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and intrauterine bleeding with formation of a subchorionic haematoma (see below). Circumvallate placentation is therefore accompanied by a risk of premature rupture of the membrane, vaginal bleeding and preterm onset of labour. This form of placen­tation is also associated with an increased incidence of low-birthweight infants.
9,13
The obstetric risks linked with circumvallate placentas has never been eval­uated prospectively. Multiple subamniotic sonolucent areas of various size and shape, located in the periphery of the placenta, are the main ultrasound features of this form of placentation.9 However, the accuracy of sonography of the pla­centa for revealing circumvallation appears to be limited.
8
Placenta accreta
This has been defined as a placenta with abnormal adherence, either in whole or in part, to the uterine wall.
6,16
This abnormality is characterized by myometrial invasion by the villi and occurs when the decidua basalis is partially or completely absent. According to the degree of myometrial invasion, this condition is subdi­vided into placenta accreta vera, when the villi are simply attached to the myome­trium, placenta increta, when the villi deeply invade the myometrium, and placenta percreta, when the villi penetrate the entire thickness of the uterine wall.
16
Placenta accreta is a rare but very serious abnormality. All conditions or pro­cedures which affect the integrity of the internal uterine walls, such as caesarean section and other uterine surgery, curettage, sepsis or fibroids, are predisposing factors for abnormal villous penetration.6 In many patients there is a combina­tion of aetiological factors and the association of high parity with prior cae­sarean scars and anterior low placental insertion (praevia) is a particular risk. Placentas accreta have an overall maternal and fetal mortality of around 10% due to antepartum or postpartum bleeding, uterine rupture and uterine inver­sion. Placenta percreta is clearly the most dangerous condition, with a perina­tal mortality rate approaching 96%.
6,16
On ultrasound, the decidual interface between placenta and myometrium (hypoechoic retroplacental zone) is absent at the level of the abnormal villous penetration.
4,6,16,29
Most placentas accreta can be detected as early as 11–14 weeks of gestation in most at-risk patients by visualization of irregular vascular spaces within the placenta basal area.4 Colour Doppler sonography highlights areas of increased vascularity with dilated blood vessels crossing the placenta and uterine wall.
16,29
In addition, in placenta percreta, an irregular uterine serosa is found in grey­scale ultrasonography and thinning of the uterine wall, found in magnetic reso­nance imaging, contributes to the diagnosis.29 The diagnosis of placenta accreta is rarely achieved antenatally by routine ultrasound examination.6 However, by contrast with placenta circumvallate, the prenatal diagnosis of this condi­tion allows the surgical team to demarcate which areas of the placenta are accreta, increta or percreta before surgery and prepare for a caesarean hyster­ectomy and more extensive pelvic surgery if a partial resection is impossible. This approach has not been investigated prospectively on many patients but there is little doubt that prenatal diagnosis of major placenta increta and per­creta will reduce fetal and maternal perinatal mortality allowing transfer to centres that are equipped for major pelvic surgery and intensive care.
Assessment of the placenta and umbilical cord
123
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Placental tumours
Mesenchymal tumours
Chorioangioma or placental hemangioma is the most common benign tumour of the placenta with an incidence at delivery of 0.5–1% of placentas examined. The incidence of large chorioangiomas is lower and varies from 1 in 8000 to 1 in 50,000 pregnancies.16 Chorioangiomas are hamartomas, which arise as a malfor­mation of the primitive angioblastic tissue of the placenta.
There are two main histopathological types of chorioangiomas: angiomatous, which are formed of numerous blood vessels, and cellular, which consist of loose mesenchymal tissue, containing a few ill-formed vessels. Degenerative changes such as necrosis, calcification, hyalinization or myxoid changes are frequently present in large tumours.9 Most chorioangiomas are small, single, round, encapsulated and intra­placental. They are occasionally observed during routine ultrasound examination14 and are likely to be discovered only by histopathological examination. Large chorio­angiomas are of variable shape, divided by fibrous septa, and most commonly pro­trude from the fetal surface of the placenta near the cord insertion.9 These tumours
Ultrasound in obstetrics and gynaecology
are well circumscribed, have a different echogenicity from the rest of the placental tissue and have been documented sonographically from 16 weeks of gestation.14 Chorioangiomas can be complicated by fetal hydrops due to the chronic shunting of large volumes of fetal blood through the tumour or to polyhydramnios. development of polyhydramnios is independent of the size of the tumour but rather linked to its vascular nature.
14,30
The fetal risk depends more on the proportion of
angiomatous versus myxoid tissue inside the tumour than on its exact size.
