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ANSWERS
CASE 86
Succenturiate Lobe
1. C
2. A
3. C
4. C
References
Hata K, Hata T, Aoki S, et al: Succenturiate placenta diagnosed by ultra-
sound. Gynecol Obstet Invest 1988; 25(4):273-276.
http://www.ncbi.nlm.nih.gov/pubmed/3042556 (Accessed on June 18, 2012.)
Nelson LH, Fishburne JI, Stearns BR: Ultrasonographic description of a suc-
centuriate placenta. Obstet Gynecol 1977; 49(1 suppl):79-80.
http://www.ncbi.nlm.nih.gov/pubmed/831187 (Accessed on June 18, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 488.
Comment
Etiology and Incidence
A rare but important placental abnormality is shown in this
case—the presence of a succenturiate lobe. A succenturiate
lobe is an accessory placental lobe that is removed from, but
is in vascular continuity with, the main placenta. Fetal vessels
under the amniochorionic membranes connect this accessory
lobe to the main placenta. This anomaly is reported to occur
in 0.28% of pregnancies. The etiology is a regional failure of
placental villi to atrophy.
Ultrasound Imaging
Despite its rarity, a succenturiate lobe is an important abnormality to detect on prenatal ultrasound. Ultrasound can detect
and localize the accessory placenta.
Prognosis
Several serious complications can ensue if a succenturiate lobe
is not detected. Prenatally and during labor, the connecting
vessels can rupture, leading to life-threatening fetal hemorrhage. A vasa previa (fetal hemorrhage) can develop at the time
of labor and delivery if these vessels are over the internal cervical os. If the succenturiate lobe is not delivered, it can lead to
postpartum maternal hemorrhage and infection. A succenturiate lobe should be suspected at the time of delivery if severed
fetal vessels are present at the torn edge of the membranes of
the placenta.
176
CASE 87
A
C
B
History: An asymptomatic patient with a second-trimester
twin pregnancy undergoes imaging.
1. What are ndings in twin-twin transfusion (TTT) syndrome? (Choose all that apply.)
A. One twin (donor) can become small and develop ane-
mia and oligohydramnios.
B. The recipient twin enlarges and develops polycythemia,
volume overload, heart failure, and polyhydramnios.
C. The donor twin can become a “stuck” twin.
D. The twins have a very low preterm birth weight.
2. Which of the following is one of the ultrasound criteria for
TTT?
A. Dichorionic twinning
B. Twins not of the same gender
C. Growth discordance between the twins of at least 50%
D. Monochorionic or fused placenta
D
3. In the second twin pregnancy in Figure D with TTT syndrome in the late third trimester, what is detected in the
freely moving twin on the spectral Doppler tracing of the
right atrium?
A. High resistance index
B. High diastolic ow
C. Myocardial infarct
D. Tricuspid regurgitation
4. What has been reported to happen to the recipient twin if
the donor twin dies?
A. Embolic phenomenon
B. Becoming a stuck twin
C. Acute myocardial infarction
D. Causing maternal death
177
ANSWERS
CASE 87
Twin-Twin Transfusion
1. A, B, and C
2. D
3. D
4. A
References
Carver AC, Haeri S, Moldenhauer J, et al: Monochorionic diamniotic twin
pregnancy. J Ultrasound Med 2011; 30(3):297-301.
http://www.ncbi.nlm.nih.gov/pubmed/21357550 (Accessed on June 18, 2012.)
Duncombe GJ, Dickinson JE, Evans SF: Perinatal characteristics and out-
comes of pregnancies complicated by twin-twin transfusion syndrome.
Obstet Gynecol 2003; 101(6):1190-1196.
http://www.ncbi.nlm.nih.gov/pubmed/12798524 (Accessed on June 18, 2012.)
Moreira de Sa RA, Salomon LJ, Takahashi Y, et al: Analysis of fetal growth
after laser therapy in twin-to-twin transfusion syndrome. J Ultrasound Med
2005; 24(9):1213-1219.
http://www.ncbi.nlm.nih.gov/pubmed/16123181 (Accessed on June 18, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 516, 521, 523-524.
