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ANSWERS
CASE 105
Umbilical Cord Cyst
1. A , B, C, and D
2. A
3. B
4. C
References
Dudiak CM, Salomon CG, Posniak HV, et al: Sonography of the umbilical
cord. Radiographics 1995; 15(5):1035-1050.
http://www.ncbi.nlm.nih.gov/pubmed/7501849 (Accessed on June 22, 2012.)
Kalter CS, Williams MC, Vaughn V, et al: Sonographic diagnosis of a large
umbilical cord pseudocyst. J Ultrasound Med 1994; 13(6):487-489.
http://www.ncbi.nlm.nih.gov/pubmed/8083952 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 491-493.
Comment
Differential Diagnosis
Cystic masses of the umbilical cord (Figures A and B) may be
true cysts (allantoic duct or omphalomesenteric duct remnants),
dilated vasculature, or pseudocysts (e.g., Wharton’s jelly cysts).
Ultrasound Imaging
When a cystic mass is detected, the exact etiology is difcult
to determine with prenatal ultrasound. Color Doppler imaging should be used to determine if the vessels of the cord are
compressed or thrombosed and to exclude a vascular anomaly
as the cause of the cyst. Follow-up scans should be performed
throughout the gestation period.
Associated Anomalies
Cysts that arise from either allantoic or omphalomesenteric
ducts can be associated with genitourinary and gastrointestinal
anomalies. Specic associations include bowel or genitourinary
obstruction, hernia, omphalocele, and patent urachus. Recognition of an association with a patent urachus is essential to
avoid the complication of transecting the urachus when cutting the umbilical cord, particularly if the cyst arises close to
the anterior abdominal wall of the fetus. Omphalomesenteric
duct cysts are lined with epithelium that can differentiate into
gastric epithelia and possibly secrete acid. Ulceration can occur,
resulting in a fetal hemorrhage. Pseudocysts are collections of
liqueed Wharton’s jelly that may resolve but have also been
reported in association with trisomy 18 and trisomy 13.
216
CASE 106
A
Used with permission from McGahan JP, Benacerraf BR: Fetal head and
brain. In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd
ed. New York: Informa Healthcare USA, 2008.
History: A 36-year-old woman presents for level II obstet-
ric ultrasound because of advanced maternal age.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Arnold-Chiari type II malformation
B. Dandy-Walker malformation
C. Alobar holoprosencephaly
D. Hydranencephaly
E. Agenesis of the corpus callosum
2. In agenesis of the corpus callosum, which portion of the
lateral ventricles is typically dilated?
A. Frontal horns
B. Body of the lateral ventricles
C. Occipital horns
D. Temporal horns
3. Which of the following statements concerning agenesis of
the corpus callosum is false?
A. Agenesis of the corpus callosum may be diagnosed in
the rst trimester of pregnancy by means of endovagi-
nal ultrasound.
B. The cavum septi pellucidi is absent.
C. Ventriculomegaly is present.
D. Lateral ventricles are parallel to each another on axial
scan.
4. Other ndings associated with agenesis of the corpus cal-
losum include all of the following except:
A. An interhemispheric cyst is present.
B. The third ventricle is always in a normal position.
C. A lipoma within the midline may be present.
D. Heterotopia of the gray matter is present.
B
C
217
ANSWERS
CASE 106
Agenesis of the Corpus Callosum
1. A, B, and E
2. C
3. A
4. B
References
Cignini P, D’Emidio L, Padula F, et al: The role of ultrasonography in
the diagnosis of fetal isolated complete agenesis of the corpus callosum: a long-term prospective study. J Matern Fetal Neonatal Med 2010;
23(12):1504-1509.
http://www.ncbi.nlm.nih.gov/pubmed/20230313 (Accessed on June 22, 2012.)
Pilu G, Segata M, Ghi T, et al: Diagnosis of midline anomalies of the fetal
brain with the three-dimensional median view. Ultrasound Obstet Gynecol
2006; 27(5):522-529.
http://www.ncbi.nlm.nih.gov/pubmed/16586477 (Accessed on June 22, 2012.)
Sandhu PS, Khong K, McGahan JP, et al: Novel presentation of Aicardi
syndrome with agenesis of the corpus callosum and an orbital cyst.
J Ultrasound Med 2010; 29(5):843-846.
http://www.ncbi.nlm.nih.gov/pubmed/20427798 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 397.
Comment
Differential Diagnosis
The differential diagnosis of mild ventriculomegaly includes
numerous different causes of hydrocephalus. However, the nding of three echogenic lines parallel to one another in the brain
on an axial scan in association with mild colpocephaly is most
commonly associated with agenesis of the corpus callosum.