Thus ultrasound examination of the vascularization of the tumour is a pivotal determining factor of pregnancy outcome (Fig. 7.1). If the tumour is avascular, no specific complications should be expected. If the tumour is vascularized, and in particular if it contains numerous large vessels, serial ultrasound and Doppler examinations are warranted to detect polyhydramnios and early features of fetal congestive heart failure. Novel intrauterine treatment options include intravas­cular transfusion, fetoscopic laser devascularization, microcoil embolization, and
9,16
9,14
The
14
124
Fig. 7.1 Heterogeneous vascular placental mass at 30 weeks, protruding from the fetal plate near the cord insertion (star). The pregnancy was complicated by polyhydramnios. Pathological examination demonstrated a vascular chorioangioma.
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intravascular injection of absolute alcohol. Quantitative flow data obtained using three-dimensional power Doppler may indicate altered haemodynamics in the tumour which can influence the management.
28
Gestational trophoblastic tumours (GTD)
Hydatidiform transformation (Fig. 7.2) of the villous tissue is a common finding in placental trophoblastic tumours. Complete or classic hydatidiform moles (CHM) are described as a generalized swelling of the villous tissue, diffuse trophoblas­tic hyperplasia and no embryonic or fetal tissue.3 Classically, patients with CHM present with vaginal bleeding, uterine enlargement greater than expected for ges­tational age and abnormally high level of maternal serum human chorionic gonado­tropin (MShCG). Medical complications include pregnancy-induced hypertension (PIH), hyperthyroidism, hyperemesis, anaemia and the development of ovarian theca-lutein cyst. With earlier diagnosis, the incidence of these complications has decreased. Molar changes can now be detected from the second month of preg­nancy by ultrasound which typically reveals a uterine cavity filled with multiple sonolucent areas of varying size and shape (‘snow-storm appearance’) without asso­ciated embryonic or fetal structure.
18,21
Theca-lutein cysts secondary to the very high MShCG levels may be diagnosed in up to 30% of cases producing either soap bubble or spoke wheel appearance of the ovaries, which are enlarged. Elevated
Assessment of the placenta and umbilical cord
Fig. 7.2 Typical ‘Swiss cheese’ appearance of the placenta on ultrasound corresponding to hydatidiform transformation in a partial mole.
125
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MShCG levels, combined with these specific sonographic features, are highly indic­ative of the presence of hydatidiform mole, even before the final histopathological diagnosis is confirmed. Ultrasound features of CHM may be different at earlier ges­tations and accuracy of diagnosis varies between studies. The ultrasound diagnosis of complete mole usually poses little problem from the third month of pregnancy onward and can be made prenatally in around 80% of cases.
2,13,29
Partial hydatidiform moles (PHM) refer to the combination of a fetus with localized placental molar degenerations, characterized by focal swelling of the villous tissue – focal trophoblastic hyperplasia.19 The abnormal villi are being scattered within macroscopically normal placental tissue, which tends to retain its shape. Partial moles are usually triploid and of diandric origin, having two sets of chromosomes from paternal origin and one from maternal origin. Most have a 69,XXX or 69,XXY genotype derived from a haploid ovum with either reduplication of the paternal haploid set from a single sperm or, less frequently, from dispermic fertilization. Triploidy of digynic origin, due to a double mater­nal contribution, is not associated with placental hydatidiform changes. Vaginal bleeding in the first or second trimester with a total incidence of 47% is the most
Ultrasound in obstetrics and gynaecology
common maternal symptom reported in both types of triploidies.19 The pheno­typic expression of both diandric and digynic triploidies includes growth restric­tion and disturbance of organogenesis that becomes obvious in fetuses surviving into the second trimester. From 16 weeks, almost all triploid fetuses have at least one measurement below the normal range and more than 70% present with severe growth restriction.19 Structural fetal defects are observed antenatally in about 93% of cases. The most common are abnormalities of the hands, bilateral cerebral ventriculomegaly, heart anomalies and micrognathia. Triploid partial moles are not associated with specific fetal anomaly but almost always with symmetrical growth restriction. Classically, triploid partial mole presents on ultrasound as an enlarged placenta (thickness >4 cm at 18–22 weeks) containing multicystic avas­cular sonolucent spaces (see Fig. 7.2) – ‘Swiss cheese’ appearance.
Following uterine evacuation, 18–29% of patients with a CHM and 1% of those with a PHM will develop a persistent trophoblastic tumour.
3,19
If the incidence of maternal complication has been reduced by an early diagnosis, the incidence of per­sistent GTD has remained unchanged since the introduction of routine ultrasound examination during pregnancy. This highlights the importance of training sonogra­phers working in early pregnancy units in the detection of placental molar changes as many cases of complete and partial moles will present as miscarriages. MShCG is significantly higher in both CHM and PHM and, in conjunction with transvaginal ultrasound, may provide the screening test required.
126

