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ANSWERS
CASE 42
In Utero Infection
1. A, B, C, and D
2. D
3. B
4. D
References
Drose JA, Dennis MA, Thickman D: Infection in utero: US ndings in
19 cases. Radiology 1991; 178(2):369-374.
http://www.ncbi.nlm.nih.gov/pubmed/1846239 (Accessed on June 11,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 214-215.
Comment
Causes of In Utero Infection
In utero infection in the United States is most often caused
by cytomegaloviruses. Other common infections include varicella, syphilis, herpes simplex type 2, listeriosis, toxoplasmosis,
and HIV. Death can occur in utero or in the neonatal period.
Infants who survive may have developmental impairment or
mental retardation.
Detection of In Utero Infection
The infection is often subclinical in the mother and is detected
by the development of serum antibodies or by isolating the
virus from urine or the cervix. Alternatively, amniocentesis or
cordocentesis documents exposure of the fetus to the infection but does not predict the impact on fetal development.
In Utero Infection, Anomalies, and Imaging
Anomalies can occur in various organs. Central nervous system malformations include hydrocephalus, microcephaly, cerebellar aplasia, encephalomalacia, and porencephaly. Imaging
may show periventricular and parenchymal calcications (Figures A to C). Much less commonly, the tubers of tuberous
sclerosis may manifest in a microcephalic fetus as hyperechoic
intracranial masses.
Cardiac anomalies include septal defects, cardiomegaly, and
pulmonary stenosis. Hepatosplenomegaly, pleural effusion,
ascites, hydrops, and intraabdominal (including hepatic) calcications may be identied. The amount of amniotic uid
can vary from oligohydramnios to polyhydramnios. Intrauterine growth restriction occurs in some cases. The placenta
was enlarged in 30% of cases in one series. In some cases
the abnormalities may not be present on the rst ultrasound
scan but may develop later in the gestation. In utero infection
should be considered whenever unusual or atypical anomalies
are detected on prenatal ultrasound.
86
CASE 43
A
Used with permission from McGahan JP, Benacerraf BR: Fetal heart.
In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008.
History: A patient presents with a family history of con-
genital heart disease.
1. What should be considered in the differential diagnosis?
(Choose all that apply.)
A. Coarctation of the aorta
B. Atrial septal defect (ASD)
C. Atrioventricular canal
D. Ebstein anomaly
E. Tricuspid atresia
2. Which of the following cardiac defects is not associated
with a VSD?
A. Tetralogy of Fallot
B. Coarctation of the aorta
C. Ventricular hypoplasia
D. Endocardial broelastosis
B
Used with permission from McGahan JP, Benacerraf BR: Fetal heart.
In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008.
3. Which of the following chromosomal abnormalities or syndromes is not usually associated with VSD or AVSD?
A. Turner syndrome
B. Trisomy 21 (Down syndrome)
C. Edwards syndrome
D. Heterotaxy syndrome
4. Which of the following is not helpful in establishing the
diagnosis of AVSD by ultrasound?
A. Performing color Doppler
B. Obtaining static images during ventricular systole
C. Noting that the atrioventricular valves insert at the same
level with one another
D. Noting a concomitant ASD
87
ANSWERS
CASE 43
Endocardial Cushion Defect
1. B and C
2. D
3. A
4. B
References
Berg C, Kaiser C, Bender F, et al: Atrioventricular septal defect in the
fetus—associated conditions and outcome in 246 cases. Ultraschall Med
2009;30(1):25-32.
http://www.ncbi.nlm.nih.gov/pubmed/18491259 (Accessed on June 11,
2012.)
Bronshtein M, Egenburg S, Auslander R, et al: Atrioventricular septal defect
in a fetus: a false negative diagnosis in early pregnancy. Ultrasound Obstet
Gynecol 2000;16(1):98-99.
http://www.ncbi.nlm.nih.gov/pubmed/11084977 (Accessed on June 11,
2012.)
Chen CP, Su YN, Hsu CY, et al: Ellis-van Creveld syndrome: prenatal
diagnosis, molecular analysis and genetic counseling. Taiwan J Obstet Gynecol
2010;49(4):481-486.
http://www.ncbi.nlm.nih.gov/pubmed/21199751 (Accessed on June 11,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 416.
Comment
Differential Diagnosis
Differential diagnosis in this case centers on defects in the
atrioventricular septum. There may be a complete ASD or a
less severe abnormality. AVSD is a defect in the crux of the
heart owing to incomplete fusion of the endocardial cushion.
