Prenatal Sonograms Research Articles

Surgical strategies for placenta percreta invading the bladder and review of literature

BackgroundMajor obstetric hemorrhage is the leading cause of maternal morbidity and mortality. In rare cases, life-threatening hematuria in pregnant women may result from invasion of the bladder by the placenta. We present our experience with 18 cases of placenta percreta with suspected bladder invasion.MethodsIt is a retrospective single-center study conducted over a period of 3 years. Total 18 patients of radiologically diagnosed placenta percreta were included in the study. All patients who are at risk for placenta percreta underwent prenatal sonograms. Patients of Placenta Accreta Spectrum presenting electively also underwent MRI pelvis. Elective patients who were high risk of placenta percreta underwent bilateral placement of the balloon catheter in internal iliac artery. In case of doubt regarding bladder invasion, patient underwent anterior cystotomy and posterior wall of the bladder was examined and proximity of the ureteric orifice to the placenta and amount of involvement of bladder wall was assessed. Ureteric catheter placement was used as adjuncts depending on the proximity of placental invasion with ureteric orifice. Postoperative outcomes in the form of maternal morbidity, maternal mortality, fetal mortality, postoperative bleeding, bladder status, vesicovaginal fistula, bladder capacity were all evaluated.ResultsIn our series, 17 cases all cases were diagnosed preoperatively by antenatal ultrasound and MRI. Only one patient presented with hematuria. Only in one patient, we attempted separation of placenta from bladder wall, and it resulted in profuse bleeding, and in rest, we excised the involved bladder. Partial cystectomy was done in 33.4% patients, 27% patients required bilateral placement of ureteric catheter due to proximity to the ureteric orifice. 33.4% patient underwent bilateral internal iliac artery ligation or balloon placement. Clot evaluation was needed in one patient. Intraoperatively—39% patients had uterus adhered to the bladder but no placental invasion into the bladder. One patient was managed with obstetric hysterectomy and methotrexate followed by clot evacuation and bilateral internal iliac artery ligation at a later date. One (5.6%) patient developed vesicovaginal fistula in postoperative period. There was one (5.6%) maternal mortality with no fetal mortality. On follow-up, patient had good bladder capacity, 3 weeks after the surgery.ConclusionMRI done preoperatively can help us guide regarding the extent or severity of placental invasion. Intraoperatively, anterior cystostomy should be done in suspected placenta percreta. Grade I or II accrete/percreta patients can be managed conservatively. Partial cystectomy with placement of bilateral ureteric catheter is safer and less morbid approach in tackling placenta percreta invading the bladder with mucosal involvement.

Motion Correction For 3D Volumetric Reconstruction from 2D Multi-Slice Stacks in Fetal MRI

Anomalies in brain development may lead to neurological disorders and developmental impairment and may even affect the survival in the prenatal period or later in the early stages of life. Prenatal ultrasonography is the most widely used imaging technique for prenatal care. However, it has limitations to detect abnormalities in brain development due to fetal position, gestational age or skull ossification. Magnetic Resonance Imaging (MRI) has been increasingly used in clinic to quantitatively assess brain growth and development during the fetal and neonatal period with millimeter-level image resolution, high soft tissue contrast and volumetric coverage. It becomes an essential and ideal technique in clinical care to evaluate fetuses who have risks of developmental abnormalities but not apparently shown on prenatal sonogram.

Prognostic significance of prenatal ultrasound in fetal arthrogryposis multiplex congenita

PurposeFetal arthrogryposis multiplex congenita (AMC) describes a heterogeneous disease entity characterized by multiple contractures affecting at least two different body areas. The aim of our study was to identify additional sonographic abnormalities in fetuses with AMC Type I–III associated with an unfavorable prognosis and to describe when those signs were first detected.MethodsThis retrospective study included 41 pregnancies of suspected AMC diagnosed 1999–2017 at our tertiary referral center. The affected pregnancies were divided into the 3 AMC subgroups; the time of detection and outcome were analyzed. Prenatal sonograms, pediatric charts, genetic tests, and autopsy reports were studied.ResultsPregnancy outcome data were verifiable in 34 out of 41 cases; in 27 cases, AMC was confirmed. Hydrops was present in 50% of postnatally deceased fetuses, 53% of cases resulting in termination of pregnancy vs. 0% of the surviving 8 children. Absent stomach filling was found in 67% of the children with neonatal death. After subcategorization, the limb-involvement-only-group, 8% showed hydrops vs. 100% in system anomaly group vs. 70% in neuromuscular dysfunction cohort (p = 0.001). Scoliosis, nuchal edema, and absent stomach filling were significantly indicating for a neurological etiology.ConclusionIn addition to disease-defining sonographic findings, those with prognostic significance were identified. Hydrops, nuchal edema, scoliosis and absent stomach filling were associated with unfavorable outcomes implicating a neuromuscular etiology. This knowledge can help to predict the further course of the disease and support patient counseling.

