Search

Introduction: Amyloidosis is a rare clinical entity characterized by the extracellular deposition of insoluble proteins. Most patients have multi-organ involvement, while localized amyloidosis accounts for only 10-20% of cases. Among localized ones, 61% of head and neck manifestations are located in the larynx, with rhinopharynx involvement being rare (3%). Objective: To report an unusual case of oropharyngeal and nasopharyngeal amyloidosis with clinical repercussions. Resumed report: Male patient, 46 years old, referred to otolaryngology due to an oropharyngeal lesion, presenting sensation of fullness in the left ear, mild hoarseness and snoring. Physical examination showed a yellowish, polypoid, lobulated, sessile-based lesion on left posterolateral wall of the oropharynx. In addition, there were smaller, similar-looking lesions in the rhinopharynx, posteriorly to the Eustachian tube, and on the laryngeal surface of the epiglottis. A biopsy of the oropharyngeal lesion was performed, stained with Congo red and resulting positive for a lambda-type immunoglobulin light chain amyloid deposit. Chest tomography without findings of amyloidoses and neck tomography with description of an rhinopharynx elongated image measuring 36 mm in its longest axis. Cardiovascular and kidney exams without change. The patient remains under clinical follow-up while waiting for surgical resection. Conclusion: Although amyloidosis localized at pharynx are rare, the differential diagnosis must be considered when examining this region. There is no evidence suggesting a progression from untreated localized amyloidosis to systemic disease. However, there is an ongoing debate between surgical excision and a wait-and-see approach. [ABSTRACT FROM AUTHOR]

Introduction: External auditory canal cholesteatoma (EAC) is a disease characterized by abnormal growth of the squamous epithelium, which may evolve with progressive bone destruction and involvement of adjacent structures. Objectives: The objective of this work is to illustrate from a case, the trauma-related EAC cholesteatoma. Resumed report: G.S.S, 13 years old, with a history of perforating trauma in the left EAC in october 2021, evolving with left repetition otorrhea. After 2 months, she reports an attempt to remove a foreign body (FB) with intense aural manipulation. She made previous use of amoxicillin + clavulanate and cephalexin, without improvement. Due to the worsening of the condition, she was admitted to a pediatric hospital on 03/26/2022 with otalgia and otorrhea on the left. She had a polypoid lesion obstructing the left EAC at otoscopy. Computed tomography of the mastoids was performed, showing opacification of mastoid cells, content with density of soft parts and erosion of the anterior and posterior wall of the ipsilateral EAC. She underwent resection of a lesion described through retroauricular access, canalplasty and meatoplasty on 03/29/2022, with two lesions being evidenced: Medial pearly lesion in the conduct and lateral granulomatous process. The histopathological study revealed a chronic inflammatory response of the FB type and left EAC cholesteatoma. Patient remains under post-surgical follow-up in the otorhinolaryngology unit of the University Hospital of Brasília. Conclusion: The EAC cholesteatoma can present several aspects and etiologies, with trauma being an etiology to be considered. [ABSTRACT FROM AUTHOR]

Introduction: Hermansky-Pudlak syndrome is an autosomal recessive multisystem disease characterized by oculocutaneous albinism, hemorrhagic diathesis and pulmonary fibrosis in some subtypes. Rare disease that occurs in 1 in 1,000,000 individuals worldwide. Deficiency of the platelet storage pool is associated with a lack of dense bodies in platelets, with an impaired response in the secondary phase of aggregation. Patients with HPS have normal clotting tests, however their bleeding time is prolonged despite normal or increased platelet counts Objectives: To describe a complication resulting from a rare genetic syndrome in the postoperative period of nasal surgery. Resumed report: Patient, 26 years old, with albinism and spontaneous nystagmus, underwent septoplasty and inferior turbinectomy bilaterally in June 2021 due nasal obstruction refractory to clinic treatment. Coagulogram, hemogram and other laboratory tests preoperative were normal. Intraoperative patient has had an usual bleeding, difficult hemostasis and recurrent epistaxis in the outpatient visits. Outpatient visits in July 2021, patient has had spontaneous epistaxis bilaterally. Anterior packing was performed, but not failed on hemostasis. Nose Bleeding stopped just after platelet transfusion. Blood tests showed dysfunctional platelet aggregation test with epinephrine and hypoagglutination with ristocetin at high concentration. This alteration was responsive to administration os 50% of platelet-poor plasma. A hypothesis of platelet pool disease was discussed, with hematologic clinic physician: the main hypothesis was Hermansky-Pudlak Syndrome. Conclusion: This is a diagnosis to be considered in patients with albinism, associated with nystagmus and better preoperative planning and postoperative care should be performed. [ABSTRACT FROM AUTHOR]

