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ANSWERS
CASE 95
Congenital High Airway Obstruction
1. C
2. B
3. C
4. D
References
Courtier J, Poder L, Wang ZJ, et al: Fetal tracheolaryngeal airway obstruc-
tion: prenatal evaluation by sonography and MRI. Pediatr Radiol 2010;
40(11):1800-1805.
http://www.ncbi.nlm.nih.gov/pubmed/20737145 (Accessed on June 21, 2012.)
Dighe MK, Peterson SE, Dubinsky TJ, et al: EXIT procedure: technique and
indications with prenatal imaging parameters for assessment of airway
patency. Radiographics 2011; 31(2):511-526.
http://www.ncbi.nlm.nih.gov/pubmed/21415194 (Accessed on June 21, 2012.)
Kohl T, Hering R, Bauriedel G, et al: Fetoscopic and ultrasound-guided
decompression of the fetal trachea in a human fetus with Fraser syndrome
and congenital high airway obstruction syndrome (CHAOS) from laryn-
geal atresia. Ultrasound Obstet Gynecol 2006; 27(1):84-88; discussion 88.
http://www.ncbi.nlm.nih.gov/pubmed/16308883 (Accessed on June 21, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 427.
Comment
Differential Diagnosis
This case is nearly an “Aunt Minnie.” A few entities should
be considered in the differential diagnosis, including a bilateral
lung mass, which is very rare. A bilateral lung mass could be
a microcystic congenital cystic adenomatoid malformation or
bilateral pulmonary sequestration. However, a bilateral process
would be rare in both of these entities. The most likely diagnosis is CHAOS. Bronchial atresia is a similar entity, but it is a
unilateral process.
Ultrasound Findings
Because the fetal upper airways are occluded, the intrathoracic pressure is elevated from the lung uid. This increased
pressure impairs cardiac lling, leading to cardiac failure and
hydrops. Hydrops developing with CHAOS is associated with
a poor prognosis. CHAOS was previously termed laryngeal atre-
sia or tracheal atresia. On ultrasound, it is difcult to identify
whether this is laryngeal or tracheal atresia, and so the term high
airway obstruction is now used. The features are nearly pathognomonic (Figures A and B). MRI may be helpful to dene
this entity better; MRI shows a dilated uid-lled trachea and
bilateral uid-lled bronchi, which are also dilated (Figure C).
In CHAOS, there is usually compression of the heart, which
is well identied in this case (Figure A). The process is symmetric, and the heart is not displaced to the right or left. In
addition, uid is present within the tiny air spaces, which are
too small to be identied by ultrasound and appear as bilateral
increased echogenicity of the lung; this has a mass effect with
displacement of the diaphragm inferiorly, which appears attened or inverted (Figure B). Ascites may develop as in this
case and fetal hydrops.
Treatment
The EXIT procedure is used to secure the fetal airway before
compete delivery of the fetus. The EXIT procedure requires
adequate exposure of the uterus with anesthesia for both the
mother and the fetus. The fetal head and neck are exposed;
for fetuses with CHAOS, a tracheostomy is usually needed.
Tracheostomy is not always needed for other EXIT procedures. The most common indication for the EXIT procedure
is to secure an airway before delivery of a fetus with CHAOS
or a fetus with a large neck mass. In the latter case, the neck
mass may be resected after successful maintenance of the
airway.
More recently, in utero fetoscopic and ultrasound-guided
decompression of the fetal trachea in CHAOS has been
reported. Laryngeal atresia has been treated with a fetoscope
and fetal laryngoscopy with balloon dilatation and subsequent
stent implantation within the atretic upper airway.
196
CASE 96
Arrow = cord insertion; C = cer vix; P = placenta.
History: An asymptomatic patient near term presents for a
check of fetal presentation.
1. What should be included in the differential diagnosis for the
lower uterine segment of a pregnancy near term? (Choose
all that apply.)
A. Umbilical cord presenting before the fetus at the time
of birth
B. Cord compression
C. Complete placenta previa
D. Marginal placenta previa
2. What is the most common risk factor for the pathology at
the lower uterine segment?
A. Large-for-dates fetus
B. Fetal breech position
C. Multiple gestations
D. Polyhydramnios
3. What is the most common clinical complication?
A. Fetal heart decelerations during uterine contractions
B. Fetal tachycardia
C. Decreased fetal movement
D. Increased fetal movement
4. What fetal monitoring is performed when this diagnosis is
made?
