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1.
Artigo em Inglês | MEDLINE | ID: mdl-38342426

RESUMO

INTRODUCTION: The enlarged vestibular aqueduct (EVA) is the most frequent malformation of the inner ear associated with sensorineural hearing loss (5-15%). It exists when the diameter in imaging tests is greater than 1.5 mm at its midpoint. The association between hearing loss and EVA has been described in a syndromic and non-syndromic manner. It can appear as a familial or isolated form and the audiological profile is highly variable. The gene responsible for sensorineural hearing loss associated with EVA is located in the same region described for Pendred syndrome, where the SCL26A4 gene is located. OBJECTIVE: To describe a series of children diagnosed with EVA in order to study their clinical and audiological characteristics, as well as the associated genetic and vestibular alterations. METHOD: Retrospective study of data collection of children diagnosed with EVA, from April 2014 to February 2023. RESULTS: Of the 17 cases, 12 were male and 5 were female. 5 of them were unilateral and 12 bilateral. In 5 cases, a cranial traumatism triggered the hearing loss. Genetic alterations were detected in 3 cases: 2 mutations in the SCL26A4 gene and 1 mutation in the MCT1 gene. 13 patients (76.5%) were rehabilitated with hearing aids and 9 of them required cochlear implantation. DISCUSSION: The clinical importance of AVD lies in the fact that it is a frequent finding in the context of postneonatal hearing loss. It is convenient to have a high suspicion to diagnose it with imaging tests, to monitor its evolution, and to rehabilitate early.


Assuntos
Perda Auditiva Neurossensorial , Aqueduto Vestibular , Humanos , Aqueduto Vestibular/anormalidades , Aqueduto Vestibular/diagnóstico por imagem , Masculino , Feminino , Estudos Retrospectivos , Perda Auditiva Neurossensorial/genética , Perda Auditiva Neurossensorial/etiologia , Lactente , Pré-Escolar , Criança , Transportadores de Sulfato/genética , Surdez/genética , Surdez/etiologia , Adolescente , Mutação
2.
Artigo em Inglês | MEDLINE | ID: mdl-37149133

RESUMO

OBJECTIVE: To determine the percentage of children with permanent bilateral postnatal hearing loss in order to study its incidence, related risk factors, diagnosis and treatment. METHODS: Retrospective study to collect data on children diagnosed with hearing loss outside the neonatal period in the Hearing Loss Unit of the Hospital Universitario Central de Asturias, from April 2014 to April 2021. RESULTS: 52 cases met the inclusion criteria. The detection rate of congenital hearing loss in the neonatal screening programme in the same study period was 1.5 children per thousand newborns per year, adding postnatal hearing loss results in a rate of infant bilateral hearing loss of 2.7 children per thousand (55.5% and 44.4% respectively). Thirty-five children presented risk factors for hearing loss, of which 23 were at retrocochlear risk. The mean age at referral was 91.9 (18-185) months. Hearing aid fitting was indicated in 44 cases (84.6%). Cochlear implantation was indicated in eight cases (15.4%). DISCUSSION: Although congenital hearing loss accounts for the majority of childhood deafness, postnatal hearing loss has a significant incidence. This may be mainly due to: (1) that hearing impairment may arise in the first years of life, (2) that mild hearing loss as well as hearing loss in severe frequencies are undetectable by neonatal screening in some cases, (3) that some children may have false negative results. CONCLUSION: postnatal hearing loss requires identification of risk factors and long-term follow-up of children with hearing loss, as it needs to be detected and treated early.


Assuntos
Surdez , Perda Auditiva Neurossensorial , Perda Auditiva , Lactente , Criança , Recém-Nascido , Humanos , Estudos Retrospectivos , Perda Auditiva/diagnóstico , Perda Auditiva/epidemiologia , Perda Auditiva/etiologia , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Bilateral/diagnóstico , Perda Auditiva Bilateral/epidemiologia , Perda Auditiva Bilateral/etiologia , Audição
3.
An Pediatr (Engl Ed) ; 2020 Sep 28.
Artigo em Espanhol | MEDLINE | ID: mdl-32998843

RESUMO

INTRODUCTION: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present experience in ototoxicity monitoring. MATERIAL AND METHODS: A review was made of the registry of paediatric cancer patients referred to the Children's Hearing Loss Unit from 1999 to 2019. RESULTS: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. CONCLUSION: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss.

