Chapter 6. Placental Abnormalities. Will Obs

 Chapter 6. Placental Abnormalities

BS. Nguyễn Hồng Anh

During pregnancy, the placenta provides the indispensable

interace between mother and etus (Chap. 5, p. 86). However, in part due to inaccessibility throughout gestation, the

placenta’s anatomy, physiology, and molecular structure still

remain some o the most understudied and intriguing topics in

obstetrics. Furthermore, the parallels between placental ormation and cancer aord opportunities to understand tumor biology and pathogenesis (Costanzo, 2018; Guttmacher, 2014).

Visual placental inspection by the obstetrician is recommended, but routine pathological examination is not mandatory. Indeed, specic conditions that merit submission or

detailed inspection are still debated. For example, the College

o American Pathologists recommends placental examination

or an extensive list o indications, however many providers

are not aware (Langston, 1997; Odibo, 2016). Moreover, data

are insucient to support all o these. At minimum, the placenta and cord should be inspected in the delivery room. Te

decision to request pathological examination should be based

on clinical and placental ndings (Table 6-1) (Redline, 2008;

Roberts, 2008).

NORMAL PLACENTA

At term, the typical placenta weighs 470 g, is round to oval

with a 22-cm diameter, and has a central thickness o 2.5 cm

(Benirschke, 2012). It is composed o a placental disc, extraplacental membranes, and three-vessel umbilical cord. Te disc

surace that lies against the uterine wall is the basal plate, which

is divided by clets into portions—termed cotyledons. Te etal

surace is the chorionic plate. Here, the umbilical cord inserts,

typically in the center. Large etal vessels that originate rom

the cord vessels then spread and branch across the chorionic

plate beore entering stem villi o the placenta parenchyma. In

tracing these, etal arteries almost invariably cross over veins.

Te chorionic plate and its vessels are covered by thin amnion,

which can be easily peeled away rom a postdelivery specimen.

During prenatal sonographic examinations, multiple societies, including the American Institute o Ultrasound in Medicine (2018), recommend identiying and recording placental

location and its relationship to the internal cervical os. As seen

sonographically, the normal placenta is homogenous and 2 to

4 cm thick, lies against the myometrium, and indents into the

amnionic sac. Te retroplacental space is a hypoechoic area that

separates the myometrium rom the basal plate and measures

less than 1 to 2 cm. Te umbilical cord is also imaged, its etal

and placental insertion sites examined, and its vessels counted.

Many placental lesions can be identied grossly or sonographically, but other abnormalities require histopathological

examination or clarication. A detailed description o these

is beyond the scope o this chapter, and interested readers

are reerred to textbooks by Benirschke (2012), Fox (2007),

and Faye-Petersen (2006) and their colleagues. Moreover, the

placenta accreta spectrum, placenta previa, and gestational

TABLE 6-1. Some Indications for Placental Pathological

Examinationa

Maternal Indications

Abruption

Antepartum infection with fetal risks

Anti-CDE alloimmunization

Cesarean hysterectomy

Oligohydramnios or hydramnios

Peripartum fever or infection

Preterm (<32 wks) delivery

Postterm (>42 wks) delivery

Severe trauma

Suspected placental injury

Systemic disorders with known placental effects

Thick meconium

Unexplained late pregnancy bleeding

Unexplained or recurrent pregnancy complications

Fetal and Neonatal Indications

Admission to an acute care nursery

Birth weight <10th or >95th percentile

Fetal anemia

Fetal or neonatal compromise

Neonatal seizures

Hydrops fetalis

Infection or sepsis

Major anomalies or abnormal karyotype

Multifetal gestation

Stillbirth or neonatal death

Vanishing twin beyond the first trimester

Placental Indications

Gross lesions

Markedly abnormal placental shape or size

Markedly adhered placenta

Term cord >32 cm or <100 cm

Umbilical cord lesions

Velamentous cord insertion

aIndications are organized alphabetically.

A B

FIGURE 6-1 Succenturiate lobe. A. Vessels extend from the main

placental disc to supply the small round succenturiate lobe located

to the left. (Reproduced with permission from Dr. Rachel Gardner.)

B. Sonographic imaging with color Doppler shows the main placental disc implanted posteriorly (asterisk). The succenturiate lobe

is located on the anterior uterine wall across the amnionic cavity.

Vessels are identified as the long red and blue crossing tubular

structures that travel within the membranes to connect these two

portions of placenta.Placental Abnormalities 109

CHAPTER 6

an actual hole in the placenta. More oten, only villous tissue is

missing, and the chorionic plate remains intact.

During pregnancy, the normal placenta increases its thickness at a rate that approximates 1 mm per week. Although not

measured as a component o routine sonographic evaluation,

this thickness typically does not exceed 40 mm (Hoddick,

1985). Placentomegaly denes those thicker than 40 mm and

commonly results rom striking villous enlargement. Underlying maternal etiologies are diabetes mellitus or severe anemia,

whereas etal sources include hydrops, anemia, syphilis, toxoplasmosis, or inection caused by parvovirus or cytomegalovirus. In these conditions, the placenta is homogeneously

thickened. In other cases placentas are thick but inhomogeneous. Partial mole is a classic example. Te thickened placenta contains edematous villi, which appear as multiple,

small, anechoic placental cysts (Chap. 13, p. 239). Cystic

vesicles also are seen with placental mesenchymal dysplasia. In

this rare condition, vesicles correspond to enlarged stem villi.

