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Chapter 128 Nonimmune Hydrops Fetalis
128
CHAPTER
Nonimmune Hydrops
Fetalis
Key Points
Nonimmune hydrops fetalis is a serious fetal
condition in which abnormal fluid accumulates in
at least two different fetal compartments, and in
which circulating antibodies against red-cell
antigens are absent in the mother.
Nonimmune hydrops fetalis is a heterogeneous
disorder, caused by a large number of underlying
pathologic processes. While the majority of cases
appear to be idiopathic, the most common
recognizable cause is cardiovascular pathology.
Following the sonographic detection of hydrops,
the most important step is to differentiate between
immune and nonimmune causes. Once immune
causes are excluded, a detailed anatomical survey
is needed to rule out congenital abnormalities,
which could be the cause of the hydrops.
Nonimmune hydrops can occur secondary to fetal
anatomical abnormalities (cardiac, thoracic,
gastrointestinal, neurologic, genitourinary,
vascular, or skeletal), placental/cord abnormalities,
fetal hematologic, neoplastic or metabolic
disorders, infection, fetal genetic anomalies, and
maternal abnormalities.
Maternal blood tests should include an indirect
Coombs antibody screen, maternal blood type,
KleihauerBetke stain, complete blood count with
differential and erythrocyte indices, hemoglobin
electrophoresis, and glucose-6-phosphate
dehydrogenase deficiency screen. Additional
maternal blood work should include TORCH titers,
syphilis screen, and parvovirus B19 IgG and IgM
titers.
Fetal echocardiography and invasive testing for
fetal karyotype should be offered.
While the optimal mode of delivery is uncertain,
cesarean section is advised for all potentially viable
fetuses due to the risk for soft-tissue dystocia.
Fetal therapy may be possible, including PUBS
with transfusion, maternal administration of
cardiac medications, and fetal shunt placement.
The long-term prognosis will depend on the
nature of the underlying abnormality.
The recurrence risk will depend on the underlying
etiology of the nonimmune hydrops.
CONDITION
Nonimmune hydrops fetalis (NIHF) is a serious fetal con-
dition in which abnormal uid accumulates in at least two
different fetal compartments, and in which circulating an-
tibodies against red cell antigens are absent in the mother.
Hydrops fetalis is associated with a pathologic increase in in-
terstitial and total fetal body water, usually appearing in fetal
soft tissues and serous cavities. It may be either immunologic
or nonimmunologic, depending on the presence or absence
of maternal antibodies against fetal red cell antigens. While
it was previously thought that the majority of cases of hy-
drops fetalis were secondary to maternalfetal blood group
incompatibilities, it is now estimated that over 90% of cases
are nonimmunologic (Santolaya et al., 1992).
NIHF is a heterogeneous disorder, caused by a large
number of underlying pathologic processes. The majority of
cases appear to be idiopathic, although some investigators
have stated that with thorough investigation an underlying
cause can be identied in as many as 84%of cases (Holzgreve
et al., 1984; Norton, 1994; Wilkins, 1999). Whenprenatallydi-
agnosedcases of NIHFandcases of intrauterine fetal deathare
included, the success rate in discovering an underling cause
for NIHFmay be as lowas 40%(Norton, 1994). The literature
on NIHF consists almost entirely of case reports or small case
series, together with reviews of these case series. A review of
these reports suggests that the most common recognizable
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cause of NIHF is cardiovascular pathology (accounting for
17% to 35% of cases), followed by chromosomal abnormal-
ities (accounting for 14% to 16% of cases), and hemato-
logic disorders (accounting for 4% to 12% of cases) (Norton,
1994). The remainder of causes of NIHF are rare conditions,
many of which are difcult to diagnose prenatally. The range
of possible underlying causes for NIHF is summarized in
Table 128-1, listed in order of expected incidence.
Other than idiopathic cases, cardiac malformations ac-
count for the majorityof cases of NIHF(Knilans, 1995). There
is no particular form of cardiac abnormality that always re-
sults in hydrops, although the more severe the malformation
the greater the likelihood of hydrops developing. The com-
mon mechanism of NIHF in cases of cardiac malformation
or arrhythmia is the development of congestive heart failure,
with increasing generalized uid overload. The prognosis for
fetuses with structural cardiac malformations and hydrops is
extremely poor, with a mortality rate approaching 100% in
some series (Crawford et al., 1988).
