Hydrops Fetalis
Dr Manal Behery 2014
Hydrops = Generalized subcutaneous edema in the fetus or neonate
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
Hydrops Fetalis
Non Immune Hydrops Fetalis (90%)
Immune Hydrops Fetalis (10%)
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
3. Infection (10% of cases) TORCH-Syphilis-Congenital
Hepatitis, Parvovirus….
4. Chromosomal (5% of cases) : Turner syndrome, trisomy
13,18,21
5. Pulmonary (5% of cases) Chylothorax, diaphragmatic
hernia
6. Gastrointestinal (5% of cases) : Meconium peritonitis
7. Renal (5% of cases) Nephrosis, RVT, urinary obstruction
…..
7. Maternal conditions (5% of cases) : Toxemia, diabetes,
thyrotoxicosis
8. Miscellaneous (10% of cases) : Cystic hygroma, wilms’
tumor – teratoma
9. -Unknown (20% of cases) :
Rh (Rhesus) Isoimmunization:
Four blood types ( A, B, AB, and O)
Each blood type is additionally classified according to the presence or absence of the Rh factor
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
•Rh Disease
•Alloimmunization
•Isoimmunization
•HDFN
•Erythroblastosis Fetalis
Confusing Terminology
When the mother produces Abs directed against fetus RBC surface Ag.
Isoimmunization
Rh Incompatibility
Exposure to fetal antigens causes the mother to produce
antibodies
The placenta usually acts as a barrier to fetal blood entering the maternal
circulation.
Fetal cells can enter the maternal circulation through a “break” in the
“placental barrier”.
Maternal production of Rhesus antibodies following introduction of Rhesus positive
blood
Maternal production of Rhesus antibodies following introduction of
Rhesus positive blood
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)
Sensitized pregnancy Non- Sensitized pregnancy
Rh Incompatibility
Sensitization = Rh neg person exponsed to the Rh (D) antigen and makes antibodies against that protein (antigen).
Rh Negative Women Man Rh positive
FetusRh 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
Presentation Mild jaundice
Erythroblastosis Fetalis Generalized Edema
Hepatomegaly Ascites
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
RhoGAM has decreased HDFN caused by anti-DGive 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 (-)
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”
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%
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
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
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.
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
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
Normal and Abnormal MCA Dopplers
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
Liley Curve•Measures the level of bilirubin and predicts
severity of hemolytic disease after 27 weeks
Suggested management after amniocentesis for ΔOD 450
Serial Amniocentesis
Lily zone ILower Zone II
Upper Zone II Zone IIIHydramnios & 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 Sampling7 to 14 days
>35 to 36 weeksAnd Fetal lung
immaturity
<35 to 36 weeks Lung maturity
present
Intrauterine Transfusion
Delivery
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
Intrauterine transfusion
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%
Diagnosis and Management contd.
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**
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