Browsing by Author "Monteiro, E."
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- Malignant tumors of the temporal bone - our experiencePublication . Silva, A.; Breda, E.; Monteiro, E.INTRODUCTION: Malignant tumors of the temporal bone are rare, with an estimated incidence of about 0.8-1.0 per 1,000,000 inhabitants per year. The vast majority of these tumors are squamous cell carcinomas and their treatment is eminently surgical. OBJECTIVE: This study is an attempt at systematizing the forms of clinical presentation, the therapeutic possibilities, and oncological outcomes of patients with malignant tumors of the temporal bone in a tertiary hospital in Portugal. METHODS: The authors present a retrospective study of temporal bone tumors treated and followed during otorhinolaryngology consultations between 2004 and 2014. A review of the literature is also included. RESULTS: Of the 18 patients included in the study, 16 had a primary tumor of the temporal bone, in most cases with squamous cell carcinoma histology. Of these, 13 patients were treated with curative intent that always included the surgical approach. Disease persistence was observed in one patient and local recurrence in five patients, on average 36.8 months after the initial treatment. CONCLUSIONS: The anatomical complexity of the temporal bone and the close associations with vital structures make it difficult to perform tumor resection with margins of safety and thus, tumor relapses are almost always local. A high level of suspicion is crucial for early diagnosis, and stringent and prolonged follow-up after treatment is essential for diagnosis and timely treatment of recurrances.
- Síndrome de Apert: caso clínicoPublication . Sampaio, B.; Nunes, S.; Monteiro, E.; Silva, A.; Pereira, A.O Síndrome de Apert (SA) é uma doença genética, rara. Recém-nascido do sexo feminino, de termo, nasceu por parto eutócico, fruto de uma gestação vigiada e sem intercorrências. As ecografias pré-natais foram relatadas como “normais”. O exame objectivo revelou turribraquicefalia, occipital plano, acrocefalia, andar médio da face recuado, pavilhões auriculares de implantação baixa, hipertelorismo, exoftalmia, pirâmide nasal curta e larga, desvio inferior dos ângulos das comissuras labiais, mandíbula proeminente, fenda palatina no palato mole, úvula bífida e sindactilia completa dos pés e dos quatro dedos das mãos com polegares livres. Foi colocada a hipótese de SA. Enquanto não obtivermos correção do defeito molecular, a abordagem desta patologia é multidisciplinar. O tratamento deverá ser individualizado às características clínicas distintas do fenótipo do doente. ABSTRACT Apert syndrome is a rare genetic disorder. A term female newborn was born by a euthocic delivery of an uncomplicated pregnancy. The prenatal ultrasound was reported as normal. On examination the girl´s skull appeared turribrachycephaly with a flat occiput. She also presented retruded midface, low-set ears, exophthalmia, slight hypertelorism, short broad nose, prominent mandible, down-turned corners, cleft palate, bifid uvula and symmetric syndactyly of the fourth digits of the hands and all the toes of the feet. A provisional diagnosis of Apert’s Syndrome was established. Until there is a means to correct the molecular defect, management must rely on a strong multidisciplinary approach. Treatment should be tailored to each individual patient’s needs.