A. Stress tests and follow-up ultrasound imaging
B. Nonstress tests and follow-up ultrasound imaging
C. Ultrasound imaging alone
D. Amniocentesis
197
ANSWERS
CASE 96
Obligate Cord
1. A and B
2. B
3. A
4. B
References
Pelosi MA: Antepartum ultrasonic diagnosis of cord presentation. Am J
Obstet Gynecol 1990; 162(2):599-601.
http://www.ncbi.nlm.nih.gov/pubmed/2178435 (Accessed on June 21, 2012.)
Sakamoto H, Takagi K, Masaoka N, et al: Clinical application of the perineal
scan: prepartum screening for cord presentation. Am J Obstet Gynecol 1986;
155(5):1041-1043.
http://www.ncbi.nlm.nih.gov/pubmed/3535515 (Accessed on June 21, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 489-490.
Comment
General Description
Obligate presentation of the cord refers to presentation of the
umbilical cord before the fetus at the time of birth.
Risk Factors
Obligate cord is most common in the setting of a fetal breech
position (particularly a footling breech) and with small fetuses,
including fetuses delivered prematurely. Other risk factors
include multiple gestations, polyhydramnios, multiparity, disproportion, incompetent cervix, and “hourglass membranes.”
Diagnosis of this condition is essential because prolapse of
the cord into the cervix during delivery can be catastrophic
for the fetus. In addition, the obligate positioning of the cord
results in cord compression and variable fetal cardiac decelerations or bradycardia during uterine contractions.
Diagnosis Including Ultrasound Imaging
The diagnosis can be made at the time of delivery by palpation; in some cases, the umbilical cord can be palpated in the
lower uterine segment on pelvic digital examination. Prenatal
diagnosis can be made with ultrasound. The umbilical cord can
be identied overlying the internal os (Figure). Transperineal
scanning is helpful in imaging the lower uterine segment and
determining the presenting part.
Management
If an obligate cord is diagnosed prenatally, nonstress fetal
cardiac monitoring is performed at weekly intervals or more
frequently if clinically warranted. Application of fundal and
suprapubic pressure may provoke fetal bradycardia, indicating
cord entrapment. If the cord remains in a primary presenting position, prompt delivery by cesarean section is required
at term.
198
CASE 97
History: A 28-year-old woman undergoes routine prenatal
ultrasound.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Hydronephrosis
B. Renal cyst
C. Multicystic dysplastic kidneys
D. Ectopic ureterocele
E. Infantile polycystic kidney disease
2. Concerning ectopic ureterocele, the ureter to the upper
pole moiety inserts in what relationship to the lower pole
moiety?
A. Lower medial
B. Lower lateral
C. Higher medial
D. Higher lateral
A
B
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; 1326.
3. What is the least common location for insertion of the
ectopic ureter in a female patient among these choices?
A. Urethra
B. Vagina
C. Portion of the bladder
D. Rectum
4. Concerning the ultrasound ndings of ectopic ureterocele,
which of the following statements is false?
A. Ureterocele associated with duplex kidneys is more
common in male patients.
B. Ureterocele may be associated with a solitary renal
pelvis.
C. Usually a normal amount of amniotic uid is present
with ectopic ureterocele.
D. A ureterocele can be mistaken for the bladder.
C
199
ANSWERS
CASE 97
Ectopic Ureterocele
1. A, B, and D
2. A
3. D
4. A
References
Adorisio O, Elia A, Landi L, et al: Effectiveness of primary endoscopic
incision in treatment of ectopic ureterocele associated with duplex system.
Urology 2011; 77(1):191-194.
http://www.ncbi.nlm.nih.gov/pubmed?term=21168903 (Accessed on June
21, 2012.)
Gloor JM, Ogburn P, Matsumoto J: Prenatally diagnosed ureterocele present-
ing as fetal bladder outlet obstruction. J Perinatol 1996; 16(4):285-287.
Sozubir S, Lorenzo AJ, Twickler DM, et al: Prenatal diagnosis of a prolapsed
ureterocele with magnetic resonance imaging. Urology 2003; 62(1):144.
http://www.ncbi.nlm.nih.gov/pubmed?term=12837449 (Accessed on June
21, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 466-467.
Comment
Differential Diagnosis
The differential diagnosis in this case would include any
obstruction of the urinary tract with a dilated ureter. Vesicoureter junction obstruction and vesicoureteric reux would
be considered. Other cystic abnormalities of the kidneys
including a simple cyst could be considered but are less likely.
In this case with a dilated upper pole of the kidney in association with a ureterocele in the bladder, the most likely diagnosis
would be ectopic ureterocele (Figures A to C).