Chromosomal causes of NIHF are common; the most
frequent is Turner syndrome, with its typical sonographic
nding of cystic hygroma. A wide range of other genetic syn-
dromes is also associated with NIHF, such as arthrogrypo-
sis, tuberous sclerosis, PenaShokeir syndrome, and Noonan
syndrome (Jauniaux et al., 1990).
The underlying mechanismfor the associationof NIHF
with hematologic abnormalities is most likely severe fetal
anemia leading to high-output cardiac failure (Arcasoy and
Gallagher, 1995). Fetal anemia canresult fromfailure to man-
ufacture normal hemoglobin (such as -thalassemia), fetal
hemorrhage (such as intracranial bleeding), hemolysis, (such
as glucose-6-phosphate dehydrogenase deciency), or failure
to form erythrocytes because of marrow destruction (such as
parvovirus B19 infection).
Congenital cystic adenomatoidmalformation(CCAM)
is the most common thoracic lesion associated with hy-
drops. Other thoracic causes of hydrops include bronchopul-
monary sequestration, thoracic masses, and diaphragmatic
hernia. The underlying mechanism for the association of
hydrops with these thoracic conditions is most likely ob-
struction to venous return because of increased intrathoracic
pressure.
Congenital infectionwitha wide variety of organisms is
a well-recognized cause of hydrops (Barron and Pass, 1995).
Causative organisms include syphilis, cytomegalovirus, par-
vovirus B19, toxoplasmosis, herpes simplex, rubella, and
coxsackievirus. Possible mechanisms for the association of
congenital infection with hydrops include fetal anemia from
suppression of erythrocyte production, fetal myocarditis, or
fetal hepatitis. In some cases of congenital infection, such as
syphilis, the presence of hydrops is associatedwitha very poor
prognosis. In other cases, hydrops from congenital infection
may be self-limited and may resolve spontaneously. Fetal par-
vovirus B19 infection results in an aplastic crisis, which leads
to profound anemia, and hydrops, the outcome of which may
be either fetal death or spontaneous resolution without long-
term morbidity (Morey et al., 1991; Levy et al., 1997).
A wide variety of structural fetal malformations has
beenassociatedwiththe development of NIHF. These include
skeletal dysplasias, which may be associated with thoracic
compression, impairment of venous return, and subsequent
hydrops. The association of other structural malformations
with NIHF, such as gastrointestinal, genitourinary, and neu-
rologic abnormalities, may represent chance occurrences, as
it is difcult to elucidate a plausible underlying mechanism
for hydrops in many cases.
Some fetal conditions, such as Finnish nephrosis and
hepatic brosis, result in profound hypoproteinemia or cir-
rhosis, which may subsequently lead to hydrops. The mech-
anism for the association between metabolic disorders and
NIHF is unclear, but may be related to soft-tissue swelling
and obstruction of venous return to the heart. Certain non-
cardiac fetal abnormalities may result in hydrops by causing
high-output cardiac failure. Examples include sacrococcygeal
teratoma, neuroblastoma, placental chorioangioma, andum-
bilical cord masses.
NIHF may very rarely occur in association with sig-
nicant maternal medical illnesses, such as severe anemia,
hypoproteinemia, or diabetes mellitus. The mirror syn-
drome, also known as Ballantyne syndrome, is the combi-
nation of fetal hydrops with generalized uid overload and a
preeclampsia-like state in the mother (Carbillon et al., 1997).
In general, the maternal clinical features resolve only with
delivery of the fetus and placenta.
INCIDENCE
The precise incidence of NIHFis difcult toelucidate, as many
cases are not detected prior to intrauterine fetal death, and
some cases may resolve spontaneously in utero. The generally
reported incidence of NIHF is 1 in 1500 to 1 in 4000 deliveries
(Romero et al., 1988; Wilkins, 1999).
