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FetalUSumbilicalarteryDopplerexamat32weeks.

FetalUSumbilicalarteryDopplerexamat34weeks.

Regarding the umbilical artery Doppler examination shown, which of the following is concerning for intrauterinefetaldistress?

A.Increasedpeaksystolicflow B.Decreasedpeaksystolicflow C.Increasedend-diastolicflow D.Reversedend-diastolicflow

18a.A25-year-oldfemalewithpreviousectopicpregnancyandCesareansectiondeliverypresentsfor

routine fetalanatomicsurveyat20 weeksofgestationalage.Animageatthelower uterine segmentis shown.

Whatisthemostlikelydiagnosis?

A.Vasaprevia B.Placentaprevia C.Choriocarcinoma D.Placentalabruption

18b.Whichofthefollowingincreasesthelikelihoodofdevelopingplacentaprevia?

A.Previousvaginaldelivery B.Firstpregnancy C.Advancedmaternalage D.Gestationaldiabetes

19.A32-year-oldG2P1undergoesobstetricultrasoundat32weeksofgestationalage.

Whichofthefollowingisthemostlikelydiagnosis?

A.Cysticfibrosis B.Meconiumperitonitis C.Meconiumileus D.Fetalhydrops

AnswersandExplanations

1a.AnswerC.ThefetalUSandMRIimagesshowasinglecentralventricleandfusedthalami.Nofalx

or interhemispheric fissure is present. These imaging findings are consistent with alobar holoprosencephaly, whichis the mostsevere form of holoprosencephaly. Inalobar holoprosencephaly, there is completelack of separation ofthecerebral hemispheres with a largeposterior monoventricle. Single midline structures such as the falx, interhemispheric fissure, septum pellucidum, and corpus callosumareabsentwhereaspairedmidlinestructuressuchasthethalamiandbasalgangliaarefused.

Holoprosencephalyis a spectrum ofcongenitalabnormalities ranging from incomplete formationof thefalxcerebriandinterhemisphericfissuretoacompletelackofseparationofthecerebralhemispheres witha largemonoventricle.Thethreetypesofholoprosencephalyare alobar(mostsevere), semilobar,

andlobar(leastsevere).Septoopticdysplasiaisconsideredthemildestformoflobarholoprosencephaly.

In severe fetal hydrocephalus (choice B), the ventricles are severely dilated, but a thin rim of preserved cortical tissue is present peripherally. Also, the middle cerebral arteries are preserved in severefetalhydrocephalus.

Inhydranencephaly(choiceA),thecerebralhemispheresofthebrainareabsentandreplacedbysacs filledwithcerebrospinalfluid.Anincompleteorcompletefalxispresent.Hydranencephalyisbelieved toresultfromocclusion ofthemiddlecerebralorinternalcarotid arteries.Usuallythe cerebellum and brainstemareformednormallybecausetheposteriorcirculationisnotaffected.

Acrania(choiceD)isararelethalcongenitalanomalycharacterizedbyanabsenceofthecalvarium.

1b.AnswerD.Themidlinefacialabnormalitiesassociatedwithalobarholoprosencephalyarecyclopia

(singlemidlineeyewithfailed developmentofnosewithorwithoutaproboscis), ethmocephaly (small narrow-seteyeswithabsenceofnose),cebocephaly(smallnarrow-seteyeswithaflattenednoseandone nostril),cleftpalateandlip,andsolitarymaxillarycentralincisor.

Facialandcalvarialabnormalitieshelppredicttheseverityofthebrainmalformation.Therefore,the presence offacial anomalies should trigger careful searchfor brainanomalies. “Theface predicts the brain.”

References: Barkovich AJ. Congenital malformations of the brain and skull. In: Barkovich AJ (ed). Pediatric neuroimaging, 4th ed. Philadelphia,PA:LippincottWilliams&Wilkins,2005:291–439.

HertzbergBS,MiddletonWD.Ultrasound:therequisites,3rded.Philadelphia,PA:Elsevier,2016:362.

WinterTC.Diagnosticimaging:obstetrics,2nded.LippincottWilliams&Wilkins,2011:1–2.

2a.AnswerB.Thefirstimageisasagittalviewofthefetalspineshowinganopenspinaldysraphismor

neuraltubedefectfromtheL2toS1levels.Thesecondimageisacoronalviewofthelumbosacralneural tube defect with fluid sac containing spinal elements. These findings are concerning for myelomeningocele.Becausealmostallfetuses withanopenspinalneuraltube defectarefoundtohave ChiariIImalformation,furtherimagingofthefetalbrainisrecommended.

2b.  Answer D.  Axial views of the fetal head demonstratea smallposterior fossa, indentation of the

frontalbones,andenlargedlateralventricles.(Fetalventriculomegalyisdefinedas>10mmwidthacross theatriaoftheposteriororanteriorhornoflateralventriclesatanygestationalage.Thelateralventricles in this example measure 12.1 and 12.8 mm, respectively, which is considered moderate ventriculomegaly).

