Hidaka H, Ishida E, Kaku K, Nishikawa H, Kobayashi T
Department of Otolaryngology-Head and Neck Surgery, Tohoku University Graduate School of Medicine, Sendai, Japan.
J Laryngol Otol. 2010 Jul;124(7):810-5. doi: 10.1017/S0022215109992209. Epub 2009 Dec 11.
We report an extremely rare case of congenital cholesteatoma of the mastoid region, presenting as acute mastoiditis. We also review the 16 previously reported cases of congenital cholesteatoma of the mastoid region.
A 65-year-old man presented with left-sided, post-auricular swelling and pain. Acute mastoiditis was diagnosed, with computed tomography demonstrating destruction of the bony plates of the posterior cranial fossa and sigmoid sinus. Initial surgery revealed a cholesteatoma in the mastoid, with no extension into the aditus ad antrum or attic. These findings were confirmed by pathological and immunohistochemical analysis of the surgical specimen, the latter using involucrin. The cholesteatoma matrix was completely removed in a second operation.
Including this case, only four of the 17 reported cases of congenital cholesteatoma of the mastoid region showed post-auricular pain or swelling, indicating acute mastoiditis. Clinicians should bear in mind that congenital cholesteatoma may be present in patients presenting with mastoiditis, particularly adults.
我们报告一例极为罕见的乳突区先天性胆脂瘤，表现为急性乳突炎。我们还回顾了此前报道的16例乳突区先天性胆脂瘤病例。
一名65岁男性，出现左耳后肿胀和疼痛。诊断为急性乳突炎，计算机断层扫描显示后颅窝骨板和乙状窦破坏。初次手术发现乳突有胆脂瘤，未延伸至鼓窦入口或上鼓室。手术标本的病理和免疫组织化学分析证实了这些发现，后者使用了外皮蛋白。在第二次手术中完全切除了胆脂瘤基质。
包括本病例在内，17例报道的乳突区先天性胆脂瘤病例中，只有4例表现为耳后疼痛或肿胀，提示急性乳突炎。临床医生应牢记，乳突炎患者，尤其是成人患者，可能存在先天性胆脂瘤。