4.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-32192730

RESUMO

INTRODUCTION: Single cochlear implantation usually provides substantial speech intelligibility benefits but bilaterally deaf, unilaterally implanted subjects will continue to experience limitations due to the head shadow effect, like single-sided deaf individuals. In the treatment of individuals with single-sided deafness one option is contralateral routing of signal (CROS) devices, which constitute a non-surgical intervention of the second ear in unilaterally implanted individuals. METHOD: Twelve experienced adult cochlear implant users with Naída Q70 processor and the CROS device used in combination participated in the study. For the study 3 conditions were provided: cochlear implant only, omnidirectional microphone mode (CROS deactivated); cochlear implant plus CROS activated, omnidirectional microphone mode and cochlear implant plus CROS activated, UltraZoom mode. Speech reception thresholds were determined in quiet and noise. Subjective feedback regarding the practical usability of the CROS device and the perceived benefit were collected. RESULTS: There was a 27.6% improvement in speech understanding in quiet and 32.5% improvement in noise when CROS device was activated. Using advanced directional microphones, a statistically significant benefit of 35% was obtained. The responses to the questionnaires revealed that the subjects perceived benefit in their everyday lives when using the CROS device with their cochlear implants. CONCLUSION: The investigated CROS device used by unilateral CI recipients in cases where bilateral implantation is not an option provides both subjective and objective speech recognition benefit when the signal is directed to the CROS device. Unfavourable conditions where speech is presented from the cochlear implant side and noise from the CROS side or diffusely were not included in this evaluation since the CROS device adds additional noise and performance is expected to decrease as has previously been shown.

5.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30579509

RESUMO

BACKGROUND AND OBJECTIVE: The risk of bacterial meningitis increases in cochlear implant patients. Therefore, pneumococcal, influenza and Haemophilus influenzae type b vaccination is indicated in this group. The aim of this study was to determine compliance with the vaccination calendar in patients implanted in a referral hospital. MATERIALS AND METHODS: Patients with cochlear implant operated between 2005 and 2015 were included. Vaccine coverage for seasonal influenza, Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes and pneumococcal polysaccharide 23-serotypes was evaluated. The sample was divided into 2 age groups (<14 years and≥14 years). A univariate and bivariate analysis was performed. RESULTS: Of the 153 patients studied (28.01% 0-13 years old and 71.9%≥14), only 2 (5.71%) had 100% adherence to the vaccination schedule, while 65.71% had compliance of 50% or less. Overall, vaccination coverage against the sequential pneumococcal pattern was 48.57%. The paediatric population exceeded 90% coverage for the vaccine against Haemophilus influenzae type b and pneumococcal conjugate 13-serotypes while in those over 14 years of age it barely exceeded 50%. Influenza coverage was less than 40%. An inverse correlation was obtained between age and compliance, although not statistically significant. CONCLUSIONS: Vaccination coverage in patients with cochlear implant is lower than expected. Close collaboration between Otolaryngology departments and the Vaccination Units is proposed as the main strategy for improvement.


Assuntos
Implantes Cocleares , Meningites Bacterianas/prevenção & controle , Complicações Pós-Operatórias/prevenção & controle , Cobertura Vacinal/estatística & dados numéricos , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Implante Coclear , Estudos Transversais , Suscetibilidade a Doenças , Vacinas Anti-Haemophilus , Humanos , Lactente , Recém-Nascido , Vacinas contra Influenza , Pessoa de Meia-Idade , Vacinas Pneumocócicas , Espanha , Adulto Jovem
6.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30086890

