To date, there is no approved local therapeutic agent for the treatment of epistaxis due to hereditary hemorrhagic telangiectasia (HHT). Several case reports suggest the topical use of timolol. This monocentric, prospective, randomized, placebo-controlled, double-blinded, cross-over study investigated whether the effectiveness of the standard treatment with a pulsed diode laser can be increased by also using timolol nasal spray. The primary outcome was severity of epistaxis after three months, while the main secondary outcome was severity of epistaxis and subjective satisfaction after one month. Twenty patients were allocated and treated, of which 18 patients completed both 3-month treatment sequences. Timolol was well tolerated by all patients. Epistaxis Severity Score after three months, the primary outcome measure, showed a beneficial, but statistically nonsignificant (p = 0.084), effect of additional timolol application. Epistaxis Severity Score (p = 0.010) and patients’ satisfaction with their nosebleeds after one month (p = 0.050) showed statistically significant benefits. This placebo-controlled, randomized trial provides some evidence that timolol nasal spray positively impacts epistaxis severity and subjective satisfaction in HHT patients when additively applied to standard laser therapy after one month. However, the effect of timolol was observed to diminish over time. Trials with larger sample sizes are warranted to confirm these findings.
Appropriate management of hereditary hemorrhagic telangiectasia (HHT) is of particular importance in females, as HHT-mediated modifications of the vascular bed and circulation are known to increase the risk of complications during pregnancy and delivery. This study was undertaken to evaluate female HHT patients’ awareness of and experience with HHT during pregnancy and delivery, with a focus on epistaxis. In this retrospective study, 46 females (median age: 60 years) with confirmed HHT completed a 17-item questionnaire assessing knowledge of HHT and its pregnancy-associated complications, the severity of epistaxis during past pregnancies and deliveries, and the desire for better education and counselling regarding HHT and pregnancy. Results revealed that 85% of participants were unaware of their disease status prior to the completion of all pregnancies. Further, 91% reported no knowledge of increased pregnancy-related risk due to HHT. In regard to epistaxis, 61% of respondents reported experiencing nosebleeds during pregnancy. Finally, approximately a third of respondents suggested that receiving counseling on the risks of HHT in pregnancy could have been helpful. Findings suggest that awareness of HHT and its potential for increasing pregnancy-related risk is poor. Best practices in HHT management should be followed to minimize negative effects of the disorder.
Hereditary hemorrhagic telangiectasia (HHT; Rendu-Osler-Weber syndrome) affects the capillary and larger vessels, leading to arteriovenous shunts. Epistaxis is the main symptom impairing quality of life. The aim of the Osler Calendar is to offer information about the extent of the systemic disease and the current state of treatment. A care plan with information on the rare disease and self-treatment of epistaxis was created. Organ examinations and ongoing treatments were recorded. A questionnaire documents the treatment success, including patient satisfaction, frequency of hemorrhage and hemoglobin levels. The patients using the Osler Calendar for at least one year (n = 54) were surveyed. Eighty-five percent of patients (n = 46) used the calendar to gain information about HHT. Seventy-two percent (n = 39) used the Osler Calendar for instructions on the self-treatment of nosebleeds. The calendar increased patients’ understanding for the need for organ screenings from 48% (n = 26) to 81% (n = 44). Seventy-nine percent (n = 43) of patients confirmed that the Osler Calendar documented their therapeutic process either well or very well. Fifty-two percent (n = 28) saw an improvement in the therapeutic process due to the documentation. The Osler Calendar records the individual intensity of the disease and facilitates the communication between attending physicians. It is a tool for specialists to review treatment strategies. Furthermore, the calendar enhances patients’ comprehension of their condition.
