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
X
- •Series Foreword
- •Book Foreword
- •Preface
- •Acknowledgments
- •Contents
- •Opening Round
- •CASE 2
- •CASE 5
- •CASE 6
- •CASE 7
- •CASE 3
- •CASE 4
- •CASE 8
- •CASE 9
- •CASE 10
- •CASE 11
- •CASE 12
- •CASE 13
- •CASE 14
- •CASE 15
- •CASE 16
- •CASE 17
- •CASE 18
- •CASE 19
- •CASE 20
- •CASE 21
- •CASE 22
- •CASE 23
- •CASE 24
- •CASE 25
- •CASE 26
- •CASE 27
- •CASE 28
- •CASE 29
- •CASE 31
- •CASE 32
- •CASE 33
- •CASE 34
- •CASE 35
- •CASE 36
- •CASE 37
- •CASE 38
- •CASE 39
- •CASE 40
- •CASE 41
- •CASE 42
- •CASE 43
- •CASE 44
- •CASE 45
- •CASE 46
- •CASE 47
- •CASE 48
- •CASE 49
- •CASE 50
- •CASE 51
- •Fair Play
- •CASE 53
- •CASE 54
- •CASE 55
- •CASE 56
- •CASE 57
- •CASE 58
- •CASE 59
- •CASE 60
- •CASE 61
- •CASE 62
- •CASE 63
- •CASE 64
- •CASE 65
- •CASE 66
- •CASE 67
- •CASE 68
- •CASE 69
- •CASE 70
- •CASE 71
- •CASE 72
- •CASE 73
- •CASE 74
- •CASE 75
- •CASE 76
- •CASE 77
- •CASE 78
- •CASE 79
- •CASE 80
- •CASE 81
- •CASE 82
- •CASE 83
- •CASE 84
- •CASE 85
- •CASE 86
- •CASE 87
- •CASE 88
- •CASE 89
- •CASE 90
- •CASE 91
- •Challenge
- •CASE 93
- •CASE 94
- •CASE 95
- •CASE 96
- •CASE 97
- •CASE 98
- •CASE 99
- •CASE 100
- •CASE 101
- •CASE 102
- •CASE 103
- •CASE 104
- •CASE 105
- •CASE 106
- •CASE 107
- •CASE 108
- •CASE 109
- •CASE 110
- •CASE 111
- •CASE 112
- •CASE 113
- •CASE 114
- •CASE 115
- •CASE 116
- •CASE 117
- •CASE 118
- •CASE 119
- •Index Of Cases
- •Index of Terms

ANSWERS
CASE 7
Oligohydramnios
1. A, C, and D
2. A
3. B
4. D
be immediately apparent (Figures A and B). Fetal kidneys can
be seen at 12 to 14 weeks’ gestation. The adrenal glands are
large in utero and can resemble kidneys; the urinary bladder
is still not present. Color Doppler imaging has been useful in
documenting the presence of renal arteries. A severe bilateral
renal obstruction or any other bilateral renal anomaly that
affects function would also lead to severe oligohydramnios.
References
Cunningham FG, MacDonald PC, Gant NF, et al: Placental disorders:
disease and abnormalities of the fetal membranes. In Cunningham FG,
Williams JW (eds): Williams Obstetrics, 20th ed. Stamford, CT, Appleton &
Lange 1997, pp 664-665.
Kilbride HW, Yeast J, Thibeault DW: Dening limits of survival: lethal pul-
monary hypoplasia after midtrimester premature rupture of membranes.
Am J Obstet Gynecol 1996; 175(3 Pt 1):675-681.
http://www.ncbi.nlm.nih.gov/pubmed/8828433 (Accessed on May 30,
2012.)
Ott WJ: Reevaluation of the relationship between amniotic uid volume and
perinatal outcome. Am J Obstet Gynecol 2005; 192(6):1803-1809.
http://www.ncbi.nlm.nih.gov/pubmed/15970814 (Accessed on May 30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 458, 460.
Comment
Ultrasound Imaging to Diagnose
Oligohydramnios
Second- and third-trimester fetuses are imaged because of a
history of abnormally small increase in uterine enlargement on
physical examination. Abnormalities in amniotic uid volume
reect underlying fetal, maternal, and placental conditions.
