sinus surgery. Despite this improvement, the high risk of early recurrence, forces us to reserve it for cases with severe nasal obstruction. The aim of this study is to present a literature review on this topic.

Purpose: Melanomas are malignant tumors that have a poor prognosis and surgical treatment is the best option when they are resectable.In this study we determined the incidence and survival what we achieved in our population of patients treated for melanomas of the nasal cavity and paranasal sinuses. Material and methods:The medical records of all patients diagnosed with rhinosinusal malignant tumors at the hospital Italiano of Buenos Aires from March 1999 to December 2020 were reviewed.Patients with melanomas were selected y and we determine its incidence within malignant rhinosinusal tumors. Clinical characteristics, location, type of treatment indicated, and results were analyzed.Results: Eight patients had melanomas, five located in the paranasal sinuses, two in the nasal cavity and one in nasal cavity and maxillary sinus.The incidence of melanomas in our population of patients with rhinosinusal malignant tumors was 11.11%.Three patients were treated with surgery and radiotherapy and three with surgery only.Two were treated with palliative criteria.In the six patients treated with curative intent, the local control and specific survival were 83.33%, and 66.66% respectively.The average follow up time was 30.99 months.Conclusions: The incidence of melanomas in our population of patients with rhinosinusal malignancies was 11.11%.In patients treated with curative intention, the local control rate was 83.33%, and disease-free survival was 66.66%.The more frequently failure pattern was the appearance of distant metastases.Endonasal surgery with endoscopes alone or associated with an external approach facilitated oncological resection.

Background: In this study, we describe our experience in the treatment of malignant rhinosinusal tumors and establish factors related to their prognosis.Objectives: To determine the local control and the survival rates in patients with malignant tumors of the paranasal sinuses and nasal cavity treated by endonasal endoscopic surgery. Design: Descriptive and prospective.Methods: All patients who had malignant tumors of the nasal cavity and paranasal sinuses who were treated by an endonasal, cranionasal or combined endonasal an external approach in the ENT Department of the Italian Hospital of Buenos Aires from March 2003 to August 2019 were selected. Patient data were collected prospectively and updated by reviewing electronic medical records.Results: Thirty patients were treated, 10 had tumors located in the nasal cavity. No patient had local recurrence.Twenty had malignant tumors originated in the paranasal sinuses. Twelve endonasal approaches were performed with endoscopes only, 9 had adjuvant treatment with radiotherapy and chemoradiotherapy. The local control rate was 50% (6/12). Six cranionasal approaches were made: All had adjuvant treatment with radiotherapy or chemoradiotherapy and two others were treated by an endonasal and external approach, and postoperative radiation therapy.The local control rate in patients treated with cranionasal and combined approaches was 75%. Conclusions:The rate of local control and survival that we obtained in patients treated for rhinosinusal malignant tumors by endonasal endoscopic surgery was 73.33%.Tumors with greater extension and infiltration, with higher T staging, localization in the paranasal sinuses and unfavorable histologies such as undifferentiated carcinoma, had a greater need for neoadjuvant or adjuvant treatment and worse prognosis than those small, T1-2, and localized neoplasms in the nasal cavity.