A Multidisciplinary Skull Base Board for Tumour and Non-Tumour Diseases: Initial Experiences.

The skull base is the area where various cancerous and non-cancerous diseases occur and represents the intersection of several medical fields. The key is an integrated treatment by specialists of multiple disciplines. We prospectively analysed patients with a skull base disease between August 2022 and 2023 and presented to the Multidisciplinary Skull Base Board (MDT-SB), which takes place once a month hybridly (in-person and remotely). Thirty-nine patients (median age of 58.2 years) were included, of which twelve (30.8%) had a benign tumour, twelve (30.8%) had a malignant tumour, five had an infection (12.8%), and ten (25.6%) had other diseases. For each patient, at least two otorhinolaryngologists, a neurosurgeon, and a neuroradiologist, as well as an infectious disease specialist, a paediatrician, an oculoplastic surgeon, a maxillofacial surgeon, and a pathologist were involved in 10%, 8%, 8%, 3%, and 3% of cases, respectively. In fifteen patients (38%), the MDT-SB suggested surgical treatment; in fourteen (36%), radiological follow-ups; in five (13%), non-surgical treatments; in two, conservative treatments (5%); in two (5%), surgical and conservative treatments; and in one (3%), a biopsy. Non-cancerous and cancerous diseases of the skull base in adults and children should be presented to the MDT-SB, which consists of at least an otolaryngologist, a neurosurgeon, and a neuroradiologist.

Sinonasal Orbital Apex Syndrome, Horner Syndrome and Pterygopalatine Fossa Infection: A Case Report and Mini-Review.

This paper presents a literature review and a case of an 83-year-old otherwise healthy female patient with a history of recent syncope, a sudden-onset right-sided temporal headache, diplopia, and vision loss. An exam revealed right-sided upper eyelid ptosis, myosis, vision loss, ophthalmoplegia, and a positive relative afferent pupillary defect on the right eye. CT showed sphenoid sinus opacification, eroded lateral sinus wall, Vidian canal, disease extension to the posterior ethmoid air cells, orbital apex, medial orbital wall, and pterygopalatine fossa. An orbital apex syndrome (Jacod's syndrome), Horner syndrome, and pterygopalatine fossa infection were diagnosed due to the acute invasive fungal sinusitis developed from a sphenoid sinus fungal ball. The patient was treated with antimicrobial therapy and transnasal endoscopic surgery twice to decompress the orbital apex, drain the abscess and obtain specimens for analysis. The right-sided ptosis, visual loss, ophthalmoplegia, and headache resolved entirely. No immune or comorbid diseases were identified, microbiological and histopathological analyses were negative, and MRI could not be performed on the presented patient. For that reason, the diagnostic procedure was non-standard. Nevertheless, the treatment outcome of this vision and life-threatening disease was satisfactory. Treating the fungal ball in an older or immunocompromised patient is essential to prevent invasive fungal rhinosinusitis and fatal complications.

Atypical Skull-Base Osteomyelitis: Comprehensive Review and Multidisciplinary Management Viewpoints.

Atypical skull-base osteomyelitis is a rare but fatal disease that usually involves infection of the ethmoid, sphenoid, occipital, or temporal bones that form the skull base. Unlike typical (so-called otogenic), atypical skull-base osteomyelitis has no otogenic cause. Instead, some authors call atypical skull-base osteomyelitis sinonasal, since the infection most often originates from the nose and paranasal sinuses. Diagnosing and treating this disease is challenging. To assist in managing atypical skull-base osteomyelitis, a review of the most recent literature, with patient cases and multidisciplinary perspectives from otolaryngologists, neurosurgeons, radiologists, infectious disease specialists, pathologists, and clinical microbiologists, is provided in this paper.

Transcriptomic Differentiation of Phenotypes in Chronic Rhinosinusitis and Its Implications for Understanding the Underlying Mechanisms.

