Evaluation of the type of fetal umblical-portal anastomosis in late-onset fetal growth restriction: A case-control study.

PURPOSE: To evaluate the type of umbilical-portal anastomosis in late-onset fetal growth restriction (LO-FGR) and appropriate for gestational age (AGA) fetuses. To investigate the impact of the type of umbilical-portal anastomosis on the adverse outcomes in LO-FGR. METHOD: This study observed 150 pregnancies with AGA fetuses and 62 pregnancies with fetuses with LO-FGR. In each case, the point of reference for measuring the abdominal circumference was established. The type of umbilical-portal anastomosis was evaluated as T-shaped, X-shaped, and H-shaped according to the shape of main portal vein and portal sinus. Incidences of the type of umbilical-portal anastomosis in AGA and LO-FGR fetuses were evaluated. RESULTS: T-shaped anastomosis was the most common (56.7%) in the AGA group and X-shaped (66.1%) in the LO-FGR group. In LO-FGR, T-shape anastomosis was significantly lower and X-shape anastomosis was significantly higher than AGA (p < 0.001). X-shaped anastomosis was associated with LO-FGR and the RR was 2.3 (95% CI 1.5-3.6; p < 0.001). Incidences of admission to NICU and emergency C/S for fetal distress were higher in fetuses with X -shaped anastomosis in the LO-FGR (p < 0.05). CONCLUSION: X-shaped umbilical-portal anastomosis have a prognostic significance in LO-FGR fetuses.

First trimester genetic sonogram for screening fetal Down syndrome: A population-based study.

OBJECTIVE: To evaluate the performance of first trimester sonomarkers in the detection of fetal Down syndrome among Thai pregnant women. MATERIALS AND METHODS: Pregnant women at 11-13+6 weeks' gestation underwent ultrasound examination for assessment of nuchal translucency (NT), nasal bone (NB), tricuspid regurgitation (TR), and abnormal ductus venosus (aDV) Doppler waveforms. The women were followed up for final outcomes. Fetal abnormalities other than trisomy 21 were excluded. The performances of each sonomarker and their combinations in predicting fetal Down syndrome were calculated. RESULTS: A total of 7820 pregnant women meeting the inclusion criteria were available for analysis, including 20 cases with fetal Down syndrome and 7800 unaffected cases. Of the four sonomarkers, NT, as a single sonomarker, had the highest detection rate (55.0% at a false positive rate of about 5%), whereas the remaining single sonomarkers had low detection rate (15-20%). The combination of all sonomarkers had the highest detection rate of 70% but the false positive rate was as high as 10.8%. The combination of NT and NB had a detection rate of 60% with an acceptable false positive rate of 6.9%, whereas the other combinations yielded relatively high false positive rates. CONCLUSION: The first trimester genetic sonogram in screening for Down syndrome among Asian women is acceptably effective and may be offered to some selected groups of the population. NT is the best sonomarker with a detection rate of 55% at 5% false positive rate and its combination with NB can improve performance with minimal increase in false positive rate.

Normal Fetal Umbilical, Portal, and Hepatic Venous System: Four-dimensional STIC Rendering.

Online supplemental material is available for this article.

Diagnosing Small for Gestational Age during second trimester routine screening: Early sonographic clues.

OBJECTIVE: Small for gestational age (SGA) is generally defined as birth weight being at or below the 10th percentile. Children with SGA have a higher risk for complications. There is a need for early predictors, as the accurate diagnosis rate is only 50%. In the current study, we aimed to evaluate diagnostic performance of ultrasound (US)/color Doppler ultrasound (CDUS) parameters (umbilical vein-UV, right portal vein-RPV diameter/flow rate, and portal sinus-PS diameter) examined at 20-22 gestational week as SGA diagnostic factors. MATERIALS AND METHODS: 93 pregnant included (32 SGA, 61 controls). All the US examinations were performed between 20 and 22 weeks of gestation. UV, RPV, and PS measurements were performed by using the same image acquired for abdominal circumference measurement. A fetus with as estimated fetal weight (EFW) below the 10th percentile was diagnosed as SGA and SGA at birth was defined as having a birth weight under the 10th percentile. RESULTS: Pregnant women in the SGA group were significantly older (30 ± 4.8 vs. 26.6 ± 5.4 years, p < 0.01). Median UV diameter was significantly lower in SGA group (2.20 vs. 2.40 mm, p = 0.001). Median RPV diameter was significantly lower in SGA group (2 vs. 2.10 mm, p = 0.018). Median PS diameter was significantly lower in SGA group (2 vs. 20.10 mm, p = 0.008). CONCLUSION: UV, RPV, and PS diameters can be earlier predictors for SGA diagnosis. Routinely evaluation of these parameters during second trimester screening can increase SGA diagnosis rates and serve for early diagnose.