In the most common form, there is a combination of ASD,
VSD, and various degrees of abnormality of the atrioventricular valves. Although a complete AVSD is the most likely
diagnosis in this case, there may be other variations, including partial AVSD, in which there is an associated ASD and a
cleft in the mitral valve. Asymmetry of the ventricular chambers may be present when the AVSD is unbalanced. Isolated
ASD or isolated VSD may be considered in the differential
diagnosis.
As mentioned previously, when an AVSD is identied,
there is a higher association of other cardiac defects, including
coarctation of the aorta, double outlet of the right ventricle,
and pulmonary valve stenosis. The incidence of congenital
heart block is also increased, especially when heterotaxy syndrome is present.
Ultrasound Findings
Ultrasound ndings include identication of an ASD at the
crux of the heart. The atrioventricular valves often form a
single common valve. This single valve can be problematic
because during systole the valve is closed and may give a false
impression of an intact septum (Figure A), especially when
there is a small VSD. The heart should be examined throughout the cardiac cycle, especially during diastole when the valves
are open and an atrioventricular defect can be identied. In
addition, color Doppler or power Doppler may be helpful to
identify the common atrium and the large VSD (Figure B).
There are other variations of AVSD, including a partial defect
in which there is an ASD with a small VSD component. There
may be unbalanced AVSDs, in which there is hypoplasia in one
of the ventricles or the pulmonary artery or aorta. These cases
may be much more complex and lead to difcult repairs.
Prognosis and Management
The prognosis is usually good if no other major associated
defect is present and the fetus does not have complete heart
block or develop hydrops. However, AVSD is highly associated with aneuploidy, including trisomy 21, trisomy 18, trisomy
13, and heterotaxy syndrome. Karyotyping may have prognostic implications in management. Ellis-van Creveld syndrome
(chondroectodermal dysplasia) has a high incidence of cardiac
defects, especially ASD. When there is an unbalanced defect
with marked hypoplasia of one of the ventricles or arteries,
this may render a ventricular repair impossible. However, survival is good when the AVSD is balanced and there are no
other associated defects or chromosomal abnormalities. Serial
ultrasound scans to watch for fetal hydrops should be incorporated into standard practice.
88
CASE 44
A
Used with permission from McGahan JP, et al: Fetal abdomen and pelvis. In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008; 1301.
B
History: A 19-year-old patient presents with an elevated
alpha fetoprotein level on screening.
1. What should be included in the differential diagnosis for
this 20-week ultrasound scan? (Choose all that apply.)
A. Omphalocele
B. Gastroschisis
C. Physiologic gut herniation
D. Bladder exstrophy
E. Amniotic band syndrome
2. What structure seen on ultrasound is critical in differentiating gastroschisis from omphalocele?
A. Stomach
B. Liver
C. Small bowel
D. Site of umbilical cord insertion
C
Courtesy of Dolores Pretorius, MD, San Diego, California.
3. Which of the following entities is least likely to be associated
with gastroschisis?
A. Chromosomal abnormalities
B. Bowel atresia
C. Necrotizing enterocolitis
D. Small anterior abdominal wall defect
4. Which of the following statements concerning gastroschisis is false?
A. Gastroschisis occurs more commonly in African-
Americans than whites.
B. Gastroschisis is commonly associated with other
abnormalities.
C. Gastroschisis is not associated with advanced maternal
age.
D. Gastroschisis is treated with a silo apparatus after birth.
89
ANSWERS
CASE 44
Gastroschisis
1. B and E
2. D
3. A
4. B
References
Christison-Lagay ER, Kelleher CM, Langer JC: Neonatal abdominal wall
defects. Semin Fetal Neonatal Med 2011; 16(3):164-172.
http://www.ncbi.nlm.nih.gov/pubmed/21474399 (Accessed on June 11,
2012.)
Durfee SM, Downard CD, Benson CB, et al: Postnatal outcome of fetuses
with the prenatal diagnosis of gastroschisis. J Ultrasound Med 2002;
21(3):269-274.
http://www.ncbi.nlm.nih.gov/pubmed/11883537 (Accessed on June 11,
2012.)
Emanuel PG, Garcia GI, Angtuaco TL: Prenatal detection of anterior
abdominal wall defects with US. Radiographics 1995; 15(3):517-530.
http://www.ncbi.nlm.nih.gov/pubmed/7624560 (Accessed on June 11,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 443-446.