Comment
Twin-Twin Transfusion Syndrome
TTT syndrome is a serious complication in 10% to 15% of
monochorionic gestations and is a heterogeneous disorder in
its clinical manifestations and progress. TTT is strictly dened
by interconnecting placental vessels between the two twins,
which result in umbilical arteriovenous shunting of blood
from one twin to the other (Figures A and B). When significant shunting occurs, one twin (donor) becomes small and
develops anemia and oligohydramnios (Figure B). The other
twin (recipient) enlarges and develops polycythemia, volume
overload, heart failure, and polyhydramnios (Figure A). In the
most severe form of TTT, the smaller twin can become stuck
to the intervening membrane, with a very high mortality rate
(Figure C). A high preterm birth rate typies TTT.
Ultrasound Imaging
Ultrasound criteria for TTT include monochorionic twinning
(a monochorionic [fused] placenta), twins of the same gender,
and a growth discordance between the twins of at least 20%.
Polyhydramnios is often present in the larger sac, and oligohydramnios is present in the smaller sac. Oligohydramnios may
be so severe that the “stuck” fetus is difcult to identify. Ultrasound evaluation of this syndrome should begin in the second
trimester.
Complications
The recipient twin is subject to several complications. Cardiomegaly results from biventricular hypertrophy and dilation.
Tricuspid regurgitation is often present (Figure D). If the
donor twin dies, the recipient can experience an embolic phenomenon through the arteriovenous connections, resulting in
cerebral damage. Color Doppler ultrasound evaluation of the
placenta has largely failed to identify the signicant vascular
connections. Laser therapy performed as laser coagulation of
chorionic plate anastomoses has altered recipient growth patterns toward a decrease, while not altering donor growth patterns. Less twin discordance has resulted. Donor twins have
been found to have a higher rate of noncardiac anomalies
compared with the recipient twins after laser therapy.
178
CASE 88
C
A
B
History: A patient with advanced maternal age undergoes a
20-week ultrasound examination.
1. Which chromosomal abnormalities should be included in
the differential diagnosis for Figure A? (Choose all that
apply.)
A. Normal
B. Trisomy 18
C. Trisomy 21
D. Turner syndrome (45, XO karyotype)
E. Trisomy 13
2. Which of the following statements regarding echogenic
bowel is true?
A. Echogenic bowel should be as bright as bone.
B. Ultrasound assessment of echogenic bowel does not
depend on technical factors.
D
C. Echogenic bowel is a highly specic marker for Down
syndrome.
D. Echogenic bowel is a highly sensitive marker for Down
syndrome.
3. Which of the following statements regarding duodenal
atresia is true?
A. Duodenal atresia is not associated with trisomy 21.
B. Duodenal atresia is diagnosed prenatally by the pres-
ence of the double bubble sign in the fetus.
C. Continuity between the gastric and duodenal bubbles
does not have to be shown to exclude other etiologies
of cystic abdominal masses.
D. Mortality is higher in cases of duodenal atresia that are
diagnosed postnatally than in those diagnosed prenatally.
4. Sandal gap deformity refers to which of the following?
A. Convex plantar surface of the foot with posterior bulg-
ing of the calcaneus
B. Curvature of the fth nger toward the adjacent fourth
nger
C. Medial displacement of the great toe, giving rise to
greater than normal space between the rst and second
toes
D. Nonperpendicular alignment between the lower leg
bones and the sole of the foot
179
ANSWERS
CASE 88
Advanced Down Syndrome (Trisomy 21)
1. A, B, C, D, and E
2. A
3. B
4. C
References
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 18,
2012.)
de Jong-Pleij EA, Ribbert LS, Manten GT, et al: Maxilla-nasion-mandible
angle: a new method to assess prole anomalies in pregnancy. Ultrasound
Obstet Gynecol 2011; 37(5):562-569.
http://www.ncbi.nlm.nih.gov/pubmed/20922777 (Accessed on June 18, 2012.)
Towner D, Gerscovich EO, Chiong BB, et al: Comparison of single versus
multiple echogenic foci in the fetal hear t regarding risk of aneuploidy.
J Ultrasound Med 2010; 29(7):1061-1067.
http://www.ncbi.nlm.nih.gov/pubmed/20587429 (Accessed on June 18, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 394, 396, 436, 442, 453, 475.
