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AIMS Patients with the Pendred syndrome suffer very often from a hearing loss. They may be good candidates for a cochlear implantation, but unfortunately, due to the fluctuating character of the hearing loss, they may escape such indication. In the study, we compared speech production and speech acquisition in 2 groups of implanted patients: those with the Pendred syndrome, and standard non-syndromic patients. METHODS Ten patients with Pendred syndrome were analyzed for speech perception and production. The control group consisted of 41 non-syndromic implanted patients. All implantees were scored according to speech perception, speech production, and the sum of both. The data were statistically analyzed. RESULTS No statistical difference was found in language acquisition and production in implantees with Pendred syndrome when compared to non-syndromic patients with cochlear implants. Nor there was any difference in speech production and acquisition between the 2 compared groups regarding surgical age, time elapsed after surgery, or age during the testing. CONCLUSION In this study evaluating language and speech production and acquisition, patients with Pendred syndrome who underwent cochlear implants show comparable results to their implanted peers with deafness of a different etiology.

Fabry disease (FD) is a lysosomal storage disorder caused by pathogenic mutations in the alpha-galactosidase A (AGALA) encoding gene region. This rare disease affects several organs including the cochlea-vestibular system. Tinnitus and sensorineural hearing loss (SNHL) are reported among otoneurological symptoms. Early and correct diagnosis of FD is important with a view to available therapy. The aim of the study was to screen for alpha-galactosidase deficiency in men with tinnitus/SNHL. A prospective multicentric study including consecutive patients with SNHL confirmed by tone audiometry or tinnitus evaluated (10/2016-8/2019). The diagnosis of AGALA deficiency was done by dry blood spot method using a threshold of 1.2 µmol/l/h. Only men aged 18-60 were included. 181 patients were subject to evaluation. SNHL was reported in 126 (70%) patients, 50 (28%) patients had unilateral, 76 (42%) patients had bilateral SNHL. Tinnitus was found in 161 (89%) patients, unilateral in 96 (53%) and bilateral in 65 (36%) patients. Suspected FD was not detected in any patient; alpha-galactosidase The AGALA values ranged 1.5-8.8 µmol/l/h, an average of 3.4 µmol/l/h. None of the 181 patients participating in the study had AGALA levels below the threshold 1.2 µmol/l/h. The occurrence of tinnitus and sensorineural hearing loss in men appears to be an irrelevant clinical sign for FD systematic screening.

Objective: Oropharyngeal/laryngeal carcinoma are common cancers of the upper aerodigestive system. Human papillomavirus (HPV) is described as the most frequent in the cancer of unknown primary. The presence of Helicobacter pylori (HP) in the oral cavity is discussed in some papers. The aim of study: To analyze the incidence of HPV and HP in oropharyngeal/laryngeal cancer persons versus persons with chronic tonsillar inflammation and healthy persons. Methods: The samples were taken in three groups: (1) tissue of oropharynx/larynx cancer (103 specimens); (2) tissue of palatine tonsils (85 specimens); and (3) healthy control group (50 specimens). We analyzed the presence of HP (PCR) and HPV genomic DNA (Sacace HPV High-Risk Screen Real-TM Quant) in the samples. Results: HP was detected in 86 samples (83.5%) and high-risk HPV in 62 samples (60.2%). We found a very high incidence of HP. In the cancer group, HP was detected in 82.5% cases and HPV positivity in 57.8%. In total, 7.2% of the cancer patients were negative for HP and HPV together. In turn, 53.6% of the cancer patients were positive for HP and HPV together. Four cases (4.2%) were positive for HPV only. VacA positivity was detected in 82 (79.6%) of the cancer cases and VacA negativity in 21 (20.4%) if the cancer cases. The incidence of HP in chronic inflammation (n = 85) was 65 cases (76.5%) and the incidence of HPV was 38 cases (44.7%). VacA positivity was detected in 59 (69.4%) of the chronic inflammation cases and VacA negativity was found in 26 (30.6%) of the chronic inflammation cases. Regarding the control group, we found HP positivity in 5 cases (11.1%) and HPV positivity in 19 cases (42.2%). There was VacA positivity in 6 cases (50.0%) of the control group. Statistically significantly lower prevalence of HP (p < 0.001) and HPV (p = 0.006) was found in the control group. Conclusions: We suggest that the palatine tonsils are colonized by HP. In our study, HP was present in oropharyngeal cancer in more cases in comparison with HPV infection. The presence of VacA from HP can have an influence on the human epithelial and immune cells’ regulation ways. Our results do not support idea that the CagA-positive HP is a primary carcinogen in oropharyngeal area.

Background Clinical examination, including pre- and postoperative assessment of olfaction function, should be included in evaluating surgical outcomes in patients with pituitary adenomas. Most of the studies are lacking assessment of olfactory function. Methods A prospective study of 143 patients who underwent surgical resection of pituitary adenomas from January 2014 to December 2017 was performed. Data on clinical presentation, pre- and postoperative neurologic, endocrinologic, and ophthalmologic examinations, complications, and follow-up outpatient examinations were recorded. Olfactory function was assessed using the Sniffin' Sticks odor identification test preoperatively, postoperatively (3 months), and 1 year after surgery. Results Normosmia was present preoperatively in 93.7% of patients, postoperatively in 95.8% of patients, and in 95.1% 1 year postsurgery. Hyposmia was present preoperatively in 4.2% of patients, postoperatively in 2.1% of patients, and in 1.4% 1 year after surgery. Anosmia was present preoperatively in 2.1% of patients, postoperatively in 2.1% of patients, and in 3.5% 1 year after surgery. In patients with preoperative normosmia, postoperative hyposmia and anosmia were present in 1.5% of patients. There were no differences according to age, sex, size, or type of pituitary adenoma. Conclusions Assessment of olfactory function should be included in the analysis of pituitary adenoma surgery results. This prospective study showed low risk of olfaction deterioration if an endoscopic endonasal approach is used without any mucosal flaps for skull base reconstruction. Further studies that include objective olfaction assessment are warranted.

