King Sarah, O'Connor Mackenzie, Winchester Arianna, Bartellas Michael, Roland J Thomas
Department of Otolaryngology-Head and Neck Surgery. University of Kansas School of Medicine, Kansas City, Kansas, USA.
Department of Otolaryngology-Head and Neck Surgery, New York University Grossman School of Medicine, New York, New York, USA.
J Int Adv Otol. 2025 May 27;21(3):1-5. doi: 10.5152/iao.2025.251894.
Congenital cholesteatomas are defined as a collection of epithelium in the absence of prior surgery or pathologic retraction. They are most commonly found in the middle ear and are thought to arise from a residual epithelial rest present since birth; however, a small number of cases present with disease isolated to the mastoid bone. One such case and a review of prior reports are presented. A 29-year-old male with a 2-year history of headaches and no otologic surgery was found to have a destructive mass centered in the right mastoid bone, invading the jugular foramen and posterior fossa without middle ear disease. He underwent a mastoidectomy with complete resection of the cholesteatoma. A literature review identified 21 prior cases of isolated mastoid congenital cholesteatoma. Congenital cholesteatoma should be considered in the differential diagnosis when patients present with postauricular pain or headaches. Mastoidectomy is considered the treatment of choice.
先天性胆脂瘤被定义为在无既往手术或病理性回缩情况下的上皮组织聚集。它们最常见于中耳，被认为起源于自出生就存在的残余上皮细胞巢；然而，少数病例的病变仅局限于乳突骨。本文报告了这样一例病例并回顾了既往报道。一名29岁男性，有2年头痛病史，未接受过耳科手术，被发现有一个以右侧乳突骨为中心的破坏性肿物，侵犯颈静脉孔和后颅窝，而中耳无病变。他接受了乳突切除术，完整切除了胆脂瘤。文献回顾确定了既往21例孤立性乳突先天性胆脂瘤病例。当患者出现耳后疼痛或头痛时，鉴别诊断应考虑先天性胆脂瘤。乳突切除术被认为是首选治疗方法。