Comment
Differential Diagnosis
The differential diagnosis of nuchal thickening (Figure A)
includes a nding in a normal fetus, trisomy 21, and other chromosomal abnormalities, including trisomy 13, trisomy 18, and
Turner syndrome (45,XO). When increased nuchal thickness
is a solitary nding, the differential diagnosis is broad. When
nuchal thickening is the only ultrasound nding detected, biochemical markers and maternal age must be considered for the
best risk assessment in these pregnancies.
Ultrasound Findings
Several anatomic abnormalities have a strong association with
trisomy 21, including endocardial cushion defect (which is discussed in more detail in Case 43: Endocardial Cushion Defect) and
duodenal atresia (which is discussed in more detail in Case 15:
Duodenal Atresia). Some of the basic ndings in trisomy 21 are
presented in Case 35.
Endocardial cushion defect consists of a large defect
involving the inferior portion of the atrial septum and the posterior portion of the ventricular septum. There is one common atrioventricular valve instead of two separate valves. The
defect can be accurately diagnosed by using the four-chamber
view. The double bubble sign of duodenal atresia on prenatal ultrasound represents an amniotic uid–lled stomach and
duodenum. It is important to show continuity between the two
bubbles to exclude other causes of an abnormal cystic mass,
such as a choledochal cyst. A dilated duodenum should be
persistently visible for several minutes because peristalsis can
cause transient dilation.
Commonly used markers of trisomy 21 on prenatal ultrasound are heart defects, duodenal atresia, hydrops, thick nuchal
fold (Figure A), absent or short nasal bone, short long bones,
echogenic bowel, echogenic intracardiac focus, and pyelectasis
(Figure B). Less commonly used markers are wide iliac angle,
at facies (Figure C), sandal gap foot (Figure D), short frontal
lobe, clinodactyly, brachycephaly, small ears, and small cerebellar diameter.
Hyperechoic bowel refers to fetal bowel that is as bright as
bone. Echogenicity of the bowel depends on the frequency
of the transducer and ultrasound technical factors; this is a
subjective nding that can be difcult to use. Sandal gap deformity (Figure D), which is medial displacement of the great toe
giving rise to greater than normal space between the rst and
second toes, and clinodactyly, which is curvature of the fth
nger toward the adjacent fourth nger, are two of the musculoskeletal markers of trisomy 21.
Prognosis and Management
A cluster of ultrasound markers on prenatal examination
greatly increases the likelihood that the patient is in a high-risk
category. A normal ultrasound examination with no markers
reduces the risk of fetal Down syndrome by 50% to 80%. This
information can be used to reassure patients and enable them
to forgo invasive procedures.
180
CASE 89
A
Used with permission from McGahan JP, et al: Fetal abdomen and pelvis. In McGahan JP, Goldberg B [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008; 1291-1336.
B
Used with permission from McGahan JP, et al: Fetal abdomen and pelvis. In McGahan JP, Goldberg B [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008; 1291-1336.
History: A patient in her third trimester undergoes an ultra-
sound scan.
1. What should be included in the differential diagnosis for
Figure A? (Choose all that apply.)
A. Infantile polycystic kidney disease
B. Adult polycystic kidney disease
C. Beckwith-Wiedemann syndrome
D. Normal
E. Bilateral multicystic dysplastic kidney
2. Which of the following types of autosomal recessive polycystic renal disease is not associated with signicant hepatic
brosis?
A. Perinatal
B. Neonatal
C. Infantile
D. Juvenile
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. Meckel-Gruber syndrome is associated with all of the following ndings except:
A. Polydactyly
B. Encephalocele
C. Cardiac anomalies
D. Enlarged echogenic kidneys
4. All of the following statements concerning the prognosis
and management of infantile polycystic kidney disease are
true except:
A. Infantile polycystic kidney disease is associated with
liver cysts in approximately 50% of cases.
B. Oligohydramnios is associated with infantile polycystic
kidney disease, with a very poor outcome owing to pulmonary hypoplasia.
C. The juvenile form of infantile polycystic disease is asso-
ciated with systemic hypertension.
D. The second-trimester ultrasound examination in
affected fetuses can appear normal.
