Differential diagnosis and causes of nonimmunological hydrops fetalis: a review

Twenty-five babies with nonimmune hydrops fetalis were born during a 20-year period from 1967 to 1987 (1/1475). Sixteen were live-born, only three survived. Although hydrops can be caused by a myriad of diseases, a simple classification is proposed in order to allow efficient diagnostic workup. For the majority of cases of nonimmune hydrops no cause can be found. Vascular causes are most frequent, followed by chromosomal, placental, hematological, infectious, and a miscellaneous group of causes. Of the cases reviewed fetal causes dominate in 95 per cent of cases. Maternal causes are relatively rare and consist mainly of infections and diabetes. In order to reduce the high mortality rate of 98 per cent one should aim at early diagnosis of hydrops fetalis, extensive investigation of both the fetus and the mother, and referral to a center for intensive perinatal care.