SONOGRAPHIC FINDINGS
The diagnosis of hydrops is made following the detection
of abnormal or increased uid accumulation in at least two
distinct fetal body cavities. Examples include pericardial ef-
fusion, pleural effusion, ascites, subcutaneous edema, cystic
hygroma, polyhydramnios, and placental thickening (Figures
128-1 and 128-2). In general, skin thickness of at least 5 mm
is required to diagnose subcutaneous edema, and a placental
thickness of at least 6 cm is required to diagnose placen-
tomegaly (Romero et al., 1988). These features do not neces-
sarily indicate hydrops; it should be noted that skin thicken-
ing of at least 5 mm may be commonly seen in macrosomic
fetuses. If abnormal uid accumulation is conned to only
one site, then the diagnosis of hydrops should not be used,
and the case should be described simply in terms of the in-
volvedsite, suchas isolatedascites or isolatedpleural effusion.
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Chapter 128 Nonimmune Hydrops Fetalis
Table 128-1
Conditions Associated with Nonimmune Hydrops Fetalis
Idiopathic hydrops Unknown cause
Cardiac malformation Hypoplastic left ventricle; Atrioventricular canal defect; Atrial septal defect; Ventricular septal defect; Tetralogy
of Fallot; Hypoplastic right ventricle; Ebsteins anomaly; Truncus arteriosus; Transposition of great vessels;
Aortic stenosis or atresia; Pulmonary stenosis or atresia; Cardiomyopathy; Endocardial broelastosis;
Premature closure of ductus arteriosus; Premature closure of foramen ovale; Rhabdomyoma; Intrapericardial
teratoma
Cardiac arrhythmia Supraventricular tachycardia; Atrial utter; Heart block; Bradyarrhythmias; WolffParkinsonWhite syndrome
Chromosomal
abnormality
Trisomy 21; Trisomy 18; Trisomy 13; Turner syndrome; Triploidy; Tetraploidy; 18q+; 13q; Others
Hematologic disorder Alpha-thalassemia; Parvovirus B19 infection; Fetomaternal transfusion; In utero hemorrhage;
Glucose-6-phosphate dehydrogenase deciency; Fetal red cell enzyme deciencies; Twin-to-twin transfusion
syndrome
Thoracic abnormality Congenital cystic adenomatoid malformation of lung; Bronchopulmonary sequestration; Diaphragmatic
hernia; Chylothorax; Pulmonary lymphangiectasia; Intrathoracic mass (teratoma, leiomyosarcoma);
Bronchogenic cyst
Genetic syndrome Arthrogryposis; Multiple pterygium syndrome; Noonan syndrome; PenaShokeir syndrome; Cornelia de Lange
syndrome; Tuberous sclerosis; Prune belly syndrome; Myotonic dystrophy; NeuLaxova syndrome
Infectious disease Cytomegalovirus; Parvovirus B19; Toxoplasmosis; Syphilis; Herpes simplex; Rubella; Coxsackievirus; Varicella;
Respiratory syncytial virus
Skeletal dysplasia Achondroplasia; Achondrogenesis; Osteogenesis imperfecta; Hypophosphatasia; Short rib polydactyly;
Thanatophoric dwarsm; Asphyxiating thoracic dysplasia; Osteochondrodystrophy; Osteochondrodysplasia;
Chondrodystrophy; Chondrodysplasia
Gastrointestinal
disorder
Diaphragmatic hernia; Esophageal or intestinal atresia; Imperforate anus; Midgut volvulus; Meconium
peritonitis; Duodenal diverticulum; Intestinal duplication; Malrotation
Genitourinary disorder Congenital Finnish nephrosis; Hypoplastic kidney; Polycystic kidneys; Renal vein thrombosis; Bladder outlet
obstruction; Dysplastic kidneys
Hepatic disorder Hepatic brosis; Cholestasis; Polycystic liver disease; Biliary atresia; Hepatic calcication; Giant cell hepatitis;
Cirrhosis with portal hypertension; Hepatic necrosis
Neoplastic disorder Neuroblastoma; Teratoma; Congenital leukemia; Pulmonary leiomyosarcoma; Hemangioendothelioma of liver
Metabolic disorder Gaucher disease; GM
1
gangliosidosis; Mucolipidoses; Mucopolysaccharidoses; Galactosialidosis; Carnitine
deciency; Pyruvate kinase deciency
Neurologic disorder Encephalocele; Fetal intracranial hemorrhage; Vein of Galen aneurysm; Porencephaly with absent corpus
callosum
Vascular disorder Arteriovenous malformation; Sacrococcygeal teratoma; Vena caval thrombosis; Hemangioendothelioma;
Arterial calcication; Cerebral angioma
Placenta/cord
abnormality
Chorioangioma; Chorionic vein thrombosis; Umbilical cord torsion; True knot of cord; Angiomyxoma of cord;
Placental hemorrhagic endovasculitis
Maternal abnormalities Mirror syndrome; Severe maternal anemia, diabetes mellitus or hypoproteinemia; Maternal indomethacin use
Adapted from:
Holzgreve W, Holzgreve B, Curry CJ: Nonimmune hydrops fetalis: diagnosis and management. Semin Perinatol 1985;9:52-57.