Fetal case of right atrial isomerism with infracardiac total anomalous pulmonary venous connection and agenesis of the ductus venosus.

After birth, the ductus venosus becomes an important route connecting the pulmonary and systemic venous systems for survival in infracardiac total anomalous pulmonary venous connection. We encountered a fetal case of right atrial isomerism with infracardiac total anomalous pulmonary venous connection and agenesis of ductus venosus. Prenatal echocardiography suggested that the fetus had severe pulmonary venous obstruction; however, no obstructive lesions were detected at the level of the vertical vein that drained into the portal veins. Therefore, we concluded that emergency surgical pulmonary venous obstruction release was the only way for the fetus to survive. However, the saturation level was maintained above 70% due to the abundant communications via the hepatic sinusoid over 1 week after birth. In conclusion, hepatic sinusoids can be a sufficient route for pulmonary venous return and may not cause severe pulmonary venous obstruction in infracardiac total anomalous pulmonary venous connection with agenesis of ductus venosus.

The types of connections between the portal sinus and main portal vein in foetuses / Tipos de conexiones entre el seno portal y la vena porta principal en fetos

In the prenatal period, the three types of connections between the portal sinus and main portal vein have been published in the literature: T, X and H-shaped. The T type is the most frequent in the literature, and the aim of our study is to define the percentage of the connection types during the prenatal period in our population. In this prospective study, 237 women between 20 and 38 weeks of pregnancy without a foetal anomaly or pregnancy-related complications were included, and the precordial veins of the foetuses were examined using a wide-band color Doppler technique. The types of connections were determined by two specialists according to the shape of the colour coded vessels in Doppler examinations. The criteria of Czubalski & Aleksandrowicz (2000) were used. All of the connection types in patients were confirmed using video clips and were stored in the picture archiving and communication system. In 237 patients, the types of connection were determined by the first specialist as 189 foetuses (79.7 %) with the X-shaped or side-to-side connection, 16 foetuses (6.8 %) with the T-shaped or end-to-side type and 32 foetuses (13.5 %) with the H-shaped or parallel-coursed vessels connected with a short segment. The most common types of connections between the portal sinus and main portal vein in foetuses are X shaped or side-to-side, which is contrary to previous studies.

En el período prenatal, se han publicado en la literatura los tres tipos de conexiones entre el seno portal y la vena porta principal: en forma de T, X y H. El tipo T es el más frecuente, y el objetivo de nuestro estudio fue definir el porcentaje de tipos de conexión durante el período prenatal en nuestra población. En este estudio prospectivo, se incluyeron 237 mujeres entre 20 y 38 semanas de embarazo, sin anomalías fetales o complicaciones relacionadas con el embarazo, y se examinaron las venas precordiales de los fetos utilizando una técnica Doppler de banda ancha. Los tipos de conexiones fueron determinados por dos especialistas según la forma de los vasos codificados por color en los exámenes Doppler. Se utilizaron los criterios del estudio de Czubalski & Aleksandrowicz. Todos los tipos de conexión en los pacientes se confirmaron mediante videoclips y se almacenaron en el sistema de comunicación y en archivo de imágenes. En 237 pacientes, el primer especialista determinó en 189 fetos (79,7 %) la conexión en forma de X o de lado a lado; en 16 fetos (6,8 %) la forma de T o Tipo de extremo a lado; y en 32 fetos (13,5 %) los vasos en forma de H o paralelos, conectados con un segmento corto. Los tipos más comunes de conexiones entre el seno portal y la vena porta principal en los fetos son en forma de X o de lado a lado, lo que es contrario a estudios anteriores.

Altered development of fetal liver perfusion in pregnancies with pregestational diabetes.

