Hereditary Hemorrhagic Telangiectasia With Multiple Ear, Nose, and Throat (ENT) Manifestations: A Case Report.

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.

Hemorrhage of Upper Digestive and Respiratory Tracts in a Child with Glanzmann Thrombasthenia.

Glanzmann thrombasthenia (GT) is an autosomal recessive platelet disorder that could cause mild to severe bleeding episodes. The incidence is approximately 1 per 1,000,000 births. Patients with GT frequently have mucocutaneous bleeding with absent platelet aggregation in response to all physiologic stimuli, but a normal platelet count and morphology. Specifically, the glycoprotein IIb/IIIa (GP IIb/IIIa) complex is either inadequate or dysfunctional. This case reports a 3.5-year-old boy with Glanzmann thrombasthenia who had two episodes with uncontrolled hemorrhage from upper digestive and respiratory tracts, the first with the bleeding point found in the left tonsil and the second in the adenoids.

Seromucinous Hamartoma of the Lateral Nasal Wall with Infiltration of the Orbit: A Rare Case Report and Review of the Literature.

Seromucinous hamartoma is a rare benign glandular proliferation arising from the respiratory epithelium of the sinonasal tract and nasopharynx. It was described for the first time in 1974 by Baillie and Batsakis. Since then, few cases have been reported in the literature with most of them occurring in the posterior nasal septum. We report the case of a 52-year-old woman that presented to our department with left periorbital edema, pain, and dacryorrhea due to seromucinous hamartoma arising from the left inferior turbinate and extending through the lateral nasal wall into the maxilla, the nasolacrimal duct, and the orbit. Endoscopic medial maxillectomy and endoscopic transnasal orbital tumor resection were performed. The patient remains symptom-free for 16 months, till her most recent follow-up. Seromucinous hamartoma of the nasal cavity is an exceedingly rare diagnosis, especially in the lateral nasal wall. It should be included in the differential diagnosis of nasal tumors. According to the literature review, this is the first case report of seromucinous hamartoma with orbit infiltration. Endonasal endoscopic resection is the treatment of choice.

Resection of Primary and Recurrent Parapharyngeal Space Pleomorphic Adenomas via a Combined Transcervical-Transparotid Approach: A Case Series.

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.

Differential Diagnosis of Cervical Lymph Nodes with Ultrasound and Virtual Touch Imaging Quantification: a Report of Three Cases.

Cervical lymphadenopathy is a common clinical presentation, and its differential diagnosis is challenging. In this article we present three cases with enlarged cervical lymph nodes evaluated with combination of ultrasound (US) and virtual touch imaging quantification (VTIQ) performed by the head and neck surgeon G.P. with a 10-year experience in ultrasound elastography (USE). In the first two cases, US and VTIQ evaluation showed predominance of areas with increased stiffness and higher share wave velocities compared with those in the third case. Malignancy was suspected in the first and second cases. The histology report revealed metastatic squamous cell carcinoma in the first two cases and chronic non-specific lymphadenopathy in the third case. The development of VTIQ share wave elastography has improved the diagnostic accuracy of ultrasonography and can guide clinical decision when dealing with cervical lymphadenopathy.

Management of an Acute Airway Obstruction Due to Tracheal Carcinoma in a Patient With Severe Glottic Stenosis.

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.

Parenchymal Brain Abscess as an Intracranial Complication After Sinusitis.

Brain abscesses caused by sinusitis are rare in the antibiotic era. The purpose of the current manuscript was to report a rare case of a brain abscess located mainly in the frontal lobe after sinusitis, which was initially thought to be meningitis or encephalitis. A 39-year-old man was transferred to our hospital from another neighbouring hospital with tonic-clonic seizures, severe headache, and purulent nasal secretions. For one week, he was taking antibiotics for sinusitis. The computed tomography indicated lesions in the right sinuses but not in the parenchymal brain and thus antibiotics along with antiepileptic drugs were given. However, due to the deterioration of symptoms, magnetic resonance imaging was executed, which revealed an abscess in the frontal lobe. Afterward, an anterior ethmoidectomy and middle maxillary antrostomy were performed in order to drain the purulent content from the right sinuses. Ten days later, the patient presented disorientation and thus an open craniotomy for successful removal of the parenchymal abscess was performed. One month later, the patient was discharged with mild irritability, which was eliminated gradually over the next two months. Conclusively, brain abscesses can be caused by local spread from an infection of the paranasal sinus. The contribution of imaging modality is very significant not only for the early diagnosis but also for the therapeutic management of such cases. Frequently antibiotic treatment is insufficient and surgery may be required.

Intraoperative flow cytometry for head and neck lesions. Assessment of malignancy and tumour-free resection margins.

Head and neck cancer poses a significant health problem worldwide. We set out to investigate the value of rapid intraoperative cell cycle analysis by flow cytometry for the intraoperative characterization of head and neck lesions and surgical margins. Seventy patients with head and neck lesions suspicious of malignancy were included in the study. There were 31 neoplastic and 39 benign lesions. Flow cytometry permitted the intraoperative detection of neoplastic lesions within 6 min with high sensitivity and specificity based on cell cycle fractions. In the cases in which surgical margins were assessed, intraoperative flow cytometry had complete concordance with pathology. Intreoperative flow cytometry is a novel promising technique for rapid intraoperative characterization of malignancy and tumour free resection margins in head and neck lesions.