Sachdeva Kavita, Upadhyay Aparaajita
Department of E.N.T., NSCB Medical College, A 202, Kachnar Sambhar, 365-Napier Town, Jabalpur, Madhya Pradesh 482003 India.
Indian J Otolaryngol Head Neck Surg. 2017 Dec;69(4):527-534. doi: 10.1007/s12070-017-1203-5. Epub 2017 Sep 9.
The destructive lesions affect midline facial areas causing extensive destruction. These lesions are challenging till date due to peculiarity in presentation and non availability of any single objective test. An attempt has been done to evaluate these different clinical presentations of lesions presenting at a single centre and the challenges faced by us at a tertiary government setup. Prospective study done on 15 patients presenting in department during period of 2006-2016. After a detailed history, hematological and biochemical markers, ESR, radiographical tests, pus culture, C ANCA, CECT PNS and histopathogical evaluation were done. 12 out of 15 patients were male; Most common age group was above 45 years of age. All cases had ulceration of nose, 9 had cartilaginous destruction and 3 had osseocartilaginous destruction. 5 cases of mucormycosis had hyperglycemia with 2 having ketonuria and 4 had nerve palsies. All patients needed multiple biopsies. 3 cases of wegners and 4 cases were suspected of IMDD and immunohistochemistry was advised. C ANCA was negative in wegners cases as they were in limited form. Mutilating diseases of face usually present at an advanced stage with diverse etiology, are slowly progressive and require detailed evaluation. Due to lack of availability of IHC markers and C-ANCA at every centre it is difficult to diagnose them. The treatment is challenging due to incomplete response and frequent relapses. These cases require proper work up plan and perseverance leads to a final diagnosis.
破坏性病变累及面部中线区域,造成广泛破坏。由于其临床表现的特殊性以及缺乏单一客观检测方法,这些病变至今仍具有挑战性。我们尝试对在单一中心出现的这些病变的不同临床表现以及在三级政府机构中所面临的挑战进行评估。对2006年至2016年期间在该科室就诊的15例患者进行了前瞻性研究。在详细询问病史后,进行了血液学和生化指标、血沉、影像学检查、脓液培养、C-ANCA、鼻窦CT平扫及增强扫描以及组织病理学评估。15例患者中有12例为男性;最常见的年龄组为45岁以上。所有病例均有鼻部溃疡,9例有软骨破坏,3例有骨软骨破坏。5例毛霉菌病患者有高血糖,2例有酮尿,4例有神经麻痹。所有患者均需要多次活检。3例韦格纳肉芽肿病患者和4例疑似免疫介导的深部真菌病患者建议进行免疫组化检查。韦格纳肉芽肿病患者的C-ANCA呈阴性,因为他们处于局限型。面部毁损性疾病通常在晚期出现,病因多样,进展缓慢,需要详细评估。由于每个中心都缺乏免疫组化标志物和C-ANCA,因此难以诊断这些疾病。由于反应不完全和频繁复发,治疗具有挑战性。这些病例需要适当的检查计划,坚持下去才能最终确诊。