Prenatal diagnosis of foetal hydrocephalus and suspected X-linked recessive inheritance of cleft lip in a Chihuahua.

A 3.5-year-old, 2.9 kg, multiparous Chihuahua presented with abdominal distension; pregnancy was diagnosed. On Day 7 before parturition, prenatal sonograms showed anechoic bilateral dilated cerebral lateral ventricles, suggesting fluid-filled regions (ventriculomegaly) in one foetus. A Caesarean section was performed and the male newborn had an abnormally enlarged dome-shaped head and a cleft lip, and died 6 days after birth. According to the family pedigree, the X-linked recessive inheritance of an orofacial cleft from the unaffected mother was suggested. This report clearly demonstrates that canine foetal ventriculomegaly (hydrocephalus) can be diagnosed in utero. For dog breeds predisposed to congenital ventriculomegaly, early detection is important for the prediction of perinatal survival and adequate supportive care can be applied at delivery.

Alobar holoprosencephaly in one of twin neonates

Holoprosencephaly (HPE) is a congenital structural anomaly of the forebrain which is often diagnosed prenatally, usually in singleton pregnancies. This is a case of alobar HPE affecting one of 19-day-old twin neonates which was only detected postnatally. The other twin was unaffected. The anomaly was not diagnosed in utero because a prenatal sonogram was not performed. This case is presented to reemphasize the need for thorough routine prenatal/obstetrics sonography. After birth, transfontanelle ultrasonography is a useful imaging modality for diagnosing this debilitating cerebral structural anomaly, but definitive classification requires cross-sectional brain imaging with computed tomography or magnetic resonance imaging.

Case 253: Thrombosed Umbilical Venous Varix in an Infant

History A 3-month-old previously healthy girl presented to an outside institution with a 4-day history of low-grade fever, irritability, and a tender "knot" in the upper abdomen. Ultrasonography (US) was performed at an outside hospital. US images were not available for review; however, they showed a mass in the left hepatic lobe, per the outside report, and the patient was referred to our institution for further evaluation. Her parents reported a normal full-term pregnancy, with regular prenatal care and normal prenatal US findings. The baby was born after an uncomplicated gestation. She was delivered at term via an uncomplicated cesarean section due to a maternal history of cesarean section. The perinatal course was uncomplicated, and there was no history of umbilical catheterization, per the parents. On arrival at our institution, the patient had a temperature of 38.2°C. All other vital signs were normal. Palpation revealed a tender and firm mass in the periumbilical region; otherwise, physical examination findings were normal. Results of laboratory work-up were normal, except for elevated white blood cell count (26 600/mm3 [26.6 × 109/L]; normal, 6000-17 500/mm3 [6-17.5 × 109/L]). The patient underwent US followed by intravenous contrast material-enhanced (10 mL ioversol, Optiray 320; Medtronic, Santa Rosa, Calif) computed tomography (CT) on the same day.

Congenital cytomegalovirus infection presenting with intrapulmonary calcifications on prenatal sonogram

Cytomegalovirus (CMV) is the most common congenital viral infection in developed countries. Although pneumonitis has been reported in infants with congenital CMV infection, involvement of the lung parenchyma is less common in congenital than in perinatal CMV infections. We report an infant with congenital cytomegalovirus (CMV) infection and multiple intrapulmonary calcifications on prenatal sonogram that had congenital pneumonia and persistent oxygen requirement after birth. To our knowledge, this is the first case report of a live-born infant with pulmonary calcifications due to CMV noted on prenatal sonograms.

