Carlo Bellini, Dario Paladini, Luca A. Ramenghi
Hydrops fetalis (HF) is a severe condition characterized by excessive fetal fluid accumulation in extravascular compartments, leading to edema, effusions, and organ enlargement. It can be classified into immune hydrops (IHF) and nonimmune hydrops (NIHF), with NIHF representing the majority and linked to high perinatal mortality. The etiology of NIHF is heterogeneous and often idiopathic, with recent studies highlighting the role of congenital lymphatic dysplasia. Pathophysiology involves fluid regulation imbalances, lymphatic dysfunction, and fetal circulatory changes. Diagnosis requires extensive evaluation, including genetic testing. Treatment strategies have improved fetal outcomes, emphasizing the need for specialist involvement in prenatal and postnatal management.