Oligohydramnios is a uid volume less than the 5th percentile
for a specic gestational age. The amniotic uid volume peaks
in the second trimester. Although the diagnosis can be made
by measuring the uid as four perpendicular measurements
added together, a subjective evaluation of the amount of uid
is accurate.
Ultrasound Imaging of Bilateral Renal Agenesis
Severe oligohydramnios should prompt an ultrasound scan to
identify the cause (Figures A to C). The anatomy should be
evaluated for the presence of kidneys (Figure B) and uid in
the urinary bladder; in cases of bilateral renal agenesis, both
kidneys and uid are absent. As this case of bilateral renal
agenesis shows, the abnormality in amniotic uid volume may
Ultrasound Imaging of Spontaneous Rupture
of Membranes
A separate case of SROM in a third-trimester fetus (Figure
C) shows that the degree of oligohydramnios can be severe.
Frequently the kidneys can be seen, and the urinary bladder
can be identied. In cases of SROM in which the amniotic
uid index is less than 1 cm, the duration of this exposure to
oligohydramnios and the gestational age at the time of membrane rupture are predictors of fetal outcome. Fetal mortality
is greater than 90% if membranes rupture before 25 weeks
and the exposure to severe oligohydramnios continues for
more than 14 days. Chorioamnionitis is a serious complication.
Lethal pulmonary hypoplasia occurs in 20% of cases of membrane rupture. In fetuses that survive severe oligohydramnios
secondary to membrane rupture, limb deformities can occur.
Of fetuses born after more than 2 weeks of this exposure,
80% had such deformities.
Additional Causes of Oligohydramnios
Alternative causes of oligohydramnios include growth restriction, chromosomal anomalies, congenital anomalies (e.g., cystic
hygroma), and fetal demise. Maternal causes include hypertension, diabetes, and preeclampsia. Placental insufciency, a
cause of oligohydramnios later in gestation, warrants umbilical artery Doppler imaging whenever the uid volume appears
low. To exclude rupture of the membranes as the cause, the
mother should be asked about uid leakage.
Postdates Gestation
In gestations that extend beyond the expected due date, amniotic uid can normally decrease. This decrease may result in
umbilical cord compression and fetal heart deceleration. Monitoring includes frequent amniotic uid index measurements
and subjective quantitative measurements of uid volume,
maternal assessment of fetal movement, and fetal nonstress
cardiac testing.
16

CASE 8
A
CC
B
History: A 55-year-old, postmenopausal woman presents
with a palpable right-sided pelvic mass.
1. What should be included in the differential diagnosis of
this right-sided pelvic mass based on the ultrasound images
(Figures C and D)? (Choose all that apply.)
A. Ovarian cancer
B. Cystadenoma
C. Tuboovarian abscess
D. Dermoid
E. Dominant ovarian follicle
2. What is the most likely diagnosis?
A. Ovarian cancer
B. Cystadenoma
DD
C. Tuboovarian abscess
D. Dermoid
3. What does the spectral waveform from the soft tissue component of the mass suggest?
A. Benign process
B. Concern for malignancy
C. High resistance
4. Which benign ovarian masses do not show high diastolic
ow?
A. Tuboovarian abscess
B. Endometriomas
C. Dermoids
D. Simple ovarian cysts
17

ANSWERS
CASE 8
Ovarian Cancer
1. A, B, C, and D
2. A
3. B
4. D
References
Alcazar JL, Galan MJ, Ceamanos C, et al: Transvaginal gray scale and color
Doppler sonography in primary ovarian cancer and metastatic tumors to
the ovary. J Ultrasound Med 2003; 22(3):243-247.
http://www.ncbi.nlm.nih.gov/pubmed/12636323 (Accessed on May 30,
2012.)
Alcazar JL, Galal MJ, Garcia-Manero M, et al: Three-dimensional sono-
graphic morphologic assessment in complex adnexal masses J Ultrasound
Med 2003; 22(3):249-254.
http://www.ncbi.nlm.nih.gov/pubmed/12636324 (Accessed on May 30,
2012.)
