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ANSWERS
CASE 115
Fetal Goiter
1. A and D
2. A
3. C
4. D
References
Marin RC, Bello-Munoz JC, Martinez GV, et al: Use of 3-dimensional sonog-
raphy for prenatal evaluation and follow-up of fetal goitrous hypothyroid-
ism. J Ultrasound Med 2010; 29(9):1339-1343.
http://www.ncbi.nlm.nih.gov/pubmed/20733190 (Accessed on June 22, 2012.)
Van Loon AJ, Derksen JT, Bos AF, et al: In utero diagnosis and treatment of
fetal goitrous hypothyroidism, caused by maternal use of propylthiouracil.
Prenat Diagn 1995; 15(7):599-604.
http://www.ncbi.nlm.nih.gov/pubmed/8532617 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 244, 246-247.
Comment
Enlargement of the Fetal Thyroid Gland
Enlargement of the fetal thyroid gland may be associated with
a hypothyroid or hyperthyroid state. Fetal goiter, shown by
coronal images of the fetal neck, can be detected and followed
after treatment with prenatal ultrasound. Both two- and threedimensional ultrasound images are useful (Figures A to C).
The vascular supply can be seen for indirect monitoring of
fetal thyroid function. MRI shows thyroid masses well (Figure D). The differential diagnosis of an anterior neck mass in
utero includes teratoma, thyroglossal duct cyst (midline), and
branchial cleft cyst (usually lateral). Rarely, a cystic hygroma
may manifest anteriorly.
Etiology of Fetal Goiter
In patients with Graves disease, thyroid-stimulating immunoglobulins cross the placenta and cause fetal hyperthyroidism.
Despite early neonatal treatment, developmental disorders
can result. Fetal hypothyroidism is caused by the transplacental transfer of medications (e.g., PTU) used to treat maternal
hyperthyroidism. Agenesis or hypoplasia of the fetal thyroid is
an additional cause.
Prognosis and Treatment
Polyhydramnios may be present with fetal goiter. The neonate may develop cardiovascular or respiratory abnormalities
and mental retardation. Cordocentesis is the only accurate
method to determine fetal thyroid hormone levels. Treatment
of hyperthyroidism is possible with maternally administered
PTU; however, hypothyroidism requires fetal intramuscular,
intravascular, or intraamniotic infusion of thyroxine.
236
CASE 116
A
History: An asymptomatic patient in the third trimester of
pregnancy undergoes imaging.
1. What should be included in the differential diagnosis of the
cord insertion in the color Doppler images of the placenta?
(Choose all that apply.)
A. Vasa previa and marginal cord
B. Prolapsed umbilical cord and marginal cord insertion
C. Marginal cord insertion
D. Velamentous cord insertion
2. In what type of pregnancy are velamentous cord insertions
most prominent?
A. Ectopic pregnancy
B. Preterm pregnancy
C. Multiple pregnancies
D. Large-for-dates pregnancy
B
3. Which of the following is not a complication or anomaly
associated with a velamentous cord insertion?
A. Preterm labor
B. Rupture during labor, causing fetal exsanguination
C. Intrauterine growth restriction (IUGR)
D. Postdates pregnancy
4. Which of the following statements about the use of ultrasound to diagnose umbilical cord insertion sites is true?
A. Ultrasound studies report a 98% sensitivity for detect-
ing an abnormal placental cord insertion.
B. Ultrasound studies report a less than 10% sensitivity for
detecting an abnormal placental cord insertion.
C. Color Doppler imaging has not been helpful in deter-
mining an abnormal insertion of the umbilical cord.
D. Ultrasound studies report a 42% sensitivity for detecting
an abnormal placental cord insertion.
237
ANSWERS
CASE 116
Velamentous Insertion of the Umbilical Cord
1. C and D
2. C
3. D
4. D
References
Pretorius DH, Chau C, Poelter DM, et al: Placental cord insertion visualiza-
tion with prenatal ultrasonography. J Ultrasound Med 1996; 15(8):585-593.
http://www.ncbi.nlm.nih.gov/pubmed/8839406 (Accessed on June 22, 2012.)
