1. 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

2. Fetal cırculatory system

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

4. upvs

5. UPVS – COLOR DOPPLER

6. UPVS – COLOR DOPPLER

7. UPVS – COLOR DOPPLER

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

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

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

11. 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)

12. 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

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

14. 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)

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

16. 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

17. 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

18. CASE – SONOGRAPHIC FINDINGS
Dilated umbilical vein

19. CASE – SONOGRAPHIC FINDINGS
Atypical artery twisting around the UV

20. CASE – SONOGRAPHIC FINDINGS
Atypical artery
Dilated part of UV
Aorta

21. 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

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

23. 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:

24. 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)

25. 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