This document provides an overview of hydrops fetalis (fetal edema). It begins by defining hydrops fetalis and listing the criteria. It then discusses the history, classifications (immune vs. non-immune), etiologies, associated conditions, investigations, management approaches, and prognosis. For non-immune hydrops, over 100 causes have been identified, most commonly cardiovascular, chromosomal, infectious, or structural anomalies. Evaluation involves detailed ultrasound, fetal testing and treatment depends on the underlying cause but prognosis remains poor, especially if diagnosed early in pregnancy or structural defects are present.

1. R
HYDROPS FETALIS
RAKESH KOTHA MD DM
Associate professor of neonatology Niloufer
hospital

2. HYDROPS
FETALIS
Hydrops fetalis (Latin) is a condition in the fetuscharacterized by an abnormal collection of fluid
with at least two of the following:
Edema
(fluid beneath the skin, more than 5 mm)
Ascites
Pleural effusion
Pericardial effusion
Frequently associated with polyhydramnios and a thickened placenta (4-6 cm) (Not
included in criteria ) compensate for the fetal hypoxia penetration into
myometrium. (%)

3. 400BC Hippocrates
1609AD First clinical report (Twin,French midwife)
1892 AD Ballantine multiple etiology of hydrops
1940AD Landsteiner Rh factor identification
1941 AD Levine et al Rh pathophysiology
1943 AD Potters immune vs non immune hydrops
History

4. Broadlydivided into 2categories:
ImmuneHydrops
Non-immunehydops

5. incidence
Accurate estimates of incidence are difficult (casesarenot detected
before intrauterine death, spontaneously resolve antenatally)
Vary depending on the region studied, differences in etiology,
ethnicity of population, availability of sophisticated investigations
Hydrops fetalis is found in about 1-6 per 1,000 births and is
categorized asimmune or nonimmune hydrops.
Immune hydrops (accounts for 10-20%of cases)
decreasing……………
Parvo: SEA CVS :WORLD

6. IMMUNE HYDROPS :
Classically Rh (Kell, Duffy) Anemia
Good prognosis as well established protocols
First baby spared because of Ig M as Ig G takes 5-6 months
Pinocytosis
Wide speared use of Anti-D (80 RBCs 50 LPF 4 ml 100ugms )

7. NON IMMUNE HYDROPS(80-90%)
Failure of absorption of inertial fluid into the venous
system
No common mechanisum
Fetus /Placenta/ Mother

8. Thismaydue to:
Cardiac failure :
Highoutput failure (sacrococcygealteratoma, fetal adrenal neuroblastoma)
Impaired venousreturn :
CCAM
Obstruction to normal lymphatic flow
:
Thoracic malformations
Increasedcapillary permeability :
CMV vasculitis
Decreased colloidal osmotic pressure
: Congenitalnephroticsyndrome
Pathogenesis (mayoverlap
(etiology))

11. Non-immune Hydrops Fetalis

12. Progressive ultrasound findings of IHF include
the
early findings of polyhydramnios and
placentomegaly,
followed by hepatomegaly and pericardial
effusions and
late findings of ascites, scalp edema, pleural
effusions,
and finally oligohydramnios.

13. More than 100 causesor associations of NIHFhavebeen found.
cardiovascular pathologies (35 %),chromosomal anomalies (20%),anemias (15%),
malformation syndromes (15%),infections (10%), liver diseases(5%),and
miscellaneous causes(5%)
About 10 to 20%of casesare idiopathic depending on the thoroughness of
investigations.
ConditionsAssociatedwithNIH

14. Cardiac
Cardiomyopathy, Ebstein's anomaly, pulmonary atresia, coarctation oftheaorta,hypoplastic left heart,
complete AVcanal, leftsided obstructive lesions, premature closure oftheforamen ovale
Intracardiac tumors (tuberous
sclerosis) Cardiac arrhythmia
SVT,flutter, heart block, WPWsyndrome
Chromosomal/GeneticSyndromes
T13, T18,T21,XO(Turners syndrome) , Noonan syndrome ,multiplepterygium syndrome, Pena-
Shokeir,arthrogryposis
FetalAnemia
Alpha (α) thalassemia, parvovirus, fetalhemorrhage, G-6-PD deficiency
Infection
Parvovirus, CMV, syphilis,coxsackie virus, rubella, toxoplasmosis, herpes,varicella, adenovirus,
enterovirus, influenza,listeria
ThoracicAbnormalities
Congenital cystic adenomatoid malformation (CCAM) ,chylothorax, diaphragmatic hernia,
mediastinal tumor,skeletal dysplasias
Twinnning
Twintotwintransfusion Severe anemia inthedonor twinor high-output failureinthe recipient
Tumors
Fetalsacrococcygeal teratoma, hemangiomas (Hepatic, Klippel-Trenaunay syndrome), fetaladrenal
neuroblastoma, placental tumors (chorioangioma)
Miscellaneous
Cystichygromas, inheritabledisorders ofmetabolism (lysosomal storage diseases) ,maternal
thyroiddisease, congenital nephrotic syndrome. Sly

