primary lymphedema EPHB4-related lymphatic-related hydrops fetalis https://www.malacards.org/card/ephb4_related_lymphatic_related_hydrops_fetalis MONDO:0035475 EPHB4-related generalised lymphatic dysplasia with atrial septal defect EPHB4-related generalized lymphatic dysplasia with non-immune hydrops fetalis A rare primary lymphedema characterized by a highly variable lymphatic phenotype ranging from severe lymphatic-related hydrops fetalis, which may cause perinatal demise or fully resolve to become completely asymptomatic, to a mild presentation in older patients with persistent varicose veins, peripheral edema, and impaired lymph drainage in the lower limbs. Atrial septal defect has been described in association and may be the only anomaly in some patients. EPHB4-related LRHF/GLD UMLS:C5680140 Orphanet:568065 MEDGEN:1802765 GARD:0022302 EPHB4-related generalised lymphatic dysplasia with non-immune hydrops fetalis EPHB4-related generalized lymphatic dysplasia with atrial septal defect