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ANSWERS
CASE 27
Pulmonary Sequestration
1. A, B, C, and D
2. D
pleural effusions are also known complications. Differential diagnoses include an intrathoracic mass such as congenital cys­tic adenomatoid malformation and congenital diaphragmatic hernia.
3. D
4. A
References
Dhingsa R, Coakley FV, Albanese CT, et al: Prenatal sonography and MR
imaging of pulmonary sequestration. AJR Am J Roentgenol 2003; 180(2). 443-437.
http://www.ncbi.nlm.nih.gov/pubmed/12540448 (Accessed on June 5,
2012.)
Hernanz-Schulman M, Stein SM, Neblett WW, et al: Pulmonary sequestra-
tion: diagnosis with color Doppler sonography and new theory of associ-
ated hydrothorax. Radiology 1991; 180(3):817-821.
http://www.ncbi.nlm.nih.gov/pubmed/1871300 (Accessed on June 5, 2012.)
Felker RE, Tonkin IL: Imaging of pulmonary sequestration. AJR Am J Roent-
genol 1990; 154(2):241-249.
http://www.ncbi.nlm.nih.gov/pubmed/2105007 (Accessed on June 5, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 426.
Comment
Differential Diagnosis
Pulmonary sequestration is diagnosed when a portion of the lung receives systemic rather than pulmonary arterial supply and is separated from the tracheobronchial tree. There are two subtypes:
1. Intralobar—sequestration is contained within normal
pleura. Because this type of sequestration is normally found in infants older than 2 months, some authors believe that it is an acquired entity. It is associated with brosis and chronic inammation.
2. Extralobar—sequestration is contained within its own
pleura. Usually this condition is diagnosed prenatally. Infec­tion and congestive heart failure are the most common associations with this disorder.
Sequestration should be included in the differential diagno­sis of any infant with congestive heart failure and suspected pneumonia or atelectasis. Lobar emphysema, hemothorax, and
Ultrasound Findings
Prenatal ultrasound shows a supradiaphragmatic or infradia­phragmatic hyperechoic mass, usually on the left side (Figures A and B). Mediastinal and cardiac shift results if the sequestra­tion is large. In some cases, as the fetus grows, the sequestration becomes relatively smaller. The diagnosis should be suspected in any fetus with a lung mass, especially when hydrothorax is present. Blood ow may be evident on color Doppler imaging, and in some cases the systemic arterial supply may be visible from the aorta, which is the usual supply (Figures A and B). MRI may be useful to dene better the location of the mass, any associated pleural effusions, and systemic arterial supply (Figure C).
Extralobar sequestrations may be diagnosed prenatally between the lower lobe and the hemidiaphragm, surround­ing the diaphragm or lung in the pleural or pericardial space, and in the retroperitoneum. Most occur on the left side. More recently, there have been cases of pulmonary sequestration with elements of cystic adenomatoid malformation or congen­ital pulmonary airway malformation. These have been called hybrid lesions.
Prognosis and Management
Perinatal mortality of a fetus with an extralobar sequestration is rare. These cases have an excellent prognosis, frequently with spontaneous regression. However, tension hydrothorax can develop secondarily from uid secreted by the mass. This condition can cause vena caval obstruction or cardiac com­pression leading to hydrops fetalis. Postnatal surgery is usually necessary for treatment of sequestration.
Acknowledgment
Special thanks are extended to Jonathan Kuo, MD, for his con­tribution to preparing this case.
56

CASE 28

A
D
History: A 30-year-old woman with a previously inserted
T-shaped intrauterine contraceptive device (IUCD) presents with heavy menstrual ow.
1. What is the most likely cause of the patient’s heavy men­strual ow?
A. Endometrial cancer B. T-shaped IUCD embedded in the uterine wall C. Endometritis D. Submucosal broid
2. What is the role of ultrasound in the evaluation of an IUCD?
A. To evaluate the endometrial cavity for the correct size
IUCD
B
B. To evaluate the length of the cervix for the correct size
IUCD C. To evaluate for the presence and location of an IUCD D. To measure the endometrial lining
3. If the string of an IUCD is not identied on physical exami-
nation, which of the following is not a serious consideration?