Chronic rhinosinusitis (CRS) is a multifaceted disease with variable clinical courses and outcomes. We aimed to determine CRS-associated nasal-tissue transcriptome in clinically well-characterized and phenotyped individuals, to gain a novel insight into the biological pathways of the disease. RNA-sequencing of tissue samples of patients with CRS with polyps (CRSwNP), without polyps (CRSsNP), and controls were performed. Characterization of differently expressed genes (DEGs) and functional and pathway analysis was undertaken. We identified 782 common CRS-associated nasal-tissue DEGs, while 375 and 328 DEGs were CRSwNP- and CRSsNP-specific, respectively. Common key DEGs were found to be involved in dendritic cell maturation, the neuroinflammation pathway, and the inhibition of the matrix metalloproteinases. Distinct CRSwNP-specific DEGs were involved in NF-kß canonical pathways, Toll-like receptor signaling, HIF1α regulation, and the Th2 pathway. CRSsNP involved the NFAT pathway and changes in the calcium pathway. Our findings offer new insights into the common and distinct molecular mechanisms underlying CRSwNP and CRSsNP, providing further understanding of the complex pathophysiology of the CRS, with future research directions for novel treatment strategies.

Sialendoscopy-Based Analysis of Submandibular Duct Papillae with a Proposal for Classification.

Identifying a submandibular (Wharton's) duct punctum often hinders sialendoscopy; however, there is a paucity of evidence on whether the appearance of Wharton's duct papilla impacts the sialendoscopic procedure. A classification of Wharton's duct papillae based on the macroscopic appearance, size of dilatation probes, and sialendoscopic approach was proposed herein. The classification describing four main types of papillae, A, B, C, and D, was prospectively evaluated on 351 Wharton's duct papillae in 315 patients. For each papillae type, the demographic/clinical data, intraoperative complications, and time required for sialendoscope introduction were analyzed. Estuary-like papilla (type A) was commonly seen after spontaneous stone extrusion, had no intraoperative complications noted, and had the shortest time required for the sialendoscope introduction. Normal papilla (type B) was the most frequently observed papilla (48.1%), reflecting diverse underlying pathology, while difficult papilla (type C) was often associated with unfavorable anatomical variations of the mandible or floor of the mouth. Substantially closed papilla (type D) had the highest rate of intraoperative complications, namely, perforation with a false passage, and required the longest time for the sialendoscope introduction. In seven patients (2.0%), the entrance into the duct was feasible only through the fistula, while the sialendoscope introduction failed in eight patients (2.3%). In conclusion, the appearance of Wharton's duct papillae may be influenced by the underlying pathology. Based on the proposed classification, papilla typology affects the duration of sialendoscope introduction and may influence the frequency of intraoperative complications.

Management of Choanal Atresia: National Recommendations with a Comprehensive Literature Review.

Choanal atresia is the most common congenital anatomical abnormality of the nasal cavities, manifested with a clinical picture of neonatal respiratory distress. The treatment requires interdisciplinary management based mainly on tertiary referral centre experiences. However, there is a lack of high-quality evidence in the available literature. Recommendations were prepared based on a systematic review of the supporting literature: on a website survey addressed to the participating authors consisting of 28 questions and on five live meetings. The initial response to the recommendations was determined at their presentation at the sectional meeting of the section for otorhinolaryngology of the Slovenian Medical Association. Then, reactions from the professional public were accepted until the recommendations were presented at the Expert Council for Otorhinolaryngology of the Slovenian Medical Association. A systematic literature review identified eight systematic reviews or meta-analyses and four randomized controlled clinical trials. Thirty-four recommendations for diagnosis, treatment and postoperative management were consolidated. The paper presents the proposal and first Slovenian recommendations for treating patients with choanal atresia. They are based on foreign medical institutions' published literature and our clinical experience. They represent the basic requirements of diagnostics and may represent an essential guide in treatment.

Sialendoscopy in Management of Juvenile Recurrent Parotitis-A Single Centre Experience.

