Chapter 5. Implantation and Placental Development. Will Obs

 Chapter 5. Implantation and Placental Development

BS. Nguyễn Hồng Anh

All obstetricians should understand the basic biological steps

required or women to achieve pregnancy. Moreover, abnormalities aecting these steps can lead to inertility or pregnancy

loss. Te biological and molecular changes involved in human

zygote implantation and subsequent etal and placental development are intricate. In the past 50 years, researchers have

delineated many o these molecular and physiological events.

Yet, much work remains in the continual challenge to improve

clinical outcomes.

OVARIAN-ENDOMETRIAL CYCLE

In most women, cyclical ovulation continues during the almost

40 years between menarche and menopause. Tus, without

contraception, approximately 400 opportunities or pregnancy exist, and these are tightly regulated by complex interactions o

the hypothalamic-pituitary-ovarian axis. Concurrently, endometrium undergoes aithully reproduced cyclical changes to

prepare or pregnancy (Fig. 5-1). Essential contributors in this

process include gonadotropin-releasing hormone (GnRH), the

gonadotropin hormones ollicle-stimulating hormone (FSH)

and luteinizing hormone (LH), and the ovarian sex steroid hormones estrogen and progesterone. For a detailed description o

menstrual cycle physiology, the reader is reerred to Chapter 16

in Williams Gynecology, 4th edition (Halvorson, 2020).

■ Ovulation

Tis dening event separates the ollicular and luteal phases o

the menstrual cycle. Following ovulation, the corpus luteum

develops rom the remains o the graaan ollicle in a process

reerred to as luteinization. Te basement membrane separating

the granulosa-lutein and theca-lutein cells breaks down, and

by day 2 postovulation, blood vessels and capillaries invade the

granulosa cell layer. During luteinization, these cells undergo

hypertrophy and increase their capacity to synthesize hormones. LH is the primary luteotropic actor responsible or

corpus luteum maintenance (Vande Wiele, 1970).

Te hormone secretion pattern o the corpus luteum diers

rom that o the ollicle. First, the greater capacity o granulosa-lutein cells to produce progesterone results rom enhanced

access to blood-borne, low-density lipoprotein (LDL)-derived

cholesterol, which is a steroidogenic precursor (Carr, 1981).

Ovarian progesterone production peaks at 25 to 50 mg/d during the midluteal phase. With pregnancy, the corpus luteum

continues progesterone production in response to placental

human chorionic gonadotropin (hCG). LH and hCG both act

via the same LH-hCG receptor.

Te human corpus luteum is a transient endocrine organ. In

the absence o pregnancy, it rapidly undergoes apoptosis 9 to

11 days ater ovulation (Vaskivuo, 2002). Te dramatic drop in 

FIGURE 5-1 Gonadotropin control of the ovarian and endometrial cycles. The ovarian-endometrial cycle is structured as a 28-day cycle.

The follicular phase (days 1 to 14) is characterized by rising estrogen levels, endometrial thickening, and selection of the dominant “ovulatory” follicle. During the luteal phase (days 14 to 21), the corpus luteum (CL) produces estrogen and progesterone, which prepare the endometrium for implantation. If implantation occurs, the developing blastocyst begins to produce human chorionic gonadotropin (hCG) and

rescues the corpus luteum, thus maintaining progesterone production. FSH = follicle-stimulating hormone; LH = luteinizing hormone.

circulating estradiol and progesterone levels initiate molecular

events that lead to menstruation.

Between days 22 and 25 ater ovulation, the secretory-phase

endometrium undergoes striking changes associated with predecidual transormation o the upper two thirds o the unctionalis layer. Te glands exhibit extensive coiling, and luminal

secretions become visible. Changes within the endometrium

can also mark the window o implantation seen on days 20 to

24. Epithelial surace cells show ewer microvilli and cilia, but

luminal protrusions appear on the apical cell surace (Nikas,

2003). Tese pinopodes help prepare or blastocyst implantation. Tey also coincide with changes in the surace glycocalyx

that allow acceptance o a blastocyst (Aplin, 2003).

Another highlight o the secretory phase is the continuing

growth and development o the spiral arteries. Tese vessels arise

rom the radial arteries, which are myometrial branches o the

arcuate and, ultimately, uterine vessels. Te morphological and

unctional properties o the spiral arteries are unique and essential to blood ow changes seen during menstruation or implantation. During endometrial growth, spiral arteries lengthen at84 Placentation, Embryogenesis, and Fetal Development

Section 3

a rate appreciably greater than the rate o endometrial tissue

thickening. Tis growth discordance obliges even greater coiling. Spiral artery development reects a marked induction

o angiogenesis, reected by widespread vessel sprouting and

extension.

At this juncture with hormonal withdrawal, menstruation

ollows. With blastocyst implantation, however, the endometrium is converted to the decidua.

DECIDUA

Tis is a specialized, highly modied endometrium o pregnancy. It is essential or hemochorial placentation, that is, one

in which maternal blood contacts trophoblast. Tis relationship requires trophoblast invasion, and considerable research

has ocused on the interaction between decidual cells and

invading trophoblasts. Decidualization transorms prolierating

endometrial stromal cells into specialized secretory cells. Tis

process depends on estrogen, progesterone, androgens, and actors secreted by the implanting blastocyst (Gibson, 2016). Te

decidua produces actors that regulate endometrial receptivity

and that modulate immune and vascular cell unctions within

the maternal–etal microenvironment. Te special immunomodulatory relationship between the decidua and invading

trophoblasts is largely mediated by decidual natural killer (NK)

cells and ensures success o the pregnancy semiallograt.

■ Decidual Structure

Te decidua is classied into three parts based on anatomical

location. Decidua directly beneath the implanted blastocyst

is modied by trophoblast invasion and becomes the decidua

basalis. Te decidua capsularis overlies the enlarging blastocyst

and initially separates the conceptus rom the rest o the uterine

cavity (Fig. 5-2). Tis portion is most prominent during the

second month o pregnancy and consists o stromal decidual

cells covered by a single layer o attened epithelial cells. Te

other side o the capsularis contacts the avascular, extraembryonic etal membrane—the chorion laeve. Te remainder o the

uterus is lined by decidua parietalis. During early pregnancy, a

space lies between the decidua capsularis and parietalis because

the gestational sac does not ll the entire uterine cavity. Te

gestational sac is the extraembryonic coelom and also called the

chorionic cavity. By 14 to 16 weeks’ gestation, the expanding sac has enlarged to completely ll the uterine cavity. Te

resulting apposition o the decidua capsularis and parietalis

creates the decidua vera, and the uterine cavity is unctionally

obliterated.

In early pregnancy, the decidua begins to thicken, eventually attaining a depth o 5 to 10 mm. With magnication, urrows and numerous small openings, representing the mouths o

uterine glands, can be detected. Later in pregnancy, the decidua

becomes thinner, presumably because o pressure exerted by the

expanding uterine contents.

Te decidua parietalis and basalis are composed o three

layers. Tere is a surace or compact zone—zona compacta; a

middle portion or spongy zone—zona spongiosa—that has remnants o glands and numerous small blood vessels; and a basal

zone—zona basalis. Te zona compacta and spongiosa together

orm the zona unctionalis. Te basal zone remains ater delivery

and gives rise to new endometrium.

Te decidual reaction is completed only with blastocyst

implantation. Predecidual changes, however, commence rst

during the midluteal phase in endometrial stromal cells adjacent to the spiral arteries and arterioles. Tereater, these alterations spread in waves throughout the uterine endometrium.

Te endometrial stromal cells enlarge to orm polygonal or

round decidual cells. Te nuclei become vesicular, and the cytoplasm becomes clear, slightly basophilic, and surrounded by a

translucent membrane.

As the embryo–etus grows, the blood supply to the decidua

capsularis is lost. However, spiral arteries persist to supply the

decidua parietalis. Tese arteries retain smooth muscle and

endothelium and thereby remain responsive to vasoactive

agents.

In contrast, the spiral arteries that supply the decidua basalis and ultimately the placental intervillous space are altered

remarkably. rophoblastic cells invade the spiral arterioles

and arteries and replace their endothelial cells. Te vessel wall

smooth muscle is destroyed, and the resulting uteroplacental

vessels become unresponsive to vasoactive agents. Deective trophoblastic invasion o spiral arteries is thought to be one underlying cause o preeclampsia (Chap. 40, p. 692). Conversely,

the etal chorionic vessels, which transport blood between the

placenta and the etus, contain smooth muscle and thus do

respond to vasoactive agents.

■ Decidual Histology

Early in pregnancy, the decidual zona spongiosa consists o

large distended glands, oten exhibiting marked hyperplasia

and separated by minimal stroma. At rst, the glands are lined

by typical cylindrical uterine epithelium with abundant secretory activity that contributes to blastocyst nourishment. With

advanced pregnancy, the glandular elements largely disappear,

and the spongy zone o the decidua basalis consists mainly o

arteries and widely dilated veins.

Cervical

canal

Decidua basalis

Chorionic

cavity

Uterine cavity

Decidua

capsularis

Decidua

parietalis

Chorionic villi

Chorionic

villi

Embryo

Yolk sac

Amnion

FIGURE 5-2 Three portions of the decidua—the basalis, capsularis, and parietalis—are illustrated.Implantation and Placental Development 85

CHAPTER 5

Te decidua basalis contributes to ormation o the placental

basal plate (Fig. 5-3). As such, it is invaded by many interstitial trophoblasts. Te Nitabuch layer is a zone o brinoid

degeneration in which invading trophoblasts meet the decidua

basalis. I the decidua is deective, as in placenta accreta, the

Nitabuch layer is usually absent (Chap. 43, p. 759). Normally,

there is also a more supercial, but inconsistent, deposition o

brin—Rohr stria—at the bottom o the intervillous space and

surrounding the anchoring villi. Decidual necrosis is a common phenomenon in the rst and probably second trimesters

(McCombs, 1964). Tus, necrotic decidua obtained through

curettage ater spontaneous abortion should not necessarily be

interpreted as either a cause or an eect o the pregnancy loss.

Both decidual layers contain numerous cell groups whose

composition varies with gestational stage (Loke, 1995). Te

primary cellular components are the true decidual cells, which

dierentiated rom the endometrial stromal cells, and numerous maternal bone marrow-derived cells. O the latter, lymphocytes with unique properties accumulate at the maternal–etal

interace and are essential to evoke immune tolerance between

mother and etus. Tese include regulatory  cells, decidual

macrophages, and decidual NK cells. Collectively, these cells

not only provide immunotolerance but also play an important

role in trophoblast invasion and vasculogenesis (PrabhuDas,

2015).

■ Decidual Prolactin

Te decidua produces prolactin, which is present in enormous

amounts in amnionic uid (Golander, 1978; Riddick, 1979).

Decidual prolactin is a product o the same gene that encodes

or anterior pituitary prolactin, but the exact physiological role

o decidual prolactin is unknown. Signaling by prolactin can

lead to the production o proangiogenic actors. Prolactin can

also be cleaved by proteases to orm vasoinhibins (Nakajima,

2015; riebel, 2015). Tese compounds have antiangiogenic

properties and may contribute to peripartum cardiomyopathy,

whose pathogenesis and treatment are discussed in Chapter 52

(p. 933).

Decidual prolactin preerentially enters amnionic uid and

may reach extraordinarily high levels o 10,000 ng/mL at 20 to

24 weeks’ gestation (yson, 1972). In contrast, maternal serum

levels are relatively low at 150 to 200 ng/mL. As discussed in

Chapter 4 (p. 57), prolactin inhibits maternal insulin action

and results in increased glucose levels or etal growth.

IMPLANTATION AND EARLY TROPHOBLAST

FORMATION

■ Fertilization

With ovulation, the ovary releases the secondary oocyte and

surrounding cells o the cumulus–oocyte complex. Te oocyte

complex is quickly enguled by the allopian tube inundibulum. Directional movement o cilia and tubal peristalsis moves

the ovum within the tubal lumen. Fertilization takes place in

the lumen within a ew hours, and no more than a day ater

ovulation. Because o this narrow window, spermatozoa must

be present in the allopian tube at the time o oocyte arrival.

