Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5789_Библиотеки_им_академика_М_И_Перельмана.pdf
ANSWERS
CASE 2
Pleural Effusions
1. B and D
2. D
3. B
4. A
References
Aubard Y, Derouineau I, Aubard V, et al: Primary fetal hydrothorax: a litera-
ture review and proposed antenatal clinical strategy. Fetal Diagn Ther 1998;
13(6):325-333.
http://www.ncbi.nlm.nih.gov/pubmed/9933813 (Accessed on May 29,
2012.)
Bianchi S, Lista G, Castoldi F, et al: Congenital primary hydrothorax: effect
of thoracoamniotic shunting on neonatal clinical outcome. J Matern Fetal
Neonatal Med 2010; 23(10):1225-1229.
http://www.ncbi.nlm.nih.gov/pubmed/20482288 (Accessed on May 29,
2012.)
Deurloo KL, Devlieger R, Lopriore E, et al: Isolated fetal hydrothorax with
hydrops: a systematic review of prenatal treatment options. Prenat Diagn
2007; 27(10):893-899.
http://www.ncbi.nlm.nih.gov/pubmed/17605152 (Accessed on May 29,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 270, 419-422.
Comment
Differential Diagnosis
The diagnosis of pleural effusions is straightforward. Any
uid within the fetal chest is abnormal. A pleural effusion is
either a primary abnormality such as chylothorax or a manifestation of a more generalized condition such as hydrops
fetalis. Primary chylothorax is often a unilateral process that
has an approximately equal occurrence on the right and left
sides. There are multiple etiologies of fetal hydrops, including
immune and nonimmune causes, but pleural effusion may be
one of the earliest signs. Secondary causes of pleural effusions
include infection, intrathoracic mass (congenital cystic adenomatoid malformation, sequestration), cardiovascular abnormalities, diaphragmatic hernia, chromosomal abnormalities,
and various syndromes.
Ultrasound Findings
A pleural effusion appears as anechoic uid noted within the
pleural spaces in the thoracic cavity and above the diaphragmatic contour. This uid surrounds the more echogenic lung
(Figure A). The uid may be either unilateral or bilateral. Bilateral pleural effusions as in this case suggest a secondary etiology of pleural effusions, such as fetal immune or nonimmune
hydrops or some other secondary etiology as listed previously.
MRI can be used to evaluate the pleural effusion and help determine if there is any other underlying abnormality (Figure B).
Prognosis and Management
Perinatal mortality of a fetus with a hydrothorax ranges from
35% to 50%. The two worst complications of fetal hydrothorax are development of pulmonary hypoplasia and hydrops.
Development of hydrops and early delivery are associated
with a poor neonatal outcome. The perinatal mortality rate of
untreated hydropic fetuses was 76% in one series compared
with only 25% of fetuses without hydrops. If the hydrothorax is large or clinical deterioration such as a hydropic fetus
develops, fetal thoracentesis or a thoracoamniotic shunt can
be considered. This treatment may not always be curative and
is associated with complications, but it has been shown to be
effective in some cases.
6
CASE 3
A
Used with permission from Anderson Publishing Ltd., from Hellinger J,
et al: Fetal MRI in the third dimension. Appl Radiol 39(7)8-19, 2010. ©
Anderson Publishing Ltd.
History: A patient presents for a routine second-trimester
ultrasound.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Enteric duplication cysts
B. Mesenteric cysts
C. Ovarian cysts
D. Choledochal cysts
E. Renal cysts
2. Which of the following is not included in the ultrasound
ndings in a fetus with ovarian cysts?
A. The cysts are usually bilateral.
B. The cysts may be seen in the upper abdomen.
C. The cysts may undergo torsion.
D. Most cysts are purely cystic.
3. In regard to treatment of fetal ovarian cysts, which of the
following statements is not true?
A. After delivery, observation alone is considered appro-
priate therapy.
B. Uncomplicated ovarian cysts usually resolve spontaneously.
C. Prenatal cyst aspiration should not be performed.
D. If ovarian torsion is present, surgery should be
considered.