Brown DL, Zou KH, Tempany CM, et al: Primary versus secondary ovarian
malignancy: imaging ndings of adnexal masses in the Radiology Diagnostic Oncology Study. Radiology 2001; 219(1):213-218.
http://www.ncbi.nlm.nih.gov/pubmed/11274559 (Accessed on May 30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 579-583.
Comment
Gray-Scale Ultrasound Features of Ovarian
Cancer
Ovarian cancer exhibits numerous ultrasound criteria for a
malignant mass (Figures A and B). The presence of a solid
component or solid papillary mural projections (Figure A),
particularly if they are nonhyperechoic, is worrisome. A hyperechoic solid component is seen more typically in a dermoid. If
a uid component is present, it is more commonly anechoic
or hypoechoic. Septations may or may not occur in a malignant mass, but if present, they are usually 3 mm or thicker.
The wall is often not discernible but can be thin or thick if
seen. A malignant cyst is accompanied by ascites in 30% of
cases, which suggests spread to the pelvis (stage 2) or abdomen
(stage 3 or 4). Multilocularity favors the diagnosis of a primary
ovarian neoplasm rather than a secondary one. A purely solid
tumor indicates a higher probability of metastatic carcinoma
rather than primary ovarian carcinoma (Figure C).
Doppler Ultrasound Features of Ovarian Cancer
Use of Doppler ultrasound to distinguish a benign from a
malignant ovarian mass has been shown to be nonpredictive.
Doppler ultrasound has been applied on the principle that low
impedance ow should indicate a malignancy. When arterial
signals are detected, the systolic and diastolic components can
be evaluated. Measurements include the resistive index, or RI:
(peak systolic velocity – end diastolic velocity) ÷ peak systolic
velocity, and the pulsatility index, or PI: (peak systolic velocity –
end diastolic velocity) ÷ mean velocity. The standard cutoff
level for malignancy is RI less than 0.4 or PI less than 1.0. It
is common for a malignant lesion to have a borderline or low
ratio that is suggestive of a malignancy (Figure D). Conversely,
arterial ow with systolic ow but little or no diastolic ow is a
high-resistance signal that is seen almost exclusively in benign
lesions.
Magnetic Resonance Imaging of Ovarian Masses
Considerable overlap has been shown between benign and
malignant masses when the spectral waveform shows an arterial waveform with a high diastolic component (low impedance). In the case presented here, RI is borderline, and the
mass was pathologically malignant. Benign masses that are
endocrine secreting or inammatory may have this ow pattern, particularly tuboovarian abscesses, endometriomas, and
ovarian dermoids. In a more recent large study, in women with
an indeterminate mass on gray-scale ultrasound, the use of
MRI contributed more to a change in probability of malignancy in both premenopausal and postmenopausal women
than did the use of CT or combined gray-scale and Doppler
ultrasound.
18

CASE 9
History: A 40-year-old woman with a late rst-trimester
pregnancy presents with right lower quadrant pain.
1. What should be included in the differential diagnosis of the
transabdominal images (Figures A to C)? (Choose all that
apply.)
A. Meckel diverticulum
B. Acute appendicitis
C. Crohn disease
D. Intussusception
2. Which nding suggests appendicitis but is also seen normally in the pregnancy state?
A. Anemia
B. Leukocytosis
C. Left lower quadrant pain
D. Thrombocytopenia
A
3. Which of the following ndings is not a complication of
acute appendicitis?
A. Large-for-dates fetus
B. Preterm delivery
C. Spontaneous abortion
D. Internal fetal sepsis
E. Neonatal neurologic injury
4. What is the worst complication of appendicitis in a thirdtrimester gestation?
A. Anemia
B. Peritonitis
C. Adhesions
B
C
19

ANSWERS
CASE 9
Acute Appendicitis
1. A and B
2. B
presenting symptom of appendicitis in pregnancy regardless
of gestational age. The location of the cecum and the appendix may be distorted during pregnancy. Fever and leukocytosis
are not clear indicators of appendicitis in pregnancy.