Almost all pregnancies result when intercourse occurs during

the 2 days preceding or on the day o ovulation.

Fertilization is highly complex. Molecular mechanisms allow

spermatozoa to pass between cumulus cells; through the zona

pellucida, which is a thick glycoprotein layer surrounding the

oocyte cell membrane; and into the oocyte cytoplasm. Fusion

o the two nuclei and intermingling o maternal and paternal

chromosomes creates the zygote.

Early human development is described by days or weeks

postertilization, that is, postconceptional (Chap. 7, p. 121).

By contrast, in most chapters o this book, clinical pregnancy

dating is calculated rom the rst day o the last menstrual

period (LMP). Tus, 1 week postertilization corresponds to

approximately 3 weeks rom the LMP in women with regular

28-day cycles. Tis convention is clinically important or dating

pregnancies conceived by in vitro ertilization (IVF), in which

the gestational age is 14 days greater than the day o ertilization. As an example, 8 weeks’ gestation reers to 8 completed

weeks ollowing the LMP but corresponds to 6 weeks postertilization.

Ater ertilization, the zygote—a diploid cell with 46 chromosomes—undergoes cleavage, and zygote cells produced by

this division are blastomeres (Fig. 5-4). In the two-cell zygote, the

blastomeres and polar body continue to be surrounded by the

zona pellucida. Te zygote undergoes slow cleavage or 3 days

while still in the allopian tube. As the blastomeres continue

to divide, a solid mulberry-like ball o cells—the morula—is

produced. Te morula enters the uterine cavity approximately

3 days ater ertilization. Gradual accumulation o uid between

the morula cells leads to ormation o the early blastocyst.

FIGURE 5-3 Section through a junction of chorion, villi, and

decidua basalis in early first-trimester pregnancy. (Reproduced with

permission from Dr. Kurt Benirschke.)86 Placentation, Embryogenesis, and Fetal Development

Section 3

Blastocyst

As early as 4 to 5 days ater ertilization, the 58-cell blastocyst

dierentiates into ve embryo-producing cells—the inner cell

mass (see Fig. 5-4). Te remaining 53 outer cells, called the

trophectoderm, are destined to orm trophoblasts (Hertig, 1962).

In the 107-cell blastocyst, the eight ormative, embryoproducing cells are surrounded by 99 trophoblastic cells. Te

blastocyst is released rom the zona pellucida secondary to secretion o specic proteases rom the secretory-phase endometrial

glands (O’Sullivan, 2002). Release rom the zona pellucida

allows blastocyst-produced cytokines and hormones to directly

inuence endometrial receptivity (Lindhard, 2002). Te blastocyst secretes interleukin-1α (IL-1α) and IL-1β, which are cytokines that likely directly inuence the endometrium. Embryos

also secrete hCG, which may inuence endometrial receptivity

(Licht, 2001; Lobo, 2001).

Te receptive endometrium is thought to respond by producing leukemia inhibitory actor (LIF), ollistatin, and colonystimulating actor-1 (CSF-1). LIF and ollistatin activate signaling

pathways that collectively inhibit prolieration and promote di-

erentiation o the endometrial epithelia and stroma to enable

uterine receptivity (Rosario, 2016b). At the maternal–etal inter-

ace, CSF-1 has proposed immunomodulatory and proangiogenic

actions that are required or implantation (Rahmati, 2015).

■ Implantation

Te blastocyst implants into the uterine wall 6 or 7 days ater

ertilization. Tis process can be divided into three phases: (1)

apposition—initial contact o the blastocyst to the uterine wall;

(2) adhesion—increased physical contact between the blastocyst and decidua; and (3) invasion—penetration and invasion

o syncytiotrophoblast and cytotrophoblasts into the decidua,

inner third o the myometrium, and uterine vasculature.

Successul implantation requires a receptive endometrium

appropriately primed with estrogen and progesterone by the

corpus luteum. Such uterine receptivity is limited to days 20

to 24 o the endometrial cycle. Adherence is mediated by cellsurace receptors at the implantation site that interact with blastocyst receptors (Carson, 2002; Lessey, 2002). I the blastocyst

approaches the endometrium ater cycle day 24, the potential

or adhesion declines because antiadhesive glycoprotein synthesis prevents receptor interactions (Navot, 1991). A mismatch

between uterine receptivity and timing o embryo transer can

lead to repeated implantation ailure in some IVF patients. Tis

has stimulated eorts to dene receptivity by gene expression

proles to improve clinical outcomes (Ruiz-Alonso, 2013).

At the time o its interaction with the endometrium, the

blastocyst is composed o 100 to 250 cells. Te blastocyst

trophectoderm loosely adheres to the decidua by apposition.

Tis appears to be closely regulated by paracrine interactions

between these two tissues and most commonly occurs on the

upper posterior uterine wall.

Successul endometrial blastocyst adhesion involves modi-

ed expression o cellular adhesion molecules (CAMs). Te

integrins—one o our amilies o CAMs—are cell-surace

receptors that mediate cell adhesion to extracellular matrix

proteins (Lessey, 2002). Endometrial integrins are hormonally regulated, and a specic set o integrins is expressed at

implantation (Lessey, 1995). Recognition-site blockade o the

integrins needed or binding will prevent blastocyst attachment

(Kaneko, 2013).

■ Trophoblast Development

Te etus depends on the placenta or pulmonary, hepatic, and

renal unctions. Tese are accomplished through the anatomical relationship o the placenta and its uterine interace. In

overview, maternal blood ows rom uteroplacental vessels into

the placental intervillous space and bathes the syncytiotrophoblast, which surround villi. Here, gases, nutrients, and other

substances are exchanged with etal capillary blood within the

core o each placental villus. Tus, etal and maternal blood do

not normally mix in this hemochorial placenta.

Human placental ormation begins with the trophectoderm,

which gives rise to a trophoblast cell layer encircling the blastocyst. rophoblast exhibits the most variable structure, unction, and developmental pattern o all placental components.

Its invasiveness promotes implantation, its nutritional role or

the conceptus is reected in their name, and its endocrine unction is essential to maternal physiological adaptations and to

pregnancy maintenance.

By the eighth day postertilization, ater initial implantation,

the trophoblast has dierentiated into an outer multinucleated

syncytium—the primitive syncytiotrophoblast, and an inner layer

o primitive mononuclear cells—cytotrophoblasts. Te latter are

solely germinal cells or the syncytium. As cytotrophoblasts di-

erentiate, they express an endogenous envelope protein called

syncytin, which aids their cell usion with the expanding outer

layer o syncytiotrophoblast. Each cytotrophoblast has a welldemarcated cell border, a single nucleus, and ability to undergo

DNA synthesis and mitosis (Arnholdt, 1991). Tese are lacking

Polar body Blastomeres

2-cell stage 4-cell stage 8-cell stage

16-cell stage (Morula) Blastocyst Inner cell

mass

Blastocyst

cavity

Trophoblast

FIGURE 5-4 Zygote cleavage and blastocyst formation. The

morula period begins at the 12- to 16-cell stage and ends when

the blastocyst forms, which occurs when there are 50 to 60 blastomeres present. The polar bodies, shown in the 2-cell stage, are

small nonfunctional cells that degenerate.Implantation and Placental Development 87

CHAPTER 5

in the syncytiotrophoblast, which provides transport unctions

o the placenta. It is so named because instead o individual

cells, it has an amorphous cytoplasm without cell borders,

nuclei that are multiple and diverse in size and shape, and a

continuous syncytial lining.

Following implantation, trophoblasts dierentiate along

two main pathways that give rise to either villous or extravillous trophoblasts. As shown in Figure 5-5, both have distinct

unctions (Loke, 1995). Villous trophoblasts generate chorionic

villi, which primarily transport oxygen, nutrients, and other

compounds between the etus and mother. Extravillous trophoblasts migrate into the decidua and myometrium (Fig. 5-6).

Tey also penetrate maternal vasculature and thus directly contact various maternal cell types (Pijnenborg, 1994). Extravillous

trophoblasts are urther classied as interstitial trophoblasts and

endovascular trophoblasts. Te interstitial trophoblasts invade

the decidua and eventually penetrate the myometrium to orm

placental-bed giant cells. Tese trophoblasts also surround spiral arteries. Te endovascular trophoblasts penetrate the spiral

artery lumens (Pijnenborg, 1983).

Recently, single-cell RNA sequencing technology has

enabled inerence o trophoblast dierentiation lineages (Liu,

2018; sang, 2017). Tis has also yielded insights into the communication between trophoblasts and maternal decidual and

immune cell populations (Vento-ormo, 2018). Furthermore,

human trophoblast stem cells, long hypothesized to exist, have

been isolated rom early gestations (Okae, 2018). Culture o

these cells will likely aid greater understanding o early trophoblast dierentiation and invasion (Haider, 2018; urco, 2018).

■ Early Invasion

Ater gentle erosion between epithelial cells o the surace endometrium, invading trophoblasts burrow deeper. At 9 days o

development, the blastocyst wall acing the uterine lumen is a

single layer o attened cells. By the 10th day, the blastocyst

becomes totally encased within the endometrium (Fig. 5-7).

Te blastocyst wall opposite the uterine lumen is thicker and

comprises two zones—the trophoblasts and the embryo-orming

inner cell mass. As early as 7.5 days postertilization, the inner

cell mass or embryonic disc dierentiates into a thick plate o

primitive ectoderm and an underlying layer o endoderm. Some

small cells appear between the embryonic disc and the trophoblasts and enclose a space that will become the amnionic cavity.

Extraembryonic mesenchyme rst appears as groups o isolated cells within the blastocyst cavity, and later this mesoderm

completely lines the cavity. Within this mesoderm, spaces orm

and then use to orm the chorionic cavity, that is, the extraembryonic coelom. Te chorion is composed o trophoblasts

and mesenchyme. Some mesenchymal cells eventually will condense to orm the body stalk. Tis stalk joins the embryo to the

nutrient chorion and later develops into the umbilical cord.

Te body stalk can be recognized at an early stage at the caudal

end o the embryonic disc (Fig. 7-3, p. 123).

Early

pregnancy

End of 1st

trimester

Midgestation 3rd trimester

Myometrium

Decidua

basalis

Interstitial extravillous trophoblast

Endovascular extravillous trophoblast

Anchoring villus

Syncytiotrophoblast

Cytotrophoblast

Extravillous trophoblasts

Endothelium

Spiral artery

FIGURE 5-5 Endovascular and interstitial extravillous trophoblasts are found outside the villus. Endovascular trophoblasts invade and

transform spiral arteries during pregnancy to create low-resistance blood flow that is characteristic of the placenta. Interstitial trophoblasts

invade the decidua and surround spiral arteries.

FIGURE 5-6 Photomicrograph of extravillous trophoblast invasion

of the decidua basalis.88 Placentation, Embryogenesis, and Fetal Development

Section 3

As the embryo enlarges, more maternal decidua basalis is

invaded by syncytiotrophoblast. Beginning approximately

12 days ater conception, the syncytiotrophoblast is permeated

by a system o intercommunicating channels called trophoblastic lacunae. Ater invasion o supercial decidual capillary walls,

lacunae become lled with maternal blood. At the same time,

the decidual reaction, characterized by decidual stromal cell

enlargement and glycogen storage, intensies (p. 84).

■ Chorionic Villi

With deeper invasion into the decidua, solid primary villi arise

rom buds o cytotrophoblasts that protrude into the primitive syncytium beore 12 days postertilization. Primary villi are

composed o a cytotrophoblast core covered by syncytiotrophoblast. As the lacunae merge, a complicated labyrinth is ormed

that is partitioned by these solid cytotrophoblastic columns.

Te trophoblast-lined channels orm the intervillous space,

and the solid cellular columns orm the primary villous stalks.

Beginning on the 12th day ater ertilization, mesenchymal

cords derived rom extraembryonic mesoderm invade the solid

trophoblast columns. Tese then orm secondary villi. Once

angiogenesis begins in the mesenchymal cords, tertiary villi are

created. Although maternal venous sinuses are tapped early in

implantation, maternal arterial blood does not enter the intervillous space until around day 15. By the 17th day, however,

etal blood vessels are unctional, and a placental circulation is

established. Te etal–placental circulation is completed when

the blood vessels o the embryo are connected with chorionic

vessels. In some villi, angiogenesis ails rom lack o circulation.