RESUMO

INTRODUCTION AND OBJECTIVES: Inadequate auditory feedback in prelingually deaf children alters the articulation of consonants and vowels. The purpose of this investigation was to compare vowel production in Spanish-speaking deaf children with cochlear implantation, and with hearing-aids with normal-hearing children by means of acoustic analysis of formant frequencies and vowel space. METHODS: A total of 56 prelingually deaf children (25 with cochlear implants and 31 wearing hearing-aids) and 47 normal-hearing children participated. The first 2 formants (F1 and F2) of the five Spanish vowels were measured using Praat software. One-way analysis of variance (ANOVA) and post hoc Scheffé test were applied to analyze the differences between the 3 groups. The surface area of the vowel space was also calculated. RESULTS: The mean value of F1 in all vowels was not significantly different between the 3 groups. For vowels /i/, /o/ and /u/, the mean value of F2 was significantly different between the 2 groups of deaf children and their normal-hearing peers. CONCLUSION: Both prelingually hearing-impaired groups tended toward subtle deviations in the articulation of vowels that could be analyzed using an objective acoustic analysis programme.


Assuntos
Transtornos da Articulação/etiologia , Implantes Cocleares , Auxiliares de Audição , Perda Auditiva/complicações , Pessoas com Deficiência Auditiva , Fonética , Transtornos da Articulação/fisiopatologia , Criança , Pré-Escolar , Retroalimentação Sensorial , Feminino , Perda Auditiva/fisiopatologia , Perda Auditiva/reabilitação , Humanos , Masculino , Acústica da Fala , Medida da Produção da Fala , Qualidade da Voz
7.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30097163

RESUMO

INTRODUCTION AND OBJECTIVES: Perceptual rating of overall voice quality and other more specific perceptual dimensions is difficult, as such judgments depend on the listener's subjectivity. Thus, finding objective, valid, and accessible clinical measures to include in comprehensive voice evaluation protocols is a priority. The purposes of this study were to 1) determine the diagnostic accuracy of a single acoustic measure, smoothed cepstral peak prominence (CPPS), to predict voice disorder status from sustained vowels and connected speech samples using the software Praat; 2) to determine the relationship between measures of CPPS and perceptual ratings of vocal quality; and 3) describe the normative values of CPPS. METHOD: Measures of CPPS were obtained from connected speech and sustained vowel recordings of 72 Spanish-speaking subjects with voice disorders and 52 nondysphonic Spanish-speaking subjects with no vocal disorders using freely downloadable Praat software. IBM SPSS Statistics software version 23 was used to complete the statistical analyses. RESULTS: results revealed a 70% sensitivity rate, a specificity rate of 85%. Estimated severity for sustained vowels and connected speech were strongly correlated and significantly associated with listener ratings of dysphonia severity. CONCLUSIONS: A single acoustic measure of CPPS was highly predictive of voice disorder status using Praat software. Clinicians may consider using CPPS to complement clinical voice evaluation and screening protocols.


Assuntos
Disfonia/diagnóstico , Espectrografia do Som , Qualidade da Voz , Adulto , Disfonia/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Processamento de Sinais Assistido por Computador , Espanha , Acústica da Fala , Adulto Jovem
8.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-28213999

RESUMO

INTRODUCTION: Over the past several decades, many acoustic parameters have been studied as sensitive to and to measure dysphonia. However, current acoustic measures might not be sensitive measures of perceived voice quality. A meta-analysis which evaluated the relationship between perceived overall voice quality and several acoustic-phonetic correlates, identified measures that do not rely on the extraction of the fundamental period, such the measures derived from the cepstrum, and that can be used in sustained vowel as well as continuous speech samples. A specific and recently developed method to quantify the severity of overall dysphonia is the acoustic voice quality index (AVQI) that is a multivariate construct that combines multiple acoustic markers to yield a single number that correlates reasonably with overall vocal quality. METHODS: This research is based on one pool of voice recordings collected in two sets of subjects: 60 vocally normal and 58 voice disordered participants. A sustained vowel and a sample of connected speech were recorded and analyzed to obtain the six parameters included in the AVQI using the program Praat. Statistical analysis was completed using SPSS for Windows, version 12.0. RESULTS: Correlation between perception of overall voice quality and AVQI: A significant difference exists (t(95) = 9.5; p<.000) between normal and dysphonic voices. CONCLUSIONS: The findings of this study demonstrate the clinical feasibility of the AVQI as a measure of dysphonia severity.