Introduction: Total thyroidectomy (TT) is one of the most common surgical endocrine surgeries. Voice impairment after TT can occur not only in patients with recurrent laryngeal nerve (RLN) transient paralysis, but also in cases of normal vocal cord mobility. Aim: To compare voice limits using a speech range profile (SRP) in patients before and 14 days after TT and to investigate the influence of the early results of voice quality after TT on the personal lives of patients. We focused on the perception of voice change before and shortly after TT. Materials and methods: A retrospective study, in the period 2018–2020, included 65 patients aged 22–75 years. We compared two groups of patients: group I (n = 45) (without RLN paresis) and group II (n = 20) (with early transient postoperative RLN paresis). Patients underwent video flexible laryngocopy, SRP, and Voice Handicap Index-30 (VHI-30). Results: In group I, the mean values of Fmax (maximum frequency) and Imax (maximum intensity) decreased in women (both p = 0.001), and VHI-30 increased (p = 0.001). In group II after TT in women, the mean Fmax and Imax values decreased (p = 0.005 and p = 0.034), and the frequency range of the voice was reduced from 5 to 2 semitones. The dynamic range of the voice was reduced by 3.4 dB in women and 5.1 dB in men.VHI-30 increased (p = 0.001). Conclusion: The study documented a worsening of the mean values of SRP, VHI-30, and voice parameters of patients in group II. Voice disorders also occurred in group I without RLN paresis. Non-paretic causes can also contribute to voice damage after TT. SRP and VHI-30 are suitable tools for comparing voice status in two groups of patients, including those with dysphonia. Our data support the claim that the diagnosis of a thyroid cancer does not necessarily imply a higher postoperative risk of impaired voice quality for the patient.

Background: Acute acoustic trauma (AAT) ranks, among others, as one common cause of inner ear function impairment, especially in terms of military personnel, who are at an increased exposure to impulse noises from firearms. Aim of this study: 1. We wanted to demonstrate whether early treatment of AAT means a higher chance for the patient to improve hearing after trauma. 2. We find the answer to the question of whether hyperbaric oxygen therapy (HBO2) has a positive effect in the treatment of AAT. Methods: We retrospectively analyzed data for the period 2004–2019 in patients with AAT. We evaluated the therapeutic success of corticosteroids and HBO2 in a cohort of patients with AAT n = 108 patients/n = 141 affected ears. Results: Hearing improvement after treatment was recorded in a total of 111 ears (79%). In terms of the data analysis we were able to ascertain, utilizing success of treatment versus timing: within 24 h following the onset of therapy in 56 (40%) ears—54 (96%) ears had improved, within seven days following the onset the therapy was used in 55 (39%) ears—41 (74%) ears had improved, after seven days the therapy started in 30 (21%) ears—16 (53%) ears had improved. Parameter latency of the beginning of the treatment of AAT was statistically significant (p = 0.001 and 0.017, respectively). The success of the medical protocols was apparent in both groups—group I (treated without HBO2): n = 61 ears, of which 50 (82%) improved, group II (treated with HBO2): n = 73 ears, of which 56 (77%) improved. Group II shows improvement at most frequencies (500–2000 Hz). The most serious sensorineural hearing loss after AAT was at a frequency of 6000 Hz. Conclusion: Analysis of our data shows that there is a statistically significant higher rate of improvement if AAT treatment was initiated within the first seven days after acoustic trauma. Early treatment of AAT leads to better treatment success. HBO2 is considered a rescue therapy for the treatment of AAT. According to our recommendation, it is desirable to start corticosteroid therapy immediately after acoustic trauma. If hearing does not improve during the first seven days of corticosteroid therapy, then HBO2 treatment should be initiated.

Background Clinical examination, including pre- and postoperative assessment of olfaction, is essential in evaluating surgical outcomes in patients with olfactory groove meningiomas (OGMs). A review of a recent series revealed a lack of assessment of olfaction in most of the studies. Tests determining olfactory detection should be used to reveal olfactory dysfunction. Specialized examination techniques (e.g., electro-olfactography, olfactory evoked potentials, and functional magnetic resonance imaging) are currently used in research. Methods Prospective analysis of 13 patients who underwent surgical resection of OGMs from December 2013 to December 2017 was performed. Data on clinical presentation, pre- and postoperative neurologic examinations, complications, recurrences, adjuvant treatment, and follow-up outpatient examinations were recorded. Olfactory function was assessed using the Sniffin' Sticks odor identification test preoperatively, postoperatively, and 1 year after surgery. Results All the meningiomas were resected via unilateral craniotomy, and gross total resection was achieved in all cases. Surgery-related permanent morbidity was 7.7% and overall mortality 0%. For the eight patients with preoperative normosmia, five remained normosmic (62.5%), one deteriorated to hyposmia (12.5%), and two deteriorated to anosmia (25%). For the two patients with preoperative hyposmia, one remained hyposmic and one deteriorated to anosmia. For the three anosmic patients, two remained anosmic, and one improved to hyposmia.The intact olfactory function preoperatively was associated with a better olfactory outcome. Overall, 62.5% of these patients remained normosmic, and none of the hyposmic or anosmic patients normalized their olfaction.Higher meningioma volume is associated with worse olfactory function before surgery (normosmia in 16.7% versus 100.0% in less voluminous) and following the surgery (normosmia in 16.7% versus 57.1% less voluminous).The unilateral surgical approach enabled the anatomical preservation of the contralateral olfactory nerve in 76.9% of our patients. Functional normosmia was achieved in 50% and hyposmia in 30% of these cases. Conclusions Assessment of olfactory function is both vital in preoperative decision making (surgical approach, radicality of resection) and when evaluating surgical outcome. Preoperative normosmia seems to be the most important prognostic factor for functional olfactory outcome. In normosmic patients the olfaction was preserved in 62.5% of cases. Moreover, higher meningioma volume is associated with worse olfactory function before and following the surgery. The greatest advantage of the unilateral surgical approach is anatomical preservation of the contralateral olfactory nerve with a satisfactory functional outcome. These results support a proactive approach, with early surgical resection using a unilateral approach even in cases with less voluminous OGMs that enables the preservation of olfactory function in a significant proportion of patients.

