Nagato Toshihiro, Otaka Ryuki, Wada Tetsuji, Kanai Naoki, Harabuchi Yasuaki
Department of Otolaryngology - Head and Neck Surgery, Asahikawa Medical University, Asahikawa, Japan.
Int J Pediatr Otorhinolaryngol. 2012 May;76(5):754-6. doi: 10.1016/j.ijporl.2012.02.032. Epub 2012 Mar 3.
Congenital cholesteatoma may originate at various sites in the temporal bone. Congenital cholesteatoma of the mastoid origin shows a variable clinical presentation, although the least common site is the mastoid process. We report an extremely rare case of congenital cholesteatoma isolated to the mastoid presenting as stricture of the external auditory canal. A 10-year-old boy presented with stricture of the left-sided external auditory canal caused by bulging of the posterior wall of the external auditory canal. Computed tomography showed destruction of the posterior wall of the external auditory canal by a lesion of soft tissue density in the left mastoid cells. At surgery, cholesteatoma was observed in the mastoid cavity. Although destruction of the posterior wall of the external auditory canal was identified, the external auditory canal skin and tympanic membrane were intact, and the aditus ad antrum mucosa was normal. Congenital cholesteatoma isolated to the mastoid was diagnosed. Diagnosis of congenital cholesteatoma isolated to the mastoid should be based on clinical examination, radiological evaluation, and surgical findings. In addition, the possibility of congenital cholesteatoma isolated to the mastoid should be considered in patients with stricture of the external auditory canal.
先天性胆脂瘤可起源于颞骨的不同部位。乳突起源的先天性胆脂瘤临床表现多样，尽管乳突是最不常见的部位。我们报告了一例极为罕见的孤立于乳突的先天性胆脂瘤，表现为外耳道狭窄。一名10岁男孩因外耳道后壁膨出导致左侧外耳道狭窄前来就诊。计算机断层扫描显示左侧乳突气房内软组织密度病变破坏了外耳道后壁。手术中，在乳突腔内观察到胆脂瘤。尽管外耳道后壁有破坏，但外耳道皮肤和鼓膜完整，鼓窦入口黏膜正常。诊断为孤立于乳突的先天性胆脂瘤。孤立于乳突的先天性胆脂瘤的诊断应基于临床检查、影像学评估和手术所见。此外，外耳道狭窄患者应考虑孤立于乳突的先天性胆脂瘤的可能性。
