Descending necrotising mediastinitis: a rare entity in children.

We present a case of descending necrotising mediastinitis (DNM) originating from a retropharyngeal abscess in a healthy early childhood patient. The patient had a history of fever, odynophagia and refusal to eat, followed by rapid deterioration of the clinical state. Cervicothoracic CT was performed, which revealed a right parapharyngeal abscess, extending to the mediastinum and occupying the retropharyngeal/visceral space, with gaseous content throughout this collection, associated with bilateral pleural effusion, aspects compatible with DNM. She started broad-spectrum antibiotic therapy and transoral drainage of the parapharyngeal and retropharyngeal collections was performed under general anaesthesia. She was admitted to the intensive care unit. The patient showed clinical, analytical and imaging improvement, having been transferred to the ear, nose and throat department, with favourable evolution. Early diagnosis of DNM by cervicothoracic CT and multidisciplinary approaches, including intensive care, broad-spectrum antibiotics and surgical intervention, are crucial to minimise the morbidity and mortality.

Children then, adults now: long-term outcomes-performance at 15, 20, and 25 years of cochlear implant use.

Motivation: Severe to profound sensorineural hearing loss interferes with a child's development at the cognitive, linguistic, academic, and social levels. Since the beginning of the pediatric auditory rehabilitation program through cochlear implantation in the Ear, Nose, and Throat (ENT) Service of the Coimbra Hospital and University Center (CHUC), Portugal, its mentors defended the early diagnosis of hearing loss followed by timely intervention, and this was considered the starting point to optimize (re)habilitation through this method. Three decades or so later, recently we conducted this study to evaluate the performance of patients implanted in the initial phase of the cochlear implantation program. Objectives: The study aimed to evaluate the performance of individuals with severe to profound congenital hearing loss who underwent pediatric cochlear implantation and have used the cochlear implant for at least 25 years, to analyze the beneficial effect of early intervention in improving performance results. Methods: The study sample is composed of 31 individuals with severe to profound congenital hearing loss and no other comorbidities, divided into two groups (Group 1: age at implantation was under 3 years; Group 2: age at implantation was over 3 years). All 31 subjects were evaluated at 15, 20, and 25 years of cochlear implant (CI) use with a comprehensive set of tests. In addition, data were collected regarding the academic level of each participant. The results of both groups were compared to find out if there is an effect of age at implantation on auditory performance, and if there is an improvement in the performance with CI over time (15, 20, and 25 years of use). Results: The results show that there is a positive effect, with statistical significance, of early implantation on auditory performance, and telephone use. In both groups, there is an increase in performance over time, but it tends to stabilize after 20 years of CI use. Discussion and conclusion: The results obtained in this work support the importance of early intervention in patients with severe to profound hearing loss who are cochlear implant users and show that CI is an effective and reliable method in the treatment of these patients, contributing to their improved socio-educational integration, and that the benefits last over time.

Quinine in Otology and Neurotology: Ototoxicity and Historic Role in Therapy.

OBJECTIVES/HYPOTHESIS: Quinine, a cinchona bark-derived antimalarial alkaloid, is a known ototoxic. Isolated and named in 1820 by the French scientists Pierre-Joseph Pelletier and Joseph-Bienaimé Caventou, it has since been employed in the treatment of different maladies. Quinine was also recommended as a local anesthetic in surgical procedures in the early 20th century. This article aims to identify early ototoxicity reports regarding quinine and to investigate if quinine was previously used in otology as an anesthetic agent or as an actual therapy. METHOD: Historical review of medical and pharmaceutical literature from the 19th and 20th centuries in databases (PubMed; Web of Science), as well as medical books on ototoxic drugs, quinine, and therapies in otology. RESULTS: The first identified reference of quinine ototoxicity was from 1824. Quinine also had a therapeutic role in otology and neurotology and was employed for its analgesic properties. It was used in Menière's disease, vertigo, otalgia, purulent otitis media, neuralgia of the plexus tympani, furuncles in the auditory canal, and herpes zoster in the auricle. CONCLUSION: Quinine was acknowledged as an ototoxic drug in the 19th century. Quinine was used in several otologic disorders, both as an analgesic (for herpes zoster, otalgia) and as a therapeutic agent (Menière's disease, vertigo, purulent otitis media, furuncles in the auditory canal). This research demonstrates that, analogously to gentamicin, quinine was used in Menière's disease specifically due to its ototoxic effects.

How to manage an unusual presentation of a thyroglossal duct cyst?

Lingual thyroglossal duct cysts are rare congenital anomalies of the neck. They can be accidentally detected or manifest with disabling symptoms. These cysts can be potentially difficult to manage, but their complete resection is curative.

Upper aerodigestive tract carcinoma: Development of a (epi)genomic predictive model for recurrence and metastasis.

Despite the increased molecular knowledge and the diagnostic and therapeutic improvements, the survival of patients with upper aerodigestive tract carcinoma remains poor. The identification of early diagnostic and prognostic biomarkers and the development of molecular models to distinguish patients that will recur and/or develop metastasis after treatment as well as to benefit with target therapies can be important to decrease mortality, improve survival rates and improve the quality of life of these patients. The current study analyzed 21 upper aerodigestive tract carcinomas through array comparative genomic hybridization and methylation-specific multiplex ligation-dependent probe amplification techniques. A number of chromosomal regions and genes were observed with copy number alterations and methylation. A predictive (epi)genomic model that comprises the 3p chromosomal region and WT1, VHL and THBS1 genes was built, highlighting a molecular signature with possible clinical use. The current study may aid in the development of a more individualized patient management and targeted drug design. The power of this genomic and epigenetic model to predict the recurrence and metastasis development should be evaluated and validated in future larger cohort study.

