婴幼儿中耳畸胎瘤3例临床分析

Middle ear teratoma in infant： report of three cases and review of the literatures

目的分析婴幼儿中耳畸胎瘤的临床特点和诊疗经验，以减少其术前误诊及漏诊，总结制定完善的诊疗方案。方法对2012--2015年首都医科大学附属北京友谊医院诊治的3例中耳畸胎瘤患者的临床资料进行回顾性分析。结果3例患者均为婴幼儿期起病，病程进展缓慢，以单侧耳流脓、听力下降为主要症状。耳镜检查耳道内可见肉芽样组织。听力学改变据病变程度轻重不一。影像学特点为：患侧咽鼓管走行区域的囊袋状或包裹状占位病变，颞骨CT示乳突、鼓室内类似于中耳炎性反应的软组织密度影，外耳道可见扩大和骨质破坏。2例术前行MRI检查，提示病变区域混杂信号影，边界清楚。3例患儿均在全麻下行中耳肿物切除术，其中1例同期行鼓室成形术。术后病理均诊断为成熟畸胎瘤。术后随访17～54个月，复查MRI均提示肿瘤完全切除，未见复发。结论中耳畸胎瘤为头颈部罕见肿瘤，如婴幼儿以单侧耳流脓及听力下降起病，外耳道可见肉芽样物，影像学提示咽鼓管、鼓室及乳突区域的边界清楚的软组织密度影，尤其是囊袋状或包裹状的软组织密度影时，应警惕畸胎瘤的可能性。颞骨高分辨率CT结合MRI检查可以提高术前中耳畸胎瘤的诊断率。一旦确诊，应尽早手术，完全切除病变。术后定期随访，必要时行影像学检查，早期排除肿瘤复发。

Objective To summarize the clinical characteristics and therapeutic experiences of the middle ear teratoma in infants. Methods Three cases of middle ear teratoma, from 2012 - 2015 in Beijing Friendship Hospital were analyzed. Results The three cases all developed slowly and presented unilateral otorrhea and hearing loss. Otoseopy showed the granulation tissue in the external ear canal. Audiological changes varied according to the degree of severity. Imaging features showed the pocket-like occupancy lesions in the Eustachian tube area. The temporal bone CT showed mass with soft tissue density usually involved in the mastoid and tympanic cavity. MRI showed mixed signal intense on both T1 and T2 weighted imaging. All the three cases received neoplasm resection of the middle ear. Only one ease received tympanoplasty surgery at the same time. And all the pathology results displayed mature teratoma. The follow- up time was 17 to 54 months. MRI showed complete removal of the tumor. Conclusions Teratoma are rare in the head and neck neoplasm. When the infants suffer from the unilateral otorrhea, hearing loss, and granulation tissue formed in the external ear canal, it should be vigilant for teratoma. The differential diagnosis is middle ear eholesteatoma, congenital first branchial cyst or fistula, and middle ear carcinoma. Temporal bone CT combined with MRI could improve the accuracy of diagnosis. It should be totally resection as soon as possible if there is no contraindication. Postoperative follow-up and imaging examination are necessary to eliminate tumor recurrence.