SECONDARY ABNORMALITIES

Vascular abnormalities
Thrombosis and infarcts
Both lesions are usually found during the third trimester of pregnancy.1 Placental thromboses are the result of focal coagulation of blood in the intervillous spaces
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and occur more frequently in pregnancies complicated by rhesus isoimmuniza-
9,15,17
tion.
Large hypoechoic areas with low flow laterally and relatively high flow in the central part on real-time imaging can be observed in the early stages of the development of an intervillous thrombosis. Abnormal haemodynamic flow in the intervillous space may result from the failure of the cotyledon to expand in response to the increasing flow of the corresponding uteroplacen­tal artery and compression of the surrounding villi which gradually atrophy as fibrin is laid down in the periphery.15 This process causes a progressive increase of the echogenicity of the lesion. Finally the maternal blood coagulates in the placental tissue, obliterating, focally, the intervillous circulation.
Placental infarcts are the result of obstruction of a uteroplacental artery leading to
focal degeneration of the overlying villous tissue.
15,17
Extensive infarcts are found in preg­nancies complicated by pre-eclampsia or essential hypertension and are associated with an increase in perinatal mortality and intrauterine growth retardation. Sonographically, placental infarcts appear as large intraplacental areas, irregular and hyperechoic in the acute stage and isoechoic in a more advanced stage.
9,17
Identifying thrombo-occlusive placental lesions before the development of pregnancy complications may prove useful in the design of trials to study the effectiveness of heparin in the prevention of clinical complications resulting from thrombo-occlusive uteroplacental disease.
9
Haematomas
Extravasation of maternal or fetal blood can result in a localized collection of blood or haematoma forming in the placenta. Such lesions may be subamniotic, subchorionic or retroplacental and can be identified on prenatal ultrasound examination.
5,20
The terminology used to describe placental vascular lesions is confusing, with ultrasound descriptions often unrelated to the pathological findings. Various terms such as subchorionic cyst, membranous cyst, thrombotic cyst and subchorionic haemorrhage have been used in the literature to describe lesions of the fetal plate of the placenta. A subchorionic or retroplacental haematoma reflects bleeding of maternal origin and is identified sonographically as a hypoechoic area between the chorion and uterine wall. Such lesions are seen in more than 10% of pregnancies, commonly in the first trimester, and may carry an increased risk of miscarriage, still­birth, preterm labour and abruptio placentae.
9,17
On the other hand, subamniotic haematoma are found situated under the amniotic layer covering the fetal (chori­onic) plate of the placenta and result from the rupture of fetal vessels branching from the cord (Fig. 7.3). These are considerably less common, with the majority reported in the third trimester or as a result of excessive traction on the umbilical cord at delivery.17 The sonographic appearance is of a single mass protruding from the fetal plate and surrounded by a thin membrane. While the newly formed clot is echogenic, with time the lesion becomes less so as the clot resolves.
Assessment of the placenta and umbilical cord

