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ANSWERS
CASE 32
Ectopic Pregnancy, Unruptured
1. A, B, and C
2. C
3. A
4. C
References
Dialani V, Levine D: Ectopic pregnancy: a review. Ultrasound Q 2004;
20(3):105-117.
http://www.ncbi.nlm.nih.gov/pubmed/15322388 (Accessed on June 6,
2012.)
Maymon R, Halperin R, Mendlovic S, et al: Ectopic pregnancies in Cesarean
section scars: the 8 year experience of one medical center. Hum Reprod 2004; 19(2):278-284.
http://www.ncbi.nlm.nih.gov/pubmed/14747167 (Accessed on June 6,
2012.)
Maymon R, Svirsky R, Smorgick N, et al: Fertility performance and obstetric
outcomes among women with previous cesarean scar pregnancy. J Ultra- sound Med 2011; 30(9):1179-1184.
http://www.ncbi.nlm.nih.gov/pubmed/21876087 (Accessed on June 6,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 357-370.
Comment
Ultrasound Imaging of Ectopic Pregnancy
Detection of a true intrauterine gestational sac is paramount to the exclusion of an ectopic pregnancy, which usually occurs in the distal fallopian tube. An intraendometrial uid collection, also known as a decidual cast or pseudogestational sac (Figure A), should not be misinterpreted as a gestational sac. These col­lections can be seen in an ectopic pregnancy and are caused by the hormonal inuence. Several characteristics of a true early intrauterine gestational sac have been shown to be helpful in distinguishing this from intraendometrial uid when the intra­uterine pregnancy is visualized before the development of a yolk sac or fetal pole.
Ultrasound Finding of Intradecidual Sign
Before the double decidual sac becomes apparent, the location of the sac is an important criterion. The intradecidual sign refers
to a sac located adjacent to, or abutting, the endometrial lining, embedded within the decidual reaction. The decidual cast, or intraendometrial uid that is seen in cases of ectopic pregnan­cies, is located within the uterine cavity. The ultrasound nding of the intradecidual sign has been shown to exclude reliably an ectopic pregnancy. Use of transvaginal ultrasound and beta­human chorionic gonadotropin evaluation has led to a reduc­tion in the need for diagnostic laparoscopy.
Double Decidual Reaction
The double decidual reaction refers to two concentric hyperechoic rings that surround the early intrauterine gestational sac. This sign may not be present with a normal intrauterine gestational sac. The chorionic rim, a hyperechoic rim bordering an intrauter­ine collection of uid, has been shown to be a more sensitive indicator of an intrauterine pregnancy, particularly if diastolic ow is high. Although color Doppler imaging can be used, pulsed Doppler should not be used on or near a normal embryo.
Decidual Cysts
Decidual cysts are simple cysts, 1 to 5 mm in diameter, that are located in the decidual reaction and are remote from the endometrial canal. They may be found at the junction of the endometrium and myometrium. They do not have a hyper­echoic trophoblastic ring and are believed to represent an early breakdown of the decidua.
Ectopic Pregnancy in a Cesarean Section Scar
Figure B shows a life-threatening ectopic pregnancy develop­ing in a cesarean section scar in a different patient. Although rare, the number of primary and recurrent cesarean scar pregnancies has been increasing. The clinical presentation is usually vaginal bleeding. Women at risk have a history of placental pathology, ectopic pregnancy, multiple cesarean sec­tions, and cesarean breech delivery. Failure to recognize this type of ectopic pregnancy may result in uterine rupture and maternal morbidity, either from progression of the disorder or from inappropriate curettage for a presumed failed intrauterine pregnancy with subsequent hemorrhage.
66

