Amyloidosis of the Upper Aerodigestive Tract: Management of a Rare Disease and Review of the Literature.

Amyloidosis in the upper aerodigestive tract is a very rare disease with mainly case reports documented so far. In the pathogenesis, amyloid protein fibers are deposited in organs and tissue. In the upper aerodigestive tract, mostly localized amyloidosis occurs with unspecific symptoms, e.g., dysphagia. We conducted a retrospective multicenter study with two study centers in Germany (tertiary referral hospitals), the University Hospital of Bonn and the University Hospital of Goettingen. For a period of the last 7 years, data were analyzed and patients were recruited consecutively. 14 cases were included to the study. The most common manifestation was in the larynx (n = 11); in one case each localized amyloidosis was found in the tongue, trachea and in the pharynx. Since the majority of our cases (n = 13; 92.6%) presented with unspecific symptoms, biopsy results confirmed the diagnosis of localized amyloidosis. Resection of the lesion was only performed in patients reporting of symptoms, in asymptomatic patients only a non-invasive biopsy was done. In two patients (14.2%), in addition to the focal lesion a systemic amyloidosis was found. Amyloidosis in the upper aerodigestive tract is a rare disease. Nonetheless, every otorhinolaryngologist should be aware of this disease. When detected it is a straight forward to treat illness. The appearance of a systemic amyloidosis needs to be ruled out; thus, there is a chance to develop a multiple myeloma. Given the slow progressive character of amyloidosis, a long-term follow-up up to 10 years is inevitable.

Transoral robotic surgery for the pediatric head and neck surgeries.

Pediatric robotic surgery is a relatively new technology that has been shown to be safe and feasible for a number of pediatric procedures. Our literature analysis was performed using Pubmed database between January 2005 and December 2015, using key words: "robotic," "robotic surgery," "TORS," "pediatric," "children," "head and neck," and "da Vinci". We selected only publications in English. Eight published reports met the selection criteria. We totally found 41 patients, and the age range was between 2 months and 19 years. The cases are 16 only lingual tonsillectomy, nine base of tongue and lingual tonsillectomy, two malignant disease in the oropharynx (high-grade undifferentiated sarcoma and biphasic synovial sarcoma), one tongue base thyroglossal duct cyst, 11 laryngeal cleft cyst, one posterior glottic stenosis, and one congenital true vocal cord paralysis surgeries. One intraoperative complication was reported. No patient needed postoperative tracheotomy. Hospital duration time had a range of 1-16 days. TORS is new for pediatric patients in head and neck areas, and there were few reports. It is becoming increasingly used in head and neck surgeries and those reports above are encouraging for pediatric robotic airway surgeries in otolaryngology in the future.

[Castleman's Disease - A Case Series of a Rare Lymphoproliferative Disease]. / Morbus Castleman ­ eine harmlose Differenzialdiagnose einer Lymphadenitis colli? ­ Eine Fallserie.

Castleman's disease is a very rare and potentially severe lymphoproliferative disorder. First sign may be cervical lymphadenitis, requiring sufficient support in diagnosis and therapy by an ENT specialist. Based on a case series the current manuscript gives an overview of the symptoms, the course of disease and the therapy options. Patients with the first diagnosis of a Castleman's disease at the ENT clinic of Ulm University during the years 2011-2015 were included. The duration of symptoms, the applied diagnostic and therapeutic algorithms were evaluated. The duration of the rather weak symptoms was inhomogenous and lasted from 14 days to 14 years. After diagnostic exstirpation a hyaline-vascular type of Castelman's disease was confirmed in all cases. One of the 5 cases proved a multicentric type with an additional axillary manifestation, the others were monocentric. In all patients the diagnostic exstirpation was sufficient for therapy without need for adjuvant medication. At the time of publication all patients are in remission for at least 18 resp. 61 months. The ENT specialist encounters Castleman's disease mostly as a long-lasting swelling of cervical lymph nodes refractory to therapy and without severe concomittant symptoms. Due to potentially unfavorable outcomes a timely diagnostic lymph node exstirpation under general anesthesia is indicated. In most cases this surgical intervention represents the sufficient therapy.

[Epithelial dysfunction associated with pyo-inflammatory diseases of the ENT organs]. / Épitelial'naia disfunktsiia pri gnoino-vospalitel'nykh zabolevaniiakh LOR-organov.