BACKGROUND: Pregestational diabetes is associated with fetal macrosomia, and umbilical perfusion of the fetal liver has a role in regulating fetal growth. We therefore hypothesized that pregestational diabetes alters fetal liver blood flow depending on degree of glycemic control. METHODS: In a prospective study, 49 women with pregestational diabetes underwent monthly ultrasound examinations during 24-36 gestational weeks. Blood flow was determined in the umbilical vein, ductus venosus and portal vein, and blood velocity was measured in the left portal vein, the latter reflecting the watershed between splanchnic and umbilical flow. The measurements were compared with reference values by z-score statistics, and the effect of HbA1c assessed. RESULTS: The umbilical venous flow to the liver (z-score 0.36, p = 0.002), total venous liver flow (z-score 0.51, p<0.001) and left portal vein blood velocity (z-score 0.64, p<0.001), were higher in the study group. Normalized portal venous flow was lower (z-score -0.42, p = 0.002), and normalized total venous liver flow tended to be lower after 30 gestational weeks (z-score -0.54, p = 0.047) in the diabetic pregnancies compared with reference values from a low-risk population. The left portal vein blood velocity was positively, and the portal fraction of total venous liver flow negatively correlated with first trimester HbA1C. CONCLUSIONS: In spite of increased umbilical blood distribution to the fetal liver, graded according to glycemic control, the total venous liver flow did not match third trimester fetal growth in pregnancies with pregestational diabetes, thus contributing towards increased perinatal risks and possibly altered liver function with long-term metabolic consequences.

Congenital portosystemic venous shunt.

Congenital portosystemic venous shunts are rare developmental anomalies resulting in diversion of portal flow to the systemic circulation and have been divided into extra- and intrahepatic shunts. They occur during liver and systemic venous vascular embryogenesis and are associated with other congenital abnormalities. They carry a higher risk of benign and malignant liver tumors and, if left untreated, can result in significant medical complications including systemic encephalopathy and pulmonary hypertension. CONCLUSION: This article reviews the various types of congenital portosystemic shunts and their anatomy, pathogenesis, symptomatology, and timing and options of treatment. What is Known: • The natural history and basic management of this rare congenital anomaly are presented. What is New: • This paper is a comprehensive review; highlights important topics in pathogenesis, clinical symptomatology, and treatment options; and proposes an algorithm in the management of congenital portosystemic shunt disease in order to provide a clear idea to a pediatrician. An effort has been made to emphasize the indications for treatment in the children population and link to the adult group by discussing the consequences of lack of treatment or delayed diagnosis.

Systematic examination of the fetal abdominal precordial veins: a cohort study.

OBJECTIVES: Examination of the fetal venous system is a necessary part of complete fetal organ scanning to confirm landmark anatomy, such as the ductus venosus and course of the umbilical veins, and, whenever cardiovascular anomalies are identified, to exclude associated anomalous development of the fetal veins. We aimed to develop a protocol for systematic examination of the fetal venous system during midtrimester targeted organ scanning. METHODS: We included low-risk women with a singleton fetus presenting between January 2011 and June 2013 to our center for routine midtrimester (20-24 weeks) targeted organ scanning. Imaging of the venous system was added to the booked scan and comprised two-dimensional color Doppler scanning of the fetal abdomen in three discrete planes, two transverse and one longitudinal. The more caudal plane was obtained in a ventral or lateral transverse abdominal plane to image the umbilical vein, left portal vein, portal sinus, anterior right portal vein, posterior right portal vein, main portal vein and splenic vein and artery. Moving cephalad, a ventral or lateral transverse plane was obtained to image the right, middle and left hepatic veins and inferior vena cava (IVC). Finally, a longitudinal anteroposterior plane showed the umbilical vein, ductus venosus, IVC and left hepatic vein. In some cases the pulsed Doppler waveform of a given target vessel was also examined. Three-dimensional/4D ultrasound was applied as necessary, when anomalous cases were encountered. RESULTS: We examined 1810 women. Their body mass index ranged from 19 to 40 (mean, 24.7). In 38 (2.1%) women, the target anatomy was not visualized satisfactorily owing to maternal body habitus. A T-shaped configuration of the portal system vessels was observed in 63% of cases, an X-shaped configuration in 25% and an H-shaped configuration in 12%. During the study period, 24 congenital anomalies of the precordial venous system were diagnosed: nine cases of persistent right umbilical vein, seven of agenesis of the ductus venosus, five of anomalous portal venous drainage and three of interrupted IVC with azygos continuation. CONCLUSIONS: Examination of the fetal venous system is feasible with the application of three abdominal planes. While a venous system scan is not practicable as part of a screening-level examination, mastery of the normal anatomy is an essential part of the professional knowledge base, in order to provide ready and complete scanning of the system in cases of suspected anomalies or disordered cardiac function.