CASE 33

A
History: A patient presents with a question of a cardiac
nding on a prior ultrasound scan.
1. What should be included in the differential diagnosis? (Choose all that apply.)
A. Physiologic B. Structural cardiac abnormality C. Chromosomal abnormalities D. Hydrops E. Fetal arrhythmia
2. Which of the following is a technical factor that causes difculty in diagnosing pericardial uid?
A. When the axis of the interventricular septum is perpen-
dicular to the ultrasound beam B. Echoic dropout of the ventricular myocardium C. Use of color Doppler D. Use of M-mode
B
3. When a pericardial effusion is present, which of the follow­ing actions is not recommended?
A. Do nothing. B. Follow up. C. Offer fetal karyotyping. D. Perform fetal echocardiography.
4. Which of the following statements regarding the prognosis of a fetus with pericardial effusion is true?
A. Larger pericardial effusions have a worse prognosis
than smaller ones.
B. The presence of hydrops or extracardiac malformations
is associated with low mortality.
C. The combination of structural heart disease with peri-
cardial effusion is associated with high mortality.
D. Pericardial effusions can never be present in normal
healthy fetuses.
67
ANSWERS
CASE 33
Pericardial Effusion
1. B, C, D, and E
2. B
3. A
4. C
References
DeVore GR, Horenstein J: Color Doppler identication of a pericardial
effusion in the fetus. Ultrasound Obstet Gynecol 1994; 4(2):115-120.
http://www.ncbi.nlm.nih.gov/pubmed/12797204 (Accessed on June 6,
2012.)
Sharland G, Lockhart S: Isolated pericardial effusion: an indication for fetal
karyotyping? Ultrasound Obstet Gynecol 1995; 6(1):29-32.
http://www.ncbi.nlm.nih.gov/pubmed/8528797 (Accessed on June 6, 2012.)
Slesnick TC, Ayres NA, Altman CA, et al: Characteristics and outcomes of
fetuses with pericardial effusions. Am J Cardiol 2005; 96(4):599-601.
http://www.ncbi.nlm.nih.gov/pubmed/16098320 (Accessed on June 6,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 419-422.
Comment
Differential Diagnosis
Fetal pericardial effusions are often seen with fetal con­gestive heart failure, sometimes along with hydrops fetalis. They may also be seen in association with chromosomal abnormalities (particularly trisomy 21); cardiac malforma­tions (heterotaxy syndrome being most common); infectious processes; fetal arrhythmias (supraventricular tachycardia occurring most frequently); or extracardiac malformations, including pericardial teratomas, congenital cystic adeno­matoid malformations, omphaloceles, and liver or kidney masses. A pericardial effusion sometimes may be present in normal healthy fetuses.
Ultrasound Findings
Fetal pericardial effusion is best identied on a four-chamber view of the heart with the ultrasound beam perpendicular to the interventricular septum (Figures A and B). Effusion is anechoic and separates the pericardium from the epicardium. Difculty arises in diagnosis because of signal dropout in the ventricular myocardium, which is noted in 94% of fetuses and can be mistaken for effusion. Color Doppler helps in these cases; in the presence of pericardial effusion, color Doppler signals in the pericardial space are opposite to the direction of blood ow entering and exiting the ventricles. Use of M-mode also aids in diagnosis, but it may be difcult to interpret if one is unfamiliar with its use. Normal rim of pericardial uid mea­sures 2 mm and is of no pathologic signicance.
Prognosis and Management
When a fetal pericardial effusion is detected, a diligent search should be performed for any ultrasound evidence of structural cardiac or extracardiac anomalies or fetal arrhythmias; this is done by a detailed fetal anatomy ultrasound scan and fetal echocardiography. Cardiac function should be evaluated, and assessment should be performed for hydrops. If the effusion is determined to be isolated, fetal karyotyping should be offered. There is a high incidence of chromosomal anomalies in fetuses with isolated effusions, particularly trisomy 21. The size of the pericardial effusion is an unreliable predictor of outcome. The presence of associated abnormalities such as hydrops or struc­tural cardiac and extracardiac defects is associated with poor prognosis. However, normal healthy fetuses may have pericar­dial effusions, and these cases have an excellent prognosis.
Acknowledgment
Special thanks are extended to Vishal Sidhar, MD, and Simran Sekhon, MD, for their contribution to preparing this case.
68

