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The original article [1] mistakenly cropped Fig. 1. The full, corrected version of Fig. 1 can instead be viewed ahead.

In the original article, there was a mistake in the order of Figures 1 and 2 as published. The figures are given in the correct order below. The authors apologize for the error and state that this does not change the scientific conclusions of the article in any way. The original article has been updated.

Lo schwannoma intralabirintico (SIL) è un raro tumore benigno (prevalenza 0.1-0.4%) che origina dalle cellule di Schwann situate a ridosso della giunzione cito-neurale dell’VIII n.c. I sintomi clinici includono ipoacusia neurosensoriale monolaterale progressiva (95%), acufeni (51%), disequilibrio (35%), vertigine (22%), fullness (2%). Alla risonanza magnetica (RM) il tumore si presenta come una massa circoscritta, iperintensa in T1, ipointensa in T2 e con enhancement dopo gadolinio nelle immagini T1-pesate. Il management prevede in prima istanza osservazioni seriali con RM “wait and scan approach”. La chirurgia, considerando le complicanze descritte quali anacusia (100%), paralisi VII n.c. (4%), fistola liquorale (5.4%), meningite (1.8%), è riservata a casi limitati e dipende da: età, condizioni generali del paziente, sede e dimensioni del tumore ed è consigliabile in caso di un pattern di crescita invasivo e presenza di sintomi vertiginosi non responsivi al trattamento medico.

Background: Cystic Fibrosis (CF) consists of multiorgan manifestations that include chronic rhinosinusitis (CRS) with or without nasal polyposis. The principal symptoms of CRS are nasal congestion, rhinorrea, mouth breathing, facial pain, and olfactory dysfunction (anosmia/hyposmia). A large percentage of CF patients present CRS therefore we evaluated the olfactory performance in: i) CF patients with CRS without polyps; ii) age-matched healthy controls. Materials and Methods: We enrolled: - group I: 29 CF patients with CRS without polyps with different mutations (mean age: 30.1±9.3 years); - group II: 29 age-matched healthy volunteers (mean age: 29.7±5.9 years). All cases were not smokers. They did not assume food or beverages other than water within 6 hours previous test. Subjects with acute nasal infection, facial trauma and/or nasal polyps were excluded. All subjects underwent the Sniffin’Sticks (Burghart Medical Technology) (SS) to assess the olfactory performance. Results: Group I: 7% of CF patients were normosmic, 69% hyposmic, 10% anosmic, 14% borderline. They presented mean odor identification of 10.55±2.99, mean odor discrimination of 10.28±2.67, mean odor threshold of 2.14±2.63, mean TDI score of 22.97±6.07. All these olfactory evaluations were statistically different to healthy controls (Group II) (p

Sino-nasal solitary extramedullary plasmacytoma (EMP) is a rare neoplasm with unpredictable progression to multiple myeloma. To improve the precision of irradiation delivery, preserving the healthy surrounding tissue and critical structures we used a CyberKnife® for the treatment of sinonasal solitary extramedullary plasmacytoma. We present the first case of sinonasal-EMP treated with CyberKnife®-stereotactic radiotherapy (SRT) with a complete remission without adverse events. Based on the post-therapeutic results and healthy tissue preservation, we believe that CyberKnife®-SRT represents a good therapeutic option for the treatment of sinonasal-EMP.

Diagnostic workup in rare tumors of rhinopharynx.

Settimi, Stefano/0000-0003-0104-1501; De Corso, Eugenio/0000-0001-5761-7018; Salvati, Antonio/0000-0002-0718-8996; Cantone, Elena/0000-0002-7389-2033 WOS: 000455133900001 PubMed: 30623894 Facial pain remains a diagnostic and therapeutic challenge for both clinicians and patients. In clinical practice, patients suffering from facial pain generally undergo multiple repeated consultations with different specialists and receive various treatments, including surgery. Many patients, as well as their primary care physicians, mistakenly attribute their pain as being due to rhinosinusitis when this is not the case. It is important to exclude non-sinus-related causes of facial pain before considering sinus surgery to avoid inappropriate treatment. Unfortunately, a significant proportion of patients have persistent facial pain after endoscopic sinus surgery (ESS) due to erroneous considerations on aetiology of facial pain by physicians. It should be taken into account that neurological and sinus diseases may share overlapping symptoms, but they frequently co-exist as comorbidities. The aim of this review was to clarify the diagnostic criteria of facial pain in order to improve discrimination between sinogenic and non-sinogenic facial pain and provide some clinical and diagnostic criteria that may help clinicians in addressing differential diagnosis.