However, unlike molar pregnancy, trophoblast prolieration

is not excessive, and chromosomal complements are diploid

(Woo, 2011).

Rather than villous enlargement, inhomogeneous placentomegaly oten may result rom collections o blood or brin,

which impart heterogeneity to the placenta. Examples o this

are discussed later and include massive perivillous brin deposition, intervillous or subchorionic thromboses, and large retroplacental hematomas (p. 110).

EXTRACHORIAL PLACENTATION

Te chorionic plate normally extends to the periphery o the placental disc and has a diameter similar to that o the basal plate.

With extrachorial placentation, however, the chorionic plate ails to

extend to this periphery and leads to a chorionic plate that is smaller

than the basal plate (Fig. 6-2). Circummarginate and circumvallate

placentas are the two types. In a circummarginate placenta, brin

and old hemorrhage lie between the placental disc and the overlying sheer amniochorion. In contrast, with a circumvallate placenta,

the chorionic plate periphery is a thickened, opaque, gray-white

circular ridge composed o a double old o chorion and amnion.

Sonographically, the circumvallate old can be seen as

a thick, linear band o echoes extending rom one placental

edge to the other. On cross section, however, it appears as two

“shelves,” with each lying above an opposing placental margin

(see Fig. 6-2). Tis anatomy can help dierentiate this shel

rom other bands (Table 6-2).

In small observational studies o circumvallate placenta

diagnosed postpartum, it was associated with increased risk

or antepartum bleeding, abruption, etal demise, and preterm birth (Suzuki, 2008; aniguchi, 2014). In a prospective

sonographic investigation o 17 cases, however, Shen and associates (2007a) ound most circumvallate placentas to be transient. Persistent cases were benign. In general, most otherwise

uncomplicated pregnancies with either type o extrachorial placentation have normal outcomes, and no increased surveillance

is usually required.

CIRCULATORY DISTURBANCES

Functionally, placental perusion disorders can be grouped

into: (1) those in which maternal blood fow to or within the

intervillous space is disrupted, and (2) those with disturbed

etal blood fow through the villi. Tese lesions are requently

identied in the normal, mature placenta. Although they can

limit maximal placental blood fow, unctional reserve within

the placental prevents harm in most cases. Indeed, some estimate that up to 30 percent o placental villi can be lost without

untoward etal eects (Fox, 2007). I extensive, however, these

lesions can prooundly limit etal growth.

Lesions that disrupt perusion are requently seen grossly

or sonographically, whereas smaller lesions are seen only histologically. With sonography, many o these, such as subchorionic brin deposition, perivillous brin deposition, and

intervillous thrombosis, appear as ocal sonolucencies within

the placenta. Greater magnetic resonance (MR) imaging use

in pregnancy has permitted detection and urther characterization o these lesions (Bockoven, 2020; Capuani, 2017).

Importantly, in the absence o maternal or etal complications, small isolated placental sonolucencies are considered

incidental ndings.

A B

FIGURE 6-2 A. In this illustration, circummarginate (left) and circumvallate (right) varieties of extrachorial placentation are shown. A circummarginate placenta is covered by a single layer of amniochorion. B. This transabdominal grayscale sonographic image shows a circumvallate placenta. The double fold of amnion and chorion creates a broad, opaque white ring and ridge on the fetal surface.110 Placentation, Embryogenesis, and Fetal Development

Section 3

■ Maternal Blood Flow Disruption

Subchorionic Fibrin Deposition

Tese collections are caused by slowing o maternal blood fow

within the intervillous space. In the upper portion o this space

near the chorionic plate, blood stasis is prominent and leads

to subsequent brin deposition. In viewing the placental etal

surace, subchorionic lesions are seen as white or yellow, rm,

round, elevated plaques just beneath the chorionic plate.

Perivillous Fibrin Deposition

Stasis o maternal blood fow around an individual villus also

results in brin deposition and can lead to diminished villous

oxygenation and necrosis o syncytiotrophoblast (Fig. 6-3).

Tese small yellow-white placental nodules are grossly visible

within the parenchyma o a sectioned placenta. Within limits,

these refect normal placental aging. Deposition that aects

>25 percent o villi is associated with etal-growth restriction

and adverse neonatal outcomes (Devisme, 2017; Spinillo, 2019).

FIGURE 6-3 Potential sites of maternally and fetally related placental circulatory disturbances.

