Giannuzzi Anna Lisa, Merkus Paul, Taibah Abdelkader, Falcioni Maurizio
Gruppo Otologico, Piacenza, Italy.
Ann Otol Rhinol Laryngol. 2011 Nov;120(11):700-6. doi: 10.1177/000348941112001102.
We evaluate 3 new cases of congenital cholesteatoma confined to the mastoid process, and compare them with cases presented in the literature in order to better define this rare lesion.
We performed a retrospective chart analysis of all congenital cholesteatomas treated surgically in a tertiary referral and skull base center. We performed a complete analysis (history, radiologic, and surgical) of all patients with congenital cholesteatoma confined to the mastoid process; we then performed a literature review and compared our findings with the presented cases.
The results of preoperative imaging were in line with the surgical findings. The most important surgical issue in this type of lesion was the management of the sigmoid sinus and the jugular bulb. Half of the cases previously reported in the literature appeared not to fulfill the definition criteria of a congenital cholesteatoma of the mastoid process.
Congenital cholesteatoma confined to the mastoid process is a rare lesion, and is even more exceptional upon critical review of the literature. Symptoms are often lacking or nonspecific, and although cases have a congenital origin, the diagnosis often is not made until adulthood. A combined congenital cholesteatoma group with middle ear and mastoid features seems to fill in the gap in the definition. Management of the sigmoid sinus and the jugular bulb is the most demanding surgical key point.
我们评估3例局限于乳突的先天性胆脂瘤新病例，并将其与文献中报道的病例进行比较，以便更好地界定这种罕见病变。
我们对在一家三级转诊及颅底中心接受手术治疗的所有先天性胆脂瘤病例进行了回顾性病历分析。我们对所有局限于乳突的先天性胆脂瘤患者进行了全面分析（病史、影像学和手术方面）；然后进行了文献综述，并将我们的发现与所报道的病例进行比较。
术前影像学结果与手术发现一致。这类病变最重要的手术问题是乙状窦和颈静脉球的处理。文献中先前报道的病例有一半似乎不符合乳突先天性胆脂瘤的定义标准。
局限于乳突的先天性胆脂瘤是一种罕见病变，经严格的文献回顾后更是如此。症状往往缺乏或不具特异性，尽管病例起源于先天性，但诊断通常要到成年期才作出。具有中耳和乳突特征的先天性胆脂瘤组合似乎填补了定义上的空白。乙状窦和颈静脉球的处理是最具挑战性的手术关键点。
