Hydrops fetalis

Hydrops fetalis: Description, Causes and Risk Factors: Hydrops fetalisHydrops fetalis is an excess accumulation of fluid in the fetus. Depending on the severity and cause of hydrops, there may be edema of fetus and placenta, ascites, pleural effusions and/or pericardial effusions. In previous years, most cases of hydrops were caused by severe erythroblastosis fetalis secondary to Rh isoimmunization. With the marked decrease in this condition (due to prophylaxis with immune globulin), most cases of hydrops fetalis are now caused by other conditions and are known as non-immune hydrops. There are two types of hydrops fetalis, immune and non-immune. Immune hydrops fetalis is no longer common in the US. Non-immune hydrops fetalis can be caused by a wide variety of factors including: Hemolytic anemia: -thalassemia, RBC enzyme deficiencies.
  • Other anemias: Feto-maternal hemorrhage, twin-twin transfusion (donor).
  • Chromosomal abnormalities: Trisomy 21, 18, Turner syndrome.
  • Infections: Viral (Parvovirus B19, Herpes, CMV), toxoplasmosis, syphilis, Chagas disease.
  • Genetic metabolic disease: Gaucher disease, mucopolysaccharidosis, Nieman-Pick disease, neonatal hemochromatosis.
  • Cardiac: Fetal arrhythmias, premature closure of foramen ovale, hypoplastic left heart, hypoplastic right heart, Ebstein's anomaly of tricuspid valve, cardiomyopathy, cardiac tumors, premature closure of ductus arteriosus, other structural anomalies.
  • Vascular malformations: Chorioangioma (placenta, umbilical vessels), liver hemangioma, cerebral A-V malformation, sacrococcygeal teratoma, Klippel-Trenaunay syndrome.
  • Vascular accidents: Intracranial hemorrhage, thrombosis of renal veins, IVC, twin-twin transfusion (recipient).
  • Lymphatic malformations: Pulmonary lymphangiectasia, cystic hygroma, multiple pterygium Syndrome, Noonan syndrome.
  • Chest masses: Cystic adenomatoid malformation, diaphragmatic hernia, pulmonary sequestration, intrathoracic mass.
  • Skeletal conditions: Asphyxiating thoracic dystrophy, osteogenesis imperfecta, chondrodysplasia.
  • Fetal Hypomobility: Arthrogryposis, Neu-Laxova syndrome, Pena-Shokier syndrome. myotonic dystrophy.
  • CNS anomalies: Absent corpus callosum, encephalocele, holoprosencephaly.
  • Other: Bowel obstruction with perforation (meconium peritonitis, volvulus), infant of diabetic mother, Prune belly syndrome, congenital Nephrosis, maternal indomethacin therapy.
In approximately 1/4 of all cases, the cause is not determined. Approximately 50% of fetuses with non-immune hydrops fetalis die in utero, and about half of the liveborn infants survive. Symptoms: Symptoms depend on the severity of the condition. Mild forms may cause: Liver swelling, change in skin color (pallor).
  • More severe forms may cause: Breathing problems, bruising or purplish bruise-like spots on the skin, heart failure, severe anemia, severe jaundice, total body swelling.
Diagnosis: Diagnostic techniques include fetal ultrasonography, fetal echocardiography, examination of maternal blood for fetal erythrocytes (Kleihauer-Betke test), amniocentesis and sampling of fetal blood. Treatment: Treatment depends on the cause. During pregnancy, treatment may include: Medicine to cause early labor and delivery of the baby.
  • Early cesarean delivery if condition gets worse.
  • Intrauterine fetal blood transfusion.
Antenatal Management: In some cases, fetal intervention is effective (e.g., fetal transfusion for anemia due to Parvovirus B19 infection, treatment of fetal tachycardia). In others, delivery corrects the underlying problem (e.g., chorioangioma of placenta). Very few hydropic infants survive if delivered before 30 weeks of gestation. Postnatal treatment includes: Vigorous resuscitation with interventions (e.g., thoracentesis, paracentesis) to remove excess fluid.
  • Treatment of asphyxia, which is common in these infants.
  • Diagnosis of cause of hydrops, both for management of the patient and counseling of the parents regarding risk of recurrence in future pregnancies. This may require extensive diagnostic interventions, including careful post-mortem examination, skin sample for karyotyping and full body radiographs in those who do not survive.
  • Treatment of underlying cause.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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