A. The string has just pulled back into the cervical or uter-
ine canal. B. The IUCD has been expelled with the string. C. The IUCD has perforated the wall, and the string
has been pulled up into the uterine canal or into the
myometrium. D. The string fell off the IUCD.
4. What is the most common complication of an IUCD? A. Infection B. Pregnancy C. Hemorrhage
C
D. Urinary frequency
57
ANSWERS
CASE 28
Intrauterine Contraceptive Device
1. B
2. C
3. D
4. A
References
Boortz HE, Margolis DJA, Ragavendra M, et al: Migration of intrauterine
devices: radiologic ndings and implications for patient care. Radiograph­ics 2012; 32:335-352.
http://www.ncbi.nlm.nih.gov/pubmed/22411936 (Accessed on June 27,
2012.)
Grimes DA, Jones KP, Knutson CC, et al: New developments in intrauterine
contraception. Association of Reproductive Health Professionals ARHP Clinical Proceedings September 2004, pp 1-20.
Shipp TD, Bromley B, Benacerraf BR: The width of the uterine cavity is nar-
rower in patients with an embedded intrauterine device (IUD) compared to a normally positioned IUD. J Ultrasound Med 2010; 29(10):1453-1456.
http://www.ncbi.nlm.nih.gov/pubmed/20876899 (Accessed on June 5,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 544-549.
Comment
General Background of Intrauterine Contraceptive Devices
IUCDs have been used since the early 1900s to prevent preg­nancy (Figures A to D). Since then, the devices have been steadily rened, and today many types are available. The two most common types of IUCD in the United States are the ParaGard IUCD and the Mirena IUCD. Visualization of the newer Mirena IUCD is most challenging. Three-dimensional ultrasound imaging is most helpful. IUCDs are better suited for older women who have already been pregnant. Younger users of IUCDs experience higher pregnancy rates and more expulsions, and they have the devices removed more fre­quently for medical reasons. Contraindications to use of an IUCD include pregnancy, a history of pelvic inammatory disease, undiagnosed vaginal bleeding, uterine anomalies, and large broid tumors. IUCDs have undergone a resurgence in the United States because they are being used for contracep­tion and the treatment of menorrhagia.
combination of both. A Copper 7 IUCD has a long arm of wrapped copper and a small arm of plastic in transaxial view in the upper body. The metal wrapping exhibits a “reverberation artifact”—a series of parallel lines that become weaker from the IUCD posteriorly when the IUCD is parallel to the ultra­sound beam. The plastic tubing appears as two parallel lines, entrance and exit echos.
Ultrasound Imaging of Intrauterine Contraceptive Device Position
Ultrasound is very accurate in detecting an IUCD when it is correctly positioned. When the uterus is normal and there is no distortion of the endometrial canal, the IUCD has a mid­line position. Fibroids may change the position of the uterine cavity so that the IUCD appears to be partially perforating the uterine wall. Perforations have been more common when the cavity is thinner. One of the most common clinical uses of ultrasound in the evaluation of IUCDs is to determine whether the IUCD is still present within the uterus when the string is no longer present on pelvic examination. Because of the potential for expulsion and for perforation (Figure A), fail­ure to detect a string within the vagina or seeing too much string (Figure B) often leads to an ultrasound examination. When clinical symptoms make perforation likely, a negative uterine study does not entirely rule out perforation, and a plain anteroposterior radiograph of the pelvis may be obtained to detect the perforated radiopaque IUCD.
Complications of Intrauterine Contraceptive Devices
IUCDs are associated with complications. Infections are the most common complications (Figures C and D); unexpected pregnancies also occur. Although infections typically are bac­terial, they may be fungal, such as actinomycosis. Expulsion may also occur. If a pregnancy occurs after implantation of an IUCD, ultrasound is indicated to detect whether it is still present. The IUCD does not create a risk to the pregnancy because it is in the endometrial cavity and is not within the chorionic sac. However, if on physical examination the string is still projecting into the vagina, the cervical plug will not form, and there is a potential for an ascending infection. The IUCD should be removed, often under ultrasound guidance.
