Cystic lymphatic malformation of the middle ear

Objectives: We review the clinical, radiologic, and histopathologic features of cystic lymphangioma of the middle ear, and discuss the developmental etiology and management of such a lesion. Methods: We present an unusual location for the development of a cystic lymphangioma with emphasis on etiology, clinical implications, and current treatment. Results: A 10-year-old girl presented with a mass involving the medial surface of the right tympanic membrane. T2-weighted magnetic resonance imaging demonstrated a hyperintense lesion in the anterior-superior middle ear cavity without evidence of vascular abnormalities. Conclusions: To our knowledge, this is the only report of lymphangioma involving the middle ear represented in the English-language literature. Such a lesion has been demonstrated to arise from abnormalities in growth factors that contribute to the tightly regulated process of lymphangiogenesis. Lymphatic malformations can be diagnosed presumptively by virtue of magnetic resonance imaging in combination with a detailed physical examination. The treatment of choice for lymphangiomas located in the middle ear is surgical excision. Definitive diagnosis of the lesion is then made by identifying specific histopathologic characteristics. Although rare and histologically benign, middle ear lymphangiomas may produce significant patient discomfort and ultimately a conductive hearing loss. Therefore, these lesions warrant early recognition and treatment.