Bosma arhinia microphthalmia syndrome (BAMS) is a rare condition, with about 100 cases identified worldwide. It is characterized by nasal and ophthalmic abnormalities, as well as disturbances in puberty and sexual development. The cardinal sign is arhinia, though some cases have partial aplasia of the external nose. In addition, several reports have revealed abnormal brain structure, including changes to the olfactory bulbs. This case describes a 29-year-old female who has suffered from BAMS since birth. On presentation, she was noted to have congenital arhinia, bilateral microphthalmia, vision loss, mouth-breathing, an unclear speaking voice, a high arched or cleft palate, and a hypoplastic maxilla. Her paranasal sinuses were ossified and underdeveloped. This syndrome occurs rarely, both within Vietnam and worldwide. It is characterized by four major features: arrhinia, complete absence of the paranasal sinuses, eye defects, and absent sexual maturation. This case report describes the presentation of the disorder to improve otolaryngologists’ understanding of BAMS. Criteria for diagnosis consist of arhinia, midface hypoplasia (with a hypoplastic maxilla), hypogonadotropic hypogonadism, and normal intellectual abilities. Additional important findings are microphthalmia with or without coloboma, anosmia, maxillary hypoplasia, a high-arched palate, and absence of paranasal sinuses and olfactory bulbs.
Background: Surgical resection of a large anterior skull base (ASB) tumor and sinonasal maglinancies with intracranial extension will result a large skull base defect. Reconstruction of large ASB defects by using traditional techniques may result in high risk of postoperative CSF leakage, meningitis and increase mortality rate. The use of pedicled double flap technique to reconstruct the anterior skull base defect may decrease the complications. In this study, we examine the clinical outcomes of patients who underwent this double flap reconstruction technique after the resection of sinonasal malignancies with significant intracranial extension at Cho Ray hospital, Vietnam. Methods: Case series study was conducted at Cho Ray hospital from 09/2010 to 09/2020. All patients with large sinonasal malignancies with intracranial invasion underwent combined transbasal - EEA approach. Reconstruction of large skull base defect ( > 2 cm ) was followed by using the pedicled double flap technique. Results: There were 75 patients who underwent the modified multi-layer with double flap reconstruction technique after the resection of ASB tumor from 09/2010 to 09/2020. The skull base defects were commonly seen at the horizontal plate of the ethmoid and the roof of the ethmoid ( 98.6%). The large skull base defects ( > 2cm) accounted for 81.3%. The risk of postoperative CSF leakage after double flap repair was very low. In this study, we had 1 patient with postoperative CSF leakage and 1 patient had postoperative meningitis. Conclusion: The use of two vascularize pedicled flap may decrease the incidence of postoperative cerebral spinal fluid (CSF) leakage and meningitis. This technique is an effective method for the reconstruction of the ASB with large defect.
Introduction: the Coronavirus disease 2019 (COVID-19) pandemic caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) continues to spread all over the world. This situation brings medical facilities into an emergency state and the huge need for medical staff in the diagnosis and treatment of infected patients. Therefore, it is necessary to standardize knowledges and therapeutics agents of hospitalized patients or field hospitals. The article was aimed to summarize the clinical progression, risk factors and treatment protocols for COVID-19 patients around the world and practical guidelines in Vietnam. These help new medical staff that update knowledges quickly. Methods: review the latest approaches and guidelines of the Vietnamese Ministry of Health on the diagnosis and treatment of Covid-19 patients. Results: we summarize specific treatment guidelines for Covid-19 patients into treatment protocols according to the level of severity. The regimen is being applied at medical facilities, field hospitals, and Covid-19 treatment hospitals in Vietnam. Conclusion: the strategy of COVID-19 treatment is monitoring and detecting cases with signs of severe progression and limiting mortalities. Management protocols according to the level of severity provides standardized regimens and comprehensive information of COVID-19.
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