Are multiple projections necessary when assessing umbilical venous catheter placement in neonates? A retrospective study.

INTRODUCTION: There is variability in clinical practice regarding the number of radiographic views required to accurately determine umbilical venous catheter (UVC) tip position. Some units prefer performing a single anteroposterior (AP) view and others do both AP and lateral views. The aim of this study was to compare the need for one versus two radiographical views to accurately determine UVC tip position. METHODS: Radiographs of 382 infants (all gestations) from two level six neonatal units were included in the study. Patients with congenital anomalies and those who had adjustment of UVC position between AP and lateral films being performed were excluded. Six clinicians reviewed anonymized AP only images and documented UVC tip position. Subsequently, they reviewed both AP and lateral views and again documented the UVC tip position. Results were compared to the expert consensus which was taken from the consensus of two paediatric radiologists. RESULTS: The study population had a mean gestational age of 32.8 weeks and birth weight of 2190 g, with 58% males. The UVC tip positions were accurately determined 76% times by the AP view alone and 82% times by using both the AP and lateral views (P < 0.001). The low placed UVC tip position which is the most inappropriate for use was more accurately determined by two images (78% times) rather than single image (70% times) (P < 0.001). CONCLUSIONS: Utilising both AP and lateral views was superior in accurately determining UVC tip position to AP view alone.

Isolated fetal umbilical vein varix and the association with intrauterine fetal death and fetal growth restriction: A systematic review, meta-analysis, and nested retrospective cohort study.

OBJECTIVES: To assess the risk of intrauterine fetal death (IUFD) and fetal growth restriction (FGR) in fetuses with an isolated fetal intra-abdominal umbilical vein varix (i-FIUVV). METHODS: A retrospective cohort study combined with a systematic review and meta-analysis of the literature was performed. In the retrospective cohort study, all singleton fetuses with an i-FIUVV in the fetal medicine units of the Amsterdam UMC (between 2007 and 2023) were analyzed. The primary outcome measures were IUFD and FGR. The sample proportions of IUFD and FGR were depicted as risk percentages. The IUFD proportion was compared to the regional reference population and the FGR proportion was compared to the reported proportions in Europe. The secondary outcome measures were gestational age at diagnosis, initial and maximal FIUVV diameter, fetal monitoring in pregnancy, turbulent flow in the varix, thrombus formation in the varix, induction of labor, gestational age at birth, and birthweight centile. The proportion of fetuses with a birthweight below the 10th centile was compared with that of the regional reference population. The systematic review included all cases from eligible literature published between 2007 and 2023 supplemented by the data of our retrospective cohort study. In the systematic review and meta-analysis, the pooled proportions of IUFD and FGR were assessed in fetuses with i-FIUVV. RESULTS: The retrospective cohort included 43 singletons with an i-FIUVV. The IUFD risk was 0% [Confidence Interval, CI: 0%-8.2%], which did not differ significantly from 0.3% in the reference population, p = 1.0. The risk of FGR was 16.3% [CI: 6.8%-30.7%] in the studied population, which is higher than the reported incidence of FGR in Europe ranging from 5%-10%. The proportion of fetuses with birthweights below the 10th centile was higher in our cohort compared with the reference population (23.3 vs. 9.9%, p < 0.01). The systematic review included 12 articles, three abstracts, and our current cohort. In total, 513 cases with an i-FIUVV were included. The pooled risk was 0.4% [CI: 0.1%-1.7%] for IUFD and 5.2% [CI: 1.1%-21.3%] for FGR. The mean gestational age at birth did not exceed 39 weeks in neither the cohort (38.7 weeks) nor the pooled literature (37.6 weeks). CONCLUSION: An i-FIUVV in singletons is not associated with an increased IUFD risk up to 39 weeks of gestation but is possibly associated with FGR. The incidence of FGR in our cohort was higher than in the pooled literature (16.3% vs. 5%) but FGR definitions in the included studies varied. The proportion of birthweights below the 10th percentile in our cohort was significantly higher than in the reference group. Thus, based on these findings, we suggest conducting sonographic growth assessments while simultaneously assessing the i-FIUVV. No further monitoring and follow-up are indicated up to 39 weeks of gestation. After 39 weeks of gestation, data on fetuses with i-FIUVV and their outcomes are lacking.

In-utero evaluation of the fetal umbilical-portal venous system among fetuses with persistent right umbilical vein: Two-and three-dimensional ultrasonographic study.