3. A
4. B
References
Glanc P, Maxwell C: Acute abdomen in pregnancy: role of sonography.
J Ultrasound Med 2010; 29(10):1457-1468.
http://www.ncbi.nlm.nih.gov/pubmed/20876900 (Accessed on May 30,
2012.)
Long SS, Long C, Macura KJ: Imaging strategies for right lower quadrant
pain in pregnancy. AJR Am J Roentgenol 2011; 10(1):4-12.
http://www.ncbi.nlm.nih.gov/pubmed/21178041 (Accessed on May 30,
2012.)
McGahan JP, Lamba R, Coakley FV: Imaging non-obstetrical causes of
abdominal pain in the pregnant patient. Appl Radiol 2010; 10-25.
http://www.appliedradiology.com/Article.aspx?id=25245 (Accessed on June
25, 2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 224-225.
Comment
Clinical Findings
Appendicitis occurs in 1 in 1500 pregnant women. It is the
most common cause of nontraumatic abdominal pain in pregnancy. It can develop in the rst, second, or third trimester. The
symptoms of appendicitis are identical to symptoms that occur
in a normal pregnancy. Leukocytosis and nausea are common.
The enlarging uterus can cause severe right lower quadrant
pain owing to round ligament strain. The clinical differential
diagnosis of right lower quadrant pain in pregnancy includes
appendicitis, renal calculus, pyelonephritis, placental abruption, degeneration of myoma, ovarian cyst, and torsion. Right
lower quadrant pain has been found to be the most common
Ultrasound Findings
Ultrasound with graded compression has been used to make
the diagnosis, avoiding the ionizing radiation of CT. Early in
pregnancy, the inamed appendix may be visualized as a noncompressible tubular structure measuring 7 mm or more, as
shown in this case (Figures A to C), with a diameter of 15 mm.
An appendicolith may be present (Figure B). Doppler ultrasound has been found to be a sensitive indicator of inammation and increased diastolic ow with a low resistive index
(see hyperemia in Figure C). Pain often occurs directly over this
area. In the setting of perforation, a collection of peritoneal
uid may be detected. As the uterus enlarges, the appendix can
move superiorly and toward the anks. Acute appendicitis is
more difcult to diagnosis in the third trimester.
Magnetic Resonance Imaging
MRI is a safe modality to evaluate pregnant patients if there is
a clinical suspicion of acute appendicitis. Unnecessary operations can be avoided when a normal appendix is imaged. In
one series, 50% of pregnant women who underwent surgery
had appendicitis.
Complications of Appendicitis
If the diagnosis of appendicitis is missed, peritonitis results.
In the third trimester, peritonitis has a poor prognosis, and
maternal mortality is approximately 5%. Other complications
include preterm labor, spontaneous abortion, and fetal neurologic injury if maternal-fetal sepsis results.
20

CASE 10
A
B
Used with permission from McGahan JP, et al: Fetal abdomen and pelvis. In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008; 1316. Courtesy of Marshal
Swartz, MD.
C
Used with permission from McGahan JP, et al: Fetal abdomen and pelvis. In McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed.
New York: Informa Healthcare USA, 2008; 1316.
History:Apatientfromanoutsideinstitutionpresentswith
anultrasoundscanshowingafetalmassandundergoesasecondultrasoundexamination.
1. What should be included in the differentialdiagnosis of
FigureA?(Chooseallthatapply.)
A. Sacralmeningomyelocele
D
B. Limb–bodywallcomplex
C. Omphalocele
D. Amnioticbandsyndrome
E. Sacrococcygealteratoma
2. Which of the following statements concerningsacrococ-
cygealteratomaisnottrue?
A. Incidenceisapproximately1:40,000.
B. Mostprenatallydetected sacrococcygeal teratomasare
malignant.
C. Sacrococcygealteratomasareassociatedwithafemale-
to-maleratioof approximately4:1.
D. Few sacrococcygeal teratomas are entirely internal
withinthesacrum.
3. Which of the followingstatementsconcerning the ultra-
soundappearanceof sacrococcygealteratomaisnottrue?