Te most striking exaggeration o this is seen with hydatidiorm

mole (Fig. 13-1, p. 236).

Villi are covered by an outer layer o syncytiotrophoblast and an inner layer o cytotrophoblasts. Prolieration o

cytotrophoblast at the villous tips produces the trophoblastic

cell columns that orm anchoring villi. Tey are not invaded by

etal mesenchyme, and they are anchored to the decidua at the

basal plate, which is the maternal side o the intervillous space.

Te chorionic plate orms the roo o the intervillous space. It

consists o the two layers o trophoblasts, which line the intervillous space, and brous mesoderm on the opposite side. Te

nal chorionic plate is ormed by 8 to 10 weeks as the amnionic

and primary chorionic plate mesenchyme use together. Tis

ormation is accomplished by expansion o the amnionic sac.

Early electron microscopic studies demonstrate prominent

microvilli on the syncytial surace (Fig. 5-8) (Wislocki, 1955).

Syncytiotrophoblast

Endometrial gland Spiral artery

Lacunar network

Amnion

Cytotrophoblast

Extraembryonic

mesoderm

Primitive

A yolk sac B

Eroded gland

Maternal blood

Lacunar network

Amnionic

cavity

Extraembryonic

endoderm

Cytotrophoblast

Embryonic disc

FIGURE 5-7 Drawing of sections through implanted blastocysts. A. At 10 days. B. At 12 days after fertilization. This stage is characterized

by the intercommunication of the lacunae filled with maternal blood. Note in (B) that large cavities have appeared in the extraembryonic

mesoderm, forming the beginning of the extraembryonic coelom. Also, extraembryonic endodermal cells have begun to form on the inside

of the primitive yolk sac. (Redrawn from Moore KL, Persaud, TV, Torchia, MG (eds): The Developing Human. Clinically Oriented Embryology,

9th ed. Philadelphia, PA: Saunders; 2013.)

FIGURE 5-8 Electron micrograph of term human placenta villus.

A villus capillary filled with fetal red blood cells (asterisks) is seen in

close proximity to the microvilli border. (Reproduced with permission from Boyd JD, Hamilton WJ: The Human Placenta. Cambridge,

Heffer, 1970.)Implantation and Placental Development 89

CHAPTER 5

Associated pinocytotic vacuoles and vesicles are involved in

both absorptive and secretory placental unctions. Microvilli increase trophoblast surace area in direct contact with

maternal blood, the dening characteristic o a hemochorial

placenta.

PLACENTA AND CHORION

■ Chorion Development

In early pregnancy, the villi are distributed over the entire

periphery o the chorionic membrane (Fig. 5-9). As the blastocyst with its surrounding trophoblasts grows and expands into

the decidua, one pole aces the endometrial cavity. Te opposite pole will orm the placenta. Here, chorionic villi in contact

with the decidua basalis prolierate to orm the chorion rondosum—or leay chorion. As growth o embryonic and extraembryonic tissues continues, the blood supply to the chorion

acing the endometrial cavity is restricted. Because o this, villi

A B

FIGURE 5-9 Complete abortion specimens. A. Initially, the entire

chorionic sac is covered with villi, and the embryo within is not

visible B. Stretch and pressure from further growth prompt partial

regression of the villi. The remaining villi form the future placenta,

whereas the smooth portion is the chorion.

in contact with the decidua capsularis cease to grow and then

degenerate. Tis portion o the chorion becomes the avascular

etal membrane that abuts the decidua parietalis and is called

the chorion laeve—or smooth chorion. Tis smooth chorion is

composed o cytotrophoblasts and etal mesodermal mesenchyme. A paracrine system between the decidua and chorion

also links mother and etus. Tis is an extraordinarily important

arrangement or maternal–etal communication and or maternal immunological acceptance o the conceptus (GuzelogluKayisli, 2009).

Until near the end o the third month, the chorion laeve is

separated rom the amnion by the exocoelomic cavity. Terea-

ter, they are in intimate contact to orm the avascular amniochorion. Tese two structures are important sites o molecular

transer and metabolic activity. Moreover, they constitute an

important paracrine arm o the etal–maternal communication

system.

■ Regulators of Trophoblast Invasion

Implantation and endometrial decidualization activate a unique

population o maternal immune cells that play critical unctions

in trophoblast invasion, angiogenesis, spiral artery remodeling,

and maternal tolerance to etal alloantigens. Decidual natural

killer cells (dNK) make up 70 percent o decidual leukocytes in

the rst trimester and directly contact trophoblasts. In contrast to

NK cells in peripheral blood, dNK cells lack cytotoxic unctions. Tey produce specic cytokines and angiogenic actors

to regulate trophoblast invasion and spiral artery remodeling

(Hanna, 2006). dNK cells promote phagocytosis o cell debris

(Faas, 2017). Tese and other unique properties distinguish

dNK cells rom circulating NK cells and rom NK cells in the

endometrium beore pregnancy (Fu, 2013; Winger, 2013).

dNK cells express both IL-8 and intereron-inducible protein

10, which bind to receptors on invasive trophoblastic cells to

promote their decidual invasion toward the spiral arteries. dNK

cells also produce proangiogenic actors, including VEGF and

placental growth actor (PlGF), which both promote vascular

growth in the decidua.

rophoblasts also secrete specic chemokines that attract

the dNK cells to the maternal–etal interace. Tus, both cell

types simultaneously attract each other. Decidual macrophages

account or approximately 20 percent o leukocytes in the

rst trimester and elicit an M2-immunomodulatory phenotype (Williams, 2009). Recall that while M1 macrophages are

proinammatory, M2 macrophages counter proinammatory

responses and promote tissue repair.

Concurrently,  cell subsets aid tolerance toward the allogenic etus. Regulatory  cells (regs) are essential or promoting immune tolerance. Other  cell subsets are present, such

as T1, T2, and T17, although their unctions are tightly

regulated (Ruocco, 2014).

Endometrial Invasion

Extravillous trophoblasts o the rst-trimester placenta are

highly invasive. Tis process occurs under low-oxygen conditions, and regulatory actors that are induced under hypoxic conditions are contributory (Soares, 2012). Invasive trophoblasts90 Placentation, Embryogenesis, and Fetal Development

Section 3

secrete numerous proteolytic enzymes that digest extracellular

matrix and activate proteinases already present in the decidua.

rophoblasts produce urokinase-type plasminogen activator,

which converts plasminogen into the broadly acting serine protease, plasmin. Tis in turn both degrades matrix proteins and

activates MMPs. One member o the MMP amily, MMP-9,

appears to be critical. Te timing and extent o trophoblast

invasion is regulated by a balanced interplay between pro- and

anti-invasive actors.

Low estradiol levels in the rst trimester are critical or trophoblast invasion and spiral artery remodeling. Animal studies

suggest that the rise in second-trimester estradiol levels suppresses and limits vessel remodeling by reducing trophoblast

expression o VEGF and specic integrin receptors (Bonagura,

2012). Namely, extravillous trophoblasts express integrin receptors that recognize the extracellular matrix proteins collagen IV,

laminin, and bronectin. Binding o these matrix proteins and

integrin receptors initiates signals to promote trophoblast cell

migration and dierentiation. However, as pregnancy advances,

rising estradiol levels downregulate VEGF and integrin receptor expression. Tis represses and controls the extent o uterine

vessel transormation.

■ Spiral Artery Invasion

One o the most remarkable eatures o human placental development is the extensive modication o maternal vasculature by

trophoblasts, which are etal in origin. Tese events occur in the

rst hal o pregnancy and are essential to uteroplacental blood

ow. Tey are also integral to conditions such as preeclampsia, etal-growth restriction, and preterm birth. Spiral artery

modications are carried out by two populations o extravillous trophoblasts—endovascular trophoblasts, which penetrate

the spiral-artery lumen, and interstitial trophoblasts, which surround the arteries (see Fig. 5-5).

Interstitial trophoblasts constitute a major portion o the

placental bed. Tey penetrate the decidua and adjacent myometrium and aggregate around spiral arteries, where they may

aid endovascular trophoblast invasion.

Endovascular trophoblasts rst enter the spiral artery lumens

and initially orm cellular plugs. Tey then promote apoptosis

o the native endothelium and replace the vessel lumen with

cells o etal origin. Fibrinoid material replaces smooth muscle

and connective tissue o the vessel media. Tis decreases resistance o blood ow into the intervillous space. Invading endovascular trophoblasts can extend several centimeters along the

vessel lumen, and they must migrate against arterial ow. O

note, invasion by trophoblasts involves only the decidual spiral

arteries and not decidual veins.

Uteroplacental vessel development proceeds in two waves

or stages (Ramsey, 1980). First, beore 12 weeks’ postertilization, spiral arteries are invaded and modied up to the

border between the decidua and myometrium. Te second

wave, between 12 and 16 weeks, involves some invasion o

the intramyometrial segments o spiral arteries. Remodeling

converts narrow-lumen, muscular spiral arteries into dilated,

low-resistance uteroplacental vessels. Molecular mechanisms o

these crucial events and their signicance or preeclampsia and

etal-growth restriction have been reviewed (Pereira de Sousa,

2017; Xie, 2016).

Although early placenta development occurs in a lowoxygen-tension environment (2 to 3 percent O2), this vascular

remodeling increases blood ow and oxygenation such that the

oxygen concentration more than doubles around the end o the

rst trimester (Chang, 2018).

■ Villus Branching

Although certain villi o the chorion rondosum extend rom

the chorionic plate to the decidua to serve as anchoring villi,

most villi arborize and end reely within the intervillous space.

As gestation proceeds, the short, thick, early stem villi branch

to orm progressively ner subdivisions and greater numbers o increasingly smaller villi (Fig. 5-10). Tis increasing

villous surace area correlates with gestational age and aids

etal growth. In cases o restricted blood ow, more highly

branched villi than expected or gestational age is a compensatory mechanism.

Each truncal or main stem villus and its ramications constitutes a placental lobule or cotyledon. Each lobule has a single

chorionic artery and vein, such that lobules constitute the unctional units o placental architecture.

■ Placental Growth and Maturation

In the rst trimester, placental growth is more rapid than

that o the etus. However, by 17 weeks’ gestation, placental

and etal weights are nearly equal. By term, placental weight

approximates one sixth o etal weight.

Te mature placenta and its variant orms are discussed in

detail in Chapter 6 (p. 108). Briey, viewed rom the maternal surace, which attaches to the uterine wall, the number o

slightly elevated convex areas, called lobes, varies rom 10 to

38. Lobes are incompletely separated by grooves o variable

depth. Tese tissue grooves orm in response to placental septa,

which rise up as projections o decidua. Te total number o

placental lobes remains the same throughout gestation, and

individual lobes continue to grow—although less actively in

late pregnancy (Craword, 1959). Although grossly visible lobes

are commonly reerred to as cotyledons, this is inaccurate. Correctly used, lobules or cotyledons are the unctional units supplied by each main stem villus.

As villi continue to branch and the terminal ramications

become more numerous and smaller, the volume o cytotrophoblasts decline. Tis layer attenuates, and by 16 weeks’ gestation, the apparent continuity o the cytotrophoblasts is lost. As

the syncytium also thins, the etal vessels become more prominent within the villus and lie closer to the intervillous space

(see Fig. 5-8). At term, villi may be ocally reduced to a thin

layer o syncytium covering minimal villous connective tissue

in which thin-walled etal capillaries abut the trophoblast and

dominate the villi.

Te villous stroma also exhibits changes as gestation progresses. In early pregnancy, the branching connective-tissueImplantation and Placental Development 91

CHAPTER 5

cells are separated by an abundant loose intercellular matrix.

Later, the villous stroma becomes denser, and the cells are more

spindly and closely packed. Another stromal change involves

inltration o Hobauer cells, which are etal macrophages.