BACKGROUND AND PURPOSE: Kallmann syndrome is a rare inherited disorder due to defective intrauterine migration of olfactory axons and gonadotropin-releasing hormone neurons, leading to rhinencephalon hypoplasia and hypogonadotropic hypogonadism. Concomitant brain developmental abnormalities have been described. Our aim was to investigate Kallmann syndrome–related brain changes with conventional and novel quantitative MR imaging analyses. MATERIALS AND METHODS: Forty-five male patients with Kallmann syndrome (mean age, 30.7 years; range, 9–55 years) and 23 age-matched male controls underwent brain MR imaging. The MR imaging study protocol included 3D-T1, FLAIR, and diffusion tensor imaging (32 noncollinear gradient-encoding directions; b-value = 800 s/mm2). Voxel-based morphometry, sulcation, curvature, and cortical thickness analyses and tract-based spatial statistics were performed by using Statistical Parametric Mapping 8, FreeSurfer, and the fMRI of the Brain Software Library. RESULTS: Corpus callosum partial agenesis, multiple sclerosis–like white matter abnormalities, and acoustic schwannoma were found in 1 patient each. The total amount of gray and white matter volume and tract-based spatial statistics measures (fractional anisotropy and mean, radial, and axial diffusivity) did not differ between patients with Kallmann syndrome and controls. By specific analyses, patients with Kallmann syndrome presented with symmetric clusters of gray matter volume increase and decrease and white matter volume decrease close to the olfactory sulci; reduced sulcal depth of the olfactory sulci and deeper medial orbital-frontal sulci; lesser curvature of the olfactory sulcus and sharper curvature close to the medial orbital-frontal sulcus; and increased cortical thickness within the olfactory sulcus. CONCLUSIONS: This large MR imaging study on male patients with Kallmann syndrome featured significant morphologic and structural brain changes, likely driven by olfactory bulb hypo-/aplasia, selectively involving the basal forebrain cortex.

Diagnosi e terapia di un raro tumore testa collo: il plasmacitoma extramidollare

effetti benefici nel ridurre timing cicatrizzazione post operatoria dopo uso di spray nasale a base di Vit A e E con liposomi

Si valuta l'associazione tra DM tipo II e cancro laringeo

descriviamo caso di un paziente con tumore di Warthin che presenta ascesso e paralisi del facciale in seguito ad un FNAC della parotide paziente

descriviamo diagnosi e clinica del melanoma mucosa naso sinusale. Tumore raro testa-collo altamente aggressivo e con rischio di recidiva locale.

Rhinogenic headache (RH) is a headache or facial pain syndrome secondary to mucosal contact points in the sino-nasal cavities, in the absence of inflammatory signs, hyperplastic mucosa, purulent discharge, sino-nasal polyps or masses. It may result from pressure on the nasal mucosa due to anatomical variations among which the pneumatization of the middle turbinate, concha bullosa, a variant of the development of ethmoidal cells, is the most commonly observed. Clinical practice suggests a close correlation between concha bullosa, mucosal contacts and rhinogenic headache, with high impact on the QoL. However diagnostic and therapeutic difficulties still remain. Aim of the present study is to evaluate the impact of medical or surgical care on the QoL of patients suffering from concha bullosa related headache from the patients' perspective.One-hundred-two subjects with concha bullosa and headache anamnesis were randomized into two groups and given medical or surgical treatment. To assess the Quality of life (QoL) we used visual analogue scale and for the first time, the migraine disability score before and after treatment.After treatment the severity of the headache decreased as well as the discomfort in the surgical group compared with medical group.The improvement of symptoms and QoL suggests that the endoscopic surgical plastic may promote the rapid resolution of concha bullosa related headache improving the and reducing health care costs.

Il Plasmocitoma è una proliferazione di un singolo clone di plasmacellule. Può presentarsi come singola lesione ossea ( 40Gy) e successivo follow-up. CONCLUSIONI La presenza di neoformazione nasosinusale monolaterale impone un protocollo diagnostico rigoroso. L’endoscopia rappresenta il primo step seguito dall’imaging. Tuttavia TC e RM non sono esaustive nella diagnosi di natura e la biopsia rappresenta l’unica indagine dirimente. Sebbene il PEM sia estremamente raro lo Specialista ORL non può trascurare un approfondimento diagnostico multidisciplinare che veda coinvolto oncoematologo, patologo e radioterapista.