Comment
Incidence and Etiology
Gastroschisis is a major anterior abdominal wall abnormality; the other major abdominal wall abnormalities include
omphalocele, amniotic band syndrome, and limb–body wall
complex. It is less common than omphalocele, occurring in
1:10,000 live births. Associated anomalies and aneuploidy are
rare. Gastroschisis results in a weakened area of the abdominal wall; this may be caused by the normal involution of
the right umbilical vein or the premature interruption of
the right omphalomesenteric artery. A full-thickness defect
occurs in the anterior abdominal wall, usually less than 4 cm
in size. Because the bowel has returned to the peritoneal cavity before this defect occurs, the protruding viscera is not
lined by peritoneum, and the umbilicus inserts normally; this
distinguishes gastroschisis from omphalocele. The exposed
bowel results in elevated maternal serum alpha-fetoprotein
levels.
Ultrasound Findings and Complications
Although the defect is small, the contents that herniate
through the defect vary. Small bowel is always herniated, and
large bowel is often herniated (Figure A). The stomach or portions of the genitourinary tract uncommonly may protrude
through the defect. The defect is lateral to insertion of the
umbilical cord, and there are free-oating loops of bowel (Figures A to C). The exposed bowel may appear thicker and more
dilated than normal. It has been suggested that the caliber of
bowel can indicate postnatal bowel complications. However,
more recent research has indicated that isolated gastrointestinal ndings in gastroschisis do not correlate with adverse
postnatal outcomes. The signicance of bowel dilation has not
been fully established. However, bowel complications occur
with gastroschisis. The bowel is exposed to amniotic uid and
fetal urine, which may result in development of peritonitis. In
addition, given the small size of the defect, there may be compression or torsion of blood vessels; this may result in bowel
stenosis atresia or perforation.
Prognosis and Management
Management includes placing the eviscerated bowel into a
protective silo and allowing the bowel to return slowly into
the abdomen, followed by denitive closure of the abdominal
defect. The prognosis for gastroschisis is determined primarily by the degree of bowel injury. The long-term outcome is
favorable in most cases, and survival is greater than 90%.
90
CASE 45
D
A
History: Four women, who range in age from 20 to 54
years, present with asymptomatic masses on ultrasound.
1. What should be included in the differential diagnosis of
the adnexal masses of patients 1 and 2, looking only at
the ultrasound images (Figures A and C)? (Choose all that
apply.)
A. Corpus luteum cyst
B. Pedunculated broid
C. Dermoid or cystic teratoma
D. Endometrioma
2. Which of the following is not a complication associated
with adnexal ovarian masses as seen in the images?
A. Torsion
B. Hemorrhage
C. Rare malignant degeneration
D. Infection
3. What structures account for the “bright lines and dots”
seen on ultrasound images?
A. Hair
B. Cholesterol
C. Teeth
B
D. Necrosis
4. What endocrine syndrome is rarely associated with this
entity?
A. Hashimoto thyroiditis
1
2
2
1
B. Thyrotoxicosis with struma ovarii in the tumor
C. Graves disease
D. Stein-Leventhal syndrome
C
91
ANSWERS
CASE 45
Dermoid
1. B and C
2. B
3. A
4. B
References
Levine D, Brown DL, Andreotti RF, et al: Management of asymptomatic
ovarian and other adnexal cysts imaged at US: Society of Radiologists in Ultrasound Consensus Conference Statement. Radiology 2010;
256(3):943-954.
http://www.ncbi.nlm.nih.gov/pubmed/20505067 (Accessed on June 20,
2012.)
Mlikotic A, McPhaul L, Hansen GC, et al: Signicance of the solid com-
ponent in predicting malignancy in ovarian cystic teratomas: diagnostic
considerations. J Ultrasound Med 2001; 20(8):859-866.
http://www.ncbi.nlm.nih.gov/pubmed/11503922 (Accessed on June 20,
2012.)
Outwater EK, Siegelman ES, Hunt JL: Ovarian teratomas: tumor types and
imaging characteristics. Radiographics 2001; 21(2):475-490.
http://www.ncbi.nlm.nih.gov/pubmed/11259710 (Accessed on June 20,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 567-570, 572-573.
Comment
Mature Cystic Teratomas
Mature cystic teratomas, also known as dermoid cysts, are the
most common ovarian tumors; they constitute 10% to 15%
of all ovarian neoplasms. Most teratomas are benign. They are
often incidental ndings on cross-sectional imaging and are
bilateral in 10% of cases.
