Expanding the spectrum of low-grade sinonasal adenocarcinoma with biphasic seromucinous differentiation and activating HRAS/AKT1 mutations.

AIMS: Low-grade non-intestinal-type sinonasal adenocarcinoma (LGSNAC) is a rare heterogeneous and poorly characterised group of tumours, distinct from intestinal- and salivary-type neoplasms. Therefore, further characterisation is needed for clearer biological understanding and classification. METHODS AND RESULTS: Clinical, histological and molecular characterisation of four cases of biphasic, low-grade adenocarcinomas of the sinonasal tract was performed. All patients were male, aged between 48 and 78 years, who presented with polypoid masses in the nasal cavity. Microscopically, virtually all tumours were dominated by tubulo-glandular biphasic patterns, microcystic, focal (micro)papillary, oncocytic or basaloid features. Immunohistochemical staining confirmed biphasic differentiation with an outer layer of myoepithelial cells. Molecular profiling revealed HRAS (p.G13R, p.Q61R) mutations, and concomitant AKT1 (p.E17K, p.Q79R) mutations in two cases. Two cases showed potential in-situ/precursor lesions adjacent to the tumour. Follow-up periods ranged from 1 to 30 months, with one case relapsing locally after 12 and > 20 years. CONCLUSION: This study further corroborates a distinct biphasic low-grade neoplasm of the sinonasal tract with seromucinous differentiation. Although morphological and molecular features overlap with salivary gland epithelial-myoepithelial carcinoma, several arguments favour categorising these tumours within the spectrum of LGSNAC.

Taste Alteration in External Auditory Canal Cholesteatoma: Indicator of Impending N VII Affection.

INTRODUCTION: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients. METHODS/STUDY DESIGN: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients. RESULTS: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results. DISCUSSION/CONCLUSION: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve.

Measuring and evaluating standardization of scrub nurse instrument table setups: a multi-center study.

OBJECTIVES: In-depth knowledge about surgical processes is a crucial prerequisite for future systems in operating rooms and the advancement of standards and patient safety in surgery. A holistic approach is required, but research in the field of surgical instrument tables, standardized instrument setups and involved personnel, such as nurses, is sparse in general. The goal of this study is to evaluate whether there is an existing standard within clinics for an instrument table setup. We also evaluate to which extent it is known to the personnel and whether it is accepted. MATERIALS AND METHODS: The study makes use of the Nosco Trainer, a scrub nurse training and simulation system developed to analyze various aspects of the workplace of scrub nurses. The system contains a virtual instrument table, which is used to perform and record instrument table setups. We introduce a metric which delivers a measurable score for the similarity of surgical instrument table setups. The study is complemented with a questionnaire covering related aspects. RESULTS: Fifteen scrub nurses of the Otolaryngology departments at three clinics in Germany and Switzerland performed a table setup for a Functional Endoscopic Sinus Surgery intervention and completed the questionnaire. The analysis of the developed metric with a leave one out cross-validation correctly allocated 14 of the 15 participants to their clinic. DISCUSSION: In contrast to the identified similarities of table setups within clinics with the collected data, only a third of the participants confirmed in the questionnaire that there is an existing table setup standard for Functional Endoscopic Sinus Surgery interventions in their facility, but almost three quarters would support a written standard and acknowledge its possible benefits for trainees and new entrants in the operating room. CONCLUSIONS: The structured analysis of the surgical instrument table using a data-driven metric for comparison is a novel approach to gain deeper knowledge about intra-operative processes. The insights can contribute to patient safety by improving the workflow between surgeon and scrub nurse and also open the way for goal-oriented standardization.

Blunt nasal trauma in children: a frequent diagnostic challenge.