The modern concept of epithelial-endothelial dysfunction and epithelial-endothelial distress-syndrome associated with pyo-inflammatory ENT diseases is presented. It has provided a basis for the analysis of the initial stages of etiopathogenesis of acute and chronic inflammation in the ENT system including the mucous and associated lymphoid tissues as well as the Pirogov-Waldeyer limphopharyngeal ring making up the first protective barrier. The leading role of dysbiosis of synanthropic microflora and endotoxins of the Gram-negative bacteria in the mechanisms of regional responsiveness of the organism to the infection and chronic endotoxic aggression is demonstrated. The regional and synthetic mechanisms underlying the interaction between the external and internal media of the organism are subjected to the analysis with special reference to those operating in epithelium. The possible variants of the outcome of these processes are considered including both the recovery and the development of chronic inflammation. It has been proved that the exhaustion of the internal reserves for the stabilization of the epithelium-associated lymphoid tissue system including the Pirogov-Waldeyer limphopharyngeal ring leads to the formation of epithelial dysfunction as the initial stage of epithelial-endothelial dysfunction and epithelial-endothelial distress-syndrome. It is concluded that the modern concept of epithelial-endothelial dysfunction and epithelial-endothelial distress-syndrome is a fundamental interdisciplinary phenomenon.

Endoscopic airway management of acute upper airway obstruction.

The conventional treatment for patients with acute upper airway obstruction is tracheostomy, which is a safe, definitive procedure in most hands. Alternatively, a debulking procedure can be considered but this requires both surgical and anaesthetic skill and expertise. However, where possible, it provides a good alternative with the advantages of removing the cause of obstruction and yielding tissue for histopathological analysis, and avoiding the need for a tracheostomy, with its associated morbidity. We evaluated all patients who presented with acute upper airway obstruction and underwent endoscopic laser debulking surgery performed by the senior author, over a three and a half year period. We recorded patient demographic data, their underlying pathologies, complication rates associated with laser debulking surgery and the conversion to tracheostomy. Thirty patients were identified, including 19 males and 11 females, with a mean age of 57.10 ± 17.20 years (19-93 years). All patients underwent debulking procedures with carbon dioxide laser under general anaesthetic. All patients had their underlying diagnosis confirmed from their debulking surgery. Twelve patients were found to have benign pathology and 18 had malignant airway obstruction. There were no laser-associated complications. One patient required conversion to emergency tracheostomy, during their debulking surgery. Endoscopic laser assisted debulking surgery has successfully been used to establish a safe airway. It allows obtaining tissue specimens, to confirm the underlying diagnosis, thus avoiding the need for further biopsies under anaesthetic. For all malignant cases, patients were subsequently able to proceed to definitive treatment. It has obviated the need for emergency tracheostomy in almost all of the cases in our patient cohort.

[Immunoglobulin G4 (IgG4)-related disease. A review of head and neck manifestations]. / Immunglobulin-G4(IgG4)-assoziierte Erkrankung. Gesichertes und Kontroverses der häufigsten Kopf-Hals-Manifestationen.

Immunoglobulin G4 (IgG4)-related disease (also known as hyper-IgG4 disease) is a recently defined emerging condition with highly heterogeneous clinicopathological features and variable disease manifestations. This disorder is characterized by unifocal or multifocal (multiorgan) involvement by tumefactive plasma cell-rich inflammatory infiltrates associated with prominent fibrosclerosis. This not uncommonly interferes with organ function resulting in diverse clinical symptoms. The autoimmune pancreatitis represents the prototype of this disease; however, to date almost all organs have been reported to be involved in this disorder. In the head and neck area several presentations of this disease may be encountered in salivary glands, lacrimal glands, thyroid gland, lymph nodes, soft tissue of the neck, ear and sinonasal tract. However, IgG4 positive plasma cells are occasionally prominent in non-specific chronic inflammatory conditions of the head and neck and the oral cavity unrelated to autoimmune diseases or systemic disorders, thus representing diagnostic pitfalls. The diagnosis of IgG4-related disease should be based on a combination of typical histological, clinical and serological findings.

[Functional and aesthetic therapy concepts of keloids in the head- and neck area]. / Funktionelle und ästhetische Therapiekonzepte von Keloiden im Kopf-Hals Bereich.

Keloids are fibroproliferative extreme variants of an impaired wound healing, developing a tumour-like growth. In the ENT area keloids arise mainly at auricle. Often caused by piercings or trauma. They grow bulging excessively over the original scar edges and adopt sometimes bizarre morphologies. The patients complain often of dysesthesias such as burning pain, severe itching and are suffering often from stigmatization. The huge number of therapy methods, for the treatment of keloids described in the literature (silicon therapy, compression, intralesional corticoids, intra- or extralesional surgical excision, kryosurgery, radiatio, Interferon- or Bleomycin-therapy) emphasize the enormous challenge for the treating doctor, particularly in facial plastic surgery.