Ultrasound Appearance of Ovarian Dermoids
The ultrasound appearance depends on which components are
present (Figures A to D). Dermoids are composed of mature
epithelial elements: skin, hair, desquamated epithelium, and
teeth. Macroscopically, dermoids contain variable amounts
of sebum, hair, and teeth. Fat and calcications are hallmarks
of teratomas. The dermoid plug, or Rokitansky protuberance,
consists of sebaceous material usually without ow on color
Doppler and calcications or teeth, hair, and other soft tissue;
this dermoid plug is present in most teratomas and varies in
size. It is seen on ultrasound as a hyperechoic nodule or mass
with shadowing, often within a more simple-appearing cyst.
The cyst is lled with homogeneous sebaceous uid, which
accounts for the lack of internal echoes.
Several characteristic ultrasound ndings have been
described for dermoids. Diffuse or regional bright echoes may
be seen (Figures A and C). As in Figures A, C, and D, the echogenicity is brightest at the top, with absorption of sound internally, known as the “tip of the iceberg” sign. The presence of
hair accounts for hyperechoic lines and dots (Figures B and C
from Case 21: Normal First-Trimester Uterine Pregnancy with Extra-
uterine Mass, a Cystic Teratoma [Dermoid]). A uid-uid level may
also be present. On CT scans, fat attenuation within a cyst is
diagnostic. On MRI, the sebaceous component is identied
with fat saturation techniques (Figure B).
Struma Ovarii
In 20% of cases, thyroid tissue is present microscopically.
When thyroid tissue constitutes a large portion of the lesion, it
is called struma ovarii. In these cases, women may present with
thyrotoxicosis or thyroid enlargement. On ultrasound, if the
mass is an endocrine-secreting mass, a solid component is seen
in the dermoid with low-impedance arterial ow detected by
Doppler ultrasound imaging.
Complications and Treatment of Ovarian
Dermoids
These adnexal masses, especially if large, can cause ovarian
torsion. Other complications include infection and rare malignant degeneration; the latter typically affects patients older
than 40 years, is usually less malignant than other ovarian neoplasms, and constitutes 1% to 2% of mature teratomas. When
a mature cystic teratoma is discovered, management is usually
by surgical excision to prevent the known complications. If
the dermoid is small, it may be removed without loss of the
entire ovary.
92
CASE 46
A
B
History: A patient presents with a fetal thoracic abnormality identied on an ultrasound examination performed at an
outside institution.
1. What should be included in the differential diagnosis for a
left-sided solid fetal chest mass? (Choose all that apply.)
A. Congenital diaphragmatic hernia (CDH) (stomach up)
B. CDH (stomach down)
C. Congenital cystic adenomatoid malformation (or con-
genital pulmonary airway malformation)
D. Bronchial atresia
E. Bronchogenic cyst
2. Which of the following statements concerning CDH is
false?
A. CDH occurs most commonly on the left side.
B. CDH occurs on the right side in approximately 10% of
cases.
C. CDH may occur bilaterally.
D. CDH is usually a defect in the anterior portion of the
diaphragm.
C
Used with permission from Anderson Publishing Ltd. from Victoria T,
et al: Fetal MRI of common non-CNS abnormalities: a review. Appl
Radiol 2011; 40[6]8-17. © Anderson Publishing Ltd.
3. Which of the following statements concerning the prognosis of CDH is false?
A. A poor prognostic factor of CDH is the presence of
the liver within the chest.
B. The amount of cardiac displacement has little prognos-
tic implication for survival.
C. The presence of a small left cardiac ventricle is a poor
prognostic factor.
D. Bilateral CDHs have a poor prognosis.
4. Prognostic features of a left CDH include all of the following except:
A. Associated chromosomal abnormalities
B. Late diagnosis
C. Intrathoracic liver
D. Cardiac anomalies
93
ANSWERS
CASE 46
Congenital Diaphragmatic Hernia,
Left-Sided Bochdalek Hernia
1. A, B, C, and D
2. D
3. B
4. B
References
Dekoninck P, Gratacos E, Van Mieghem T, et al: Results of fetal endo-
scopic tracheal occlusion for congenital diaphragmatic hernia and the
set up of the randomized controlled TOTAL trial. Early Hum Dev 2011;
87(9):619-624.
http://www.ncbi.nlm.nih.gov/pubmed/21907109 (Accessed on June 11,
2012.)
