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Transcript
 15thWorldCongressinFetalMedicine
Acaseofpersistenthyperplasticprimaryvitreous
DağlarHK,TürkmenGG,SanhalCY,KirbasA,TimurH,KaraÖ,YılmazZ,DanismanN
ZekaiTahirBurakMaternityHospital,Ankara,Turkey
Objective
Persistenthyperplasticprimaryvitreousalsoknownaspersistentfetalvasculatureisararedevelopmentalmalformationoftheeyethatresultfromfailureofinvolutionofthe
hyaloidartery.Hyaloidarterycanbeseenonultrasonographicexaminationuptothe30weeksofgestationalage.Thisisthetimethatconversionoftheprimaryvitreousto
maturesecondaryvitreousiscompleted.
Methods
It can be present in three forms: purely anterior, purely posterior and a combination of both.Approximately 90% of cases are unilateral and sporadic but also autosomal
recessivepatternofinharitancehasbeenreported.
Results
A 25-year-old primigravida woman was referred to our department at 22 weeks' gestation for ultrasound evaluation because of ventriculomegaly on routine ultrasound
examination.Hermedicalhistorywasunremarkableexceptconsanguinity.Shewasmarriedwithherfirstcousin.Downsyndromescreeningtestshadnotbeenperformed.
TORCHprofilewasnormal.Ourultrasoundexaminationrevealedsevereventriculomegaly.Amniocentesiswasoffered;buttheparentdeclinedanyfurtherevalution.At37
weeksshewasreevaluatedviaultrasoundexamination.Hydrocephalywaspresent.Examinationoftheeyeshowedpersistenthyperplasticvitreous.Thebabywasdelivered
atterm.Sixhoursafterdeliverythebabydied.
Conclusion
Hyaloid artery should not be identified on ultrasound examination past 30 weeks gestation. Bilateral persistent hyperplastic primary vitreous is frequently associated with
trisomysyndromesandotherformsofabnormalbraindevelopment.Whenabrainabnormlitiyisdetected,fetaleyesshouldbeexaminedthoroughly.