2. OBJECTIVES:
To define fetal hydrops
Identify those affected by hydrops fetalis
What are the maternal symptoms of hydrops
fetalis ?
classification?
How is hydrops fetalis diagnosed ?
Treatment for hydrops fetalis
3. DEFINITION:
Hydrops fetalis is a condition in the fetus
characterized by an abnormal accumulation of fluid in
two or more fetal compartments resulting in
generalized edema of the fetus, with fluid build up in
extravascular components and body cavities-Edema
, Ascites , Pleural or Pericardial effusion.
4. Hydrops fetalis is typically diagnosed during
ultrasound evaluation for other complaints such as :
is frequently associated with polyhydramnios
and a thickened placenta (>6 cm).
Size greater than dates
Fetal tachycardia
Decreased fetal movement
Abnormal serum screening
Antenatal hemorrhage
5. Epidemiology
•
1 per 2,000 births
Internationally 1 in 500 in Asia,
Mortality-:60-90%
Race: Asian, African descent-hemolytic diseases
Sex: male
6. CLASSIFICATION:
IMMUNE
HYDROPS
Isoimmunization of fetus
NON-IMMUNE
HYDROPS
cardiovascular
Genetic abnormaliy
Intra-thoracic
malformation
Hematological
disorders
Infectious conditions
Idiopathic forms
8. Pathophysiology
The basic mechanism for the formation of fetal hydrops is an
imbalance of interstitial fluid production and the lymphatic
return. Fluid accumulation in the fetus can result from
congestive heart failure
obstructed lymphatic flow
decreased plasma osmotic pressure.
The fetus is particularly susceptible to interstitial fluid
accumulation because of its greater capillary permeability,
compliant interstitial compartments, and vulnerability to
venous pressure on lymphatic return
9.
10. Immune hydrops
(accounts for 10-20%of cases)
This phenomenon was reported by Levine in 1941
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
1. High-output congestive heart failure.
2. Increased red blood cell production by the spleen and
liver leads to hepatic circulatory obstruction (portal
hypertension)
11. 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.
12. Development of Rh- D antibodies
When Rh +Ve RBCs enters the Rh-Ve mother’s blood circulation; they
stimulate the maternal immune response producing RBC antigen –
specific antibodies –isoimmunization (sensitization ) .
After few weeks , IgM antibodies ( Saline antibodies ) are produced. IgM
do not cross the placenta barrier Hence can not cause fetal damage .
On subsequent exposure of fetal Rh D antigen , her previously
primed B cells act swiftly to produce IgG ( albumin antibodies ) which
can cross placenta ---and bounds to antigen of fetal RBCs causing
sequestration and destruction of fetal RBCs.
Antibodies are formed in 16% of the mothers significantly after
6months of larger amount of fetomaternal bleeding and once formed
remain in circulation through the life. And each subsequent pregnancy
will have booster effect especially when fetus is Rh +Ve fetus is in
utero.
13.
14. 3 D image of fetus with features of
immune hydrops
16. Feto- maternal haemorrhage During Antenatal Period
O.1ml of fetal RBCs are found in 5-15 % of women’s
circulation by 8 weeks of gestation.
75% cases it is always < 1ml .
1% show atleast 5ml fetal RBCs.
0.25% have more than 30ml of fetal RBCs hence only
1.5 % women get sensitised in Antenatal period .
It can be prevented by anti D therapy in antenatal
period ---at 28 and 36 weeks of gestation and
repating it after delivery.
If any factor precipitating feto-maternal hemorrhage
need immediate anti d therapy in appropriate dose.
17. Non-immune 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:
1. Cardiac failure (High output failure from anemia, sacrococcygeal
teratoma, fetal adrenal neuroblastoma, etc.)
2. Impaired venous return (Metabolic disorders)
3. Obstruction to normal lymphatic flow (Thoracic malformations)
4. Increased capillary permeability
5. Decreased colloidal osmotic pressure
(Congential nephrosis)
24. 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.
25. 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 Gaucher’s, 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
26.
27.
28. 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.
29. Delivery
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 .
30. Prophylaxis
Universal ABO Rh grouping of all girls / expactant mother.
Premarrital councellig regarding The Rh factor in both life
partners --- advising marriage of identical Rh factor.
Rh Immunoglobulin (Rh-IgG) therapy within 24-48 hrs to
all Rh –Ve women after ectopic/MTP/ abortion/ delivery
/amnioscentasis ,Version , APH bleeding and at 28th and
36th week of gestation in primigravida too..
This therapy was introduced in1966 and its world wide
acceptance has remarkably reduced the incidence of
immune hydrops in developed countries , but it is still
common type of hydrops in developing world.
31. Prognosis
Perinatal death rate is 40-98%.
Worst prognosis is seen in anatomic malformations found in 40% of
cases.
Only cases with CMV and arrhythmias without structural
malformations have spontaneous resolution.
If hydrops was evident before 24 weeks then fetal mortality rate was
95%. Euploid fetuses with hydrops who survived upto 24weeks and
have a structurally normal heart had survival rate of 20%.(Mc Coy
and colleagues, 1995).
Risk of recurrence is low unless etiology is genetic.
Hydrops usually persists or worsens with time, it occasionally
resolves spontaneously.
OVERALL OUTCOME FOR HYDROPS FETALIS OF ANY CAUSE
IS GENERALLY POOR.
Most patients with hydrops diagnosed in early fetal life have chromosomal anomalies, whereas cases diagnosed after the second trimester are caused mainly by cardiovascular diseases.
Causes can be grouped in 6 broad categories: cardiovascular, genetic abnormalities, intrathoracic malformations, hematological disorders, infectious conditions, and idiopathic forms.