Tese are round with vesicular, oten eccentric nuclei and with

very granular or vacuolated cytoplasm. Tey grow in number

and maturational state throughout pregnancy and appear to be

important mediators o protection at the maternal–etal inter-

ace (Johnson, 2012). Tese macrophages are phagocytic, have

an immunosuppressive phenotype, produce various cytokines,

and provide paracrine regulation o trophoblastic unctions

(Cervar, 1999; Reyes, 2017).

Some architectural changes, i substantive, can lower placental exchange efciency. Tese include thickening o the basal

lamina o trophoblast or capillaries, obliteration o certain etal

vessels, greater villous stroma, and brin deposition on the villous surace (Chap. 6, p. 109).

■ Placental Circulation

Te gross anatomy o the placenta reects the intimate

approximation o the etal capillary bed to maternal blood.

Te etal surace is covered by the transparent amnion,

beneath which chorionic vessels course. A section through

the placenta includes amnion, chorion, chorionic villi and

intervillous space, decidual (basal) plate, and myometrium

(Figs. 5-11 and 5-12).

A B

C D

E F

FIGURE 5-10 Illustration (A, B), histology (C, D), and electron microscopy (E, F) of early (top panel) and late (bottom panel) human placenta villi. Limited branching of villi is seen in the early placenta. With maturation, increasing villous arborization is seen, and villous capillaries lie closer to the surface of each villus. (Electron micrographs reproduced with permission from King BF, Menton DN: Scanning electron

microscopy of human placental villi from early and late in gestation. Am J Obstet Gynecol 122:824, 1975.)

Myometrium

Decidua vera

Chorion

Amnion

Chorion

Amnion

Decidua

vera

Decidua

basalis

Placenta

FIGURE 5-11 Uterus showing a normal placenta and its membranes in situ.92 Placentation, Embryogenesis, and Fetal Development

Section 3

Fetal Circulation

Deoxygenated venous-like etal blood ows to the placenta

through the two umbilical arteries. As the cord joins the placenta, these umbilical vessels branch repeatedly beneath the

amnion as they run across the chorionic plate. Branching

continues within the villi to ultimately orm capillary networks

in the terminal villous branches. Blood with signicantly higher

oxygen content returns rom the placenta via a single umbilical

vein to the etus.

Te branches o the umbilical vessels that traverse along the

chorionic plate are called placental surace vessels or chorionic

vessels. Tey respond to vasoactive substances, but anatomically, morphologically, histologically, and unctionally, they are

unique. Chorionic arteries always cross over chorionic veins.

Vessels are most readily recognized by this anatomical relationship, but they are difcult to distinguish by histological criteria.

runcal arteries are perorating branches o the surace arteries and pass through the chorionic plate. Each truncal artery

supplies one main stem villus and thus one cotyledon. As the

artery penetrates the chorionic plate, its wall loses smooth muscle, and its caliber increases. Te loss o muscle continues as the

truncal arteries and veins branch into their smaller rami.

Beore 10 weeks’ gestation, end-diastolic ow is not detected

within the umbilical artery at the end o the etal cardiac cycle

(Fisk, 1988; Loquet, 1988). Ater 10 weeks, however, end-diastolic ow appears and is maintained throughout normal pregnancy. Clinically, these ow patterns are studied with Doppler

sonography to assess etal well-being (Chap. 4, p. 262).

Maternal Circulation

Mechanisms o placental blood ow must allow blood to leave

maternal circulation; ow into an amorphous space lined by

syncytiotrophoblast; and return through maternal veins without

producing arteriovenous-like shunts that would prevent adequate

exchange between maternal blood and etal villi. For this, maternal blood enters through the basal

plate and is driven high up toward

the chorionic plate by arterial pressure beore laterally dispersing

(Fig. 5-13). Ater bathing the external microvillous surace, maternal

blood drains back through venous

orices in the basal plate and enters

uterine veins. Tus, maternal blood

traverses the placenta randomly

without preormed channels. rophoblast invasion o the spiral

arteries creates low-resistance vessels that can accommodate massive

increase in uterine perusion during

gestation. Generally, spiral arteries

are perpendicular to, but veins are

parallel to, the uterine wall. Tis

arrangement aids closure o veins

during a uterine contraction and

prevents the exit o maternal blood

rom the intervillous space. Te

number o arterial openings into

the intervillous space is gradually

reduced by cytotrophoblastic invasion to approximately 120 entry

sites at term (Brosens, 1963). Tese

discharge blood in spurts to bath

FIGURE 5-12 Photomicrograph of implantation site with partial

section of an early embryo. Anchoring villi are seen with extravillous trophoblasts invading the decidua basalis. CNS = central

nervous system. (Reproduced with permission from Dr. Kurt

Benirschke.)

Chorionic plate

Umbilical Umbilical artery

vein

Umbilical cord

Spiral

artery

Basal

plate

Chorionic

villus

Decidual septum

FIGURE 5-13 Schematic drawing of a section through a full-term placenta. Maternal blood

flows into the intervillous spaces in funnel-shaped spurts, and umbilical arteries carry deoxygenated fetal blood to the placenta. Exchanges between the maternal and fetal systems occur as

maternal blood flows around the villi. The umbilical vein carries oxygenated blood back to the

fetus. Inflowing arterial blood pushes maternal venous blood into the endometrial veins, which

are scattered over the entire surface of the decidua basalis. Placental lobes are separated from

each other by placental (decidual) septa.Implantation and Placental Development 93

CHAPTER 5

the adjacent villi (Borell, 1958). Ater the 30th week, a prominent venous plexus lies between the decidua basalis and myometrium and helps develop the cleavage plane needed or placental

separation ater delivery.

Both inow and outow are curtailed during uterine contractions. Bleker and associates (1975) used serial sonography during normal labor and ound that placental length,

thickness, and surace area grew during contractions. Tey

attributed this to distention o the intervillous space by

impairment o venous outow compared with arterial inow.

During contractions, thereore, a somewhat larger volume o

blood is available or exchange even though the rate o ow

is decreased. Similarly, Doppler velocimetry has shown that

diastolic ow velocity in spiral arteries is diminished during

uterine contractions. Tus, principal actors regulating intervillous space blood ow are arterial blood pressure, intrauterine pressure, uterine contraction pattern, and actors that act

specically on arterial walls.

■ Breaks in the Placental “Barrier”

Te placenta does not maintain absolute integrity o the etal

and maternal circulations, and cells trafc between mother and

etus in both directions. Tis situation is best exemplied clinically by erythrocyte D-antigen alloimmunization (Chap. 18,

p. 353). Fetal cell transer is small in most cases, although rarely

the etus exsanguinates into the maternal circulation.

Fetal cells can also engrat in the mother during pregnancy

and still be detected decades later. Fetal lymphocytes, mesenchymal stem cells, and endothelial colony-orming cells all reside

in maternal blood, bone marrow, or uterine vasculature (Huu,

2006; Piper, 2007; Sipos, 2013). Tis requent phenomenon,

termed microchimerism, is implicated in the disparate emale:

male ratio o autoimmune disorders (Greer, 2011; Stevens,

2006). As discussed in Chapter 62 (p. 1109), etal cell engratment has been associated with the pathogenesis o lymphocytic

thyroiditis, scleroderma, and systemic lupus erythematosus.

■ MaternalFetal Interface

Tis maternal–etal interace is an active hub o immunological interactions that allows implantation, appropriate placental

development, and immunotolerance o the etus. At the same

time, a unctional immune system must be maintained to protect the mother.

Immunogenicity of the Trophoblasts

rophoblastic cells are the only etus-derived cells in direct

contact with maternal tissues and blood. Fetal syncytiotrophoblast synthesizes and secretes numerous actors that regulate the

immune responses o maternal cells both at the implantation

site and systemically.

Human leukocyte antigens (HLAs) are the human analogue

o the major histocompatibility complex (MHC) (Hunt, 1992).

Tere are 17 HLA class I genes and include three classic genes,

HLA-A, -B, and -C, that encode the major class I (class Ia)

transplantation antigens. Tree other class I genes, designated

HLA-E, -F, and -G, encode class Ib HLA antigens. MHC class

I and II antigens are absent rom villous trophoblasts, which

appear to be immunologically inert at all gestational stages

(Weetman, 1999). Invasive extravillous trophoblasts do express

MHC class I molecules but avoid rejection by the maternal

immune system.

Moett-King (2002) reasoned that normal implantation

depends on controlled trophoblastic invasion o maternal

decidua and spiral arteries. Such invasion must proceed ar

enough to provide or normal etal growth and development

but avoid the pathogenic invasion seen in placenta accreta spectrum disorders (Chap. 43, p. 759). She suggests that dNK cells

combined with extravillous trophoblasts’ unique expression o

three specic HLA class I genes act in concert to permit and

subsequently limit trophoblast invasion.

Extravillous trophoblasts express the class Ia antigen HLA-C

and nonclassic class Ib molecules HLA-E and HLA-G. HLA-G

antigen is expressed only in humans and is restricted to extravillous trophoblasts in contact with maternal tissues. Expressed

in both membrane-bound and soluble isoorms detectable in

maternal circulation, HLA-G is thought to protect extravillous

trophoblasts rom immune rejection by modulating the unction o both decidual and circulating NK populations (Apps,

2011; Rajagopalan, 2012). Te importance o this molecule is

highlighted by the observation that IVF embryos ail to implant

i they do not express a soluble HLA-G isoorm (Fuzzi, 2002).

Tus, HLA-G may act through multiple mechanisms to aid

tolerance o the maternal–etal antigen mismatch (LeBouteiller,

1999). Abnormal HLA-G expression in extravillous trophoblasts rom women with preeclampsia suggests immune dysregulation as one etiology (Goldman-Wohl, 2000).

Decidual Immune Cells

O leukocytes, NK cells predominate in midluteal phase endometrium and in rst-trimester decidua, but numbers decline

by term (Johnson, 1999). In rst-trimester decidua, dNK cells

lie close to extravillous trophoblasts and purportedly regulate

invasion. Teir inltration is increased by progesterone and by

stromal cell production o IL-15 and decidual prolactin (Dunn,

2002; Gubbay, 2002). Although dNK cells have the capacity

or cytotoxicity, they are not cytotoxic toward etal trophoblasts. Teir cytotoxic potential is prevented by molecular cues

rom decidual macrophages. As noted, specic HLA molecule

expression protects against dNK cells’ damaging actions.

Decidual macrophages are another decidual immune cell type

and are distinct rom proinammatory M1 or antiinammatory

M2 macrophages. Tese cells regulate adaptive  cell responses;

control dNK dierentiation, activation, and cytotoxicity; and

produce antiinammatory cytokines such as IL-10.

Dendritic cells are antigen-presenting cells that educate

maternal  cells. Tey aect development o a receptive endometrium or implantation.

Maternal T cells, as part o the adaptive immune response,

increase in number and unction ater encounter with a specic

antigen. Tese cells subsequently retain the ability to respond

rapidly in a subsequent encounter with the same antigen. In

contrast, regs are immunosuppressive, and during pregnancy

systemic maternal populations expand. Specic reg cell populations persist and protect against aberrant immune responses.94 Placentation, Embryogenesis, and Fetal Development

Section 3

AMNION

At term, the amnion is a tough and tenacious but pliable membrane. Tis innermost avascular etal membrane is contiguous

with amnionic uid and provides almost all o the tensile strength

o the etal membranes. Its resilience to rupture is vital to success-

ul pregnancy outcomes. Indeed, preterm rupture o etal membranes is a major cause o preterm delivery (Chap. 45, p. 787).

Bourne (1962) described ve separate amnion layers. Here,

progression o discussed layers moves rom amnionic uid to

the chorion. Te innermost layer, which is bathed by amnionic

uid, is a single-layer cuboidal epithelium (Fig. 5-14). Tis epithelium attaches rmly to a distinct basement membrane. Next,

an acellular compact layer composed primarily o interstitial collagens is ollowed by the fbroblast-like mesenchymal cell layer.

Te outermost layer is the relatively acellular zona spongiosa,

which is contiguous with the chorion laeve. Te amnion also

contains a ew etal macrophages, which predominate in the

outer two layers. Te amnion lacks smooth muscle cells, nerves,

lymphatics, and importantly, blood vessels.