CASE 34

A
B
History: A patient undergoes a 22-week prenatal ultrasound
examination.
1. What should be included in the differential diagnosis for an enlarged kidney with multiple hypoechoic uid-lled spaces in a fetus on prenatal examination? (Choose all that apply.)
A. Hydronephrosis B. Autosomal recessive polycystic kidney disease C. Multicystic dysplastic kidney (MCDK) D. Bilateral subdiaphragmatic pulmonary sequestrations E. Bilateral mesonephric blastomas
2. Which of the following entities is the most likely cause of noncommunicating cyst?
A. MCDK B. Posterior urethral valves C. Ureteropelvic junction obstruction D. Vesicoureteral reux
C
Used with permission from Anderson Publishing Ltd., from Victoria T, et al: Fetal MRI of common non-CNS abnormalities: a review. Appl Radiol 40(6):8-17, 2011. © Anderson Publishing Ltd.
3. Which of the following studies is not part of the standard workup of the differential diagnosis?
A. Postnatal ultrasound B. CT C. MRI D. Voiding cystourethrogram
4. Without intervention, noncomplicated MCDK tends to result in which of the following?
A. Renal hypertension B. Malignant degeneration C. Partial involution D. Gartner cyst and seminal vesicle cyst
69
ANSWERS
CASE 34
Multicystic Dysplastic Kidney
1. A and C
2. A
3. B
4. C
References
Feldenberg LR, Siegel NJ: Clinical course and outcome for children with
multicystic dysplastic kidneys. Pediatr Nephrol 2000; 14(12):1098-1101.
http://www.ncbi.nlm.nih.gov/pubmed/11045394 (Accessed on June 6,
2012.)
Hains DS, Bates CM, Ingraham S, et al: Management and etiology of the
unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol 2009; 24(2):133-242.
http://www.ncbi.nlm.nih.gov/pubmed/18481111 (Accessed on June 6,
2012.)
Mercado-Deane MG, Beeson JE, John SD: US of renal insufciency in
neonates. Radiographics 2002; 22(6):1429-1238.
http://www.ncbi.nlm.nih.gov/pubmed/12432113 (Accessed on June 6,
2012.)
ischemia during embryogeneis, genetic disturbances, terato­gens, and in utero infections. Contralateral renal abnormali­ties, such as vesicoureteral reux, can occur. Evaluation of the integrity of the contralateral kidney is imperative to identify at-risk patients because reux or obstruction can impair and damage the remaining functioning kidney.
Typically, MCDK is found to be unilateral in children because bilateral MCDKs lead to oligohydramnios, which is incompatible with life. However, prenatally MCDK is asso­ciated with contralateral renal abnormalities, such as hydro­nephrosis, renal agenesis, or MCDK. The key to survival is adequate urine production with resultant normal amniotic uid volume. Severe bilateral disease leads to oligohydramnio­sis and death. Noncommunicating cysts are a key feature to distinguish MCDK from hydronephrosis; ultrasound shows organized positioning of symmetric uid-lled spaces. The noncommunicating cysts of MCDK are well identied on MRI (Figure C). The affected kidney is nonfunctional, which can be conrmed by nuclear medicine studies after birth. After birth, chronic MCDK in children exhibits curvilinear calcied cystic walls, creating a “cluster of grapes” sign.
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 465-466.
Comment
Differential Diagnosis
The differential diagnosis of multiple uid-lled structures in an enlarged kidney includes MCDK and hydronephrosis. Hydronephrosis of varying types would be considered in the differential diagnosis, although these cystic structures are sym­metrically arranged and communicate with the dilated renal pelvis.
Ultrasound Findings
Ultrasound shows a disorganized pattern of noncommuni­cating hypoechoic areas of various shapes and sizes without connections or with dominant medial cyst and without rec­ognizable renal parenchyma (Figures A and B). Scant dysplas­tic parenchyma may be visible between the cysts, but normal renal parenchyma and reniform shape are lost. Pathologically, the renal parenchyma in MCDK is nearly completely replaced by multiple thin-walled cysts. Many etiologies have been pro­posed, including ureteric bud atresia, outow obstruction or
Prognosis and Management
MCDK is usually diagnosed during routine prenatal ultra­sound scanning or is diagnosed in a neonate presenting with an abdominal mass. In the past, nephrectomy was the stan­dard of care. However, most individuals with MCDK are asymptomatic. More recent observations show high rates of spontaneous involution with age, low rates of hypertension, and a risk of tumorigenesis that is likely no greater than in normal kidneys. If MCDK is suspected prenatally, postnatal ultrasound conrms the diagnosis, and screening for other urinary tract anomalies is critical. If screening test results are normal, follow-up ultrasound in 6 weeks to reduce false­positive errors is performed. Further follow-up at 2, 5, and 10 years is considered reasonable to document involution of the MCDK and compensatory hypertrophy of the contralat­eral kidney.
Acknowledgment
Special thanks are extended to Holly Marciniak Thompson, MD, for her contribution to preparing this case.
70

