Hemoglobin Electrophoresis versus Kleihauer-Betke to Determine Bone Marrow Suppression in Fetuses Undergoing Intrauterine Transfusion.

OBJECTIVE: Mainstay therapy for fetuses affected by maternal red cell alloimmunization is serial intrauterine transfusion (IUT). Testing to determine when fetal red cells have been replaced with donor cells historically involves the use of the Kleihauer-Betke (KB) test. Hemoglobin (Hgb) electrophoresis testing may be more rapid with a reduced cost of analysis. We aimed to determine the correlation between fetal Hgb electrophoresis versus the traditional KB test. STUDY DESIGN: This is a retrospective analysis of all alloimmunized singleton pregnancies undergoing IUT between January 1, 2021, and July 1, 2023. Maternal and fetal characteristics were collected along with the indication for IUT. A final fetal blood sample was obtained at the conclusion of each transfusion and sent for KB testing and Hgb electrophoresis. The primary outcome was the assessment of these parameters in their ability to predict the replacement of the fetal circulating red cell population with donor cells. Linear regression analysis and repeated measures analysis of variance were performed, and p-values less than 0.05 were considered significant. RESULTS: A total of 56 IUTs were performed in 16 patients. There were 39 (69.6%) final KB test values collected and compared with 30 (53.6%) final Hgb electrophoresis values. Hgb electrophoresis when compared with the KB test demonstrated a significant correlation (R 2 = 0.93; 95% confidence interval, 0.61-0.76; p < 0.001). This same finding held true when examining the correlation at each individual IUT as well. The final KB test and Hgb electrophoresis values significantly decreased with each transfusion (p = 0.003). A predominance of adult donor blood was noted by the third transfusion for both laboratory indices. CONCLUSION: Fetal Hgb electrophoresis obtained at the time of IUT demonstrates a significant correlation with the traditional KB test. KEY POINTS: · Fetal Hgb electrophoresis following IUT is underexplored. · Hgb electrophoresis is an automated evaluation. · The traditional KB test is a manual evaluation. · These two tests demonstrate significant correlation.

Placental delayed villous maturation is associated with fetal congenital heart disease.

BACKGROUND: The placenta is crucial for the overall development and lifelong health of the fetus. Abnormal placental development and function occur in pregnancies with fetal congenital heart disease. However, studies that use standardized diagnostic criteria and incorporate control populations are lacking. This limits the generalizability of current research and the ability to determine the specific placental abnormalities associated with congenital heart disease. OBJECTIVE: This study applied consensus statement guidelines (known as the Amsterdam criteria) for placental pathology interpretation to compare the frequency and pattern of abnormalities in pregnancies with fetal congenital heart disease to demographically matched control pregnancies and evaluate for differences in placental abnormalities by cardiac physiology. STUDY DESIGN: A single-center retrospective cohort study was conducted from January 2013 to June 2019. Infants with a prenatal diagnosis of moderate-severe congenital heart disease who were born at ≥37 weeks of gestation were included. A control group born at ≥37 weeks of gestation but without fetal congenital heart disease or other major pregnancy complications was matched to the congenital heart disease group on maternal race and ethnicity and infant sex. Using the Amsterdam criteria, placental pathology findings were categorized as delayed villous maturation, maternal vascular malperfusion, fetal vascular malperfusion, and inflammatory lesions. The frequency of placental abnormalities was compared between groups, and logistic regression was performed to evaluate the association of clinical and sociodemographic factors with delayed villous maturation, maternal vascular malperfusion, and fetal vascular malperfusion. RESULTS: There were 194 pregnancies with fetal congenital heart disease and 105 controls included, of whom 83% in the congenital heart disease group and 82% in the control group were of non-Hispanic White race and ethnicity. Compared with controls, pregnancies with fetal congenital heart disease had higher rates of delayed villous maturation (6% vs 19%; P<.001) and maternal vascular malperfusion (19% vs 34%; P=.007) but not fetal vascular malperfusion (6% vs 10%; P=.23). Infants with congenital heart disease with 2-ventricle anatomy displayed the highest odds of delayed villous maturation compared with controls (odds ratio, 5.5; 95% confidence interval, 2.2-15.7; P<.01). Maternal vascular malperfusion was 2.2 times higher (P=.02) for infants with 2-ventricle anatomy and 2.9 times higher (P=.02) for infants with single-ventricle physiology with pulmonic obstruction. Within the congenital heart disease group, delayed villous maturation was associated with higher maternal body mass index, polyhydramnios, larger infant birth head circumference, and infant respiratory support in the delivery room, whereas maternal vascular malperfusion was associated with oligohydramnios. In multivariable models adjusting for cardiac diagnosis, associations of delayed villous maturation persisted for infant birth head circumference (odds ratio, 1.2; 95% confidence interval, 1.0-1.5; P=.02) and infant respiratory support in the delivery room (odds ratio, 3.0; 95% confidence interval, 1.3-6.5; P=.007). CONCLUSION: Pregnancies with fetal congenital heart disease displayed higher rates of delayed villous maturation and maternal vascular malperfusion than controls, suggesting that placental maldevelopment may relate to maternal factors. Future investigations are needed to determine the association of these abnormalities with postnatal infant outcomes.