MAJOR STRUCTURAL ABNORMALITIES OF THE UMBILICAL CORD

The umbilical cord anatomy can often be visualized from 12 weeks' gestation by grey-scale imaging but a precise diagnosis of a particular cord abnormality may be
127
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Fig. 7.3 Hypoechoic mass protruding from the fetal plate of the placenta (star) and corresponding after delivery to subamniotic haematoma due to the rupture of a fetal vessel.
difficult and time consuming before 18–20 weeks. Various factors such as oligo-
Ultrasound in obstetrics and gynaecology
hydramnios or multiple loops in the cord can make accurate visualization of the cord vessels impossible, even near term.17 High-resolution colour Doppler imag­ing has an important role in early and accurate diagnosis of cord abnormalities and is also of clinical value in viewing invasive procedures such as amniocentesis or cordocentesis.
128

CONGENITAL ABNORMALITIES

Abnormalities of the cord insertion
Velamentous insertion of the cord or placenta velamentosa is a well-defined path­ological entity with a frequency of around 1% of pregnancies.17 From a clinical point of view, attachment of the cord to the extraplacental membranes is impor­tant because of the risk of severe fetal haemorrhage during labour. Antenatal diagnosis of attachment of the cord to the membranes rather than the placental mass can be easily performed before labour by means of grey-scale and colour Doppler imaging. at routine obstetric ultrasound has the potential to identify pregnancies with velamentous insertion and, therefore, those at risk for obstetric complications, including vasa praevia.
Single umbilical artery (SUA) syndrome
The absence of one umbilical artery (Fig. 7.4) is amongst the most common con­genital fetal malformations with an incidence of approximately 1% of all deliver­ies.17 The highest incidence of SUA is found among western Europeans and there is no evidence of familial tendency for this anomaly. SUA occurs three to four times more frequently in twins and almost invariably accompanies the acardia malfor­mation and sirenomelia or caudal regression syndrome. increase of the incidence of velamentous insertion of the cord among SUA infants.
17,27
Systematic assessment of the placental cord insertion site
17
There is also a sixfold
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Fig. 7.4 Longitudinal view of an umbilical cord containing one vein and one artery.
Fetal major anatomical defects are largely responsible for the high fetal and neonatal loss from this pathology.23 Fetal malformations are present in about 50% of cases of SUA and can affect any organ system. The comparison of sonographic and postnatal findings in cases of SUA syndrome has shown that minor malfor­mations of the musculoskeletal and cardiovascular systems or of the genitouri­nary tract are often misdiagnosed by ultrasonography, in particular when they are isolated.17 The discovery of a SUA in the perinatal period justifies a detailed ultra­sound examination of the neonate to exclude minor anomalies of internal organs such as the kidney or heart, which may lead to deleterious sequelae if untreated until late infancy. The antenatal discovery of a SUA together with another fetal structural abnormality should raise the question of antenatal chromosome inva­sive testing,23 in particular in early pregnancy24 when the diagnosis of structural defect is less accurate. However, it is most likely that, as for many other isolated fetal defects, an isolated SUA does not increase the risk for trisomy 21.17 Thus, the SUA is not a specific marker of chromosomal abnormalities and the higher incidence of SUA found in trisomies 13 and 1824 is probably only related to the higher incidence in these cases of major fetal defects which are known to be asso­ciated with the aplasia or atrophy of one of the umbilical arteries.
The detection rate of SUA remains low in routine ultrasound.7 However, the incidence of fetal growth restriction (FGR) is significantly elevated among fetuses with a SUA and may be present without any other congenital anomalies in about 20% of cases.17 Since the incidence of FGR is about 10% in the third trimester of pregnancy then 1 in 10 neonates with a low birthweight may have a SUA. The presence of a single umbilical artery is associated with a poorer perinatal out­come compared to that in fetuses with three vessels in the cord.7 Recognizing the importance of this cord anomaly in counselling and management of pregnancies should provide the stimulus to improve detection rates.
Assessment of the placenta and umbilical cord
Cord tumours
Umbilical cord tumours are infrequent perinatal findings and are always benign. From a pathological point of view, primary cord tumours can be divided into
129
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Fig. 7.5 Transverse view of a cord tumour containing dense tissue and pseudocysts and corresponding to a cord angiomyxoma.
angiomyxomas or haemangiomas derived from embryonic vessels, teratomas derived from germ cells and vestigial cysts derived from remnants of the allantois
Ultrasound in obstetrics and gynaecology
or the omphalomesenteric duct.17 A cord angiomyxoma appears sonographically as a heterogeneous mass made of a strong echogenic area, embedding the umbilical vessels12 and surrounded by large echo-poor areas (Fig. 7.5). The prenatal diagnosis of a cord teratoma has rarely been documented but this type of tumour is mainly composed of dense tissue26 and appears echogenic on ultrasound. Conversely, ves­tigial cysts appear sonographically as a single fluid-filled mass.17 Vestigial cysts and pseudocysts can sometimes be associated with small abdominal wall defects and a precise early prenatal diagnosis can be more difficult to establish.10 These embryonic cysts are usually small but some may exceed 5 cm in size and appear as a poorly reflective round mass adjacent to or within the cord. Colour Doppler shows no blood flow within the mass.17 The prevalence of these cysts is around 3% in the first trimester and is an important differential diagnosis as more than 20% of cases are associated with fetal chromosomal or structural defects, especially when located close to the placental insertion of the cord.
25
130