Romero R, Pilu G, Jeanty P, et al. Nonimmune hydrops fetalis. In Romero R, Pilu G, Jeanty P, Ghidini A, Hobbins JC (eds): Prenatal Diagnosis of Congenital
Anomalies. Norwalk, Appleton And Lange, 1988;414-426.
Jones DC: Nonimmune fetal hydrops: diagnosis and obstetrical management. Semin Perinatol 1995;19:447-461.
Wilkins I: Nonimmune hydrops. In Creasy RK, Resnik R (eds): Maternal-Fetal Medicine (4th edition). Philadelphia, WB Saunders, 1999;769-782.
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Figure 128-1 Prenatal ultrasound image of fetus at 26 weeks
with nonimmune hydrops. Sagittal viewdemonstrates extensive
ascites and pleural effusions.
NIHF may initially present with an isolated uid accumula-
tion in one site, such as pleural effusion with congenital cystic
adenomatoid malformation, but as intrathoracic pressure in-
creases andvenous returndecreases, generalizedhydrops may
present.
Fetal ascites is diagnosed sonographically by the visu-
alization of an echolucent rim encompassing the entire fetal
abdomen in a transverse view (see Figure 128-1). Loops of
bowel and the outline of the fetal liver, spleen, bladder, and
diaphragm are generally more easily seen in the presence of
Figure 128-2 Prenatal ultrasound image of fetus at 30 weeks
with nonimmune hydrops. Transverse view through fetal chest
demonstrates extensive pleural effusionwithdeviationof cardiac
axis to left.
ascites. Pericardial effusion is diagnosed by the appearance of
an echolucent rimof at least 1 to 3 mmthickness around both
cardiac ventricles. Pleural effusion, which may be unilateral
or bilateral, also presents as an echolucent space outlining the
diaphragm.
Other sonographic features of NIHF will depend on
the cause of the hydrops. A careful fetal anatomical survey,
including fetal echocardiography, should be performed in
all cases to detect any associated structural fetal malforma-
tions. Measurement of the middle cerebral artery peak sys-
tolic velocityusingDoppler ultrasoundcanhelpidentifycases
of NIHF associated with fetal anemia (Hernandez-Andrade
et al., 2004).
DIFFERENTIAL DIAGNOSIS
Following the sonographic diagnosis of hydrops the rst step
is to differentiate between immune and nonimmune causes.
This is easily accomplished by the performance of a maternal
indirect Coombs test to screen for antibodies associated with
blood group incompatibility.
Following the exclusion of immune causes of hydrops,
each condition listed in Table 128-1 should be considered
when trying to determine the cause of NIHF. This is accom-
plished by a review of maternal and paternal histories, de-
tailed fetal sonographic survey, and the use of appropriate
diagnostic laboratory studies.
ANTENATAL NATURAL HISTORY
The precise antenatal natural history of NIHF depends en-
tirely on the underlying cause. The natural history of hydrops
is poorly understood, so little information is available regard-
ing prognostic factors to predict in utero progression. In gen-
eral, NIHF is associated with a 75% to 90% fetal mortality
rate (Romero et al., 1988). The progression of fetal signs and
development reects theunderlyingcause. Cases of NIHFsec-
ondary to fetal parvovirus B19 infection, for example, may
result in intrauterine fetal death or may result in spontaneous
resolution. By contrast, cases of NIHF associated with struc-
tural cardiac malformation almost always result in intrauter-
ine fetal death or early neonatal death. No series are available
for review describing sufcient cases of NIHF caused by each
individual underlying cause, to enable accurate prediction of
antenatal natural history.