OBJECTIVE: The aim of this study was to describe the anatomy of the portal system in fetuses with persistent right umbilical vein (PRUV). METHODS: Prospective observational study of fetuses diagnosed with PRUV. All patients underwent a comprehensive portal system anatomy scan supplemented by two-dimensional (2D) and three-dimensional (3D) color doppler modalities. RESULTS: 29 fetuses with PRUV were studied. We perceived an identical anatomical pattern in 28 fetuses. The right umbilical vein drains to the portal sinus (future right portal vein, RPV), which has a configuration of a left portal vein (LPV) in the normal left portal system, with three emerging branches: inferior (RPVi), medial (RPVm) and superior (RPVs). The RPV then courses to the left, towards the stomach to the point of the bifurcation of the main portal vein (MPV) to become the left portal vein. The LPV has an RPV configuration in a normal portal system with anterior (LPVa) and posterior (RPVp) branches. This anatomical layout mimics a mirror image of the normal anatomy of left portal system. CONCLUSION: PRUV has unique umbilical portal venous anatomy, which is a mirror image of the normal left portal system. It can be demonstrated prenatally and serve as an additional tool for prenatal diagnosis of PRUV.

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.

Effects of umbilical vein flow on midbrain growth and cortical development in late onset fetal growth restricted fetuses: a prospective cross-sectional study.

OBJECTIVES: To investigate midbrain growth, including corpus callusum (CC) and cerebellar vermis (CV) and cortical development in late fetal growth restricted (FGR) subclassified according to the umbilical vein blood flow (UVBF) values. METHODS: This was a prospective study on singleton fetuses late FGR with abnormal placental cerebral ratio (PCR). FGR fetuses were further subdivided into normal (≥fifth centile) and abnormal (

Absence of Ductus Venosus: A Comparison of 2 Distinctive Fetal Autopsy Cases and Embryologic Perspectives.

In fetal circulation, oxygenated blood from the placenta flows through the umbilical vein into the ductus venosus (DV), then enters the inferior vena cava, and subsequently reaches the right atrium of the heart. The DV serves as a shunt, allowing this oxygen-rich blood to bypass the liver. The absence of the DV (ADV), also known as agenesis of the DV, is a rare congenital anomaly. Without a DV, blood from the umbilical vein must follow alternative routes to the heart. In ADV cases, blood from the umbilical vein must follow 1 of 2 primary drainage patterns: either an extrahepatic shunt or an intrahepatic shunt. This report details the antenatal ultrasound and postmortem findings of 2 fetuses diagnosed with ADV by prenatal imaging studies. The first case involved a fetus with a persistent right umbilical vein connected directly to the suprahepatic IVC, accompanied by early obliteration of the left umbilical vein and true agenesis of the DV. This fetus also had additional congenital anomalies. In contrast, the second case involved a fetus with a normal left umbilical vein that entered the liver. However, despite an ultrasound diagnosis of "absence" of the DV, a DV was present, though markedly hypoplastic and probably minimally functional or non-functional. In this case, blood from the umbilical vein likely followed an alternate intrahepatic route through the portal and hepatic veins, before reaching the heart (intrahepatic shunt). These contrasting cases emphasize the heterogeneity of vascular anomalies and embryologic origins captured by the term "ADV." Additionally, the terminology of "absence" or "agenesis" may be misleading in some purported ADV cases. Specifically, in the second case, the DV was not absent; it was markedly hypoplastic instead. This also appears to be the first reported case of a hypoplastic DV in a fetus. Both cases underscore the importance of effective collaboration and clear communication between maternal-fetal medicine specialists and pathologists.

Ultrasonographic classification of 26 cases of fetal umbilical-portal-systemic venous shunts and the correlations with fetal chromosomal abnormalities.

OBJECTIVE: To investigate the ultrasonographic classification of fetal umbilical-portal-systemic venous shunts (UPSVS) and the correlations with fetal chromosomal abnormalities. METHODS: We retrospectively analyzed the ultrasound characteristics and the corresponding chromosomal abnormalities of 26 cases of fetal UPSVS prenatally diagnosed. RESULTS: A total of 26 fetuses diagnosed as UPSVS were included, including four cases of type I UPSVS, ten of type II, three of type IIIA, and nine of type IIIB. Four cases of type I were all complicated by fetal heart enlargement and heart insufficiency, of which one case had multiple malformations, and all four cases terminated pregnancies. Six of ten cases of type II terminated pregnancies, including four of Down's syndrome, one of twin reversed arterial perfusion sequence, one of fetal edema but with normal copy number variation (CNV) by chorionic villus sampling. The other four of ten cases were isolated type II with normal chromosomes, which were delivered at full term and were normal in growth and development when followed up 34 months after birth. Three cases of type IIIA all terminated pregnancies, of which one had multiple malformations, one had right multicystic dysplastic kidney, and one had fetal heart enlargement and heart failure. Among nine of type IIIB, seven with chromosomal abnormalities and/ or complicated malformations terminated pregnancies, and two with isolated type IIIB and normal chromosomes were delivered at full term, and were normal in growth and development (one was followed up to 33 months after birth and the other 20 months after birth). CONCLUSION: Fetal UPSVS can be clearly diagnosed and typed by prenatal ultrasonography. Fetal prognosis is determined by the types of UPSVS and complicated malformations and/ or chromosomal abnormalities. The probability of fetal chromosomal abnormalities in UPSVS fetuses is related to the ultrasonographic classification.