Raga F, Ballester MJ, Osborne NG, et al: Role of color ow Doppler ultra-
sonography in diagnosing velamentous insertion of the umbilical cord and
vasa previa. J Reprod Med 1995; 40(11):804-808.
http://www.ncbi.nlm.nih.gov/pubmed/8592318 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 489-490.
Comment
Marginal Cord Insertion versus Velamentous
Cord Insertion
Marginal cord insertion is dened as peripheral insertion of
the cord in the placenta, within 2 cm of the edge (Figure A).
Velamentous insertion is distinguished by aberrant vessels
traversing between the amnion and chorion before placental
insertion. Velamentous cord insertion occurs in 1.1% of single intrauterine gestations, with a 10 times higher incidence in
multiple pregnancies. It is also associated with uterine anomalies and indwelling intrauterine devices (IUDs) (Figure B).
Complications of Velamentous Cord Insertion
Velamentous cord insertion is associated with several complications. During active labor, a velamentous insertion is
not anchored and can tear, resulting in exsanguination. Preterm delivery occurs in 17% of cases. IUGR, single umbilical
artery, congenital anomalies, and low Apgar scores have also
been reported. A vasa previa caused by a velamentous insertion in front of the presenting part of the fetus may also
occur.
Ultrasound Imaging of Placental Cord Insertions
Ultrasound studies have reported a 42% sensitivity for
detecting an abnormal placental cord insertion, particularly
later in the gestation period. Nonetheless, this is an important portion of the prenatal ultrasound evaluation. The use
of color Doppler imaging facilitates visualization of the
insertion site.
238
CASE 117
C
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; 1308.
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; 1308.
D
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; 1308.
History: A 28-year-old patient presents from an outside
institution with an abnormal four-chamber view of the fetal
heart.
1. What should be included in the differential diagnosis based
on the imaging ndings in Figures A and B? (Choose all
that apply.)
A. Tetralogy of Fallot
B. Hypoplastic left heart
C. Ebstein anomaly
D. Atrioventricular septal defect
E. Single ventricle
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; 1308.
2. Taking into account Figures A and B, to what does the cys-
tic structure in Figure C most likely correspond?
A. Ovarian cyst
B. Hydronephrosis
C. Fetal stomach
D. Sacrococcygeal teratoma
239
3. Which of the following statements is false?
A. Solitus is the usual arrangement of organs and vessels
within the body.
B. Situs inversus totalis occurs when the fetal stomach is
present on the right side but the fetal heart is directly
toward the left side.
C. Asplenia is part of a condition called situs ambiguus.
D. Polysplenia syndrome is part of a condition called situs
ambiguus.
ANSWERS
CASE 117
4. Which of the following statements is false concerning
prognosis?
A. Situs inversus totalis has a low rate of associated cardiac
abnormalities.
B. One of the most common cardiac abnormalities associ-
ated with heterotaxy syndrome is atrioventricular septal
defect.
C. Congenital heart block has been associated with hetero-
taxy syndrome, especially polysplenia syndrome.
D. The prognosis for infants with heterotaxy syndrome is
usually good.
Heterotaxy Syndrome and Cardiosplenic
Syndrome
1. D and E
2. C
3. B
4. D
References
Berg C, Geipel A, Smrcek J, et al: Prenatal diagnosis of cardiosplenic syn-
dromes: a 10-year experience. Ultrasound Obstet Gynecol 2003; 22(5):451-459.
http://www.ncbi.nlm.nih.gov/pubmed/14618656 (Accessed on June 22, 2012.)
Cohen MS, Schultz AH, Tian ZY, et al: Heterotaxy syndrome with functional
single ventricle: does prenatal diagnosis improve survival? Ann Thorac Surg
2006; 82(5):1629-1636.
http://www.ncbi.nlm.nih.gov/pubmed/17062216 (Accessed on June 22, 2012.)