MANAGEMENT OF PREGNANCY
Following the sonographic detection of hydrops, the preg-
nant woman should be promptly referred to a tertiary care
facility, with availability of a multidisciplinary team con-
sisting of perinatologists, neonatologists, clinical geneticists,
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Chapter 128 Nonimmune Hydrops Fetalis
and other pediatric subspecialists. Careful maternal and pa-
ternal histories should be obtained, to evaluate for possible
health problems, family histories, or ethnic predisposition to
any of the conditions listed in Table 128-1. Maternal blood
work should include an indirect Coombs antibody screen,
maternal blood type, KleihauerBetke stain, complete blood
count with differential and erythrocyte indexes, hemoglobin
electrophoresis, and glucose-6-phosphate dehydrogenase de-
ciency screen. Additional maternal blood work should in-
clude anevaluationfor infectious diseases, including TORCH
(toxoplasmosis, rubella, cytomegalovirus, and herpes sim-
plex) titers, rapid plasma reagent test, and parvovirus B19
IgG and IgM titers.
A detailed fetal sonographic anatomy survey should be
performed to evaluate for possible associated structural mal-
formations. Because of the strong association between NIHF
and cardiac malformations, fetal echocardiography should
also be performed, with particular attention paid to possible
fetal rhythm disturbances. Fetal heart rate monitoring for 12
to 24 hours should be considered to rule out an underlying
fetal arrhythmia.
Invasive testing for fetal karyotype should also be of-
fered. This can be performed by means of amniocentesis,
with the use of uorescence in situ hybridization or poly-
merase chain reaction (PCR), to facilitate a rapid diagnosis
of the common aneuploidies. While previously the preferred
invasive diagnostic procedure at late gestational age was a fe-
tal percutaneous umbilical blood sample (PUBS), this is now
rarely necessary. The previously cited advantage of a PUBS
was that it allowed rapid determination of fetal karyotype,
in addition to providing details of fetal hematocrit, platelet
count, liver-function tests, hemoglobin electrophoresis, and
fetal IgM specic to the various infectious causes of NIHF. In
addition, it alsoallowedthe opportunity totransfuse the fetus
with packed red cells immediately if profound fetal anemia is
diagnosed. However, given the precision of middle cerebral
artery Doppler evaluation in predicting the anemic fetus, and
the easy availability of either FISH or PCR for rapid chromo-
some evaluation, the need for PUBS continues to decrease.
Amniotic uid (obtained either at the same time as a PUBS
or by amniocentesis) may be sent for -fetoprotein level (in-
creased in Finnish nephrosis and sacrococcygeal teratoma),
and polymerase chain reaction (PCR) testing for infectious
agents. Metabolic testing can also be performed on amniotic
uid when a specic disorder is suspected.
Following the laboratory and sonographic evaluation,
a diagnosis of idiopathic NIHF, or NIHF secondary to a spe-
cic condition, will be made. If a specic underlying cause
has beendiscovered, patient counseling will be dictatedby the
particular abnormality. In general, the presence of hydrops
together with a structural malformation, such as cardiac ab-
normality, is associated with a very poor prognosis. Coun-
seling should therefore also include the possibility of elective
pregnancy termination, depending on the gestational age at
presentation. Patients should be counseled by a multidisci-
plinary teamthat includes a perinatologist, neonatologist, ge-
neticist, and appropriate additional pediatric subspecialists.
Options for fetal interventiontotreat the underlying problem
and hydrops are available and are discussed in the following
section.
If expectant management of the fetus withNIHFis cho-
sen, careful sonographic surveillance should be performed,
including biophysical proles on a frequent basis. The pre-
cise timing of fetal surveillance testing is uncertain and de-
pends on the gestational age at presentation. If the fetus is
considered viable, it may be reasonable to admit the mother
and perform daily fetal testing with nonstress tests or bio-
physical proles. If the hydrops appears to be resolving it
would be reasonable to decrease the frequency of testing,
provided that fetal testing has been reassuring to date. Re-
peat sonographic fetal anatomy surveys should be performed
every 2 weeks to conrm appropriate fetal growth and to im-
prove the chances of detecting an underlying structural fetal
malformation.