SECONDARY ABNORMALITIES

Vascular abnormalities
Haematomas and thrombosis
Spontaneous cord haematomas are occasional perinatal findings and are usually located near the fetal umbilicus.17 Mechanical trauma of the cord such as pro­lapse, torsion, strangulation or dissecting aneurysm are all potential causes of cord haematoma. At ultrasound examination, the cord appears markedly thickened, sausage-shaped and extremely echogenic. Thrombosis of one or more umbili­cal cord vessels is a rare complication with an incidence of approximately 0.08% among placentas examined prospectively at delivery.17 Thrombosis of the umbili­cal vein occurs more frequently than thrombosis of one or both arteries and peri­natal morbidity or mortality is more likely with umbilical artery thrombosis than
 
with umbilical vein thrombosis. Thrombosis of the umbilical vessels may also be secondary to localized increased resistance in the umbilical circulation in cases of torsion, compression and knotting of haematoma. Intense echogenic material within the lumen of the umbilical vessels is the main sonographic finding.
17
Vascular abnormalities
Abnormal cord position
Looping of the cord may occur around the fetal neck, body or shoulder17 and can be diagnosed by sonography. Although in singleton pregnancies looping of the cord around the neck is an uncommon cause of fetal death, in monoamniotic twins a significant portion of the high mortality can be attributed to umbilical cord problems. Short-term fetal complications of the nuchal cord theoretically include variable fetal heart rate decelerations during the first and second stages of labour and lower mean umbilical artery and venous pH at birth. However, because of the small size of these studies the clinical significance of a single nuchal cord remains undetermined. The sensitivity of around 90% for colour Doppler imag­ing in detecting the presence of a nuchal cord increases with advancing gestation and is always higher than that of grey-scale imaging.11 Colour mapping allows single loops to be differentiated from multiple loops of nuchal cord. However, the sensitivity of the routine ultrasound diagnosis of a nuchal cord is low prior to induction of labour at term.22 Furthermore, a nuchal cord does not appear to increase the risk of caesarean section or poor neonatal outcome. The low ultra­sound detection rate of a nuchal cord limits its use in decision making prior to induction of labour in high-risk pregnancies.
11,22
Assessment of the placenta and umbilical cord

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