Ultrasound Findings
Ultrasound ndings are fairly classic and include a dilated
upper pole moiety (Figure A) and dilated ureter with a ureterocele within the bladder (Figure B). However, if the ureter
is dilated and inserts below the bladder neck and if no ureterocele is present, a diagnosis of ectopic ureterocele is more difcult. In rare cases, ureteroceles may insert into the region of
the urethra and cause bladder outlet obstruction. Visualization
of the ureterocele within the bladder may be difcult if the
bladder is empty. The upper pole moiety usually inserts lower
and medial to the lower pole moiety; this has been called the
“Weigert-Meyer rule.”
Prognosis and Management
The prognosis of ectopic ureterocele is usually good because
in most cases only one kidney is affected. The amount of
amniotic uid is usually normal, leading to normal development of the other kidney. There are rare associated abnormalities with a normal contralateral kidney. In utero therapy is not
indicated. Postnatal therapy includes cystoscopy with puncture
of the ureterocele or ureteral reimplantation. The condition is
sporadic, and risk of recurrence is low.
200
CASE 98
A
History: In two different patients, similar ndings were
identied on second-trimester ultrasound scans.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Ectopia cordis
B. Pentalogy of Cantrell
C. Amniotic band syndrome
D. Limb-body wall complex
E. Omphalocele
2. Which of the following abnormalities is not included in
pentalogy of Cantrell?
A. Congenital cardiac defects
B. Infraumbilical omphalocele
C. External cleft of the sternum
D. Diaphragmatic defect
B
3. Which of the following abnormalities is not most frequently
associated with ectopia cordis?
A. Omphalocele
B. Cardiovascular malformations
C. Craniofacial defects
D. Amputational defects of the limbs
4. Which of the following statements concerning the prognosis and management of ectopia cordis is false?
A. A cleft sternum alone has an excellent prognosis.
B. True ectopia cordis has a high mortality rate.
C. Cardiac defects are the primary determinant of survival
in infants with ectopia cordis.
D. Chromosomal abnormalities with ectopia cordis and
pentalogy of Cantrell are nearly nonexistent.
201
ANSWERS
CASE 98
Ectopia Cordis
1. A, B, C, and D
2. B
3. D
4. D
References
Hannoun A, Usta IM, Sawaya F, et al: First trimester sonographic diagnosis
of ectopia cordis: a case report and review of the literature. J Matern Fetal
Neonatal Med 2011; 24(6):867-869.
http://www.ncbi.nlm.nih.gov/pubmed?term=21534855 (Accessed on June
21, 2012.)
Twomey EL, Moore AM, Ein S, et al: Prenatal ultrasonography and neonatal
imaging of complete cleft sternum: a case report. Ultrasound Obstet Gynecol
2005; 25(6):599-601.
http://www.ncbi.nlm.nih.gov/pubmed?term=15810036 (Accessed on June
21, 2012.)
Zidere V, Allan LD: Changing ndings in pentalogy of Cantrell in fetal life.
Ultrasound Obstet Gynecol 2008; 32(6):835-837.
http://www.ncbi.nlm.nih.gov/pubmed?term=18951546 (Accessed on June
21, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 446-447.
Comment
Differential Diagnosis
The differential diagnosis in this case would include anything
that causes a defect in the sternum. Cleft sternum may be
present without ectopia cordis; this situation causes some bulging of the cardiac apex on ultrasound. True ectopia cordis may
or may not include pentalogy of Cantrell with its associated
abnormalities. Also, amniotic band syndrome and limb-body
wall complex may produce bizarre defects that could involve
the anterior chest, but these appear much more unusual and
complex than the cases presented in Figures A and B.
Ultrasound Findings
Ultrasound ndings of ectopia cordis are straightforward.
Ectopia cordis is dened as partial or complete displacement
of the heart outside of the thorax. An extrathoracic heart is a
dramatic nding on ultrasound. In an isolated cleft sternum,
the heart may appear to protrude through the chest, although
the chest wall is intact. Pentalogy of Cantrell is more severe,
involving not only ectopia cordis but also a supraumbilical ventral wall defect, intrinsic cardiac abnormalities, a cleft sternum,
and diaphragmatic defects.
Prognosis and Management
Prognosis for ectopia cordis is poor, and the mortality rate is
very high. The surgical approach is tailored to the severity of
defects and other complicating factors, such as the intrinsic
cardiac abnormalities or associated omphalocele.
202
CASE 99
A
C
L = liver.
B
U = uterus.
