Most cases prevented by use of immunoglobulin (Ig) prophylaxis in at-risk mothers.
About
- A severe condition seen in the fetus characterized by abnormal accumulation of fluid
- If untreated, hydrops fetalis can lead to fetal heart failure, organ damage, and in many cases, stillbirth.
- Hydrops fetalis is characterized by fluid accumulation in more than two fetal body compartments.
- Common sites of fluid accumulation include ascites, pleural effusion, pericardial effusion, and skin oedema.
Aetiology
- Immune Causes (Erythroblastosis Fetalis): Primarily due to Rh blood group isoimmunization, where maternal antibodies attack fetal red blood cells.
- Non-Immune Causes: These include chromosomal anomalies, liver disease, heart or lung disorders, and infections (e.g., parvovirus B19, cytomegalovirus, syphilis).
Risk Factors
- Maternal Rh-negative status with an Rh-positive fetus, leading to isoimmune antibody formation.
- Previous administration of blood products, which can increase sensitization risks.
- Substance abuse, medications such as Indomethacin, and maternal collagen-vascular disease.
- Extremes of maternal age, including young mothers (<16 years) or older mothers (>35 years).
Clinical Presentation
- Severe fetal anaemia, often resulting in high-output cardiac failure.
- Fetal haemorrhage with hepatosplenomegaly (enlarged liver and spleen).
- Significant abdominal swelling due to ascites and pleural/pericardial effusions.
- Skin oedema as a result of fluid overload and cardiac insufficiency.
Investigations
- Ultrasound (USS): In the 1st or 2nd trimester, reveals fluid collections, polyhydramnios, and placental oedema, along with enlargement of the liver, spleen, and heart.
- Fetal Blood Sampling: Performed by inserting a needle through the uterus into a fetal blood vessel or umbilical cord to assess fetal haemoglobin and other parameters.
- Amniocentesis: Involves withdrawing a sample of amniotic fluid to test for chromosomal abnormalities or infections.
Management
- Prevention: Administration of immunoglobulin (Ig) prophylaxis in Rh-negative mothers significantly reduces immune-related hydrops fetalis cases.
- Delivery: Often by Caesarean section, with resuscitation, intubation, and ventilation support prepared if needed.
- Neonatal Care:
- Ventilation and oxygen support to manage respiratory distress.
- Transfusions to address severe anaemia and improve fetal oxygenation.
- Management of hypoglycaemia and drainage of any ascites or pleural effusions as needed.
- Administration of Vitamin K to prevent bleeding and Furosemide to manage fluid overload.