4. Which of the following statements is not true?
A. Ovarian cysts may result in fetal ascites.
B. In the newborn, enteric duplication cysts are rarely
symptomatic.
B
C. Type I choledochal cysts are the most common type.
D. Omental cysts may have a variety of appearances.
C
7
ANSWERS
CASE 3
Ovarian Cyst
1. A, B, and C
2. A
3. C
4. B
References
Akın MA, Akın L, Ozbek S, et al: Fetal-neonatal ovarian cysts—their moni-
toring and management: retrospective evaluation of 20 cases and review
of the literature. J Clin Res Pediatr Endocrinol 2010; 2(1):28-33.
http://www.ncbi.nlm.nih.gov/pubmed/21274333 (Accessed on May 29,
2012.)
Dimitraki M, Koutlaki N, Nikas I, et al: Fetal ovarian cysts: our clinical expe-
rience with 16 cases and review of the literature. J Matern Fetal Neonatal
Med; 2011 May 26.
http://www.ncbi.nlm.nih.gov/pubmed/21615230 (Accessed on May 29,
2012.)
Galinier P, Carfagna L, Juricic M, et al: Fetal ovarian cysts management
and ovarian prognosis: a report of 82 cases. J Pediatr Surg 2008; 43(11):
2004-2009.
http://www.ncbi.nlm.nih.gov/pubmed/18970932 (Accessed on May 29,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 447.
Comment
Differential Diagnosis
The differential diagnosis for a fetal cystic abdominal or pelvic
mass is large. The location of the mass is important in the differential diagnosis. In this case, the mass is not located close to
the kidneys, so hydronephrosis and bladder outlet obstruction
are not strong considerations. Choledochal cysts are quite rare
and usually seen in the right upper quadrant of the abdomen
associated with the biliary ductal system. In this case, the cyst
is not within the liver or spleen, and splenic or liver cysts are
excluded. Meconium pseudocysts occur as a cystic abdominal
mass but usually not until later in pregnancy. Urachal cysts are
usually near the umbilical cord insertion site, and they can communicate with the urinary bladder. Umbilical vein varices are
a dilation of the umbilical veins seen with color ow imaging.
Ovarian cysts are the most common cystic abdominal
mass in female fetuses (Figures A and B). With improved fetal
imaging, more ovarian cysts are being diagnosed. Other differential diagnoses that may be considered include mesenteric
or omental cysts. Another simple cyst that may be included in
the differential diagnosis is an enteric duplication cyst, which is
located adjacent to the bowel (Figure C).
Ultrasound Findings
Ovarian cysts are typically benign functional cysts that result
from enlargement of otherwise normal follicles. These cysts
usually are less than a few millimeters and are too small to
be visualized, but with hormonal stimulation of the ovary by
the placenta and maternal hormones, larger cysts may develop.
These cysts are usually unilateral and cystic. They are usually
small, but they may become quite large—greater than 5 cm and
in reported cases 8 to 10 cm (Figures A and B). These cysts
may become complex and have a uid debris level, a retracting
clot, or internal septation usually from ovarian torsion (Figure
C). Ascites has been associated with ovarian cysts, thought to
be secondary to torsion or rupture of the cysts. Polyhydramnios has been reported in a few cases, probably secondary to
small bowel obstruction by mechanical compression by these
cysts.
Prognosis and Management
Birth dystocia, respiratory distress, and gastrointestinal
obstruction have been reported with very large cysts. A few
cases of prenatal aspiration have been reported. After delivery,
management is usually observation alone. An intervention such
as oophorectomy has been performed when there is a complication of the cyst such as ovarian torsion. Oophorectomy may
be performed if there are symptoms in the neonate such as
abdominal distention by the cyst or mechanical obstruction of
the bowel resulting in vomiting. Additionally, cystectomy with
salvage of the remainder of the ovary has been performed.