Juvenile recurrent parotitis is a rare inflammatory disease of the parotid gland that shares diverse therapeutic management between institutions. Sialendoscopy has been demonstrated as an efficient diagnostics and therapeutic method with minimal complications; however, due to the rarity of the disease and limited data, there is a lack of universal guidelines on its optimal management. Herein, we retrospectively analysed patients with juvenile recurrent parotitis who had the sialendoscopy performed at our tertiary centre. Descriptive data were retrieved along with the number of swelling episodes one year before and after the sialendoscopy intervention. In the last decade, twenty-nine sialendoscopic procedures were performed at our clinics on twenty-one patients diagnosed with juvenile recurrent parotitis. Most of them underwent the procedure under general anaesthesia (86%). In the year before and after the sialendoscopic procedure, the patients had 3.9 ± 2.7 and 0.2 ± 0.4 episodes of swelling per year, respectively. The difference proved to be statistically significant (p < 0.0001). The complete resolution was noted in sixteen patients (76%); however, the procedure was not repeated on the same side of any patient. Solely one patient had a relapse of the disease reported more than twelve months after the sialendoscopy, nonetheless, one of his exacerbation episodes was already reported in the first year after the sialendoscopy. The mean follow-up period of patients was 48.6 months (range, 13−116 months). All things considered, this study emphasises sialendoscopy as an effective minimally invasive diagnostic and therapeutic tool for the management of juvenile recurrent parotitis.

Indicators of pediatric peripheral vestibular disorder: A retrospective study in a tertiary referral center.

OBJECTIVES: This study aims to present characteristics of pediatric patients with peripheral vertigo and dizziness and their clinical workup results in the tertiary otorhinolaryngology center. We wanted to investigate whether the detailed history could replace the extensive vestibular testing and whether the clinical presentation could guide the first contact physician to appropriately directed specialist referral. METHODS: Retrospective case review of consecutive pediatric vertigo and dizziness patients referred to the tertiary otorhinolaryngology center from 2015 to 2020. The data about the signs and symptoms of vertigo and dizziness and the results of audiological and vestibular tests were collected. RESULTS: Of 257 children aged 10.9±4.3 years (R: 1-17 years), 32 (12.5%) had peripheral, and 49 (19%) had central vertigo and dizziness. Acute vestibulopathy was diagnosed in 22/257 (8.5%) children, sudden sensorineural hearing loss and benign paroxysmal positional vertigo in 5/257 (2%) children each. 60% of children with peripheral vertigo and dizziness had emesis, and 55.6% had nausea. 8% of children had spontaneous horizontal-rotatory nystagmus that followed Alexander's law. Goodman and Kruskal's л for determining whether the type of nystagmus could predict the type of vertigo and dizziness (central or peripheral) was 0.481 (p = 0.001). 12/26 (60%) of children with peripheral vertigo and dizziness had emesis compared to 14 (30.04%) children without emesis, a difference in proportions of 0.296 (p = 0.024, chi-square test of homogeneity). Binomial logistic regression to ascertain the effects of duration, nausea and emesis on the likelihood of the presence of peripheral vertigo was statistically significant (χ2(3) = 10.626, p = 0.014). CONCLUSION: Unlike adults, peripheral vestibular causes of vertigo and dizziness in children may be rare but have the same typical signs and symptoms. The detailed history and careful clinical examination are crucial in differentiating between peripheral and central causes. This guides the first contact physician for further referral to appropriate specialists included in a multidisciplinary workup. Namely, nausea, emesis, horizontal nystagmus and a longer duration of symptoms in a child with vertigo and dizziness indicate a peripheral etiology. Therefore, a referral to an otorhinolaryngologist is reasonable.

Clinical Implementation of Platelet- and Extracellular Vesicle-Rich Product Preparation Protocols.

