Fetal cystic hygroma, web neck and trisomy 13 syndrome

The term fetal cystic hygroma is applied to a condition of marked lymphoedema and lymphatic dilatation occurring particularly in the cervical region in fetal life. It has been suggested that the condition results from delayed communication of the jugular lymphatic sacs with the jugular veins which normally occurs at about 40 days of development. It is also believed that fetal cystic hygroma, the type of cystic lymphangioma commonly called cystic hygroma, and pterygium colli, are all manifestations of the “jugular lymphatic obstruction sequence” (Burck et al, 1981). Delayed lymphatic-venous communication produces lymphatic dilatation and lymphoedema, leaving redundant skin when the lymphatic-venous connection is established.