Quality of Life Improvement in Concurrent Septorhinoplasty and Endoscopic Sinus Surgery.

OBJECTIVES: Septorhinoplasty (SRPL) and functional endoscopic sinus surgery (FESS) are two frequently performed surgeries to improve quality of life (QoL) in patients with nasal symptoms. It has been demonstrated as a safe combination regarding complication rates, but patient satisfaction in concurrent surgery has not been adequately studied yet. METHODS: Patients undergoing sole FESS due to chronic rhinosinusitis (n = 57), sole SRPL (n = 148), and concurrent surgery (n = 62) were prospectively evaluated for their disease-specific QoL before and one year after surgery. Each procedure was performed by the same surgeon (OCB). For SRPL, the patient-reported outcome measures Rhinoplasty Outcomes Evaluation (ROE) and Functional Rhinoplasty Outcome Inventory (FROI-17) were utilized, and for chronic rhinosinusitis, the Sino-Nasal Outcome Test-22 (SNOT22). RESULTS: All three groups showed significant improvement in the postoperative QoL measurements (all p < 0.01). The postoperative improvements were slightly smaller in the concurrent surgery group compared with the single surgery groups measured with ROE (combined: +55.2 ± 9.3, single: +58.8 ± 9.8, p = 0.02), FROI total score (combined: +47.6 ± 5.2, single: +49 ± 5.4; p = 0.08) and SNOT22 (combined: +33.1 ± 6.7, single +34.5 ± 7, p = 0.26). CONCLUSIONS: SRPL, FESS, and combined surgery improve disease-specific QoL. When applicable, surgeons may offer the benefits of a combined procedure without compromising the QoL gain. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1239-1245, 2024.

Quality of Life and Screening on Body Dysmorphic Disorder, Depression, Anxiety in Septorhinoplasty.

OBJECTIVES: Septorhinoplasty (SRPL) can improve quality of life (QoL) in functional and aesthetical aspects of the nose. A key factor compromising postoperative satisfaction is symptoms of body dysmorphic disorder (BDD), defined by excessive concerns and distress over slight or imagined physical defects. Although a high prevalence of BDD in SRPL patients is evident, the effect of positive screening on BDD and other psychiatric disorders is understudied. It was hypothesized that patients screening positive for BDD, depression or anxiety do not show increased postoperative QoL. METHODS: A multicenter, prospective study including 259 patients. For psychiatric evaluation, the BDD concern questionnaire-aesthetic version and the Hospital Anxiety and Depression Scale were used; for disease-specific QoL the Rhinoplasty Outcomes Evaluation (ROE) and the Functional Rhinoplasty Outcome Inventory (FROI-17). RESULTS: In preoperative evaluation, 32.5% had a positive screening for BDD, 42.2% for increased anxiety, and 32.9% for depression. Mean QoL improved in the whole cohort (FROI total score: 54.4 ± 21.8 to 32.8 ± 23.7 and ROE: 32.3 ± 15.6 to 69.8 ± 23.1, both p < 0.001). Patients screening positive for BDD, depression or anxiety did show an increased postoperative QoL, but to a significantly lower degree and with lower pre- and postoperative QoL levels. CONCLUSIONS: SRPL patients show a high prevalence of BDD symptoms, elevated anxiety, and depression. These subgroups show lower QoL levels and an impaired QoL increase after surgery. Rhinoplasty surgeons must be aware of the disorders and their symptoms, discuss potential concerns with the patients, and potentially refer them to a specialist. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:2187-2193, 2024.

Neratinib + fulvestrant + trastuzumab for HR-positive, HER2-negative, HER2-mutant metastatic breast cancer: outcomes and biomarker analysis from the SUMMIT trial.