A long styloid process and Collet-Sicard syndrome / Proceso estiloides alargado y síndrome de Collet-Sicard

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Genomic predictive model for recurrence and metastasis development in head and neck squamous cell carcinoma patients.

The head and neck squamous cell carcinoma (HNSCC) population consists mainly of high-risk for recurrence and locally advanced stage patients. Increased knowledge of the HNSCC genomic profile can improve early diagnosis and treatment outcomes. The development of models to identify consistent genomic patterns that distinguish HNSCC patients that will recur and/or develop metastasis after treatment is of utmost importance to decrease mortality and improve survival rates. In this study, we used array comparative genomic hybridization data from HNSCC patients to implement a robust model to predict HNSCC recurrence/metastasis. This predictive model showed a good accuracy (>80%) and was validated in an independent population from TCGA data portal. This predictive genomic model comprises chromosomal regions from 5p, 6p, 8p, 9p, 11q, 12q, 15q and 17p, where several upstream and downstream members of signaling pathways that lead to an increase in cell proliferation and invasion are mapped. The introduction of genomic predictive models in clinical practice might contribute to a more individualized clinical management of the HNSCC patients, reducing recurrences and improving patients' quality of life. The power of this genomic model to predict the recurrence and metastases development should be evaluated in other HNSCC populations.

Genetic and epigenetic characterization of the tumors in a patient with a tongue primary tumor, a recurrence and a pharyngoesophageal second primary tumor.

BACKGROUND: The choice of therapeutic modality for oral carcinoma in recurrent or second primary tumors remains controversial, as the treatment modalities available might be reduced by the treatment of the first tumor, and the overall survival is lower when compared with patients with a single or first tumor. Identifying biomarkers that predict the risk of relapse and the response to treatment is an emerging clinical issue. CASE PRESENTATION: A Caucasian 49-years-old man was treated with chemotherapy followed by chemoradiotherapy for a primary left side tongue tumor, achieving a complete response. After 49-months of follow-up, a local recurrence was diagnosed. After 3 months, a second primary tumor at the pharyngoesophageal region was detected. Genomic and epigenetic characterization of these three tumors was performed using array Comparative Genomic Hybridization, Multiplex Ligation-dependent Probe Amplification (MLPA) and Methylation Specific MLPA. RESULTS: The three tumors of this patient shared several imbalances in all chromosomes excluding chromosomes 9, 20 and 22, where genes related to important functional mechanisms of tumorigenesis are mapped. The shared genomic imbalances, such as losses at 1p, 2p, 3p, 4q, 5q, 6q, 7q, 8p, 10p, 11q, 12p, 12q, 13q, 15q, 16p, 16q, 17p, 17q, 18q, 19p, 19q, 21q and Xp and gains at 3q, 7q, 14q and 15q showed a common clonal origin for the diagnosed relapses. We identified some chromosomal imbalances and genes mapped in the chromosomes 2, 3, 4, 6, 7, 11, 14, 17, 18 and 22 as putative linked to chemoradioresistance and chemoradiosensitivity. We also observed that gains in short arm of chromosomes 6, 7, 8 and 18 were acquired after treatment of the primary tumor. We identified losses of VHL gene and promoter methylation of WT1 and GATA5 genes, as predictors of relapses. CONCLUSIONS: A common clonal origin for the diagnosed relapses was observed and we identified some putative candidate biomarkers of prognosis, relapse risk and treatment response that could guide the development of management strategies for these patients.

USPIO-enhanced magnetic resonance imaging for nodal staging in patients with head and neck cancer.

PURPOSE: To determine the accuracy of ultrasmall superparamagnetic iron oxide (USPIO)-enhanced magnetic resonance imaging (MRI) for nodal staging in patients with head and neck cancer. MATERIALS AND METHODS: Twenty patients with carcinomas of the upper aerodigestive tract were prospectively enrolled. MRI was performed before and 24-36 hours after intravenous infusion of an USPIO agent, ferumoxtran-10 (Sinerem; Guerbet, France; and Combidex; Advanced Magnetics) at a dose of 2.6 mg Fe/kg using T2-weighted spin-echo and gradient-echo sequences. Surgery was performed the same day or the day after the ferumoxtran-10-enhanced MR examination. Based on MRI, selected nodes were surgically removed and directly correlated with pathology using hematoxylin-eosin (H&E) and Perls stainings. RESULTS: A total of 63 nodes were studied; 36 were nonmetastatic, 25 metastatic, and two inflammatory. Ferumoxtran-10-enhanced MRI allowed diagnosis of 24 metastatic and 30 nonmetastatic nodes, yielding a sensitivity of 96%, a specificity of 78.9%, a positive predictive value of 75%, and a negative predictive value of 96.8%, compared to 64%, 78.9%, 66.6%, and 76.9%, respectively, for nonenhanced MRI. Accuracy of ferumoxtran-10-enhanced MRI was 85.7%. The gradient-echo T2-weighted sequence was the most accurate to detect signal loss in nonmetastatic nodes. CONCLUSION: USPIO-enhanced MRI is useful for nodal staging of patients with head and neck cancers.