Introduction: Paragangliomas are a rare neuroendocrine tumor which is divided in sympathetic or parasympathetic paragangliomas, differentiated by its ability to secrete catecholamines. Jugulotympanic glomus is less common pathology of parasympathetic. Usually in glomus jugulare tumor symptoms of mass effect, specially in middle ear presenting with pulsatile tinnitus or hearing loss are very common, without infectious or inflammatory history. Objectives: Report a history of otorrhea without tinnitus in Jugulotympanic glomus case Resumed report: T.F.L., female, 57 years complaining otorrhea in right ear for 2 months associated in headache, hearing loss without pulsatile tinnitus. Otoscopy presenting whitish, fetid secretion. After aspiration, tympanic membrane without complete definition, but opaque. Audiometry show a mixed deep hearing loss ipsilaterally and tomography imaging 'chronic inflammatory otomastoidopathy, not being able to rule out the possibility of cholesteatoma'. An assistant otolaryngology review, carotid canal dehiscence was suspected but discussed with neuroradiology it was not possible to confirm. The patient was proceeded to a mastoidectomy (by her inflammation history) and it was identified a pulsatile and violet mass at mastoid antrum. Then stopped the surgery and led the patient to an embolization assessment. Retrospectively we must suspect by a minimum possibility of jugular or carotid dehiscence. By that the inflammation cumbersome history was a pitfall of suspected tumors diagnosis, which could be assessed by MRI imaging. Conclusion: Jugulotympanic glomus are often silent and not aggressive tumors. Few cases could be suspected in an inflammation otorrhea history. [ABSTRACT FROM AUTHOR]

Introduction: Mucormycosis of the nose and paranasal sinuse is a rare, invasive fungal infection that mainly affects immunocompromised patients with higher morbidity and mortality. Objective: To report a non-fatal case of a patient with type 2 diabetes diagnosed with infiltrative mucormycosis of the nose and paranasal sinuse. Case report: A.P.L, 54 years, male, rural producer, uncontrolled type 2 dyabetes, hospitalized on 02/24/2022 due to left facial pain, ipsilateral edema and a painless lesion in the left hard palate, after tooth extraction surgery despite the use of Amoxicillin with Clavulanate and Levofloxacin. Tracheostomy followed by left maxillectomy and surgical debridement in left necrotic tissue was performed in urgency. Histopathological analysis suggestive of infiltrative mucormycosis, and culture of fungi with growth of Rhizoppus sp. During the hospitalization, glycemic control was achieved and polymyxin B, Meropenem and Amphotericin B lipid complex was used. However, he evolved with kidney dysfunction, elevation of canalicular enzymes, sepsis and an episode of acute myocardial infarction. After two months, CT paranasal sinus and brain resonances was performed with evidence of undefined lesion in right maxillary sinus and left orbit, followed by right maxillary antrostomy, Caldwell-Luc on the right and debridement of the left orbital floor with removal of fungus suggestive material. After prolonged hospitalization for 3 months, he was discharged with clinical improvement in use of oral isavuconazole Conclusion: Treatment of nasosinusal mucormycosis is complex. It involves a multidisciplinary team, and surgical treatment is essential for complete improvement. [ABSTRACT FROM AUTHOR]

Introduction: Paranasal sinus mucocele is defined as the accumulation and retention of mucoid secretion within the sinus, leading to distension and erosion of its bony walls. Sphenoid mucoceles, although rare, should be considered in patients with headache, visual disorders and eye paralysis. The differential diagnosis of lesions at skull base is made with other pathologies with a more reserved prognosis. Objectives: To report a case of large volume sphenoid mucocele, initially diagnosed as an inoperable tumor, evolving with facial paresthesia and visual loss due to resolution delay. Resumed report: 48-year-old female patient, with longstanding headache, underwent cranial CT during hospitalization for Covid-19 and an expansive lesion was seen at the skull base, suprasellar, compromising the cavernous sinus, measuring 53 mm in its longest axis, eroding the sellar floor and in close contact with optic chiasm. Referred to the otolaryngology for investigation, she presented with frontal headache, hypoesthesia of middle third of the left face, nasal obstruction on the left side and temporary left amaurosis evolving with left temporal hemianopia. The patient's nasal endoscopy revealed an expansive lesion medial to the middle turbinates, filling the left sphenoethmoidal recess. During the surgical approach, a large amount of purulent secretion was drained and the diagnosis of mucocele was confirmed. The patient got better of the temporal left hemianopia. Conclusion: The reported case became a surgical urgency due to impaired visual acuity. An earlier approach could avoid patient fatigue and decrease intraoperative risk. [ABSTRACT FROM AUTHOR]

Introduction: Johanson-Blizzard syndrome (JBS) is a rare autosomal recessive disorder with a frequently reported history of inbreeding. In addition to facial features, associated conditions include congenital heart disease, pancreatic dysfunction, hypothyroidism, hypopituitarism, mental retardation, sensorineural hearing loss. Sensorineural hearing loss occurs in most cases with a structurally normal inner ear. Objectives: To register a case of cochlear implant in a patient with JBS with an anatomical alteration of the inner ear. Resumed report: Female patient, 2 years old, child of a non-consanguineous couple, diagnosed with JBS. An image of the mastoids was performed, which showed cochleovestibular cystic dysplasia, characterized by the absence of modiolus and coalescence of the middle and apical turns. She underwent a left ear cochlear implant on 08/2021. She evolved with left peripheral facial palsy in the immediate postoperative period, and a cycle of corticotherapy was performed with maintenance of House-Brackmann grade II facial palsy. The patient presented a fall from his own height on 09/21 and evolved with an otocerebrospinal fluid leak, which was then submitted to surgical correction. Conclusion: According to the literature, approximately 20% of children with sensorineural hearing loss have radiological abnormalities associated with the temporal bone. Cochlear dysplasia accounts for 30% of these anomalies. The rate of postoperative complications is higher in patients with anomalous inner ear, with a higher risk of meningitis, cerebrospinal fluid fistula, and facial nerve paralysis. Despite this, the functional results achieved by these children are still poorly described. [ABSTRACT FROM AUTHOR]