Assuntos
Acústica , Disfonia/diagnóstico , Disfonia/fisiopatologia , Qualidade da Voz , Adulto , Humanos , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Adulto Jovem
9.
Acta Otorrinolaringol Esp ; 67(4): 193-200, 2016.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-26454645

RESUMO

INTRODUCTION AND OBJECTIVES: Conventional audiometry is the gold standard for quantifying and describing hearing loss. Alternative methods become necessary to assess subjects who are too young to respond reliably. Auditory evoked potentials constitute the most widely used method for determining hearing thresholds objectively; however, this stimulus is not frequency specific. The advent of the auditory steady-state response (ASSR) leads to more specific threshold determination. The current study describes and compares ASSR, auditory brainstem response (ABR) and conventional behavioural tone audiometry thresholds in a group of infants with various degrees of hearing loss. METHODS: A comparison was made between ASSR, ABR and behavioural hearing thresholds in 35 infants detected in the neonatal hearing screening program. RESULTS: Mean difference scores (±SD) between ABR and high frequency ABR thresholds were 11.2 dB (±13) and 10.2 dB (±11). Pearson correlations between the ASSR and audiometry thresholds were 0.80 and 0.91 (500Hz); 0.84 and 0.82 (1000Hz); 0.85 and 0.84 (2000Hz); and 0.83 and 0.82 (4000Hz). CONCLUSION: The ASSR technique is a valuable extension of the clinical test battery for hearing-impaired children.


Assuntos
Audiometria de Tons Puros , Potenciais Evocados Auditivos , Perda Auditiva Neurossensorial/diagnóstico , Potenciais Evocados Auditivos do Tronco Encefálico , Feminino , Perda Auditiva Condutiva/diagnóstico , Perda Auditiva Condutiva/fisiopatologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Triagem Neonatal , Emissões Otoacústicas Espontâneas/fisiologia , Reprodutibilidade dos Testes
10.
Head Neck ; 35(5): E147-52, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-22319002

RESUMO

BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare, inheritable, multisystem disorder characterized by numerous basal cell carcinomas (BCCs), maxillary keratocyst, and musculoskeletal malformations. Occasionally, it is associated with malignancies like rhabdomyoma, melanoma, and sinonasal undifferentiated carcinoma, to name a few. METHODS: A patient presented with NBCCS with a medullary thyroid carcinoma. Clinical, surgical details, and germline genetic analysis are herein described. RESULTS: A 32-year-old woman was referred to our department with suspicion of medullary thyroid carcinoma, which was confirmed by histopathological examination. The patient was diagnosed as also having NBCCS. Germline mutation analysis indicated wild-type genes PTCH1 and RET. DNA copy number analysis by high resolution microarray comparative genomic hybridization (CGH) revealed a small interstitial loss at chromosomal band 2q37.3. CONCLUSION: To our knowledge, this is the first described patient with NBCCS carrying a medullary thyroid carcinoma and a 2q37 deletion, which confirms that this syndrome can be associated with many different malignancies.


Assuntos
Síndrome do Nevo Basocelular/complicações , Deleção Cromossômica , Neoplasias da Glândula Tireoide/complicações , Adulto , Carcinoma Neuroendócrino , Cromossomos Humanos Par 2 , Hibridização Genômica Comparativa , Variações do Número de Cópias de DNA , Feminino , Mutação em Linhagem Germinativa , Humanos , Neoplasias da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/patologia
11.
Acta Otorrinolaringol Esp ; 64(2): 115-23, 2013.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-23122366

RESUMO

INTRODUCTION AND OBJECTIVES: Intestinal-type sinonasal adenocarcinomas are malignant epithelial tumours. Around 8-25% of all sinonasal malignant tumours are intestinal-type adenocarcinomas, which are related to wood dust exposure. Four histological subtypes have been described: papillary, colonic, solid and mucinous. We performed a pathological and immunohistochemical study in order to describe characteristics with prognostic, diagnostic and therapeutic value, and also to compare our results with previous studies. METHODS: Sixty six tumour samples were analysed and protein expression of p53, p16, E-cadherin, ß-catenin, epidermal growth factor receptor (EGFR), human epidermal growth factor receptor 2 (HER2/neu) and cyclooxygenase-2 (COX-2) was performed by tissue microarray blocks. RESULTS: The 63% of cases were p53 positive; 37% showed nuclear staining with ß-catenin and 100% with E-cadherin, while 98% showed membrane staining with ß-catenin, 7% with EGFR, 8% with HER2/neu and 52% with COX-2; and 59% of the cases lost p16 expression. CONCLUSIONS: Intracranial invasion was the worst prognostic associated event. Solid and mucinous tumours were the most aggressive histological subtypes. Intracranial invasion was more frequent in mucinous subtype tumours. Immunohistochemical results were similar in all tumour subtypes, except for mucinous tumours, which showed weak expression of E-cadherin and ß-catenin. Comparing with previous studies, we found a lower expression of EGFR, HER2/neu and COX-2. The p16 expression was associated with worse survival and metastatic disease.


Assuntos
Adenocarcinoma/patologia , Neoplasias dos Seios Paranasais/patologia , Adenocarcinoma/química , Adenocarcinoma/classificação , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Feminino , Humanos , Imuno-Histoquímica , Intestinos , Masculino , Pessoa de Meia-Idade , Neoplasias dos Seios Paranasais/química , Neoplasias dos Seios Paranasais/classificação , Prognóstico , Estudos Retrospectivos
12.
Acta Otorrinolaringol Esp ; 63(3): 212-7, 2012.
Artigo em Espanhol | MEDLINE | ID: mdl-22425204

RESUMO

INTRODUCTION: Peritonsillar infection is the most frequent complication of acute tonsillitis. Peritonsillar infections are collections of purulent material, usually located between the tonsillar capsule and the superior constrictor of the pharynx. Peritonsillar infection can be divided into abscess and cellulitis. MATERIAL AND METHODS: We prospectively analysed the clinical data from 100 patients with peritonsillar infection from 2008 to 2010. The diagnosis of abscess or peritonsillar cellulitis was primarily based on obtaining pus through fine-needle aspiration. RESULTS: Seventy-seven per cent of patients had no history of recurrent tonsillitis and 55% were receiving antibiotic treatment. Sixty-two cases were peritonsillar abscess and the rest were cellulitis. Trismus, uvular deviation and anterior pillar bulging were statistically associated with peritonsillar abscess (P<.005). All patients were admitted to hospital and treated with puncture-drainage, intravenous antibiotics (amoxicillin/clavulanate in 83% of cases) and a single dose of steroids. All patients were discharged on oral antibiotic therapy. The mean length of hospital stay was 3 days and the recurrence rate was 5%. CONCLUSIONS: Due to the absence of clinical practice guidelines, there are different therapeutic protocols. According to our experience, puncture-aspiration and administration of intravenous antibiotics is a safe, effective way to treat these patients. To determine the efficacy and safety of outpatient management, controlled studies would be needed.


Assuntos
Celulite (Flegmão)/epidemiologia , Abscesso Peritonsilar/epidemiologia , Faringite/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Antibacterianos/uso terapêutico , Celulite (Flegmão)/tratamento farmacológico , Celulite (Flegmão)/cirurgia , Criança , Pré-Escolar , Terapia Combinada , Suscetibilidade a Doenças , Drenagem , Feminino , Humanos , Infusões Intravenosas , Tempo de Internação , Macrolídeos/efeitos adversos , Macrolídeos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Abscesso Peritonsilar/tratamento farmacológico , Abscesso Peritonsilar/cirurgia , Faringite/tratamento farmacológico , Faringite/cirurgia , Estudos Prospectivos , Recidiva , Espanha/epidemiologia , Tonsilectomia , Tonsilite/epidemiologia , Adulto Jovem
13.
Acta Otorrinolaringol Esp ; 63(2): 79-84, 2012.
Artigo em Espanhol | MEDLINE | ID: mdl-22100049