Monochorionic Twins: TTTS, TAPS, and Selective Fetal Growth Restriction.

With an increasing incidence of twin gestations, understanding the inherent risks associated with these pregnancies is essential in modern obstetrics. The unique differences in placentation in monochorionic twins leads to unique complications, including twin-to-twin transfusion syndrome, the twin anemia-polycythemia sequence, and selective fetal growth restriction. Not only does the understanding of the monochorionic placenta lead to an understanding of the pathophysiology of the complications of monochorionic twins, but it also has led to the development of highly effective directed fetal therapy via fetoscopic laser coagulation used in twin-to-twin transfusion syndrome.

Missouri's Abortion law: Access to care for Patients with Lethal Fetal Anomalies at a Midwest Tertiary care Center.

OBJECTIVES: Missouri passed an 8-week gestational age abortion in August 2019. The objective of this study was to compare distance and time from referral to evaluation between patients who continued their pregnancy and those who terminated in patients with severe and lethal fetal anomalies and estimate the impact of the Missouri gestational age abortion ban on distance to abortion care in this patient population. METHODS: This is a retrospective cohort study of patients seen at the Washington University in St. Louis (WUSTL) Fetal Care Center (FCC) with a severe or lethal fetal anomaly between July 2018 and June 2019. Patient characteristics including gestational age at referral and distance traveled to the FCC were compared between patients who underwent abortion and who continued their pregnancies. RESULTS: From July 2018 to June 2019, 463 patients were seen in the Fetal Care Center and 13% (60/463) were diagnosed with severe or lethal fetal anomalies comprising the study population for this analysis. Of these, 21 (35%) patients underwent an abortion, and 39 (65%) patients continued their pregnancy. Patients who underwent abortion were referred at a significantly earlier gestational age (median 19 weeks [IQR 17, 20 weeks] v. 20 weeks [IQR 18, 24 weeks]), p = 0.04. There was a statistically significant difference between the median latency time between patients who underwent an abortion and who continued their pregnancy (median 8 days [IQR 4,13 days] v. 14 days [IQR 9, 22 days], p < 0.01). CONCLUSION: Patients with severe or lethal fetal anomalies are often evaluated at later gestational ages, which may preclude their access to abortion services.

The Special Delivery Unit: A Novel Concept for Mother-Baby Continuity for Major Fetal Abnormalities.