Sacrococcygeal teratoma: A population-based study of incidence and prenatal prognostic factors

BackgroundSacrococcygeal teratoma (SCT) is a rare congenital tumor associated with high rates of perinatal mortality and morbidity. This study evaluated the incidence, prenatal detection rate, and early predictors of a complicated outcome following diagnosis of SCT. MethodsWe retrospectively identified all fetuses and newborns diagnosed with SCT in southern Sweden from 2000 to 2013. Prenatal sonograms, charts, and pathology reports were reviewed and analyzed. Each case of SCT was defined as complicated or uncomplicated based on the postnatal outcome. All cases with a fatal outcome or that required cardiac resuscitation during birth or surgery were classified as complicated. ResultsThe overall incidence of SCT was 1:13,982 (19 children in a cohort of 265,658 live births). A prenatal diagnosis was made in 74% of cases, there were no stillbirths or intrauterine deaths, and the overall mortality rate was 11%. Four cases of SCT (21%) were classified as complicated, and these cases had a significantly larger tumor size at gestational week 20 (P=0.048), had a significantly higher tumor growth rate (P=0.003), and were more often associated with polyhydramnios (P=0.01), and mainly solid/mixed morphology (P=0.001). ConclusionsThe incidence of SCT in southern Sweden was higher than those reported in most previous studies; however, the associated mortality rate was relatively low. Fetuses with large tumors, rapidly growing tumors, and polyhydramnios were more likely to experience a complicated outcome during the postnatal period.

In memory: Floyd Dunn.

I was deeply saddened when I heard that Professor Floyd Dunn, a groundbreaking pioneer in the science of ultrasound and bioacoustics, passed away on January 24, 2015. His death came just 30 days after that of his wife Elsa. He was born in Kansas City, MO, USA in 1924. He attended the University of Illinois (UI) at Urbana-Champaign in the Department of Electrical and Computer Engineering, earning three degrees, including his Ph.D. in 1956. He remained at UI for 50 years, and during that period he directed the Bioacoustics Research Laboratory at UI for more than 20 years. His research interests included bioeffects, safety of ultrasound, ultrasonic imaging used for prenatal sonograms, the ultrasonic microscope, and numerous measurement tools. He carried an appointment at the University of Arizona, Tucson after his retirement from UI in 1995. He was a member of both the National Academy of Sciences and the National Academy of Engineering. He was also the past President of the Acoustical Society of America (ASA). He was a recipient of the IEEE Edison Medal, the ASA Gold and Silver Medals, as well as the AIUM William J. Fry Memorial Lecture Award in 1984 and the AIUM Joseph P. Holmes Basic Science Pioneer Award in 1990. Not only in the US, USSR, or United Kingdom, he also taught and led ultrasound research in Japan. Of note, he annually visited Sendai for more than 15 years after he spent 1 year on his sabbatical at Tohoku University in 1989. During his stay in Sendai, he taught young researchers topics ranging from the principle of ultrasound to advanced technology in medical ultrasound. I was grateful that he edited my papers for both content and English grammar before submission to Ultrasound in Medicine and Biology again and again. Together with Professors Motonao Tanaka, Shigeo Ohtsuki, and me, he edited a monogram titled ‘‘Ultrasonic Tissue Characterization’’ published by Springer in 1996. He influenced many of Japan’s leading researchers in medical ultrasound including Professors Daitaro Okuyama, Noriyoshi Chubachi, Motonao Tanaka, Hiroshi Inoue, Jun-ichi Kushibiki, Nobuyuki Endoh, Shin-ichiro Umemura, Hiroaki Okawai, Hiroshi Kanai, and Hideyuki Hasegawa. For his achievements in research and education in medical ultrasound in Japan, he was awarded as an honorary member of the Japan Society of Ultrasonics in Medicine in 1990. Personally, I visited him in 2012 when the AIUM annual meeting was held in Phoenix, AZ. At that time, I drove a Ford rent-a-car to Tuscan from Phoenix and he drove his Lexus sports car to the meeting venue. He was very sharp and remembered the names of most people he met in Sendai, not only researchers but also their families and secretaries. I will forever be grateful for Dr. Dunn’s generosity and support. My deepest sympathies and prayers for his family, friends, and colleagues. & Yoshifumi Saijo saijo@idac.tohoku.ac.jp

Term Infant with Echogenic Fetal Lung Mass on Prenatal Sonogram

Postnatal management of infant with prenatally diagnosed fetus with Echogenic fetal lung depends on the infant’s system at the time of delivery. Infant with large lesions can present with symptomatology at birth whereas a symptomatic infant with normal X-ray requires CT scan or more advance MRI studies between 4-6 weeks prior to discharge from birth facility.

Cardiology patient page. Screening for critical congenital heart disease in newborns.