181
ANSWERS
CASE 89
Autosomal Recessive Polycystic Kidney Disease
1. A, B, and C
2. A
3. C
4. A
References
Jang DG, Chae H, Shin JC, et al: Prenatal diagnosis of autosomal recessive
polycystic kidney disease by molecular genetic analysis. J Obstet Gynaecol Res
2011; 37(11):1744-1747.
http://www.ncbi.nlm.nih.gov/pubmed/21790888 (Accessed on June 18, 2012.)
Liu SS, Cheong ML, She BQ, et al: First-trimester ultrasound diagnosis of
Meckel-Grüber syndrome. Acta Obstet Gynecol Scand 2006; 85(6):757-759.
http://www.ncbi.nlm.nih.gov/pubmed/16752273 (Accessed on June 18, 2012.)
Nasu K, Yoshimatsu J, Anai T, et al: Magnetic resonance imaging of fetal
autosomal recessive polycystic kidney disease. J Obstet Gynaecol Res 1998;
24(1):33-36.
http://www.ncbi.nlm.nih.gov/pubmed/9564103 (Accessed on June 18, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 461-462.
Comment
Differential Diagnosis
The most likely differential diagnosis in this case would be
autosomal recessive polycystic kidney disease. In this entity,
there are bilateral enlarged echogenic kidneys. Very rarely,
adult polycystic kidney disease may be detected in the third trimester of pregnancy with slightly enlarged echogenic kidneys.
Meckel-Gruber syndrome also has bilateral enlarged echogenic
kidneys but with polydactyly and an encephalocele. BeckwithWiedemann syndrome may have enlarged echogenic kidneys
with macrosomia, an enlarged liver and spleen, macroglossia,
and occasionally an omphalocele. Trisomy 13 and other syndromes may have enlarged echogenic kidneys. Rarely, cytomegalic inclusion virus may manifest with enlarged echogenic
kidneys, but it is also associated with other features such as
microcephaly, enlarged cerebral ventricles, and intracranial
calcications.
Ultrasound Findings
Ultrasound ndings of infantile polycystic kidney disease are
classic as in this case with bilaterally enlarged echogenic kidneys, associated oligohydramnios, and absent or small bladder
(Figures A and B). Other entities included in the differential
diagnosis usually have a normal amount of amniotic uid
except for Meckel-Gruber syndrome, which is associated with
oligohydramnios. Also, in less severe forms of infantile polycystic kidney disease, the amniotic uid amount may be normal and a urinary bladder may be present. MRI may be useful
in these cases (Figure C).
Prognosis and Management
The prognosis for the perinatal form of infantile polycystic
kidney disease is dismal. Affected newborns die within hours
to weeks after birth. There is an association of pulmonary
hypoplasia owing to oligohydramnios with poor lung development. In addition, these fetuses have renal failure. Less
severe forms of infantile polycystic kidney disease, including
juvenile forms, have a better prognosis. The less severe forms
of disease have less severe renal involvement; however, they
have more severe hepatic brosis and associated liver disease,
including portal hypertension and its complications. In cases
with a family history of autosomal recessive polycystic kidney
disease, the outlook is poor. In cases with a family history, the
karyotype would be known. In other cases, karyotyping may be
helpful, especially if a particular syndrome is suspected.
182
CASE 90
A
DD
History: A 30-year-old woman presents with pelvic “full-
ness” on the left side on bimanual pelvic examination.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Corpus luteum
B. Dermoid
C. Endometriosis
B
D. Hemorrhagic ovarian cyst
2. What is the classic ultrasound appearance of an endometrioma?
A. Homogeneous low-level echoes
B. Mass containing many shadowing calcications
C. Reticular, web pattern
D. Anechoic simple cyst
3. How would the sensitivity of ultrasound for detecting
endometriosis be described?
A. Highly sensitive—greater than 90%
B. Rarely detects endometriosis—32%
C. Mildly sensitive—detects about 71%
D. Sensitivity of 50% to detect endometriosis
4. Which of the following statements about endometriosis is
true?
A. Endometriosis does not adversely affect fertility.
B. Endometriosis does not occur outside the female pelvis.
C
C. An endometrioma is a cyst containing altered blood,
usually arising from an ovary.