Ultrasound Findings
Ultrasound features of complete agenesis of the corpus callosum include absent cavum septi pellucidi and complete
agenesis of the corpus callosum. However, because the corpus
callosum develops late in gestation, it is not completed until
18 weeks’ gestation. Early diagnosis of agenesis of the corpus
callosum is difcult. There is usually ventriculomegaly involving the atria and occipital horns; this is termed colpocephaly
(Figures A and B). Separate parallel lines are noted on axial
scan (Figure C). The ventricles form a “teardrop” conguration and are parallel to one another (Figure C). Displacement
of the third ventricle and other intracranial abnormalities are
often present and may be better diagnosed with MRI.
Prognosis and Management
The prognosis of complete agenesis of the corpus callosum
depends on various factors. Malformations are identied in
association with agenesis of the corpus callosum with a high
frequency. Agenesis of the corpus callosum is associated with
the major trisomies and various syndromes, including MeckelGruber syndrome, Miller-Dieker syndrome, and Aicardi syndrome. A complete survey of the entire fetal anatomy should
be performed. Karyotyping is advisable. In addition, multiple
systemic anomalies are associated with agenesis of the corpus
callosum, including cardiovascular, genitourinary, and gastrointestinal malformations. In cases of isolated agenesis of the
corpus callosum, children may have normal intelligence, but
they may have peculiar neurologic ndings or cognitive decits
ranging from subtle to more severe impairment.
218
CASE 107
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 woman presents in the early second trimester
with left lower quadrant pain.
1. What should be included in the differential diagnosis of a
pregnancy with a mass and pain based on the imaging ndings in Figures A and B? (Choose all that apply.)
A. Acute appendicitis
B. Uterine broids
C. Ovarian torsion
D. Adnexal masses
E. Heterotopic pregnancy
2. Based on the imaging ndings in Figure C, what is the most
likely diagnosis of this right-sided cyst in pregnancy?
A. Appendiceal abscess
B. Corpus luteum cyst
C. Ovarian torsion
D. Heterotopic pregnancy
3. Which of the following statements concerning pregnancy
and abdominal pain is false?
A. Ultrasound is a rst-line tool in imaging a pregnant
patient with pain.
B. Gadolinium is useful with MRI in evaluation of abdom-
inal pain during pregnancy.
C. In pregnant trauma patients, the focused abdominal
scan in trauma (FAST) scan should be used before CT.
D. In trauma patients, the diagnosis of abruptio placentae
is often missed.
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.
4. Which of the following statements concerning etiologies
of nontraumatic abdominal pain of pregnancy is false?
A. MRI may have improved sensitivity over ultrasound in
evaluation of appendicitis.
B. In a pregnant patient, pancreatitis is most likely second-
ary to medications during pregnancy.
C. In a pregnant patient, bowel obstruction occurs more
often in the third trimester than the rst trimester.
D. Appendicitis and acute cholecystitis are the most com-
mon nonobstetric indications for emergent surgery in
pregnant patients.
C
219
ANSWERS
CASE 107
Mass and Pain during Pregnancy
1. B, C, D, and E
2. C
3. B
4. B
References
Mkpolulu CA, Ghobrial PM, Catanzano TM: Nontraumatic abdominal pain
in pregnancy: imaging considerations for a multiorgan system problem.
Semin Ultrasound CT MR 2012; 33(1):18-36.
http://www.ncbi.nlm.nih.gov/pubmed/22264900 (Accessed on June 22, 2012.)
Richards JR, Ormsby EL, Romo MV, et al: Blunt abdominal injury in the
pregnant patient: detection with US. Radiology 2004; 233(2):463-470.
http://www.ncbi.nlm.nih.gov/pubmed/15516618 (Accessed on June 22, 2012.)
Wallace GW, Davis MA, Semelka RC, et al: Imaging the pregnant patient with
abdominal pain. Abdom Imaging 2011 Dec 13. [Epub ahead of print]
http://www.ncbi.nlm.nih.gov/pubmed/22160283 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 567-569.
Comment
Differential Diagnosis
The differential diagnosis of an adnexal mass on the right
side occurring in pregnancy includes appendicitis with appendiceal abscess; this is a common nonobstetric cause of acute
abdominal pain and a mass in pregnancy. Other common nonobstetric etiologies of pain, such as acute cholecystitis, bowel
obstruction, and renal colic, usually do not produce a mass. An
adnexal mass with or without torsion seems to be the most
likely etiology of adnexal pain with a mass in pregnancy. Heterotopic pregnancy manifesting as an adnexal mass is very rare.
