Evaluation of the Hydropic Fetus

Henry L. Galan, MD

Professor

Department of OB/GYN and Division of MFM

Henry L. Galan, MD

Discloses no relevant financial

relationships with commercial interests.

Objectives

Following this lecture, the learner should be able

to:

• Define hydrops fetalis

• Discuss immune and non-immune causes of hydrops

• Discuss the use of ultrasound and MCA PSV in

assessment and management of isoimmunization

• Describe the work-up for hydrops fetalis

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Hydrops Fetalis

• Fluid accumulation in two or more

potential spaces within the conceptus

Hydrops FetalisUltrasound Findings

Pleural Effusion Pericardial Effusion

Hydrops FetalisUltrasound Findings

Skin/Scalp Edema Ascites

>5mm

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Hydrops FetalisUltrasound Findings

Gross HydropsAnasarca

Hydrops Fetalis

Placentomegaly and/or polyhydramnios are

considered part of the diagnostic criteria for

hydrops (e.g. they are two of the potential spaces

that can be counted towards hydrops diagnosis).

A.True

B.False

Hydrops FetalisOther Ultrasound Findings

Polyhydramnios Placentomegaly

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Hydrops FetalisOther Ultrasound Findings

PlacentomegalyHoddick WK et al. JUM 1985;4:479

2 >4cm

3 >6cm

Lee et al. JUM 2012;31:213

Hoddick et al. JUM 1985;4:479

NIHFDoes Placentomegaly or Polyhydramnios Matter?

• 1994-2013; 153 NIHF w/ vs w/o poly and/or PM (P/PM)• Endpoints: 1o IUFD; 2o PTB

Etiology %

Chest Mass (CPAM & CHAOS) 35.3

Cardiac Dz 15.7

Primary hydrothorax 12.4

Hematologic (non‐RBC iso)/lymphatic dz) 11.1

CDH 7.8

Aneuploidy 5.8

Other (idopathic, anomalies, GU, SCT) 11.8

• 121/153 (80%) had P/PM• P/PM more common in chest vs non-chest mass etiology

88.89% versus 73.74%; P=.037JUM 2018;37:1185

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Underlying Pathogenesis of NIHF?

Imbalance in the regulation of fluid movement between the vascular and interstitial spaces, with an increase in

interstitial fluid production OR a decrease in lymphatic return.

Pathogenesis Mechanisms of NIHF?

• Increased right heart pressure: resulting in increased central venous pressure (eg, structural heart defects)

• Obstruction of venous or arterial blood flow: pulmonary masses

• Inadequate diastolic ventricular filling: arrhythmias

• Hepatic venous congestion: leading to decreased hepatic function and hypoalbuminemia.

• Increased capillary permeability congenital infection

• Anemia: leading to high output cardiac failure and extramedullary hematopoiesis, often with resultant hepatic dysfunction.

• Lymphatic vessel dysplasia and obstruction: eg, cystic hygroma) and reduced osmotic pressure.

Classification of Hydrops

Immune Non-Immune (NIFH)

Obstet Gynecol 1992;79:256

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Maternal-Fetal Interface

Mother Fetusm

Ag

fAg

f

B

+

IgM IgG

Ag

fIgG

Ag

fAg

fBB BB

B B +

IgG IgG IgG IgG

IgG IgG IgG

IgG IgG

+

Immune Hydrops Fetalis

Management of Rh Disease

Maternal Type and Rh with Ab screen

+Ab screen (paternal serotype & genotype)

Serial Maternal Titers (<24w q 4w; >24w q2w)

Critical Titer Threshold Reached

Serial MCA Doppler Studies

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Extramedullary Hematopoesis

Smrcek et al. Usg Ob Gyn 2001;17:403

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Hepatomegaly

Smrcek et al. Usg Ob Gyn 2001;17:403

Liver Nomogram

Live

rLe

ngth

Gestational Age (weeks)

Liver Nomogram

Splenomegaly

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Fetal Spleen Diameters

Splenomegaly

Etiology of NIHF

Obstet Gynecol 1992;79:256AJOG 2015 Feb;212(2):127Am J Med Genet 1989;34:366 Pediatr Neurol 1994;11::18Prenat Diagn 2011;31:186Pediatrics 2007;120:84Pediatrics 2009;123:1191

Cardiovascular 17‐35%

Chromosomal 7‐16%

Hematologic 4‐12%

Infectious 5‐7%

Thoracic 6%

TTTS 3‐10%

GUanomalies 2‐3%

GI anomalies 0.5‐4%

LymphaticDysplasia 5‐6%

Tumors (including

chorioangiomas)2‐3%

Skeletal dysplasias 3‐4%

Syndromic 3‐4%

Inborn erros of metabolism 1‐2%

Misc 3‐15%

Unknown 15‐25%

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Causes of NIHF: Cardiovascular

• Cardiovascular abnormalities are the most common cause of NIHF, accounting for about 20% of cases.

