1/4. A rare case of upper airway obstruction in an infant caused by basal encephalocele complicating facial midline deformity.A four-month-old male infant with basal encephalocele of the transsphenoidal type presented with upper airway obstruction and facial midline deformity, including cleft lip, cleft palate, hypertelorism and exophthalmos. Basal encephalocele is a rare disease, and usually not detectable from the outside. In this case, initially the cause of an upper airway obstruction was considered to be posterior rhinostenosis, and posterior rhinoplasty with inferior nasal conchectomy was scheduled. However, in preoperative examination, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a bony defect in the sphenoidal bone and a cystic mass in communication with cerebrospinal fluid, herniating into the nasal cavity through the bony defect. The mass was diagnosed as a transsphenoidal encephalocele, the scheduled operation cancelled, and tracheostomy performed for airway management. The possibility of basal encephalocele should be considered in the case of upper airway obstruction with facial midline deformity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. An 80-year-old woman with chronic nasal congestion.Nasal congestion is a common problem in outpatient allergy and immunology. Here, we present the case of an 80-year-old woman with long-standing nasal congestion of uncommon cause and discuss its diagnosis, treatment, and prognosis. Although most patients with chronic nasal congestion do not have a life-threatening condition, it is important to remain vigilant for warning signs that a rare disease is at work behind this common complaint.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Eosinophilic angiocentric fibrosis.Eosinophilic angiocentric fibrosis (EAF) is a rare disease of the sinonasal tract, with histologic characteristic features like thick collagen bundles whirling around vessels in a fibrotic stroma with inflammatory cells rich in eosinophils. The Authors present a case of a 31-year-old man with bilateral nasal obstruction with no history of allergies or other systemic disease. The patient underwent a septoplasty with symptoms relieving. An EAF diagnosis was made. Differential diagnosis must rule out other lesions that may mimic EAF such as granuloma faciale, Kimura disease, wegener granulomatosis, churg-strauss syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Primary localized nasopharyngeal amyloidosis. A case report.Primary localized amyloidosis of the nasopharynx is a rare disease. We present the case of a 13-year-old girl, to our knowledge only the second pediatric case reported in the literature. Symptoms were nasal obstruction and bleeding from the oral cavity. physical examination revealed a mass in the nasopharynx and left side of the soft palate. The diagnosis was made by biopsy and histopathologic study of the surgical specimen. Immunohistochemical study revealed amyloid light chains (AL). The main treatment was surgical. At follow-up 9 months later, no recurrence had developed. While rare, localized amyloidosis should be considered in the differential diagnosis of nasal obstruction, epistaxis and glue ear, and must be recognized and understood by the otolaryngologist to allow appropriate diagnostic and therapeutic planning.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |