Hydrops fetalis is characterized by excess serous fluid in fetal or neonatal spaces and is primarily non-immune (90%) with various etiologies including hematologic, cardiovascular, infection, chromosomal, and more. The management of Rh incompatibility involves the administration of Rhogam to prevent hemolytic disease in sensitized pregnancies, and monitoring can include tests like the Kleihauer-Betke test and MCA Doppler for fetal anemia. Preventive measures, timely diagnosis, and appropriate interventions are crucial for managing potential complications and outcomes associated with hydrops fetalis.

1. Hydrops Fetalis
Dr Manal Behery
2014

2. Hydrops = Generalized subcutaneous
edema in the fetus or neonate

3. Definition
1. Excess serous fluid in at least
one space (ascites, pleural effusion, or
pericardial effusion) + skin edema (> 5 mm thick)
1. Excess fluid in two spaces without edema

4. Hydrops Fetalis
Non Immune Hydrops Fetalis (90%(
Immune Hydrops Fetalis (10%(

5. Etiology
1. Hematologic: Due to anemia (10% of cases) :
A. Isoimmune hemolytic disease (RH incompatibility).
2.Cardiovascular: Due to heart failure (20% cases)
A. Rhythm disturbances
B. Major cardiac disease

6. 3. Infection (10% of cases) TORCH-Syphilis-
Congenital Hepatitis, Parvovirus….
4. Chromosomal (5% of cases) : Turner
syndrome, trisomy 13,18,21

7. 5. Pulmonary (5% of cases) Chylothorax,
diaphragmatic hernia
6. Gastrointestinal (5% of cases) : Meconium
peritonitis
7. Renal (5% of cases) Nephrosis, RVT, urinary
obstruction
…..

8. 7. Maternal conditions (5% of cases) :
Toxemia, diabetes, thyrotoxicosis
8. Miscellaneous (10% of cases) : Cystic
hygroma, wilms’ tumor – teratoma
9. -Unknown (20% of cases) :

9. Rh (Rhesus) Isoimmunization:

10. Four blood types ( A, B, AB, and O)
Each blood type is additionally classified according to the presence or
absence of the Rh factor

11. CDE (Rhesus) System
• Clinically Important
• Includes c, C, D, e, E
• Rh negative status indicates the absence of D
antigen
• 87% of Caucasians carry the D antigen

12. •Rh Disease
•Alloimmunization
•Isoimmunization
•HDFN
•Erythroblastosis Fetalis
Confusing Terminology

13. When the mother produces Abs
directed against fetus RBC surface Ag.
Isoimmunization

14. Rh Incompatibility
Exposure to fetal antigens
causes the mother to
produce antibodies

15. The placenta usually acts as a barrier to fetal
blood entering the maternal circulation.

16. Fetal cells can enter the maternal circulation
through a “break” in the “placental barrier”.

17. Maternal production of Rhesus antibodies
following introduction of Rhesus positive blood

18. Maternal production of Rhesus antibodies
following introduction of Rhesus positive blood

19. Causes of RBC Transfer:
“A break in the barrier”
• Abortion/Ectopic
Pregnancy
• Partial molar pregnancy
• Blighted ovum
• Antepartum bleeding
• Procedures
(amniocentesis,
cordocentesis, CVS)
• External version
• Platelet transfusion
• Abdominal trauma
• Inadvertent transfusion
Rh+ blood
• Postpartum (Rh+baby)

20. Sensitized pregnancy Non- Sensitized pregnancy
Rh Incompatibility
Sensitization = Rh neg person exponsed to the Rh (D) antigen and makes
antibodies against that protein (antigen).

21. Rh Negative Women Man Rh positive
⇓
Fetus
Rh positive Fetus
→
Rh+ve R.B.C.s enter Maternal
circulation⇐
previously sensitized 2nd
immune response
IgM…IgG antibodies
⇓
Non sensitized Mother Primary
immune response
1st
Baby usually escapes. Mother
gets sensitized? ±
Fetus
Haemolysis
⇓
Pathogenesis Of Rh Iso - immunisation
Rh –VE Fetus
no harm

22. Presentation
Mild jaundice
Erythroblastosis Fetalis
Generalized Edema
Hepatomegaly
Ascites

23. Natural History
•50%of affected infants have mild
anemia, requiring either phototherapy
or no treatment.
•25%have hepatosplenomegaly ,
moderate anemia and progressive
jundice ending in kernicterus, neonatal
death
•25%are hydropic and die in utero or in
the neonatal period