Ultrasound Imaging Characteristics of Intrauterine Contraceptive Devices
IUCDs have many different appearances on ultrasound. They are composed of thin plastic tubing, metal wrapping, or a
58

CASE 29

History: A patient has a routine second-trimester ultra­sound scan with a question of a cystic structure in the fetal abdomen.
1. What are potential causes of upper abdominal cystic struc-
tures in the fetus? (Choose all that apply.) A. Enlarged fetal stomach B. Enlarged duodenum C. Gastric duplication cyst D. Esophageal duplication cyst E. Pericardial cyst
2. What is a potential cause for not identifying the fetal
stomach? A. Esophageal atresia B. Duodenal atresia C. Jejunal atresia D. Ileal atresia
3. If an echogenic structure is identied within the lumen, what could this indicate?
A. Pseudomass B. Choledochal cyst C. Duodenal atresia D. Annular pancreas
4. At what gestational age can the fetal stomach rst be identied?
A. 9 weeks B. 15 weeks C. 19 weeks D. 21 weeks
59
ANSWERS
CASE 29
Enlarged Fetal Stomach
1. A, B, and C
2. A
3. A
4. A
References
Bonin B, Gruslin A, Simpson NA, et al: Second trimester prenatal diag-
nosis of congenital gastric outlet obstruction. J Ultrasound Med 1998;
17(6):403-406.
http://www.ncbi.nlm.nih.gov/pubmed/9623481 (Accessed on June 5, 2012.)
Wax JR, Hamilton T, Cartin A, et al: Congenital jejunal and ileal atresia: natu-
ral prenatal sonographic history and association with neonatal outcome.
J Ultrasound Med 2006; 25(3):337-342.
http://www.ncbi.nlm.nih.gov/pubmed/16495494 (Accessed on June 5,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 433-440.
Comment
Differential Diagnosis
The differential diagnosis of an anechoic cystic structure in the upper abdomen includes a normal or enlarged fetal stomach, gastric duplication cyst, choledochal cyst, and ovarian cyst. An enlarged stomach is seen with duodenal atresia or any potential obstruction of the duodenum such as Ladd bands or annular pancreas. The fetal stomach can be identied on ultrasound at 9 weeks’ gestation. The characteristic morphology is shown at 14 weeks.
Ultrasound Findings
The stomach is usually identied in the left upper quadrant as a uid-lled anechoic structure. If the position of the stomach is difcult to determine or obviously on the right, a search for
other anatomic anomalies should be undertaken, specically, cardiac anomalies because of the high association with cardiac disorders. If the stomach is midline, this may be associated with heterotaxia syndrome and major cardiac malformation. If the stomach appears to be residing in the chest, this likely represents a congenital diaphragmatic hernia.
Absence of the fetal stomach may be secondary to a multi­tude of causes, including but not limited to oligohydramnios, problems with swallowing (neurogenic), or tracheoesophageal stula. Most commonly with a tracheoesophageal stula, there is a distal communication between the trachea and the distal esophagus that connects to the stomach and as such a small stomach may be identied with this entity. If no stomach is identied and esophageal atresia is suspected, a search for other abnormalities or other syndromes should be undertaken. Finally, the fetal stomach may be displaced by an enlarged abdominal mass, and in such cases the mass is the primary abnormality and not absence of the fetal stomach.
Prognosis and Management
Enlargement of the fetal stomach (see Figure) has been asso­ciated with duodenal atresia when occurring with an enlarged duodenum; however, it was also shown to be present in 5 of 15 cases of jejunal or jejunoileal atresia in a single-center, 10-year retrospective review. There have been a few case reports of enlarged fetal stomach manifesting as the only abnormality on fetal ultrasound associated with a postnatal diagnosis of pyloric stenosis. If duodenal atresia is associated with a chromosomal abnormality, such as trisomy 21 (which has the highest asso­ciation), a comprehensive fetal ultrasound survey should be undertaken and genetic karyotyping should be considered.
Acknowledgment
Special thanks are extended to Vishal Sidhar, MD, and Simran Sekhon, MD, for their contribution to preparing this case.
60
History: An asymptomatic woman with a second-trimester
pregnancy undergoes ultrasound.