D. Endometriomas never have ow on color Doppler.
183
ANSWERS
CASE 90
Endometriosis
1. C and D
2. A
3. C
4. C
References
Bazot M, Darai E, Hourani R, et al: Deep pelvic endometriosis: MR imag-
ing for diagnosis and prediction of extension of disease. Radiology 2004;
232(2):379-389.
http://www.ncbi.nlm.nih.gov/pubmed/15205479 (Accessed on June 18, 2012.)
Kuligowska E, Deeds L, Lu K III: Pelvic pain: overlooked and underdiag-
nosed gynecologic conditions. Radiographics 2005; 25(1):3-20.
http://www.ncbi.nlm.nih.gov/pubmed/15653583 (Accessed on June 18, 2012.)
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 18, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 570-571, 573-574.
Comment
Definition
Endometriosis is a disorder in which benign endometrial glands
and stroma are located outside of the uterus (Figures A to D).
The diagnosis is made denitively with laparoscopy. Implants
of endometrial tissue are commonly seen on the ovary, uterus,
and ligament in the cul-de-sac and can be found less commonly
on the rectosigmoid colon, bladder (Figures B to D), cervix, and
vagina. Small implants are not seen with ultrasound, accounting
for the low sensitivity of ultrasound to detect endometriosis.
MRI has been shown to be more sensitive because of the ability to detect small endometriomas (<1 cm) and to characterize
blood accurately based on signal intensity. In addition, MRI can
reveal implants or evidence of hemosiderin along the peritoneum and adhesions that are the sequelae of endometriosis.
Endometriosis can seed abdominal scars.
Ultrasound Imaging
An endometrioma is a cyst that contains altered blood, usually arises from an ovary, and is often bilateral. On ultrasound,
endometriomas usually have internal echoes and often have
homogeneous low-level echoes (Figure A). The internal material can be hyperechoic. Internal septations or uid-uid levels
may be present. There are usually no solid components. Blood
ow on Doppler is not seen in ne septations but can be seen
in thick septations. An endometrioma is the only form of
endometriosis that can be readily diagnosed with transvaginal
ultrasound. Initial follow-up is in 6 to 12 weeks and then yearly
if the endometrioma is not removed.
MRI
Endometriomas are often hyperintense on T1-weighted MRI.
On T2-weighted sequences, they may show a relative decrease
in signal intensity (“shading”), or they can be hyperintense
(Figure D). The presence of shading is the most accurate criterion to distinguish an endometrioma from other hemorrhagic
or nonhemorrhagic adnexal masses. MRI has been found to
aid in the detection of deep pelvic endometriosis, also referred
to as deep inltrating endometriosis, located under the surface
of the peritoneum.
184
CASE 91
A
Used with permission from McGahan JP, Benacerraf BR: Fetal abdomen
and pelvis. In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound,
2nd ed. New York: Informa Healthcare USA, 2008.
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.
B
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.
History: A patient undergoes a prenatal ultrasound scan
that shows an unusual appearance to the fetus.
1. What should be included in the differential diagnosis for
Figure A? (Choose all that apply.)
A. Neural tube defect
B. Limb–body wall complex
C. Amniotic band syndrome
D. Trisomy 21
E. VACTERL syndrome
2. What is the most common genetic abnormality that occurs
with limb–body wall complex?
A. No known association
B. Trisomy 18
C. Trisomy 21
D. XO Turner syndrome karyotype
D
Used with permission from McGahan JP, Benacerraf BR: Fetal abdomen
and pelvis. In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound,
2nd ed. New York: Informa Healthcare USA, 2008.
3. Which of the following ndings is not associated with limb–
body wall complex?
A. Normal maternal serum alpha-fetoprotein
B. High percentage of cardiac defects
C. Absent diaphragm
D. No body part is precluded from this defect.
4. Which of the following statements concerning limb–body
wall complex is false?
A. This defect is not usually detected until the third trimes-
ter of pregnancy.
B. Often the fetal parts are fused with the placenta.
C. Amniotic band syndrome and limb–body wall complex
may have similar features.
D. The umbilical cord in limb–body wall complex is usually
short.
185