PRESENTATION

By: Dr. Saba Kalhoro

HYDROPS
FETALIS
Table of contents
Introduction
Why Hydrops Fetalis is a concern?
Types
Epidemiology
Causes of immune hydrops fetalis
Causes of non-immune hydrops fetalis
Pathogenesis
Symptoms
Complications
Management
Diagnosis
Treatment


Introduction:
Hydrops fetalis refers to the presence of two or
more of the following abnormal fetal fluid
collections:
Ascites
Pleural effusion
Pericardial effusion
Skin edema(more than 5mm)


Why Hydrops Fetalis is a concern?

It has a very poor outcome (>80% mortality),
regardless of the aetiology.








Dewhursts Textbook Of Obstetrics & Gynaecology Eighth Edition
Types:
In 1943, Potter defined two forms of
hydrops fetalis based upon etiology:

Immune-mediated (10 percent )

Non-Immune ( 90 percent )



Potter EL. Universal edema of the fetus unassociated with erythroblastosis.
Am J Obstet Gynecol 1943; 46:130.


Epidemiology:


The reported incidence of NHF ranges from 1
in 1500 to 3800 births. It accounts for more
than 90% of all cases of hydrops.


Epidemiology...contd
Review from a large national data set from the
United States:

598 live-born infants with hydrops fetalis

The most common causes of hydrops fetalis
were:

Congenital heart problems (14 percent)
Heart rate abnormalities (10 percent)
Twin to twin transfusions (9 percent)


Epidemiology...contd
Chromosomal abnormalities (8 percent)
Congenital viral infections (7 percent)
Congenital anemia (5 percent)
Congenital chylothorax (3 percent)



Abrams ME, Meredith KS, Kinnard P, Clark RH. Hydrops fetalis: a retrospective review of cases
reported to a large national database and identification of risk factors associated with death.
Pediatrics 2007; 120:84.




In Asia, the most common causes of hydrops
fetalis are homozygous alpha thalassemia and
cardiac disease.


Evidence Based Text Book of Obstetrics & Gynaecology - 2
nd
Edition
Causes of immune hydrops
fetalis:
Anti-D antibodies
Anti-C antibodies
Anti-Kell antibodies

RhD iso-immunisation


IgG antibodies to RhD Antigen will persist in the circulation
and the numbers of antibodies can be multiplied hugely if
there is repeated contact with these foreign RhD red cells

IgG Antibodies are small molecules and cross freely into the
placenta

If the fetal cells are RhD positive they are targeted by the
IgG molecules and are destroyed by the fetal Reticullo-
endothelial system (hemolysis) which may leads to anemia
Causes of non-immune hydrops
fetalis:
Cardiac
Hypoplastic left/right heart
Fetal arrhythmias
Premature closure of foramen ovale
Cardiomyopathy
Sacrococcygeal teratoma
Causes:
Chromosomal abnormalities
Trisomy 13, 18, 21
Turner syndrome

Causes:
Thoracic anomalies
Diaphragmatic hernia
Pulmonary sequestration
Intrathoracic mass
Bronchogenic cyst

Causes:
Infections
Viral (Parvovirus B19, Herpes, CMV)
Toxoplasmosis
Syphilis

Causes:
Hematologic
Alpha Thalassemia
Red-cell enzyme deficiencies

Other causes:
Skeletal conditions
Osteogenesis imperfecta
Chondrodysplasia

Genetic metabolic disease
Gaucher Disease
Mucopolysaccharidosis
PATHOGENESIS:
Compensatory response activated due to fetal
anemia in extramedullary hematopoiesis.

Spleen and liver enlarge in size

Immature red cell called erythroblast enter into
circulation help in O2 carrying to cells.

If this response failed then there is compensatory
placental hyperplasia
PATHOGENESIS:contu
In extreme case compensatory response
exceed and baby goes into high cardiac out
put and result in cardiac failure


Result in accumulation of fluid in body cavity
including scalp edema, ascites, pleural and
pericardial effusion ( hyfrops fetalis )
Features of hydrops fetalis :
Depend on the severity of the condition.
Mild forms may cause:
Liver swelling
Change in skin color (pallor)
More severe forms may cause:
Breathing problems
Bruising on the skin
Heart failure
Severe anemia
Severe jaundice
Total body swelling

Maternal Complications:
Polyhydraminos
Placental abruption
Uterine atony
Premature-labour
Hydropic placenta ( more than 6cm)
Retained placenta
Pre-eclampsia
Mirror Syndrome:

Mother develops pre-eclampsia along with
severe oedema that is similar to that of the
fetus

Caused by vascular changes in the swollen
hydropic placenta
Fetal complications:
Babies born with hydrops are very swollen and
have a large, round abdomen due to the fluid
collection in the abdominal cavity.
Severe respiratory distress
Hypoglycemia
Apnea
Anemia
Neurological injury and fetal death



MANAGEMENT OF NON
IMMUNE HYDROPIC FETALIS
Diagnosis & Investigations for non
immune
Ultrasound - Several etiologies are confirmed or
excluded based upon ultrasound findings
twin-to-twin transfusion
cardiac arrhythmias
structural anomalies
Ultrasound doppler
Fetal echo
Amniocentesis
Maternal thyroid antibodies

Diagnosis & Investigations for non
immune
Viral serology.
Assessment of maternal blood type
Treatment of non immune :
Fetal anemia


Fetal arrythmia

Intrinsic thoracic
malformations

Fetal blood
sampling followed
by in utero
transfusion.
Medications such as
digoxin, propanolol.
Thoracentesis or
thoracoamniotic
shunt for pleural
effusions.