Timing of delivery is also uncertain with NIHF. De-
pending on the gestational age, premature delivery may be
indicated if fetal testing becomes nonreassuring. Otherwise,
the most common practice is to continue expectant manage-
ment until 37 weeks of gestation, or until fetal lung matu-
rity has been conrmed by amniocentesis. It is possible that
mature lecithin:sphingomyelin ratios may be less frequently
obtained near term in the fetus with hydrops, as compared
with nonhydropic fetuses (Romero et al., 1988). Expectant
management may also be complicated by preeclampsia in up
to 50% of cases, and this may necessitate immediate preterm
delivery. Inaddition, NIHFis frequently associatedwithpoly-
hydramnios, whichmayprecipitate pretermlabor due touter-
ine overdistention. Periodic reduction amniocenteses may be
required to relieve maternal discomfort from severe polyhy-
dramnios.
The optimal mode of delivery for the hydropic fetus is
uncertain, as the overall prognosis is poor regardless of the
form of delivery. Because of the risk of soft-tissue dystocia
associated with hydrops, it is generally advisable to deliver all
potentially viable fetuses with NIHF by cesarean. This should
minimize the chances of maternal and fetal trauma. In cases
in which the fetus is not expected to survive, the option of
therapeuticthoracentesis or paracentesis tooptimizeavaginal
delivery is also reasonable. However, patients should be made
aware of the extremely small likelihood of neonatal survival.
The optimal location of delivery should be a tertiary care cen-
ter, with the immediate availability of skilled neonatologists
and other appropriate pediatric subspecialists.
FETAL INTERVENTION
The option of fetal therapy in select cases of NIHF is possible.
The underlying pathology that is most amenable to prena-
tal therapy is fetal anemia. When a diagnostic PUBS is un-
dertaken for any fetus with NIHF, immediate transfusion of
packedredcells, if profoundfetal anemia is diagnosed, should
be performed. Arrangements prior to performance of a PUBS
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in such cases should include preparation of group O, irra-
diated, packed red blood cells that are anticytomegalovirus
negative, Rh
o
(D) negative, Kell negative, and cross-matched
compatible with maternal serum. The unit of red cells should
be tightly packed with a hematocrit of at least 80%, and a
blood transfusion setup should be primed as the diagnostic
PUBS is performed. This will ensure that prompt intravascu-
lar fetal transfusion can be performed as soon as signicant
fetal anemia is diagnosed, and prior to withdrawal of the nee-
dle from the umbilical vein. Once a fetal hematocrit of less
than 30% is detected, fetal intravascular transfusion should
begin. Fetal paralysis with pancuronium or vecuronium is
generally not required in cases of hydrops, as fetal activity is
often minimal.
The volume of packed red cells to be transfused is de-
rivedusing formulas that take intoaccount the starting hema-
tocrit, hematocrit of transfused blood, target hematocrit, and
a correction factor for the volume of blood in the placental
circulation. A commonly used formula for volume of blood
to be transfused is
V
T
=
(desired nal hematocrit initial fetal hematocrit)
(donor blood hematocrit)
(150) (EFW)
where V
T
is volume of blood transfused, 150 is a placental
correction factor, and EFW is the estimated fetal weight in
kilograms (Kaufman and Paidas, 1994). It is generally not
advisable to transfuse a hydropic fetus to a nal hematocrit
that is either greater than 25% or greater than four times
the initial hematocrit (Radunovic et al., 1992). This has been
associated with uid overload and sudden intrauterine fetal
death. In cases of NIHF secondary to fetal anemia, the goal
of the rst intrauterine transfusion should be a hematocrit
of 20% to 25%, and the transfusion should then be repeated
in 48 to 72 hours to bring the nal hematocrit to a level of
40% to 45%. For many fetuses with NIHF and anemia sec-
ondary to parvovirus B19 infection, this transfusion may be
all that is needed to maintaina normal fetal hematocrit, as the
initial insult to the fetal marrowis generally self-limiting. An-
other therapeutic option for fetuses with NIHF and anemia
is to perform a combined intravascular and intraperitoneal
transfusion, with the intravascular aliquot of blood designed
to provide an acute increase in fetal hematocrit and the in-
traperitoneal aliquot designed to provide a slower sustained
increase in hematocrit. To date there are no adequate series
comparing these different approaches for the treatment of
NIHF secondary to fetal anemia.