BACKGROUND: HER2 mutations are targetable alterations in patients with hormone receptor-positive (HR+) metastatic breast cancer (MBC). In the SUMMIT basket study, patients with HER2-mutant MBC received neratinib monotherapy, neratinib + fulvestrant, or neratinib + fulvestrant + trastuzumab (N + F + T). We report results from 71 patients with HR+, HER2-mutant MBC, including 21 (seven in each arm) from a randomized substudy of fulvestrant versus fulvestrant + trastuzumab (F + T) versus N + F + T. PATIENTS AND METHODS: Patients with HR+ HER2-negative MBC with activating HER2 mutation(s) and prior cyclin-dependent kinase 4/6 inhibitor (CDK4/6i) therapy received N + F + T (oral neratinib 240 mg/day with loperamide prophylaxis, intramuscular fulvestrant 500 mg on days 1, 15, and 29 of cycle 1 then q4w, intravenous trastuzumab 8 mg/kg then 6 mg/kg q3w) or F + T or fulvestrant alone. Those whose disease progressed on F + T or fulvestrant could cross-over to N + F + T. Efficacy endpoints included investigator-assessed objective response rate (ORR), clinical benefit rate (RECIST v1.1), duration of response, and progression-free survival (PFS). Plasma and/or formalin-fixed paraffin-embedded tissue samples were collected at baseline; plasma was collected during and at end of treatment. Extracted DNA was analyzed by next-generation sequencing. RESULTS: ORR for 57 N + F + T-treated patients was 39% [95% confidence interval (CI) 26% to 52%); median PFS was 8.3 months (95% CI 6.0-15.1 months). No responses occurred in fulvestrant- or F + T-treated patients; responses in patients crossing over to N + F + T supported the requirement for neratinib in the triplet. Responses were observed in patients with ductal and lobular histology, 1 or ≥1 HER2 mutations, and co-occurring HER3 mutations. Longitudinal circulating tumor DNA sequencing revealed acquisition of additional HER2 alterations, and mutations in genes including PIK3CA, enabling further precision targeting and possible re-response. CONCLUSIONS: The benefit of N + F + T for HR+ HER2-mutant MBC after progression on CDK4/6is is clinically meaningful and, based on this study, N + F + T has been included in the National Comprehensive Cancer Network treatment guidelines. SUMMIT has improved our understanding of the translational implications of targeting HER2 mutations with neratinib-based therapy.

[Recommendations of the German Society of Otorhinolaryngology, Head and Neck Surgery (DGHNO-KHC) for the granting of resident training program authorizations]. / Empfehlungen der Deutschen Gesellschaft für HNO-Heilkunde, Kopf- und Hals-Chirurgie (DGHNO-KHC) für die Erteilung der fachspezifischen Weiterbildungsbefugnis.

The 2018 template for otorhinolaryngology specialist training of the German Medical Association is increasingly implemented by the federal associations. In this regard, the German Society recommended an otorhinolaryngology resident training plan for Otorhinolaryngology, Head and Neck Surgery (DGHNO-KHC) together with the Professional Association of German Otorhinolaryngologists as a suggested guideline for the federal medical associations. In this context, the state medical associations currently work on criteria on the basis of which otorhinolaryngologists and their training institutions can be granted authority for such a certified otorhinolaryngology resident training program.The DGHNO-KHC last made recommendations for the granting of authorizations for specialist training in otorhinolaryngology in 1999. Many contents have changed as a result of the 2018 model specialist training regulations. Therefore, a scientifically formulated proposal for the granting of continuing education authorizations is herewith provided as recommendation to the federal state medical associations.

Fragmentomic analysis of circulating tumor DNA-targeted cancer panels.

BACKGROUND: The isolation of cell-free DNA (cfDNA) from the bloodstream can be used to detect and analyze somatic alterations in circulating tumor DNA (ctDNA), and multiple cfDNA-targeted sequencing panels are now commercially available for Food and Drug Administration (FDA)-approved biomarker indications to guide treatment. More recently, cfDNA fragmentation patterns have emerged as a tool to infer epigenomic and transcriptomic information. However, most of these analyses used whole-genome sequencing, which is insufficient to identify FDA-approved biomarker indications in a cost-effective manner. PATIENTS AND METHODS: We used machine learning models of fragmentation patterns at the first coding exon in standard targeted cancer gene cfDNA sequencing panels to distinguish between cancer and non-cancer patients, as well as the specific tumor type and subtype. We assessed this approach in two independent cohorts: a published cohort from GRAIL (breast, lung, and prostate cancers, non-cancer, n = 198) and an institutional cohort from the University of Wisconsin (UW; breast, lung, prostate, bladder cancers, n = 320). Each cohort was split 70%/30% into training and validation sets. RESULTS: In the UW cohort, training cross-validated accuracy was 82.1%, and accuracy in the independent validation cohort was 86.6% despite a median ctDNA fraction of only 0.06. In the GRAIL cohort, to assess how this approach performs in very low ctDNA fractions, training and independent validation were split based on ctDNA fraction. Training cross-validated accuracy was 80.6%, and accuracy in the independent validation cohort was 76.3%. In the validation cohort where the ctDNA fractions were all <0.05 and as low as 0.0003, the cancer versus non-cancer area under the curve was 0.99. CONCLUSIONS: To our knowledge, this is the first study to demonstrate that sequencing from targeted cfDNA panels can be utilized to analyze fragmentation patterns to classify cancer types, dramatically expanding the potential capabilities of existing clinically used panels at minimal additional cost.