Background and Aim. The otolaryngologist often meets with fractures of the orbital floor. The most serious complication is diplopia, arising as a result of herniation of the orbital contents, with or without fixation of the inferior rectus muscle. The aim of our work was to create a mathematical model to calculate the volume of prolapsed soft tissue of the orbit in blow-out fractures, as a factor in deciding on the need for surgical treatment. Patients and Methods: In a retrospective study (2007-2013), we evaluated 80 patients with blow-out fractures, divided into two equal groups: 40 conservatively treated and 40 surgically treated patients. We created the model by measuring the fracture lines and herniation of the orbital soft tissues in the coronal and sagittal sections from CT images, equivalent to half the volume of a rotating ellipsoid. Results: According to the proposed model, posterior and anterior fractures with a prolapse volume above 500 mm3, and anteroposterior fractures with a volume over 1400 mm3, are indicated for surgery. Conclusion: The volume of prolapsed soft tissue relative to the location of the fracture is the main indicator for selecting the best treatment procedure immediately after injury.

Head and neck paragangliomas Paragangliomas and pheochromocytomas are rare, mostly benign neuroendocrine tumors, which are embryologically derived from neural crest cells of the autonomic nervous system. Paragangliomas are essentially the extra-adrenal counterparts of pheochromocytomas. As such this family of tumors can be subdivided into head and neck paragangliomas, pheochromocytomas and thoracic and abdominal extra-adrenal paragangliomas. Ten out of fifteen genes that contribute to the development of paragangliomas are more susceptible to the development of head and neck paragangliomas when mutated. Gene expression profiling revealed that pheochromocytomas and paragangliomas can be classified into two main clusters (C1 and C2) based on transcriptomes. These groups were defined according to their mutational status and as such strongly associated with specific tumorigenic pathways. The influence of the main genetic drivers on the somatic molecular phenotype was shown by DNA methylation and miRNA profiling. Certain subunits of succinate dehydrogenase (SDHx), von Hippel-Lindau (VHL) and transmembrane protein 127 (TMEM127) still have the highest impact on development of head and neck paragangliomas. The link between RAS proteins and the formation of pheochromocytoma and paragangliomas is clear due to the effect of receptor tyrosine-protein kinase (RET) and neurofibromatosis type 1 (NF1) in RAS signaling and recent discovery of the role of HRAS. The functions of MYC-associated factor X (MAX) and prolyl hydroxylase 2 (PHD2) mutations in the contribution to the pathogenesis of paragangliomas still remain unclear. Ongoing studies give us insight into the incidence of germline and somatic mutations, thus offering guidelines to early detection. Furthermore, these also show the risk of mistakenly assuming sporadic cases in the absence of definitive family history in head and neck paragangliomas.

Olfaction is an important sensory input that obviously affects many daily activities. However, olfactory dysfunction (hyposmia and anosmia) leads to a pronounced decrease in quality of life. Surprisingly, little attention has been paid to olfactory changes after transsphenoidal surgery for pituitary tumors. In this review, we summarize current knowledge of the effects of transsphenoidal pituitary surgery on olfaction and compare different surgical techniques. Based on selected studies, the endoscopic approach, in comparison with the microscopic approach, seems to be superior in terms of preservation of olfactory function, although the quality of data from these studies is generally poor. The best results were observed when the endoscopic approach was used without harvesting of the nasoseptal flap.

Idiopathic sensorineural hearing loss (ISNHL) is a hearing impairment that occurs suddenly with onset over a period of 24 to 72 hours, affecting at least three adjacent frequencies with hearing loss of 30 decibels or more, caused by damage to the inner ear or auditory nerve without clear reasons. The treatment of ISNHL is still a hot topic. In a retrospective study, we examined 88 patients with ISNHL (2008-2013). The objective of the study was to compare the effects of two different types of therapy: vasodilator infusion treatment combined with hyperbaric oxygen (HBO₂) therapy vs. vasodilator infusion therapy alone. We investigated the correlation between the type of audiometric curve and hearing gain after treatment, the correlation between the age and the effectiveness of treatment, plus the effect of therapy on tinnitus. We found a statistically significant (p⟨0.0001) therapeutic effect in both treatment modalities: 55% with HBO₂ therapy vs. 65% without HBO₂. The difference between the two methods is not statistically significant. The best effect of treatment was observed in patients with apicocochlear hearing impairment. The age of patients in our group had no statistically significant relevance. Alleviation of tinnitus was observed mainly in patients with apicocochlear type of hearing loss.

Backgroung and Aim. This work builds on our publication on the subject of creating a mathematical model for calculating the volume of proplaped soft tissue of the orbit in blow-out fractures, which aids us greatly in our choice of the most effective treatment immediately post-accident. Patients and Methods: In this prospective study (2014 - 2016) we treated 29 patients with blow-out fractures. 18 (62%) were treated conservatively and in 11 (38%) we proceeded surgically. We decided whether surgical or non-surgical therapy was appropriate on the basis of clinical ENT, eye examination and the total volume of prolapsed orbital soft tissue. All procedures were perfomed by the same operating team with a uniform subciliary approach and using PMR splints adapted to the correct size and shape. Results: On the basis of the mathematical model we reassessed findings in 2 patients: in one we decided against a surgical solution and in the other a surgical approach was indicated. All 18 patients treated conservatively, fully recovered and are free of diplopia. The 11 operated patients are also free of diplopia, only 1 patient (3%) displays clinically insignificant postoperative diplopia in extreme posisitons when looking upwards. Conclusion: With proper selection of the optimal treatment, the rate of complete disappearance of diplopia and fully preserved motility of the eyeball ranges from 91 to 97%. Surgical treatment of orbital floor fractures is important, mainly to minimise persistent post-traumatic diplopia which significantly reduces a patient's quality of life.