We have come a long way in our ability to treat critical congenital heart conditions, which affect about 3 of every 1000 live births and account for 30% of all infant fatalities in the United States annually.1 Prenatal sonograms can often identify structural heart disease; however, the sensitivity of congenital heart disease (CHD) detection is highly variable, depending on operator expertise, gestational age, fetal position, and the type of cardiac defect. As a result, prenatal sonography will miss some patients with critical CHD and is known to miss many newborns with simple CHD. Newborns who might benefit from early treatment can often be identified in their first days of life through pulse oximetry screening—a painless, readily available noninvasive examination that is easy to incorporate into newborn assessments. This noninvasive test measures the percentage of hemoglobin in the blood that is saturated with oxygen and pulse rate. The addition of pulse oximetry to fetal ultrasound and physical examination as a screening tool can reduce the chance of overlooking critical congenital heart disease (CCHD) in newborns.2 Oxygen breathed in through the lungs attaches to the red blood cell protein hemoglobin. The newly oxygenated blood then circulates to the tissues. Arterial hemoglobin saturated with oxygen is bright red, and venous hemoglobin with less oxygen is darker. Pulse oximetry uses the principles of spectrophotometry and …

Postnatal Outcome of Fetuses With the Prenatal Diagnosis of Gastroschisis

The purpose of this study was to assess the postnatal outcome and complications that arise in infants with the prenatal diagnosis of gastroschisis. Prenatal sonograms with the diagnosis of gastroschisis were identified. Maternal age, indication for sonography, gestational age at diagnosis, other sonographic abnormalities, and postnatal outcome were recorded. Ninety-eight fetuses at 14.3 to 36 weeks' gestation had the diagnosis of gastroschisis on sonography. In 14 cases (14%), other fetal anomalies were identified, including hydronephrosis, hydrocephalus, coarctation of the aorta, and a limb anomaly. Bowel dilatation developed in 72 of 84 cases (86%) followed prenatally with sonography, and bowel wall thickening developed in 40 of 73 cases (55%). On postnatal follow-up, 57 of 68 infants (84%) had postnatal complications, many with multisystem complications, including 6 deaths, 40 with bowel-related complications, 30 with infectious complications, and 32 with anomalies involving other systems (genitourinary, cardiac, and central nervous system). The postnatal outcome did not correlate with the presence of bowel dilatation or bowel wall thickening on prenatal sonography. Only 11 infants (16.2%) had a completely uncomplicated postsurgical course. Hospital stays in survivors (n = 92) ranged from 8 to 307 days (mean, 53 days). Although reported survival rates are good for gastroschisis, the postoperative hospital stay is often lengthy, and complications are very common, especially those related to the gastrointestinal tract. Associated anomalies were more common in our study than previously reported.

Propranolol as a First‐line Treatment for Diffuse Infantile Hepatic Hemangioendothelioma

Propranolol as a First‐line Treatment for Diffuse Infantile Hepatic Hemangioendothelioma

Fetal intra-abdominal tumors: assessment of spectrum, accuracy of prenatal diagnosis, perinatal outcome and therapy at a tertiary referral center

To describe the varieties and ultrasound characteristics of prenatally diagnosed fetal abdominal tumors and to scrutinize the accuracy of prenatal diagnosis as well as the postnatal outcome and therapy of affected pregnancies. Retrospective study of 354 fetuses found to have abdominal tumors on prenatal sonogram, identified from 1993 to 2009 at a tertiary referral center for prenatal medicine. The cohort was classified into subgroups according to the sonographic appearance of the fetal tumor and the affected anatomic structure (urinary, gastrointestinal and genital tracts and other locations). Sensitivity, specificity, positive predictive value and false-positive rate of ultrasonography in identifying the system of origin were calculated. Relationships between relevant outcome domains and the different subgroups were assessed using the chi-square test and Fisher's exact test. Our cohort comprised 222 urinary tract lesions, 37 genital tract lesions, 80 gastrointestinal lesions and 15 tumors of other origins. The mean gestational age at diagnosis was 26+0 wks. The prenatally established diagnosis was exactly concordant with postnatal findings in 88.9%. Sensitivity, specificity, positive predictive value and false-positive rate of ultrasonography in identifying the system of origin (urinary, gastrointestinal, genital tracts and other locations) were 98.3%, 97.6%, 92.6% and 2.4%, respectively. The favorable postnatal outcome rate was highest among fetuses with genital tract lesions (95%) and lowest among those with tumors of the urinary tract (62%, p=<0.001). Twenty per cent of tumors regressed spontaneously, mostly gastrointestinal tumors (36%, p=<0.001). In 75/354 cases (21%) the parents opted to terminate the pregnancy: intra-uterine fetal demise and neonatal death were each noted in 4%. Prenatal therapy was performed in 24 of 354 cases (7%) and postnatal surgery in 64 cases (18%). The majority of fetal abdominal anomalies were accurately diagnosed and the vast majority of affected fetuses had a favorable outcome, some tumors even resolved with advancing pregnancy. Pre- and post-natal invasive surgical interventions were mandatory in only a small number of cases.