Ultrasound Findings
Ultrasound features of heterotopic pregnancy include a normal intrauterine pregnancy plus pregnancy located elsewhere,
usually within the pelvis (Figure A). In this case, the ectopic
pregnancy was in the left adnexa and ruptured, causing free
uid in the pelvis (Figure B). Other locations of heterotopic
pregnancy include the cervix and cornua. Cornual pregnancy
may be difcult to recognize.
Ovarian torsion tends to occur with increased frequency in
pregnancy, probably secondary to the mass effect of the pregnancy. An estimated 45% of cases of ovarian torsion manifest
during the second or third trimester of pregnancy. Twisting
of the ovarian vascular pedicle may obstruct lymphatic ow
and create an enlarged ovary. Venous ow may be obstructed,
and arterial ow may be decreased (Figure C), but there may
be decreased arterial diastolic Doppler signal. Free uid in the
abdomen may be an associated nding. Ovarian torsion may
also occur because of an underlying ovarian mass as in this
case; the patient had a cystadenoma, which was present before
pregnancy.
Prognosis and Management
Prognosis and management of an abdominal mass with pain
during pregnancy depend on the etiology. Surgery is usually
needed for ovarian torsion or heterotopic pregnancy, as in this
case.
220
CASE 108
A
P = placenta.
History: An asymptomatic patient in her third trimester
undergoes imaging.
1. What is the name of the outpouching (arrow) at the edge of
the placenta (P) into the amniotic uid shown in Figure A?
A. Succenturiate lobe
B. Amniotic shelf
C. Circumvallate placenta
D. Amniotic sheet
2. Which of the following is not a complication associated
with a circumvallate placenta?
A. Large birth weight
B. Low birth weight
C. Premature labor
D. Placental abruption
B
P = placenta.
3. What is the large soft tissue band not abutting a fetal part
(arrow) in another third-trimester pregnancy shown in Figure B?
A. Amniotic band
B. Amniotic shelf
C. Circumvallate placenta
4. What is the etiology of a circumvallate placenta?
A. Mismatch in growth at the placental margin
B. Trisomy 21
C. Maternal diabetes mellitus
D. Vascular malformation
221
ANSWERS
CASE 108
Circumvallate Placenta (Placenta Extrachorialis)
1. C
2. A
3. B
4. A
References
Arlicot C, Herve P, Simon E, et al: Three-dimensional surface rendering of
the chorionic placental plate: the “tire” sign for the diagnosis of a circum-
vallate placenta. J Ultrasound Med; 31:337-341, 2012.
http://www.ncbi.nlm.nih.gov/pubmed/22298880 (Accessed on July 2, 2012.)
Harris RD, Wells WA, Black WC, et al: Accuracy of prenatal sonogra-
phy for detecting circumvallate placenta. AJR Am J Roentgenol 1997;
168(6):1603-1608.
http://www.ncbi.nlm.nih.gov/pubmed/9168736 (Accessed on June 22, 2012.)
McCarthy J, Thurmond AS, Jones MK, et al: Circumvallate placenta: sono-
graphic diagnosis. J Ultrasound Med 1995; 14(1):21-26.
http://www.ncbi.nlm.nih.gov/pubmed/7707472 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 520.
Comment
Etiology and Incidence
Circumvallate placenta, or placenta extrachorialis, is a common disorder. Complete circumvallate placenta occurs in 1%
of pregnancies; partial circumvallate placenta is estimated to
occur 10 to 20 times more commonly. This condition results
from a mismatch in growth at the placental margin. In typical placentation, there is a smooth transition from the parenchymal villous chorion to the membranous chorion. In partial
or complete circumvallate placenta, the parenchymal villous
chorion overgrows and bulges into the amniotic uid. This
leaves villous tissue of the chorionic frondosum that is not
completely covered by the amniochorionic membrane—hence
the term placenta extrachorialis.
Ultrasound Imaging
In circumvallate placenta, the parenchymal villous chorionic
tissue bulges out peripherally and the edge of the placenta
develops a rolled appearance (Figure A). Amniotic shelves or
sheets (Figure B), thought to be due to synechiae covered by
amniochorionic membrane, are usually larger but otherwise
similar in appearance to a circumvallate placenta. Ultrasound
is neither sensitive nor specic for diagnosis, as shown by one
study by experienced sonologists.
Clinical Significance
The clinical signicance of circumvallate placenta is uncertain. It has been associated with low birth weight, prematurity,
intrauterine growth restriction, placental abruption, and perinatal death. In addition, congenital anomalies may be present,
although the exact etiology of this nding is uncertain.