TABLE 6-2. Sonographic Bands During Pregnancy

Condition Sonographic Findings

Normal early chorioamnionic

separation

Crescent-shaped amnion mirrors the chorion’s curve; distinct from the fetus; fuses after

16 weeks’ gestation

Subchorionic hematoma Echogenic blood lies between the myometrium and chorioamnion, which appears as a

thin band crossing the cavity. Hemorrhage and band resolve over time

Uterine synechiae (Amnionic

sheet)

2.5- to 4.0-mm-thick, broad-based band crosses the cavity. Appears shelflike on cross

section

Circumvallate placenta Broad-based band extends from one placental edge to the other, just above the

placental surface. Appears shelflike on cross section

Amnionic band Thin strands cross and appear to tether fetal parts

Pseudoamnionic band syndrome Thin strands tether fetal parts and form after fetoscopic surgeries or amniocenteses that

are complicated by membrane laceration

Uterine septum Chorioamnionic sac of an early pregnancy fills one horn of a septate or partial

bicornuate uterus. Thick band of echoes, which may be wedge-shaped, extend from

uterine fundus in midline

Membranes from vanishing twin Depending on chorionicity either a thin amnion or thicker chorioamnion spans the cavity

Placental vessels supported by

membranes: velamentous

insertion, succenturiate lobe

With grayscale imaging, vessels appear as bands. Color Doppler will clarify (see Figs. 6-1

and 6-6)

Modified from Dashe, 2017; Lafitte, 2017.Placental Abnormalities 111

CHAPTER 6

Maternal Floor Infarction. Tis extreme variant o perivillous

brin deposition is a dense brinoid layer within the placental

basal plate and is erroneously termed an inarction. Maternal

oor inarction has a thick, yellow or white, rm corrugated

surace that impedes normal maternal blood fow into the intervillous space. In specic cases that extend up and beyond the

basal plate to entrap villi and obliterate the intervillous space,

the term massive perivillous fbrin deposition is used. Te etiopathogenesis is unclear, but maternal auto- or alloimmunity

appears contributory (Faye-Peterson, 2018; Romero, 2013).

Antiphospholipid antibody syndrome and angiogenic actors

involved with preeclampsia also have been implicated (Sebire,

2002; Whitten, 2013).

Tese lesions are not reliably imaged with prenatal sonography, but they may create a thicker basal plate. Aected pregnancies are associated with miscarriage, etal-growth restriction,

preterm delivery, and stillbirth (Andres, 1990; Mandsager,

1994). Importantly, these adverse outcomes can recur in subsequent pregnancies.

Intervillous Thrombus

Tis is a collection o coagulated maternal blood normally ound

in the intervillous space mixed with etal blood rom a break in

a villus. Grossly, these round or oval collections vary in size up

to several centimeters. Tey appear red i recent or white-yellow

i older, and they develop at any placental depth. Intervillous

thrombi are common and typically not associated with adverse

etal sequelae. Tese refect potential communication between

maternal and etal circulations, and thus large lesions are one

cause o elevated maternal serum alpha-etoprotein (MSAFP)

levels (able 17-5, p. 338). (Salaa, 1988).

Infarction

Chorionic villi themselves receive oxygen solely rom maternal circulation and specically rom blood supplied into the

intervillous space. Any uteroplacental disease that diminishes

or obstructs this supply can result in inarction o an individual

villus. Tese are common lesions in mature placentas and are

benign in limited numbers. I numerous, however, placental insuciency can develop. When they are thick, centrally

located, and randomly distributed, they may be associated with

preeclampsia or lupus anticoagulant.

Hematoma

Te maternal–placental–etal unit can develop several hematoma

types. As depicted in Figure 6-3, these include: (1) retroplacental hematoma—ormed between the placenta and its adjacent

decidua; (2) marginal hematoma—ormed between the chorion

and decidua at the placental periphery—known clinically as subchorionic hemorrhage; (3) subamnionic hematoma—derived o etal

vessel origin and ound beneath the amnion but above the chorionic plate, and (4) subchorial thrombus along the roo o the intervillous space and beneath the chorionic plate. With this last type,

massive subchorionic hematomas are also known as Breus moles.

Sonographically, hematomas evolve with time and appear

hyperechoic to isoechoic in the rst week ater hemorrhage,

hypoechoic at 1 to 2 weeks, and nally, anechoic ater 2 weeks.

Most subchorionic hematomas visible sonographically are airly

small and o no clinical consequence (Naert, 2019). However,

extensive retroplacental, marginal, and subchorial collections

are associated with higher rates o miscarriage, stillbirth, placental abruption, and preterm delivery (uuli, 2011). In essence,

placental abruption is a large, clinically signicant retroplacental hematoma.

■ Fetal Blood Flow Disruption

Fetal Vascular Malperfusion

Placental lesions that arise rom etal circulatory disturbances are

also depicted in Figure 6-3. Normally, deoxygenated etal blood

fows rom the two umbilical arteries into arteries within the

chorionic plate. Tese surace arteries divide and send branches

out across the placental surace. Tese eventually supply individual stem villi. Remember that etal blood is oxygenated

within each villus by passive diusion o oxygen rom maternal

blood contained within the intervillous space. Tus, with etal

vessel thrombosis, portions o the villus distal to the obstruction

become nonunctional. Normally, thrombi in limited numbers

are ound in mature placentas. I many villi are aected, which

can be seen with preeclampsia, the etus may suer growth

restriction, stillbirth, or nonreassuring etal heart rate patterns

(Chisholm, 2015; Lepais, 2014; Saleemuddin, 2010).

Villous Vascular Lesions

Villous capillaries show a spectrum o histological lesions. Chorangiosis describes an increased number o capillaries within terminal

villi. Its denition requires ≥10 capillaries to be present in ≥10

villi in ≥10 elds viewed through a 10× lens (Altshuler, 1984).