OBJECTIVE: The clinical challenge in blunt nasal trauma in children, is to identify cases requiring specialized care among frequently encountered banalities, whilst trying to minimize the exposure to diagnostic procedures. We aim to evaluate the related diagnostic and therapeutic pathways and its outcome during follow-up. METHODS: This retrospective cohort study includes children up to 16 years presenting at the emergency department with blunt nasal trauma of our tertiary reference center. RESULTS: The incidence of blunt nasal injuries was estimated 1750 cases in 7 years. A total of 459 consecutive cases with suspected complications were enrolled. Univariate comparison between age groups revealed a statistically significant diminution of downfall related injuries with growing up, whereas blows (including violence) significantly increased with age (p < 0.001 each). The logistic regression model identified male sex as an independent risk factor for soft tissue lesions (OR 1.699, p = 0.017) and for frontobasal fractures (OR 2.415, p = 0.050). Age was not identified to play a significant role regarding localization of injuries. Delayed septorhinoplasties became necessary in 2 cases only (0.4%). The logistic regression model identified nasal bone fracture (OR 17.038, p < 0.001) and mandibular fracture (OR 4.753, p = 0.004) as independent risk factor for a surgical intervention. CONCLUSIONS: Blunt trauma to the nose is frequent in children. Trauma mechanisms differ significantly between age groups, whereas localization and concomitant injuries do not. Male sex was identified as an independent risk factor for soft tissue lesions and frontobasal fractures. In our experience, initial triage by the pediatric department with consecutive involvement of the ENT specialists in case of suspected complications is safe and effective and may help to reduce unnecessary diagnostic procedures/irradiation to the young patients.

Endoscopic Ear Surgery for External Auditory Canal Cholesteatoma.

BACKGROUND: Treatment of external auditory canal cholesteatoma (EACC) has been a question of debate. To our knowledge and according to a systematic review of endoscopic ear surgery (EES) in 2015, this study describes for the first time the technique and outcome by solely transcanal EES for EACC. STUDY METHOD: Retrospective case series, level of evidence IV. METHODS: Between October 2014 and December 2016, nine patients with unilateral EACC have been treated by EES. Using a bimanual technique, canaloplasty has been performed using tragal perichondrium, cartilage, or artificial bone. Symptoms, signs, and reconstruction technique have been assessed and the primary endpoint: healing time was compared with benchmark values in the literature. RESULTS: During the 26 months study period all of our nine Naim stage III EACCs were successfully treated by EES with median healing time of 23.8 days. EACC limited to the external auditory canal (Naim stage III) represented an ideal target for EES minimizing tissue damage and thus median healing time compared with retroauricular (42-56 d) or endaural (59 d) surgical techniques. DISCUSSION: Shorter healing time helped to reduce skepticism toward a surgical treatment of EACC from the patient's perspective. Moreover, EES relied on reduced bulky equipment, dressing time, and complex maintenance compared with microscopic techniques. CONCLUSION: Transcanal endoscopic surgery is a valid treatment option for EACC up to Naim stage III. Moreover, the described procedure fosters in our eyes the teaching of our residence to get familiar with the basic steps of EES.

Design and evaluation of an interactive training system for scrub nurses.

OBJECTIVE: The current trend toward increasingly integrated technological support systems and the rise of streamlined processes in the OR have led to a growing demand for personnel with higher levels of training. Although simulation systems are widely used and accepted in surgical training, they are practically non-existent for perioperative nursing, especially scrub nursing. This paper describes and evaluates an interactive OR environment simulation to help train scrub nurses. METHODS: A system comprising multiple computers and monitors, including an interactive table and a touchscreen combined with a client-server software solution, was designed to simulate a scrub nurse's workplace. The resulting demonstrator was evaluated under laboratory conditions with a multicenter interview study involving three participating ear, nose, and throat (ENT) departments in Germany and Switzerland. RESULTS: The participant group of 15 scrub nurses had an average of 12.8 years hands-on experience in the OR. A series of 22 questions was used to evaluate various aspects of the demonstrator system and its suitability for training novices. DISCUSSION: The system received very positive feedback. The participants stated that familiarization with instrument names and learning the instrument table setup were the two most important technical topics for beginners. They found the system useful for acquiring these skills as well as certain non-technical aspects. CONCLUSIONS: Interactive training through simulation is a new approach for preparing novice scrub nurses for the challenges at the instrument table in the OR. It can also improve the lifelong training of perioperative personnel. The proposed system is currently unique in its kind. It can be used to train both technical and non-technical skills and, therefore, contributes to patient safety. Moreover, it is not dependent on a specific type of surgical intervention or medical discipline.

Topographic bone thickness maps for Bonebridge implantations.