Structured clinical assessment of the ear, nose and throat in patients with granulomatosis with polyangiitis (Wegener's).

The objective of this prospective cross-sectional study is to describe the clinical otorhinolaryngological manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) in a prospective cohort. All patients suffering from GPA seen in a tertiary centre between March 2007 and November 2008 had a detailed clinical assessment by an ENT surgeon of their ear, nose and head and neck complaints. An evaluation of whether there was disease activity and/or infection in each ENT area was made using the European Vasculitis Study Group guidelines. The number of patients assessed was 144. The proportion of female patients was 47 % (n = 69) and the median age was 57.7 years (IQ range 42.5-68.5). The prevalence of ENT involvement was 87 % (125/144). Hearing loss and abnormal tympanic membrane appearance were more common in patients with active disease and no infection (7/8 and 6/8, respectively, in active disease cf. 59/131 and 52/131, respectively, in remission). Nasal crusting was the most common nasal complaint recorded (52/144, 36 %) and bloody rhinorrhoea was the most common symptom in patients with disease activity. Rhinoscopy was highly sensitive in diagnosing disease activity (100 %). Subglottic stenosis was the most common head and neck manifestation (27/121, 22 %) and 74 % were symptomatic. In conclusion, the pattern and frequency of clinical ENT manifestations in GPA have been described in a large patient cohort. The use of tools readily available in the ENT clinic was essential to assess these patients accurately. This dataset will form the basis of an objective scoring system to measure disease activity in the ENT system.

[Measurement of tissue-shift for head neck rotation]. / Messung von Strukturverschiebungen bei Kopf-Hals-Rotation.

BACKGROUND: During neck surgery the head of the patient is located in such a position that the neck-tissue is deformed compared to the normal head-position. The consideration of this tissue deformation of the neck during head rotation is the first step to using preoperative image data for medical navigation. MATERIAL AND METHODS: The tissue shift was measured and analyzed based on MRT-image data of a subject group of 5 patients. RESULTS: The big vessels, lying contralateral to the rotation, demonstrated a larger shift than those vessels, lying ipsilateral. The shift was nonlinear to the head rotation and the shift of the V. jugularis interna was less pronounced than the shift of the A. carotis communis even though they both run in the same adventitia. In the cranial neck section, the movement of the tissue was larger than in the caudal neck section. CONCLUSION: The knowledge about tissue shifts in different head positions should promote intraoperative, minimally invasive procedures. The expectation is that such examinations facilitate the navigation in neck surgery with online calculation of tissue shifts.

[Primary mucosal tuberculosis of head and neck region: a clinicopathologic analysis of 47 cases].

OBJECTIVE: To study the clinicopathologic features, histologic diagnosis and differential diagnosis of primary mucosal tuberculosis (TB) in the head and neck region. METHODS: Forty-seven cases of primary mucosal TB of the head and neck region were studied by hematoxylin-eosin and Ziehl-Neelsen stains. The clinical and pathologic features were analyzed with review of the literature. RESULTS: The patients included 26 male and 21 female, with mean age 47.1 years (range 14-84 years). There were three sinonasal TB, 19 nasopharyngeal TB, two oropharyngeal TB, 18 laryngeal TB, four middle ear TB, one salivary gland TB and one laryngeal TB complicating laryngeal cancer. The initial symptoms were nasal obstruction, mucopurulent rhinorrhea, epistaxis, snoring, hoarseness, dysphagia, odynophagia, serous otitis, hearing loss, tinnitus, and otalgia. Physical examination result was variable, from an apparently normal mucosa, to an evident mass, or a mucosa with an adenotic or swollen appearance, ulcers, leukoplakic areas, and various combinations thereof. CT and MRI findings included diffuse thickening, a soft-tissue mass, calcification within the mass and bone destruction resembling malignancy. Histologic examination showed granulomas with a central necrotic focus surrounded by epithelioid histiocytes and multinucleated Langhan's giant cells. Acid-fast bacilli were difficult to demonstrate but found in 13/45 cases. Follow-up data were available in 42 patients. CONCLUSIONS: Primary TB arising in the head and neck mucosa is rare. It may mimic or co-exist with other conditions. The characteristic histopathology is a granuloma with central caseous necrosis and Langhans'giant cells. Identification of acid-fast bacilli and bacteriologic culture confirm the diagnosis of mycobacterial disease.

[The mechanisms of local protection of the mucous membrane of the nasal cavity and paranasal sinuses].