Clinically, long-standing hypoperusion or hypoxia is thought to

be causative (Stanek, 2016). Focal chorangiosis is increased capillary

vascularity in a signicant portion o the placenta but not diusely.

In one small study, lower Apgar scores and etal vascular malperusion were associated outcomes (Sung, 2019). Prenatal detection o

chorangiosis has been reported (Inubashiri, 2017). Chorangiomatosis describes increased capillary number in stem villi, but terminal villi are spared. Tis nding has been linked with etal-growth

restriction and anomalies (Bagby, 2011). Despite these associations, the clinical signicance o both vascular conditions remains

unclear. Chorioangiomas are described subsequently.

Subamnionic Hematoma

As noted earlier, these hematomas lie between the chorionic

plate and amnion. Tey most oten are acute iatrogenic events

o no clinical consequence during third-stage labor when cord

traction ruptures a vessel near the cord insertion.

Large, chronic antepartum lesions may cause etomaternal

hemorrhage or etal-growth restriction (Deans, 1998). Tey

also may be conused with other placental masses such as chorioangioma. In most cases, color Doppler interrogation will

show absent internal blood fow within a hematoma and permit

dierentiation (Sepulveda, 2000).

PLACENTAL CALCIFICATION

Calcium salts can be deposited throughout the placenta but

are most common on the basal plate. Calcication accrues112 Placentation, Embryogenesis, and Fetal Development

Section 3

with advancing gestation, and greater degrees are associated

with smoking and higher maternal serum calcium levels (Bedir

Findik, 2015). Tese hyperechoic deposits can easily be seen

sonographically, and a grading scale rom 0 to 3 refects increasing calcication with increasing numerical grade (Grannum,

1979). Following this scheme, a grade 0 placenta is homogeneous, lacks calcication, and displays a smooth, fat chorionic

plate. A grade 1 placenta has scattered echogenicities and subtle

chorionic plate undulations. Grade 2 shows echogenic stippling

at the basal plate. Large, echogenic comma shapes originate

rom an indented chorionic plate, but their curve alls short o

the basal plate. Last, a grade 3 placenta has echogenic indentations extending rom the chorionic plate to the basal plate,

which create discrete components that resemble cotyledons.

Basal plate densities also increase.

Tis grading scale poorly predicts neonatal outcome near

term (McKenna, 2005; Mirza, 2018). However, data rom

two small studies link grade 3 placenta prior to 32 weeks with

stillbirth and some other adverse pregnancy outcomes (Chen,

2011, 2015; Mirza, 2018).

PLACENTAL TUMORS

■ Chorioangioma

Tese benign tumors have components similar to the blood

vessels and stroma o the chorionic villus. Also called chorangiomas, these placental tumors have an incidence that

approximates 1 percent (Guschmann, 2003). In some cases,

etal-to-maternal hemorrhage across tumor capillaries leads to

elevated levels o MSAFP. Tis typically prompts sonographic

evaluation to exclude a neural-tube deect, which also shows

high MSAFP levels. Sonographically, chorangiomas appear

as a well-circumscribed, rounded, predominantly hypoechoic

lesion lying near the chorionic plate and protruding into the

amnionic cavity (Fig. 6-4). Documenting increased blood fow

by color Doppler helps to distinguish these lesions rom other

placental masses such as hematoma, partial hydatidiorm mole,

teratoma, metastases, and leiomyoma (Prapas, 2000). Although

rare, chorangiocarcinoma tumors mirror chorioangiomas clinically (Huang, 2015).

Small chorioangiomas are usually asymptomatic. Large

tumors, typically those measuring >4 cm, can create signicant

arteriovenous shunting within the placenta to cause high- output

heart ailure, hydrops, and etal death (Al Wattar, 2014). Compression or shearing o etal erythrocytes within tumor vessels

can lead to hemolysis and microangiopathic anemia (Bauer,

1978). Hydramnios, preterm delivery, and etal-growth restriction are other sequelae (Dong, 2020). Large tumor size and

etal hydrops are the primary determinants and signal a potential adverse perinatal outcome (Buca, 2020).

Grayscale and color Doppler interrogation o the placenta

and amnionic fuid volume are used to identiy these tumors.

Diagnostic tools that can arm associated etomaternal hemorrhage include MSAFP level and Kleihauer-Betke stain (Chap.

18, p. 358). With etal concern, echocardiography assesses cardiac unction, whereas middle cerebral artery interrogation is

used to identiy etal anemia.

Several etal therapies interere with the vascular supply to

the tumor and reverse etal heart ailure. At specialized perinatal centers, endoscopic laser ablation o eeder vessels to the

tumor is most requently used and is associated with avorable

etal outcomes (Hosseinzadeh, 2015). Discussed in Chapter

16, etal transusion can treat serious anemia, amnioreduction

can temporize hydramnios, and digoxin therapy can assist etal

heart ailure.

■ Metastatic Tumors

Maternal malignant tumors rarely metastasize to the placenta.

O those that do, melanomas, leukemias and lymphomas,

A B

FIGURE 6-4 Placental chorioangioma. A. Color Doppler imaging

displays blood flow through a large chorioangioma with its border

outlined by white arrows. B. Grossly, the chorioangioma is a round,

well-circumcised mass protruding from the fetal surface.Placental Abnormalities 113

CHAPTER 6

and breast cancer are the most common (Al-Adnani, 2007).

umor cells usually are conned within the intervillous space.

