Lymphatic Malformation of the Sphenoid Sinus in a Pediatric Patient

Abstract

Lymphatic malformation is a benign vascular lesion resulting from lymphatic tissue being isolated from the remainder of the lymphatic system. They are present at birth and up to 90% are diagnosed by 2 years of age. More aggressive lesions are usually diagnosed earlier, with low-grade lesions presenting later with fewer complications. These lesions are hamartomas and not true neoplasms. The term hamartoma is used to describe an abnormally large mass of histologically normal tissue in a normal location. The lymphatic malformation is composed of lymph-filled channels lined with a single layer of flat endothelial cells on a basement membrane. They present with either generalized edema and poorly defined borders (microcystic) or a localized area of multilocular cysts (macrocystic). The term lymphatic malformation has replaced many other outdated terms, such as lymphangioma, cystic hygroma, lymphangioma circumscriptum, and lymphangiomatosis. In this study, we present a case report of a pediatric lymphatic malformation of the sphenoid sinus. To our knowledge, this lesion has not been described in the pediatric population and has been described only once in an adult in the German literature. 1 EBM rating: C.