Paladini D, Sglavo G, Masucci A, et al: Role of four-dimensional ultrasound
(spatiotemporal image correlation and sonography-based automated vol-
ume count) in prenatal assessment of atrial morphology in cardiosplenic
syndromes. Ultrasound Obstet Gynecol 2011; 38(3):337-343.
http://www.ncbi.nlm.nih.gov/pubmed/21404356 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 416-419.
Comment
Differential Diagnosis
The differential diagnosis for a large atrioventricular canal
or single ventricle with a midline stomach is limited. Large
atrioventricular canals are associated with trisomy 21. In this
case, however, a combination of a single ventricle and large
atrioventricular canal with a midline stomach points to the
diagnosis of situs ambiguus.
Ultrasound Findings
Heterotaxy syndrome and cardiosplenic syndrome are the
general terms used for indeterminate situs. Ultrasound ndings depend on whether there is polysplenia with bilateral leftsidedness or asplenia with bilateral right-sidedness, although
overlap exists. Often a large atrioventricular septal defect (Figures A and B) is present with cardiosplenic syndrome. There
may be congenital heart block, and venous abnormalities are
often associated with this syndrome. Usually the stomach is in
an abnormal position and opposite that of the cardiac apex
(Figure C). Other associated abnormalities are often seen.
Sometimes the liver may be displaced or lie in midline (Figure D). In both abnormalities, there may be other anomalies,
including duodenal atresia and central nervous system lesions
such as hydrocephalus and spinal bida.
Prognosis and Management
Fetal karyotyping may be offered, but the risk of aneuploidy
seems to be low. Serial ultrasound scans are recommended
because hydrops or bradycardia may develop. Otherwise, standard obstetric management may be appropriate. However,
because of congenital heart block and the complex structural
defects, the mortality of heterotaxy syndrome is high, especially when hydrops develops.
240
CASE 118
A
History: A 24-year-old patient undergoes routine second-
trimester ultrasound.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Choledochal cyst
B. Ovarian cyst
C. Urachal cyst
D. Mesenteric cyst
E. Umbilical vein varix
2. All of the following statements concerning the umbilical
cord or umbilical vein and arteries are true except:
A. There are two arteries in the umbilical cord.
B. Umbilical arterial ow is away from the fetus.
C. Umbilical venous ow is toward the fetus.
D. There are two vessels within the umbilical cord.
3. All of the following statements concerning the umbilical
vein varix are true except:
A. Umbilical vein varix is not always an isolated abnormality.
B. An association of umbilical vein varix and chromo-
somal abnormalities has been reported.
C. Normal umbilical vein size is approximately 8 mm at 20
weeks’ gestation and 16 mm at term.
D. One of the criteria for diagnosis of varix of the umbili-
cal vein is that it is at least 50% larger than the diameter
of the intrahepatic umbilical vein.
4. Concerning management of umbilical vein varix, all of the
following statements are true except:
A. Umbilical vein varix has been associated with fetal
demise.
B. Associated abnormalities may accompany umbilical
vein varix.
C. When umbilical vein varix is identied, a complete level
II obstetric ultrasound scan should be performed.
D. The prognosis for umbilical vein varix is ominous.
B
C
241
ANSWERS
CASE 118
Umbilical Vein Varix
1. E
2. D
3. C
4. D
References
Byers BD, Goharkhay N, Mateus J, et al: Pregnancy outcome after ultrasound
diagnosis of fetal intra-abdominal umbilical vein varix. Ultrasound Obstet
Gynecol 2009; 33(3):282-286.
http://www.ncbi.nlm.nih.gov/pubmed/19115263 (Accessed on June 22, 2012.)
Mahony BS, McGahan JP, Nyberg DA, et al: Varix of the fetal intra-
abdominal umbilical vein: comparison with normal. J Ultrasound Med 1992;
11(2):73-76.
http://www.ncbi.nlm.nih.gov/pubmed/1560496 (Accessed on June 22, 2012.)
Mankuta D, Nadjari M, Pomp G: Isolated fetal intra-abdominal umbilical
vein varix: clinical importance and recommendations. J Ultrasound Med
2011; 30(2):273-276.
http://www.ncbi.nlm.nih.gov/pubmed/21266567 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 434.