RESUMO

INTRODUCTION AND OBJECTIVES: The literature on the involvement of microsatellite instability in head and neck squamous cell carcinoma shows great variability, probably due to differences in the testing methods. Using a consensus detection system, we aimed to reach a reliable estimate of microsatellite instability prevalence in a subset of head and neck squamous cell carcinoma cases. METHODS: The microsatellite instabilityI status of 43 patients with previously untreated primary laryngeal squamous cell carcinomas was analyzed by a multiplex polymerase chain reaction assay including 5 mononucleotide repeat markers. RESULTS: Thirty-six cases showed a stable phenotype or a microsatellite stable phenotype (83.7%) and 7 cases (16.3%) showed an microsatellite instability-positive phenotype. One case showed instability in 3 of 5 markers, 1 case in 2 markers and 5 cases in 1 marker. The microsatellite instability-positive and stable cases did not differ with respect to age, tumour stage, lymph node or distant metastases. CONCLUSIONS: Our data showed that a proportion of laryngeal squamous cell carcinomas are microsatellite instability positive. Knowledge of microsatellite instability patient status will allow adjusting anticancer therapy at an individual level.


Assuntos
Carcinoma de Células Escamosas/genética , Neoplasias Laríngeas/genética , Instabilidade de Microssatélites , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/radioterapia , Carcinoma de Células Escamosas/cirurgia , Diferenciação Celular , Terapia Combinada , Reparo do DNA , DNA de Neoplasias/genética , Feminino , Humanos , Estimativa de Kaplan-Meier , Neoplasias Laríngeas/patologia , Neoplasias Laríngeas/radioterapia , Neoplasias Laríngeas/cirurgia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Reação em Cadeia da Polimerase/métodos
14.
Acta Otolaryngol ; 131(12): 1349-52, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21905794

RESUMO

The patient was a 48-year-old man complaining about right hemicranial pain and deafness of 1 year's duration. MRI showed a solid well-vascularized mass destroying the mastoid and petrous bone, and with an epidural component that pulled the right cerebellar hemisphere. The patient underwent a right modified type A infratemporal approach, which allowed complete resection of the tumor, with a low morbidity. The pathological diagnosis was solitary fibrous tumor. The patient received complementary treatment with radiotherapy. At 24 months after the initial treatment the patient is free of disease. We conclude that solitary fibrous tumor of the petrous bone can be satisfactorily treated with surgical excision followed by radiotherapy, with low morbidity and excellent facial function. To our knowledge this is the first description of a solitary fibrous tumor of the petrous bone. Due to the lack of consensus in treating rare tumors, we want to offer this management approach for treating this kind of tumor.


Assuntos
Neoplasias Ósseas/terapia , Osso Petroso/cirurgia , Tumores Fibrosos Solitários/terapia , Neoplasias Ósseas/patologia , Doenças dos Nervos Cranianos/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Osso Petroso/patologia , Radioterapia Adjuvante , Tumores Fibrosos Solitários/patologia
15.
J Craniofac Surg ; 20(4): 1282-6, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19625850

RESUMO

Malignant triton tumor is a very aggressive type of sarcoma that comprises rhabdomyoblasts and malignant Schwann cells. It is a different entity from malignant schwannoma, characterized by their aggressiveness and poor prognosis. Head and neck location is frequent, and early diagnosis and complete resection followed by radiation therapy is important for long-term survival. However, the therapeutic plan should be individualized, taking into account the location and size of the primary tumor. The use of adjuvant chemotherapy and molecular therapies should be considered in the treatment of these tumors. We report an unusual presentation of a malignant triton tumor located in the infratemporal fossa, describing its clinical and pathologic features, and we try to update the knowledge in the management of these tumors, including the use of molecular therapies.


