HYDROPS FETALISPEADIATRICS ROTATION

GEORGTOWN PUBLIC HOSPITAL

08/2014

DR. SANDY S. SOLOMON

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

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.

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

Epidemiology

1 per 2,000 births

Internationally 1 in 500 in Asia,

Mortality-:60-90%

Race: Asian, African descent-hemolytic diseases

Sex: male

•

CLASSIFICATION:

IMMUNE HYDROPS

Isoimmunization of fetus

NON-IMMUNE HYDROPS

cardiovascular

Genetic abnormaliy

Intra-thoracic malformation

Hematological disorders

Infectious conditions

Idiopathic forms

NIHF

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

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)

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.

Development of Rh- D antibodies

When Rh +Ve RBCs enters the Rh-Ve mother’s blood circulation; theystimulate the maternal immune response producing RBC antigen –specific antibodies –isoimmunization (sensitization ) .

After few weeks , IgM antibodies ( Saline antibodies ) are produced. IgMdo not cross the placenta barrier Hence can not cause fetal damage .

On subsequent exposure of fetal Rh D antigen , her previouslyprimed B cells act swiftly to produce IgG ( albumin antibodies ) whichcan cross placenta ---and bounds to antigen of fetal RBCs causingsequestration and destruction of fetal RBCs.

Antibodies are formed in 16% of the mothers significantly after6months of larger amount of fetomaternal bleeding and once formedremain in circulation through the life. And each subsequent pregnancywill have booster effect especially when fetus is Rh +Ve fetus is inutero.

3 D image of fetus with features of immune hydrops

Rh –is0 immune –Hyderops ( aNasarca)

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.

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)

Pathogenesis

Conditions Associated with NIH (This list is not

exhaustive)

Cardiac

Cardiomyopathy, Ebstein's anomaly, pulmonary atresia, coarctationof 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 (α) 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.

Non Immune Hydrops Fetalis( USG) Trisomy case

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 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

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 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 .

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.

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.

bibliography

http://www.perinatology.com/conditions/Hydrops.htm

http://emedicine.medscape.com/article/974571-overview

http://www.stanfordchildrens.org/en/topic/default?id=hydrops-fetalis-90-P02374