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CASE 52
A
2
3
1
1
2
C
1
B
History: A 40-year-old woman presents with right adnexal 
pain.
 1.   To evaluate an ovarian cyst, what questions do you need to  ask the patient? (Choose all that apply.)
  A.   Date of  last menstrual period   B.   Whether the patient is postmenopausal   C.   Whether the patient has ever been pregnant   D.   Whether the patient is receiving a hormone treatment
 2.   What is the usual appearance of  a corpus luteum?
  A.   A dominant follicle   B.   A cyst with a spongelike, lacelike, or reticular pattern   C.   A complex cyst with echogenic and shadowing foci   D.   A homogeneous echogenic cyst
D
 3.   What is the size of  a usual corpus luteum?   A.   Less than 1 cm   B.   Greater than 10 cm   C.   2.5 to 10 cm
3
3
3
  D.   There are no size guidelines for a corpus luteum.
 4.   Which statement is  correct regarding simple ovarian cysts  in postmenopausal women?
  A.   Simple ovarian cysts do not occur normally.   B.   These cysts are present in  5%  of   asymptomatic post-
menopausal patients.   C.   These cysts disappear in 50% of  women within 2 years.   D.   Simple ovarian cysts less than 6 cm in diameter can be 
safely followed.
107
ANSWERS
CASE 52
Ovarian Cyst
 1.   A, B, and D
 2.   B
 3.   C
 4.   C
References
Levine D, Brown DL, Andreotti RF: Management of  asymptomatic ovarian 
and other adnexal cysts imaged at US: Society of  Radiologists in Ultra-
sound Consensus Conference Statement. Radiology 2010; 256(3):943-954.
http://www.ncbi.nlm.nih.gov/pubmed/20505067 (Accessed on June 12, 2012.)
Patel MD, Feldstein VA, Filly RA: The likelihood ratio of  sonographic nd-
ings for the diagnosis of  hemorrhagic ovarian cysts. J Ultrasound Med 2005; 
24(5):607-614.
http://www.ncbi.nlm.nih.gov/pubmed/15840791 (Accessed on June 12, 2012.)
Timor-Tritsch IE, Goldstein SR: The complexity of  a “complex mass” and 
the simplicity of  a “simple cyst.” J Ultrasound Med 2005; 24(3):255-258.
http://www.ncbi.nlm.nih.gov/pubmed/15723838 (Accessed on June 12, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 560-561, 563, 567, 568, 570-571.
Comment
Complex Ovarian Cysts in Premenopausal Women
A  hemorrhagic  corpus luteum (also called  a  cyst)  is  a  com­mon cause of   pelvic pain. It  is a physiologic  cyst that forms  on  release  of   the  oocyte.  The  corpus  luteum  may  rupture,  resulting  in  a  hemorrhagic  cyst  that  may  be  symptomatic.  These  cysts  vary  in  size  from  2.5  to  10  cm2;  the  wall,  from  2 to 22 mm. The cyst composition causes posterior through­transmission. However,  the  internal  echo pattern varies with  the stage of the hemorrhage, best appreciated by transvaginal  ultrasound ( Figure A). A classic appearance of  hemorrhage is  a spongelike, lacelike, or  reticular pattern. Fibrin strands or  a  retracting clot were found in 90% of hemorrhagic cysts. Color  ow  Doppler  ultrasound  can  show  a  typical  “ring  of   re.”  These complex cysts were shown to resolve in 4- to 12-week  follow-up studies (Figure B, which was obtained 6 weeks after  Figure A).
The  differential  diagnosis  of   a  hemorrhagic  ovarian  cyst  includes  ectopic  pregnancy,  adnexal  torsion,  neoplasm,  pel­vic  inammatory  disease,  endometrioma,  and  degenerating  broid. If the complex ovarian  cyst is not  typical for corpus  luteum or is larger than 3 cm,  a follow-up ultrasound scan is  recommended. This repeat study is planned after 6 to 12 weeks 
so  that  the  cyst  is  reimaged  during  a  different  phase  of   the  menstrual  cycle.  If   the  patient  is  postmenopausal,  a  hemor­rhagic cyst is abnormal, usually an indication for surgery.