24. RhoGAM has decreased HDFN caused by anti-D
Give 300 mcg dose within 72 hrs of delivery to
unsensitized Rh (-) women (Rh positive infant)
• ACOG: 300 mcg at 28 weeks UNLESS father known
to be Rh (-)

25. Mechanism of action
• Administered antibodies will
bind the fetal Rh- positive cells
• Spleen captured these cells by
Fc-receptors
• Suppressor T cell response is
stimulated
• Spleen remove anti-D coated
red cells prior to contact with
antigen presenting cells
“antigen deviation”

26. Kleihauer-Betke Test
• % fetal RBC in maternal circulation
• Fetal erythrocytes contain Hbg F which is more
resistant to acid elution than HbgA so after
exposure to acid, only fetal cells remain & can be
identified with stain
• 1/1000 deliveries result in fetal hemorrhage >
30ml
• Risk factors only identify 50%

28. 1.1-Direct Coomb’s Test (DAT(
Detects RBCs that have
already been sensitized with
IgG
Demonstrates that in vivo
coating of RBC by Ab has
occurred but does NOT
identify the antibody
Deepa Babin @TMC Kollam 28

29.  Detects antibodies to RBC antigens
present in the patient’s serum
 Detects in vitro red cell sensitization
if red cells contain antigen
corresponding to serum antibody
 Procedure:
 STEP 1:
patient’s serum (with unknown Ab) +
RBC (with known Ag)
 STEP 2: product of step 1 + Coomb’s
reagent
IAT((2. Indirect Coomb’s Test
Deepa Babin @TMC Kollam 29

30. Recognition of pregnancy at risk
•First ante-natal visit check blood group,
antibody screening.
• If indirect coombs test is positive, the
father’s Rh should be tested.
• Serial maternal Anti D titers should be
done every 2- 4 weeks.
• If titer is less than 1/16 the fetus is not
at risk.
• If titer is more than 1/16 then
severity of condition should be
evaluated.

31. Prevention
• Test for excessive fetal-maternal hemorrhage
after blunt trauma, abruption, cordocentesis,
and bleeding with previa
• Give RhoGAM for partial molar pregnancy,
ectopic, chorionic villus sampling,
amniocentesis, external version

32. MCA Doppler and Fetal Anemia
• Fetuses with anemia show an
• increased peak velocity of systolic
blood flow in MCA.
• MCA Doppler is also used to follow
fetal response to intrauterine transfusion
and to assist in timing subsequent transfusions.
• Non-invasive,
• no risk for worsening isoimmunization

33. Normal and Abnormal MCA Dopplers

34. Amniocentesis
- There is an excellent correlation
between the amount of bilirubin in
amniotic fluid and fetal hematocrit.
-
• - Perform serial amniocenteses
• to the optical density deviation at 450
nm measures the amniotic fluid
unconjugated bilirubin.
• Plot values on Liley Curve
at 16 weeks of gestation

35. Liley Curve
•Measures the level of bilirubin and predicts
severity of hemolytic disease after 27 weeks

36. Suggested management after amniocentesis for ΔOD 450
Serial Amniocentesis
Lily zone I
Lower Zone II
Upper Zone II Zone III
Hydramnios & Hydrops
Repeat
Amniocentesis every
2-4 weeks
Delivery at or near term
Repeat Amniocentesis in 7
days or FBS
Hct < 25%
Hct > 25%
Intrauterine
Transfusion
Repeat Sampling
7to 14 days
<35to 36 weeks
And Fetal lung
immaturity
>35to 36 weeks
Lung maturity
present
Intrauterine
Transfusion
Delivery

37. Cordocentesis
• Gold standard for detection of fetal anemia
• Complications
• 2.7% total risk of fetal loss
• Reserved for patients with
increased MCA-PSV or delta OD450

38. Intrauterine transfusion

39. Exchange Transfusion
•.
 Considered if the total serum bilirubin level is approaching 20
mg/dL and continues to rise despite intense in-hospital
phototherapy.
 Mortality rate 1%

40. Diagnosis and Management contd.

41. Review of Management for Rh
Isoimmunization
• Monthly indirect coombs titer (in first sensitized
pregnancy)
• If critical titer reached, determine paternal and fetal
antigen status
• Amniocentesis and delta OD450 OR MCA-PSV
** For 2nd
or greater sensitized pregnancy,
initiate amnio or MCA at 18-20 weeks**