19 July 2022 in European Archives of Oto-Rhino-Laryngology
Purpose: Several theories have been proposed regarding the origin of lateral neck cysts (LNC). Besides complete surgical resection ipsilateral tonsillectomy and dissection of a tract or its remnants is sometimes recommended. In this retrospective trial we wanted to evaluate if patients, who received LNC resection only, develop complications or recurrence to justify this surgical strategy. Methods: Patients who received LNC resection between 2004 and 2017 at the Ear Nose and Throat Department of a university hospital were included. Data was collected from the clinic database and through a structured telephone interview. Results: A total of 126 patients met the inclusion criteria. In this collective, the diagnosis of a lateral neck cyst was confirmed histologically. Mean age at time of operation was 38 years (± 14.6). The median follow-up time was 7 years (range 3–18). None of the participants experienced recurrent unilateral pharyngitis or tonsillitis during follow-up. Furthermore, there was no case of postoperative peritonsillar, neck phlegmon or neck abscess. No patient reported recurrence of LNC. Conclusions: Sole complete resection of LNCs is sufficient to avoid postoperative infections and recurrences. Therefore, ipsilateral tonsillectomy and tract dissection is not necessary in routine cases of LNC surgery.
20 January 2026 in European Archives of Oto-Rhino-Laryngology
Purpose: Hereditary hemorrhagic telangiectasia (HHT) is a genetically inherited vascular disorder transmitted in an autosomal dominant manner. It is defined by the presence of arteriovenous malformations in the nasal mucosa, resulting in severe recurrent epistaxis. This study investigates the long-term efficacy and safety of blue light laser therapy for managing epistaxis in patients with HHT. Methods: A retrospective analysis was conducted on 71 patients treated between 09/2017 and 02/2023 at the Department of Otorhinolaryngology, University Hospital Regensburg. All patients underwent endonasal treatment using a pulsed diode laser at 445 nm as part of a structured stepwise protocol including nasal care. Of the 86 patients initially treated, 17.4% were excluded due to uncontrollable epistaxis requiring surgery under general anesthesia or concomitant medication. Results: A total of 351 laser treatments were administered, with no reported occurrences of nasal septal perforation. A modified Epistaxis Severity Score (ESS, range 0–10) decreased by an estimated mean difference of –0.66 (95% CI = –1.05, –0.27). Subgroup analyses revealed that patients with moderate to severe baseline ESS experienced a significant reduction of –1.53 (95% CI = –2.11, –0.95), achieving the minimal important difference (MID) of 0.71. Patients’ satisfaction with their nasal bleeding, measured by the Numeric Rating Scale (0–10), improved significantly from 5.08 to 6.67 ( p < 0.001). Conclusions: Our study indicates that blue light laser therapy plus mucosal care is associated with reduced nosebleed severity, improved patient satisfaction and a favorable safety profile, supporting its use as a long-term treatment option for managing HHT in patients with mild to moderate epistaxis.
Intracranial sinogenic and otogenic complications and high-grade orbital complications of sinusitis in children are rare, but case numbers appeared to increase after COVID-19 health measures were lifted. As similar trends were reported internationally, we conducted a multicenter retrospective case series to evaluate this “perceived accumulation”. The study population included otherwise healthy children treated at eight participating centers from seven countries including a period from 2012 to 2023 for above-mentioned complications. Recorded parameters included age, diagnosis, timing, duration and type of inpatient therapy, as well as microbiological and virological findings, and differential blood count. A total of 289 data sets were received from the eight centers. Cases per semiannual increased significantly from 6 to 28 in the sinogenic and 3 to 12 in the otogenic cohort comparing pre- to post-pandemic period. Main patient characteristics were preserved. However, the number of surgical interventions per patient rose slightly in the post-pandemic sinogenic cohort. No association with acute COVID-19 infection was identified. In summary, intracranial sinogenic and otogenic complications in children rose significantly after the lifting of COVID-19-related restrictions. As immunity to respiratory viruses is not permanent, COVID-19 protective measures may have resulted in a lack of stimulation of the immune system, favoring the occurrence of superinfections. This highlights the importance of global respiratory infection research and surveillance programs, as well as open data sharing and maintaining vaccine development and use.