Comment
Differential Diagnosis
The differential diagnosis in this case is broad unless color
Doppler ultrasound is performed. The differential diagnosis
would include any cystic structure within the abdomen, including normal structures such as the gallbladder and stomach and
cystic masses such as duplication or mesenteric cysts, choledochal cysts, or ovarian cysts. However, when venous Doppler
ow is shown in a cystic intraabdominal mass originating from
the anterior abdominal wall, the diagnosis of an umbilical vein
varix is straightforward.
Ultrasound Findings
Ultrasound features include a dilated intraabdominal extrahepatic portion of the umbilical vein (Figure A). Rarely a varix
may manifest as a fairly large mass. Color Doppler ow imaging shows the continuity with the umbilical vein and permits
denitive diagnosis of umbilical vein varix (Figures B and C).
Diagnostic criteria include umbilical vein diameter greater than
9 mm or enlargement of the varix to at least 50% larger than
the diameter of the intrahepatic umbilical vein. The umbilical
vein normally measures up to 3 mm at 15 weeks and increases
to 8 mm at term.
Prognosis and Management
In most cases, the prognosis of an isolated umbilical vein
varix is good. However, nearly every potential complication
one can imagine has been reported in different series of cases
of umbilical vein varix. In the rst report by Mahony et al.,
four of nine fetuses (44%) with umbilical vein varix subsequently died, including one fetus with trisomy 21, and the fth
fetus in that series developed hydrops. Other series of fetuses
with umbilical vein varix have shown associated anomalies that
have led to fetal demise; thrombosis of the varix was reported,
which could be the cause of the demise. However, in most
series, the outcome is excellent.
When umbilical vein varix is identied, at least a level II
obstetric ultrasound scan is recommended to exclude other
abnormalities. Some authors advocate repeat examination
every 2 weeks with close fetal surveillance. Others disagree,
stating that no specic data support alternative obstetric management based on umbilical vein varix alone.
242
CASE 119
A
1
2
2
1
C
History: A 20-year-old pregnant patient presents with acute
left-sided pelvic pain.
1. What is the abnormality in this patient with cyclical leftsided pelvic pain? (Choose all that apply.)
A. Corpus luteum cyst
B. Ovarian mass
C. Polycystic ovary
D. Ovarian torsion
2. What is the typical age (stage of life) of a patient with this
diagnosis?
A. Postmenopausal
B. Perimenopausal
C. Pregnant after age 40
D. Premenopausal, including pregnant
B
D
3. Which of the following factors is not a risk factor for ovarian torsion?
A. Pregnancy
B. Anorexia
C. Ovarian mass
D. Hyperstimulation syndrome
4. Which of the following is the most denite sign of ovarian
torsion?
A. Lack of arterial Doppler ow
B. Lack of venous Doppler ow
C. High diastolic arterial ow
D. Positive whirlpool sign
243
ANSWERS
CASE 119
Ovarian Torsion
1. B and D
2. D
3. B
4. D
References
Di Salvo DN: Sonographic imaging of maternal complications of pregnancy.
J Ultrasound Med 2003; 22(1):69-89.
http://www.ncbi.nlm.nih.gov/pubmed/12523613 (Accessed on July 9, 2012.)
Mashiach R, Melamed N, Gilad N, et al: Sonographic diagnosis of ovarian
torsion. J Ultrasound Med 2011; 30(9):1205-1210.
http://www.ncbi.nlm.nih.gov/pubmed/21876091 (Accessed on July 9, 2012.)
Vijayaraghavan SB: Sonographic whirlpool sign in ovarian torsion. J Ultra-
sound Med 2004; 23(12):1643-1649.
http://www.ncbi.nlm.nih.gov/pubmed/15557307 (Accessed on July 9, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 567, 569, 576, 578, 579.