Complex Ovarian Cysts in Postmenopausal Women
In postmenopausal women with complex ovarian cysts, 55%  of   cysts  resolved  in  60  days  and  45%  persisted.  Malignant  ovarian tumors were found in this group. Subsequent studies  led to the suggestion that postmenopausal women with simple  unilocular  cysts  less  than  7  cm  in  diameter  can be followed  with ultrasound yearly. However, complex ovarian cysts, even  if   small,  are  associated  with  malignancy  and  should  be  fol­lowed in early menopause or removed if they develop in later  menopause.
Simple Ovarian Cysts in Premenopausal Women
Simple  ovarian  cysts  in  premenopausal  women  are  usually  physiologic follicles or dominant follicles,  typically not larger  than 3 cm in diameter. They do not cause pain unless there is  torsion, which is rare. A large simple cyst may be followed into  the next cycle to check for physiologic change.
Simple Ovarian Cysts in Postmenopausal Women
Ovarian  cysts  are  of   more  concern  in  postmenopausal  women  than  in  premenopausal  women.  It  is  important  to  detect  any  ovarian  neoplasm  occurring  more commonly  in  the older age group as early as possible because with ovarian  malignancies, disease is usually widespread before symptoms  occur. Simple ovarian cysts are common in  postmenopausal  women. These cysts were present in one study in 15% to 17%  of   asymptomatic postmenopausal  patients. In follow-up  of   cysts over 2 years in  the  study, approximately  53%  of  these  simple cysts disappeared, 28% remained stable, 11% enlarged  by 3 mm or more, and 3% decreased by 3 mm or more. The  study recommended that simple adnexal cysts less than 3 cm  (Figure C) that have a normal resistive index less than 0.4 in  patients with a normal cancer antigen 125 (CA 125) level are  probably  benign  and  can  be  safely  followed  by  ultrasound.   Figure  D  is  the  same  postmenopausal  patient  as  in  Figure  C, 3  months later after the cyst has resolved. A more recent  investigation  of  asymptomatic  postmenopausal women that  used 10 cm as maximum cyst diameter also showed that many  of  the ovarian cysts resolved spontaneously. Of patients with  unilocular  cysts,  49% resolved within 60 days and 51%  per­sisted. No cancers were detected in the examined ovaries after  surgery in the patients with persistent cysts.
108

CASE 53

A
D
History: A patient presents for a routine ultrasound scan at
20 weeks’ gestation.
1. What should be included in the differential diagnosis of Figure A? (Choose all that apply.)
A. Ventriculomegaly B. Choroid plexus cysts C. Intracranial hemorrhage D. Semilobar holoprosencephaly E. Dandy-Walker malformation
2. Which of the following is not a true statement concerning choroid plexus cysts?
A. These cysts are most frequently benign ndings. B. These cysts are often transient ndings and resolve by
the third trimester.
C. These cysts may be either unilateral or bilateral.
B
D. These cysts are associated with an increased risk of
bilateral hydrocephalus.
3. Which of the following is not a typical feature of trisomy 18?
A. Fixed position of the fetal ngers B. Abnormally shaped fetal head C. Umbilical cord with two vessels D. A sandal gap between the rst and second toe
4. Which of the following is a rare ultrasound nding in a fetus with trisomy 18?
A. Cardiac defects B. Intrauterine growth restriction C. Cyclopia and proboscis D. Omphalocele
C
109
ANSWERS
CASE 53
Trisomy 18
1. A, B, and C
2. D
3. D
4. C
Reference
Watson WJ, Miller RC, Wax JR, et al: Sonographic ndings of trisomy 18 in
the second trimester of pregnancy. J Ultrasound Med 2008; 27(7):1033-1038; quiz 1039-1040.
http://www.ncbi.nlm.nih.gov/pubmed/18577667 (Accessed on June 12, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 395-397.