Bonebridge™ (BB) implantation relies on optimal anchoring of the bone-conduction implant in the temporal bone. Preoperative position planning has to account for the available bone thickness minimizing unwanted interference with underlying anatomical structures. This study describes the first clinical experience with a planning method based on topographic bone thickness maps (TBTM) for presigmoid BB implantations. The temporal bone was segmented enabling three-dimensional surface generation. Distances between the external and internal surface were color encoded and mapped to a TBTM. Suitable implant positions were planned with reference to the TBTM. Surgery was performed according to the standard procedure (n = 7). Computation of the TBTM and consecutive implant position planning took 70 min on average for a trained technician. Surgical time for implantations under passive TBTM image guidance was 60 min, on average. The sigmoid sinus (n = 5) and dura mater (n = 1) were exposed, as predicted with the TBTM. Feasibility of the TBTM method was shown for standard presigmoid BB implantations. The projection of three-dimensional bone thickness information into a single topographic map provides the surgeon with an intuitive display of the anatomical situation prior to implantation. Nevertheless, TBTM generation time has to be significantly reduced to simplify integration in clinical routine.

18 FDG-PET/CT computer-assisted biopsies for suspected persistent or recurrent malignant skull base disease.

Background: FDG-PET/CT is increasingly used for the evaluation of regional or distant metastasis in head and neck oncology. However, positive PET findings lack specificity, which is especially challenging for localized disease at the skull base. Methods: An optically tracked navigation system for multimodal image-guided biopsies was tested to evaluate PET-positive skull base lesions between 2009 and 2013. Results: FDG-PET/CT navigated biopsies of patients with suspected persistence or recurrence of carcinoma (sinonasal, n=3; nasopharyngeal, n=1; adenocarcinoma, n=2; and carcinoma of unknown primary origin, n=1) have been safely performed. Histology confirmed local persistent or recurrent malignant disease (n=5), radio-osteonecrosis (n=1) and super-infection (n=1). Conclusions: In the follow-up of tumor patients, FDG-PET/CT-navigated biopsies are a valid tool to evaluate PET-positive skull base lesions. This is an especially useful technique if functional anomalous areas in FDG-PET/CT do not cause structural alterations in MRI/CT, and if endoscopic visualization is impossible because of post-treatment alterations. Head Neck, 2014.

Diagnostic value of biopsies in identifying cytoplasmic antineutrophil cytoplasmic antibody-negative localized Wegener's granulomatosis presenting primarily with sinonasal disease.

BACKGROUND: A substantial proportion of Wegener's disease (WG) patients present with localized disease of the upper airways, i.e., sinonasal and other ear/nose/throat (ENT) symptoms. Because of the oligosymptomatic presentation a timely diagnosis of this potentially fatal disease is challenging. This study evaluates diagnostic peculiarities between WG in its localized and generalized form of the disease. METHODS: Retrospective analysis was performed of 82 patients with suspected WG manifesting in the ENT region between 1989 and 2009. Comparison was performed of the clinical and laboratory results between patients with localized (n = 15) and generalized stage (n = 16) as well as non-WG patients (n = 50). RESULTS: ENT signs and symptoms were subtle, especially in the population presenting with localized disease. Therapy refractory rhinosinusitis or serous otitis media were the most frequent presentations of WG. In testing for localized WG, mucosal biopsy had the highest sensitivity (53%) compared with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCAs) with a lower sensitivity (47%) but highest specificity (96%) and highest positive predictive value (PPV; 78% versus 73%). Patients with generalized WG typically revealed a pathological urine sediment, hemoptysis, or rheumatological symptoms. In the generalized stage, c-ANCA had the highest sensitivity (81%), specificity (96% versus 95%), and highest PPV (87%). CONCLUSION: Timely diagnosis and treatment of localized WG limited to the ENT region remains problematic. Even with adequate therapy, nearly one-half of patients with sinonasal localization suffer from relapse, at least 1 in 10 will progress to generalized disease, and up to two-thirds may develop permanent tissue damage. Unfortunately, the diagnostic usefulness of c-ANCA is significantly reduced at this early stage compared with cases with generalized disease (p = 0.04). Hence, the relative diagnostic value of mucosal biopsy increases especially for the significant proportion of c-ANCA(-) localized WG patients (47%). Sinonasal tissue sampling represents a cornerstone for diagnosis, which unlike c-ANCA testing can be repeated in short intervals and is associated with low morbidity.