The objective of the preset study was to elucidate the mechanisms of local protection of the mucous membrane of the nasal cavity and paranasal sinuses based on the results of original morphological investigations. The materials for the study were obtained at autopsy on the patients who had died from the diseases unrelated to otorhinolaryngological pathology. The preparations for histological and immunohistochemical studies were obtained by routine staining techniques. Moreover, some immunohistochemical studies were carried out with the use of monoclonal antibodies against specific markers of macrophages, T- and B-lymphocytes. It was shown that the mucous membranes of the nasal cavity and paranasal sinuses contain mucociliary, lymphoepithelial, mucoglandular, and stromal-vascular (interstitial) barriers totally different in terms of structural and functional characteristics. Under normal conditions, these barriers serve to protect mucosa at different levels. At the same time, functional or structural disturbance of one of the superficially located mechanisms of local protection induces activation of another.

[The prevalence of chronic pathology of ENT organs and oral mucosa in the HIV-infected patients depending on their immune status].

The objective of the present study was to estimate the prevalence of chronic pathology of ENT organs and mucous membrane of the oral cavity in the HIV-infected patients depending on the number of cells containing CD4 markers in 1 mcl of blood plasma. It was shown that the prevalence of chronic pathology of ENT organs and oral mucosa in the HIV-infected patients depends on the number of cells carrying CD4 markers in 1 mcl of blood plasma. This pathology was found in 64.6% of the patients at immunological stage I of infection with human immunodeficiency virus (over 500 CD4 per 1 mcl plasma), in 73.4% of the patients at stage II of HIV infection (200-499 CD4/1 mcl), and in 90.7% of the patients at stage III of HIV infection (less than 200 CD4/1 mcl). The frequency of individual nosological forms of ENT disease at different stages of infection with human immunodeficiency virus is discussed. Certain diseases pathognomonic for HIV-infected adult subjects were identified including adenoid vegetations, cheilitis, oral hairy leukoplakia, and oropharyngeal candidiasis. Combined chronic pathology of ENT organs as well as certain clinical features of the above diseases may suggest diagnosis of immunodeficiency and require the assessment for the HIV status.

Head and neck cancers manifested as deep neck infection.

The incidence of head and neck cancers in patients with an initial presentation of deep neck infection is unclear and may be underestimated. Thus, the aim of this study was to assess the incidence of head and neck cancers initially manifested as deep neck infection. Also, the possible risk factors and pathophysiology are discussed. This study was a retrospective medical chart review in a tertiary referral center. A total of 81 consecutive patients admitted with a diagnosis of deep neck infection over a 46-month period were analyzed. The demographic data, physical examinations, laboratory findings, radiographic studies, and pathology report were analyzed. Among the 81 deep neck infection patients, head and neck cancers were histologically demonstrated in four patients (4.9%) with the initial symptom of a painful neck mass. The incidence of head and neck cancer initially manifested as deep neck infection was found to increase in patients aged over 40 years (6.7%; 3/45 vs. 2.8%; 1/36). A detailed history of all patients with deep neck infection should be taken. Furthermore, endoscopic examination, thyroid examination and routine pathological examination should be performed, especially in those aged over 40. Also, careful explanation to the patient and his/her family about the possibility of underlying head and neck cancer (incidence 1-5%) may be needed. If the neck swelling diminishes, but does not disappear completely after full course of antibiotics, repeated fine needle aspiration, endoscopy, or image study should be considered.

[Intraoperative frozen section diagnosis of head and neck tumors: possibilities, limitations, pitfalls and tips for the daily practice]. / Intraoperative Gefrierschnittdiagnostik von Kopf-Hals-Tumoren : Möglichkeiten, Grenzen, Fallstricke und Ratschläge für die tägliche Praxis.

Intraoperative consultation (synonym: frozen section diagnostics) has increasingly gained significance for the daily practice in head and neck surgery. The main aim of this investigation method which is usually associated with much stress and effort is to facilitate an optimal and timely oncological surgical treatment of neoplastic diseases with a minimum rate of postoperative functional disturbance. In order to achieve this purpose pathologists are expected to deliver as much correct information as possible to accurately influence intraoperative surgical decisions. At the same time this aim should be reached without significantly and unnecessarily increasing the workload for the pathology laboratory and without significantly inducing tissue artifacts. This would otherwise negatively influence the tissue quality for permanent section examination and consequently the overall quality of diagnosis and tumor staging. Thus, the quality and efficacy of frozen sections span a spectrum with the highest quality having the least possible false negative rate on the one hand and a false positive result of approximately zero on the other hand. Sticking to this approach would result in a high positive impact on the surgical treatment of a variety of neoplastic diseases and help to minimize or even eliminate the rate of medicolegal consequences.