■ Amnion Development

Early during implantation, a space develops between the

embryonic cell mass and adjacent trophoblastic cells (see

Fig. 5-7). Small cells that line this inner surace o trophoblasts

are precursors o amnionic epithelium, and the amnion is rst

identiable on the 7th or 8th day o embryo development. It is

initially a minute vesicle, which then develops into a small sac

that covers the dorsal embryo surace. As the amnion enlarges,

it gradually enguls the growing embryo, which prolapses into

its cavity (Benirschke, 2012).

Distention o the amnionic sac eventually brings it into contact with the interior surace o the chorion laeve. Apposition

o the chorion laeve and amnion near the end o the rst trimester obliterates the extraembryonic coelom. Te amnion and

chorion laeve, although slightly adhered, are never intimately

connected and can be separated easily. Placental amnion covers

the placental surace and thereby is in contact with the chorionic vessels. Umbilical amnion covers the umbilical cord. As

discussed in Chapter 48 (p. 842), with monochorionic-diamnionic placentas, no tissue intervenes between the used amnions. With dichorionic-diamnionic twin placentas, amnions are

separated by used chorion laeves.

Amnionic uid lls the amnionic sac. As pregnancy progresses, this normally clear uid increases in volume until

approximately 34 weeks’ gestation. Ater this, the volume

declines. At term, it averages 1000 mL, although this may vary

widely in normal and especially abnormal conditions. Amnionic uid origin, composition, circulation, and unction o are

discussed urther in Chapter 14 (p. 256).

■ Amnion Cell Histogenesis

Amnionic epithelium derives rom etal ectoderm o the embryonic disc and not rom trophoblasts. Tis is an important consideration both embryologically and unctionally. For example,

HLA class I gene expression in amnion is more akin to that in

embryonic cells than to that in trophoblasts.

Te broblast-like mesenchymal cell layer likely originates

rom embryonic mesoderm. Early in human embryogenesis,

the amnionic mesenchymal cells lie immediately adjacent to

the basal surace o the amnion epithelium. At this time, the

amnion has two-cell layers and approximately equal numbers o

epithelial and mesenchymal cells. Simultaneously with growth

and development, interstitial collagens are deposited between

these two cell layers. Tis marks ormation o the amnion compact layer, which separates the two early layers.

Amnionic epithelium early in pregnancy replicates at a rate

appreciably aster than mesenchymal cells. Tus, as the amnionic sac expands, its epithelial cells orm a continuous, uninterrupted layer. Instead, mesenchymal cells become more sparsely

distributed. Connected by a lattice network o extracellular

matrix, they appear as long, slender brils.

Amnion Epithelial Cells

Te apical surace o the amnionic epithelium is replete with

highly developed microvilli. Tis structure reects its unction

as a major site o transer between amnionic uid and amnion

layers. Tis epithelium is metabolically active, and its cells synthesize tissue inhibitor o MMP-1, prostaglandin E2 (PGE2),

and etal bronectin (FN) (Rowe, 1997). Although epithelia

produce FN, studies suggest that FN acts in the underlying

mesenchymal cells. Here, FN promotes synthesis o MMPs that

break down strength-bearing collagens. It also enhances prostaglandin synthesis to prompt uterine contractions (Mogami,

2013). Tis pathway is upregulated with premature rupture o

membranes induced by thrombin or inection-induced release

o FN (Chigusa, 2016; Mogami, 2014).

Epithelial cells may respond to signals derived rom the etus

or the mother, and they are responsive to various endocrine or

paracrine modulators. Examples include oxytocin and vasopressin, both o which increase PGE2 production in vitro (Moore,

1988). Tese cells may also produce cytokines such as IL-8 during labor initiation (Elliott, 2001).

FIGURE 5-14 Photomicrograph of fetal membranes. From left to

right: AE = amnion epithelium; AM = amnion mesenchyme; S =

zona spongiosa; CM = chorionic mesenchyme; TR = trophoblast;

D = decidua. (Reproduced with permission from Dr. Judith R. Head.)Implantation and Placental Development 95

CHAPTER 5

Amnionic epithelium also synthesizes vasoactive peptides,

which unction in both maternal and etal tissues in diverse

physiological processes. Tese peptides include endothelin

and parathyroid hormone-related protein (Economos, 1992;

Germain, 1992). Others are brain natriuretic peptide (BNP)

and corticotropin-releasing hormone, which are peptides that

invoke smooth-muscle relaxation (Riley, 1991; Warren, 1995).

BNP production is positively regulated by mechanical stretch

in etal membranes and is proposed to unction in uterine quiescence. Epidermal growth actor, a negative regulator o BNP,

is upregulated in the membranes at term and leads to a decline

in BNP-regulated uterine quiescence (Carvajal, 2013).

Amnion Mesenchymal Cells

Tese cells are responsible or other major unctions. Mesenchymal cells synthesize the interstitial collagens that compose

the amnionic compact layer—the major source o its tensile

strength (Casey, 1996). At term, the generation o cortisol by

11β-hydroxysteroid dehydrogenase may contribute to membrane rupture by reducing collagen abundance (Mi, 2017).

Mesenchymal cells also synthesize cytokines that include IL-6,

IL-8, and MCP-1. Cytokine synthesis rises in response to bacterial toxins and IL-1. Tis ability o amnion mesenchymal

cells to synthesize chemokines is an important consideration in

interpreting studies o labor-associated accumulation o inammatory mediators in amnionic uid (Garcia-Velasco, 1999).

Last, mesenchymal cells may be a greater source o PGE2 than

epithelial cells, especially in the case o premature membrane

rupture (Mogami, 2013; Whittle, 2000).

■ Tensile Strength

During tests o tensile strength, the decidua and then the chorion laeve give way long beore the amnion ruptures. Indeed,

the membranes are elastic and can expand to twice normal

size during pregnancy (Benirschke, 2012). Te amnion tensile

strength resides almost exclusively in the compact layer, which

is composed o cross-linked interstitial collagens I and III, and

lesser amounts o collagens V and VI.

Amnion tensile strength is regulated in part by brillar collagen assembly. Tis process is inuenced by the interaction

between brils and proteoglycans such as decorin and biglycan (Chap. 21, p. 405). Reduction o these proteoglycans is

reported to perturb etal membrane unction (Horgan, 2014;

Wu, 2014). Fetal membranes overlying the cervix have a

regional shit in gene expression and lymphocyte activation that

set in motion an inammatory cascade (Marcellin, 2017). Tis

change may contribute to tissue remodeling and loss o tensile

strength in the amnion (Moore, 2009).

■ Metabolic Functions

Te amnion is metabolically active, is involved in solute and

water transport or amnionic uid homeostasis, and produces

an impressive array o bioactive compounds. Te amnion is

responsive both acutely and chronically to mechanical stretch,

which alters amnionic gene expression (Carvajal, 2013;

Nemeth, 2000). Tis in turn may trigger both autocrine and

paracrine responses that include production o MMPs, IL-8,

and collagenase (Bryant-Greenwood, 1998; Mogami, 2013).

Such actors may modulate changes in membrane properties

during labor.

UMBILICAL CORD

Te yolk sac and the umbilical vesicle into which it develops

are prominent early in pregnancy. Initially, the embryo is a

attened disc interposed between amnion and yolk sac (see

Fig. 5-7). Te embryonic dorsal surace, in association with

the elongation o its neural tube, grows aster than the ventral

surace. As a result, the embryo bulges into the amnionic sac,

and the embryo body incorporates the adjacent yolk sac to

orm the gut. Te body stalk connects the caudal embryo to

the chorion. Te etal allantois orms as a diverticulum rom the

caudal wall o the yolk sac and projects into the base o

the body stalk.

As pregnancy advances, the yolk sac becomes smaller and

its pedicle relatively longer. By the middle o the third month,

the expanding amnion uses with the chorion laeve to obliterate the extraembryonic coelom. In its expansion, the amnion

covers the bulging placental disc and the lateral surace o the

body stalk. Te latter is then called the umbilical cord or unis. A

more detailed description o this cord and potential abnormalities is ound in Chapter 6 (p. 113).

Te cord at term normally has two arteries and one vein.

Te right umbilical vein usually disappears early during etal

development, leaving only the original let vein. Te umbilical

cord extends rom the etal umbilicus to the etal surace o

the placenta, that is, the chorionic plate. As discussed in detail

in Chapter 7 (p. 126), blood ows rom the umbilical vein

toward the etus. Blood then takes a path o least resistance via

two routes within the etus. One is the ductus venosus, which

empties directly into the inerior vena cava. Te other route

consists o numerous smaller openings into the hepatic circulation. Blood rom the liver ows into the hepatic vein and

then the inerior vena cava. Resistance in the ductus venosus is

controlled by a sphincter situated at the origin o the ductus at

the umbilical recess and is innervated by a vagus nerve branch.

Blood exits the etus via the two umbilical arteries. Tese

are anterior branches o the internal iliac artery and become

obliterated ater birth to orm the medial umbilical ligaments.

PLACENTAL HORMONES

Te production o steroid and protein hormones by human

trophoblasts is greater in amount and diversity than that o

any single endocrine tissue in all o mammalian physiology.

Table 5-1 is a compendium o average production rates or various steroid hormones in nonpregnant and in near-term pregnant women. It demonstrates the remarkable increase in steroid

hormone production during pregnancy. Te human placenta

also synthesizes an enormous amount o protein and peptide

hormones, summarized in Table 5-2. Te successul physiological adaptations o pregnant women to this unique endocrine

milieu is discussed throughout Chapter 4.96 Placentation, Embryogenesis, and Fetal Development

Section 3

■ Human Chorionic Gonadotropin

Biosynthesis

Chorionic gonadotropin is a glycoprotein with biological activity similar to that o LH, and both act via the LH-hCG receptor.

hCG varies in molecular weight rom 36,000 to 40,000 Da and

is highly glycosylated with the most carbohydrate content o

any human hormone—30 percent. Tis glycosylation protects

the molecule rom catabolism and results in a 36-hour plasma

hal-lie or intact hCG compared with 2 hours or LH. Te

hCG molecule is composed o two dissimilar subunits, α and

β. Tese are noncovalently linked but held together by electrostatic and hydrophobic orces. Isolated subunits are unable to

bind the LH-hCG receptor and thus lack biological activity.

Te hCG hormone is structurally related to three other

glycoprotein hormones—LH, FSH, and thyroid-stimulating

hormone (SH). All our glycoproteins share a common

α-subunit. However, each o their β-subunits is characterized

by a distinct (although related) amino acid sequence.

Synthesis o the α- and β-chains o hCG is regulated separately rom gene loci on dierent chromosomes. A single gene

TABLE 5-1. Steroid Production Rates in Nonpregnant

and Near-Term Pregnant Women

Production Rates (mg/24 hr)

Steroida Nonpregnant Pregnant

Estradiol 0.1–0.6 15–20

Estriol 0.02–0.1 50–150

Progesterone 0.1–40 250–600

Aldosterone 0.05–0.1 0.250–0.600

Deoxycorticosterone 0.05–0.5 1–12

Cortisol 10–30 10–20

aEstrogens and progesterone are produced by placenta.

Aldosterone is produced by the maternal adrenal in

response to the stimulus of angiotensin II. Deoxycorticosterone

is produced in extraglandular tissue sites by way of the

21-hydroxylation of plasma progesterone. Cortisol production during pregnancy is not increased, even though the

blood levels are elevated because of decreased clearance

caused by increased cortisol-binding globulin.

TABLE 5-2. Protein Hormones Produced by the Human Placenta

Hormone

Primary Non-placental

Site of Expression

Shares Structural or

Function Similarity Functions

Human chorionic

gonadotropin (hCG)

— LH, FSH, TSH Maintains corpus luteum function

Regulates fetal testis testosterone

secretion

Stimulates maternal thyroid

Placental lactogen (PL) — GH, prolactin Aids maternal adaptation to fetal energy

requirements

Adrenocorticotropin (ACTH) Hypothalamus —

Corticotropin-releasing

hormone (CRH)

Hypothalamus — Relaxes smooth-muscle; initiates

parturition?