Il sarcoma granulocitico (SG) è una rara (3%) manifestazione extramidollare della Leucemia Acuta Mieloide (LAM). Rapporto M:F 1.42:1, età media alla diagnosi 37. Può essere isolato (8-20%), svilupparsi nel contesto di LAM o esserne espressione di recidiva (2.5-9.1%) e secondario a disordini mieloproliferativi/mielodisplastici. La diagnosi si effettua mediante biopsia, aspirato midollare, esami di laboratorio, imaging e va integrata con immunoistochimica e citochimica. La radioterapia (RT) è il trattamento di scelta in forme isolate, la chemioterapia combinata (induzione e consolidamento) in forme sistemiche. Riportiamo una raro caso di SG rinofaringe in paziente con pregressa LAM. MATERIALI E METODI Donna di 53 anni, non fumatrice, anamnesi positiva per LAM in remissione completa dopo chemioterapia ad alte dosi e trapianto di cellule staminali autologhe, praticate 7 anni prima. La paziente lamentava ipoacusia soggettiva sinistra da 5 mesi. L’endoscopia nasale evidenziava una massa peritubarica sinistra. Assenza di linfonodi latero-cervicali. L’imaging (RM e PET-TC) orientava per neoformazione rinofaringe (6 x 3,5 cm), SUV 5.7, assenza di erosione ossea. La biopsia rinofaringea evidenziava presenza di cellule immature. Al pattern immunoistochimico forte positività per CD43, CD34 e CD99 e negatività per mieloperossidasi (MPO) e CD117; elevata attività proliferativa Ki67 (circa 40%). Aspirato midollare negativo. RISULTATI I dati clinico-strumentali suggerivano ipotesi di localizzazione secondaria da processo ematologico. L’indagine immunoistochimica orientava per SG scarsamente differenziato (MPO-). La paziente praticava RT e follow-up. CONCLUSIONI Il SG è un tumore solido maligno, extramidollare, composto da mieloblasti o cellule mieloidi immature a vario grado di differenziazione. Spesso, nei soggetti non leucemici, le forme scarsamente differenziate sono diagnosticate erroneamente come linfoma a grandi cellule/linfoblastico/Burkitt e carcinoma anaplastico. L’immunoistochimica è necessaria per la diagnosi di SG e va sempre programmata in caso di patologia espansiva rinofaringea soprattutto se sussiste anamnesi positiva per patologia linfoproliferativa, anche se pregressa. Il caso riportato MPO-, raro in letteratura, orienta per diagnosi di SG, scarsamente differenziato, dunque a prognosi peggiore. Per questo la paziente è entrata in un protocollo multidisciplinare di follow-up tipo “short time”.

Background and objective. Sleep-disordered breathing (SDB) may develop in children with primary ciliary dyskinesia (PCD), leading to these diseases worsening one another. Assessment of SDB depends on detection of sleep disruption and quality parental report. Methods. Sixteen stable PCD patients (4.9-17.2 years) and forty-two controls underwent overnight respiratory polysomnography (rPSG) and Sleep Disturbances Scale for Children (SDSC). In PCD we assessed nasal obstruction by nasal endoscopy, and lung disease by pulmonary function tests and chest high resolution computed tomography (HRCT). Results. Compared to controls, PCD had higher obstructive apnoea (4.7 versus 0.2, p 0.05), with total score and subscores of disorders in initiating and maintaining sleep, and sleep-wake transition lower than controls. Nasal endoscopy revealed chronic rhinosinusitis and adenoidal hypertrophy in 100% and 50% of PCD, respectively. Total HRCT score was 7 (range, 0-14). ODI was related to functional residual capacity (r=0.8, p=0.02), total HRCT (r=0.6, p=0.03) and peribronchial thickening scores (r=0.7, p=0.02). Mean oxygen saturation was associated with bronchiectasis severity score (r=-0.6, p=0.02). Conclusions. Parents of children with PCD may underestimate SDB. As nocturnal desaturation is associated to PCD lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.