History: A woman treated with gonadotropins for infertility
presents with pelvic discomfort (Figures A to D).
1. What should be included in the differential diagnosis of
the transabdominal axial images of the pelvis and the sagittal image of the right upper quadrant? What organs are
denoted by the arrows in Figure A? (Choose all that apply.)
A. Ovarian hyperstimulation syndrome (OHSS) caused by
assisted reproduction with the patient pregnant. Hyperstimulated ovaries are seen.
B. OHSS caused by ovulation induction or assisted repro-
duction. Bilateral cystadenomas of the ovaries are seen.
C. OHSS caused by ovarian cystadenomas.
D
2. What patients are at increased risk?
A. Women older than 40 years
B. Women with polycystic ovary syndrome
C. Multiparous women
3. Which of the following is not a sign or symptom of OHSS?
A. Ovarian enlargement
B. Ascites
C. Anemia
4. What is the pathophysiology of this disorder?
A. Decreased capillary permeability
B. Increased capillary permeability
C. Decreased creatinine clearance
203
ANSWERS
CASE 99
Ovarian Hyperstimulation Syndrome
1. A and B
2. B
3. C
4. B
Reference
Berendonk CC, Van Dop PA, Braat DD, et al: Ovarian hyperstimulation
syndrome: facts and fallacies. Obstet Gynecol Surv 1998; 53(7):439-449.
http://www.ncbi.nlm.nih.gov/pubmed/9662730 (Accessed on June 21, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 561-563.
Comment
Etiology
OHSS occurs in women undergoing ovulation induction or
hyperstimulation for assisted reproduction. Increased capillary
permeability secondary to the large ovarian cysts leads to third
spacing, which can result in hypovolemic shock and stroke and
electrolyte abnormalities. The diagnosis is made by measuring serum estradiol levels in a patient with enlargement of the
ovaries on ultrasound.
Risk Factors
A previous history of OHSS is a risk factor. Young, thin
women are at increased risk. In addition, women with the
“necklace” sign of multiple peripheral follicles in the ovary
before assisted reproduction or polycystic ovary disease are
at increased risk. As seen in the case presented (Figures A
to D), pregnancy is not a prerequisite for OHSS; however,
hyperstimulation is more common in women who become
pregnant. Luteinization is an integral contributor to the development of OHSS. In particular, the administration of human
chorionic gonadotropin increases the risk. Women who
become pregnant are at higher risk for the more severe forms
of OHSS.
Ultrasound Findings
Ultrasound ndings include enlarged ovaries (Figure A) and
ascites (Figures A to C). Unilateral pleural effusion has been
described but is rarely an isolated nding.
Complications and Treatment
Complications in addition to hypovolemic shock (and rarely
death) include thromboembolic disease and stroke (Figure D),
liver and kidney dysfunction, and acute respiratory distress
syndrome. The enlarged ovaries are susceptible to torsion.
Treatment is supportive to maintain hemodynamic stability.
Paracentesis guided by transabdominal or transvaginal ultrasound has been shown to be an effective treatment. Mild
forms of OHSS can be managed on an outpatient basis; however, patients with severe OHSS require hospitalization and
monitoring in the intensive care unit.
204
CASE 100
A
C
B
History: A 16-year-old girl presents with fever; a tender,
painful uterus; and pelvic pain.
1. What should be included in the differential diagnosis for
a premenopausal patient presenting with a tender, painful
uterus and fever, with the pelvic ultrasound images shown
in Figures A to C? (Choose all that apply.)
A. Menses
B. Endometritis
C. Endometrial cancer
D. Retained products of conception
2. Which of the following is not a salient nding in the patient
shown in Figure D, who is postpartum after premature
rupture of the membranes with ensuing chorioamnionitis
1 week after emergency cesarean section? Her blood cultures grew Escherichia coli.
A. Small amounts of hemoperitoneum
B. Uterine dehiscence
C. Gas foci within endometrial cavity
D. Bowel obstruction
D
Figure courtesy of Cheryl Sadow, MD.
3. Which mode of delivery carries the highest risk of postpartum uterine infection?
A. Vaginal delivery without instrumentation
B. Vaginal delivery with forceps
C. Cesarean section
4. Which of the following statements is true?
A. Small foci of gas in the endometrial cavity in a postpar-
tum woman indicate infection.
B. A mucopurulent discharge in a postpartum woman sug-
gests infection.
C. In acute endometritis, ultrasound can make the diagno-
sis in 90% of cases.
D. In acute endometritis, ultrasound can make the diagno-
sis in 50% of cases.
205