Histological analyses demonstrate the temporary contribution of yolk sac, liver, and bone marrow to hematopoiesis during chicken development.

The use of avian animal models has contributed to the understanding of many aspects of the ontogeny of the hematopoietic system in vertebrates. However, specific events that occur in the model itself are still unclear. There is a lack of consensus, among previous studies, about which is the intermediate site responsible for expansion and differentiation of hematopoietic cells, and the liver's contribution to the development of this system. Here we aimed to evaluate the presence of hematopoiesis in the yolk sac and liver in chickens, from the stages of intra-aortic clusters in the aorta-genital ridges-mesonephros (AGM) region until hatching, and how it relates to the establishment of the bone marrow. Gallus gallus domesticus L. embryos and their respective yolk sacs at embryonic day 3 (E3) and up to E21 were collected and processed according to standard histological techniques for paraffin embedding. The slides were stained with hematoxylin-eosin, Lennert's Giemsa, and Sirius Red at pH 10.2, and investigated by light microscopy. This study demonstrated that the yolk sac was a unique hematopoietic site between E4 and E12. Hematopoiesis occurred in the yolk sac and bone marrow between E13 and E20. The liver showed granulocytic differentiation in the connective tissue of portal spaces at E15 and onwards. The yolk sac showed expansion of erythrocytic and granulocytic lineages from E6 to E19, and E7 to E20, respectively. The results suggest that the yolk sac is the major intermediate erythropoietic and granulopoietic site where expansion and differentiation occur during chicken development. The hepatic hematopoiesis is restricted to the portal spaces and represented by the granulocytic lineage.

Prehepatic portal hypertension with aneurysm of the portal vein: unusual but treatable malformative pattern.

INTRODUCTION: Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. METHODS: Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. RESULTS: Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. CONCLUSIONS: These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.

Fetal venous circulation in monochorionic twin pregnancies with placental insufficiency: prediction of acidemia at birth or intrauterine fetal death.

OBJECTIVES: To investigate fetal venous Doppler measurements in monochorionic twin pregnancies complicated by placental insufficiency and the relationship between fetal venous flow and acidemia at birth or intrauterine fetal death. METHODS: This was a prospective study of 18 monochorionic twin pregnancies with placental insufficiency. Inclusion criteria were monochorionic-diamniotic twin pregnancy, abnormal umbilical artery (UA) Doppler indices, intact membranes and absence of fetal congenital abnormalities. Cases of twin-to-twin transfusion syndrome were excluded. The following Doppler measurements were studied: UA pulsatility index (PI), ductus venosus PI, middle cerebral artery PI and peak systolic velocity, intra-abdominal umbilical vein (UV) time-averaged maximum velocity (TAMXV) and left portal vein (LPV) TAMXV. Doppler parameters were transformed into Z-scores (SD values from the mean) or multiples of the median according to normative references. RESULTS: UA pH < 7.20 occurred in nine (25.0%) neonates, pH < 7.15 in four (11.1%) and intrauterine death in four (11.1%) fetuses. The UV-TAMXV and LPV-TAMXV Z-scores were significantly lower in the group with pH < 7.20 or intrauterine fetal death (-1.79 vs -1.22, P = 0.006 and -2.26 vs -1.13, P = 0.04, respectively). In cases with pH < 7.15 or intrauterine fetal death, UV pulsations were more frequent (50.0% vs 10.7%, P = 0.03) and UV-TAMXV Z-score was significantly lower (-1.89 vs -1.26, P = 0.003). Mixed effects logistic regression analysis, accounting for the paired nature of the outcomes for the two twins in each pregnancy, demonstrated that the UV-TAMXV Z-score significantly predicted UA pH at birth < 7.20 or intrauterine fetal death. The Doppler parameter that independently predicted pH < 7.15 or intrauterine fetal death was presence of pulsation in the UV. CONCLUSION: UV Doppler parameters may predict acidemia at birth or intrauterine fetal death in monochorionic twins complicated by placental insufficiency.