The aim of this prospective, randomised study was to evaluate the effects of nasal douches with sodium hyaluronate on clinical and endoscopic variables, on parental perception of their child’s health-related quality of life (HR-QoL), and on parental workdays lost in preschool recurrent upper respiratory tract infections (URTIs). Children aged 2-6 years with recurrent or persistent URTIs underwent at baseline the evaluation of upper respiratory tract symptoms in the previous two weeks, and nasal endoscopy. Parents of enrolled children were assessed for self-perception of their children’s HR-QoL using a standardised questionnaire. The same variables were reassessed after a 2-week treatment with either 9 mg sodium hyaluronate plus saline solution or saline alone by nasal douches. Forty of the 48 children enrolled completed the study (22 assigned to the combined treatment). Compared to baseline, the combined treatment resulted in a significant reduction of the prevalence of children with missed daycare days (45% vs 14%, p=0.04) and of parents with workdays lost (36% vs 5%, p=0.02), and in a significant improvement of HR-QoL score (3.7 vs 2.8, p=0.004). At endoscopy, the secretion and mucosal oedema score significantly improved after the combined treatment (6 vs 2, p < 0.001), and there was a trend towards a reduction of the adenoid hypertrophy score (p=0.06). No clinical, HR-QOL or endoscopy changes were found in the saline group. In preschool children with recurrent or persistent URTIs, sodium hyaluronate by nasal douche significantly improves endoscopic features. Additional benefits include the children’s HR-QoL and daycare attendance, and parental work.

Mucopolysaccharidoses (MPSs) are lysosomal storage disorders caused by deficiency of enzymes involved in the degradation of glycosaminoglycans (GAGs). These disorders are associated with the accumulation of GAGs in tissues with organomegaly, mental retardation and short stature. Otologic and upper respiratory tract pathologies are among the earliest clinical manifestations. We analyzed 20 patients (13 male and 7 female, median age at the beginning of the observation 6 years) with MPS (35% type I, 30% type II, 20% type III, 5% type IV, 10% type VI), focusing on their otorhinolaryngologic problems and the impact of surgery on quality of life. We found ear, nose and throat manifestations in all types of MPS; in particular, recurrent otitis media was present in 30% of cases, hearing loss in 75% (mixed in 43.33%, conductive in 43.33%, sensorineural in 13.33%), adenotonsillar hypertrophy in 75%, frequent infections of the upper airway in 75% and obstructive sleep apnoea syndrome in 45% of cases. Fifty percent of patients required surgical therapy (adenotonsillectomy, adenoidectomy with insertion of middle ear ventilation tubes, tonsillectomy, tracheotomy and exeresis of vocal cord polyps). In our experience the ENT surgery reduced the frequency and severity of ear infections and relieved symptoms related to upper airway obstruction, thereby improving the quality of life in affected patients.Le mucopolisaccaridosi (MPS) sono patologie da accumulo lisosomiale causate da carenza di enzimi coinvolti nella degradazione dei glicosaminoglicani (GAGs) che si associano ad organomegalia, ritardo mentale e ridotta statura. Le patologie a carico dell'orecchio e delle alte vie respiratorie sono tra le più precoci manifestazioni cliniche. Abbiamo analizzato 20 pazienti (13 maschi e 7 femmine, età mediana all'inizio dell'osservazione 6 anni) con MPS (35% tipo I, 30% tipo II, 20% tipo III, 5% tipo IV, 10% tipo VI) focalizzando l'attenzione sui problemi ORL e sugli effetti della chirurgia sulla qualità della vita. Abbiamo riscontrato manifestazioni ORL in tutti i tipi di MPS; in particolare l'otite media era presente nel 30% dei casi, l'ipoacusia nel 75% (mista nel 43.33%, trasmissiva nel 43.33% e neurosensoriale nel 13.33%), ipertrofia adenotonsillare nel 75%, frequenti infezioni delle alte vie aeree nel 75%, sindrome da apnea notturna nel 45% dei casi. Nel 50% dei pazienti è stato necessario ricorrere alla terapia chirurgica (adenotonsillectomia, adenoidectomia con timpanocentesi e apposizione di drenaggio trans-timpanico, tonsillectomia, tracheotomia ed exeresi di polipo cordale). Nella nostra casistica la chirurgia ORL riduce la frequenza e la severità delle infezioni auricolari e attenua i sintomi da ostruzione delle alte vie respiratorie migliorando la qualità di vita dei pazienti affetti da mucopolisaccaridosi.

Tratta dei tumori dell'orofaringe