As a result, metastasis to the etus is uncommon but is most

oten seen with melanoma (Alexander, 2003).

Similarly, cases in which etal malignancy metastasizes to

the placenta are rare (Rei, 2014). Tese are predominantly

etal neuroectodermal tumors, and only one case in the literature describes transplantation o tumor to the maternal uterus

(Nath, 1995).

AMNIOCHORION

■ Chorioamnionitis

Normal genital-tract fora can colonize and inect the membranes, umbilical cord, and eventually the etus. Bacteria most

commonly ascend ater prolonged membrane rupture and during labor to cause inection. Organisms initially inect the chorion and adjacent decidua in the area overlying the internal os.

Subsequently, progression leads to ull-thickness involvement

o the membranes—chorioamnionitis. Organisms oten then

spread along the chorioamnionic surace to colonize and replicate in amnionic fuid. Infammation o the chorionic plate

and o the umbilical cord—unisitis—may ollow (Kim, 2015;

Redline, 2012).

Most commonly, chorioamnionitis is microscopic or

occult and caused by a wide variety o microorganisms. Tis

is requently cited as a possible explanation or many otherwise unexplained cases o ruptured membranes, preterm

labor, or both (Chap. 45, p. 789). In some cases, gross inection is characterized by visible membrane clouding and is

sometimes accompanied by a oul odor that depends on bacterial species.

■ Other Membrane Abnormalities

Amnion nodosum is a condition characterized by numerous

small, light-tan nodules axed to the amnion that overlies the

chorionic plate. Tese may be scraped o the etal surace and

contain deposits o etal squames and brin that refect prolonged and severe oligohydramnios (Adeniran, 2007).

wo notable bands can be ormed by the etal membranes.

First, amnionic band sequence is an anatomical disruption

sequence in which amnion bands tether, constrict, or amputate etal parts. Bands may orm spontaneously or ollow etal

surgery procedures (see able 6-2) (Latte, 2017). Amnionic

bands commonly cause limb-reduction deects, acial clets,

or encephalocele (Barzilay, 2015; Guzmán-Huerta, 2013).

Umbilical cord compromise is another sequela (Barros, 2014).

Severe deects o the spine or ventral wall that accompany

amnionic bands suggest a limb-body wall complex, described in

Chapter 15 (p. 297).

Sonography oten rst identies the sequelae o this

sequence rather than the bands themselves. As with any etal

anomaly, targeted sonography is indicated. Identication o a

limb-reduction deect, an encephalocele in an atypical location,

or an extremity with edema or positional deormity should

prompt careul evaluation or amnionic bands.

Management depends on the degree o anatomic deormity

(Society o Maternal–Fetal Medicine, 2019). Fetoscopic laser

interruption o the band may be suitable in highly selected

antepartum cases (Gueneuc, 2019; Javadian, 2013).

Second, an amnionic sheet in contrast is ormed by normal

amniochorion draped over a preexisting uterine synechia. Generally, these sheets pose little etal risk, although slightly higher

rates o preterm membrane rupture and placental abruption

have been described (Nelson, 2010; uuli, 2012).

UMBILICAL CORD

■ Length

Most umbilical cords at delivery measure 40 to 70 cm long, and

very ew measure <30 cm or >100 cm. Cord length is infuenced positively by both maternal parity and body mass index

(Linde, 2018). In retrospective studies, short cords have been

linked with congenital malormations and intrapartum distress

(Krakowiak, 2004; Linde, 2018; Yamamoto, 2016). Excessively

long cords are linked with cord entanglement or prolapse and

with etal anomalies (Olaya-C, 2015; Rayburn, 1981).

Because antenatal determination o cord length is technically limited, cord diameter has been evaluated as a predictive

marker or etal outcomes. Some have linked lean cords with

poor etal growth and large-diameter cords with macrosomia

(Proctor, 2013). However, the clinical utility o this parameter

is still unclear (Cromi, 2007; Raio, 2003).

■ Coiling

Cord coiling characteristics are not currently part o standard

sonographic evaluation. Usually the umbilical vessels spiral

through the cord in a sinistral, that is, let-twisting direction

(Fletcher, 1993; Lacro, 1987). Te number o complete coils

per centimeter o cord length is termed the umbilical coiling

index—UCI (Strong, 1994). A normal, antepartum, sonographically derived UCI is 0.4, and this contrasts with a normal, postpartum, physically measured value o 0.2 (Sebire,

2007). UCIs <10th percentile are considered hypocoiled, and

those >90th percentile are hypercoiled.

Clinically, the signicance o coiling extremes is controversial. Some studies evaluating large, unselected cohorts nd no

associations between UCI values and poor neonatal outcome

(Jessop, 2014; Pathak, 2010). In others, extremes are linked

with various adverse outcomes but most consistently with

intrapartum etal heart rate abnormalities, preterm labor, or

etal-growth restriction (Chitra, 2012; de Laat, 2006; Pergialiotis, 2019).