• Cardiac structural abnormalities, arrhythmias, cardiomyopathy, cardiac tumors, or vascular abnormalities.

• Results from increased central venous pressure due to a structural malformation or from inadequate diastolic ventricular filling.

• The most common congenital heart defects reported in association with NIHF are right heart defects.

• The prognosis is poor, with combined fetal and infant mortality reported as 92%.

Cardiomyopathy

Cardiomegaly

CT ratio >0.4

SVT

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Fetal Arrhythmias

• Tachyarrhythmia

– SVT

– Atrial Flutter

• Bradycardia

– Congenital heart block

• Antibodies (Ro/La)

• Endocardial cushion defects with heterotaxy

• 20 year year-old G2 P0010 at 36w 1d gestation

• Referred by regional MFM for intracardiac echogenicities and bilateral cystic kidneys

• Patient has TS

- Initially presented with cutaneous skin lesions; workup revealed tubular sclerosis with lesions in her brain and associated findings in her kidneys

- No seizure history

• Renal function has not been tested years and she has had no recent diagnostic cranial studies.

• FmHx: negative

Case

• tii¥I II M H1 11.8cm / L3 / 36Hz Tlb 0.1 LW 11/12/2012 11:15:36 AMTCH FETAL

Har-mid

G11 6C8 / M7FF4 i E2

SR I II 4 i CR I 3

- ...---.--:

...

- ꞏ~ꞏ,' -Page: 42 of 331

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Tlb 0.1 LW15.4cm / 2.0 / 31Hz

-..BM¥WEM6!

Voh.J .oo

E!J.

11/12/2012 11:12: 58AM2+3 Trim.

Har -low

....' - 'f

Gn IJC 6 / M7FF3 JE2

SR I II 4, CR I 3

.,. ,.

•

'

.,

• Most common infectious

- Parvovirus, CMV, Toxoplasmosis, syphilis

• Rare

- Coxsackie

- Trypanosomiasis

- Varicella

- HHV 6 and 7

- HSV

- RSV

Causes of NIHF: Infectious

Viral Cardiomyopathy

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27 year old G2 P1001 at 21 weeks and 6 days gestation referred to CFCC for fetal hydrops.

Case

MCA PSV 59.9cm/s = 2.15 MOM

• Insert jpegs of MCA

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1st attempt at PCI

-- - --- ꞏ- -_-~----.,,-..65"/ 35Hz

RoutineHar-highGn 0

C 6 / M 8Ff 2/ El

SRI D 2/ CRI 3

•-•- .•., -•-

.....-.- .....

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2nd attempt - intrahepatic

3rd attempt – Free Loop

• Post IUT hct 24%.• Two PUBS and IUTs on 8/7 and 8/10• FISH/karyotype normal and microarray normal.• Positive amniotic fluid Parvovirus PCR.• Progressive improvement in skin edema, pericardial effusion,

and ascites (which persisted for a long time). Placenta remains thickened.

• Last MCA PSV before delivery: 0.8 MOM.

Plan:• Delivered at 37 weeks

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Hydrops Fetalis

The most common type of infection that results in

fetal hydrops is:

A. CMV

B. Parvovirus

C. Toxoplasmosis

D. Coxsackie

E. Computer

Infectious Causes of NIHFParvovirus

• Most common viral etiology.

• Attacks erythroid progenitor cells inhibition of erythropoiesis and subsequent anemia.

• Poorest fetal outcomes when the congenital infection occurs in the early second trimester (< 20 weeks of gestation).

• Risk of fetal death 15% at 13-20 weeks of gestation, and 6% after 20 weeks. In most cases.

• Anemia is transient and fetal intravascular transfusion can support a fetus through this aplastic crisis.