Extracellular vesicles (EVs) are considered essential mediators of regenerative roles of autologous platelet- and extracellular vesicle-rich plasma (PVRP) and platelet- and extracellular vesicle-rich gel (PVRG). PVRP and PVRG are novel blood-derived products gaining attraction in regenerative medicine. However, despite their reported good efficacy, their preparation protocols are too time-consuming. Moreover, patient-tailored preparation protocols are desired to optimize platelet and EV count in PVRP and PVRG. This article presents the clinical implementation of one-step, patient-tailored erythrocyte sedimentation rate (ESR)-based, PVRP and PVRG preparation protocols through the presentation of three cases: (1) large chronic tympanic membrane (TM) perforation, (2) osteoradionecrosis of the lateral skull base, and (3) cerebrospinal fluid (CSF) leak in the sphenoid sinus. These were treated with PVRP and PVRG, prepared according to our preclinically constructed mathematical sedimentation model of cells and EVs based on the patient's ESR. (1) TM healed completely after the treatment with 3.6 mL of PVRP and PVRG (high platelet and EV protocol). The speech discrimination score and air conduction pure tone average improved from 75% to 95% and from 65 to 25 dB, respectively. (2) The osteoradionecrotic surface area decreased from 46 to 18 cm2, and infection was eradicated after six applications of 13-65 mL of PVRG ("half-volume" protocol). (3) No CSF leak recurrence was detected after surgical closure with 30 mL of PVRG postoperatively. Reproducible preparation protocols proved effective, safe, fast, and straightforward enough for the surgical staff to prepare PVRP and PVRG intraoperatively. To alleviate preparation, a calculator is provided. This pilot study presents a sound basis for further studies, which are needed to assess the therapeutic effect of PVRP and PVRG. Impact statement We introduce a clinical implementation of a patient-tailored, erythrocyte sedimentation rate-based platelet- and extracellular vesicle-rich plasma (PVRP) and gel (PVRG) preparation protocol based on a mathematical model. Products proved beneficial in wound healing and were, to our knowledge, used for the first time in the treatment of osteoradionecrosis of the lateral skull base. Furthermore, this reproducible preparation protocol is fast and straightforward to implement in clinical practice. A calculator is provided to alleviate PVRP and PVRG preparation for various clinical scenarios.

Desmoid-type fibromatosis of paranasal sinuses with intracranial extension in a child-acase-based review.

PURPOSE: Desmoid-type fibromatosis (DF) is clonal fibroblastic proliferation that arises in the deep soft tissues, tends to reoccur, and is locally invasive. Desmoid-type fibromatosis of paranasal sinuses with intracranial extension is a rare condition that is even rarer in a small child. We aim to share with the reader our literature review, decision-making, and endoscopic endonasal operation procedure that combined gained us favorable results against this benign tumor with unpredictable natural history and disease course. CASE REPORT: We describe the decision-making process in the management of a 3-year-old boy with a history of sudden vision loss and vomiting. MR showed an expansive well-delineated homogeneous tumor in the sphenoid sinus with intracranial extension and optic nerves compression. The diagnosis of a sporadic form of desmoid-type fibromatosis was made using genetic testing of tumor tissue. A total gross removal was carried out with endoscopic endonasal microsurgical approach. At a 3-month follow-up, the patient is without any signs of recurrance. CONCLUSION: The treatment of children with desmoid-type fibromatosis requires a multidisciplinary approach by clinicians experienced with the management of pediatric cancer. While the desmoid-type fibromatosis is a benign, locally invasive tumor, observation should be the first step in the management. In case of life-threatening or symptomatic cases, operations that preserve function and structure should be the first choice for this benign tumor with unpredictable natural history and disease course.

Unraveling the Etiology of Pediatric Vertigo and Dizziness: A Tertiary Pediatric Center Experience.

Background and Objectives: Numerous authors have reported that the commonest type of vertigo in children is migraine-associated vertigo (vestibular migraine and benign paroxysmal vertigo of childhood-BPV). We aimed to provide the possible etiological background of vertigo and dizziness in Slovenian children. Materials and Methods: A retrospective case series of pediatric vertigo and dizziness children referred to the tertiary pediatric otorhinolaryngology center from 2015 to 2020. Children received a complete audiological and vestibular workup and were referred to pediatric specialists depending on the clinical presentation. Results: Of 257 children (42% male, 58% female) aged 1-17 years (M = 10.9, SD = 4.3 years) in 19.1% vertigo and dizziness were classified as central, in 12.4% as a peripheral vestibular, in 10.9% as a hemodynamic, in 5.8% as a psychological and none as visual by pediatric neurologists, otorhinolaryngologists, cardiologists, psychologists or ophthalmologists, respectively. 40.8% (20) children with central vertigo had BPV (7.8% of all children) and 8.2% (4) migrainous vertigo. In 43.6% (112 children), the etiology remained unclassified. Conclusions: After a thorough multidisciplinary workup, the etiology of vertigo and dizziness was unraveled in the majority of children referred to our tertiary otorhinolaryngology center. The most common cause was central; however, in a considerable number, the etiology remained unclassified. The latter could be attributed to the self-limiting nature of vertigo spells. Hence, a child presenting with dizziness and vertigo requires a multidisciplinary approach, in which referral to a neurologist is, in most cases, essential.