ChiariIImalformation(alsoknownasArnold-Chiarimalformation),isacongenitalmalformationof thespineandposteriorfossa.Itischaracterizedbymyelomeningocele,smallposteriorfossawithdescent of the brainstem and cerebellar tonsils, and ventriculomegaly. It is essential to obtain sagittal and transverseviewsofthefetalspineonantenatalimagingtoevaluateforneuraltubedefects.

SonographicfindingsseeninChiariIImalformationincludethelemonsignandthebananacerebellum sign. Thelemon sign istheindentation/concavedeformityofthefrontal bones. The bananacerebellum signdescribestheabnormalcurvedshapeofthecerebellarhemispheresthatiscausedbyobliterationof thecisternamagnaduetodownwardshiftingoftheposteriorfossastructures.

Meckel-Grubersyndrome(choiceA)isalethalautosomalrecessivedisordercharacterizedbycystic dysplastic kidneys, occipital encephalocele, and postaxial polydactyly. In Dandy-Walker malformation

(choiceB),thefourthventricleisdilatedandcommunicateswiththecisternamagna.Theposteriorfossa isenlarged,andthetentoriumiselevated.ThemajorcongenitalanomaliesinDownsyndrome/Trisomy21 (choice C) include ventriculoseptal defect, atrioventricular septal defects, duodenal atresia, and ventriculomegaly.

References:HertzbergBS,MiddletonWD.Ultrasound:therequisites,3rded.Philadelphia,PA:Elsevier,2016:375–377.

RumackCM,WilsonSR,CharboneauWJ.Diagnosticultrasound,4thed.Philadelphia,PA:ElsevierHealthSciences,2011:1133,1257,1365.

WoodwardPJ.DiagnosticImaging:Obstetrics,2nded.SaltLakeCity,UT:Amirsys,2011.

3a. AnswerB. The axial images at the level of the fetal chest(4-chamber heartviews) demonstrate

rounded, echogenic soft tissue masses occupying the left atrium and left ventricle (the most posterior chamber of a normal fetal heart is the left atrium). The calipers in the second image correspondto a pericardialeffusion.

Thethreemostcommonfetalcardiacmassesarerhabdomyoma(58%),teratoma(19%),andfibroma (12%).Rhabdomyoma is a benignmyocardial tumor(hamartoma).Mostofthesetumorsare diagnosed beforetheageof1yearandareoftenmultiple.Inmostcases,theselesionsregressspontaneouslyandno treatmentisrequired.Hemodynamicallysignificantlesionscanresultincongestiveheartfailure,hydrops, pericardialeffusion, andarrhythmiasbyobstructingoutflowtracts or AV valves.Theyrespond well to surgicalexcision.

3b.  Answer D.  There is well-known association of cardiac rhabdomyomas with tuberous sclerosis.

Greaterthan50%ofpatientswithcardiacrhabdomyomasarefoundtohavetuberoussclerosis.Although tuberoussclerosis hasmanyother manifestationsin adultpatients, theyarerareinfetallife,exceptfor subependymaltubersinthebrain.

References:HertzbergBS,MiddletonWD.Ultrasound:therequisites,3rded.Philadelphia,PA:Elsevier,2016:397.

RumackCM,WilsonSR,CharboneauWJ.Diagnosticultrasound,4thed.Philadelphia,PA:ElsevierHealthSciences,2011:1317–1318.

WoodwardPJ.DiagnosticImaging:Obstetrics,2nded.SaltLakeCity,UT:Amirsys,2011.

4.AnswerA.Ultrasoundimagesshowahyperechoic,wedge-shaped,solidmass, nearthebaseofthe

leftlung.Dopplerinterrogationdemonstratesanarteryarisingfromtheaortaandsupplyingbloodflowto themass.Theseimagingfindingsaremostconsistentwithabronchopulmonarysequestration.

Bronchopulmonary(BP)sequestrationisacongenitallungmalformationinwhichnonfunctioninglung tissueis presentwithout communication to the tracheobronchial tree. Bronchopulmonarysequestrations receivevascular supplyfrom asystemicartery arisingeither fromthe thoracicorabdominalaorta.The two main types of bronchopulmonary sequestrations are intralobar and extralobar. Intralobar sequestration shares pleura with the normal lung, and extralobar sequestration is covered by its own separatepleura.Sequestrationsareoftenfoundinthelowerlobesandaremorecommonontheleft.

Bronchopulmonary sequestrations are not supplied by pulmonary artery(choiceB). Anylargechest mass,includingalargeBPsequestration,canresultinmediastinalshift.Thepresenceorabsenceofthis findingdoesnotaidindistinguishingitfromotherlesions(choicesCandD).

Reference:HertzbergBS,MiddletonWD.Ultrasound:therequisites,3rded.Philadelphia,PA:Elsevier,2016:404.

5a. AnswerB. Ultrasoundimages ofthefetal thorax at 18 weeks of gestationalage show echogenic

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