Overall prognosis is usually good. Other associated anomaly
with fetal ovarian cyst is rare. Rare cystic neoplasms of the
ovary in the fetus have been reported.
8
CASE 4
A
B
C
History: A patient presents with an abnormality identied
on an ultrasound obtained at 18 weeks’ gestation at an outside
institution.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Bilateral ureterovesical junction obstruction
B. Bilateral ureteropelvic junction (UPJ) obstruction
C. Posterior urethral valves
D. Megacystis-microcolon-intestinal hypoperistalsis syn-
drome (MMIHS)
E. Bilateral multicystic dysplastic kidneys
2. Which of the following statements regarding UPJ obstruc-
tion is not true?
A. The renal pelvis can enlarge to a massive proportion
and produce an abdominal cyst.
D
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. Rupture of the renal pelvis calyx results in urine ascites
or perinephric urinoma.
C. UPJ obstruction is more commonly a bilateral process.
D. UPJ obstruction can be associated with renal dysplasia.
3. Which of the following statements regarding the treatment
of UPJ obstruction is not true?
A. Double-J pigtail stent placement is commonly used for
the relief of obstruction in utero.
B. In utero follow-up scans are performed to reassess the
degree of hydronephrosis and amniotic uid volume.
C. After delivery, if surgery is performed, pyeloplasty
would be the surgery of choice.
D. Poor prognostic indicators include development of a
perinephric urinoma or development of a large abdom-
inal cyst.
4. Which of the following entities is associated with the best
prognosis?
A. MMIHS
B. Posterior urethral valves
C. Urethral atresia
D. UPJ obstruction
9
ANSWERS
CASE 4
Ureteropelvic Junction Obstruction
1. A, B, C, and D
2. C
3. A
4. D
References
Al-Shibli AI, Chedid F, Mirghani H, et al: The signicance of fetal renal pel-
vic dilatation as a predictor of postnatal outcome. J Matern Fetal Neonatal
Med 2009; 22(9):797-800.
http://www.ncbi.nlm.nih.gov/pubmed/19557659 (Accessed on May 30,
2012.)
Bethune M: Literature review and suggested protocol for managing ultra-
sound soft markers for Down syndrome: thickened nuchal fold, echogenic
bowel, shortened femur, shortened humerus, pyelectasis and absent or
hypoplastic nasal bone. Australas Radiol 2007; 51(3):218-225.
http://www.ncbi.nlm.nih.gov/pubmed/17504311 (Accessed on May 30,
2012.)
Chertin B, Pollack A, Koulikov D, et al: Conservative treatment of ure-
teropelvic junction obstruction in children with antenatal diagnosis of
hydronephrosis: lessons learned after 16 years of follow-up. Eur Urol 2006;
49(4):734-738.
http://www.ncbi.nlm.nih.gov/pubmed/16504374 (Accessed on May 30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, p 465.
Comment
Differential Diagnosis
A cystic structure is noted in the retroperitoneum in the location of the fetal kidneys. First, it must be determined if this
cystic structure is a cystic mass, such as a multicystic dysplastic
kidney, or if it is hydronephrosis. With hydronephrosis, the
cystic structure representing the dilated renal pelvis connects
to the prominent calyceal system, making diagnosis of hydronephrosis easier (Figures A and B). Unilateral UPJ obstruction is more common than bilateral. Bilateral hydronephrosis
can occur with bilateral UPJ obstruction (Figure A). In this
entity, the bladder is normal in size. Similarly, with bilateral
vesicoureteric junction obstruction, the bladder is normal in
size, but there are bilateral dilated ureters. Vesicoureteric reux
could be considered; this entity can have varying amounts of
dilation of the ureter and pelvis from side to side. Reux is a
common problem in the newborn.
Posterior urethral valves and urethral atresia result in a large,
thick bladder wall. With posterior urethral valves, the bladder neck has a “keyhole” appearance. There is usually severe
hydronephrosis and often oligohydramnios in both conditions.