Accuracy of Sonography to Predict Estimated Weight in Fetuses With Gastroschisis

The purpose of this study was to determine whether sonographic formulas for estimating fetal weight are as accurate for fetuses affected with gastroschisis as they are for healthy fetuses. We hypothesized that because the most commonly used Hadlock formulas rely on the abdominal circumference as a biometric variable, estimates of birth weight are less reliable in fetuses with gastroschisis than in healthy fetuses. We performed a chart review of all fetuses with a prenatal diagnosis of gastroschisis at 3 tertiary care institutions from 1990 to 2008. Charts were reviewed for clinical and sonographic data. The estimated fetal weight at the prenatal sonogram closest to delivery was compared to the birth weight. Published Hadlock formulas using 4 biometric parameters were used to calculate the estimated fetal weight. Data analysis was performed using the Student t test and χ(2) test for continuous and categorical variables, respectively. One hundred eleven patients with gastroschisis were identified. Sixty-six patients had a prenatal sonogram with a calculated estimated fetal weight within 7 days of delivery; 88 patients had a sonogram within 14 days. The mean birth weights ± SD were 2292 ± 559 and 2477 ± 531 g in the 0- to 7- and 8- to 14-day groups, respectively. Sonographic biometric measurements underestimated the birth weight by an average of 5.6%. Intrauterine growth restriction was predicted in 72% of all pregnancies but was only present in 52%. Our study shows a systematic error of birth weight underestimation when using the Hadlock formulas in fetuses affected with gastroschisis.

P19.10: Prenatal imaging findings in cloacal extrophy

Background: congenital inguinal hernias are rarely detected in utero, with prenatal diagnoses often made in the 3rd trimester. While most reported cases are isolated, fetal inguinal hernia has also been reported with rib and vertebral anomalies in the setting of Jarcho-Levin syndrome. Case: we report a case and associated images and video of a male infant prenatally diagnosed with a large inguinal hernia and multiple other anomalies. Prenatal ultrasound at 32 weeks revealed a large homogeneous solid mass anterior to the fetal pelvis measuring 4.70 × 4.62 × 4.88 cm adjacent to a normalappearing phallus (image 1). Peristalsis was noted within the mass, with bowel loops extending from the fetal abdomen into the mass suggesting an inguinal hernia (video). A normal cord insertion was noted. Additional anomalies apparent by prenatal sonogram included lumbosacral myelomeningocele with Chiari malformation, d-transposition of the great arteries, multiple hemivertebrae, and lower extremities fixed in flexion with short longs bones and bilateral talipes. Prenatal MRI confirmed the above findings. Amniocentesis revealed a normal male karyotype with a negative microarray. Cesarean delivery of a 2630 g male was performed at term due to breech presentation. Postnatal examination confirmed prenatal findings with no clinical or molecular evidence of a genetic abnormality. Conclusion: Inguinal hernias may be detected prenatally by identification of peristalsing bowel loops extending from the fetal abdomen into an external pelvic mass. Close evaluation for associated anomalies is required, as other structural anomalies may be present even the absence of a syndromic diagnosis.

Is radical lobectomy required in congenital cystic adenomatoid malformation?