Can Sex Improve Nasal Function?-An Exploration of the Link Between Sex and Nasal Function.

OBJECTIVES: This study was conducted to examine the impact of sexual activity on nasal breathing and compare such effect to that of a nasal decongestant. METHODS: We evaluated nasal breathing at 5 different times: (1) before sexual activity (baseline), (2) immediately after sexual activity, (3) 30 minutes, (4) 1 hour (5), and 3 hours after sexual climax. Same measurements were taken on the second day following application of nasal decongestant spray. For evaluation of nasal breathing, we used a visual analogue scale (VAS). Additionally, we used a portable rhinometric device to measure resistance and nasal flow. RESULTS: Nasal breathing improved significantly after sexual intercourse with climax to the same degree as after application of nasal decongestant for up to 60 minutes, as measured subjectively with the VAS (sex -3.6, P < .001; spray -3.2, P < .001). This was confirmed in the objective rhinometric data as mean nasal flow (mL/s) increased while resistance decreased immediately (flow sex +214, P < .001; flow spray +235, P < .001), 30 (flow sex +249, P < .001; flow spray +287, P < .001), and 60 minutes (flow sex +180, P < .001; flow spray +287, P < .001) post-intervention. Nasal breathing was back to the baseline level after 3 hours following sexual intercourse, while it continued to be improved for longer after application of nasal decongestant. Only participants having nasal obstruction (Nasal Obstruction Symptom Evaluation score >30) showed nasal function improvement after sex. CONCLUSIONS: Sexual intercourse with climax can improve nasal breathing to the same degree as application of nasal decongestant for up to 60 minutes in patients having nasal obstruction.

[Analysis of ENT emergency patients during the COVID-19 pandemic in Germany]. / Analyse von HNO-Notfallpatienten während der COVID-19-Pandemie in Deutschland.

BACKGROUND: This retrospective observational study was undertaken to assess the ENT emergency workload during the COVID-19 pandemic caused by the severe acute respiratory coronavirus­2 (SARS-CoV-2). MATERIALS AND METHODS: All 3230 patients who were treated as an emergency from 23.01.2020 to 06.08.2020 in the Department of Otolaryngology at the SLK-Kliniken Heilbronn were included in this study. Demographic data, diagnostics, diagnosis, and treatment (in-/outpatient) were retrospectively retrieved. Not only did the physicians on call triage the emergency department (ED) ENT patients, but the patients also self-assessed their urgency of treatment. RESULTS: The number of patients consulting our ED decreased significantly during the pandemic, by 42.2%. However, the top diagnoses remained almost constant, with epistaxis being the most frequent diagnosis before, during, and after COVID-19. Facial trauma remained the second most frequent consultation reason. The hospitalization rate decreased from 21.9% before COVID-19 to 16.2% during the pandemic. Surgical therapy was necessary in 17.6% of patients before COVID-19 and this increased to 23.5% during COVID-19. The self-referral rate increased from 61 to 66% during the pandemic. More men than women consulted the ED during COVID-19. Regarding the triage assessment by the physician on call and the patient's self-assessment, a significant discrepancy was noted before, during, and after COVID-19. CONCLUSION: The reasons for reduction in ENT ED visits are multifactorial. The clinical consequences of decreased hospitalizations remain uncertain. However, health authorities need to advocate the safety of the hospital environment to limit potential damage.

Validating the Xerostomia Inventory in a radiation-induced xerostomia population in German language.

OBJECTIVES: Though xerostomia is a frequent oral symptom, there is no validated disease-specific questionnaire in German. The purpose of this study was to translate and validate versions of the Xerostomia Inventory and the Summated Xerostomia Inventory in a German-speaking population. PARTICIPANTS AND METHODS: Thirty-nine patients including 18 patients suffering from radiation-induced xerostomia enrolled in this study. Both questionnaires were translated into German language according to international accepted guidelines. For validation, we evaluated reliability, validity, and responsiveness using the COSMIN manual for cross-cultural adaptation. RESULTS: Cronbach's α was 0.92 for XI and 0.91 for SXI, showing both high internal consistency. Patients suffering from xerostomia showed significantly higher average scores demonstrating its discriminant validity. Confirmatory factor analysis showed excellent "goodness-of-fit" values for SXI and good to moderate values for XI, confirming the assumed factor structures. The Xerostomia Inventory and its summated version both showed excellent test-retest reliability in the non-xerostomia group (ICC = 0.85 and 0.84). CONCLUSIONS: The XI and SXI in their cross-cultural adapted versions are the first validated self-report assessments for xerostomia in German language. They are characterized by practical design and can be easily interpreted by the treating physician.