Rare causes of bilateral dilation of the renal pelvis and ureters
include bilateral ureteroceles or MMIHS.
Ultrasound Findings
Dilation of the renal pelvis is seen with UPJ obstruction. The
degree of dilation has been classied using pelvic diameter and
the stage in pregnancy. The degree of caliectasis can also be
determined as none, moderate, or marked (Figure C). When
there is a question of dilation of the renal pelvis, postnatal
ultrasound is essential to determine the degree of renal tissue.
Several grading systems of fetal renal pelvic dilation have been
suggested, but there is no consensus regarding any specic
grading system. Fetal MRI may be helpful to obtain a better
overview of the severity of hydronephrosis and other abnormalities (Figure D).
Evaluation of the contralateral kidney for any abnormality
is also helpful. Contralateral renal anomalies are uncommon;
they occur in 20% of cases of UPJ obstruction and include
bilateral UPJ obstruction, contralateral multicystic dysplastic
kidney, and, more rarely, renal agenesis. The most important
prognostic factor in terms of fetal or neonatal survival is the
presence of a normal amount of amniotic uid.
Prognosis and Management
Management includes follow-up prenatal ultrasound scan to
determine the amount of renal pelvis dilation and the presence
of caliectasis. Most important, prenatal management includes
determining whether the amount of amniotic uid is normal.
After delivery, most cases are managed conservatively unless
hydronephrosis is increasing or renal function is poor.
10
CASE 5
History: A pregnant patient presents for a routine 20-week
ultrasound scan.
1. Which of the following would be included in the differential diagnosis? (Choose all that apply.)
A. Ventriculomegaly
B. Choroid plexus cyst
C. Intracranial hemorrhage
D. Semilobar holoprosencephaly
E. Dandy-Walker malformation
2. Which of the following is not a true statement concerning
choroid plexus cyst?
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.
D. These cysts are associated with an increased risk of
hydrocephalus.
3. Choroid is present in all portions of the lateral ventricles
except:
A. The frontal horn of the lateral ventricle
B. The body of the lateral ventricle
C. The trigone of the lateral ventricle
D. The temporal horn
4. Which chromosomal abnormality is most commonly asso-
ciated with choroid plexus cysts?
A. Trisomy 13
B. Trisomy 18
C. Trisomy 21
D. XO karyotype
11
ANSWERS
CASE 5
Choroid Plexus Cyst
1. A, B, and C
2. D
3. A
4. B
References
Beke A, Barakonyi E, Belics Z, et al: Risk of chromosome abnormalities in
the presence of bilateral or unilateral choroid plexus cysts. Fetal Diagn Ther
2008; 23(3):185-191.
http://www.ncbi.nlm.nih.gov/pubmed/18417976 (Accessed on May 30,
2012.)
Bethune M: Time to reconsider our approach to echogenic intracardiac focus
and choroid plexus cysts. Aust N Z J Obstet Gynaecol 2008; 48(2):137-141.
http://www.ncbi.nlm.nih.gov/pubmed/18366485 (Accessed on May 30,
2012.)
Fong K, Chong K, Toi A, et al: Fetal ventriculomegaly secondary to isolated
large choroid plexus cysts: prenatal ndings and postnatal outcome. Prenat
Diagn 2011; 31(4):395-400.
http://www.ncbi.nlm.nih.gov/pubmed/21328578 (Accessed on May 30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 395–397.
Comment
Differential Diagnosis
This case shows a well-demarcated hypoechoic structure
within both lateral ventricles. This structure is a classic nding for a choroid plexus cyst. The most common differential
diagnosis to consider would be intraventricular hemorrhage,
which may result in a blood clot that eventually cavitates
and becomes hypoechoic. However, intraventricular hemorrhage usually occurs late in the second trimester or early in
the third trimester. Choroid plexus cysts are usually observed
on a screening ultrasound scan in the 18th to 20th week of
gestation. Although unusual large and bilateral, choroid plexus
cysts could be mistaken for ventriculomegaly. Choroid plexus
cysts by themselves rarely cause ventriculomegaly; however,
they may be so large that they mimic 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 cyst is straightforward and
includes a well-demarcated hypoechoic structure with a welldened wall within the echogenic choroid of either the downside or upside lateral ventricle. The cysts are more commonly
identied in the downside lateral ventricle because the overlying skull reduces visualization of the upside lateral ventricle.