Promotes fetal and maternal glucocorticoid

production

Gonadotropin-releasing

hormone (GnRH)

Hypothalamus — Regulates trophoblast hCG production

Thyrotropin (TRH) Hypothalamus Unknown

Growth hormone-releasing

hormone (GHRH)

Hypothalamus — Unknown

Growth hormone variant

(hGH-V)

— GH variant not found

in pituitary

Potentially mediates pregnancy insulin

resistance

Neuropeptide Y Brain Potential regulates CRH release by

trophoblasts

Parathyroid-releasing

protein (PTH-rp)

— Regulates transfer of calcium and

other solutes; regulates fetal mineral

homeostasis

Inhibin Ovary/testis Potentially inhibits FSH-mediated ovulation;

regulates hCG synthesis

Activin Ovary/testis Regulates placental GnRH synthesis

GH = growth hormone; FSH = follicle-stimulating hormone; LH = luteinizing hormone; TSH = thyroid-stimulating

hormone.Implantation and Placental Development 97

CHAPTER 5

on chromosome 6 encodes the α-subunit. Chromosome 19

encodes the β-hCG–β-LH amily o subunits with six genes or

β-hCG and one or β-LH (Miller-Lindholm, 1997). Both subunits are synthesized as larger precursors and then cleaved by

endopeptidases to their mature orm. Intact hCG is assembled

and released by secretory granule exocytosis (Morrish, 1987).

Modications during synthesis and subsequent enzymatic

degradation give rise to multiple orms o hCG in maternal

plasma and urine that vary enormously in bioactivity and

immunoreactivity.

Beore 5 weeks, hCG is expressed both in the syncytiotrophoblast and cytotrophoblasts (Maruo, 1992). Later in the rst

trimester, hCG is produced almost solely in the syncytiotrophoblast, peaks around 9 weeks’ gestation, and then declines

to a plateau or the remainder o gestation (Beck, 1986;

Kurman, 1984). Dynamic changes in hCG concentration in

the rst trimester highlight the importance o accurate gestational age estimation when interpreting aneuploidy screening

strategies that include hCG.

Circulating levels o ree β-subunit are low to undetectable

throughout pregnancy, and their concentration is the limiting

actor or secretion o complete hCG molecules. Levels o the

α-subunit rise gradually and roughly correspond to placental

mass, until they plateau at approximately 36 weeks’ gestation

(Cole, 1997).

Concentrations in Serum and Urine

Te combined hCG molecule is detectable in plasma o pregnant women 7 to 9 days ater the midcycle LH surge preceding ovulation. Tus, hCG likely enters maternal blood at the

time o blastocyst implantation. Plasma levels rise rapidly,

doubling approximately every 2 days in the rst trimester

(Fig. 5-15). Appreciable uctuations in levels or a given patient

are observed on the same day.

Intact hCG circulates as multiple, highly related isoorms

that have variable cross-reactivity between commercial assays.

Tis emphasizes the need to use the same assay type when measuring serial hCG levels or clinical indications such as evaluating pregnancy o unknown location or medical management o

ectopic pregnancy. Peak maternal plasma levels reach approximately 50,000 to 100,000 mIU/mL between the 60th and

80th days ater menses. Plasma levels then decline, and a nadir

is reached by approximately 16 weeks’ gestation. Plasma levels

remain at this lower level or the rest o pregnancy.

Although maternal urine, like plasma, contains various

hCG degradation products, the principal urinary orm is the

terminal product o hCG degradation, namely, the β-core ragment. Concentrations o this ragment ollow the same general

pattern as that in maternal plasma, peaking at approximately

10 weeks’ gestation. Importantly, the β-subunit antibody used

in most pregnancy tests reacts with both intact hCG (the major

orm in the plasma) and with ragments o hCG (the major

orm ound in urine).

hCG Regulation

Placental GnRH is likely involved in the regulation o hCG

ormation. Both GnRH and its receptor are expressed by

cytotrophoblasts and syncytiotrophoblast (Wolahrt, 1998).

GnRH administration elevates circulating hCG levels, and cultured trophoblasts respond to GnRH treatment with increased

hCG secretion (Iwashita, 1993; Siler-Khodr, 1981). Pituitary

GnRH production is regulated by inhibin and activin. Likewise, in cultured placental cells, activin stimulates and inhibin

inhibits GnRH and hCG production (Petraglia, 1989; Steele,

1993).

hCG is cleared by the kidney (about 30 percent), and the

remainder is likely cleared by liver metabolism (Wehmann,

1980). Tus, levels can be markedly altered in gravidas with

chronic renal disease.

Biological Functions

Noted earlier, hCG maintains corpus luteum unction—that

is, continued progesterone production. Both hCG subunits are

required or binding to the LH-hCG receptor in the corpus

luteum. However, maximum plasma hCG concentrations are

attained well ater hCG-stimulated corpus luteum secretion o

progesterone has ceased. Specically, luteal progesterone synthesis begins to decline at approximately 6 weeks’ gestation

despite continued and increasing hCG production. Tereore,

this incompletely explains the physiological unction o hCG

in pregnancy. LH-hCG receptors are present in various other

tissues, and roles are discussed subsequently.

In pregnancies with male etuses, hCG stimulates etal testicular testosterone secretion. Tis reaches a maximum when

hCG levels peak. Tus, at a critical time in male sexual dierentiation, hCG enters etal plasma rom the syncytiotrophoblast.

In the etus, it acts as an LH surrogate to stimulate Leydig

cell replication and testosterone synthesis to promote male

sexual dierentiation (Chap. 3, p. 35). Beore approximately

110 days, the etal anterior pituitary lacks vascularization rom

the hypothalamus and produces minimal LH secretion. Tereater, as hCG levels all, pituitary LH maintains modest testicular stimulation.

Te maternal thyroid gland also is stimulated by large

quantities o hCG. In women with gestational trophoblastic

disease, biochemical and clinical evidence o hyperthyroidism sometimes develops (Chap. 13, p. 238). Some orms o

140 500

400

300

200

100

120

100

80

60

40

hCG

hPL

CRH

20

0

0 10 20

Weeks’ gestation

hCG (IU/mL)

hPL(µg/mL)

CRH(pmol/mL)

30 40

8 7 6 5 4 3 2 1 0

FIGURE 5-15 Distinct profiles for the concentrations of human

chorionic gonadotropin (hCG), human placental lactogen (hPL),

and corticotropin-releasing hormone (CRH) in serum of women

throughout normal pregnancy.98 Placentation, Embryogenesis, and Fetal Development

Section 3

hCG bind to SH receptors on thyrocytes (Hershman, 1999).

Te thyroid-stimulatory activity in plasma o rst-trimester

pregnant women varies appreciably rom sample to sample.

Modications o hCG oligosaccharides likely are important in

the capacity o hCG to stimulate thyroid unction. Acidic iso-

orms stimulate thyroid activity, and some more basic isoorms

stimulate iodine uptake (Kraiem, 1994; suruta, 1995). Last,

the LH-hCG receptor is also expressed by thyrocytes, which

suggests that hCG stimulates thyroid activity via the LH-hCG

receptor as well (omer, 1992).

LH-hCG receptors are ound in myometrium and in uterine vascular tissue. It has been hypothesized that hCG may

promote uterine vascular vasodilation and myometrial smooth

muscle relaxation (Kurtzman, 2001). hCG also modulates

maternal immune cell unctions in the decidua during early

stages o placentation (Schumacher, 2019; Silasi, 2020).

Abnormally High or Low Levels

Several clinical circumstances display substantively higher

maternal plasma hCG levels. Some examples include multietal

pregnancy, erythroblastosis etalis associated with etal hemolytic anemia, and gestational trophoblastic disease. Relatively

higher hCG levels may be ound in women carrying a etus with

Down syndrome, and this has been incorporated into screening

strategies (able 17-4, p. 336). Various malignant tumors also

produce hCG, sometimes in large amounts— especially trophoblastic neoplasms (Chap. 13, p. 241).

Relatively lower hCG plasma levels are ound in women

with ailing early pregnancies and ectopic pregnancy (Chap.

12, p. 222). hCG is produced in very small amounts in normal

tissues o men and nonpregnant women, perhaps primarily in

the anterior pituitary gland. Nonetheless, the detection o hCG

in blood or urine almost always indicates pregnancy (Chap. 10,

p. 176).

■ Human Placental Lactogen

Biosynthesis

Tis single, nonglycosylated polypeptide chain shares a 96-

percent amino-acid-sequence homology with human growth

hormone (hGH) and a 67-percent homology with human prolactin (hPRL). Because o these similarities, it was called chorionic growth hormone or human placental lactogen. Currently,

the latter term is used by most.

Five genes in the growth hormone–placental lactogen gene

cluster are linked and located on chromosome 17: GH1, GH2,

CSHL1, CSH1, and CSH2. Human placental lactogen (hPL) is

encoded by the last two genes. hPL is concentrated in syncytiotrophoblast, but similar to hCG, hPL is demonstrated in cytotrophoblasts beore 6 weeks (Grumbach, 1964; Maruo, 1992).

Within 5 to 10 days ater conception, hPL is demonstrable in

the placenta and can be detected in maternal serum as early as

3 weeks. Levels o mRNA or hPL in syncytiotrophoblast

remain relatively constant throughout pregnancy. Tis nding

supports the idea that the hPL secretion rate is proportional

to placental mass. Levels rise steadily until 34 to 36 weeks’

gestation. Te hPL production rate near term—approximately

1 g/d—is by ar the greatest o any known hormone in humans.

It is rapidly cleared and has a hal-lie between 10 and 30 minutes (Walker, 1991). In late pregnancy, maternal serum concentrations reach levels o 5 to 15 μg/mL (see Fig. 5-15).

Very little hPL is detected in etal blood, and amnionic uid

levels are somewhat lower than that in maternal plasma. Tus,

although hPL may have a direct eect on etal tissues, such as

modulating etal vasculature ormation, its primary role is to

act on maternal physiology to ensure adequate nutrient delivery

to the placenta (Corbacho, 2002).

Metabolic Actions

hPL has putative actions in several important maternal metabolic processes. First, hPL promotes lipolysis to raise circulating

ree atty acid levels. Tis provides an energy source or maternal metabolism and etal nutrition. In vitro studies suggest that

hPL inhibits secretion by term syncytiotrophoblast o leptin

(Coya, 2005). Prolonged maternal starvation in the rst hal o

pregnancy leads to higher hPL plasma concentrations.

Second, hPL aids maternal adaptation to etal energy requirements (Hill, 2018). For example, increased maternal insulin

resistance ensures nutrient ow to the etus. It also avors protein synthesis and provides a readily available amino acid source

to the etus. o counterbalance the greater insulin resistance

and prevent maternal hyperglycemia, maternal insulin levels

rise. Both hPL and prolactin signal through the prolactin receptor to increase maternal beta cell prolieration, which augments

insulin secretion (Georgia, 2010). In animals, prolactin and

hPL upregulate serotonin synthesis, which increases beta cell

prolieration (Kim, 2010). Short-term changes in plasma glucose or insulin, however, have relatively little eect on plasma

hPL levels. In vitro studies o syncytiotrophoblast suggest that

hPL synthesis is stimulated by insulin and insulin-like growth

actor-1 and inhibited by PGE2 and PGF2α (Bhaumick, 1987;

Genbacev, 1977).

■ Other Placental Protein Hormones

Te placenta has a remarkable capacity to synthesize numerous peptide hormones, including some that are analogous or

related to hypothalamic and pituitary hormones. In contrast

to their counterparts, some o these placental peptide/protein

hormones are not subject to eedback inhibition.

Hypothalamic-Like Releasing Hormones

Te known hypothalamic-releasing or -inhibiting hormones

include GnRH, corticotropin-releasing hormone (CRH),

thyrotropin-releasing hormone (RH), growth hormone–

releasing hormone (GHRH), and somatostatin. For each o

these, the human placenta produces an analogous hormone

(Petraglia, 1992; Siler-Khodr, 1988).