BackgroundThe extent of surgical resection in the management of congenital cystic adenomatoid malformation (CCAM) located in 1 pulmonary lobe remains controversial. Although lobectomy seems to be the best treatment to avoid recurrent pulmonary infection and neoplasia, neither retrospective nor prospective studies have been done to our knowledge. Besides, many reports claim that there are advantages of parenchyma-saving resections. The aim of our study was to determine whether it was necessary to perform an extended resection in these cases. Materials and MethodsWe retrospectively reviewed all patients who had undergone a thoracoscopic lobectomy for CCAM located to 1 pulmonary lobe between January 2007 and January 2009. A thoracic radiologist performed a single blind review of all preoperative computed tomographic (CT) scans, mentioning the presence or absence of distant lesions from the main cysts of CCAM within the pulmonary lobe. The pathologist who analyzed the pulmonary lobectomy specimen was aware of the diagnosis but not the CT report. ResultsTwenty-five patients underwent thoracoscopic lobectomy for congenital lung malformations, including 12 with CCAM. All had a positive prenatal sonogram. The median age at surgery was 12 months (range, 2-24 months). The preoperative CT showed only 2 cases with distant lesions within the affected pulmonary lobe, whereas the histologic study of the surgical specimen identified 6 cases. The sensitivity of the CT scan was low, 33%, whereas its specificity was high, 100%. Furthermore, the preoperative CT negative predictive value was 60%. The median time of follow-up was 7.5 months. ConclusionAdequate treatment of CCAM in children requires a lobectomy because of poor sensitivity and very poor negative predictive value (60%) of the preoperative CT to determine distal adjacent lesions. A prospective study on a larger number of patients is required to confirm these observations.

Acrania-Anencephaly Associated with Hypospadias. Prenatal Ultrasound and MRI Diagnosis and Molecular Folate Metabolism Pathway Analysis

Acrania may occur as a single isolated malformation or associated with extracranial defects. Hypospadias is one of the most common congenital abnormalities of the genitalia frequently missed on prenatal sonograms. Second trimester two- and three-dimensional ultrasound and MRI diagnosis with necropsy and folate metabolism pathway analysis. The mechanisms leading to closure of both neural and urethral tubes, are far from being demonstrated, and molecular studies of this very rare association are lacking although it might be based on a common genetic mechanism, leading to a disturbed development pathway at the molecular level.

Malformaciones urinarias del recién nacido: Estudio ECLAMC 1998-2010

Introduction: Congenital abnormalities of the Urinary Tract are frequent and prevalence has increased since the introduction of routine prenatal sonogram. Objectives: To determine the prevalence rate of congenital urinary malformations at birth at Hospital Clinico de la Universidad de Chile. These data will be compared to chilean hospitals participating in ECLAMC (Estudio Colaborativo Latino Americano de Malformaciones Congenitas - Latin American Collaborative Study of Congenital Malformations). A longitudinal study will serve to evaluate if significant variation has occurred, and risk factors will be investigated. Patients and Methods: All births occurring between January, 1998 and December, 2010 were included. Prevalence rate of urinary malformations were calculated, and compared to those obtained in previous years. Statistical analysis of proportions was calculated through mean and average comparison was made through Student t test. Results: Urinary anomalies appeared at a rate of 64.5 per 10.000 births. This represents a significant increase from previous studies. Highest risk factor seemed to be other family members with disease. Most frequent anomaly was Hydroureteronphrosis (24,2%) followed by Hypospadias (17%). The rate of these malformations in ECLAMC participating Chilean hospitales was 23.37/10.000 births. Conclusion: A significant increase in the diagnosis of these anomalies was shown, being the most important risk factor the presence of family members with similar congenital disorders.

Persistent right umbilical vein: a prenatal condition worth mentioning?

To investigate the incidence and clinical impact of prenatally diagnosed persistent right umbilical vein (PRUV) in a referral population and to evaluate the findings together with those from previous publications. This was a retrospective analysis of 39 cases with PRUV diagnosed in three tertiary referral centers for prenatal medicine between 1996 and 2009. Fetuses with situs inversus, situs ambiguous and heterotaxy (left and right isomerism) were excluded. During the study period 46 653 consecutive high- and low-risk pregnancies were examined. The prenatal sonograms and neonatal outcome data of affected individuals were reviewed. Our findings were analyzed together with findings retrieved from the scientific literature. Twenty-nine fetuses had an isolated PRUV as a single anomaly, whereas 10/39 (25.6%) were found to have PRUV accompanied by additional minor and major abnormalities. These anomalies comprised mainly cardiovascular, genitourinary and gastrointestinal malformations. In common with our series, previously published cases of isolated PRUV (n = 197) displayed an uneventful course of pregnancy and a favorable postnatal outcome. Sixty-six previously reported cases of PRUV with additional anomalies were identified in the literature. Intrahepatic umbilical drainage is the most frequent type of PRUV. Including our cases, there were 16 cases (5.3%) with extrahepatic drainage of PRUV, all of which had additional anomalies. Consistent with previous reports, in the majority of cases (74.8%) PRUV is an isolated finding. While these cases carry an excellent prognosis, PRUV can be associated with severe congenital anomalies, so this finding should prompt detailed prenatal assessment of the fetus.