Objectives The aim of this pilot study was to investigate an association between laryngopharyngeal reflux detected by combined multiple intraluminal impedance and pH monitoring and Helicobacter pylori in adenoid hyperplasia detected with real time polymerase chain reaction (PCR). Methods The study group consisted of 30 children (median age 5.34 years) with extraesophageal symptoms of gastroesophageal reflux disease with adenoid hyperplasia. All children underwent adenoidectomy with subsequent PCR detection of H. pylori DNA in the tissue and multiple intraluminal impedance and pH monitoring. The most proximal impedance sensor was located 1 cm caudal to the entrance of the oesophagus. Results We found significant differences in the number of reflux episodes among patients with PCR positivity (median 35) and negativity (median 0) of H. pylori (p-value of Mann–Whitney U-test 0.0056). Patients with PCR positivity of H. pylori had significantly more reflux episodes reaching the upper oesophageal sphincter (p-value of Mann–Whitney U-test 0.023). The absence of reflux episode was the only independent factor for PCR negativity of H. pylori in the multiple logistic regression model. Conclusions These results support the hypothesis that reflux episodes reaching the upper oesophageal sphincter may play an important role in the transmission of H. pylori into lymphoid tissue of the nasopharynx and thus may contribute to adenoid hyperplasia in children.

Radiofrequency surgery is a widely used modern technique for submucosal volume reduction of the tonsils. So far there is very limited information on morphologic changes in the human tonsils after radiofrequency surgery. We performed histopathological study of tonsillectomy specimens after previous bipolar radiofrequency induced thermotherapy (RFITT). A total of 83 patients underwent bipolar RFITT for hypertrophy of palatine tonsils. Tonsil volume reduction was measured by 3D ultrasonography. Five patients subsequently underwent tonsillectomy. Profound histopathological examination was performed to determine the effect of RFITT on tonsillar architecture. All tonsillectomy specimens showed the intact epithelium, intact germinal centers, normal vascularization, and no evidence of increased fibrosis. No microscopic morphological changes in tonsillectomy specimens after bipolar RFITT were observed. RFITT is an effective submucosal volume reduction procedure for treatment of hypertrophic palatine tonsils with no destructive effect on microscopic tonsillar architecture and hence most probably no functional adverse effect.

Sialendoscopy (SE) represents nowadays one of the standard diagnostic and therapeutic procedures in the treatment of major salivary glands lithiasis. We know from experience that it is successful only in small percentage of patients, when used in monotherapy. However, it represents an indispensable part of all of the combined minimally invasive gland-preserving treatment techniques, the success rate of which is around 90%. In this work, we focused on the role of sialendoscopy in the treatment of patients with larger inflamed fixed stones in glandula parotis. We conducted a total of 364 sialendoscopy procedures in 332 patients on our site. We have confirmed lithiasis as a cause of salivary gland obstruction in 246 (74%) patients. In 9 patients there was larger, single, or multiple inflamed fixed lithiasis of glandula parotis. In this subgroup of patients endoscopically assisted sialolithectomy from external mini-incision has become the method of choice. In 9 of the 9 (100%) cases we have achieved complete elimination of stones, and in 8 of the 9 (89%) cases we have achieved complete elimination of complaints. Sialoendoscopically assisted sialolithectomy of glandula parotis from external mini-incision has proved to be highly effective technique to eliminate stones with minimal complications.

Primary hyperparathyroidism in children is very rare. It is caused by overproduction of parathormone by a pathologically changed parathyroid gland. We carried out a retrospective study in 10 patients (age 10-17 years) who had surgical treatment between 1996 and 2007. The main signs of primary hyperparathyroidism were urolithiasis, nephrolithiasis, nephrocalcinosis and bone resorption, as well as non-specific signs such as fatigue, torpidness and muscle weakness. Patients were examined using sonography, MIBI-scintigraphy, CT and MRI. Calcium was measured before and after surgery; parathormone was monitored postoperatively. Surgery was curative in nine patients; reoperation was necessary in one patient because an ectopic parathyroid gland was not detected during the primary operation. Other major complications were not observed. Removal of pathologically changed parathyroid glands offers definitive and safe treatment of primary hyperparathyroidism in children. Special care should be taken if an ectopic parathyroid gland is suspected.

The incidence of well-differentiated low-risk thyroid cancer have increased globally over the last three decades. Thyroid cancer treatment relates to a suitable surgical procedure and the use of adjuvant radio-iodine therapy in selected patients. Evaluation of prognostic factors and risk stratification are critical for determining appropriate treatment. Survival of patients with low-risk thyroid cancer is excellent. Appropriate choice of medical treatment resulted in full recovery in most patients. Relapse risk increases with the size of the primary tumor, along with the findings of the risk factors in men.Our study included a total of 1 980 patients in whom were diagnosed T1a and T1b tumors between the years 2003 to 2012. The population included 1 675 women (84.6 %) of average age of 45.22 years and 305 men (15.4 %) of average age of 50.0 years. The bulk of the file represented papillary carcinomas (1 868; 94.4 %), and smaller group of follicular carcinomas (112; 5.6 %). Patients were divided into four groups according to tumor size. Patients were evaluated according to risk factors: unifocality no other risk factors, multifocality - more bearings in thyroid tumor, metastases in regional lymph nodes, distant metastases or combination of risk factors. Group A: In the monitored set of 678 patients with papillary and follicular microcarcinoma up to 5 mm, during histological input, the findings revealed one bearing (unifocal type of cancer) in 566 patients. Multifocality was found in 112 patients, local nodal metastasis were demonstrated in 24 cases and pulmonary metastasis was discove-red in 1 case. Group B: In this group there were 576 study patients with papillary and follicular microcarcinoma size of 5-10 mm. Histological findings were captured input one bearing carcinoma in 434 patients, 142 patients with multifocality, in 53 cases of local nodal metastasis, and 1 case of bone metastases. Group C: In this group there were 467 study patients with papillary and follicular microcarcinoma size 10-15 mm. The histological initial finding captured unifocal type of cancer in 344 patients, multifocality in 123 patients, in 45 cases local metastases and in 3 cases of pulmonary metastases. Group D: 259 patients were monitored in this group with breast size 16-20 mm. At the initial finding was captured one bearing cancer in 188 patients, multifocality in 71 patients, in 24 cases evidence of local metastases and 2 patients had a case of distant lung metastases. In patients in whom risk factors were found, radioiodine treatment was indicated. This included 744 patients. In this group of patients after a year or more, relapse was observed in 74 patients (9.94 %). In 1 236 patients who did not undergo radioiodine treatment, there was a relapse in 49 patients (3.96 %).Based on our analysis, it is necessary to stratify the risk of relapse according to risk factors. In case of missed radioiodine therapy in patients with low-risk cancer without confirmed risk factors, it is also necessary to have regular clinical, laboratory and ultrasound examination. It is important to distinguish patients with risk factors that may contribute to disease recurrence. Only in this way, on one hand we prevent excessive treatment of patients with low-risk thyroid cancer which leads to increased cost of health care, and on the other hand prevent reduced level of care for patients with an increase in relapses.