GnRH in the placenta shows its highest expression in the rst

trimester (Siler-Khodr, 1978, 1988). Interestingly, it is ound

in cytotrophoblasts but not syncytiotrophoblast. Placentaderived GnRH unctions to regulate trophoblast hCG production and extravillous trophoblast invasion via regulation o

MMP-2 and MMP-9 (Peng, 2016). Placenta-derived GnRH

is also the likely source o elevated maternal GnRH levels in

pregnancy (Siler-Khodr, 1984).Implantation and Placental Development 99

CHAPTER 5

CRH is a member o a larger amily o CRH-related peptides that includes CRH and urocortins (Dautzenberg, 2002).

Maternal serum CRH levels rise rom 5 to 10 pmol/L in the

nonpregnant woman to approximately 100 pmol/L in the early

third trimester and then to almost 500 pmol/L abruptly during the last 5 to 6 weeks (see Fig. 5-15). Ater labor begins,

maternal plasma CRH levels rise even urther (Petraglia, 1989,

1990). Urocortin, involved in the stress response, is also produced by the placenta and secreted into the maternal circulation, but at much lower levels than that seen or CRH (Florio,

2002).

Te unction o CRH synthesized in the placenta, membranes, and decidua has been somewhat dened. For example,

trophoblast, amniochorion, and decidua express both CRH-R1

and CRH-R2 receptors and several variant receptors (Florio,

2000). Both CRH and urocortin enhance trophoblast secretion

o adrenocorticotropic hormone (ACH), and this suggests an

autocrine–paracrine role (Petraglia, 1999). Large amounts o

trophoblast CRH enter maternal blood.

CRH receptors are also present in many tissues outside

the placenta. Proposed biological roles include induction o

smooth-muscle relaxation in vascular and myometrial tissue

and immunosuppression. Te physiological reverse, however,

induction o myometrial contractions, has been proposed or

the rising CRH levels seen near term. Some hypothesize that

CRH may be involved with parturition initiation (Wadhwa,

1998).

Glucocorticoids act in the hypothalamus to inhibit CRH

release. But, in the trophoblast, glucocorticoids stimulate CRH

gene expression (Jones, 1989a; Robinson, 1988). Tus, a novel

positive eedback loop in the placenta may allow placental

CRH to stimulate placental ACH and thereby prompt etal

and maternal adrenal glucocorticoid production, with subsequent stimulation o placental CRH expression (Nicholson,

2001; Riley, 1991).

GHRH is expressed in placenta, but its unction is unclear

(Berry, 1992). GHRH may play an autocrine role in trophoblast survival via the GHRH receptor (Liu, 2016). Ghrelin is

another regulator o hGH secretion and is produced by placental tissue (Horvath, 2001). rophoblast ghrelin expression

peaks at midpregnancy and is a paracrine regulator o dierentiation or is a potential regulator o human growth hormone

variant production, described next (Fuglsang, 2005; Gualillo,

2001).

Pituitary-Like Hormones

A human growth hormone variant (hGH-V) that is not expressed

in the pituitary is expressed in the placenta. Te gene encoding

hGH-V is located in the hGH–hPL gene cluster on chromosome 17. Sometimes reerred to as placental growth hormone,

hGH-V is a 191-amino-acid protein that diers in 15 amino

acid positions rom the sequence or hGH. Although hGH-V

retains growth-promoting and antilipogenic unctions that are

similar to those o hGH, it has reduced diabetogenic and lactogenic unctions relative to hGH (Vickers, 2009). Placental

hGH-V presumably is synthesized in the syncytiotrophoblast.

It is believed that hGH-V is present in maternal plasma by 21 to

26 weeks’ gestation, rises in concentration until approximately

36 weeks, and remains relatively constant thereater. hGH-V

levels in maternal plasma and those o insulin-like growth actor 1 positively correlate. Moreover, hGH-V secretion by trophoblast in vitro is inhibited by glucose in a dose-dependent

manner (Patel, 1995). Overexpression o hGH-V in mice

causes severe insulin resistance, making it a likely candidate to

mediate insulin resistance o pregnancy (Liao, 2016).

Pro-opiomelanocortin (POMC) is a polypeptide produced

in the pituitary and other tissues including the placenta. It is

proteolytically cleaved into numerous active hormones including ACH, β-lipotropic hormone, melanocyte-stimulating

hormone (α-, β-, and γ-MSH), and β–endorphin (Harno,

2018). Tese hormones play a role in maintaining energy balance. As discussed, placental CRH stimulates synthesis and

release o placental ACH, demonstrating the autocrine and

paracrine unctions o the placenta in addition to its systemic

endocrine activity.

Relaxin

Te peptide is expressed in human corpus luteum, decidua,

and placenta (Bogic, 1995). wo o the three relaxin genes—

H2 and H3—are transcribed in the corpus luteum (Bathgate,

2002; Hudson, 1983, 1984). Decidua, placenta, and membranes express H1 and H2 (Hansell, 1991). Relaxin is synthesized as a single, 105-amino-acid preprorelaxin molecule that is

cleaved to A and B molecules. Relaxin is structurally similar to

insulin and insulin-like growth actor.

Te rise in maternal circulating relaxin levels in early pregnancy is attributed to corpus luteum secretion, and levels parallel those o hCG. Relaxin, along with rising progesterone

levels, may act on myometrium to promote relaxation and the

quiescence o early pregnancy (Chap. 21, p. 404). In addition,

the production o relaxin and relaxin-like actors within the

placenta and etal membranes may play an autocrine-paracrine

role in postpartum regulation o extracellular matrix remodeling (Qin, 1997a,b). One important relaxin unction is enhancement o the maternal glomerular ltration rate that is apparent

early in gestation (Chap. 4, p. 68).

Parathyroid Hormone-Related Protein

Levels o this peptide are elevated within maternal but not

etal circulation (Bertelloni, 1994; Saxe, 1997). Parathyroid

hormone-related protein (PH-rP) synthesis is ound in several normal adult tissues, especially in reproductive organs

that include myometrium, endometrium, corpus luteum, and

lactating mammary tissue. PH-rP is not produced in the

parathyroid glands o normal adults. Although yet undened,

placenta-derived PH-rP may regulate genes involved in trans-

er o calcium and other solutes. It also contributes to mineral

homeostasis in etal bone, amnionic uid, and the etal circulation (Simmonds, 2010).

Leptin

Tis hormone is normally secreted by adipocytes, but cytotrophoblasts and syncytiotrophoblast also synthesize leptin

(Henson, 2002). Relative contributions o leptin rom maternal adipose tissue versus placenta are currently undened.

Leptin unctions as an antiobesity hormone that decreases ood100 Placentation, Embryogenesis, and Fetal Development

Section 3

intake through its hypothalamic receptor. It also regulates bone

growth and immune unction (Cock, 2003; La Cava, 2004).

Placental leptin promotes placental cell prolieration, protein

synthesis, and activation o immune tolerance and antiapoptotic responses (Rosario, 2016a; Schanton, 2018). Maternal

serum levels are signicantly higher than those in nonpregnant

women. Fetal leptin levels correlate positively with birthweight

and likely unction in etal development and growth. Studies suggest that reductions in leptin availability contribute to

adverse etal metabolic programing in intrauterine growthrestricted ospring (Nusken, 2016, Perez-Perez, 2018).

Neuropeptide Y

Tis 36-amino-acid peptide is widely distributed in brain. It

also is ound in sympathetic neurons innervating the cardiovascular, respiratory, gastrointestinal, and genitourinary systems.

Neuropeptide Y has been isolated rom the placenta and localized in cytotrophoblasts (Petraglia, 1989). rophoblasts possess

neuropeptide Y receptors, and treatment o these with neuropeptide Y causes CRH release (Robidoux, 2000).

Transforming Growth Factor Beta Superfamily

Tis amily o cytokines regulates various cellular unctions that

include placental development, trophoblast dierentiation, and

invasion into the decidua (Adu-Gyama, 2020). Tis process

is nely tuned, and extreme invasion results in placenta accreta

spectrum disorders, whereas shallow implantation can lead to

early pregnancy loss or preeclampsia. Members o the amily

include transorming growth actor beta (GF-β), activin,

inhibin, nodal, bone morphogenic proteins (BMPs), antimüllerian hormone, and growth dierentiation actors (GDFs).

Tese cytokines bind to a suite o receptors composed o

a type 1 subunit and a type 2 receptor subunit to ultimately

direct gene expression. Tis combinatorial diversity leads to

divergent unctions in the placenta and decidua. Activin A

promotes syncytialization, extravillous trophoblast ormation,

and invasion. BMP2 enhances invasion, and BMP4 may direct

embryonic stem cells toward a trophoblast lineage (Zhang

2018, Xu 2002). Inhibin A promotes syncytialization but

inhibits invasion (Debiève, 2000; Jones, 2006). Nodal inhibits

prolieration, extravillous trophoblast ormation, and invasion.

Te complete role this superamily in normal and abnormal

placental development is yet to be ully dened.

■ Placental Progesterone Production

Ater 6 to 7 weeks’ gestation, little progesterone is produced in

the ovary (Diczalusy, 1961). Surgical removal o the corpus

luteum or even bilateral oophorectomy during the 7th to 10th

week does not decrease excretion rates o urinary pregnanediol,

the principal urinary metabolite o progesterone. Beore this

time, however, corpus luteum removal will lead to spontaneous

abortion unless an exogenous progestin is given, and Chapter

66 (p. 1170) lists suitable dosing. Ater approximately 8 weeks,

the placenta assumes progesterone secretion, and maternal

serum levels throughout pregnancy gradually rise (Fig. 5-16).

By term, these levels are 10 to 5000 times o those in nonpregnant women, depending on the ovarian cycle stage.

Te daily production rate o progesterone in late, normal,

singleton pregnancies approximates 250 mg. In multietal pregnancies, the daily production rate may exceed 600 mg. Progesterone is synthesized rom cholesterol in a two-step enzymatic

reaction. First, cholesterol is converted to pregnenolone within

the mitochondria in a reaction catalyzed by cytochrome P450

cholesterol side-chain cleavage enzyme. Pregnenolone leaves

the mitochondria and is converted to progesterone in the endoplasmic reticulum by 3β-hydroxysteroid dehydrogenase. Progesterone is released immediately by diusion.

Although the placenta produces a prodigious amount o

progesterone, the syncytiotrophoblast has a limited capacity or

cholesterol biosynthesis. Te rate-limiting enzyme in its biosynthesis is 3-hydroxy-3-methylglutaryl coenzyme A (HMGCoA) reductase. Because o this, the placenta must rely on an

exogenous source, that is, maternal cholesterol, or progesterone ormation. Te trophoblast preerentially uses LDL cholesterol or progesterone biosynthesis (Simpson, 1979, 1980).

Tis mechanism diers rom placental production o estrogens,

which relies principally on etal adrenal precursors.

Although etal well-being and placental estrogen production

show a relationship, this is not the case or placental progesterone. Tus, placental endocrine unction, including progesterone biosynthesis and ormation o protein hormones such as

hCG, may persist or weeks ater etal demise.

Estetrol

Gestational age (weeks)

Plasma unconjugated steroid (ng/mL)

4

0.05

0.1

0.5

1.0

5.0

10.0

50.0

100.0

8 12 16 20 24 28 32 36 40

Estrone

Estriol

Estradiol

Estradiol

(nonpregnant)

Progesterone

(nonpregnant)

Progesterone

FIGURE 5-16 Plasma levels of progesterone, estradiol, estrone,

estetrol, and estriol in women during the course of gestation.

(Modified and redrawn with permission from Mesiano S: The endocrinology of human pregnancy and fetoplacental neuroendocrine

development. In Strauss JF, Barbieri RL (eds) Yen and Jaffe’s Reproductive Endocrinology: Physiology, Pathophysiology, and Clinical

Management, 6th ed. Philadephia, PA: Saunders; 2009.)Implantation and Placental Development 101

CHAPTER 5

Te metabolic clearance rate o progesterone in pregnant

women is similar to that ound in men and nonpregnant women.