Cardiovascular transition to extrauterine life in growth-restricted neonates: relationship with prenatal Doppler findings.

OBJECTIVE: Cardiovascular status in fetal growth restriction (FGR) can be classified by the severity of individual Doppler abnormalities (early and late) or by the rate of clinical progression. We tested the hypothesis that aspects of the fetal cardiovascular status in FGR affect neonatal cardiovascular findings. STUDY DESIGN: FGR cases [abdominal circumference <5th percentile and an elevated umbilical (MCA) artery (UA) pulsatility index] had UA, middle cerebral artery and ductus venosus (DV) Doppler. Positive UA end-diastolic velocity and/or a low MCA pulsatility index denoted early and absent/reversed UA end-diastolic velocity, whereas an increased DV pulsatility index for veins denoted late responses. The rate of progression was classified into mild, progressive and severe. After delivery, shunt dynamics and blood flow across the patent ductus arteriosus (PDA), foramen ovale and atriaventricular valves, myocardial contractility and pharmacologic pressor requirement were noted at neonatal echocardiography. These findings were related to prenatal Doppler parameters. RESULTS: In 94 patients, only individual Doppler parameters related to neonatal echocardiographic findings. Absent/reversed UA DV significantly predicted PDA with right to left shunt (p = 0.016). The pressor need for cardiovascular instability was observed in neonates with abnormal prenatal DV Doppler and with lower birth weights delivered at earlier gestational age (p < 0.0001 for both). Pressor need was significantly related to neonatal death (Nagelkerke R² = 0.35, p = 0.002). CONCLUSION: A markedly abnormal UA Doppler predisposes growth-restricted neonates to persistence of fetal circulation associated with right to left shunting. Abnormal venous Doppler is a risk factor for cardiovascular instability which in turn significantly contributes to neonatal mortality. Further clarification of the neonatal cardiovascular transition may be helpful in guiding early neonatal assessment and management.

Missing ductus venosus: a case report.

BACKGROUND: The ductus venosus is a short vessel, present in the newborn infant on the dorsal surface of the liver, connecting the portal and umbilical circulation with the inferior vena cava. Agenesis of the duct is a rare anomaly. CASE: A 28-year-old woman was referred to our department for the first trimester ultrasound evaluation. Detailed scanning revealed agenesis of the duct. Fetal echocardiography showed cardiac disproportion at the level of the ventricles. CONCLUSION: Agenesis of the duct can be related to either cardiac or congenital abnormalities.

Updated reference ranges for the ductus venosus pulsatility index at 11-13 weeks.

OBJECTIVE: To update the reference ranges for the ductus venosus pulsatility index (DVPI) at 11+0 to 13+6 gestational weeks. METHODS: DVPI was calculated in 14,444 singleton fetuses at 11+0 to 13+6 weeks in two Fetal Medicine Centers, during a 4-year period. Using previously described medians, DVPI evolution was assessed both over the study period on a yearly basis and over gestation, grouping fetuses according to 5-mm crown-rump length (CRL) ranges. Weighted DVPI medians, the 5th and 95th percentiles and distribution parameters for unaffected and trisomy 21 fetuses were newly calculated. RESULTS: A significant DVPI multiple of the median decrease was observed over both the study period (p < 0.01) and over gestation (p < 0.01) using previous medians, in the two centers. Newly calculated weighted medians were lower than those previously described, decreasing with CRL. Distribution parameters calculated using the new medians were different from those previously described. CONCLUSION: DVPI reference ranges were lower than those previously reported and decreased with CRL. Updated medians and distribution parameters should be considered to include the DVPI as a Gaussian marker in trisomy 21 screening and for quality control purposes.

Evolution of congenital malformations of the umbilical-portal-hepatic venous system.

OBJECTIVE: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. MATERIALS AND METHODS: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. RESULTS: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. CONCLUSION: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.