A Rare Massive Congenital Cervical Lymphangioma: Case Report with Systematic Review of the Literature

Abstract

Congenital anomalies of the lymphatic system resulting from sequestration of primitive lymphatic sacs from the normal lymphatic circulation and consisting of benign cystic masses of enlarged lymphatic vessels are referred to as lymphangiomas. They are believed to manifest from the 6th week of pregnancy when the forming lymphatic sacs unsuccessfully fail to connect to the rest of the lymphatic system or to the internal jugular vein. 1 Thus, lymphatic malformations are swellings that are made up of multiple lymphatic cysts, 1-4 which comprise many lymphatic vessels that do not connect to normal lymphatic circulation. 3,4 Their presentation ranges from small masses affecting localized areas of the skin and subcutaneous tissues to extensive swellings that can affect a whole limb or region. The sizes of the cysts vary from very small or very large-up to several centimeters across, depending on the amount of fluid they contain. The term cystic hygroma is applied to lymphangiomas developing within loose areolar connective tissue and denotes the mass effect and the cystic nature of these lesions. The term "hygroma" can be directly translated into "moist tumor" 3,4 or "water-containing tumor." 1,4 Lymphangiomas are commonly found in the neck but they can occur almost anywhere in the body. 1,3,4 Moreover, lymphatic malformations often appear to affect multiple separate parts of the body, but when investigated with MRI, it is revealed that the different swellings are linked 1,