Selective RET kinase inhibition for patients with RET-altered cancers.

Background: Alterations involving the RET kinase are implicated in the pathogenesis of lung, thyroid and other cancers. However, the clinical activity of multikinase inhibitors (MKIs) with anti-RET activity in RET-altered patients appears limited, calling into question the therapeutic potential of targeting RET. LOXO-292 is a selective RET inhibitor designed to inhibit diverse RET fusions, activating mutations and acquired resistance mutations. Patients and methods: Potent anti-RET activity, high selectivity, and central nervous system coverage were confirmed preclinically using a variety of in vitro and in vivo RET-dependent tumor models. Due to clinical urgency, two patients with RET-altered, MKI-resistant cancers were treated with LOXO-292, utilizing rapid dose-titration guided by real-time pharmacokinetic assessments to achieve meaningful clinical exposures safely and rapidly. Results: LOXO-292 demonstrated potent and selective anti-RET activity preclinically against human cancer cell lines harboring endogenous RET gene alterations; cells engineered to express a KIF5B-RET fusion protein -/+ the RET V804M gatekeeper resistance mutation or the common RET activating mutation M918T; and RET-altered human cancer cell line and patient-derived xenografts, including a patient-derived RET fusion-positive xenograft injected orthotopically into the brain. A patient with RET M918T-mutant medullary thyroid cancer metastatic to the liver and an acquired RET V804M gatekeeper resistance mutation, previously treated with six MKI regimens, experienced rapid reductions in tumor calcitonin, CEA and cell-free DNA, resolution of painful hepatomegaly and tumor-related diarrhea and a confirmed tumor response. A second patient with KIF5B-RET fusion-positive lung cancer, acquired resistance to alectinib and symptomatic brain metastases experienced a dramatic response in the brain, and her symptoms resolved. Conclusions: These results provide proof-of-concept of the clinical actionability of RET alterations, and identify selective RET inhibition by LOXO-292 as a promising treatment in heavily pretreated, multikinase inhibitor-experienced patients with diverse RET-altered tumors.

Photodynamic therapy for recurrent respiratory papillomatosis.

BACKGROUND: Recurrent respiratory papillomatosis (RRP) is a benign condition of the mucosa of the upper aerodigestive tract. It is characterised by recurrent papillomatous lesions and is associated with human papillomavirus (HPV). Frequent recurrence and rapid papilloma growth are common and in part responsible for the onset of potentially life-threatening symptoms. Most patients afflicted by the condition will require repeated surgical treatments to maintain their airway, and these may result in scarring and voice problems. Photodynamic therapy introduces a light-sensitising agent, which is administered either orally or by injection. This substance (called a photo-sensitiser) is selectively retained in hyperplastic and neoplastic tissue, including papilloma. It is then activated by light of a specific wavelength and may be used as a sole or adjuvant treatment for RRP. OBJECTIVES: To assess the effects of photodynamic therapy in the management of recurrent respiratory papillomatosis (RRP) in children and adults. SEARCH METHODS: We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; Cambridge Scientific Abstracts; ICTRP and additional sources for published and unpublished trials. The date of the search was 27 January 2014. SELECTION CRITERIA: Randomised controlled trials utilising photodynamic therapy as sole or adjuvant therapy in participants of any age with proven RRP versus control intervention. Primary outcome measures were symptom improvement (respiratory distress/dyspnoea and voice quality), quality of life improvement and recurrence-free interval. Secondary outcomes included reduction in the frequency of surgical intervention, reduction in disease volume and adverse effects of treatment.   DATA COLLECTION AND ANALYSIS: We used the standard methodological procedures expected by The Cochrane Collaboration. Meta-analysis was not possible and results are presented descriptively. MAIN RESULTS: We included one trial with a total of 23 participants. This study was at high risk of bias. None of our primary outcomes and only one of our secondary outcomes (reduction in volume of disease, assessed endoscopically) was measured in the study. There was no significant difference between the groups (very low-quality evidence). Adverse effects reported included airway swelling requiring intubation in a child with severe RRP a few hours after photodynamic therapy. AUTHORS' CONCLUSIONS: There is insufficient evidence from high-quality randomised controlled trials to determine whether photodynamic therapy alters the course of disease or provides an added benefit to surgery in patients with recurrent respiratory papillomatosis. Multicentre randomised controlled trials with appropriate sample sizes and long-term follow-up are required to evaluate whether photodynamic therapy is of benefit. Outcomes such as improvement in symptoms (respiratory function and voice quality) and quality of life should be measured in future trials.