Foetal death due to extensive extra-abdominal umbilical vein Varix with umbilical vein thrombosis: a case report.

BACKGROUND: Foetal anaemia and umbilical vein thrombosis are rare pregnancy complications that can increase the risk of perinatal adverse events, which, in severe cases, can lead to foetal death. During pregnancy, umbilical vein varix (UVV) commonly occurs in the intra-abdominal part of the umbilical vein and is associated with an increased risk of foetal anaemia and umbilical vein thrombosis. However, UVV occurring in the extra-abdominal part of the umbilical vein is rare, especially when accompanied by thrombosis. In this case report, we describe a rare case of an extensive extra-abdominal umbilical vein varix (EAUVV), which ultimately resulted in foetal death due to umbilical vein thrombosis. CASE PRESENTATION: In this report, we describe a rare case of an extensive EAUVV that was discovered at 25 weeks and 3 days of gestation. During the examination, there were no abnormalities in foetal haemodynamics. The estimated weight of the foetus was only 709 g. In addition to refusing to be hospitalized, the patient refused close monitoring of the foetus. As a result, we were limited to choosing an expectant therapy. The foetus died 2 weeks after diagnosis and was confirmed to have EAUVV with thrombosis after the induction of labour. CONCLUSION: In the case of EAUVV, lesions are extremely rare, and it is very easy for thrombosis to form, which may result in the death of the child. When determining the next step in the treatment of the condition, the degree of UVV, possible complications, gestational age, foetal haemodynamics, and other relevant factors are strongly connected to the clinical therapy decision, and these factors should be considered comprehensively when making a clinical decision. We recommend close monitoring with hospital admission (to facilities capable of handling extremely preterm foetuses) after variability in delivery for worsening haemodynamic status.

Umbilical vein blood flow: State-of-the-art.

Placental blood supply to the fetus can be measured by evaluating the umbilical vein blood flow. Despite its potential application in healthcare, the umbilical vein blood flow volume is still used only in research setting. One of the reasons is a concern regarding its reproducibility, partly due to technology issues. Nowadays, technology improvements make this evaluation accurate and reproducible. The aim of this review is to refresh basic elements of the physiology of umbilical vein blood flow and its analysis. Its evaluation in normal and abnormal fetal growth is also discussed.

Correlation of Prenatal and Postnatal Diagnosis in Umbilical-Portal-Systemic Venous Shunts.

INTRODUCTION: Umbilical-portal-systemic venous shunts (UPSVS) are rare anomalies in the development of the fetal venous system. There are several postnatal and prenatal classifications of hepatic venous anomalies but the link between them is missing. We aimed to review the prenatal to postnatal diagnosis correlation in UPSVS at our center. METHODS: It is a retrospective study of patients diagnosed with UPSVS between 2019 and 2021 at our institution. Demographic, obstetric, genetic, and neonatal data were reviewed with special focus on prenatal and postnatal ultrasounds. RESULTS: A total of seven patients were diagnosed with UPSVS at a median of 24 (20-34) weeks of gestational age. All patients were male and 62% were Caucasian. None of the patients had chromosomopathies or cardiac anomalies. One patient had renal ectopia, another one had a single umbilical artery, and a third one suffered from intrauterine growth retardation. An umbilico-systemic shunt (USS) was found in two patients and a ductus venosus-systemic shunt (DVSS) in the rest. Patients with USS were diagnosed postnatally with intrahepatic portosystemic shunts. One of the DVSS patients was transferred to another hospital and the other four had normal postnatal cardiac ultrasounds, with normal abdominal ultrasounds in two patients and lack of postnatally abdominal control in the other two. All babies were found to be doing well at a median follow-up of 1 month (0-24). CONCLUSION: There is a knowledge gap in the natural history of UPSVS between fetal and neonatal life. Building bridges between prenatal and postnatal research is mandatory in order to understand these rare anomalies.