The cyst may be unilateral or bilateral and can be multiple (see
Figure). The denition of choroid plexus cyst is usually a cyst
greater than 2 mm in diameter in the choroid. Often there
is some normal heterogeneity of the choroid plexus, which
should not in itself be called a choroid plexus cyst.
Prognosis and Management
In the absence of an associated anomaly, a choroid plexus
cyst should be considered a normal anatomic variant. Parents are often concerned about fetal “cysts in the brain,” but
they should be reassured that these cysts are usually normal
and decrease rapidly in size by the third trimester. Remnants
of the cyst are only rarely identied after birth.
Although choroid plexus cysts are benign, this transient
nding is associated with increased risk of trisomy 18. When
a choroid plexus cyst is identied, careful scanning of the
fetus should be performed. The patient should be reassured
that in most cases these cysts are benign and do not affect
outcome. With the use of high-quality ultrasound equipment,
choroid plexus cysts are probably detected more frequently
than previously.
When a choroid plexus cyst is detected, 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.
Trisomy 18 is also known as trisomy E or Edwards syndrome.
12
CASE 6
A
B
History: A pregnant patient presents with a prior history of
an abnormal fetus.
1. What should be included in the differential diagnosis of
Figures A and B? (Choose all that apply.)
A. Fetal tachyarrhythmia
B. Fetal anemia
C. Fetal cystic hygroma
D. Chromosomal abnormalities
E. Rh incompatibility
2. Which of the following statements concerning fetal hydrops
is not true?
A. Rh incompatibility is the most common cause of fetal
hydrops.
B. Nonimmune hydrops is associated with two or more
areas of body cavity effusion.
C. Fetal hydrops includes one or more sets of body cavity
effusions plus anasarca.
D. Cardiac anomalies are an etiology of nonimmune fetal
hydrops.
3. Which of the following statements concerning fetal cystic
hygroma is not true?
A. Fetal cystic hygromas usually result from failure of
communication of the thoracic duct with the jugular
veins.
B. Septated cystic hygromas have a better prognosis than
nonseptated hygromas.
C. Cystic hygromas may be associated with XO karyotype.
D. Fetal cystic hygromas often result in fetal demise.
4. Which of the following statements concerning Turner syn-
drome is not true?
A. Turner syndrome is associated with coarctation of the
aorta.
B. Turner syndrome is associated with horseshoe kidneys.
C. Ovarian dysgenesis is associated with Turner syndrome.
D. Adult patients with Turner syndrome have normal
stature.
13
ANSWERS
CASE 6
Cystic Hygroma and Fetal Hydrops
1. A, B, C, D, and E
2. A
3. B
4. D
References
Ganapathy R, Guven M, Sethna F, et al: Natural history and outcome of
prenatally diagnosed cystic hygroma. Prenat Diagn 2004; 24(12):965-968.
http://www.ncbi.nlm.nih.gov/pubmed/15614877 (Accessed on May 30,
2012.)
Has R: Non-immune hydrops fetalis in the rst trimester: a review of 30
cases. Clin Exp Obstet Gynecol 2001; 28(3):187-190.
http://www.ncbi.nlm.nih.gov/pubmed/11530871 (Accessed on May 30,
2012.)
Tanriverdi HA, Hendrik HJ, Ertan AK, et al: Hygroma colli cysticum: prena-
tal diagnosis and prognosis. Am J Perinatol 2001; 18(8):415-420.
http://www.ncbi.nlm.nih.gov/pubmed/11733855 (Accessed on May 30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 406-410, 419-422.
Comment
Differential Diagnosis of Neck Mass
In this case two differential diagnoses must be considered.