Comment
Differential Diagnosis
This case shows well-demarcated hypoechoic structures noted within both lateral ventricles. These ndings are classic for a choroid plexus cyst (CPC). The most common differential diagnosis would be intraventricular hemorrhage, which may result in a blood clot that eventually cavitates and becomes hypoechoic; however, this usually occurs late in the second tri­mester or early in the third trimester. Choroid plexus cysts are usually observed in a screening ultrasound performed at 18 to 20 weeks of gestation. Finally, although unusual, large bilateral choroid plexus cysts could be mistaken for ventriculomegaly. In this case the feature of well-demarcated hypoechoic struc­tures within the echogenic choroid of the lateral ventricles is almost pathognomonic of a choroid plexus cyst.
Ultrasound Findings
The diagnosis of choroid plexus cysts is straightforward. Find­ings include a well-demarcated hypoechoic structure with a well-dened wall within the echogenic choroid of the lateral
ventricle. These structures are more commonly identied in the downside lateral ventricle because there may be poor visu­alization of the upside lateral ventricle owing to the overly­ing skull. The cyst may be unilateral or bilateral, and cysts can be multiple (Figure A). The denition of choroid plexus cyst is usually a cyst greater than 2 mm in diameter in the cho­roid plexus. Often there is some normal heterogeneity of the choroid plexus, which should not in itself be called a choroid plexus cyst.
Prognosis and Management
A choroid plexus cyst, in the absence of an associated anom­aly, should be considered a normal anatomic variant. Parents are often concerned about having “cysts in the brain,” but they should be reassured that these are usually normal and decrease rapidly in size by the third trimester. Only rarely are any remnants of the cyst identied after birth. Although cho­roid plexus cysts are benign, this transient nding is associated with increased risk of trisomy 18. Trisomy 18 is also known as trisomy E or Edwards syndrome. With the detection of a choroid plexus cyst, the patient’s age and biochemical marker values should be reviewed. In addition, a targeted ultrasound examination should be performed to check for potential abnormalities associated with trisomy 18. This genetic syn­drome is associated with numerous ndings, including cho­roid plexus cysts, strawberry-shaped head, small low-set ears, a single umbilical artery, umbilical cord cyst, intrauterine growth restriction, clubbed or rocker-bottom feet (Figure B), and clenched hands (Figure C). Structural abnormalities are often present in fetuses with trisomy 18, including central nervous system ndings such as spinal bida, hydrocephalus, or an abnormal posterior fossa. In one series, there was associated mild increased risk of alobar holoprosencephaly, although this is rare. Heart anomalies also are often detected in fetuses with trisomy 18, including ventricular septal defect and atrioven­tricular canal and complex cardiac malformations (Figure D). Other ndings include omphalocele and genitourinary abnor­malities such as hydronephrosis.
110

CASE 54

A
History: Three asymptomatic patients present in the second
trimester for fetal ultrasound imaging.
1. What should be included in the differential diagnosis for the lemon sign seen in Figure A? (Choose all that apply.)
A. Encephalocele B. Chiari II malformation and myelomeningocele C. Dandy-Walker malformation D. Agenesis of the corpus callosum
2. What entity is most commonly associated with a strawberry-shaped skull?
A. Trisomy 21 B. Trisomy 13 C. Trisomy 18 D. Alobar prosencephaly
3. Which syndrome is associated with a cloverleaf skull (Fig­ure B)?
A. Thanatophoric dwarsm B. Trisomy 13 C. Trisomy 21 D. Encephalocele
4. What is Spalding sign (Figure C)?
A. Kleeblattschädel B. Severe hydrocephalus C. Overlapping skull bones D. Lobar prosencephaly
B
C
111
ANSWERS
CASE 54
Calvarial Abnormalities
1. A, B, C, and D
2. C
3. A
4. C
References
Ball RH, Filly RA, Goldstein RB, et al: The lemon sign: not a specic indica-
tor of meningomyelocele. J Ultrasound Med 1993; 12(3):131-134.
http://www.ncbi.nlm.nih.gov/pubmed/8492374 (Accessed on June 12, 2012.)