A. FetalMRImaybehelpfultodetecttheinternalpresa-
cralcomponentsof sacrococcygealteratoma.
B. Sacrococcygealteratomasare often associatedwithan
abnormalkaryotype.
C. Color Dopplerultrasoundmayshowa highlyvascular
masswithlargesolidteratomas.
D. Thesetumorsmaybecystic,solid,ormixed.
4. Whichof thefollowingisnotapoorprognosticfactorasso-
ciatedwithsacrococcygealteratomas?
A. Developmentoffetalhydrops
B. Malignanthistologyof thelesion
C. Presence of external rather than internal sacral
components
D. Sizeof thetumor
21

ANSWERS
CASE 10
Sacrococcygeal Teratoma
1. A,B,D,andE
2. B
3. B
4. C
References
GucciardoL,UyttebroekA,DeWeverI,etal:Prenatalassessmentandman-
agementof sacrococcygealteratoma.Prenat Diagn2011;31(7):678-688.
http://www.ncbi.nlm.nih.gov/pubmed/21656530(AccessedonMay30,
2012.)
HoKO,SoundappanSV,WalkerK,etal:Sacrococcygealteratoma:the
13-yearexperienceof atertiarypaediatriccentre.J Paediatr Child Health
2011;47(5):287-291.
http://www.ncbi.nlm.nih.gov/pubmed/21599781(AccessedonMay30,
2012.)
WilsonRD,HedrickH,FlakeAW,etal:Sacrococcygealteratomas:prenatal
surveillance,growthandpregnancyoutcome.Fetal Diagn Ther2009;
25(1):15-20.
http://www.ncbi.nlm.nih.gov/pubmed/19122459(AccessedonMay30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES,2nded,pp408-410.
mesoderm, and endoderm. Theyare thought to have originatedfrom totipotentcells of theHensennode.Sacrococcygealteratomasarelocatedmidlineinthepresacralregionand
are uniformly attached to the coccyx. Several patterns may
beshownwithsacrococcygealteratomas, includingpredominantlyasolidmasswithsmallanechoicregionsasidentiedin
thiscase(FiguresAandB),aunilocularcysticmass(FiguresC
andD),oramixedcysticandsolidmass.
Four types of sacrococcygeal teratomas have been
described:
• TypeI—predominantly external with minimal presacral
component
• TypeII—predominantlyexternalwithsignicantintrapel-
viccomponent
• Type III—predominantly internal with abdominal
extension
• TypeIV—entirelyinternalwithnoexternalcomponent
TypesIandIIaccountformostcases.Only10%ofsacrococcygealteratomasaretypeIV.TypeIVsacrococcygealteratomashaveahigherrateof malignancy.MRImaybehelpful
to dene better the extent of the sacrococcygeal teratoma,
especiallyinternal(presacral)components(FiguresCandD).
Dependingonthesizeandvascularityoftheteratoma,polyhydramniosandfetalhydropsmayresult,bothofwhicharepoor
prognosticindicators.
Comment
Differential Diagnosis
The differential diagnosis of a presacral massisstraightforward. Sacrococcygeal teratomas may be cystic or solid or
mixed. They may be quite large. The differential diagnosis
is small because sacrococcygeal teratomas have a pathognomonic appearance. However, myelomeningocele can be
consideredwithinthedifferentialdiagnosis.Alesslikelyconsideration wouldbeamniotic bands leading to amputational
defectswhetherwithinthepelvisorelsewherewithinthefetus.
Likewise,thereisaspectrumofabnormalitiesthatcanoccur
withlimb–bodywallcomplex.Inthisanomaly,thefetusfuses
withtheplacentaandotheramputationaldefectsoccurinthe
fetus. When a meningomyelocele is present, the fetus usually has a lemon-shaped head and abanana-shaped cerebellum,whichishelpfulforultrasoundndingsassociatedwith
meningomyelocele.
Ultrasound Findings
Onultrasound,asacrococcygealteratomaappearsasacystic,
solid,ormixed cysticandsolidmass arisingfromthesacrococcygeal region (Figures A to D). Teratomas are tumors
consisting of tissues fromall three germlayers—ectoderm,
Prognosis and Management
Theprognosisdependsonthedevelopmentof fetalhydrops,
malignantversusbenignhistology,andthesizeof thetumor.