Successful rigid endoscopic removal of an esophageal subtotally covered nitinol stent 11 months after initial placement.

Formation of granulation tissue and stricture at the stent's ends are well-known long-term complications of esophageal subtotally covered externally self-expandable metal stents (SEMS). Removal is associated with an increased risk of mucosal injury, severe bleeding, mediastinis, and consecutive stenosis. We report on a case of successful endoscopic removal of an esophageal SEMS 11 months after original placement to cover an iatrogenic esophageal perforation created during rigid esophagoscopy in a patient with cancer of unknown primary (CUP) syndrome. This case shows that safe SEMS late removal is achievable and at the same time illustrates the disadvantages of using SEMS for benign esophageal pathology. Particularly in cases of accidental esophageal injury during endoscopy, esophageal SEMS placement should be considered as a therapeutic option only if conventional surgery is contraindicated.

Endovascular carotid reconstruction in palliative head and neck cancer patients with threatened carotid blowout presents a beneficial supportive care measure.

ABSTRACT Carotid blowout is a devastating complication in patients with head and neck malignancy. The traditional surgical treatment for carotid blowout is often technically difficult and is associated with an unacceptably high morbidity and mortality. Recently, endovascular therapy has been proposed for head and neck surgical patients. Preliminary reports showed a better outcome with less morbidity and mortality compared to the previous treatment modalities. The use of such techniques in cases of impending or acute carotid blowout syndrome has been previously described to be beneficial for palliative head and neck cancer patients as well. We introduce a case of a head and neck cancer patient receiving palliative care, presenting with threatened carotid blowout, who was managed with endovascular placement of a covered stent under elective conditions in order to prevent an inevitable carotid rupture. In the present case endovascular carotid stenting allowed preservation of the vessel, prevented the dramatic situation of carotid rupture, and facilitated a rapid hospital discharge without any neurologic or stenting sequelae.

Stentgraft-implantation for treatment of internal carotid artery injury during endonasal sinus surgery.

BACKGROUND: Damage of the internal carotid artery (ICA) is very rare but can be a dramatic complication of endonasal sinus surgery. In the literature only a few cases are reported, some of them with fatal results. METHODS: We present two cases with massive bleeding of the ICA caused by endonasal sinus surgery. RESULTS: In both cases bleeding could be stopped sufficiently by implanting a stentgraft. CONCLUSION: The lumen of the artery could be preserved and no neurological deficits were observed.

Forty-one cases of congenital choanal atresia over 26 years--retrospective analysis of outcome and technique.

This retrospective analysis reflects the outcome of various techniques used in a series of 41 cases of choanal atresia treated at the Department of Otoloaryngology, Head- and Neck Surgery at the University of Mainz between 1980 and 2006. Thirteen bilateral and 28 unilateral cases are included. After endonasal management in 38 and a transpalatine approach in 3 cases a total of 15 patients needed revision surgery between 1 and 5 times to establish a stable result. Postoperative stenting was used in 23 patients with a failure rate of 35%, whereas only 11% of the 18 patients without stenting had to be revised. None of those 5 cases where Mitomycin C had been applied intraoperatively in combination with postoperative transnasal dilations needed surgical revision. We conclude that the endonasal micro-endoscopic surgical approach is successful if combined with postoperative dilations for up to one year. Stenting should be abandoned as it stimulates granulation formation that frequently leads to restenosis. The intraoperative application of Mitomycin C offers a promising adjunct in achieving a stable

Separation of beta2-transferrin by denaturing gel electrophoresis to detect cerebrospinal fluid in ear and nasal fluids.

