Hydrops Fetalis

Danna Premer, MD
Former Fellow, Division of Neonatology

(some content from outline by Dr. CM Bendel)

• Peripheral edema may be significant
• Ascites, pleural and pericardial effusions frequently noted
• Uneven distribution of excess fluid common
• features noted in utero: polyhydramnios, thickened placenta (>6cm)

HYDROPS FETALIS: Differential Diagnosis

• Immune Hydrops
  • Alloimmune hemolytic disease / Rh isoimmunization
  • Etiology for the majority of cases prior to 1960s
• Nonimmune Hydrops
  • Currently the majority of cases fall into this category, ~ 75%
  • Incidence estimated to be 1 per 3000 deliveries
• Common Pathophysiology
  • Conditions leading to abnormal transport of water between the capillary plasma and extravascular tissues
    • increased capillary hydrostatic pressure
    • decreased plasma colloid osmotic pressure
    • increased capillary permeability
    • decreased lymph flow

NONIMMUNE HYDROPS FETALIS: Differential Diagnosis

• Hematologic
  • Fetal hemolytic anemia
  • a-Thalassemia
  • Fetomaternal or twin-to-twin transfusion
• Congenital Infections
  • Viruses
  • Cytomegalovirus
  • Parvovirus B19
  • Toxoplasmosis
  • Syphilis
  • Chagas Disease
• Cardiovascular
  • Arrhythmias
  • Cardiomyopathy
  • Structural anomalies: lesions that result in increased right atrial pressure and volume
    • primarily with atrioventricular regurgitation
    • left sided obstructive lesions
    • Ebstein's
    • Premature closure of the foramen ovale
  • Intracardiac tumors (tuberous sclerosis)
  • Vascular malformations
    • Chorangioma of the placenta, chorionic or umbilical vessels
    • Hemangiomas (Hepatic, Klippel-Trenaunaysyndrome)
• Lymphatic Abnormalities
  • Lymphangiectasia
  • Cystic hygroma
  • Turner's syndrome (XO)
  • Noonan's syndrome
  • Multiple ptergium syndrome
• Pulmonary Malformations
  • Lymphangiectasia
  • Chylothorax
  • Cystic adenomatoid malformation
  • Hypoplasia
• Other
  • Obstructive uropathy
  • Congenital nephrosis
  • Chromosomal abnormalities
    • Trisomy 15, 18, 21
    • XX/XY
  • Neoplasms
  • Storage diseases
  • Bone diseases
  • Placental abnormalities
  • Neurologic abnormalities
• Idiopathic

NONIMMUNE HYDROPS FETALIS: Diagnosis

• History/Prenatal Course
  • Noncontributory here, but often significant
  • Prenatal diagnosis helpful
    • Evaluation should include:
      • exclusion of isoimmunization
      • complete ultrasound examination with fetal echocardiography
      • chromosomal evaluation
      • maternal serology
    • Evaluation may include:
      • amniotic fluid analysis with cultures/PCR
      • fetal thoracentesis/paracentesis
      • PUBS for determination of blood counts
      • intrauterine transfusion
• Physical Examination
  • Generalized edema
  • Ascites with hepatoslenomegaly
  • Respiratory distress
  • Cardiac murmur
  • Evidence for congestive heart failure
  • No arrhythmia
• Diagnostic Imaging
  • X-rays of the chest, abdomen, long bones and cranium
  • Complete ultrasound evaluation
    • cranial, thoracic and abdominal
  • Echocardiography
• Laboratory Evaluation
  • Blood type and Coombs' test (direct and indirect)
  • Blood cell count and platelet count with peripheral smear
  • Kleihauer-Betke (maternal blood)
  • Chromosomal analysis
  • Electrolytes, renal and liver function tests
  • Hemoglobin electrophoresis
  • Bacterial and viral cultures / PCR
  • Serology for congenital infections (maternal and infant)
  • Diagnostic thoracentesis and/or paracentesis
  • Placental anatomy and histology
  • Electrocardiogram

NONIMMUNE HYDROPS FETALIS: Therapy

• Co-ordinated delivery room resuscitation
• Vigorous cardiorespiratory stabilization
  • PPHN common, therefore aggressive ventilatory management often necessary including HFOV and NO
  • thoracentesis, paracentesis and fluid replacement as needed
  • inotropic support as indicated
  • cautious diuretic administration
  • factors to consider in resuscitation:
    • airway edema
    • chest wall edema
    • pulmonary edema/pleural effusions/pulmonary hypoplasia/RDS
    • perinatal depressions/hypoxia/acidosis
• Supportive care (especially if born prematurely)
• Specific therapy based on underlying etiology

NONIMMUNE HYDROPS FETALIS: Prognosis

• 50% of all cases diagnoses in utero result in fetal death
• 50% of all live born infants with nonimmune hydrops die
• Exact prognosis depends on the underlying condition
  • idiopathic - 20-25% survive the newborn period
  • anemia that can be treated in utero or in the newborn period -- very good >90%
  • structural cardiac disease with or without an arrhythmia -- very poor
  • isolated arrhythmia -- >50% survival of live born infants
  • Poor Prognosis associated with:
    • prematurity
    • pleural effusions
    • pulmonary hypoplasia
    • severe hydrops
    • chromosomal disorder
    • identification prior to 24w GA
    • structural malformation

References

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