Summary Authors present 114 children and adolescents of the total 3865 patients that underwent surgery on the Department of Otorhinolaryngology and Head and Neck Surgery, 1st Faculty of Medicine, Charles University, Prague, Department of Otorhinolaryngology of IPVZ, Prague because of a thyroid disease in years 1991–2000. The male/female ratio in the group was 1:6.5, 16 (13.9%) males and 99 (86.1%) females. For non-malignant disease, the male/female ratio was 1:6.5 and for malignant disease, it was 1:5.4. Thyroid surgery was indicated for non-malignant disease in 82 (71.9%) cases – 11 males and 71 females. Most frequently it was indicated because of Graves–Basedow and Hashimoto toxicosis – in 39 children (34.2%), in 5 children (4.4%) for toxic adenoma. Because of the polynodular goiter surgery was indicated in 13 cases (11.4%), in 22 cases (19.3%) for mononodular goiter. Three girls (2.6%) were recommended for surgery because of Hashimoto thyreoiditis and for mechanic syndrome in diffuse goiter one girl (0.9%). Malignant tumor was found in 32 children (28.1%) – 5 males and 27 females. Most frequent histological type was papillary cancer – in 25 cases (21.9%). Follicular cancer was diagnosed in four cases (3.5%) and medullar cancer in three cases (2.6%). TNM classification in children and adults is summarized in Table 1. Surgery was carried out in two children with distant metastases (lung localization). In both children the pre-disease stage lasted more than 12 months. The first manifestation of the disease was a finding of enlarged and palpable nodes on the neck. The authors summarize indications for surgery of thyroid diseases in children and adolescents. A treatment of the thyroid gland is a teamwork. Indication for surgery is carried out by a pediatric endocrinologist in cooperation with a surgeon specialized in thyroid surgery. The possible identification of the recurrent laryngeal nerve by a surgery microscope is an unquestionable advance. Children and adults should be centralized into health-care centers capable not only of surgery, but also of endocrinology care. In children with a malignant disease there is a need for cooperation with a pediatric oncologist and a following oncological treatment on a nuclear medicine department. The authors summarize the information about today’s thyroid surgery care possibilities for children and adolescents. They point out the differences in indications and the decision about extent of surgery in children and adolescents.

We report a case of thyroid abscess in a 17-year-old girl. She was presented with swelling and pain in the region of the left thyroid lobe. The diagnosis was confirmed by ultrasonography and fine needle aspiration. After incision, evacuation of the pus and antibiotic treatment manifestations of disease disappeared. Barium swallow did not prove any hypopharyngeal fistula. The girl is now without any problems for 3 years. The patient had infectious mononucleosis 2 months prior development of the thyroid abscess. We contemplate that it may had changed the immunological state that facilitated this rare inflammation.

Minimally invasive video-assisted thyroidectomy (MIVAT) and minimally invasive nonendoscopic thyroidectomy (MINET) represent well accepted and reproducible techniques developed with the main goal to improve cosmetic outcome, accelerate healing, and increase patient’s comfort following thyroid surgery. Between 2007 and 2011, a prospective nonrandomized study of patients undergoing minimally invasive thyroid surgery was performed to compare advantages and disadvantages of the two different techniques. There were no significant differences in the length of incision to perform surgical procedures. Mean duration of hemithyroidectomy was comparable in both groups, but it was more time consuming to perform total thyroidectomy by MIVAT. There were more patients undergoing MIVAT procedures without active drainage in the postoperative course and we also could see a trend for less pain in the same group. This was paralleled by statistically significant decreased administration of both opiates and nonopiate analgesics. We encountered two cases of recurrent laryngeal nerve palsies in the MIVAT group only. MIVAT and MINET represent safe and feasible alternative to conventional thyroid surgery in selected cases and this prospective study has shown minimal differences between these two techniques.

Narrow band imaging (NBI) HDTV (high definition television) magnifying endoscopy is considered to be superior for the accurate display of the microvascular patterns of superficial mucosal lesions. Observation of changes in intraepithelial papillary capillary loops (IPCL) can help distinguish benign from malignant lesions as part of an “optical biopsy.” However, IPCL changes in papillomas may be mistaken for spinocellular cancer (SCC). The aim of the study was to determine whether observing microvascular changes alone is sufficient for discriminating between laryngeal SCC and papillomatosis. An additional aim was to identify associated characteristics that could clarify the diagnosis. The study included 109 patients with a suspected laryngeal tumor or papilloma. HDTV NBI magnifying endoscopy was performed during direct laryngoscopy. It was possible to visualize IPCL changes in 82 out of 109 patients (75.2%). In 71 (86.6%) patients, the diagnosis was correctly determined. In 4 (4.9%) cases, the diagnosis of SCC was expressed on the basis of finding pathologic IPCL, but histology did not demonstrate malignancy. To achieve a correct diagnosis using HDTV NBI magnifying endoscopy, it is important not only to observe changes in the shape of IPCL but also to note possible papillary structures with central-axis capillaries typical of papillomatosis.

Objectives The purpose of this study is to evaluate the characteristics of thyroid gland surgery focusing on malignancies at the pediatric age with the main concern on treatment results and complications in extensive primary treatment. Methods The records of all patients 18 years and younger with surgically treated thyroid diseases in the Prague Hospital, Motol, between 1991 and 2006 were retrospectively reviewed. Results Thyroid surgery was performed on 148 pediatric patients (including 56 carcinomas). The youngest patient involved in the study was seven years old, the oldest patient 18 years old (mean 13.7 years). Most frequent histological cancer type was PTC (42 cases, 75%). Follicular cancer was diagnosed in five cases (8.9%) and medullar cancer in nine cases (16.1%). A prophylactic thyroidectomy was performed in three cases (5.4%) without clinical signs of thyroid tumor with diagnosed RET gene mutation. Conclusions We consider total thyroidectomy with subsequent radioiodine ablation and TSH suppression as the basic approach in the treatment protocol of pediatric WDTC. The observed 100% recurrence-free and overall survival together with a low incidence of postoperative complications strongly supports the idea of a total thyroidectomy with selective neck dissection in the treatment of metastases of WDTC and MTC.