■ Vessel Number

Counting cord vessel number is a standard component o anatomical evaluation during etal sonographic examination and

immediately ater delivery (Fig. 6-5). Embryos initially have

two umbilical veins. In the rst trimester, the right vein typically atrophies to leave one large vein to accompany the two,

thick-walled umbilical arteries. Four-vessel cords are rare and

oten associated with congenital anomalies (Puvabanditsin,114 Placentation, Embryogenesis, and Fetal Development

Section 3

2011). I it is an isolated nding, however, prognosis can be

good (Avnet, 2011).

Te most common aberration is that o a single umbilical

artery (SUA). Its cited incidence is 0.63 percent in liveborn neonates, 1.92 percent in perinatal deaths, and 3 percent in twins

(Heietz, 1984). Fetuses with major malormations requently

have an SUA. Tus, its identication oten prompts consideration

or targeted sonography and possibly etal echocardiography. Te

most requent anomalies are cardiovascular and genitourinary

(Hua, 2010; Murphy-Kaulbeck, 2010). In an anomalous etus,

an SUA greatly increases the aneuploidy risk, and amniocentesis

is recommended or karyotype assessment (Dagklis, 2010).

I targeted sonography nds otherwise normal anatomy, an

isolated SUA in an otherwise low-risk pregnancy does not signicantly raise the etal aneuploidy risk. However, as an isolated

nding, it has been associated with etal-growth restriction and

perinatal death in some but not all studies (Chetty-John, 2010;

Ebbing, 2019; Voskamp, 2013). Tus, clinical monitoring o

growth is reasonable, but the value o sonographic surveillance

is unclear.

In contrast, a used umbilical artery with a shared lumen

is rare. It arises rom ailure o the two arteries to split during

embryological development. Te common lumen may extend

through the entire cord, but, i partial, it is typically ound

near the placental insertion site (Yamada, 2005). In one report,

these malormations were associated with a higher incidence o

marginal or velamentous cord insertion but not o congenital

etal anomalies (Fujikura, 2003).

Found in most placentas, the Hyrtl anastomosis is a connection between the two umbilical arteries, and it lies near the

cord’s insertion into the placenta. Tis anastomosis acts physiologically to equalize pressures between the arteries (Gordon,

2007). Te resulting redistribution o pressure gradients and

blood fow improves placental perusion, especially during uterine contractions or during compression o one umbilical artery.

Fetuses with an SUA lack this saety valve (Raio, 1999, 2001).

■ Remnants and Cysts

Several structures are housed in the umbilical cord during

etal development, and their remnants may be seen when the

mature cord is inspected transversely. Indeed, in grossly sectioned cords, remnants o the allantoic duct, vitelline duct, and

embryonic vessels are ound in 25 to 50 percent (Grottling,

2019; Jauniaux, 1989). Tese are not associated with congenital malormations or perinatal complications.

Cysts occasionally are ound along the course o the cord.

Tey are designated according to their origin. True cysts are

epithelium-lined remnants o the allantoic or vitelline ducts

and tend to be located closer to the etal insertion site. In contrast, the more common pseudocysts orm rom local degeneration o Wharton jelly and occur anywhere along the cord. Both

have a similar sonographic appearance. Single umbilical cord

cysts identied in the rst trimester tend to resolve completely,

however, multiple cysts may portend miscarriage or aneuploidy

(Ghezzi, 2003; Hannaord, 2013). Cysts persisting beyond this

time are associated with a risk or structural deects and chromosomal anomalies (Bonilla, 2010; Zangen, 2010).

■ Insertion

Te cord normally inserts centrally into the placental disc, but

eccentric, marginal, or velamentous insertions are variants.

O these, eccentric insertions in general pose no identiable

etal risk. Marginal insertion is a common variant—sometimes

reerred to as a battledore placenta—in which the cord anchors

at the placental margin. In one population-based study, the

rate was 6 percent in singleton gestations and 11 percent in

twins (Ebbing, 2013). Tis common insertion variant rarely

causes problems, but it and velamentous insertion occasionally result in the cord being pulled o during delivery o the

placenta (Ebbing, 2015; Luo, 2013). In monochorionic twins,

marginal insertion may be associated with weight discordance

(Kent, 2011).

A B

FIGURE 6-5 Two umbilical arteries are typically documented sonographically in the second trimester. They encircle the fetal bladder

(asterisk) as extensions of the superior vesical arteries. A. In this color Doppler sonographic image, a single umbilical artery, shown in red,

runs along the bladder wall before joining the umbilical vein (blue) in the cord. Remember with color Doppler that color signifies only

blood flow direction relative to the transducer. B. A cross section of a floating cord segment shows the two vessels of the cord. The smaller

circle is the single umbilical artery and the larger circle, the umbilical vein.Placental Abnormalities 115

CHAPTER 6

With velamentous insertion, the umbilical vessels characteristically travel within the membranes beore reaching the

placental margin (Fig. 6-6). Te incidence o velamentous

insertion approximates 1 percent but is 6 percent with twins

(Ebbing, 2013). It is more commonly seen with placenta previa (Papinniemi, 2007; Räisänen, 2012). Antenatal diagnosis

is possible sonographically, and cord vessels are seen traveling

along the uterine wall beore entering the placental disc. Clinically, vessels are vulnerable to compression, which may lead

to etal hypoperusion and acidemia. Higher associated rates

o low Apgar scores, stillbirth, preterm delivery, and small or

gestational age have been noted (de Los Reyes, 2018; Ebbing,

2017; Esako, 2015; Vahanian, 2015). Accordingly, monitoring o etal growth is reasonable either clinically or sonographically (Vintzileos, 2015).