Sialendoscopy and CT navigation assistance in the surgery of sialolithiasis.

BACKGROUND: A sialendoscopy-assisted combined approach is well established in the surgery of sialolithiasis. In cases of proximal salivary stones, transcutaneous sialendoscopy-assisted extractions with parotid and submandibular gland preservation is the primary intention of treatment. We recently added computer tomography (CT) navigation to improve the results of this challenging surgery equally in both localizations. PATIENTS AND METHODS: Al l the patients who submitted to sialendoscopy and sialendoscopy-assisted procedures at the tertiary institution between January 2012 and October 2020 were included in the present study. From November 2019, CT navigation was added in cases with sialolithiasis and a presumably poor sialendoscopic visibility. We evaluated the parameters of the disease, diagnostic procedures, sialendoscopic findings and outcomes, with or without optical surgical navigation. RESULTS: We performed 178 successful salivary stone removals in 372 patients, of which 118 were combined sialendos-copy-assisted approaches, including 16 transcutaneous proximal, 10 submandibular and 6 parotid stone operations. Surgical navigation was used in six patients, four times for submandibular and twice for parotid sialolithiasis. These were all non-palpable, sialendoscopically invisible or partially visible stones, and we managed to preserve five of the six salivary glands. CONCLUSIONS: The addition of CT navigation to sialendoscopy-assisted procedures for non-palpable, sialendoscopically invisible and fixed stones is a significant advantage in managing sialolithiasis. By consistently performing sialendoscopy and related preservation procedures, we significantly reduced the need for sialoadenectomies in patients with obstructive salivary gland disease.

Emergent tracheostomy during the pandemic of COVID-19: Slovenian National Recommendations.

PURPOSE: Emergent tracheostomy under local anaesthesia is a reliable method of airway management when orotracheal intubation is not possible. COVID-19 is spread through aerosol making the emergent tracheostomy a high-risk procedure for surgeons. The surgical establishment of the air conduit in emergency scenarios must be adjusted for safety reasons. METHODS: To establish the Slovenian National Guidelines for airway management in cannot intubate-cannot ventilate situations in COVID-19 positive patients. RESULTS: Good communication and coordination between surgeon and anaesthesiologist is absolutely necessary. Deep general anaesthesia, full muscle relaxation and adequate preoxygenation without intubation are initial steps. The surgical cricothyrotomy is performed quickly, the thin orotracheal tube is inserted, the cuff is inflated and ventilation begins. Following patient stabilisation, the conversion to the tracheostomy is undertaken with the following features: skin infiltration with vasoconstrictor, a vertical incision, avoidance of electrical devices in favour of classical manners of haemostasis, the advancement of the tube towards the carina, performing the tracheal window in complete apnoea following adequate oxygenation, the insertion of non-fenestrated canulla attached to a heat and moisture exchanger, the fixation of canulla with stitches and tapes, and the cricothyrotomy entrance closure. Appropriate safety equipment is equally important. CONCLUSION: The goal of the guidelines is to make the procedure safer for medical teams, without harming the patients. Further improvements of the guidelines will surely appear as COVID-19 is a new entity and there is not yet much experience in handling it.

Cross-cultural adaptation and validation of nasal obstruction symptom evaluation questionnaire in Slovenian language.