15. It is difficult to find aplausible explanation for the development of
hydrops in many caseslisted in the previous slide
(such asmalformations, hepatic causesor metabolic diseases) which
mayrepresent chance associations.
However, asageneral rule, NIHF presenting before 24 weeksis usually
due to chromosomal aberrations,
Whilehydrops presenting after this is usually due to structural
anomalies
(such ascardiac and pulmonary)

16. Fetal supraventricular tachyarrhythmias (SVT)
Congenital heart block (CHB)
NIHFin SEAasaresult of homozygous a-thalassemia (Hb Barts). 86 %(25/29)
Congenital cystic adenomatoid malformation of the lung (CCAM)is the most common
thoracic lesion associated with hydrops.
Neurologicmalformations area rare causeof hydropswhich includesencephalocele,
porencephalywith absent corpuscallosum,fetal intracranial hemorrhage,andveinof Galen
aneurysm.
Skeletaldysplasias thoracic compression,

17. parvovirus B19,infection maybe self limitedand mayresolve spontaneously
.Approximately 85%of fetusestreated by in-utero transfusion survive.
NIHF mayrarely occurin associationwith severematernal medicalconditions suchas
severeanemia,diabetesmellitus, or hypoproteinemia.
Spontaneous resolution seen in
Cardiac arrhythmias
Twin to Twin transfusion
CCAM
Parvo & CMV
Placental Chorangioma

18. Presentation
Hydrops fetalis is typically diagnosedduring ultrasound evaluation for other complaints such
as:
Polyhydramnios,Sizegreater than dates,Fetal tachycardia ,Decreasedfetal movement
Abnormal serum screening,Antenatal hemorrhage
May be diagnosedon routine sonographic screeningor maybe diagnosed after fetal death.
Themother maydevelop edema , hypertension, and proteinuria during conservative
management of hydrops acondition known asMirror syndrome
(pseudotoxemia or Ballantyne syndrome)
Thematernal condition can quickly deteriorate into fulminant pre- eclampsia, and
eventually, eclampsia.
early onset severe pre-eclampsia or polyhydramnios beginning at 28 weeks

19. Extensive clinical workup is required to attempt to identify the specific etiology
In patients in whom NIHFis suspected, the search for acausestarts with amaternal
evaluation.
Initial clinical history taking should be directed toward the presence of hereditary or
metabolic diseases,diabetes, infections, anemias, Consanguinity
INVESTIGATIONS

20. Detailed Ultrasound Scan
Fetal Echocardiography
Doppler Blood Flow Studies (MCA PSV 1.5MOM)
Liquor Volume Assessment (AFI)
Placental Thickness & Echogenicity

21. MCA?

22. Initial investigations include an indirect Coombstest to exclude immunecauses,
Routine blood counts and indices to exclude thalassemias;
maternal blood chemistry testing for G-6-PDdeficiency;
Betke-Kleihauer testing for fetal-maternaltransfusion;
and screening for toxoplasmosis, other infections, rubella, CMV,and herpes simplex
(TORCH)infection during intrauterinepregnancy.

23. Amniocentesis is needed to perform fetal karyotyping,
amniotic fluid culture
testing for CMVinfections
assessmentof α-fetoprotein (AFP)levels
testing for thalassemia, and determination of the lecithin-sphingomyelin (L/S)rati
Karyotyping canalsobe performed with tissue obtained by chorionic villous sampling (CVS)
or with fluid obtained from one of the fetal cavities
TheFISHtechnique canalsohelp in the detection of specific deletions and chromosomal
rearrangements, and the results are often available within 24-48 hours
Enzyme analysis ,P
C
R.,Histologicalexamination of fetal tissues.

25. MANAGE
ME
NT
Counseling
Longterm prognosisdepends on underlying causeand severity of the heart failure
If the causeof NIHcannot be determined, the perinatal mortality is approximately 50%
Prognosis is much poorer if diagnosed at less than 24 weeks , pleural effusion is present, or
structural abnormalities are present
Pulmonary hypoplasia is acommon causeof death in neonates with plerual effusions.
Fetal hydrops associated with a structural heart defect is associated with an almost 100%
mortality rate
If early in pregnancy (less than 24 weeks) with no treatable cause the option of termination
may be a
consideration.
Recurrence is uncommon unless related to blood group incompatibility
(isoimmunization) or inheritable disorder

26. 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
fetusmay
be followed with repeat sonograms every 1 to 2 weeks and antenatal
testing
Patients treated for immune hydrops are usually delivered at 34- 37 weeks'
orwhen fetal lung maturity hasbeenconfirmed.
Consultation with the neonatologist may help to decide when it is
appropriate to proceed with preterm delivery for possible postnatal
treatment
Themother should be evaluated frequently for signsof "mirror"syndrome

27. Fetaltherapy:
Only about 20 to 30%of fetuses with NIHFare suitable for fetal intervention even in thebest
equipped feto-maternal centers. Currently, only hydrops associatedwith these conditions
maybe amenable for antenatal treatment:
a) anemia,
b) arrhythmias,
c) pulmonary conditions,
d) twin-twin transfusion,
e) tumours, CPAM
f) miscellaneous egpleural effusion.