Last, with the rare urcate insertion, umbilical vessels lose

their protective Wharton jelly shortly beore they insert. As a

result, they are covered only by an amnion sheath and prone to

compression, twisting, and thrombosis.

Vasa Previa

With this condition, vessels travel within the membranes and

overlie the cervical os. Tere, they can be torn with cervical dilation or membrane rupture, and laceration can lead to

rapid etal exsanguination. Over the cervix, vessels can also be

compressed by a presenting etal part (Matsuzaki, 2019). Vasa

previa may be more common than previously estimated, and

rates are 1 case in 338 to 365 pregnancies (Hasegawa, 2012;

Klahr, 2019). Vasa previa is classied as type 1, in which vessels are part o a velamentous cord insertion, and type 2, in

which involved vessels span between portions o a bilobate or

a succenturiate placenta (Catanzarite, 2001). wo other risks

are conception with in vitro ertilization and second-trimester

placenta previa, with or without later migration (Baulies, 2007;

Schachter, 2003).

Compared with intrapartum diagnosis, antepartum diagnosis greatly improves the perinatal survival rate, which ranges

rom 97 to 100 percent (Oyelese, 2004; Swank, 2016; Zhang,

2021). Tus, vasa previa is ideally identied early, although this

is not always possible. Eective screening or vasa previa begins

during scheduled midtrimester sonographic examination. In

suspicious cases, transvaginal sonography is added and shows

cord vessels inserting into the membranes and vessels running

above the cervical internal os (Fig. 6-7). Routine color Doppler

interrogation o the placental cord insertion site, particularly

in cases o placenta previa or low-lying placenta, may aid its

detection. With this, the vessel waveorm refects the etal heart

rate. In one systematic review, the median prenatal detection

rate was 93 percent (Ruiter, 2015).

Once vasa previa is identied, subsequent imaging is reasonable because up to 39 percent o cases ultimately resolve (Erani,

2019; Klahr, 2019). Bed rest apparently has no added advantage. Antenatal corticosteroids can be provided as indicated or

given prophylactically at 28 to 32 weeks’ gestation to cover possible urgent preterm delivery. Antenatal hospitalization may be

considered at 30 to 34 weeks to permit surveillance and expedited delivery or labor, bleeding, or rupture o membranes.

Data supporting this are limited, and admission may best serve

women with risk actors that portend early delivery (Society

or Maternal-Fetal Medicine, 2015). A ew cases o antepartum etoscopic surgery with vessel laser ablation are described

(Hosseinzadeh, 2015; Johnston, 2014). However, current practice is early scheduled cesarean delivery. Te American College

o Obstetricians and Gynecologists (2021) recommends cesarean delivery at 34 to 36 weeks’ gestation.

At delivery, the etus is expeditiously delivered ater the hysterotomy incision in case a vessel is lacerated during uterine

entry. Delayed cord clamping is not encouraged.

In all pregnancies, otherwise unexplained vaginal bleeding

either antepartum or intrapartum should prompt consideration

o vasa previa and a lacerated etal vessel. In many cases, bleeding is rapidly atal, and neonatal salvage is not possible. With

less hemorrhage, however, it may be possible to distinguish

etal versus maternal bleeding. Various tests can be used, and

A B

FIGURE 6-6 Velamentous cord insertion. A. The umbilical cord inserts into the membranes (arrow). From here, the cord vessels branch

and are supported only by membrane until they reach the placental disc. B. When viewed sonographically and using color Doppler, the

cord vessels appear to lie against the myometrium as they travel to insert into the margin of the placental disc (P).116 Placentation, Embryogenesis, and Fetal Development

Section 3

each relies on the increased resistance o etal hemoglobin to

denaturing by alkaline or acid reagents (Odunsi, 1996).

■ Knots, Strictures, and Loops

Various mechanical abnormalities in the cord can impede

blood fow and sometimes cause etal harm. O these, true knots

are ound in approximately 1 percent o births. Tese orm

rom etal movement, and associated risks include hydramnios

and diabetes mellitus (Hershkovitz, 2001; Räisänen, 2013).

Knots are especially common and dangerous in monoamnionic

twins, which are discussed in Chapter 48 (p. 845). In singleton

etuses, the stillbirth risk is increased our- to tenold compared

with those without knots (Airas, 2002; Sørnes, 2000).

Knots can be ound incidentally during antepartum sonography, and a “hanging noose” sign is suggestive (Ramon y

Cajal, 2006). Tree-dimensional and color Doppler aid diagnostic accuracy (Hasbun, 2007). With these knots, optimal

etal surveillance is unclear but may include umbilical artery

Doppler velocimetry, nonstress testing, or subjective etal

movement monitoring (Rodriguez, 2012). Allowing vaginal

delivery is suitable, and intrapartum etal heart rate tracings do

not dier rom unaected pregnancies (Carter, 2018). In these

cases, cesarean delivery rates are not increased and cord blood

acid-base values are usually normal (Airas, 2002; Maher, 1996).

