ABSTRACT
Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare autosomal dominant multisystem disorder. It is a mucocutaneous and fibrovascular dysplasia, the diagnosis of which is based on the fulfillment of the four Curaçao criteria: 1) recurrent epistaxis; 2) dermatovascular mucosal telangiectasias at characteristic sites: skin of the face, ears, fingertips, lips, tongue, and oral and nasal cavity; 3) arteriovenous malformations (AVMs) of visceral organs and central nervous system; and 4) family history: diagnosis of HHT in a first-degree relative. We describe a case of a 76-year-old patient who presented to our department with clinical manifestations of HHT in the skin (face, fingertips), lips, hard palate, tongue, ears, and nasal cavities. Individual and family history was obtained, as well as clinical laboratory examination, pan-endoscopy of the ear, nose, and throat (ENT) systems, and treatment of active foci of bleeding from the above areas. The otolaryngologist may be the first doctor to suspect Rendu-Osler-Weber syndrome and the one responsible for treating patients with HHT since recurrent epistaxis is the most frequent (90-96% of patients) and the earlier manifestation of the disease and the main reason for the arrival of these patients in the Emergency Department. The purpose of this study is to present a clinical case of Rendu-Osler-Weber syndrome with multiple ENT manifestations, as well as a review of the literature on their management and treatment.
ABSTRACT
Primary parapharyngeal space tumors are rare, and due to the complex anatomy of the parapharyngeal space, their diagnosis and treatment are challenging. Pleomorphic adenoma is the most common histologic type followed by paragangliomas and neurogenic tumors. They can present as a neck lump or an intraoral submucosal mass with the displacement of the ipsilateral tonsil or might be asymptomatic and discovered incidentally on imaging obtained for other reasons. Magnetic resonance imaging (MRI) with gadolinium is the imaging of choice. Surgery remains the treatment of choice and many approaches have been described. In this study, we present three patients with PPS pleomorphic adenoma (two primary and one recurrent), which were resected successfully with a transcervical-transparotid approach without mandibulotomy. Division of the following anatomical structures: the posterior belly of the digastric muscle, stylomandibular ligament, stylohyoid muscle and ligament, and styloglossus muscle is a very important tip for the surgeons because enables displacement of the mandible providing excellent exposure for complete tumor excision. The only postoperative complication was temporary facial nerve palsy in two patients who fully recovered within two months. The aim of this mini case series is to present our experience, together with some tips and benefits of the transcervical-transparotid approach for the resection of pleomorphic adenomas of the PPS.
ABSTRACT
In this paper, we present the case of acute airway obstruction due to tracheal carcinoma in a patient with glottic stenosis due to previously treated laryngeal carcinoma. Because of severe dyspnea from the obstructive endotracheal mass, tracheotomy under local anesthesia was immediately performed. Intubation with pediatric size (I.D. 4.5 mm) cuffed endotracheal tube was performed by the surgeon through tracheostomy under endoscopic visualization. Blakesley forceps and electrocautery were used for tumor debulking. Postoperatively there were no complications and the patient was discharged after four days. The histopathology report showed a squamous cell carcinoma. The tumor board decided on adjuvant chemoradiotherapy for the treatment of the patient.