OBJECTIVES: Nasal obstruction is highly subjective perception with numerous efforts being made towards objective measuring. Many instruments in quality of life studies encompass subjective symptom of nasal obstruction, but only NOSE has been properly validated and is easy to use in every day practice. METHODS: Multicenter prospective instrument validation and cross-cultural adaptation cohort study was conducted on patients with deviated nasal septum, with or without inferior turbinate hypertrophy, to develop the Slovenian version of NOSE questionnaire. A cross-cultural adaptation of the original questionnaire was done in five steps, producing Slovenian NOSE-si, used on a pilot group to confirm the quality of adapted tools and, afterwards, on the main study and control group. Symptoms were lasting for more than 12 months and all had an indication for septal surgery. A control group was selected from a pool of healthy subjects, self-assessed as having no rhinological complaints. RESULTS: NOSE-si was used on 116 patients (58 from the study group vs. 58 from the control group). High degree of internal consistency - Cronbach's a 0.971 and reliability after retesting - Goodman-Kruskal gamma coefficient 0.984 was proven. Responsiveness was confirmed in the surgery subgroup with standardized response mean (SRM) 2.76 (p<0.001). CONCLUSIONS: The study produced a valid Slovenian version of NOSE questionnaire through rigorous and well defined five-phase effort to maintain scientifically comparable QoL instrument, and may be used by clinicians and researchers.

The assessment of mucosal surgical margins in head and neck cancer surgery with narrow band imaging.

OBJECTIVES/HYPOTHESIS: The diagnostic gain of narrow band imaging in the definition of surgical margins in the treatment of head and neck cancer was evaluated. STUDY DESIGN: A prospective study, blinded to the pathologist, with historical comparison. METHODS: The study group included 45 patients subjected to the intraoperative definition of margins by narrow band imaging. The control group included 55 patients who had undergone standard definition of margins. All patients underwent resection of the tumor and frozen section analysis of superficial margins. The rate of initial R0 resection and the ratio of histologically negative margins for both groups were statistically compared. RESULTS: The rate of initial R0 resection in the study group and in the control group was 88.9% and 70.9% (P = .047), and the ratio of histologically negative margins was 95.9% and 88.4% (P = .017), respectively. CONCLUSIONS: Narrow band imaging reveals a microscopic extension of the tumor that could be effectively used to better define superficial margins and to achieve a higher rate of initial R0 resections. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:1577-1582, 2017.

Coupling treatment planning with navigation system: a new technological approach in treatment of head and neck tumors by electrochemotherapy.

BACKGROUND: Electrochemotherapy provides highly effective local treatment for a variety of tumors. In deep-seated tumors of the head and neck, due to complex anatomy of the region or inability to cover the whole tumor with standard electrodes, the use of long single needle electrodes is mandatory. In such cases, a treatment plan provides the information on the optimal configuration of the electrodes to adequately cover the tumor with electric field, while the accurate placement of the electrodes in the surgical room in patients can remain a problem. Therefore, during electrochemotherapy of two head and neck lymph-node metastases of squamous cell carcinoma origin, a navigation system for placement of electrodes was used. PATIENT AND METHODS: Electrochemotherapy of two lymph-node metastases of cutaneous squamous cell carcinoma, one in the left parotid gland and the other in the neck just behind the left mandibular angle, was performed using intravenous administration of bleomycin and long single needle electrodes. The tumors were treated according to the prepared treatment plan, and executed with the use of navigation system. RESULTS: Coupling of treatment plan with the navigation system aided to an accurate placement of the electrodes. The navigation system helped the surgeon to identify the exact location of the tumors, and helped with the positioning of the long needle electrodes during their insertion, according to treatment plan. Five electrodes were inserted for each metastasis, one centrally in the tumor and four in the periphery of the tumor. Five weeks after electrochemotherapy, computed tomography images demonstrated partial response of the first metastasis and complete response of the second one. Six weeks after electrochemotherapy, fine-needle aspiration biopsy specimen obtained from the treated lesions revealed necrosis and inflammatory cells, without any viable tumor cells. CONCLUSION: We describe a new technological approach for electrochemotherapy of deep-seated head and neck tumors, coupling of the treatment planning with navigation system for accurate placement of the single long needle electrodes into and around the tumors, according to the treatment plan. Evidence of its effectiveness on two lymph-node metastases of cutaneous squamous cell carcinoma origin in neck lymph is provided.