1. What is the most likely diagnosis of the images of the right foot?
A. Osteogenesis imperfecta B. Clubfoot C. Rocker-bottom foot D. Pes planus
2. What is an extrinsic cause of this abnormal ankle-foot relationship?
A. Amniotic sheet B. Polyhydramnios C. Oligohydramnios D. Cytomegalovirus infection
A
B
3. What percentage of cases are bilateral?
A. Slightly more than 50% B. Slightly more than 10% C. Slightly more than 85% D. Less than 5%
4. What is the most common cause of the abnormal relation­ship of the foot to the lower leg?
A. Trisomy 18 B. Trisomy 13 C. Idiopathic D. Pierre Robin syndrome
ANSWERS
1. B
2. C
3. A
4. C
References
Hashimoto BE, Filly RA, Callen PW: Sonographic diagnosis of clubfoot in
utero. J Ultrasound Med 5 1986; 2:81-83.
http://www.ncbi.nlm.nih.gov/pubmed/3514941 (Accessed on June 5, 2012.)
Mammen L, Benson C: Outcome of fetuses with clubfeet diagnosed by
prenatal sonography. J Ultrasound Med 2004; 23(4):497-500.
http://www.ncbi.nlm.nih.gov/pubmed/15098867 (Accessed on June 5,
2012.)
Shipp TD, Benacerraf BR: The signicance of prenatally identied
isolated clubfoot: is amniocentesis indicated? Am J Obstet Gynecol 1998;
178(3):600-602.
http://www.ncbi.nlm.nih.gov/pubmed/9539532 (Accessed on June 5, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 480-482.
Comment
Incidence of Clubfoot
Clubfoot is a common congenital anomaly that occurs bilater­ally in slightly more than 50% of cases. Many cases are familial, and the risk is 25% for the fetus if the parent has a clubfoot. Although the most common cause is idiopathic, possible asso­ciated abnormalities warrant a careful search with prenatal ultrasound when clubfoot is detected.
fails to detect other malformations, the incidence of a karyo­type abnormality is 6%. The rates for aneuploidy have been found to be similar whether clubfoot is unilateral or bilateral.
Clubfoot and Other Associated Anomalies
In addition to chromosomal anomalies, clubfoot deformity is associated with other malformations in 10% of cases. Cleft lip and palate, micrognathia, facial deformities, congenital heart disease, and hip dislocations are among the associated anoma­lies. Neurologic abnormalities associated with clubfoot include meningomyelocele and hydrocephalus. Numerous congenital syndromes and musculoskeletal disorders include clubfoot in their spectrum of anomalies: Gordon syndrome (camptodac­tyly and cleft palate), distal arthrogryposis (xated hands and feet), nail-patella syndrome, muscular dystrophies, and Pierre Robin syndrome (congenital heart disease). Musculoskeletal anomalies besides clubbed feet, neural tube defects, and car­diovascular anomalies have been found to be more common in fetuses with bilateral clubbed feet than in fetuses with unilateral deformity. Extrinsic causes can also lead to clubfoot deformity, including oligohydramnios and amniotic band syndrome.
Ultrasound Appearance of Clubfoot
The ultrasound appearance relies on an unusual conguration between the foot and the lower leg (Figures A and B). Once this is seen, the other extremities should be carefully evaluated for bilaterality and clubhands. The more the limbs are affected, the greater is the possibility of the presence of a congenital syndrome or a musculoskeletal disorder.
Clubfoot and Chromosomal Anomalies
Clubfoot is associated with chromosomal anomalies such as trisomy 13 and trisomy 18. Even if the prenatal ultrasound scan

CASE 31

D
A
fetus undergoes imaging (Figures A and B).
1. What should be included in the differential diagnosis of the abdominal anterior cysts in the two presented fetuses? (Choose all that apply.)