Comment
Incidence and Clinical Findings
Ovarian torsion is a cause of acute abdominal pain in women
and requires rapid diagnosis and treatment. Torsion occurs
most commonly in women in their reproductive years; a nonadherent ipsilateral adnexal mass, most often a benign teratoma, is present in 50% to 81% of cases. Nonetheless, torsion
of a normal ovary can occur in prepubertal girls because of
adnexal mobility. Ovarian torsion is slightly more common on
the right side. Risk factors include the presence of an adnexal
mass, ovarian hyperstimulation syndrome, and pregnancy.
Ovarian torsion during pregnancy is facilitated by the presence of a corpus luteum during the rst trimester. A large cyst
that develops after ovulation induction can also be an inciting
factor. The pain is classically cyclical and worsens with each
cycle, although constant or vague pain may be the presenting
symptom.
Gray-Scale Ultrasound Imaging
Ovarian torsion has been detected with ultrasound, CT, and
MRI. With ultrasound, the ndings vary, depending on the age
of the patient (Figures A to D). However, an enlarged ovary
or mass should be present as the lead point of the torsion
(Figures A to D). The appearance of an ovary in torsion is
variable, depending on whether an underlying mass is present
and the degree of hemorrhage or necrosis resulting in cystic and hyperechoic components. Free uid is present in two
thirds of cases.
Color Doppler and Pulsed Doppler Imaging
Color Doppler ultrasound and pulsed Doppler ultrasound have
been shown to be unreliable in the denitive diagnosis of ovarian torsion. Although an enlarged abnormal-appearing ovary
with no arterial or venous ow may indicate torsion (Figures B
and C), arterial and venous ow has been shown in surgically
proven cases of ovarian torsion. Torsion can be intermittent.
The persistence of blood ow depends on the degree of torsion. Arterial ow with no diastolic component or absence of
venous ow can be seen early. Venous thrombosis may precede arterial occlusion. The twisted vascular pedicle is dened
as the rotation site of the ovarian pedicle and has been shown
on ultrasound. The pedicle is located either adjacent to the
ovary or ovarian mass or between the ovary and the uterus,
where vessels usually run in a straight course. A positive whirlpool sign in the twisted vascular pedicle has been reported as
the most denitive sign of ovarian torsion. In cases in which
ow is still identied in the twisted pedicle, the ovary is viable
at surgery and the pedicle is untwisted. The condition of no
ow in the twisted pedicle and ow in the artery alone but not
the vein has been predictive of nonviability of the ovary.
244
INDEX OF CASES
1 Tamoxifen, 3
2 Pleural Effusions, 5
3 Ovarian Cyst, 7
4 Ureteropelvic Junction Obstruction, 9
5 Choroid Plexus Cyst, 11
6 Cystic Hygroma and Fetal Hydrops, 13
7 Oligohydramnios, 15
8 Ovarian Cancer, 17
9 Acute Appendicitis, 19
10 Sacrococcygeal Teratoma, 21
11 Dandy-Walker Complex, 23
12 Central Nervous System Arteriovenous
Malformation, 25
13 Placenta Previa, 27
14 Anencephaly, 29
15 Duodenal Atresia, 31
16 Nuchal Thickness in the Second
Trimester, 33
17 Myelomeningocele, 35
18 Aqueductal Stenosis, 37
19 Omphalocele, 39
20 Congenital Uterine Anomalies and
Pregnancy, 41
21 Normal First Uterine Pregnancy with
Extrauterine Cystic Teratoma (Dermoid), 43