The cardiac-fetal-placental unit: fetal umbilical vein flow rate is linked to the maternal cardiac profile in fetal growth restriction.

BACKGROUND: The functional maternal-fetal hemodynamic unit includes fetal umbilical vein flow and maternal peripheral vascular resistance. OBJECTIVE: This study investigated the relationships between maternal and fetal hemodynamics in a population with suspected fetal growth restriction. STUDY DESIGN: This was a prospective study of normotensive pregnancies referred to our outpatient clinic for a suspected fetal growth restriction. Maternal hemodynamics measurement was performed, using a noninvasive device (USCOM-1A) and a fetal ultrasound evaluation to assess fetal biometry and velocimetry Doppler parameters. Comparisons among groups were performed with 1-way analysis of variance with Student-Newman-Keuls correction for multiple comparisons and with Kruskal-Wallis test where appropriate. The Spearman rank coefficient was used to assess the correlation between maternal and fetal hemodynamics. Pregnancies were observed until delivery. RESULTS: A total of 182 normotensive pregnancies were included. After the evaluation, 54 fetuses were classified as growth restricted, 42 as small for gestational age, and 86 as adequate for gestational age. The fetus with fetal growth restriction had significantly lower umbilical vein diameter (P<.0001), umbilical vein velocity (P=.02), umbilical vein flow (P<.0001), and umbilical vein flow corrected for fetal weight (P<.01) than adequate-for-gestational-age and small-for-gestational-age fetuses. The maternal hemodynamic profile in fetal growth restriction was characterized by elevated systemic vascular resistance and reduced cardiac output. The umbilical vein diameter was positively correlated to maternal cardiac output (rs=0.261), whereas there was a negative correlation between maternal systemic vascular resistance (rs=-0.338) and maternal potential energy-to-kinetic energy ratio (rs=-0267). The fetal umbilical vein time averaged max velocity was positively correlated to maternal cardiac output (rs=0.189) and maternal inotropy index (rs=0.162), whereas there was a negative correlation with maternal systemic vascular resistance (rs=-0.264) and maternal potential energy-to-kinetic energy ratio (rs=-0.171). The fetal umbilical vein flow and the flow corrected for estimated fetal weight were positively correlated with maternal cardiac output (rs=0.339 and rs=0.297) and maternal inotropy index (rs=0.217 and r=0.336), whereas there was a negative correlation between maternal systemic vascular resistance (rs=-0.461 and rs=-0.409) and maternal potential energy-to-kinetic energy ratio (rs=-0.336 and rs=-0.408). CONCLUSION: Maternal and fetal hemodynamic parameters were different in the 3 groups of fetuses: fetal growth restriction, small for gestational age, and adequate for gestational age. Maternal hemodynamic parameters were closely and continuously correlated with fetal hemodynamic features. In particular, a maternal hemodynamic profile with high systemic vascular resistance, low cardiac output, reduced inotropism, and hypodynamic circulation was correlated with a reduced umbilical vein flow and increased umbilical artery pulsatility index. The mother, placenta, and fetus should be considered as a single cardiac-fetal-placental unit. The correlations of systemic vascular resistance, cardiac output, and inotropy index with umbilical artery impedance indicate the key role of these 3 parameters in placental vascular tree development. The umbilical vein flow rate and, therefore, the placental perfusion seems to be influenced not only by these three parameters but also by the maternal cardiovascular kinetic energy.

A Rare Complication of Umbilical Vein Catheterization: TPNoma: US, X-ray, and MRI Findings.

