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Hydrops Fetalis

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Hydrops fetalis is a condition in the fetus characterized by an abnormal collection of fluid with at
least two of the following:

Edema
(fluid beneath the skin, more than 5 mm).
Ascites
(fluid in abdomen)
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).

Presentation

Hydrops fetalis is typically diagnosed during ultrasound evaluation for other complaints such as
:

Polyhydramnios
Size greater than dates
Fetal tachycardia
Decreased fetal movement
Abnormal serum screening
Antenatal hemorrhage

Causes

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 cause hemolytic
disease of the newborn.

Nonimmune hydrops (accounts for 80 -90% of cases)

Any other cause besides immune.

 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
(High output failure from anemia, sacrococcygeal teratoma, fetal adrenal neuroblastoma,
etc.)
Impaired venous return
(Metabolic disorders)
Obstruction to normal lymphatic flow
(Thoracic malformations)
Increased capillary permeability
Decreased colloidal osmotic pressure
(Congential nephrosis)

Normal return of of interstitial fluid to veins though lymphatic system

(high pressure to low pressure)

Lymph
Blood Interstitial Large
capillaries
capillaries Fluid Veins
and veins

Mechanisms that may cause increased interstitial fluid

Increased
capillary
permeability
Lymph
OR Large
capillaries
Veins
and veins
Decreased
colloidal
osmotic
pressure

Obstruction
Blood Large
to lymph
capillaries Veins
flow

Impaired
venous
return

Lymph OR
Blood
capillaries
capillaries
and veins Cardiac
failure

Some conditions may involve more than one mechanism . For example, parvovirus may cause
cardiomyopathy and anemia from marrow suppression.

Conditions Associated with NIH

(This list is not exhaustive)
 Cardiac
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, Pena-Shokeir, arthrogryposis
Fetal Anemia
Alpha () thalassemia, parvovirus, fetal hemorrhage, G-6-PD deficiency
Infection
Parvovirus, CMV, syphilis, coxsackie virus, rubella, toxoplasmosis,
herpes,varicella, adenovirus, enterovirus, influenza, listeria
Thoracic Abnormalities
Congenital cystic adenomatoid malformation (CCAM) , chylothorax,
diaphragmatic hernia, mediastinal tumor, skeletal dysplasias
Twinnning
Twin to twin transfusion Severe anemia in the donor twin or high-output
failure in the recipient
Tumors
Fetal sacrococcygeal teratoma, hemangiomas (Hepatic, Klippel-Trenaunay
syndrome), fetal adrenal neuroblastoma, placental tumors (chorioangioma)
Miscellaneous
Cystic hygromas, inheritable disorders of metabolism (lysosomal storage
diseases) ,maternal thyroid disease, congenital nephrotic syndrome.

Evaluation

Obtain maternal history (including pedigree)

Evaluate for immune hydrops
Obtain maternal indirect Coombs test to screen for antibodies associated with
blood group incompatibility.
Evaluate for nonimmune hydrops
Level II sonogram with Doppler measurement of the peak systolic velocity
(PSV) in 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 deficiency screen.
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
suspected.
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
recurrent nonimmune hydrops test for :
Storage disorders such as Gauchers, gangliosidosis, sialidosis, beta-
glucuronidase deficiency, and mucopolysaccharidosis
Enzyme analysis and carrier testing in parents and/or analysis of
fetal or neonatal blood or urine.
Histological examination of fetal tissues.
Maternal thyroid antibodies

Treatment
 Cause Treatment

Fetal blood sampling followed by in utero

Fetal anemia
transfusion

Medications such as digoxin, sotalol,

Fetal Arrhythmia
propanolol , flecainide, amiodarone

Thoracentesis or thoracoamniotic shunt for

Intrinsic thoracic malformations
pleural effusions in select cases

Fetoscopic laser ablation of communicating

Twin to twin transfusion
vessels

Syphilis Penicillin

Maternal complications

The mother may develop edema , hypertension, and proteinuria during conservative
management of hydrops a condition known as Mirror syndrome (also known as pseudotoxemia
or Ballantyne syndrome) . Symptoms may persist after delivery.

Counseling

Long term prognosis depends on underlying cause and severity of the heart failure.
If the cause of NIH cannot be determined, the perinatal mortality is approximately 50%
Prognosis is much poorer if diagnosed at less than 24 weeks , pleural effusion is
present, or structural abnormalities are present .
Pulmonary hypoplasia is a common cause of death in neonates with plerual
effusions.
Fetal hydrops associated with a structural heart defect is associated with an
almost 100% mortality rate.
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.

Antepartum

Follow up of the fetus will depend on the gestational age of the fetus, and the mother's
wishes regarding intervention.
If treatment has been successful or hydrops is resolving spontaneously, the fetus may be
followed with repeat sonograms every 1 to 2 weeks and antenatal testing.
Patients treated for immune hydrops are usually delivered at 37 weeks' or when
fetal lung maturity has been confirmed.
Consultation with the neonatologist may help to decide when it is appropriate to proceed
with preterm delivery for possible postnatal treatment .
The mother should be evaluated frequently for signs of "mirror" syndrome.

Delivery

The fetus should be delivered at tertiary care center with neonatologists and other
 The fetus should be delivered at tertiary care center with neonatologists and other
appropriate specialists.
There is no evidence that delivery by cesarean section has a marked effect on outcome.
Cord blood should be obtained at delivery

A postmortem evaluation should be performed in all cases of hydrops that result in neonatal
death. One study showed that a combined approach of a thorough antenatal assessment and
autopsy may be more likely to determine the cause of non-immune hydrops .

Online Resources:

Idiopathic Hydrops Fetalis

OMIM
Hydrops Fetalis
eMedicine
Non-immune Hydrops(Powerpoint)
OBGYN.net
Hydrops Fetalis
Lucile Packard Childrens Hospital

References

Wilkins, I. Nonimmune hydrops. In Creasy and Resnick's Maternal Fetal- Medicine Principles
and Practice sixth ed.Ed Creasy R et al. , 2009, Saunders. pp505-517

Bianchi DW, Crombleholme TM, D'Alton ME. Fetology: Diagnosis & Management of the Fetal
Patient.1st ed McGraw-Hill Professional 2000 pp 959-965

Rodriquez MM, Chaves F, Romaguera RL, Ferrer PL, delaGuardia C, Bruce JH. Value of
autopsy in nonimmune hydrops fetalis: series of 51 stillborn fetuses. Pediatr Devel Path
2002;5:365-374.

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