Peralta CF, Sbragia L, Bennini JR, et al: Fetoscopic endotracheal occlusion for
severe isolated diaphragmatic hernia: initial experience from a single clinic
in Brazil. Fetal Diagn Ther 2011; 29(1):71-77.
http://www.ncbi.nlm.nih.gov/pubmed/21325860 (Accessed on June 11,
2012.)
Ruano R, Takashi E, da Silva MM, et al: Prediction and probability of neo-
natal outcome in isolated congenital diaphragmatic hernia using multiple
ultrasound parameters. Ultrasound Obstet Gynecol 2012; 39(1):42-49.
http://www.ncbi.nlm.nih.gov/pubmed/21898639 (Accessed on June 11,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 422-424.
Comment
Differential Diagnosis
The differential diagnosis depends on whether the mass
appears complex or solid. The differential diagnosis of an
intrathoracic mass includes congenital cystic adenomatoid
malformation (or congenital pulmonary airway malformation), sequestration, bronchial atresia, and CDH. If a complex mass is present, the differential diagnosis is slightly
broader and includes various forms of congenital cystic
adenomatoid malformation and sequestration and CDH. In
addition, bronchogenic cyst and neuroenteric cyst may be
considered in the differential diagnosis when an intrathoracic
cyst is visualized.
Ultrasound Findings
Ultrasound ndings of CDH include the presence of a solid
or complex mass usually within the left thorax (Figures A
and B). Most CDHs are on the left side, although right-sided
CDH occurs in 10% of cases. Rarely, bilateral CDHs have
been reported. One of the most common ndings of CDH is
the presence of uid-lled loops of bowel within the thorax.
Loops of small bowel may be identied in the thorax, but they
appear fairly echogenic. Peristalsis may be observed. The liver
may be herniated into the thorax; this is best evaluated with
color Doppler with identication of the portal veins.
If the mass is larger, there is considerable mass effect with
compression of the heart to the opposite side and compression of both the ipsilateral and the contralateral lung. Fetal
MRI may be useful to visualize and characterize the defect better (Figure C).
Prognosis and Management
The prognosis is often poor and is determined by numerous other ndings, including associated morphologic anomalies, chromosomal abnormalities, and the size of the defect,
including the presence of an intrathoracic liver. The size of
the remaining lung tissue is probably the most important prognostic factor. Also, disproportion of the cardiac ventricles with
a small left ventricle has been associated with a poor outcome.
Earlier diagnosis is associated with a poor outcome, probably
because the defect is larger and is recognized early. An evaluation of the size of the contralateral lung in cases of CDH
is most important with various measurements, including lung
diameter, lung area, and ratio of the lung area to the head circumference. In utero repair of CDH has been performed, but
results were poor. However, fetal endoscopic tracheal occlusion has been shown to be an effective treatment of CDH. This
procedure allows better expansion of the fetal lung. “Liver up”
in the chest is associated with a poor prognosis. Total fetal lung
volume may also be a better predictor of outcome compared
with measurement of only the contralateral lung size.
A side effect or serious complication of CDH is pulmonary
hypertension. Many of these fetuses will require extracorporeal membrane oxygenation to permit surgical repair in the
neonatal period.
94
CASE 47
A
B
History: Two patients with rst-trimester pregnancies and
a patient with a third-trimester pregnancy present with vaginal
bleeding.
1. What should be included in the differential diagnosis of
vaginal bleeding that can be seen on ultrasound of the
uterus (not necessarily on the images presented)? (Choose
all that apply.)
A. Spontaneous abortion
B. Subchorionic hemorrhage
C. Periimplantation bleed
D. Ectopic pregnancy
2. What is the overall spontaneous abortion rate associated
with a subchorionic hemorrhage?
A. 50%
B. 90%
C. 1%
1
2
2
1
D. 9%
3. Which of the following factors does not inuence the out-
come of a fetus with subchorionic hemorrhage?
A. Size of the hemorrhage area
B. Maternal age
C. Gestational age of the fetus
D. Diabetes mellitus in the mother
4. How does the echogenicity of the hemorrhage change with
time?
A. It is stable.
B. It becomes continually more echogenic as it gets older.
C. It becomes more liqueed as it gets older.
D. It is anechoic acutely, becomes heterogeneous, and then
is anechoic again.
C
D
95