Assuntos
Hamartoma/diagnóstico , Neoplasias da Base do Crânio/diagnóstico , Biópsia , Terapia Combinada , Diagnóstico Diferencial , Endoscopia , Evolução Fatal , Feminino , Hamartoma/patologia , Hamartoma/terapia , Humanos , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/terapia , Tomografia Computadorizada por Raios X , Adulto Jovem
16.
J Craniofac Surg ; 20(4): 1178-81, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19553844

RESUMO

Parapharyngeal and retropharyngeal infections, which can potentially cause life-threatening complications, could be treated conservatively with no need for surgical drainage. A retrospective analysis of all patients diagnosed with retropharyngeal and parapharyngeal infections was performed. Information regarding age, sex, presenting symptoms, physical examination, laboratory and imaging evaluations, management, duration of hospital stay, and complications were reviewed. Seven children were identified, 4 with retropharyngeal abscess and 3 with parapharyngeal abscess. All but 1 patient were under 7 years old, and all were treated with intravenous amoxicilin/clavulanic acid and corticosteroids. Torticollis and fever were present in all the patients. The mean length of hospital stay was 7 days. There were no complications associated. We demonstrate that retropharyngeal and parapharyngeal abscesses can be treated medically, reserving the surgical drainage for complicated cases. Treatment with intravenous antibiotics and corticosteroids is a safe option, reducing the durations of symptoms and the length of hospital stay.


Assuntos
Abscesso/tratamento farmacológico , Corticosteroides/uso terapêutico , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Antibacterianos/uso terapêutico , Doenças Faríngeas/tratamento farmacológico , Abscesso Retrofaríngeo/terapia , Anti-Inflamatórios não Esteroides/uso terapêutico , Criança , Pré-Escolar , Quimioterapia Combinada , Humanos , Ibuprofeno/uso terapêutico , Tempo de Internação/estatística & dados numéricos , Masculino , Resultado do Tratamento
17.
Acta Otorrinolaringol Esp ; 60(1): 19-24, 2009.
Artigo em Espanhol | MEDLINE | ID: mdl-19268125

RESUMO

INTRODUCTION AND OBJECTIVES: Tumours of the parapharyngeal space are rare, representing only 0.5 % of head and neck oncology. The aim of this study is to present our experience with the diagnosis and therapies for these tumours. PATIENTS AND METHOD: This study is a retrospective review of 51 patients diagnosed as having tumours of the parapharyngeal space and treated surgically between 1984 and 2006. Tumours originating in the deep lobe of parotid gland were excluded. RESULTS: Seventy percent of the parapharyngeal space neoplasms were benign and thirty percent malignant. Pleomorphic adenoma was the most common neoplasm (37 %), followed by miscellaneous benign tumours (34 %), paraganglioma (21 %), and neurogenic tumours (8 %). Surgery was chosen in all case, with the transcervical approach used in 32 cases, the cervical-transparotid approach in 13 cases, the transmandibular approach in 4 cases (with mandibulectomy in 1 patient), the transoral approach in 1 case, and the infratemporal fossa approach in 1 case. The most common complications were those deriving from damage to nerve structures. CONCLUSIONS: Most parapharyngeal space tumours can be removed surgically with a low rate of complications and recurrence. The transcervical approach is the most frequently used.


Assuntos
Neoplasias Faríngeas , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Neoplasias Faríngeas/diagnóstico , Neoplasias Faríngeas/cirurgia , Estudos Retrospectivos , Adulto Jovem
18.
Acta Otorrinolaringol Esp ; 60 Suppl 1: 97-105, 2009 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-19245780