CASE 35

A
C
History: A 32-year-old patient undergoes routine prenatal
second-trimester ultrasound screening.
1. Which second-trimester ultrasound markers aid in detect­ing Down syndrome? (Choose all that apply.)
A. Thickened nuchal fold B. Absent or short nasal bone C. Echogenic intracardiac focus (EIF) D. Pyelectasis E. Short long bones
2. Which of the following statements regarding EIF is true?
A. It is a highly specic marker for Down syndrome. B. It can be seen in normal fetuses. C. It is best detected when the fetal heart is horizontal with
respect to the ultrasound beam.
D. Any echogenic focus is signicant.
3. To evaluate the nasal bone correctly in the second trimester, what should the angle of insonation be from the longitudi­nal axis of the fetal nose?
A. 45 degrees B. 60 degrees
B
C. 90 degrees D. 75 degrees
4. How is pyelectasis dened in the second trimester?
A. Renal pelvis anteroposterior (AP) dimension 4 mm or
greater in transverse plane
B. Renal pelvis AP dimension 2 mm or greater in trans-
verse plane
C. Renal pelvis AP dimension 3 mm or greater in trans-
verse plane
D. Renal pelvis AP dimension 8 mm or greater in trans-
verse plane
71
ANSWERS
CASE 35
Trisomy 21 (Down Syndrome)—Basic
1. A, B, C, D, and E
2. B
3. C
4. A
Reference
Benacerraf BR: The history of the second-trimester sonographic markers for
detecting fetal Down syndrome, and their current role in obstetric practice. Prenat Diagn 2010; 30(7):644-652.
http://www.ncbi.nlm.nih.gov/pubmed/20572106 (Accessed on June 6,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 394-453.
Comment
Differential Diagnosis
Commonly used markers and anomalies associated with Down syndrome in second-trimester fetuses are as follows:
• Heart defect, especially atrioventricular canal (endocardial cushion defect) but also other cardiac malformation
• Ventriculomegaly
• Duodenal atresia (after 22 weeks’ gestation)
• Thickened nuchal fold
• Absent or short nasal bone
• Short long bones (femur and humerus)
• Hyperechoic bowel
• EIF
• Pyelectasis
Ultrasound Findings
Specic cardiac features associated with Down syndrome include ventricular disproportion and septal defects. Duo­denal atresia is identied as two uid-lled upper abdominal structures, the stomach and proximal duodenum—the double bubble sign. The nuchal fold measurement is made on trans­verse section across the fetal head, angled caudally to include
the occipital bone and the cerebellum. The measurement is made from the outside of the occipital bone to the skin edge and is considered thickened at 6 mm or greater (Figure A). Although most centers use 6 mm as the measurement for abnormal nuchal thickness, some other authors use 5 mm or greater. This measurement improves sensitivity but increases the number of false-positive examinations. Absent or short nasal bone is one of the most promising newest markers. For correct evaluation of the nasal bone in the second trimester, it is important that the angle of insonation be 90 degrees from the longitudinal axis of the fetal nose (Figure B). An individual with Down syndrome has a short stature with a short femur and humerus (Figure B). These are considered minor markers because of their prevalence in the normal population and vari­ability in different ethnic groups. Hyperechoic bowel is dened as fetal bowel that is as echogenic as bone. However, this is a subjective nding and difcult to use because echogenicity of fetal bowel is affected by transducer frequency and machine settings. EIF is identied as an echogenic focus that is as bright as bone and best detected when the heart is vertically aligned with the ultrasound beam (Figure C). Pyelectasis in a second­trimester fetus is dened as renal pelvis AP dimension 4 mm or greater in transverse plane.
Prognosis and Management
A cluster of markers has a much higher likelihood ratio than any individual markers, indicating that when multiple markers are present, the patient could go from a low-risk to a high­risk category. A normal ultrasound examination with no mark­ers results in a 50% to 80% reduction in risk of fetal Down syndrome; this could be used to lower a prior risk and reas­sure patients, enabling them to forgo invasive procedures. The results of the ultrasound scan need to be taken into consider­ation with other risk factors, including maternal age and serum biochemical markers, to determine best the true risk factors.
Acknowledgment
Special thanks are extended to Simran Sekhon, MD, for her contribution to preparing this case.
72