222
CASE 109
A
B
History: A woman in the late third trimester presented for
late prenatal care.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Heterozygous achondroplasia
B. Thanatophoric dysplasia
C. Osteogenesis imperfecta
D. Intrauterine growth restriction
E. Syringomyelia
2. All of the following entities have a small thorax except:
A. Achondrogenesis
B. Asphyxiating thoracic dysplasia (Jeune syndrome)
C. Osteogenesis imperfecta type II
D. Cleidocranial dysostosis
3. Signicant shortening of the fetal limbs associated with an
abnormal skull contour can occur with all of the following
except:
A. Thanatophoric dysplasia
B. Osteogenesis imperfecta
C. Crouzon syndrome
D. Homozygous achondroplasia
4. Which of the following is not associated with thanatophoric
dysplasia?
A. Hydrocephalus
B. Oligohydramnios
C. Renal abnormalities
D. Cardiac septal defects
C
D
223
ANSWERS
CASE 109
Thanatophoric Dysplasia (Thanatophoric
Dwarfism)
1. B and C
2. D
3. C
4. B
References
Bekdache GN, Begum M, Al-Gazali L, et al: Prenatal diagnosis of thana-
tophoric dysplasia and obstetrical challenges. J Obstet Gynaecol 2010;
30(6):628-630.
http://www.ncbi.nlm.nih.gov/pubmed/20701518 (Accessed on June 22, 2012.)
Fink AM, Hingston T, Sampson A, et al: Malformation of the fetal brain
in thanatophoric dysplasia: US and MRI ndings. Pediatr Radiol 2010;
40(suppl 1):S134-S137.
http://www.ncbi.nlm.nih.gov/pubmed/20499057 (Accessed on June 22, 2012.)
Khalil A, Pajkrt E, Chitty LS: Early prenatal diagnosis of skeletal anomalies.
Prenat Diagn 2011; 31(1):115-124.
http://www.ncbi.nlm.nih.gov/pubmed/21210484 (Accessed on June 22, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 475-481.
Comment
Differential Diagnosis
The differential diagnosis in this case is broad and includes
all forms of severe short limb dwarsm: homozygous
achondroplasia, asphyxiating thoracic dysplasia (Jeune syndrome), chondroectodermal dysplasia, and thanatophoric
dysplasia. Four major types of skeletal dysplasias account for
nearly two thirds of all dysplasias. The most common skeletal
dysplasia is thanatophoric dysplasia, followed by heterozygous
achondroplasia, osteogenesis imperfecta, and achondrogenesis. Of these, thanatophoric dysplasia is the most common
lethal dysplasia, whereas heterozygous achondroplasia is the
most common nonlethal skeletal dysplasia.
Ultrasound Findings
A striking ultrasound nding is marked discrepancy between
the femur length measurements and other biometric data as
seen in this case (Figure A). There are two different types of
thanatophoric dysplasia: type I and type II. Features seen in
both types include a small thorax and short femur. In type II,
a cloverleaf skull is prominent. Progressive hydrocephalus is
often present when the cloverleaf skull deformity is identied (Figures B to D). Polyhydramnios may develop in many
cases. In addition to hydrocephalus, associated abnormalities
include renal abnormalities, atrial septal defects, and imperforate anus.
Prognosis and Management
Thanatophoric dysplasia is uniformly a lethal condition. The
cause of death is respiratory failure secondary to hypoplastic
lungs. Most cases result from new mutations. There is a small
risk of recurrence.
224
CASE 110
A
B
D
History: A 35-year-old patient presents for baseline ultra-
sound assessment for infertility.
1. What should be included in the differential diagnosis based
on ultrasound and MRI (Figures A to C)? (Choose all that
apply.)
A. Endometrioma
B. Hypoechoic solid ovarian mass with shadowing, a sex
cord-stromal tumor
C. Ovarian dermoid
D. Pedunculated broid tumor of the uterus
2. Which subtype of this tumor is more likely to be malignant?
A. Granulosa-stromal cell type
B. Sertoli-Leydig cell tumor
C. Thecoma-broma
3. What is a Call-Exner body?
A. Microfollicular and macrofollicular patterns (rosettelike)
that are seen with Leydig cells when viewed cytologically
B. Microfollicular and macrofollicular patterns (rosette-
like) that are seen with granulosa cells when viewed
cytologically
C. Rosettelike patterns that are seen in all hormonally
active ovarian tumors
D. Rosettelike patterns that are seen in all non-hormonally
C
active ovarian tumors
4. Which subtype is most likely to have an associated endome-
trial cancer?
A. Fibroma
B. Thecoma
C. Sertoli-Leydig tumor
225