OBJECTIVE: This article describes the experience in the planning and development of a special delivery unit (SDU) at our free-standing children's hospital in Austin, Texas. STUDY DESIGN: Description of various aspects of the development of the SDU. In addition, telephone surveys were obtained from five other institutions regarding the planning and current status of their SDUs. RESULTS: Since the advent of the SDU at Children's Hospital of Philadelphia in 2008, several free-standing children's hospitals have opened similar units at their institutions. Developing an obstetrical unit in a children's hospital can be a daunting task on many fronts. The costs of providing 24-hour obstetrical, nursing, and anesthesiology coverage must be considered. Although most SDUs are associated with a fetal center and fetal surgery/interventions, some units function exclusively for the delivery of pregnancies complicated by major fetal conditions where the neonate will require immediate surgical care or other interventions. CONCLUSION: Research on the cost-effectiveness and the effect of SDUs on clinical outcome, teaching, and patient satisfaction is warranted. KEY POINTS: · Specialized delivery units are becoming more common at free-standing children's hospitals.. · The primary aim of the SDU is to maintain mother-baby continuity in cases of congenital anomalies.. · Developing an obstetrical unit at a pediatric hospital is a daunting task..

Cell free fetal DNA to triage antenatal rhesus immune globulin: Is it really cost-effective in the United States?

OBJECTIVE: To compare the efficacy and costs of three different strategies of antenatal rhesus immune globulin (RhIG) administration in a US population. METHODS: A decision tree analysis was undertaken for universal antenatal RhIG administration based on RhD serologic paternity testing, universal administration without paternity, and selective antenatal RhIG administration using cell free fetal DNA (cfDNA) for RHD fetal typing. Rates of alloimmunization were calculated. Charges were determined for laboratory testing and obstetrical and neonatal treatments for the first pregnancy and cases of alloimmunization in the following pregnancy. RESULTS: The largest number of new RhD alloimmunization cases resulted from a strategy of universal RhIG that included paternity. Fewer cases resulted from a selective strategy; the least number of cases were associated with a universal approach that discounted paternity. When the costs of first pregnancies and alloimmunized second pregnancies were combined, a universal strategy that excludes paternity had the least costs followed by a selective strategy followed by a universal strategy that included paternity. CONCLUSION: The use of cfDNA to determine the selective use of antenatal RhIG would not be cost-effective in the United States. Universal antenatal RhIG without paternity is more effective in preventing new cases of alloimmunization than the current ACOG guideline.

Recipient umbilical artery elongation (redundancy) in twin-twin transfusion syndrome.

BACKGROUND: Chronic hypertension in adults causes arterial lengthening in major arteries, but the effects of early fetal hypertension on the twin-twin transfusion syndrome recipient's vascular architecture remains unknown. OBJECTIVE: We hypothesize that arterial cord redundancy is related to recipient hypertension and subsequent heart failure. Our objectives were to: (1) establish a 3-dimensional color Doppler ultrasound method of measuring umbilical arterial length relative to its corresponding venous segment in the umbilical cord using artery vein angle; (2) compare recipient artery vein angle to gestational age-matched controls; and (3) test the association of artery vein angle with recipient heart failure. STUDY DESIGN: We compared 3 groups prospectively: twin-twin transfusion syndrome pregnancies undergoing fetoscopic laser surgery (preoperatively) and 2 groups of gestational age-matched controls: uncomplicated monochorionic-diamniotic twin pregnancies and healthy singletons. Using a 3-dimensional color-Doppler volume image of 5 cm of cord near the placental insertion, we traced the umbilical artery and vein producing umbilical artery:vein length, (artery vein index) and measured the artery vein angle between umbilical artery and vein. Correlation of artery vein angle to twin-twin transfusion syndrome stage, maximum vertical pocket, umbilical arterial indices, ductus venosus Doppler, and brain natriuretic peptide were performed. We used pulsed-wave and tissue Doppler to measure tissue Doppler velocities and indexed cardiac output and correlated these with artery vein angle. Comparative statistics, including multivariable linear regression, examined the relationship between umbilical arterial Doppler indices and artery vein angle. RESULTS: Artery vein angle and artery vein index correlated significantly (R2, 0.86; P < .0001), hence, artery vein angle was used for analysis. Mean artery vein angle was 33.1 ± 31.5 degrees in recipients (n = 44), 9.5 ± 6 degrees in monochorionic-diamniotic (n = 11; 22 fetuses), and 8.9 ± 8.3 degrees in singleton controls (n = 16) (P < .001). An artery vein angle ≥26 degrees (>95th percentile for controls) was measured in 52% recipients. Artery vein angle was higher in twin-twin transfusion syndrome stage 3R vs 1 (P = .001). Artery vein angle increased with increasing umbilical arterial pulsatility index (P < .001), and decreased with increasing resistance index (P = .02) after adjusting for gestational age. Interrater agreements to categorize abnormal artery vein angle values was 95% (P < .001). Abnormal ductus venosus Doppler and elevated recipient amniotic fluid N-terminal pro-brain natriuretic peptide/protein levels correlated significantly with artery vein angle. Abnormal artery vein angles were associated with decreased indexed cardiac output, lower tissue Doppler velocities, higher right-sided Tei indices, and severe tricuspid regurgitation. CONCLUSION: Umbilical arterial lengthening occurs in 52% of recipients and is associated with abnormal Doppler flows, low systolic tissue Doppler velocities, reduced cardiac output, and elevated markers of cardiac failure. This may reflect chronicity and severity of hypertension in the recipient fetus. Further research is needed to explore the mechanisms of elongation and long-term implications.

