ATYPICAL VASCULAR COMMUNICATION BETWEEN INTRA-ABDOMINAL AORTA AND UMBILICAL VEIN IN A FETUS WITH DOWN SYNDROME: A CASE REPORT DR BURCU AYKAN YÜKSEL ANTALYA TRAINING AND RESEARCH HOSPITAL DEPARTMENT OF OBSTETRICS & GYNECOLOGY

Fetal cırculatory system

Fetal umbılıcal-portal venous system (upvs) UMBILICAL VEIN + PORTAL SYSTEM DUCTUS VENOSUS

upvs

UPVS – COLOR DOPPLER

UPVS – COLOR DOPPLER

UPVS – COLOR DOPPLER

ANOMALIES OF UPVS Anomalies of the umbilical cord Anomalies of portal venous system Anomalies of the ductus venosus

Venous anomalıes of the umbılıcal cord Four –vessel cord (two arteries-two veins) (persistent right umbilical vein)

Venous anomalıes of the umbılıcal cord Umbilical cord varix (Abnormal dilatation of the umbilical vein)

Congenıtal agenesıs of the portal venous system (capvs) COMPLETE AGENESIS There is complete diversion of portal blood into the inferior vena cava (portosystemic shunt) PARTIAL AGENESIS Any portal vein (+) but a certain amount of portal blood is diverted into the systemic venous circulation (portohepatic shunt)

CAPVS COMPLETE AGENESIS Complete failure of the vitelline veins to anastomose with the hepatic sinusoids Portal veins do not exist Possible liver problems later in life

CAPVS PARTIAL AGENESIS Right or left portal vein is present. Pregnancies have almost normal outcome but the neonate should be observed meticulously

DUCTUS VENOSUS AGENESIS The drainage of the umbilical blood may be * extrahepatic (IVC, iliac vein, renal vein, right atrium) or * intrahepatic ( portal sinus as usual without giving rise to ductus venosus)

Two seperate ductus venosus or in the form of duplication Two Ductus venosus Two seperate ductus venosus or in the form of duplication

case 27 years old G1 Medical and family history unremarkable Abnormal double test result Amniocentesis at 16 weeks (result trisomy 21) Family did not accept termination of pregnancy

Case – sonographIC FINDINGS Biometric measurements consistent with LMP Umbilical cord: two arteries- one vein with normal Doppler measurements Fetal echocardiogram showed normal cardiac anatomy Examination of UPVS: An atypical vessel emerging directly from the abdominal aorta turned around the UV like a helix before entering the vein. The high velocity flow in the atypical vessel has caused a pseudodiverticle below the point where the vessel entered the vein

CASE – SONOGRAPHIC FINDINGS Dilated umbilical vein

CASE – SONOGRAPHIC FINDINGS Atypical artery twisting around the UV

CASE – SONOGRAPHIC FINDINGS Atypical artery Dilated part of UV Aorta

case The patient was under follow-up until 36 weeks when her contractions started Because of late decelerations at the beginning of labor, she underwent C/S An otherwise healthy baby of 2600 gr was delivered and taken under observation by the pediatricians for further examination

Case - postpartum mrı Dilated part of UV Abdominal aorta Atypical artery

dıscussıon Second case First case the only two cases wıth arterıo-venous fıstula of upvs diagnosed prenatally ın the lıterature (hartung et al.) 11st First case Fetal hydrops at 14 weeks Fistula between umbilical vein and an atypical artery emerging directly from the aorta CVS Trisomy 21 Spontaneous abortion at 16 weeks Second case Fetal hydrops +polyhydramnios at 31 weeks Fistula between umbilical vein and hepatic artery, between epigastric artery and hepatic artery and between another atypical artery emerging from coeliac truncus and umbilical vein Cordocentesis  Trisomy 21 Fetus stillborn at 36 weeks Hartung J, Cahoui R, Kalache K, Tennstedt C, Bollmann R. Prenatal diagnosis of intrahepatic communications of the umbilical vein with atypical arteries (A-V fistulae) in two cases of trisomy 21 using color Doppler ultrasound. Ultrasound Obstet Gynecol 2000;16: 271-74.

dıscussıon Venous or veno-venous anomalies of the umbilical-portal venous system are significantly more common than arteriovenous fistula in Down Syndrome cases (especially abnormal insertion of the umbilical vein into the inferior vena cava) So a question arises; Are arteriovenous fistula really very very rare? Or, Maybe they are not rare but pregnancies with this anomaly may be resulting in stillbirths or are being terminated because of the severity of the associated malformation or the abnormal karyotype (hence the anomaly is overlooked)

conclusıon UPVS anomalies may occur with or without abnormal karyotype. Careful examination of umbilical vein and the ductus venosus in every patient with color Doppler will reveal more cases in the future