Causes Treatment
Treatment of non immune
Twin to twin
transfusion



syphilis
Fetoscopic laser
ablation of
communicating
vessels

penicillin
Management:
Antepartum
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.
Consultation with the neonatologist

Signs of "mirror" syndrome.



Management:
Delivery
In tertiary care center with neonatologists and
other appropriate specialists.

Delivery by caesarean section has no marked
effect on outcome.

Cord blood should be obtained at delivery

Counselling and fetal outcome:
Long term prognosis and severity of
the disease.

Prognosis is much poorer if diagnosed at less
than 24 weeks , pleural effusion is present, or
structural abnormalities are present, option of
termination of pregnancy may be a
consideration.
Counselling and fetal
outcomecontd


In survivors of hydrops fetalis, poor
neurodevelopment outcome appears to be the
most significant morbidity.




Nakayama H, Kukita J, Hikino S, et al. Long-term outcome of 51 liveborn neonates with non-immune
hydrops fetalis. Acta Paediatr 1999; 88:24.

Immune Hydrops Fetalis
Two major problem :
1) Fetal anemia

2) Hyperbilirubinemai
Investigation of rhD
immunized
Anti D Anitibodies level :shows risk of
fetal hemolysis
1) Indirect coombs test : >1:16 titer
2)Immunoassay measure : maternal serum
level shows ; <4iu/ml ------ mild
>10iu/ml------ moderate
>30iu/ml------ sever
Investigations for RhD
immunized
Fetal blood group
1) Paternal genotype : RhD +is 100% in
homozygous

2)Invasive procedure :
-fetal blood sampling :umbilical cord at placental
insertion site is most common site for FBS

-Free fetal DNA :The maternal blood can now be
tested to determine the fetal Rh status

NICE GUIDE LINE 2008
Invasive and Non-invasive
Methods
of assessing for fetal anaemia
Doppler ultrasound : Middle Cerebral Artery

Amniocentesis for bilirubin level (indirect
measurement of fetal haemolysis)

Cordocentesis ( direct Hb measurement and
transfusion if needed)
How does blood flow in the Middle
Cerebral Artery alter in an anaemic
fetus
Fetuses suffering from hypoxia will preferentially divert
blood to the brain
Anaemia will result in a low blood viscosity and a higher
cardiac output
All blood vessels in the fetus will shows high blood
velocities but the Middle Cerebral Artery shows this to
exaggerated effect(peak systolic blood flow )
MANAGEMENT OF IMMUNE
HYDROPIC FETALIS
Dose and Timing of Anti-D
Immunoglobulin
Routine Antenatal Anti-D Prophylaxis
Anti-D should be administered at 28 and 34 weeks of
pregnancy ( 500iU im)
OR
Single dose regime
1500 iU im at 28-30 weeks
A KLEIHAUER BETKE test. Fetomaternal
transfusion (calculate 100iu of antiD antibodies can
nuterlize 1ml of fetal blood ) should be performed
after delivery
After delivery within 72 hr anti D 500IU im

NICE GUIDE LINE JUNE 2008
Management of immune
MODE OF DELIVERY :

In moderate to severely affected babies < 37 week
gestational age caesarean section .. more prone to
develop hypoxia during labour and due to large
size of bay and placenta .


If mild anemia labour may be induced after 37
week by vaginal delivery
Precaution after delivery of
baby
Once baby delivered transfusion of fetal blood
from placenta into maternal circulation should
be reduced by
early cord clamping
Avoiding milking of umbilical cord toward fetus
Maintaining the fetal level at , or above the
maternal level
Avoid manual removal of placenta
Recurrence:
The risk of recurrence depends upon the
underlying etiology. The recurrence rate is
greatest in families with infants who have a
chromosomal abnormality.
Etiology and outcome
of Hydrops fetalis a ten year
experience
Objective
To determine etiology and outcome of hydrops fetalis in a large
Irish tertiary referral centre.
Study Design
All antenatally diagnosed cases of hydrops fetalis from January
2000 to January 2010 were studied prospectively
Conclusions
Cardiac cause (most common)
Majority die (Antenatally/Neonatally)
66% with diagnosed Etiology
53% survived who undergone intervention

American Journal of Obstetrics and Gynecology - Volume 204, Issue 1 Suppl 1 (January 2011)
References
Evidence Based Text Book of Obstetrics & Gynaecology
Dewhursts Text Book of Obstetrics & Gynaecology
Ballantyne JW. The diseases and deformities of the foetus: An attempt
towards a new system of ante-natal pathology. Edinburgh, Oliver & Boyd,
1892.
Potter EL. Universal edema of the fetus unassociated with erythroblastosis.
Am J Obstet Gynecol 1943; 46:130.
Non-immunological hydrops fetalis. J Obstet Gynaecol Br Commonw 1970;
77:226.
Clark RH. Hydrops fetalis: a retrospective review of cases reported to a
large national database and identification of risk factors associated with
death. Pediatrics 2007; 120:84.
American Journal of Obstetrics and Gynecology - Volume 204, Issue 1
Suppl 1 (January, 2011)


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