Indications for Submandibulectomy Within a 20-Year Period.

Purpose: Different pathologies of the submandibular gland are an indication of submandibular gland excision-ranging from inflammatory causes and sialolithiasis to malignant tumors. The purpose of this study was to get an overview of the different indications for submandibular gland excision. Methods: The main goal of this study was to evaluate the different indications for submandibular gland excision during a 20-year period. In addition, epidemiological information and therapy concepts were investigated with a special focus on Tumor Lymph nodes Metastasis (TNM) classification and recurrence rate. Procedures during which the submandibular gland was removed while not being the primary cause for surgery (eg, neck dissection in Level Ib) were not included. Results: During the period of observation, 359 submandibular gland excisions were performed. The most common cause for submandibular gland excision was sialolithiasis (n = 129) with intraparenchymal stone localization. Up next were inflammatory causes (n = 115) in particular chronic submandibular sialadenitis followed by only a few cases of Sjögren's syndrome, sarcoidosis, and tuberculosis. In 115 cases, surgery was performed for tumors of the submandibular gland, with 88 of them being benign and 27 malignant. Malignancies were then divided into lymphomas (n = 9) and primary salivary gland malignancies (n = 18). Conclusion: This retrospective study of a large cohort of patients displays a representative overview of the indications for submandibular gland excision. Sialolithiasis was the most common underlying cause of gland excision. The malignancy rate in our cohort was lower than described in the literature.

[Virtual DEGUM-certified course in the head and neck region-a useful complement to conventional course formats?] / Virtuell abgehaltene DEGUM-zertifizierte Kurse im Kopf-Hals-Bereich ­ eine sinnvolle Ergänzung zum konventionellen Kursformat?

BACKGROUND: Training in clinical ultrasound has become highly relevant for working as an otorhinolaryngologist. While there is a high demand for standardized and certified training courses, until recently, there was no possibility to attend web-based and exclusively virtual head and neck ultrasound courses certified by the Deutsche Gesellschaft für Ultraschall in der Medizin (DEGUM; German Society for Ultrasound in Medicine). OBJECTIVE: The aim of this study was to provide a qualitative and semi-quantitative analysis of the first purely virtual DEGUM-certified head and neck ultrasound courses. MATERIALS AND METHODS: In 2021, three purely web-based DEGUM-certified head and neck ultrasound courses were carried out and then qualitatively analyzed using questionnaires including an examination. RESULTS: The purely virtual implementation of head and neck ultrasound courses proved to be a viable alternative to the conventional course format, with a high level of acceptance among the participants. The lack of practice among the participants remains a relevant criticism. CONCLUSION: A more dominant role of web-based and remote ultrasound training is likely and should be considered as an alternative depending on existing conditions. Nevertheless, acquisition of practical sonographic skills remains a major hurdle if courses are purely digital.

[Neuro-otological manifestations in Fabry disease-a retrospective single center study]. / Neurootologische Manifestationen bei Morbus Fabry ­ eine retrospektive Analyse.

BACKGROUND: Fabry disease (FD) is one of the X­linked lysosomal storage diseases that can affect any organ. They have a specific lysosomal dysfunction in common, which results in substrate accumulation in lysosomes instead of metabolite degradation. Due to the deficiency/absence of α­galactosidase, globotriaosylceramides (Gb3) are deposited in lysosomes of the organs. In addition to acroparesthesia, angiokeratomas, autonomic dysfunction, vortex keratopathies, ischemic cerebral or cardiac complications and chronic renal failure, also vestibulocochlear dysfunctions with sudden or progressive asymmetric hearing loss, tinnitus and vertigo may be observed. PATIENTS AND METHODS: In this retrospective study, 33 patients (men = 16 and women = 17) with FD were evaluated. All patients presented to us in interdisciplinary cooperation as part of routine examinations by the specialized center for lysosomal storage diseases of the in-house department of nephrology. This presentation is carried out as a screening examination independent of neuro-otological symptoms. RESULTS: The mean age at diagnosis was 34.76 (±11.55) years. The first presentation in our ENT department was at 40.45 (±11.71) years. We were able to demonstrate a significant correlation between neurological symptoms or apoplexy and hearing loss (p = 0.001) and between cardiac manifestations and hearing loss (p = 0.024). CONCLUSION: Hearing loss is a potential symptom of Fabry disease and is not limited to the classic male phenotype. Due to possible positive correlations with neurological and cardiological manifestations of the disease, routine ENT screening examinations should be carried out to be able to identify and treat neuro-otological deficits at an early stage. In addition, FD should also be considered and tested as a differential diagnosis, especially in younger patients with sudden unilateral or bilateral hearing loss and a family history.

A Retrospective Analysis of Long-Term Prophylaxis with Berotralstat in Patients with Hereditary Angioedema and Acquired C1-Inhibitor Deficiency-Real-World Data.

Hereditary angioedema (HAE) and acquired C1-inhibitor deficiency (AAE-C1-INH) are orphan diseases. Berotralstat is a recently licensed long-term prophylaxis (LTP) and the first oral therapy for HAE patients. No approved therapies exist for AAE-C1-INH patients. This study is the first to report real-world clinical data of patients with AAE-C1-INH and HAE who received Berotralstat. All patients treated with Berotralstat were included in this retrospective, bi-centric study. Data was collected from patients' attack calendars and the angioedema quality of life (AE-QoL) and angioedema control test (AECT) questionnaires before treatment, and at 3, 6, and 12 months after treatment and was then analyzed. Twelve patients were included, 3 patients with AAE-C1-INH, 7 patients with HAE type I, and 2 patients with HAE-nC1-INH. One patient (HAE I) quit treatment. Berotralstat was associated with fewer attacks in all groups. After 6 months of treatment, a median decrease of attacks per month was noted for HAE type I patients (3.3 to 1.5) and AAE-C1-INH patients (2.3 to 1.0). No aerodigestive attacks were noted for AAE-C1-INH patients. For HAE-nC1-INH patients, a mean decrease from 3.8 to 1.0 was noted (3 months). For HAE I patients, the total AE-QoL lowered a mean of 24.1 points after 6 months, for HAE-nC1-HAE patients 8.0 points, and for AAE-C1-INH patients 13.7 points. AECT scores increased for HAE I patients (mean: 7.1), HAE-nC1-INH patients (9.0), and AAE-C1-INH patients (4.2) after 6 months. Patients with HAE, HAE-nC1-INH, and AAE-C1-INH treated with Berotralstat showed reduced angioedema attacks and improved AE-QoL and AECT scores.