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1078 PART IV Obstetric and Fetal Sonography

and partial), invasive mole, choriocarcinoma, and placental

trophoblastic tumor. 133

Hydatidiform Molar Pregnancy

Hydatidiform molar pregnancy is the most common and benign

form of gestational trophoblastic disease, with an incidence of

1 in 1000 pregnancies in North America. 134 he incidence is

much higher in the Asian population. here is an increased risk

in teenagers, in women older than 35 years of age, and in women

with a previous molar pregnancy. he risk also increases with

the number of previous spontaneous abortions. 135 Molar pregnancy

is characterized histologically by cystic (hydatidiform)

degeneration of chorionic villi, with absent or inadequate vascularization

and abnormal trophoblastic proliferation. Embryos

or fetuses are either absent or abnormal.

he most frequent presenting symptom is vaginal bleeding,

which occurs in more than 90% of cases. Passage of vesicles

(hydropic villi) through the vagina occurs frequently and is

considered speciic for the diagnosis of molar pregnancy. 136 he

uterus may be enlarged for dates, and there may also be rapid

uterine enlargement. Medical complications include pregnancyinduced

hypertension, hyperemesis gravidarum, preeclampsia,

and hyperthyroidism. he routine use of ultrasound for any

woman with bleeding in pregnancy allows for early diagnosis,

and few women in current practice show the classic features of

hyperemesis and preeclampsia. 137

Serum β-hCG levels in molar pregnancy are abnormally

elevated, usually greater than 100,000 mIU/mL. he ovaries may

be greatly enlarged in complete molar pregnancy by multiple,

bilateral theca lutein cysts. hese are large, usually multilocular,

and may undergo hemorrhage or torsion and can be a source

of pelvic pain. heca lutein cysts are most marked when trophoblastic

proliferation is severe, and when hCG is elevated.

hey are seen much less oten in the irst trimester. 138,139

Molar pregnancy is treated by uterine evacuation, which

is adequate in most patients. Approximately 80% of complete

moles and 95% of partial moles will subsequently follow a

benign course. 136,140 However, accurate diagnosis and classiication

of molar pregnancy are important because of the risk

of persistent trophoblastic neoplasia (PTN). he American

College of Obstetrics and Gynecology currently recommends

follow-up testing for hCG for 6 months ater levels become

undetectable. 141

Hydatidiform molar pregnancy is classiied as either complete

molar pregnancy or partial molar pregnancy on the basis of

cytogenetic and pathologic features.

Complete Molar Pregnancy

Complete molar pregnancy is characterized by a diploid karyotype

of 46,XX in 80% to 90% of cases, with the chromosomal

DNA being exclusively paternal in origin. 142 his occurs when

an ovum with absent or inactive maternal chromosomes is fertilized

by a normal haploid sperm. Occasionally, fertilization of

an empty ovum by two haploid sperm results in a 46,XY pattern. 135

As the embryo dies at an early stage, no fetal parts are seen. 142

he placenta is entirely replaced by abnormal, hydropic chorionic

villi with excessive trophoblastic proliferation.

he classic sonographic features of complete molar pregnancy

include an enlarged uterus with a central heterogeneous echogenic

mass that expands the endometrial canal. he mass contains

multiple cystic spaces of varying size, representing the hydropic

villi (Fig. 30.38). hese cystic spaces may vary in size from a few

millimeters to 2 to 3 cm. In the second trimester, TAS diagnosis

is highly accurate. he second trimester appearance typically

presents as an echogenic mass containing multiple tiny cysts

due to the hydropic villi in what used to be termed a “snowstorm”

pattern. However, with the higher frequencies now used the

“snowstorm” has become a “bunch of grapes.”

In the irst trimester, molar pregnancies have a variable

appearance on ultrasound, sometimes appearing as a predominantly

solid, echogenic mass or even as an intrauterine luid

collection because tiny hydropic villi may not be adequately

resolved (Fig. 30.39). In these cases, a molar pregnancy may not

be able to be diferentiated from a miscarriage.

Partial Molar Pregnancy

Partial molar pregnancy has a triploid karyotype of 69,XXX,

69,XXY, or 69,XYY and is also referred to as “triploidy.” Most

partial moles have one set of maternal chromosomes and two

sets of paternal chromosomes, resulting from fertilization of a

normal ovum by two haploid sperm. Triploidy of maternal origin

is not associated with gestational trophoblastic disease. 143 Pathologically,

partial molar pregnancy has well-developed but generally

anomalous (triploid) fetal tissues. Hydropic degeneration of

placental villi is focal, interspersed with normal placental villi

(Fig. 30.40).

Triploidy is discussed in more detail in Chapter 31.

Placental hydropic degeneration (unrelated to trophoblastic

neoplasia) may also show similar sonographic features. Hydropic

degeneration occurs frequently in irst-trimester abortion of any

cause. he associated cystic spaces may be diicult or impossible

to diferentiate from an early mole. herefore in cases with

equivocal ultrasound features, the products of conception should

be carefully evaluated to avoid missing a hydatidiform mole.

Recent studies have shown that sonography is much more

accurate in diagnosing complete molar pregnancy than partial

molar pregnancy. Kirk et al. 144 evaluated sonography in the irst

trimester and found an accuracy of 95% for the diagnosis of

complete mole and 20% for partial mole, with an overall accuracy

for hydatidiform mole of 44%. In 859 pathologically diagnosed

hydatidiform moles, Fowler et al. 145 found that sonography

performed in the irst and early second trimester had a similar

44% overall accuracy and an accuracy of 79% and 29% for

complete and partial moles, respectively. Also, the sonographic

detection rate improved ater 14 weeks’ gestation.

Coexistent Hydatidiform Mole and Normal Fetus

In complete moles, an embryo or fetus is absent except in the

rare event of a coexistent twin pregnancy. hese twin pregnancies

with an apparently normal fetus and a hydatidiform mole are

uncommon, with an estimated incidence of 1 in 20,000 to 100,000

pregnancies. 146,147 It is diferentiated from a partial molar pregnancy

by identifying a normal-appearing fetus with a corresponding

normal placenta adjacent to a mass of placental tissue that

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