CASE 36

History: A pregnant patient presents with an infectious
process.
1. What is the diagnostic nding on the ultrasound in the Fig­ure in addition to gender?
A. Oligohydramnios B. Abruption C. Polyhydramnios D. Subchorionic bleed
2. What two fetal organ systems have abnormalities associated with this condition?
A. Gastrointestinal (GI) tract and central nervous system
(CNS) B. GI tract and urinary tract C. CNS and urinary tract D. Cardiac system and CNS
3. What renal mass is associated with this condition? A. Mesoblastic nephroma B. Single renal cyst C. Hypernephroma D. Multicystic dysplastic kidney
4. What percentage of cases with polyhydramnios have fetal
anomalies? A. 90% B. Less than 1% C. 50% to 60% D. 12% to 20%
73
ANSWERS
CASE 36
Polyhydramnios
1. C
2. A
3. A
4. D
Reference
Barnhard Y, Bar-Hava I, Divon MY: Is polyhydramnios in an ultrasonograph-
ically normal fetus an indication for genetic evaluation? Am J Obstet Gynecol 1995; 173(5):1523-1527.
http://www.ncbi.nlm.nih.gov/pubmed/7503195 (Accessed on June 6, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 375-376, 378, 435-437.
Comment
Causes and Measurement of Polyhydramnios
Polyhydramnios (see Figure) may not manifest until after 24 weeks’ gestation. Causes include maternal, fetal, and placen­tal abnormalities. The quantity of uid can be an indicator of the cause. Mild increases in amniotic uid are often idiopathic. Larger volumes of uid more commonly indicate the presence of an anomaly. The ability to make the diagnosis based on a single ultrasound image emphasizes the importance of subjec­tive evaluation of the uid volume; in addition, the amniotic uid index can be calculated and compared with the range of normal for the specic gestational age.
Fetal Anomalies
Fetal anomalies are present in approximately 12% to 20% of cases. These anomalies relate to the inability of the fetus to swallow the amniotic uid or an obstruction to the passage of amniotic uid through the GI tract. CNS malformations such as anencephaly, encephalocele, and Dandy-Walker mal­formation can cause decreased swallowing. Esophageal atresia and duodenal atresia result in GI obstruction. Thoracic masses that obstruct the esophagus, such as a large congenital cystic adenomatoid malformation or diaphragmatic hernia, result in polyhydramnios. Fetal hydrops that develops from any under­lying cause is another etiology.
Fetal Masses
Fetal masses manifest with secondary polyhydramnios. These masses include head, neck, and sacrococcygeal teratoma. Unusual associations include mesoblastic nephroma and a large fetal ovarian cyst.
Fetal Chromosome Anomalies
Fetal chromosomal anomalies are present in 4% of cases. These should be suspected if specic associated fetal anom­alies are documented or if the fetus develops intrauterine growth restriction.
Other Causes of Polyhydramnios
Certain causes may not be apparent on prenatal ultrasound, including maternal diabetes mellitus or infection (the cause in this case) triggered by cytomegalovirus or Toxoplasma gondii.
74

CASE 37

A
History: Two images of placentas are presented. Figure A is from one asymptomatic pregnant patient, and Figure B is from a second asymptomatic pregnant patient.
1. What should be included in the differential diagnosis for
the placental lesion shown in Figure A? (Choose all that
apply.) A. Fibrin deposition B. Hematoma C. Placental infarct D. Intervillous thrombus
2. Which lesion occurs more commonly on the maternal side? A. Fluid deposition B. Hematoma C. Placental infarct D. Intervillous thrombus
3. Which laboratory abnormality is associated with the above-
mentioned placental abnormalities? A. Anemia B. Elevated alpha-fetoprotein C. Leukocytosis D. Erythrocytosis
4. What is the diagnosis of the placental mass shown in Figure
B? A. Uterine contraction B. Chorioangioma C. Hematoma D. Mucin deposition
BB
75