fetalis is a condition in the fetus
characterized by an abnormal collection of fluid with at least two of the
(fluid beneath the skin, more than 5 mm).
- Pleural effusion
(fluid in the pleural cavity, the
fluid-filled space that surrounds the lungs)
- Pericardial effusion
(fluid in the pericardial sac,
covering that surrounds the heart)
In addition, hydrops fetalis is frequently associated with polyhydramnios
and a thickened placenta (>6 cm).
Hydrops fetalis is typically diagnosed during ultrasound evaluation for other
complaints such as :
Size greater than dates
Decreased fetal movement
Abnormal serum screening
Hydrops fetalis is found in about 1 per 2,000 births, and is categorized as
immune or nonimmune hydrops.
Immune hydrops (accounts for 10-20%of cases)
- Maternal antibodies against red-cells of the fetus cross the placenta and coat fetal red cells which are
then destroyed (hemolysis) in the fetal spleen.
- The severe anemia leads to
- High-output congestive heart failure.
- Increased red blood cell production by the spleen and liver leads to hepatic circulatory obstruction (portal hypertension)
- Anti-D, anti-E, and antibodies directed
against other Rh antigens comprise the majority of antibodies responsible
for hemolytic disease of the newborn .
- However, there are numerous, less commonly encountered, antibodies such as
anti-K (Kell), anti-Fya (Duffy) , and anti-Jka (Kidd) that may also
causes of hemolytic disease of the newborn.
Nonimmune hydrops (accounts for 80 -90% of cases)
- Any other cause besides immune.
- In general nonimmune hydrops (NIH) is caused by a failure of the
interstitial fluid (the liquid between the cells of the body) to return into the
venous system .
This may due to:
- Cardiac failure
- Impaired venous return
- Obstruction to normal lymphatic flow
- Increased capillary permeability
- Decreased colloidal osmotic pressure
Conditions Associated with NIH
(This list is not exhaustive)
- Cardiomyopathy, Ebstein's anomaly, pulmonary atresia,
coarctation of the aorta, hypoplastic left heart, complete AV canal, left sided obstructive lesions, premature closure of the foramen ovale
- Intracardiac tumors (tuberous sclerosis)
- Cardiac arrhythmia
- SVT, flutter, heart block, WPW syndrome
- Chromosomal /Genetic Syndromes
- T13, T18, T21, XO (Turners syndrome) ,Noonan syndrome ,
multiple pterygium syndrome
- Fetal Anemia
- Alpha (α) thalassemia, parvovirus, fetal hemorrhage, G-6-PD deficiency
- Parvovirus, CMV, syphilis, coxsackie virus, rubella, toxoplasmosis, herpes,varicella, adenovirus, listeria
- Thoracic Abnormalities
- Congenital cystic adenomatoid malformation (CCAM) , chylothorax, diaphragmatic hernia, skeletal dysplasias
- Twin to twin transfusion Severe anemia in the donor twin or high-output failure in the recipient
- Fetal sacrococcygeal teratoma, hemangiomas (Hepatic, Klippel-Trenaunay syndrome), placental tumors (chorioangioma) , cystic hygromas,
inheritable disorders of metabolism (lysosomal
storage diseases) ,maternal thyroid disease
- Obtain maternal history (including pedigree)
- Evaluate for immune hydrops
- Obtain maternal indirect Coombs test to screen for antibodies associated with blood
- Evaluate for nonimmune hydrops
- Level II sonogram with Doppler measurement of the peak systolic velocity
the fetal middle cerebral artery (MCA) to assess for fetal anemia. If
there is evidence for anemia or equivocal result obtain:
- Maternal blood counts and hemoglobin electrophoresis (with hemoglobin DNA
analysis), Kleihauer-Betke stain, glucose 6-phosphate dehydrohgenase
- Maternal TORCH titers, RPR, listeria, parvovirus B19, coxsackie, adenovirus,
and varicella IgG and IgM, as indicated.
- Fetal echocardiogram
- Consider fetal heart rate monitoring for 12 to 24 hours if fetal arrhythmia is
- Amniocentesis for fetal karyotype and PCR (polymerase chain reaction) for
infections OR fetal percutaneous blood sampling for same and in
addition fetal liver function; and
metabolic testing if indicated.
- In the presence of a family history of an inheritable metabolic disorder or
nonimmune hydrops test for :
- Storage disorders such as
Gaucherís, gangliosidosis, sialidosis, beta-glucuronidase deficiency, and
- Maternal thyroid antibodies
||Fetal blood sampling followed by
in utero transfusion
||Medications such as digoxin,
sotalol, propanolol , flecainide, amiodarone
|Intrinsic thoracic malformations
||Thoracentesis or thoracoamniotic
shunt for pleural effusions in select cases
|Twin to twin transfusion
||Fetoscopic laser ablation of
- Long term prognosis depends on underlying cause and severity of the heart
- If the cause of NIH cannot be determined, the perinatal mortality is
- Prognosis is much poorer if diagnosed at less than 24 weeks , pleural effusion
is present, or structural abnormalities are present .
- If early in pregnancy (less than 24 weeks) with no treatable cause
the option of termination may be a consideration.
- Recurrence is uncommon unless related to blood group incompatibility (isoimmunization)
or inheritable disorder.
(also known as pseudotoxemia or Ballantyne syndrome)
may develop during conservative management of hydrops
And may also have HTN and proteinuria
Repeat sonogram periodically to further evaluate fetal anatomy and progression
Antenatal testing as indicated after fetal viability.
Unless there is evidence dleiver at 34 weeks untless hydrops is resolvoing or
tretament has been efective
Manage until 37 weeks or until fetal lung maturity has been confirmed.
Plan for delivery at tertiary care center with neonatologists and other
Most of the time fetuses are delivered with elective cesarean section.
However, there is no evidence that mode of delivery has a marked effect on
Pediatric team should be aware about the nature of fetal problem before
delivery as resuscitation is often difficult and adequate senior assistance
must be available.
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:
chest wall edema
pulmonary edema/pleural effusions/pulmonary hypoplasia/RDS
Supportive care (especially if born prematurely)
Specific therapy based on underlying etiology
Postmortem examination in all cases of NIFH that result in neonatal death.
An accurate diagnosis may not always be possible, and this limitation must be
clearly explained to parents of affected fetuses who opt for another
A combined approach of a thorough antenatal assessment and autopsy when
indicated was able to determine the cause of non-immune hydrops in over 90% of
cases in one study.
Cord blood at delivery - irrespective of outcome .
1 . Blood Group Serology and tying
2 . Chromosome analysis.
3 . Total protein and albumin
4 . TORCH titre
5 . Hb and Hb - EPP . ( H.P.L.C. )
6 . Vacuolated lymphocytes
7 . Enzymology ( red cell and lysosomal enzyme ).
Wilkins, Isabelle. Nonimmune hydrops. In Creasy and Resnick's
Maternal Fetal- Medicine Principles and Practice sixth ed.Ed Creasy R et al. ,