RESUMO

Vagal paragangliomas are much less frequent than carotid and jugulo-tympanic paragangliomas and, like these other forms, can be sporadic or familial. The familial forms usually occur as isolated tumors or within syndromic entities such as multiple endocrine neoplasia type IIA-B, Von Hippel- Lindau syndrome and neurofibromatosis type 1. The genes causing the disease are located in three loci: SDHD (11q23), SDHC (1q21) and SDHB (1p36.1p35). Approximately one-third of apparently sporadic paragangliomas also show germline SDH mutations. In familial forms, the rate of multicentric tumors is up to 30-40%. Vagal paragangliomas arise from arise from paraganglia associated with the vagus nerve. Lower cranial nerve deficits are frequently observed. An essential feature in the diagnosis of vagal paraganglioma is that they are situated behind the internal carotid artery. The treatment of vagal paraganglioma should be based on the biological behavior of the tumor, the age and health status of the patient, tumoral size and the possible treatment-associated morbidity. Surgery is important in the treatment of vagal paraganglioma but this option is controversial in patients without lower cranial nerve palsy. The approach of choice is usually cervical or transcervical. If the tumor involves the jugular fossa, in addition to the cervical approach, a transpetrous approach is also required. Expectant management and radiotherapy are often valid options.


Assuntos
Corpos Aórticos , Paraganglioma/cirurgia , Humanos , Paraganglioma/diagnóstico , Paraganglioma/terapia , Procedimentos Cirúrgicos Operatórios/métodos
19.
J Craniofac Surg ; 19(5): 1287-91, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18812853

RESUMO

The aim of this study was to report our experience on the management of radiation-induced sarcomas (RISs). A retrospective study from 1994 to 2003 was done at our institution reviewing the medical records of 5 patients who had RISs. Five patients diagnosed of head and neck cancer received irradiation to treat their diseases. Later on, these patients developed new neoplasms in the irradiation fields (3 malignant fibrous histiocytoma, 1 osteosarcoma, and 1 angiosarcoma). The mean period of latency between irradiation and diagnosis of new tumors was 13 years. Early symptoms included neck or face swelling, odynophagia, or trigeminal hypoesthesia. All of the patients underwent surgical treatment. In 4 cases, regional and free flaps for head and neck reconstruction were required. Three patients also needed neoadjuvant chemotherapy. In the follow-up, 2 patients are alive and free of disease. Wide excision is the treatment of choice for RISs. Previous radiation therapy limits the dose that can be administered to the involved area, and the response rate to the chemotherapy is always poor.


Assuntos
Irradiação Craniana/efeitos adversos , Neoplasias de Cabeça e Pescoço/etiologia , Neoplasias Induzidas por Radiação/cirurgia , Segunda Neoplasia Primária/etiologia , Sarcoma/etiologia , Adulto , Idoso , Feminino , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/radioterapia , Neoplasias de Cabeça e Pescoço/cirurgia , Hemangiossarcoma/etiologia , Hemangiossarcoma/cirurgia , Histiocitoma Fibroso Maligno/etiologia , Histiocitoma Fibroso Maligno/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Terapia Neoadjuvante , Neoplasias Induzidas por Radiação/tratamento farmacológico , Segunda Neoplasia Primária/tratamento farmacológico , Segunda Neoplasia Primária/cirurgia , Osteossarcoma/etiologia , Osteossarcoma/cirurgia , Estudos Retrospectivos , Sarcoma/tratamento farmacológico , Sarcoma/cirurgia , Adulto Jovem
20.
Acta Otorrinolaringol Esp ; 59(6): 308-10, 2008.
Artigo em Espanhol | MEDLINE | ID: mdl-18588792

RESUMO

Optic neuritis are clinically demonstrated by a temporary but severe loss of vision and can be caused by a wide variety of diseases. It is unusual for sphenoidal sinusitis to co-exist with acute optic neuritis, so the simultaneous appearance of both diseases would invite aetiological suspicion. We present two cases where the first clinical manifestation of infectious sphenoidal pathology was retrobulbar optic neuritis, which reverted with treatment, medical in one case and surgical in the other, of the sinusitis.


Assuntos
Neurite Óptica/etiologia , Sinusite Esfenoidal/complicações , Adulto , Idoso , Feminino , Humanos , Masculino , Sinusite Esfenoidal/terapia
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