Diffuse swelling of the buccal mucosa and palate as first and only manifestation of an extranodal non-Hodgkin 'double-hit' lymphoma: report of a case.

BACKGROUND: Most of the lymphomas arising in the oral cavity are of B-cell origin. Among these, diffuse large B-cell lymphomas are the most common. Diffuse large B-cell lymphomas may exhibit more than one chromosomal rearrangement and are then referred to as 'double-hit' or 'triple-hit' lymphomas. CASE REPORT: We present a case of an intraoral 'double-hit' lymphoma in a 76-year-old male who had been referred by an oral surgeon in private practice. Intraoral examination exhibited a firm, exophytic lesion in the region of the right hard palate and buccal mucosa with extension to the soft palate. Radiographic examination exhibited a massive thickening of the right sinus membrane, and arrosion of the lateral and basal cortical sinus walls in the right maxilla. After diagnosis of the lesion, the patient was treated with six cycles of chemotherapy. DISCUSSION: Lymphomas arising within the oral cavity account for less than 5% of all oral malignancies and typically affect the palatine tonsils and the palate. 'Double-hit' lymphomas are associated with older age, usually present with an advanced stage of disease, and show an aggressive clinical behaviour. They normally have a poor prognosis, even when treated with intensive chemotherapy regimens. Nevertheless, in the case presented, the patient was free of symptoms 1 year after initial diagnosis.

Characteristics and dimensions of the Schneiderian membrane: a radiographic analysis using cone beam computed tomography in patients referred for dental implant surgery in the posterior maxilla.

OBJECTIVES: To determine the dimensions of the Schneiderian membrane using limited cone beam computed tomography (CBCT) in individuals referred for dental implant surgery, and to determine factors influencing the mucosal thickness. MATERIAL AND METHODS: The study included 143 consecutive patients referred for dental implant placement in the posterior maxilla. A total of 168 CBCT images were taken using a limited field of view of 4 × 4 cm, 6 × 6 cm, or 8 × 8 cm. Reformatted coronal CBCT slices were analyzed with regard to the thickness and characteristics of the Schneiderian membrane in nine standardized points of reference. Factors such as age, gender, or status of the remaining dentition that could influence the dimensions of the Schneiderian membrane were evaluated using univariate and multivariate linear regression models. RESULTS: The thickness of the Schneiderian membrane exhibited a wide range, with a minimum value of 0.16 mm and a maximum value of 34.61 mm. The highest mean values, ranging from 2.16 to 3.11 mm, were found for the mucosa located in the mid-sagittal regions of the maxillary sinus. The most frequent mucosal findings diagnosed were flat thickenings of the Schneiderian membrane (62 positive findings, 37%). For the multivariate linear regression model, only gender had a statistically significant influence on the mean overall and mid-sagittal thickness of the sinus mucosa. CONCLUSION: There is great interindividual variability in the thickness of the Schneiderian membrane. Gender seems to be the most important parameter influencing mucosal thickness in asymptomatic patients. Future studies are needed to assess the therapeutic and prognostic consequences of mucosal alterations in the maxillary sinus.

Ear canal cholesteatoma: meta-analysis of clinical characteristics with update on classification, staging and treatment.

PURPOSE OF REVIEW: We present an update on clinical evaluation, staging, classification and treatment of canal cholesteatoma, including a meta-analysis of clinical data of the last 30 years. RECENT FINDINGS: Ear canal cholesteatoma is frequently associated secondarily to other canal pathologies. The cause for the rare idiopathic form of the disease remains enigmatic. Epidemiologic and experimental studies of its pathogenesis have increased; however, the main explanatory theory of a deficient migratory capacity of the canal epithelium affected has been falsified only recently. Therefore, the debate on the pathogenesis has gained additional impetus and more data is needed. SUMMARY: Canal cholesteatoma is a rarity in otologic pathology, often leading to misdiagnosis as external otitis or otomycosis by physicians unfamiliar with the disease. It presents typically with otorrhea, focal erosion and keratin accumulation in the osseous ear canal and has to be distinguished from keratosis obturans, which leads to otalgia and bilateral conductive hearing loss by ceruminal plugs, with circumferential distention of the ear canal. Treatment by canaloplasty is curative and highly successful. Alternative conservative treatment is feasible, however, requiring long-term follow up, with often painful cleaning of the lesion.