Hemangiosarcoma of the thyroid gland is a very rare entity. The therapy of this tumor is difficult because of its locally aggressive and destructive behavior with a high recurrence rate. We report a case of 60-year-old man with history of 1 month rapidly enlarging neck mass in the thyroid region. Ultrasound examination and CT scan showed resistance arising from the left thyroid lobe. FNAC revealed necrotic tumor mass without any cytological specification. Because of the clinical findings, a total thyroidectomy was indicated. The surgery was complicated by invasion of the tumor mass into the surrounding tissues. Therefore, only left side hemithyroidectomy was performed. Histopathology including histochemistry proved positive staining for Factor VIII, Fasciin, and CD31 in tumor cells. In this case, adjuvant oncological treatment was planned, but the patient died because of massive bleeding from the tumor mass. The histological diagnosis of thyroid hemangiosarcoma is mostly difficult. The treatment of the first choice is radical surgery. If the tumor cannot be treated by surgery, then radiotherapy, eventually chemotherapy is indicated. The prognosis is not favorable.

The authors present their experience with surgical treatment for nodal metastases of thyroid carcinoma based on neck dissections. The specificity of the surgical approach to the lymph nodes was determined by the biologic behavior of each thyroid tumor. Using the available literature on metastases from thyroid tumors, an opinion is supported that surgery for differentiated carcinomas (papillary and follicular neoplasms) can be more conservative and can be safely limited to modified neck dissections. In contrast, a more extended type of selective neck dissection, and only rarely a comprehensive neck dissection, is needed for medullary carcinoma. Because of its rapid spread to distant sites local aggressivity, extirpation of individual lymph nodes or neck dissection is not justified in patients with anaplastic thyroid carcinoma.

Our results encourage the notion that the pharynx could be an extragastric reservoir of Helicobacter pylori (HP). The study confirmed the presence of HP in adenotonsillar tissue in children. It could have importance in the pathophysiology of upper respiratory diseases. However, its precise role in these processes remains unclear and requires further studies.A prospective study was carried out to evaluate the presence of HP in tonsillar and adenoid tissue in children. The study focused on real-time PCR analyzing CagA and VacA genotypes of HP strains.A total of 37 consecutive pediatric patients with adenotonsillar hypertrophy indicated for surgery were observed in a prospective study. Adenoidectomy and/or tonsillectomy was performed in each patient; 49 specimens were taken, 32 from adenoids and 17 from tonsils. The presence of HP and its genotype were tested in all samples by real-time PCR analysis.Of 49 samples analyzed, 48 were positive for the presence of HP (98%), so only 1 sample was negative. While the genotype VacAs1bm2 was definitely dominant in adenoid tissue, wider distribution was observed in tonsillar tissue. Cag(+) strains represented one-fifth of all samples (21%).

Helicobacter pylori, a well-known gastric pathogen, has been detected in the oral cavity and oropharynx in tonsillar tissue. In our study, the presence of H. pylori in the tonsillar tissue of patients with chronic tonsillitis and sleep apnoea syndrome (SAS) was investigated. The aim was to detect and genotype H. pylori for a collection of data supporting the possible role of H. pylori in the aetiology of chronic tonsillitis and SAS. Helicobacter pylori was detected by real-time polymerase chain reaction (rt-PCR). 89 patients, 60 with a diagnosis of chronic tonsillitis and 29 with SAS, were tested. In the chronic tonsillitis group, Helicobacter was detected in 48 (80 %) specimens, cagA gene was detected in 12 samples (25 %) and 12 samples were negative. In SAS group, Helicobacter was found in 24 samples (82.76 %), cagA gene was detected in 5 (20.83 %) and 5 samples (17.24 %) were negative. Helicobacter pylori-specific immunoglobulins were tested by ELISA in the serum of 57 patients only with 41 (71.93 %) showing positive. Our results on H. pylori DNA detection and H. pylori seropositivity show 26.32 % discrepancy, slightly in favour of rt-PCR (15.79 % compared to 10.53 %). The H. pylori presence in tonsillar tissue does not depend on the type of oropharyngeal disease (p = 0.756). This study shows that oropharynx constitutes an extragastric reservoir of H. pylori infection which could serve as an aetiopathogenetic factor for chronic tonsillitis and tonsillar hyperplasia by SAS. No conclusion has yet been drawn about the mechanism of the process.

Helicobacter pylori (HP) is considered a major gastric pathogen with oncogenic potential. The aim of this study was to determine whether HP is present in oropharyngeal lymphoid tissue and whether oropharyngeal HP strains carry virulence factor genes known to be involved in gastric carcinogenesis. The study included 104 subjects (41 patients with tonsillar carcinoma, 38 with chronic tonsillitis and 25 with obstructive sleep apnoea syndrome—OSAS). Detection of specific serum anti-HP antibodies was performed with an ELISA. The presence of HP in tissue was determined by culture and real-time PCR. Detection of virulence factors genes was also performed. Specific antibodies were found in 78.05 % of tumour cases, 34.21 % of chronic tonsillitis cases, and 72.0 % of OSAS cases. The presence of HP in the tissue was detected in 73.91 % of tonsillar tumours, 70.0 % of tonsillitis cases, and 69.23 % of OSAS specimens. The results of the virulence factor gene analysis showed the majority of the s1b (52.4 %) and m2 (59.5 %) alleles of vacA gene and limited abundance of cagA gene (12.5 %). Results confirm that HP may colonise oropharyngeal lymphoid tissue. Oropharyngeal HP colonisation was frequently found in the oropharyngeal cancer group and in patients with benign oropharyngeal diseases. A virulence factor gene analysis showed differences from the predominant strains most commonly found in the stomach. The strains obtained from the oropharynx differed primarily by the lower abundance of the cagA gene and carried the less virulent vacA gene allele combination.