BACKGROUND: Cerebrospinal fluid (CSF) leakage is a critical condition with a substantial risk of meningitis. We investigated the use of transferrin isoform analysis as a diagnostic marker for detection of CSF leakage in fluid samples. METHODS: We analyzed 241 samples from patients with CSF leakage, most commonly presenting as otorrhea or rhinorrhea, by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) with subsequent Western blotting and immunostaining for transferrin. Tears, saliva, nasal fluid, and ear secretions (20 samples each) were analyzed in parallel, and normal human serum served as a control in each experiment. We compared the minimum volume of added CSF that could be detected in secretions by our assay with the minimum volume detected by the prostaglandin-D synthase (beta-trace) test. CSF was admixed with blood in different proportions to determine the influence of blood contamination on the transferrin pattern. RESULTS: In all CSF samples, beta1- and beta2-transferrin were present in nearly equal amounts. In tears and ear secretions, beta2-transferrin migrated in the gel in the same manner as in CSF, but its concentration was noticeably lower than that of beta1-transferrin, a difference that allowed a clear distinction from the transferrin pattern of CSF. In saliva, both transferrin isoforms were also present but could be distinguished from those of other fluids by electrophoretic migration pattern rather than relative concentrations. With the beta-trace test, a minimum of 5 microL of CSF was needed for detection, whereas our beta2-transferrin assay yielded a signal of comparable intensity with a minimum of 2 microL of CSF. CONCLUSION: Analysis of the transferrin microheterogeneity pattern by SDS-PAGE for the identification of CSF leakage is a highly sensitive and specific method that merits consideration as a routine technique.

Natural history and control of epistaxis in a group of German patients with Rendu-Osler-Weber disease.

INTRODUCTION: Epistaxis is the most common symptom of a complex, genetically determined vasculopathy, which is known under the notion hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber-syndrome). This study was initiated to gain more knowledge about the natural history of epistaxis in a German HHT-population. PATIENTS AND METHODS: Data of 49 HHT patients were ascertained by interviewing these patients with a standardized disease specific questionnaire. Patients' files were retrospectively reviewed for data concerning age, gender, past medical history, laboratory parameters, number of hospital admissions for epistaxis, conservative and operative types of therapy, treatment results and follow-up. MAIN RESULT: Epistaxis was the first and most prominent symptom in 93% of the patients and could be triggered most frequently by stress. Half of the patients had experienced first episodes of epistaxis in childhood, but usually epistaxis did not become troublesome before the age of 35 years. The effects of hormonal changes or therapies with systemic hormones were inconclusive with regard to impact on epistaxis. Patients with septal perforations had to be admitted for inpatient epistaxis treatment more frequently than patients with an intact nasal septum. An overall reduction of frequency and intensity of epistaxis could be achieved in 89% of the patients through the daily use of nasal lubricants and a minimum of two treatment sessions with the Nd:YAG laser. However in none of the cases the treatment results were permanent. More than 50% of the patients, who had been screened for visceral arteriovenous malformations, were positive for pathologic vascular lesions. PRINCIPAL CONCLUSION: The natural history of epistaxis in German HHT patients is similar to previously described entities from other parts of the world. First clinical signs of HHT may be present at an earlier age than previously thought.

Morphology and distribution of nasal telangiectasia in HHT-patients with epistaxis.

BACKGROUND: Epistaxis in hereditary hemorrhagic telangiectasia (HHT; Rendu-Osler-Weber syndrome) is a frequent symptom that may be caused by a multitude of different genetic and epigenetic phenomena. This investigation analyzes the distribution of nasal telangiectasia in 21 patients with HHT. METHODS: The patients were examined for endonasal telangiectasia by videoendoscopy with rigid endoscopes; in addition, the anterior portion of the nose was examined under the operating microscope. The endonasal findings were recorded on videotape and then evaluated in the media laboratory. RESULTS: Morphology of the nasal telangiectasia showed wide variations: the vessels were shaped like spots, loops, or spiders or they clustered and resembled raspberries. Gender did not have an influence on the phenotype of telangiectasia, whereas advancing age correlated with a higher density of telangiectasia. Patients with an intact nasal septum exhibited the bulk of telangiectasia in the anterior nasal cavity but also on the middle turbinates, the floor of the nose, and within the valve area. Patients with septal perforations displayed the majority of telangiectasia around the edge of the perforations, on the floor of the nose, and on the turbinates. Scattered telangiectasia also could be found in the profound parts of the nasal cavity and in the nasopharynx, especially in patients with septal perforations. CONCLUSION: The shapes of endonasal telangiectasia in HHT patients are very heterogeneous; predilection sites could first of all be found within the anterior portion of the nose. Morphology and distribution of endonasal telangiectasia change as a result of therapeutic interventions, development of septal perforations, and with advancing age. Therefore, repeat endoscopies are recommended to assess the actual stage of the disease before epistaxis therapy.