37yo G2 P1001 at 30 weeks referred to CFCC by a MFM colleague with skin edema, polyhydramnios, and non-reassuring BPP (2/8).

Case

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LMP II GA(EDD) 30w0d IED

D

011041201.9 I G _j Ab

_j

DOC. I ꞏGA(AlJA) 31.w3d l:DD(AUA} 06/24/2019 p _J Ee _jAge [lange GP H dlodꞏEfW (Hadlock}

AC/BPO/H/1--IC

Value

1i'02g (31b12o:t)

Range

± .248gI I

II 75.5%I j

20 Mci i ' l isurnmcnls m2 m3 Mc1h. -GP Age

BPD {Hadlock) 32w6d-- - -- tI

HC (HadIock) --1

AC ( adlock)

AUA Valun

.,

.,

.I 26.40

FL {hladlockJ .I :--lHL (Jeanty)

Ceret:J (I lifO

8.17 cm

29.76 cm

26.02 rm

6.09 rm

J.18 cm

J.70 tm

ml

6.1'1

29.76

25.63

6.09

5.18

11!! t70

a1,1g.

avg.

avg.

avg.

avg.

avg.

97.7%

90.7%

50.14H,

79.8%

60.4%

50.0%

33w0d

30wld

31w4d

30wld

30w3d'iCM

Vp6 .52 mm

6.14 mm

652

7_.q5 4.82

.a vg.

avg.

20 Meas.u1ements Vafue

Afl

Ql 12.91 cm

ml

12,g1

ml' m3 rn-4 mS m6 Mellt.

avg,

'E

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NIHFWork-Up

• Include: fetal echoumbilical cord, placenta & AF

SMFM. Nonimmune hydrops fetalis. AJOG 2015

• Consider chorioangioma picture and then flip back to workup algorithm

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Maternal Impact of NIHFMirror Syndrome

• Mirror Syndrome – An uncommon complication

• Maternal edema that “mirrors” her hydropic fetus

• May represent a form of PreE (90% edema, 60% HTN, and 40% proteinuria)

– Other findings of PreE /HELLP possible

• Review of 56 cases

– Main maternal morbidity was pulmonary edema (21%)

Fetal Diagn Ther 2010;27:191

Maternal Impact of NIHFMirror Syndrome

• Resolution: with treatment of hydrops or delivery

– With treatment of hydrops or delivery

Fetal Diagn Ther 2010;27:191 Hypertens Pregnancy 2011;30:322

– Other findings of PreE /HELLP possible

Fetal Diagn Ther 2013;34:176 Obstet Gynecol 2007;110:540

UOG 2012;40:367EJOGRB 2000;88:201

• Imbalance of angiogenic/antiangiogenic as in PreE

– Imbalance resolves with treatment/resolution ofNIHF

Fetal Diagn Ther 2013;34:176UOG 2012;40:367

NIHFPrognosis

• Depends on: cause, GA, AS, extent of resuscitation in delivery room and if transport required

s Am J Perinatol 2007;24:33

• <24 weeks:

– 50% had aneuploidy; prognosis extremely poor

– <50% survival with normal karyotypeObstet Gynecol 1992;79:256

• Series of 71 cases beyond 20 weeks w/o aneuploidy

– Survival 50%; 25%w/o major morbidities

Prenatal Diagn 2011;31:186

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NIHFPrognosis

• In newborn infants born alive with NIFH:

– NN mortality (NNM) has high as 60%

– Chylothorax: NNM as low as 6%

– Treatable causes (fetal arrhythmia, parvo): better prognosis

• Long-term prognosis:

– Depends on underlyingcause

Pediatrics 2007;120:84;AJP20007;24:33

JMFNM 2011;24:258

– After IUT for Parvo, still risk for ND delay

– Fetuses with SVT may develop WPWObstet Gynecol 2007;109:42

Early Human Dev 2011;87:83

Key Points / Clinical Pearls• NIHF is uncommon; Incidence of 1/1700 – 1/3000

• Immune causes have decreased dramatically since

introduction of Rhogam

• 70%NIHF (2/3) due to CV, aneuploidy, hematologic &

infection

• Ultrasound anatomy survey & MCA PSV are central to the

evaluation of NIHF

• NIHF risk to mother via Mirror syndrome (uncommon)

• Prognosis depends on underlying etiology, but is

generally poor with approximately 50% mortality overall.

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