Mutations in SLC26A4 cause Pendred syndrome (PS) - hearing loss with goitre - or DFNB4 - non-syndromic hearing loss (NSHL) with inner ear abnormalities such as Enlarged Vestibular Aqueduct (EVA) or Mondini Dysplasia (MD). We tested 303 unrelated Czech patients with early hearing loss (298 with NSHL and 5 with PS), all GJB2-negative, for SLC26A4 mutations and evaluated their clinical and radiological phenotype. Among 115 available HRCT/MRI scans we detected three MD (2.6%), three Mondini-like affections (2.6%), 16 EVA (13 bilateral - 19.2% and 15.6% respectively) and 61 EVA/MD-negative scans (73.4%). We found mutation(s) in 26 patients (8.6%) and biallelic mutations in eight patients (2.7%) out of 303 tested. In 18 of 26 (69%) patients, no second mutation could be detected even using MLPA. The spectrum of SLC26A4 mutations in Czech patients is broad without any prevalent mutation. We detected 21 different mutations (four novel). The most frequent mutations were p.Val138Phe and p.Leu445Trp (18% and 8.9% of pathogenic alleles respectively). Among 13 patients with bilateral EVA, six patients (50%) carry biallelic mutations. In EVA -negative patients no biallelic mutations were found but 4.9% had monoallelic mutations. SLC26A4 mutations are present mostly in patients with EVA/MD and/or progressive HL and those with affected siblings.

Background: Activating point mutation of the BRAF gene, the most common genetic alteration reported in papillary thyroid carcinomas (PTC), has been associated with poor prognostic characteristics. Aim: Our objective was to determine the frequency of BRAFV600E mutation in PTC tumor tissues from the period 1960–2007 and to correlate it with clinicopathological parameters. Subjects and methods: DNAs were extracted from 242 PTCs, 23 sporadic medullary carcinomas, one anaplastic carcinoma and 6 poorly differentiated carcinomas. The presence of BRAFV600E mutation was determined using single strand conformation polymorphism method and verified by direct sequencing. Results: BRAFV600E mutation was detected in 81 of 242 PTCs (33.5%), in one of 6 poorly differentiated carcinomas (16.7%) and in anaplastic carcinoma. BRAFV600E mutation was much less frequent in the follicular variant compared to classical variant and mixed follicular-classical variant of PTCs (p=0.001). BRAF V600E mutation was significantly associated with presence of nodal metastasis (p=0.029), more advanced TNM stage (p=0.014) and recurrence of disease (p=0.008). The mutation correlated with a higher age at diagnosis (p=0.049) and with a greater tumor size (p=0.041). Multivariate analysis confirmed these findings. The prevalence of BRAFV600E mutation before 1986 was significantly lower than after it (p=0.008). Conclusions: Our data suggest that BRAFV600E mutation is associated with high-risk clinicopathological characteristics of PTC and worse prognosis of patients. The frequency of the mutation significantly varied during the observed period but rather because of the different age distribution of patients in particular periods than as a consequence of Chernobyl accident.

Helicobacter pylori from patients with different diseases, including so-called autoimmune thyroiditis, chronic tonsillitis and tonsillar cancer, was isolated and cultured. It was identified according to the genotype using labeled hybridization probes complementary to six sequences of cagA and vacA genes. Different types of strains were found in isolates from gastrointestinal tract and patients suffering from thy- roiditis. Six out of seven genotyped isolates from patients in our Department of Otorhinolaryngology and Head and Neck Surgery exhibited the same genotype, differing from isolates obtained from other patients; the 7th isolate originated from a patient who had undergone surgery for deviatio septi nasi, at the same time suffering from autoimmune thyroiditis, having confirmed gastric infection by H. pylori from biopsy. This data made it possible to formulate the hypothesis on probable association of specific H. pylori genotype with chronic tonsillitis and tonsillar cancer. We assessed commercial transport media and improved nucleic acid isolation techniques and the RT-PCR-based tests, which allowed us to skip a culture step and to test directly the patients' samples; however, for full confirmation of our hypothesis and explanation of possible mecha- nisms of the contribution of Helicobacter sp. to the pathogenesis of the disease further data are to be col- lected and evaluated.

The aim of the presented study was to perform the immunohistochemical detection of endothelial (eNOS) and inducible (iNOS) isoform of nitric oxide synthase in the adenomatous and hyperplastic parathyroid gland in relation to the apoptotic process.Tissue samples from 12 patients with parathyroid gland adenoma (PGA) and 10 patients with secondary parathyroid gland hyperplasia (PGH) were collected during surgery at the Department of Otorhinolaryngology and Head and Neck Surgery of The First Faculty of Medicine in Prague.Three-step immunoperoxidase reaction on acetone-fixed cryostat sections was performed using both polyclonal and monoclonal antibodies against eNOS and iNOS. The detection of apoptotic cells was done using antibody against cleaved caspase-3 as an apoptotic marker.The immunoreactivity to eNOS antibody was observed in the endothelial lining of vessels in PGA, PGH and in the rim of normal parathyroid gland adjacent to PGA sample. Variable expression of eNOS was confirmed in arteries, arterioles, capillaries and veins in the glandular parenchyma as well as in the surrounding connective tissue. There was no iNOS immunoreactive cell detected in any examined sample. No apoptotic cells were detected.Our findings confirm that eNOS is regularly expressed in the vasculature of PGA and PGH.eNOS observed in the vasculature of the enlarged parathyroid glands can serve as a factor that contributes to the viability of hypertrophic pathologic tissue. The lack of stimulating signals may be a reason for negative iNOS detection and negligible apoptotic rate.