In contrast, alse knots orm rom ocal redundancy and olding o an umbilical cord vessel rather than knotting. Tese lack

clinical signicance.

A cord stricture is a ocal narrowing o the diameter that usually develops near the etal cord insertion site (Peng, 2006).

Pathological eatures typically include absent Wharton jelly at

the narrowed segment and obliteration o cord vessels (Sun,

1995). In most instances, the etus is stillborn (French, 2005).

Even less common is a cord stricture caused by an amnionic

band.

Cord loops are requently encountered and are caused by coiling around various etal parts during movement. A cord around

the neck—a nuchal cord—is common, and vaginal delivery is

suitable. One loop is reported in 20 to 34 percent o deliveries;

two loops in 2.5 to 5 percent; and three loops in 0.2 to 0.5 percent (Kan, 1957; Sørnes, 1995; Spellacy, 1966). During labor,

up to 20 percent o etuses with a nuchal cord have moderate to

severe variable heart rate decelerations, and these are associated

with a lower umbilical artery pH (Hankins, 1987). Decelerations are not relieved by amnioinusion (Spong, 1996). Cords

wrapped around the body can have similar eects (Kobayashi,

2015). Despite their requency, nuchal cords are not associated

with greater rates o adverse perinatal outcome (Henry, 2013;

Masad, 2019).

Last, in a unic presentation, the umbilical cord is the presenting part. Tese are uncommon and most oten are associated

with etal malpresentation (Kinugasa, 2007). A unic presentation in some cases is identied with placental sonography and

color fow Doppler (Ezra, 2003). Overt or occult cord prolapse

can complicate labor. Tus, once identied at term, cesarean

delivery is typically recommended.

■ Vascular

Cord hematomas are rare and generally ollow rupture o an

umbilical vessel, usually the vein, and hemorrhage into the

Wharton jelly. Hematomas have been associated with abnormal

cord length, umbilical vessel aneurysm, trauma, entanglement,

umbilical vessel venipuncture, and unisitis (Gualandri, 2008).

Most are identied postpartum, but hematomas are recognized

A B

FIGURE 6-7 Vasa previa. A. Using color Doppler, an umbilical

vessel (red linear structure) is seen overlying the internal os and cervical canal (arrows). At the bottom, the Doppler waveform seen with

this vasa previa has the typical appearance of an umbilical artery.

B. The amniotomy site (held open by hemostat), which was created

at the time of cesarean hysterotomy, illustrates how fetal vessels

may be lacerated and why fetal delivery should be prompt (Reproduced with permission from Dr. Julie Lo.)Placental Abnormalities 117

CHAPTER 6

sonographically as hypoechoic masses that lack blood fow

(Chou, 2003). Sequelae include stillbirth or intrapartum abnormal etal heart rate pattern (Abraham, 2015; Barbati, 2009;

Sepulveda, 2005; owers, 2009). However, case reports have

described normal outcomes (Sanchex-Codez, 2018).

Umbilical cord vessel thromboses are rare in utero events and

seldom diagnosed antepartum. Approximately 70 percent are

venous, 20 percent are venous and arterial, and 10 percent are

arterial thromboses (Heietz, 1988). Tese all have high associated rates o stillbirth, etal-growth restriction, and intrapartum

etal distress (Minakami, 2001; Sato, 2006; Shilling, 2014). I

these are identied antepartum as hypoechoic masses without

blood fow, data rom case reports support consideration o

prompt delivery o viable-aged etuses (Kanenishi, 2013).

An umbilical vein varix can complicate either the intraamnionic or etal intraabdominal portion o the umbilical vein.

Sonographically and complemented by color Doppler, rare

intraamnionic varices show cystic dilation o the umbilical vein

that is contiguous with a normal-caliber portion. O complications, an intraamnionic varix may compress an adjacent umbilical artery or can rupture or thrombose. A systematic review

o 250 cases ound that approximately one th is associated

with other anomalies. Isolated cases had reassuring outcomes

but typically required antenatal surveillance (di Pasquo, 2018).

Te rare umbilical artery aneurysm is caused by congenital thinning o the vessel wall with diminished support rom

Wharton jelly. Indeed, most orm at or near the cord’s placental insertion site, where this support is absent. Tese are associated with SUA, trisomy 18, amnionic fuid volume extremes,

etal-growth restriction, and stillbirth (Hill, 2010; Vyas, 2016).

At least theoretically, these aneurysms could cause etal compromise and death by compression o the umbilical vein.

With aneurysms measuring >5 cm, the blood reservoir within

the aneurysm may pose a risk or high-output heart ailure

(Matsuki, 2017). Tese aneurysms may appear sonographically

as a cyst with a hyperechoic rim. Within the aneurysm, color

fow and spectral Doppler interrogation demonstrate either

low-velocity or turbulent nonpulsatile fow (Olog, 2011; Shen,

2007b). Although not codied, management may include etal

karyotyping, antenatal etal surveillance, and early delivery to

prevent stillbirth (Doehrman, 2014). Some recommend delivery by cesarean to avoid aneurysm rupture

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