22 Conjoined (Siamese) Twins in the First
Trimester, 45
23 Congenital Cystic Adenomatoid Malforma-
tion (CCAM)/Congenital Pulmonary Airway
Malformation (CPAM), 47
24 Holoprosencephaly, 49
25 Polycystic Ovarian Disease, 51
26 Hydrocephalus, 53
27 Pulmonary Sequestration, 55
28 Intrauterine Contraceptive Device, 57
29 Enlarged Fetal Stomach, 59
30 Clubfoot, 61
31 Cystic Abdominal Mass, 63
32 Ectopic Pregnancy, Unruptured, 65
33 Pericardial Effusion, 67
34 Multicystic Dysplastic Kidney, 69
35 Trisomy 21 (Down Syndrome)—Basic, 71
36 Polyhydramnios, 73
37 Placental Infarct, 75
38 Hypoplastic Left Heart Syndrome, 77
39 Tetralogy of Fallot, 79
40 Ventricular Septal Defect, 81
41 Endometrial Cancer, 83
42 In Utero Infection, 85
43 Endocardial Cushion Defect, 87
44 Gastroschisis, 89
45 Dermoid, 91
46 Congenital Diaphragmatic Hernia,
Left-Sided Bochdalek Hernia, 93
47 Subchorionic Hemorrhage, 95
48 Single Umbilical Artery (Two-Vessel
Umbilical Cord), 97
49 Fetal Demise, 99
50 Ruptured Ectopic Pregnancy, 101
51 Ebstein Anomaly, 103
52 Ovarian Cyst, 107
53 Trisomy, 18, 109
54 Calvarial Abnormalities, 111
55 Three-Vessel View of Heart, 113
56 Umbilical Artery Doppler, 115
57 Posterior Urethral Valves, 117
58 Early Intrauterine Gestational Sac, 119
59 Hydranencephaly, 121
60 Retained Products of Conception, 123
61 Nonimmune Fetal Hydrops, 125
62 Uterine Masses, 127
63 Incompetent Cervix, 129
64 Intracranial Hemorrhage, 131
65 Placenta Accreta, 133
66 Small Bowel Obstruction, 135
67 Meconium Pseudocyst, 137
68 Fetal Liver Calcications, 139
69 Placental Abruption, 141
70 Cleft Lip and Palate, 143
71 Esophageal Atresia, 145
72 Nuchal Skin Measurement, 147
73 Ovarian Vein Thrombosis, 149
74 Large Bowel (Anorectal) Atresia, 151
75 Second-Trimester Twin Gestation and Fetal
Demise, 153
76 Osteogenesis Imperfecta, 155
77 Heterotopic Pregnancy, 157
78 Fetal Gallbladder, 159
79 d-Transposition of the Great Vessels, 161
80 Cardiac Rhabdomyoma, 163
81 Endometrial Polyp, 165
82 Pelvic Inammatory Disease, 167
83 Trisomy, 13, 169
84 Gestational Trophoblastic Disease, 171
85 Cervical Ectopic Pregnancy, 173
86 Succenturiate Lobe, 175
87 Twin-Twin Transfusion, 177
88 Advanced Down Syndrome
(Trisomy 21), 179
89 Autosomal Recessive Polycystic Kidney
Disease, 181
90 Endometriosis, 183
91 Limb–Body Wall Complex, 185
92 Encephalocele, 189
93 VATER (VACTERL) Association, 191
94 Cornual (Interstitial) Ectopic Pregnancy, 193
95 Congenital High Airway Obstruction, 195
96 Obligate Cord, 197
97 Ectopic Ureterocele, 199
98 Ectopia Cordis, 201
99 Ovarian Hyperstimulation Syndrome, 203
100 Endometritis and Endomyometritis, 205
101 Incarcerated Uterus, 207
102 Congenital Uterine Anomalies, 209
103 Acardiac Twin, 211
104 Porencephaly, 213
105 Umbilical Cord Cyst, 215
106 Agenesis of the Corpus Callosum, 217
107 Mass and Pain during Pregnancy, 219
108 Circumvallate Placenta (Placenta
Extrachorialis), 221
109 Thanatophoric Dysplasia (Thanatophoric
Dwarsm), 223
110 Sex Cord-Stromal (Thecoma-Fibroma)
Tumor, 225
111 Subdural Hematoma, 227
112 Adenomyosis, 229
113 Abdominal Ectopic Pregnancy, 231
114 Fetal Bladder Outlet Obstruction, 233
115 Fetal Goiter, 235
116 Velamentous Insertion of the Umbilical
Cord, 237
117 Heterotaxy Syndrome and Cardiosplenic
Syndrome, 239
118 Umbilical Vein Varix, 241
119 Ovarian Torsion, 243
245