BACKGROUND: Umbilical vein catheterization (UVC) is an early venous access route in newborns and is frequently used for delivering total parenteral nutrition (TPN) and medications. Vascular, hepatic parenchymal, and infectious complications of UVC can be seen rarely. OBJECTIVE: We present preterm neonates' X-ray, US, and MRI findings with parenchymal TPN extravasation and portal vein thrombosis. Our case was the first case about MRI findings of TPNoma. CASE PRESENTATION: A 30 week female with a birth weight of 1340 g was born via Cesarean section. Due to the diagnosis of transient tachypnea of the newborn and prematurity, the infant was hospitalized in the neonatal intensive care unit. On the first day of hospitalization, UVC was inserted and TPN with 20% lipid content was started. After 10 days, UVC was removed and TPN treatment was continued with a piccline catheter. Abdominal US and portal venous Doppler examination was applied to the patient whose general condition deteriorated on the 12th day. US and Doppler revealed a lesion of 17x17x18mm in size with lobulated contour, hyperechoic heterogeneous internal structure, and no central or peripheral blood supply was observed in the left lobe of the liver. Moreover, a filling defect compatible with a thrombus was observed in the proximal part of the left portal vein. An abdominal MRI was performed to characterize this liver lesion. Axial T1 weighted and T2- weighted images showed a heterogeneous hyperintense lesion without contrast enhancement. Axial fat saturation T1-weighted and out of phase T1-weighted images showed a reduction in signal intensity. US and MRI examinations showed that the thrombosed umbilical vein ended superior to the lesion. In the differential diagnoses, fat-containing lesions such as lipoma-teratoma and fat-containing collection secondary to extravasation of TPN treatment via UVC were thought. CONCLUSION: In the differential diagnosis of liver localized lesions in newborns, UVC-related liver injury should be considered and the localization of the catheter tip should be checked. In case of the doubt based on US and X-ray findings, presence of fat on MRI could be diagnosed. Serious complications should be avoided with catheter revision or removal.

Fetal intrahepatic Umbilical-Porto-Systemic venous shunts (IHUPSVS): In-utero anatomic classification.

OBJECTIVE: Congenital intrahepatic shunts divert highly oxygen and nutrients rich placental blood flow from the liver into the systemic flow having a negative influence on normal fetal growth and postnatal development. The ability to recognize this anomaly helps assess the possible clinical impact, counseling, and management of pregnancy. The present study aimed to propose in utero classification for the Intrahepatic Umbilical-Porto-Systemic Venous Shunt (IHUPSVS) based on our experience. STUDY DESIGN: A prospective study. Grayscale ultrasound with two and three-dimensional high-definition Doppler modalities was used. IHUPSVS was defined as a diversion of blood from the liver tissue by abnormal communication between a branch of the intrahepatic Umbilical vein or the Portal veins with the systemic circulation (the Hepatic veins or the Sub-Diaphragmatic Vestibulum). RESULTS: Twenty-five fetuses were diagnosed with IHUPSVS. We identified three main anatomic types: I) Porto-hepatic shunt which was divided into Ia) regular single shunt (15/25, 60%) and Ib) regular multiple shunts, (6/25, 24%) II) Umbilical-Porto-Hepatic shunt divided into a) Umbilical or b) Umbilical combined with Portal hepatic shunt (2/25, 8%) and III) Cavernous- aneurysmatic shunt (2/25, 8%). All the shunts were verified by postnatal targeted sonography. CONCLUSIONS: This study creates the anatomic basis for common nomenclature and future probable updating for this anomaly.

Ultrasound-Guided Umbilical Venous Catheter Insertion to Reduce Rate of Catheter Tip Malposition in Neonates: A Randomized, Controlled Trial.

OBJECTIVE: To investigate whether ultrasound-guided umbilical venous catheter (UVC) insertion (US group) reduced the rate of malpositioning of the catheter tip compared to the standard method of insertion (SD group). METHODS: In this open-label, randomized, controlled trial, neonates admitted to NICU within the first week of life were randomly assigned to the US group (n = 26) or SD group (n = 27). Neonates with major congenital anomalies of the thorax and abdomen were excluded. The primary outcome was the rate of malpositioning of the catheter tip. RESULTS: The rate of malpositioning of the catheter tip was observed in a significantly lower number of neonates in the US group as compared to the SD group (11/26, 42.3% vs. 20/27, 74%; RR = 0.57, 95% CI: 0.34 to 0.94, p = 0.019). As more of the UVCs were positioned optimally in the first attempt in the US group than SD group, the need for repeated attempts at catheter repositioning was reduced, resulting in reduced procedure time (minutes) [mean (SD), 23.96 (6.42) vs. 30 (1.83); mean difference 6.04 (95% CI: 3.46 to 8.62), p = 0.005]. This also led to a reduction in the additional X-ray exposure in the US group (n = 11) compared to the SD group (n = 20) [95% CI: 3.12 to 44.26; p = 0.020]. CONCLUSION: Ultrasound-guided UVC insertion significantly reduced the rate of catheter tip malposition. It also reduced the number of attempts at catheter manipulation, procedure time, and X-ray exposures. With adequate training, it could be incorporated into routine bedside practice during UVC insertion for optimum placement. TRIAL REGISTRATION: Clinical Trial Registry of India ( www.ctri.nic.in ) CTRI/2021/03/031894.