One metabolite is 5α-dihydroprogesterone (5α-DHP), and levels disproportionately rise due to synthesis in syncytiotrophoblast

rom both placenta-produced progesterone and etus-derived

precursor (Dombroski, 1997). Tus, the concentration ratio o

5α-DHP to progesterone is elevated in pregnancy, although the

mechanisms or this are incompletely dened. Progesterone also

is converted to the potent mineralocorticoid deoxycorticosterone in pregnant women and in the etus. Te concentration

o deoxycorticosterone is strikingly higher in both maternal and

etal compartments (see able 5-1). Te extraadrenal ormation

o deoxycorticosterone rom circulating progesterone accounts

or most o its production in pregnancy (Casey, 1982a,b).

■ Placental Estrogen Production

During the rst 2 to 4 weeks or pregnancy, rising hCG levels

maintain production o estradiol in the corpus luteum. Ovarian production o both progesterone and estrogens drops signicantly by the 7th week o pregnancy. At this time, there is

a luteal–placental transition. Subsequently, more than hal o

estrogen entering maternal circulation is produced in the placenta, and it produces a continually increasing magnitude o

estrogen (MacDonald, 1965a; Siiteri, 1963, 1966). Near term,

normal human pregnancy is a hyperestrogenic state, and syncytiotrophoblast is producing estrogen in amounts equivalent

to that produced in one day by the ovaries o no ewer than

1000 ovulatory women. Tis hyperestrogenic state terminates

abruptly ater delivery o the placenta.

Biosynthesis

In human trophoblast, neither

cholesterol nor in turn progesterone can serve as precursor or

estrogen biosynthesis. Tis is because

steroid 17α-hydroxylase/17,20-lyase

(CYP17A1) is not expressed in the

human placenta. Tis essential

enzyme converts 17-OH progesterone (a C21 steroid) to androstenedione, which is a C19 steroid and an

estrogen precursor. Consequently,

conversion o C21 steroids to C19

steroids is not possible.

However, dehydroepiandrosterone (DHEA) and its sulate

(DHEA-S) are also C19 steroids

and are produced by maternal

and etal adrenal glands. Tese

two steroids can serve as estrogen precursors in the placenta

(Fig. 5-17). Ryan (1959a) ound

that the placenta had an exceptionally high capacity to convert

appropriate C19 steroids to estrone

and estradiol. Te conversion o

DHEA-S to estradiol requires

placental expression o our key enzymes that are located

principally in syncytiotrophoblast (Bonenant, 2000;

Salido, 1990). First, the placenta expresses high levels o

steroid sulatase (SS), which converts the conjugated DHEA-S

to DHEA. DHEA is then acted upon by 3β-hydroxysteroid

dehydrogenase type 1 (3βHSD) to produce androstenedione.

Cytochrome P450 aromatase (CYP19) then converts androstenedione to estrone, which is then converted to estradiol

by 17β-hydroxysteroid dehydrogenase type 1 (17βHSD1).

DHEA-S is the major precursor o estrogens in pregnancy

(Baulieu, 1963; Siiteri, 1963). However, maternal adrenal

glands do not produce sufcient amounts o DHEA-S to

account or more than a raction o total placental estrogen biosynthesis. Te etal adrenal glands are quantitatively the most

important source o placental estrogen precursors in human

pregnancy. Tus, estrogen production during pregnancy reects

the unique interactions among etal adrenal glands, etal liver,

placenta, and maternal adrenal glands.

Directional Secretion

O estradiol and estriol ormed in syncytiotrophoblast, >90

percent enters maternal plasma (Gurpide, 1966). O placental

progesterone production, ≥85 percent enters maternal plasma,

and little maternal progesterone crosses the placenta to the etus

(Gurpide, 1972).

Tis directional secretion stems rom the architecture o

hemochorioendothelial placentation. Steroids produced in the

syncytiotrophoblast are secreted directly into maternal blood.

o reach the etus, they must rst traverse the cytotrophoblast

layer and then enter the stroma o the villous core and then etal

capillaries. From either o these spaces, steroids can reenter the

FIGURE 5-17 Schematic presentation of estrogen biosynthesis in the human placenta. Dehydroepiandrosterone sulfate (DHEA-S) is secreted in prodigious amounts by the fetal adrenal glands,

and a portion is converted to 16α-hydroxydehydroepiandrosterone sulfate (16OH-DHEA-S) in the

fetal liver. DHEA-S and 16OH-DHEA-S are converted in the placenta to the estrogens 17β-estradiol

(E2) and estriol (E3). These estrogens then enter the maternal circulation. Near term, half of E2 is

derived from fetal adrenal DHEA-S and half from maternal DHEA-S. On the other hand, 90 percent

of E3 in the placenta arises from fetal 16OH-DHEA-S and only 10 percent from all other sources.

3βHSD1 = 3β-hydroxysteroid dehydrogenase type 1; 17βHSD1 = 17β-hydroxysteroid dehydrogenase type 1; CYP17 = steroid 17α-hydroxylase/17,20-lyase; LDL = low-density lipoprotein;

SCC = cholesterol side-chain cleavage enzyme; StAR = steroidogenic acute regulatory protein.102 Placentation, Embryogenesis, and Fetal Development

Section 3

syncytium. Te net result o this hemochorial arrangement is

that entry o steroids into the maternal circulation is substantially greater than that into etal blood.

FETAL ADRENAL GLAND–PLACENTAL

INTERACTIONS

As noted, the etal adrenal gland is a vital source o steroid

precursors or placental estrogen synthesis. Tis etal gland is

remarkable both morphologically and unctionally. At term, its

mass exceeds that in adults (Chap. 7, p. 134). More than 85

percent o the etal gland is composed o a unique etal zone,

which has a great capacity or steroid biosynthesis. Its daily steroid production near term is 100 to 200 mg/d, which exceeds

the adult steroid secretion o 30 to 40 mg/d. Te unique etal

zone subsequently regresses in the rst year o lie.

In addition to responding to ACH rom the etal brain,

etal adrenal gland growth is inuenced by actors secreted by

the placenta. Tis is exemplied by the continued adrenal gland

growth throughout gestation and by its rapid involution immediately ater birth and placenta delivery.

■ Placental Estriol Synthesis

Estradiol is the primary placental estrogen product at term.

In addition, signicant levels o estriol and estetrol are ound

in the maternal circulation, particularly late in gestation (see

Fig. 5-16). Tese hydroxylated orms o estrogen derive rom

the placenta using substrates ormed by the combined eorts o

the etal adrenal gland and etal liver enzymes (see Fig. 5-17).

For this, high levels o hepatic 16α-hydroxylase act on adrenal-derived steroids (MacDonald, 1965b; Ryan, 1959b). Tus,

the disproportionate rise in estriol ormation during pregnancy

is accounted or by placental synthesis rom plasma-borne

16-OH-DHEA-S. Near term, the etus produces 90 percent o

placental estriol and estetrol precursors in normal human pregnancy. Tus, in the past, levels o these steroids were used as an

indicator o etal well-being.

■ Fetal Adrenal Steroid Precursor

Cholesterol is the precursor or etal adrenal steroidogenesis.

Here, the steroid biosynthesis rate is so great that its steroidogenesis alone is equivalent to a ourth o the total daily LDL

cholesterol turnover in adults. Fetal adrenal glands synthesize

cholesterol rom acetate, and all enzymes involved in this biosynthesis are elevated compared with those o the adult adrenal

gland (Rainey, 2001). Tus, the de novo cholesterol synthesis

rate by etal adrenal tissue is extremely high. Even so, it is insu-

cient to account or the steroids produced by etal adrenal

glands. Tereore, cholesterol must be assimilated rom the etal

circulation and mainly rom LDL produced in the etal liver

(Carr, 1980, 1984; Simpson, 1979).

■ Fetal Conditions Affecting Estrogen

Production

Several etal disorders alter the availability o substrate or placental steroid synthesis and thus highlight the interdependence

o etal development and placental unction. Fetal demise is

ollowed by a striking reduction in maternal urinary estrogen

levels. Similarly, ater ligation o the umbilical cord with the

etus and placenta let in situ, placental estrogen production

declines markedly (Cassmer, 1959). However, as previously

discussed, placental progesterone production is maintained.

Tese observations indicate that an important source o precursors or placental estrogen—but not or progesterone—biosynthesis derive rom the etus.

Anencephalic etuses have markedly atrophic adrenal glands

due to absent hypothalamic and pituitary structures that

would otherwise release ACH or adrenal stimulation. In the

absence o the adrenal cortical etal zone development, placental ormation o estrogen is severely limited because o diminished availability o C19 steroid precursors. Indeed, urinary

estrogen levels in women pregnant with an anencephalic etus

approximate only 10 percent o those ound in normal pregnancy (Frandsen, 1961). With an anencephalic etus, almost

all estrogens produced arise rom placental use o maternal

plasma DHEA-S.

Fetal adrenal cortical hypoplasia occurs in perhaps 1 in

12,500 births (McCabe, 2001). Estrogen production in these

pregnancies is also limited, which suggests the absence o C19

precursors.

Fetal–placental sulatase defciency is associated with very

low estrogen levels in otherwise normal pregnancies (France,

1969). Namely, sulatase deciency precludes the hydrolysis o

C19 steroid sulates, the rst enzymatic step in the placental

use o these circulating prehormones or estrogen biosynthesis.

Tis deciency is an X-linked disorder, and thus all aected

etuses are male. Its estimated requency is 1 case in 2000 to

5000 births and is associated with delayed labor onset. It also

is associated with development o ichthyosis in aected males

later in lie (Bradshaw, 1986).

Fetal–placental aromatase defciency is a rare autosomal recessive disorder in which individuals cannot synthesize endogenous

estrogens (Grumbach, 2011; Simpson, 2000). Fetal adrenal

DHEA-S is converted in the placenta to androstenedione, but

in cases o placental aromatase deciency, androstenedione cannot be converted to estradiol. Rather, androgen metabolites o

DHEA produced in the placenta, including androstenedione

and some testosterone, are secreted into the maternal or etal

circulation, or both. Tis can virilize the mother and the emale

etus (Belgorosky, 2009; Harada, 1992).

Trisomy 21—Down syndrome—screening searches or

abnormal levels o hCG, alpha-etoprotein, and other analytes

(Chap. 17, p. 338). O these, serum unconjugated estriol levels can be low in women with Down syndrome etuses (Benn,

2002). Tis likely stems rom inadequate ormation o C19 steroids in the adrenal glands o these trisomic etuses.

Fetal erythroblastosis in some cases o severe etal D-antigen

alloimmunization can lead to elevated maternal plasma estrogen levels. A suspected cause is the greater placental mass rom

hypertrophy, which can be seen with such etal hemolytic anemia (Chap. 18, p. 360).

Complete hydatidiorm mole and gestational trophoblastic neoplasias lack a etus and also a etal adrenal source o C19 steroid precursors or trophoblast estrogen biosynthesis. Placental

estrogen ormation is consequently limited to the use o C19Implantation and Placental Development 103

CHAPTER 5

steroids rom the maternal plasma, and thus estradiol is principally produced (MacDonald, 1964, 1966).

■ Maternal Conditions Affecting

Estrogen Production

Maternal conditions can likewise aect placental estrogen production. Glucocorticoid treatment can inhibit ACH secretion

rom the maternal and etal pituitary glands. Tis diminishes

maternal and etal adrenal secretion o the placental estrogen

precursor DHEA-S and leads to a striking reduction in placental estrogen ormation.

With Addison disease, pregnant women show lower estrogen

levels, principally estrone and estradiol levels (Baulieu, 1956).

However, the etal adrenal gland contribution to estriol synthesis, particularly in later pregnancy, is quantitatively much more

important than that o the maternal adrenal gland.

Maternal androgen-producing tumors can present the placenta with elevated androgen levels. Fortunately, placenta is

extraordinarily efcient in the aromatization o C19 steroids.

For example, virtually all androstenedione entering the intervillous space is taken up by syncytiotrophoblast and converted

to estradiol (Edman, 1981). None o the C19 steroid enters

the etus, and a emale etus is rarely virilized by a maternal

androgen-secreting tumor. Indeed, virilized emale etuses o

women with an androgen-producing tumor may be cases in

which a nonaromatizable C19 steroid androgen is produced

by the tumor—or example, 5α-dihydrotestosterone. Alternatively, tumor-derived testosterone could be produced very early

in pregnancy in amounts that exceed the placental aromatase

capacity at that time

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