Other forms of fetal intervention for NIHF include
medical therapy for the mother to correct fetal arrhythmias
(see Chapters 42 and 54). Digoxin administration to preg-
nant women has been successful in the treatment of fetal ar-
rhythmias, withresolutionof hydrops insome cases (Knilans,
1995).
Surgical treatment for the fetus withNIHFis alsopossi-
ble. Fetal thoracentesis may be performed under sonographic
guidance with resolution of pleural effusions (Jones, 1995).
If repeat thoracenteses are needed, consideration should be
given to placement of a thoracoamniotic shunt. Such proce-
dures are likelytobe benecial onlyfor cases of NIHFinwhich
pleural effusions are secondary to intrinsic thoracic malfor-
mations. Other surgical procedures that canbe consideredfor
fetuses with NIHF and underlying structural malformations
include open fetal surgical resection of thoracic masses or
sacrococcygeal teratoma (Bullard and Harrison, 1995). How-
ever, few data are available conrming whether such invasive
approaches have a signicant impact onfetal or neonatal out-
come.
TREATMENT OF THE NEWBORN
Infants withhydrops shouldbe deliveredina tertiarycare cen-
ter withimmediate availabilityof amultidisciplinaryneonatal
resuscitation team. Immediate neonatal endotracheal intu-
bation will almost certainly be needed, and such intubations
can be technically difcult (Carlton et al., 1989). A high-
frequency ventilator and high airway-pressure settings may
be needed to achieve adequate gas exchange. Paracentesis and
thoracentesis, with placement of bilateral chest tubes, may
also be needed to allow adequate ventilation and effective gas
exchange. Placement of umbilical artery and umbilical vein
lines may be needed to aid in the resuscitation (McMahan
and Donovan, 1995). Use of blood products, albumin, and
diuretics may be needed to effectively maintain adequate
intravascular volume without signicant uid overload or
soft-tissue edema.
If the neonate can be stabilized in the delivery room,
transport to a neonatal intensive care unit should be arranged
promptly. This should be followed by a thorough physical ex-
amination, with the aid of appropriate radiologic investiga-
tions andechocardiography, toconrmabsence of signicant
structural malformation. If additional structural malforma-
tions are detected, specic therapy should be tailored to the
individual abnormalities. Appropriate pediatric subspecialist
consultation, including a clinical geneticist, should also be
arranged.
SURGICAL TREATMENT
Possible surgical treatments for fetal hydrops include fetal
thoracoamniotic shunt placement for cases of NIHF asso-
ciated with thoracic masses or persistent pleural effusions.
Open fetal surgical resection of thoracic masses, such as con-
genital cystic adenomatoid malformation (see Chapter 35)
and bronchopulmonary sequestration (see Chapter 34), are
also possible (Bullard and Harrison, 1995). Open surgical
resection of fetal sacrococcygeal teratoma with associated hy-
drops is also possible, although there are so few cases that
it is currently difcult to evaluate the role of such invasive
procedures (see Chapter 115).
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Chapter 128 Nonimmune Hydrops Fetalis
LONG-TERMOUTCOME
The prognosis for infants with hydrops depends entirely on
the nature of any underlying abnormalities. The perinatal
mortality rate ranges from 40% to 90%, depending on the
cause, but may approach 100% in cases of NIHF associated
with structural cardiac malformations (Romero et al., 1988;
Wilkins, 1999; Jones, 1995). No long-term studies are avail-
able for counseling parents onthe likely survival or morbidity
if a successful neonatal resuscitation is achieved.
GENETICS AND RECURRENCE RISK
A postmortem examination is indicated in all cases of NIHF
that result in fetal or neonatal death. This will maximize the
number of cases in which a denite underlying cause is iden-
tied and will facilitate appropriate genetic counseling and
prediction of recurrence risk (Steiner, 1995). Recurrence of
idiopathic NIHF is rare, although case series of recurrences
have been documented (Wilkins, 1999). In one case series,
one mother had three consecutive pregnancies complicated
by recurrent idiopathic NIHF and another had two consec-
utive pregnancies complicated by recurrent idiopathic NIHF
(Onwude et al., 1992). Patients should therefore be made
aware that, while idiopathic NIHF is extremely rare, recur-
rences can and do occur.
References
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P1: OVY/SCF P2: OVY
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