The rst is the fetal neck mass. The differential diagnosis
of a fetal neck mass includes cystic hygroma, neck tumor,
or encephalocele. In this case, however, skin thickening and
uid collection in other cavities are also present. The most
likely diagnosis in this case is a fetal cystic hygroma. Fetal
cystic hygroma has many different names, including diffuse
lymphangiectasia, diffuse lymphangioma, hygroma colli, and
hygroma cysticum. Primitive lymphatic sacs located lateral
to the jugular vein develop in utero. Right and left thoracic
ducts join the jugular sacs with the cisterna chyli. Failure of
communication between these primitive structures and the
jugular veins results in accumulation of lymphatic uid in
the jugular lymphatic sacs and tissue, giving the appearance
of a cystic hygroma. This condition is often associated with a
thick brous band called a nuchal ligament. When a nuchal ligament is present, the prognosis is much worse and includes
an increased risk of aneuploidy, hydrops, other anomalies,
and pregnancy loss.
Differential Diagnosis of Fetal Hydrops
The differential diagnosis of fetal hydrops is large and includes
both immunologic and nonimmunologic causes of hydrops. In
the United States with effective prophylaxis against Rh immunization, most cases of fetal hydrops are nonimmune. The
many etiologies of nonimmune hydrops include high-output
cardiac failure, obstruction of venous return (e.g., congenital
cystic adenomatoid malformation or congenital pulmonary
airway malformation [CPAM]), decreased plasma oncotic pressure with decreased albumin formation, increased capillary
permeability such as with a congenital infection, and obstruction of lymphatic ow such as in this case with cystic hygroma.
Ultrasound Findings
Ultrasound ndings of cystic hygroma include a septated or
nonseptated neck mass, usually associated with diffuse anasarca of the fetus (Figures A and B). This anasarca is a fairly
characteristic feature in the second trimester. In addition, there
may be a focal form of cystic hygroma with a separate cystic
mass in the cranial, neck, or thoracic region without hydrops.
A focal cystic hygroma is associated with a good prognosis.
Prognosis and Management
The prognosis of fetal hydrops depends on the specic diagnosis. Fetal cystic hygroma is associated with an increased
risk of other abnormalities, including Turner syndrome (XO
karyotype) and pseudo-Turner syndrome (Noonan syndrome).
Newborns with Turner syndrome have short stature, webbed
neck, cardiac defects, and ovarian dysgenesis. In cases of fetal
hydrops, it is important to perform a complete ultrasound
examination. A basic approach for fetal hydrops could include
amniocentesis for karyotyping and a comprehensive ultrasound examination to detect other abnormalities. If immediate diagnosis is needed, cordocentesis may be performed.
Other therapy can be based on the precise etiology. If there is
tachyarrhythmia, this may be identied by M-mode ultrasound,
and treatment such as pharmacologic therapy can be initiated
to convert the abnormal fetal heart rate to sinus rhythm.
14
CASE 7
A
History: A patient with a second-trimester pregnancy
undergoes imaging because of an abnormally small increase in
uterine enlargement on physical examination.
1. What should be included in the differential diagnosis for
the main nding of the very small amount of amniotic uid
in the second-trimester fetus of the patient (Figures A and
B) and a different third-trimester fetus (Figure C)? (Choose
all that apply.)
A. Spontaneous rupture of the membranes (SROM)
B. Gestation extending beyond the due date
C. Potter syndrome
D. Fetal demise
2. What quantitative measure is used to evaluate the severity
of decreased amniotic uid?
A. Amniotic uid index
B. Resistive index
C. Pulsatility index
3. What is the most likely cause of death of the newborn?
A. Congestive heart failure
B. Pulmonary hypoplasia
C. Renal failure
D. Growth restriction
4. What is the potentially serious short-term complication of
membrane rupture?
A. Hip dysplasia
B. Congestive heart failure
C. Clubfoot
D. Chorioamnionitis
B
C
15