28. Decisions ?:
a)the natural history for many conditions are uncertain orunknown,
b)very few properly designed clinical trials are available regarding treatment
efficacy and safety
c)agood fetal outcome cannot be guaranteed despite significant fetal and
maternal risks
entailed in invasive fetal procedures, and
d)spontaneous remission of the hydropic processhasbeen documented
e)invasive interventions, suchasfetal transfusion, mayneed to proceed before
definitive tests results, such askaryotype, become available.

29. Fetal anemia is the pathology most amenable to prenatal therapy
Ahematocrit of lessthan 30%is an indication for transfusion
Transfusion with irradiated Kell and Rhesusnegative, type O,crossedmatched blood
compatible with maternal serum has led to an improved outcomes
Umbillical and peritonial transfusion
Formula of Bowman [volume = (weeks’ gestation − 20) Å~ 10 mL]

30. Fetal surgery is not yet commonly available inIndia.
Interventions performed include:
Needle aspiration
Feto-amniotic shunting
repair of congenital diaphragmatic hernia, resection of cystadenomatoid
malformation of the lung, and excision oflarge sacrococcygealteratomas.
Fetal endoscopic surgery for TRAP,PLUGfor casesofCDH.

31. DE
LIVE
R
Y
NeonatalManagement:
Hydropic fetuses should be delivered ascloseto term aspossible in atertiary center with a
multidisciplinary neonatal resuscitation team supported by aneonatal intensive care unit
(NICU).
LSCS/SVD
Earlydelivery, however, is indicated in the following situations in addition to usual
obstetricindications:
a)if fetal testing becomesnonreassuring
b)if pre-eclampsia complicates hydrops (which canbe up to 50%of cases)
c) if preterm labour is established (often precipitated by polyhydramnios, hence repeated
amniocentesesmayhelp prevent this and help relieve maternal discomfort)

32. Delivery
a) ultrasound should be repeated early in labour to assesswhether effusions inthe
lung, pericardium, or peritoneum mayrequire aspiration before or at delivery (eg.
thoracocentesis, paracentesis and chest tube drainage) to facilitate adequate
postnatal ventilation andcirculation.
Preparation of appropriate equipment forintubation
Umblical lines
Medications
Thoracocentesis and paracentesis

34. Neonatal Management
intubation is often difficult
high-frequency ventilator and high airway pressure settings will oftenbe needed to
achieve adequate oxygenation,
umbilical artery and vein lines mayhelp with administration ofvarious agents and
allows monitoring of blood gasesand arterial and venouspressures,
cautious useof inotropic agents, diuretics, blood products, albumin, and fluids to
maintain an adequate cardiac function without fluid overload or soft-tissue edema

35. once the neonate is stabilized, promptly transfer the baby toNICU,
Full physical examination aided by relevant echocardiographic,and radiologic
investigations to exclude structural malformation, and hematologic tests to rule out
sepsis,biochemical and karyotypic anomalies; the placenta should be sent for
histology andculture,
specific treatment is based on the underlying etiology and appropriate referral to the
relevant pediatric subspecialist including clinical geneticist and pediatricsurgeon.

36. C
ONC
LUS
IONS
Non-immune hydrops fetalis is an uncommon but serious disorder associated withan
overall poor prognosis. Theexact pathophysiology of NIHFis still obscure, but araised
central venous pressure is an important prerequisite tothe development of hydrops.
Thepregnant woman with newly diagnosed hydrops should be promptly referred toa
subspecialist feto-maternal medicine unit with amultidisciplinaryteam.
Thisteam usually consists of perinatologists, neonatologists, clinical geneticists,and
other pediatric sub-specialists all of whom are better trained to deal with this
condition and provide parents with accurate information and psychologicalsupport.

37. C
ONC
LUS
IONS
Theultrasonographic detection of hydrops fetalis is usually straightforward,
butits etiology Detailedprenatal diagnosticinvestigationsallow identification of
disorders amenable to treatment, avoidance of inappropriateinvasive fetal
interventions.
Fetal interventions include both medical and surgical modalities. Fetal surgery isstill
in its infancy applicable to only afew conditions

38. C
ONC
LUS
IONS
Neonatal management of NIHFrequires askilled and coordinated resuscitation team
backedby awell equipped NICU.
Fetal hydrops mortality depends on cause
In Pleural effusion pulmonary hypolasia is most common cause of death
Parentsshould be offered apostmortem after the death, Genetic evaluation

39. R
E
F
E
R
E
N
C
E
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