Efficacy of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis.

PURPOSE: Our work aims to add evidence on the effectiveness of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis. MATERIALS AND METHODS: We conducted an observational retrospective cohort study at the Cystic Fibrosis Center of a tertiary care hospital to investigate the effect of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis patients, aged 12 or older. The study's endpoints were the change in the occurrence of acute exacerbations of chronic rhinosinusitis, and the variation of the endoscopic and radiologic findings scored using the Lund-Kennedy endoscopic scale, Lund-Mackay, and modified Lund-Mackay radiologic scales, in patients who underwent both pre-treatment and post-treatment examinations. RESULTS: The study population comprised 136 patients, of which 28 underwent both pre-treatment and post-treatment nasal endoscopy and 15 had pre- and post-treatment CT scans. Elexacaftor-Tezacaftor-Ivacaftor provided a significant improvement in chronic rhinosinusitis. The mean number of acute exacerbations of chronic rhinosinusitis per year in the pre-treatment time was 0.55 versus 0.35 during the treatment (p < 0.0021). The Lund-Kennedy scale had a pre-treatment average score of 4.21 points versus 1.5 points after the start of Elexacaftor-Tezacaftor-Ivacaftor (p < 0.0001). The average Lund-Mackay and modified Lund-Mackay scores in the pre-treatment time were respectively 14.6 and 16.45 points; and after the start of the therapy, they became 5.87 and 6.73 (p < 0.0001). CONCLUSION: Elexacaftor-Tezacaftor-Ivacaftor was associated with fewer acute exacerbations of chronic rhinosinusitis, and a significant improvement of chronic rhinosinusitis evaluated endoscopically and radiologically. To our knowledge, this is the first study investigating the change in the occurrence of acute exacerbation of chronic rhinosinusitis in patients affected by cystic fibrosis in therapy with Elexacaftor-Tezacaftor-Ivacaftor.

Endoscopic endonasal repair of bilateral choanal atresia in a 1200 g preterm infant: Is it feasible? (With video).

INTRODUCTION: Bilateral choanal atresia (CA) is a rare congenital anomaly, that causes neonatal respiratory distress. No consensus exists regarding the most appropriate surgical approach in low-birth weight preterm infants. CASE REPORT: We present (with video) the case of a male born at 29 weeks' gestation, 1200 g, affected by bilateral CA. He was successfully treated with an endoscopic transnasal approach performed on day 4 of life which allowed a very early extubation. Otologic instruments and 2.7 mm wide endoscope were used to be able to operate in very narrow nasal cavities. Thulium LASER® was used to limit bleeding. At the follow-up visit, four months after surgery, the neochoana was widely patent. DISCUSSION: In low-birth weight preterm infants, endoscopic transnasal surgery (ETS) may be considered technically not feasible or with a high risk of early restenosis. With early ETS, we were able to avoid the morbidity of a prolonged intubation and sedation. CONCLUSION: Bilateral CA is a life threatening and challenging clinical entity, particularly difficult to treat in low-birth preterm infants. Early surgery to reduce intubation and sedation is preferable, but requires experienced teams with a surgical technique and instruments tailored to the narrowness of the nasal cavity.

3D exoscopic surgery of lateral skull base.

PURPOSE: The aim of the study is to assess whether the 3D exoscopic surgery technique could be used in lateral skull base surgery and if it could ultimately replace the microscope in the future. METHODS: This is a retrospective study in which were included 24 patients affected by lateral skull base pathologies, who underwent surgery using the 3D exoscope or the operative microscope at the Department of Otolaryngology-Head and Neck Surgery at the University Hospital of Verona. The exoscope and microscope groups each included 12 cases. The feasibility of all the surgical steps solely using the 3D exoscope was evaluated. The exoscope group and microscope group were compared taking into account the following factors: time of the surgery, facial and hearing functions outcomes, as well as the intraoperative and postoperative complications. RESULTS: No intraoperative complication occurred during all the procedures. Postoperatively, only one minor complication emerged. The average operative time was 289 in the exoscope group and 313 min in the microscope one. No significant statistical differences were identified between the two groups (p > 0.05). The facial and hearing function outcomes were fully comparable. CONCLUSION: Our experience demonstrated that the exclusive use of the 3D exoscope, as that of the traditional microscope during lateral skull base surgery, is feasible for all open approaches. The use of the 3D exoscopic technique is very promising for future lateral skull base surgeries.

Lung abscess as a complication of Lemierre Syndrome in adolescents: a single center case reports and review of the literature.

BACKGROUND: Fusobacterium necrophorum is an anaerobic, gram-negative, non-motile, filamentous, non-spore forming bacillus found in the oral cavity, gastrointestinal tract, and female genital tract, responsible of a rare disease named Lemierre Syndrome, characterized by septic thrombophlebitis of the internal jugular vein, which mainly affects previously healthy adolescents and young adults; some risk factors are reported, as smoking or primary viral or bacterial infection leading to the disruption of mucosa. The syndrome originates commonly from an upper respiratory infection such as pharyngotonsillitis, acute otitis media, cervical lymphadenitis, sinusitis, or odontogenic abscess, and may result in multiorgan metastasis, more frequently leading to pulmonary complications, especially lung abscesses. CASE PRESENTATION: We describe two cases of adolescents with atypical Lemierre Syndrome evaluated in a tertiary care center, one with a confirmed infection by Fusobacterium necrophorum and one with a presumptive diagnosis based on clinical features, who developed lung abscesses needing a prolonged antibiotic course and hospitalization. Of interest, both were user of electronic cigarette, configuring a possible new risk factor. The proper diagnosis of Lemierre Syndrome is often difficult to establish, so a high degree of suspicion is needed, especially in the case of lung abscesses in otherwise healthy adolescents. CONCLUSION: The current study will contribute to providing insight into Lemierre Syndrome clinical presentation and management in adolescents, promoting awareness for a rare but potentially fatal disease. Moreover, it suggests a possible relationship between Lemierre syndrome and the use of electronic cigarette, that should be investigated by future studies.

Acquired subglottic cysts in children: A rare and challenging clinical entity. A systematic review.

INTRODUCTION: Subglottic cysts (SGCs) are a rare cause of laryngeal stenosis that mainly seem to affect preterm infants with an intubation history. PURPOSE: To review the related literature and compare different management protocols for patients presenting SGCs, as well as briefly report our clinical case treated at Verona University Hospital by Pediatric Airways Team. METHODS: The articles resulting from a PubMed and MEDLINE search were analysed and selected using previously established criteria. A systematic review of the selected papers was conducted following PRISMA guidelines. RESULTS: The search yielded 571 related articles; cross-checking of articles led to the identification and exclusion of 239 duplicates. The remaining 332 papers were screened according to previously established eligibility criteria. The final number of selected articles was 13. CONCLUSIONS: Well-planned teamwork, with active collaboration between the ENT specialists, pediatricians and anesthesiologists, is the key to achieve multidisciplinary management of patients diagnosed with SGCs. Long-term follow-up is crucial considering the high recurrence rate of this disease.