Nicolaides KH, Salvesen DR, Snijders RJ, et al: Strawberry-shaped skull in
fetal trisomy 18. Fetal Diagn Ther 1992; 7(2):132-137.
http://www.ncbi.nlm.nih.gov/pubmed/1503648 (Accessed on June 12, 2012.)
Shiroyama Y, Ito H, Yamashita T, et al: The relationship of cloverleaf skull to
hydrocephalus. Childs Nerv Syst 1991; 7(7):382-385.
http://www.ncbi.nlm.nih.gov/pubmed/1794118 (Accessed on June 12, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 404, 406, 477, 479-480.
Comment
General Evaluation of the Fetal Calvaria
Evaluation of the fetal skull begins with a measurement of the biparietal diameter (BPD). If the rst ultrasound examination is performed at 12 weeks of gestation or later, the BPD is used to calculate the mean estimated gestational age. For follow-up examinations, the gestational age is always dictated by the rst study, and BPD, abdominal circumference, and femur length are measured to assess for interval growth. In addition to measure­ment of the BPD, the shape of the fetal skull should be evaluated. Several syndromes have been associated with skull abnormalities.
Lemon Sign
A lemon-shaped head (Figure A) (seen before 24 weeks’ gesta­tional age) is a well-known nding in Chiari II malformation,
which includes myelomeningocele with a small posterior fossa and a banana-shaped cerebellum. However, one series showed that a lemon-shaped head can be seen in fetuses with other central nervous system malformations, including encephalo­cele, Dandy-Walker malformation, and agenesis of the corpus callosum. In addition, a lemon-shaped head was present in a few cases with unrelated anomalies (umbilical vein varix with a two-vessel cord and fetal hydronephrosis). If the deformity is mild, it may be a normal variant.
Strawberry-Shaped Skull
A strawberry-shaped skull can be seen in some cases of tri­somy 18. It is considered to be secondary to hypoplasia of the frontal lobes of the brain. Choroid plexus cysts may also be present. Limb anomalies include rocker-bottom feet, clubbed feet, and overlapping ngers.
Cloverleaf Skull
A cloverleaf skull, or kleeblattschädel (Figure B), can be seen in some cases of thanatophoric dwarsm in association with a narrow, bell-shaped thorax and shortened femurs shaped like telephone receivers. However, as shown in Figure B, it can also be caused by an isolated craniosynostosis. Several rare syndromes may involve a cloverleaf skull deformity, includ­ing atypical Apert syndrome, the syndrome of marfanoid phenotype with craniosynostosis (Shprintzen- Goldberg syndrome), and Pfeiffer syndrome type 2. The shape of the skull can cause both communicating and noncommunicating hydrocephalus.
Spalding Sign
Spalding sign describes overlapping skull bones seen with fetal death (Figure C). The bone collapse results from autolysis.
112

CASE 55

A
Courtesy of Roy Filly, MD.
History: A patient with a family history of congenital heart
disease undergoes imaging.
1. What should be included in the differential diagnosis for Figure A? (Choose all that apply.)
A. Tetralogy of Fallot B. Hypoplastic left heart C. Truncus arteriosus D. Normal three-vessel view E. Ventricular septal defect
2. In Figure B, all of the following structures may be identied
except:
A. Pulmonary artery B. Aorta C. Inferior vena cava D. Descending aorta E. Trachea
3. In examination of the heart using the ve-short-axis view, all of the following views would be included except:
A. Upper abdomen including the fetal stomach B. Traditional four-chamber view C. Long-axis view D. Three-vessel view
4. All of the following abnormalities may be detected on the ve-short-axis view of the heart except:
A. Hypoplastic left heart B. Tetralogy of Fallot C. Ductus arteriosus D. Truncus arteriosus
B
C
113
ANSWERS
CASE 55
Three-Vessel View of Heart
1. A
2. C
3. C
4. C
References
DeVore GR, McGahn JP: Cardiac anatomy and sonographic approach. In
Nyberg DA, McGahan JP, Pretorius DH, et al (eds): Diagnostic Imaging of Fetal Anomalies. Philadelphia: Lippincott Williams & Wilkins, 2003, pp 421-450.