Long-term damage to the ENT system in Wegener's granulomatosis.

The objectives of the study are to describe long-term ENT damage and assess risk factors in patients with newly diagnosed and treated Wegener's granulomatosis (WG) using the vasculitis damage index (VDI). Data from four randomised controlled trials carried out by the European Vasculitis Study Group was used. Patients newly diagnosed with WG with complete data at 5 years were included. Patients enrolled into the trials without 5-year data were excluded. Total and ENT VDI scores were recorded at 12 months and after at least 5 years. Logistic regression models were constructed to assess risk factors using total ENT and overall VDI score over the follow-up period, the proportion of patients with increased VDI score and the presence or absence of damage as the main outcomes. One hundred and thirty-eight patients were included. Ninety patients (65%) had long-term damage and 81% of these (73/90) developed some damage in the first 12 months. Positive ENT activity score (BVAS) at baseline and relapses were associated with higher ENT VDI scores long-term (OR = 6.90, 95% CI 2.01-23.75; OR = 2.65, 95% CI 1.20-5.82). Increasing BVAS score showed a trend towards lower VDI scores (OR = 0.93, 95% CI 0.88-0.99). Only ENT relapses and number of relapses were associated with an increase in VDI over time (OR = 8.38, 95% CI 3.10-22.68; OR = 1.79, 95% CI 1.24-2.58). In conclusion, most of the ENT damage in these patients was accrued within 12 months of diagnosis. We have shown an association between later ENT damage and the presence of ENT disease at baseline; lower initial BVAS and higher rate of disease relapse.

[Optical coherence tomography in otolaryngology]. / Az optikai koherenciatomográfia alkalmazási lehetoségei a fül-orr-gégészetben.

Optical coherence tomography is an imaging technique based on coherence interferometry. It is used in many medical fields due to its non-invasive imaging capabilities with micrometer resolution. The aim of the authors was to review the applicability of the optical coherence tomography in otolaryngology. Literature data and their own studies show that optical coherence tomography is a reliable method for identifying and targeted biopsy of inflammated, pre-malignant or cancer tissue in human laryngeal and pharyngeal mucosa. Another emerging field for optical coherence tomography is diagnosis of middle ear diseases, especially stapes ankylosis and cholesteatoma.

[Head and neck tuberculosis - still current problem in ENT practice]. / Gruzlica narzadów glowy i szyi - wciaz aktualny problem w praktyce laryngologicznej.

INTRODUCTION: It is estimated that between XVI and XIX century one fourth of European population died of tuberculosis. Nowadays tuberculosis is still one of the most important infectious diseases. In 2009 estimately 9 million new cases were registered worldwide, mortality due tuberculosis reached 1.5 million. In Poland extrapulmonary tuberculosis is rare (7% of overall morbidity) and most commonly affects pleura, lymph nodes, bones, joints and genitourinary system. Head and neck tuberculosis is rare and causes many diagnostics problems. METHODS: Retrospective analysis of case histories of patients admitted to ENT Department of Silesian Medical University in Katowice. RESULTS: Between 1993 and 2010 four cases of head and neck tuberculosis were diagnosed in our Clinic. Two of patients were admitted to the hospital with symptoms of laryngeal tumor such as difficulty in swallowing. During direct laryngoscopy tissue specimens were taken. Examination of the third patient showed tumor located below left angle of mandibule. During superficial parotidectomy tumor was removed. In the fourth registered case tuberculosis manifested as tumor of nasopharynx. In every case which is mentioned above pathology reports revealed epithelioid cell granulomas with caseous necrosis typical for tuberculosis. Patients underwent tuberculostatic treatment. CONCLUSION: Rare occurrence and lack of characteristic symptoms of head and neck tuberculosis often lead to misdiagnosis. Histopathological examination is the most important diagnostic procedure. Microbiological examination is difficult in extrapulmonary tuberculosis, because of low concentration of pathogens in specimens. Tuberculostatic therapy is the leading method of treatment in every case of tuberculosis.

Otorhinolaryngology Manifestations of Systemic Illness.

Ear-nose-throat (ENT) manifestations are among the most frequently observed clinical features of systemic illnesses. The patients often present with overt findings of head and neck lesions such as salivary gland swelling or lymphadenopathy. In contrast, patients may present with covert findings of auditory, nasal, and laryngeal symptoms that are less obvious and are often overlooked. Therefore, clinicians should have a high index of suspicion to identify the underlying disease. Early recognition and prompt treatment or referral to specialists may prevent morbidity and mortality. This article discusses various systemic illnesses with ENT manifestations that are commonly encountered.