Hydropsoccurstypicallywithalargersolidtumor,whichhasa
signicantvascularcomponentthatcanresultinhydropsand
fetaldemise.Thereisalowerriskof malignancyintypeIand
typeIIsacrococcygealteratomas,whereastype IVteratomas
have a higher rate of malignancy and are associated with a
poorprognosis.Fetalhydropsresultsfromhigh-outputcardiac
failure, similar to what is observed in arteriovenous malformationssuchasveinofGalenaneurysmorchorioangiomas.
Theseconditionsare referred toasa vascularstealphenomenon.Emergencytherapysuchasdeliverybycesareansection
maybeindicatedwhenthereisincreaseintumorgrowth,signs
of cardiacfailure,orhydrops.Ifnoneofthesefactorsispresent,thefetusmaybemonitoredweekly with either planned
vaginal or cesarean delivery depending on the size of the
tumor.Rarefetalinterventionsincludemajorvesselablation,
amniodrainagetopreventpretermlabor,andcystdecompressionbeforedelivery.Inrareinstances,suchasinthepresence
of earlyfetalhydrops,inuteroresectionhasbeenperformed.
Evenwithsuccessfulsurgery,theremayberesultantinjuryto
thebowelorbladderafterbirth.
22

CASE 11
A
Used with permission from McGahan JP, et al: Fetal head and brain. In
McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed. New
York: Informa Healthcare USA, 2008; 1151.
C
Used with permission from McGahan JP, et al: Fetal head and brain. In
McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed. New
York: Informa Healthcare USA, 2008; 1151.
B
History: Three separate cases of ultrasound through the
fetal head between 20 and 22 weeks’ gestation are presented.
1. What should be included in the differential diagnosis?
(Choose all that apply.)
A. Arachnoid cyst
B. Dandy-Walker malformation
C. Dandy-Walker variant
D. Mega cisterna magna
E. Hydrocephalus
2. Concerning Dandy-Walker syndrome, which of the following statements is not true?
A. This syndrome includes cystic dilation in the fourth
ventricle.
B. There is complete or partial agenesis of the cerebellar
vermis.
C. It is rarely associated with other structural abnormalities.
D. It is often associated with karyotypic abnormalities.
3. Which of the following entities is not an abnormality related
to Dandy-Walker complex?
A. Dandy-Walker malformation
B. Dandy-Walker variant
C. Arachnoid cyst
D. Mega cisterna magna
4. Which of the following statements is true?
A. There are rarely karyotypic abnormalities with Dandy-
Walker variant.
B. Dandy-Walker syndrome has a higher association of
neonatal death than Dandy-Walker variant.
C. Ventriculomegaly is an uncommon nding with Dandy-
Walker malformation.
D. Dandy-Walker variant is commonly associated with fetal
anatomic defects.
23

ANSWERS
CASE 11
Dandy-Walker Complex
1. A, B, and C
2. C
3. C
4. B
References
Bromley B, Nadel AS, Pauker S, et al: Closure of the cerebellar vermis: evalu-
ation with second trimester US. Radiology 1994; 193(3):761-763.
http://www.ncbi.nlm.nih.gov/pubmed/7972820 (Accessed on May 30,
2012.)
Ecker JL, Shipp TD, Bromley B, et al: The sonographic diagnosis of Dandy-
Walker and Dandy-Walker variant: associated ndings and outcomes.
Prenat Diagn 2000; 20(4):328-332.
http://www.ncbi.nlm.nih.gov/pubmed/10740206 (Accessed on May 30,
2012.)
Shekdar K: Posterior fossa malformations. Semin Ultrasound CT MR 2011;
32(3):228-241.
http://www.ncbi.nlm.nih.gov/pubmed/21596278 (Accessed on May 30,
2012.)
Cross-Reference
Ultrasound: The REQUISITES, 2nd ed, pp 390-395.