A. Mesenteric cyst B. Ovarian cyst C. Omental cyst D. Enteric duplication cyst
2. How is a meconium pseudocyst formed?
A. From walled-off complicated ascites B. From walled-off simple ascites C. From a ruptured mesenteric cyst D. From a ruptured duplication cyst
3. Which of the following is not a genitourinary cyst seen in a fetus?
History: An asymptomatic patient with a third-trimester
B
A. Urachal cyst B. Bladder outlet obstruction C. Urinoma D. Medullary sponge kidney
4. What fetal abdominopelvic masses typically have both cys­tic and solid components?
A. Ovarian cyst B. Teratoma C. Multicystic dysplastic kidneys D. Obstruction of the vagina
C
63
ANSWERS
CASE 31
Cystic Abdominal Mass
1. A, B, C, and D
2. A
3. D
4. B
References
Bryant AE, Laufer MR: Fetal ovarian cysts: incidence, diagnosis and manage-
ment. J Reprod Med 2004; 49(5):329-337.
http://www.ncbi.nlm.nih.gov/pubmed/15214704 (Accessed on June 8,
2012.)
Foster MA, Nyberg DA, Mahoney BS, et al: Meconium peritonitis: prenatal
sonographic ndings and their clinical signicance. Radiology 1987;
165(3):661-665.
http://www.ncbi.nlm.nih.gov/pubmed/3317498 (Accessed on June 8, 2012.)
Richards DS, Langham MR, Anderson CD: The prenatal sonographic
appearance of enteric duplication cysts. Ultrasound Obstet Gynecol 1996;
7(1):17-20.
http://www.ncbi.nlm.nih.gov/pubmed/8932626 (Accessed on June 8, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 447, 464.
Comment
Differential Diagnosis of Cystic Abdominal or Pelvic Mass
The list of differential diagnoses for a cystic abdominal or pel­vic mass in a fetus is long. Identication of associated ndings may aid in determining the precise etiology. In the two cases presented, the cystic masses do not extend back to the spine and are not related to the kidney, so renal and retroperitoneal masses do not need to be considered.
greater numbers of ovarian cysts are being diagnosed. The risks of acute and long-term complications must be weighed against the possibility of cyst regression. Cyst size is important. Simple cysts (Figures A to D) and teratomas can arise from the ovary. Obstruction of the vagina or uterus may appear as a cystic mass in the pelvic midline.
Meconium Pseudocysts and Bowel-Related Cysts
A meconium pseudocyst forms from walled-off complicated ascites secondary to meconium peritonitis. The cyst typically contains internal echoes. Dilation of the small bowel, peri­toneal calcications, and polyhydramnios can be seen. The obstructed bowel loop in bowel atresia can also manifest as an abdominal cystic mass, usually with hyperperistalsis. A simple cyst may represent a mesenteric or omental cyst; additionally, enteric duplication cysts are located in the mesentery. Enteric duplication cysts are directly contiguous with the bowel and may communicate with the bowel lumen. Approximately 30% of fetuses with enteric duplication cysts have associated anom­alies. Spinal or gastrointestinal malformations may be present with foregut or hindgut duplications.
Genitourinary Cysts
Genitourinary cysts can arise from several sites. Renal causes such as multicystic dysplastic kidney have been eliminated in this case. However, posterior urethral valve obstruction can result in urinoma formation. An obstructed bladder in poste­rior urethral valve and other types of bladder outlet obstruction appears as a large cystic abdominopelvic mass. The existence of a urachal cyst is an additional possibility.
Ovarian Cysts and Pathology of the Uterus and Vagina
Ovarian cysts are among the most common cystic abdominal masses in female patients. With improved neonatal imaging,
64

CASE 32

A
B
History: A woman with a rst-trimester pregnancy presents
with pelvic pain.
1. What should be included in the differential diagnosis for the ultrasound image shown in Figure A? (Choose all that apply.)
A. Early intrauterine pregnancy B. Tubal ectopic pregnancy C. Cornual ectopic pregnancy
2. What is the intradecidual sign?
A. Intrauterine pseudogestational sac B. Corpus luteum C. Early intrauterine sac located adjacent to the endome-
trium and embedded in the decidua
3. What is double decidual reaction? A. Two concentric hyperechoic rings that surround the
early intrauterine gestational sac B. Twin gestational sacs C. Corpus luteum D. Decidual cysts
4. What is the nding in Figure B? A. Cervical ectopic pregnancy B. Ruptured tubal ectopic pregnancy C. Ectopic pregnancy in a cesarean section scar
65