Intensity-modulated radiotherapy for a rendu-osler-weber disease patient with recurrent severe epistaxis: a case report.

We present a case of a Rendu-Osler-Weber disease patient with recurrent life threatening epistaxis demanding multiple blood transfusions despite of repetitive endoscopic laser and electrocoagulations, endovascular embolisation, septodermoplasty, and long-term intranasal dressings. As alternative treatment modalities repeatedly failed and the patient became almost permanently dependent on nasal dressing, we performed a highly conformal intensity-modulated radiotherapy of the nasal cavity; a total dose of 50 Gy in 2 Gy single fractions was applied. The therapy was very well tolerated, no acute toxicities occurred. Two weeks after the last radiation dose had been applied, the nasal dressing could be removed without problems. Endoscopical control revealed an almost avascular white mucosa without any trace of bleeding spots; previously existing hemangiomas and crusts had disappeared. After a 1-year-follow up, the patient had no significant recurrent epistaxis.

External auditory canal cholesteatoma: reassessment of and amendments to its categorization, pathogenesis, and treatment in 34 patients.

OBJECTIVE: External auditory canal cholesteatoma (EACC) is a rarity. Although there have been numerous case reports, there are only few systematic analyses of case series, and the pathogenesis of idiopathic EACC remains enigmatic. STUDY DESIGN: In a tertiary referral center for a population of 1.5 million inhabitants, 34 patients with 35 EACC (13 idiopathic [1 bilateral] and 22 secondary) who were treated between 1994 and 2006 were included in the study. RESULTS: EACC cardinal symptoms were longstanding otorrhea (65%) and dull otalgia (12%). Focal bone destruction in the external auditory canal with retained squamous debris and an intact tympanic membrane were characteristic. Only 27% of the patients showed conductive hearing loss exceeding 20 dB. Patients with idiopathic EACC had lesions typically located on the floor of the external auditory canal and were older, and the mean smoking intensity was also greater (p < 0.05) compared with patients with secondary EACC. The secondary lesions were assigned to categories (poststenotic [n = 6], postoperative [n = 6], and posttraumatic EACC [n = 4]) and rare categories (radiogenic [n = 2], postinflammatory [n = 1], and postobstructive EACC [n = 1]). In addition, we describe 2 patients with EACC secondary to the complete remission of a Langerhans cell histiocytosis of the external auditory canal. Thirty of 34 patients were treated surgically and became all free of recurrence, even after extensive disease. DISCUSSION: For the development of idiopathic EACC, repeated microtrauma (e.g., microtrauma resulting from cotton-tipped applicator abuse or from hearing aids) and diminished microcirculation (e.g., from smoking) might be risk factors. A location other than in the inferior portion of the external auditory canal indicates a secondary form of the disease, as in the case of 2 patients with atypically located EACC after years of complete remission of Langerhans cell histiocytosis, which we consider as a new posttumorous category and specific late complication of this rare disease.

Late-onset posttraumatic septal hematoma and abscess formation in a six-year-old Tamil girl--case report and literature review.

Nasal septal hematoma with abscess (NSHA) is an uncommon complication of trauma and studies on children are especially rare. We discuss the case of a 6-year-old girl, who was initially evaluated independently by three doctors for minor nasal trauma but had to be re-hospitalized 6 days later with NSHA. Although septal hematoma had initially been excluded (5, 7 and 24 hours after trauma), a secondary accumulation of blood seems to have occured. Delayed hematoma formation has been described in the orbit as a result of possible venous injuries after endoscopic sinus surgery. However, such an observation is new for septal hematoma in children. Thus, we recommend re-evaluation for septal hematoma 48h to 72h after paediatric nasal trauma. Such a scheduled re-examination offers a chance to treat delayed subperichondral hematoma on time before almost inevitable superinfection leads to abscess formation and destruction of the nasal infrastructure. We suggest that parents should be vigilant for delayed nasal obstruction as possible herald of hematoma accumulation within the first week.