Comment
Differential Diagnosis
The differential diagnosis for a midline cystic abnormality in
the posterior fossa would include classic Dandy-Walker malformation, Dandy-Walker variant, and perhaps a posterior
fossa arachnoid cyst. Mega cisterna magna, in which there is
an enlarged cisterna magna but with integrity of the cerebellar vermis and the fourth ventricle, would be less likely to be
included in the differential diagnosis.
Ultrasound Findings
Dandy-Walker complex refers to the spectrum of anomalies
of the posterior fossa (Figures A to C). Ultrasound ndings
of Dandy-Walker complex can include classic Dandy-Walker
malformation, characterized by cystic dilation in the fourth
ventricle, partial or complete agenesis of the cerebellar vermis, and an enlarged posterior fossa with displacement of the
tentorium superiorly. The cyst communicates with the fourth
ventricle through the defect in the cerebellar vermis (Figures
A and B). This was originally termed Dandy-Walker syndrome.
In the less severe form of Dandy-Walker complex called
Dandy-Walker variant (Figure C), there is variable hypoplasia
of the cerebellar vermis with or without enlargement of the
posterior fossa. It would seem that this variant would carry a
much better prognosis than classic Dandy-Walker malformation; however, ultrasound abnormalities, including ventriculomegaly, cardiac defects, and karyotypic abnormalities, are
common. The prognosis of Dandy-Walker variant is slightly
better than the prognosis of Dandy-Walker malformation;
normal outcomes have been reported in infants with isolated
ndings of Dandy-Walker variant. Care must be taken not to
suggest the diagnosis of Dandy-Walker variant too early. The
cerebellar vermis does not close from superior to inferior until
17 to 18 weeks’ gestation. At 15 to 16 weeks, it is common to
nd the cerebellar vermis not completely closed. One must be
careful not to “overcall” Dandy-Walker variant at this stage of
pregnancy because there may still be communication with the
fourth ventricle and the posterior fossa.
Prgnosis and Management
Prognosis and management depend on associated abnormalities and karyotypic ndings. In one series, 85% of fetuses with
Dandy-Walker malformation or Dandy-Walker variant had
other abnormalities identiable on ultrasound. In addition,
approximately one third of cases of Dandy-Walker malformation or Dandy-Walker variant have an abnormal karyotype.
However, in Dandy-Walker variant, there is a higher rate of
neonatal survival and otherwise normal infants compared with
Dandy-Walker malformation. Overall, the presence of other
abnormalities is associated with the worst prognosis. An isolated Dandy-Walker variant has the highest chance of leading
to a normal neonate.
24

CASE 12
A
Used with permission from McGahan JP, et al: Fetal head and brain. In
McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed. New
York: Informa Healthcare USA, 2008; 1156.
B
Used with permission from McGahan JP, et al: Fetal head and brain. In
McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed. New
York: Informa Healthcare USA, 2008; 1156.
C
Used with permission from McGahan JP, et al: Fetal head and brain. In
McGahan JP, Goldberg BB [eds]: Diagnostic Ultrasound, 2nd ed. New
York: Informa Healthcare USA, 2008; 1156.
History: A pregnant patient presents with a suspected cystic
fetal brain abnormality.
1. What should be included in the differential diagnosis for
a midline cystic abnormality in the fetal brain? (Choose all
that apply.)
A. Choroid plexus cyst
B. Porencephaly
C. Schizencephaly
D. Arachnoid cyst
E. Vein of Galen aneurysm
2. Which of the following entities is not an abnormality that
may be identied within the brain of the fetus or newborn
in cases of arteriovenous malformations (AVMs)?
A. Ventriculomegaly
B. Porencephaly
C. Brain edema
D. Occlusion of the internal carotid artery
3. Which of the following conditions is not associated with
brain AVMs?
A. Fetal hydrops
B. Cardiomegaly
C. Arachnoid cyst
D. Pleural effusion
4. Which of the following options would not be considered in
management of fetal cerebral AVM?
A. Performing serial scans
B. In utero embolization of fetal AVM
C. Early delivery with development of fetal hydrops
D. Ruling out vaginal delivery
25
Соседние файлы в папке Библиотека им академика М.И. Перельмана