HGF (Hepatocyte Growth Factor), TGFbeta1 (Transforming Growth Factor beta1) and IGF-I (Insulin Like Growth Factor I) are cytokines that are involved in the parathyroid tumors formation and growth. We tried to determine, if there are changes and relationships in the production of these cytokines by tumor cells of parathyroid tumors.We determined concentrations of HGF, TGFbeta1 and IGF-I in serum from peripheral blood of 16 patients with parathyroid adenoma and of 8 patients with parathyroid secondary hyperplasia before and after parathyroidectomy. Results were compared with serum levels in healthy people.Both preoperative and postoperative HGF serum levels in patients with parathyroid adenoma and secondary hyperplasia are significantly higher than in healthy people. Preoperative and postoperative serum levels of TGFbeta1 in parathyroid adenoma and postoperative TGFb1 serum levels in parathyroid secondary hyperplasia are higher, compared with those in the healthy population and in parathyroid secondary hyperplasia preoperatively. There are no significant differences of IGF-I serum levels among the all investigated groups of patients.Changes in the growth factors production by parathyroid tumor cells are reflected by their concentrations in peripheral blood. The elevation of HGF serum levels in patients with parathyroid adenoma and hyperplasia can be explained by very high HGF production by tumor cells. Nevertheless, there is no decrease of HGF serum levels after the parathyroidectomy. That may be the result of the extratumoral production of this cytokine. Also TGFbeta1 and IGF-I serum levels indicate high possibility of the extratumoral production of these cytokines. Higher postoperative IGF-I serum levels (but not significantly) in parathyroid secondary hyperplasia are in accordance with its bone production.

Angiogenic growth factors bFGF and VEGF ensure vascularisation of the growing tumor tissue. We decided to investigate their peripheral serum concentrations in patients with thyroid gland adenoma and papillary carcinoma and with parathyroid adenoma. We wanted to find the possible serum marker of these tumor diseases.28 patients with thyroid gland tumor (14x adenoma, 14x papillary carcinoma) and 12 patients with parathyroid gland adenoma. Growth factors serum levels were measured by ELISA method.We found significantly higher serum levels of bFGF in both groups of patients with thyroid adenoma (4.93 +/- 3.42 ng/ml) and papillary carcinoma (5.69 +/- 5.58 ng/ml) compared to the healthy population (1.47 +/- 1.77 ng/ml). There were no significant differences of VEGF serum levels between all examined groups of patients (adenoma 213 +/- 197, papillary carcinoma 210 +/- 179, healthy 227 +/- 231 pg/ml). We found significantly higher serum levels of bFGF in patients with parathyroid gland adenoma (7.59 +/- 9.12 ng/ml) compared to those in healthy people (1.47 +/- 1.77 ng/ml).Higher bFGF serum concentrations in patients with thyroid and parathyroid tumors are in accordance with their immunohistochemical tissue levels described in the literature. Not so in VEGF. bFGF may be a serum marker of thyroid and parathyroid neoplasms.

Endogenously produced anti-microbial peptides beta-defensins and nitric oxide (NO) are important components of the immune response. When this response is disturbed it can have damaging consequences. Nasal polyposis, a common disorder, is an example of such immune dysregulation. In order to establish the possible role of human beta defensin-2 (hBD-2), human beta defensin-3 (hBD-3) and NO in the etiopathogenesis of nasal polyposis, the immunohistochemical detection of hBD-2, hBD-3 and endothelial nitric oxide synthase (eNOS) was performed in healthy and pathological human nasal mucosa. Another aim of this study was to find out whether there is a correlation between the level and localization of these antibacterial agents and the occurrence of cell proliferation and programmed cell death. The apoptotic marker cleaved caspase–3 and proliferative marker Ki67 were used for the immunohistochemical detection of these processes. METHODS

Aims and BackgroundThe coincidence of benign or malignant thyroid tumors with thyroglossal duct remnant (TDR) cysts is rare. Although the precise etiology is still unclear, thyroid origin and spread from a primary site have been suggested and this obviously has important implications for the therapeutic approach. Three cases of thyroglossal duct carcinoma are presented and its management is discussed on the basis of the current rationale for treatment of thyroid cancer. The indication for surgery depends on positive findings in the thyroid gland (nodules, FNAB). The aim of this study was to review our experience in the management of papillary thyroid diseases associated with TDR.Materials and MethodsThe records of three patients with thyroid tumors associated with TDR treated at the Department of ENT and Head and Neck Surgery of the First Medical Faculty UK of Prague between January 1991 and January 2001 were analyzed. We searched for risk factors of thyroid carcinoma: history of ionizing radiation, history of thyroid diseases, age, tumor size, tumor spread and histopathological factors.ResultsWe used a triple approach consisting of clinical and ultrasound examination and fine-needle aspiration biopsy for preoperative assessment. Our diagnostic and therapeutic procedures included TDR excision (Sistrunk or Schlange procedure) and total thyroidectomy. Although the therapeutic approach could be a matter of discussion, most patients agreed with our suggestion of relatively radical but non-mutilating treatment. Postoperative radiation or radioiodine ablation is considered in cases of TDR carcinoma or thyroid carcinoma associated with TDR. Oncological follow-up included clinical and ultrasound examination three times during the first year, twice in the second year, and once yearly thereafter. Tumor marker evaluation and/or scintigraphy were performed 6, 12 and/or 24 months following surgery.

Helicobacter pylorihas been recently detected in the oral cavity and oropharynx. However, the role it plays in oral and oropharyngeal pathogenesis remains unclear. The virulence ofH. pyloristrains can be distinguished according to the virulence factors genes carried. Our research has been focused on realtime PCR analysis ofcagAandvacAgenes ofH. pyloristrains in tonsils and tonsillar squamous cell cancer and their comparison withH. pyloristrains obtained from the gastric mucosa of the same patients. Urea breath test (UBT) test was used to detect a gastricH. pyloriinfection in 20 patients with previously provenH. pyloriin the oropharynx. Genotyping ofH. pyloriin gastric biopsies was performed in patients with positive gastric infection. Out of 20 patients positive for oropharyngealH. pylori, 8 were positive for concurrent gastricH. pyloriinfection. In 6 of them gastric biopsies were obtained. Comparison of oropharyngeal and stomachH. pylorigenotypes showed important differences. Four of 6 patients had differentH. pyloristrains in the oropharynx and stomach. The differences were found incagAgene as well as invacAgene. The finding of oral presence ofH. pyloriwithout concurrent stomach infection was confirmed using UBT. The results show that more than oneH. pyloristrain can be present in oropharynx and stomach in the same patient. The oropharyngeal infection seems to be independent to the gastric infection.