Sarkar Shrita, Seth Chandan, Dutta Mainak, Bandyopadhyay Saumendra Nath
Department of Otorhinolaryngology and Head-Neck Surgery, Medical College and Hospital, Kolkata, West Bengal, India.
Department of Otorhinolaryngology and Head-Neck Surgery, M R Bangur Super Speciality Hospital, Kolkata, West Bengal, India.
Ear Nose Throat J. 2024 Apr 18:1455613241249022. doi: 10.1177/01455613241249022.
A 24-year-old man with von Recklinghausen's disease presented with complaints of difficulty in swallowing for 6 months and change of voice for 3 months. He also had recent-onset difficulty in breathing. Telelaryngoscopy and subsequent contrast-enhanced computed tomography scan revealed a well-defined, smooth submucosal mass in the oropharynx (attached to the posterior pharyngeal wall, superior to the level of left aryepiglottic fold), obscuring the ipsilateral pyriform fossa, and nearly blocking the pharyngeal lumen. The mass was removed with endoscopic coblation-assisted laryngeal surgery, and subsequent histopathology revealed it to be neurofibroma. Neurofibromas are rare neoplasms to be encountered in the oropharynx. However, in the setting of von Recklinghausen's disease (neurofibromatosis type 1), one or more well-demarcated, submucosal nodular lesions in the upper aerodigestive tract may be considered as neurofibromas, and workup and treatment should be directed accordingly based on this clinical presumption. Endoscopic coblation during laryngeal surgery can effectively be used as a surgical tool to excise such lesions. It provides a relatively bloodless field compared to the conventional cold steel excision, and reduces the risk of complications at surgery and during the follow-up period. This clinical record illustrates the presentation and management of a solitary, isolated oropharyngeal neurofibroma in a man suffering from von Recklinghausen's disease. It further emphasizes the role of endoscopic coblation-assisted laryngeal surgery in this setup, and the need to maintain a low threshold of suspicion in having a provisional clinical diagnosis of such lesions.
一名患有冯雷克林霍增氏病的24岁男性,出现吞咽困难6个月及声音改变3个月的症状。他近期还出现了呼吸困难。电子喉镜检查及随后的增强计算机断层扫描显示,口咽部有一个边界清晰、表面光滑的黏膜下肿物(附着于咽后壁,位于左侧杓会厌襞水平上方),遮挡了同侧梨状窝,几乎阻塞了咽腔。该肿物通过内镜下低温等离子辅助喉部手术切除,随后的组织病理学检查显示为神经纤维瘤。神经纤维瘤在口咽部较为罕见。然而,在冯雷克林霍增氏病(1型神经纤维瘤病)的情况下,上呼吸道消化道出现的一个或多个边界清晰的黏膜下结节性病变可被视为神经纤维瘤,应基于这一临床推测进行相应的检查和治疗。喉部手术中的内镜下低温等离子技术可有效用作切除此类病变的手术工具。与传统的冷钢切除相比,它提供了一个相对无血的手术视野,并降低了手术及随访期间的并发症风险。本临床记录阐述了一名患有冯雷克林霍增氏病的男性患者孤立性口咽部神经纤维瘤的表现及治疗。它进一步强调了内镜下低温等离子辅助喉部手术在此类病例中的作用,以及在对此类病变进行初步临床诊断时保持低怀疑阈值的必要性。