Yagel S, Cohen SM, Achiron R: Examination of the fetal heart by ve
short-axis views: a proposed screening method for comprehensive cardiac
evaluation. Ultrasound Obstet Gynecol 2001; 17(5):367-369.
http://www.ncbi.nlm.nih.gov/pubmed/11380958 (Accessed on June 12, 2012.)
Yoo SJ, Lee YH, Kim ES, et al: Three-vessel view of the fetal upper medias-
tinum: an easy means of detecting abnormalities of the ventricular outow
tracts and great arteries during obstetric screening. Ultrasound Obstet Gynecol
1997; 9(3):173-182.
http://www.ncbi.nlm.nih.gov/pubmed/9165680 (Accessed on June 12, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 419-420.
Comment
Differential Diagnosis
The differential diagnosis in this case would include any abnor­mality in which the aortic arch is enlarged and there is a small pulmonary artery, including abnormalities in which there is obstruction of the outow tract of the right ventricle. Abnor­malities such as pulmonary atresia associated with a ventricular septal defect or pulmonary atresia with an intact ventricular septum would be included in the differential diagnosis. In this
case, there was pulmonary atresia with a ventricular septal defect and overriding aorta as identied in tetralogy of Fallot. Left-sided cardiac malformations would not be considered in the differential diagnosis because there is atresia of the aortic or mitral valve in such cases. There would be a small central aorta in these cases.
Ultrasound Findings
Conotruncal abnormalities may be detected by obtaining a ve-short-axis view of the fetal heart. This group of ve views as described by Yagel includes (1) the traditional coronal plane view showing the fetal stomach on the left; (2) the traditional four-chamber view of the heart; (3) a ve-chamber view of the heart, in which the aortic root is identied centrally; (4) a slightly more cephalad view, in which the pulmonary artery is noted bifurcating into the left and right pulmonary arteries and the aorta is noted more centrally; and (5) the three-vessel view, the most cephalad view, in which the pulmonary artery is to the right side, slightly larger than the more central aorta (Figures A to C). The pulmonary artery is more anterior within the chest in this view. The superior vena cava is to the left side. By fol­lowing these views in real time, the right ventricle is identied giving rise to the pulmonary artery and the left ventricle is iden­tied giving rise to the aorta. Transposition of the great arter­ies should be detected if the ve-short-axis view is obtained in real time. Additionally, other conotruncal abnormalities and abnormalities detected on a traditional four-chamber view can be detected on the ve-short-axis view of the heart.
Prognosis and Management
Prognosis depends on the particular abnormalities. In this case, there is tetralogy of Fallot, which is discussed in Case 39.
114

CASE 56

AA
BB
History: A patient presents for a routine second-trimester
prenatal ultrasound scan.
1. What should be included in the differential diagnosis for reversed diastolic ow of the umbilical artery? (Choose all that apply.)
A. Intrauterine growth restriction (IUGR) B. Fetal asphyxia C. Perinatal mortality D. Permanent fetal neurologic sequelae
2. What is the purpose of measuring the ow in the middle cerebral artery (MCA)?
A. To determine whether a compensatory, brain-sparing
fetal response has occurred as a result of decreased
umbilical artery diastolic ow B. To look for an aneurysm C. To look for an infarct D. To look for a stenotic vessel
3. What is the normal change in umbilical artery Doppler as
gestational age increases? A. The resistance increases. B. The systolic velocity increases. C. The resistance decreases. D. A tardus parvus waveform develops after the rst
trimester.
4. How is umbilical artery impedance measured? A. Ratio of peak systolic velocity divided by end-diastolic
velocity
B. Ratio of end-diastolic velocity divided by peak systolic
velocity
C. Ratio of midsystolic velocity divided by end-diastolic
velocity
D. The highest systolic peak velocity
CC
115