Correlating Prenatal Imaging Findings of Fetal Ventriculomegaly with the Need for Surgical Intervention in the First 3 Months after Birth.

BACKGROUND/AIMS: This study evaluates the predictive value of prenatal imaging measurements regarding the need for cerebrospinal fluid (CSF) diversion for fetal hydrocephalus in the first 3 months after birth. METHODS: We retrospectively reviewed a consecutive case series of patients with fetal hydrocephalus from January 2011 to December 2014 (n = 45). Prenatal measurements included head circumference (HC), biparietal diameter (BPD), and lateral ventricle (LV) width. Patients requiring CSF diversion within 12 weeks of birth were compared to those who did not require CSF diversion using the Wilcoxon rank sum test, and receiver-operating characteristic analysis was used to evaluate threshold values. RESULTS: CSF diversion was required within 12 weeks of birth in 30 of 45 patients. Mean LV width (mm) during the entire pregnancy was greater for the surgery group than the nonsurgery group. Neither BPD nor HC showed differences between the groups. A mean LV size ≥15 mm predicted the need for CSF diversion with a sensitivity of 67% and specificity of 73%. CONCLUSION: LV width is the prenatal imaging measurement that best predicts the need for postnatal CSF diversion.

Survey of Parent Experiences in Prenatal Visits for Infants With Cleft Lip and Palate.

OBJECTIVE: The purpose of this study was to identify the factors that influence the parent's choice of cleft team/surgeon. DESIGN: A 10-question survey was used to elucidate factors that influenced parents in choosing their cleft surgeon. No identifiers of the origin of the study were used to improve parent objectivity. SETTING: The setting for this study was an online survey. PARTICIPANTS: Participants in this study were the parents of children who were born with cleft lip and/or palate. INTERVENTIONS: Parents were contacted anonymously via national, established social media websites that were independently run by the parents themselves. MAIN OUTCOME MEASURES: The main outcome measures were information regarding choice of cleft team/surgeon, source of patient referrals, and use of online media in decision making. RESULTS: A total of 112 responses were received. Of the parents, 77% sought prenatal evaluation with at least one cleft surgeon. Maternal-fetal medicine specialists were the most frequent (42%) referral source, followed by primary obstetricians (14%) and pediatricians (12%). The surgeon/cleft team's experience level and overall personality were ranked as the most important, whereas the least important was distance traveled. Of the parents, 95% used the Internet or social media for research prior to their prenatal visit; 96% of the parents found the prenatal visit helpful, and the most useful topics discussed were treatments (surgical, nonsurgical) and feeding techniques. CONCLUSION: This study identifies factors used to choose a cleft team/surgeon. Parents are more concerned with the experience level, reputation, and environment of the cleft team/surgeon than the distance traveled to get to the center.

Selective reduction in complex monochorionic gestations.

The early determination of chorionicity in twin gestations is a critical factor in the management of twin gestations. While twins and higher order multiple gestations have increased risks of almost any complication of pregnancy, those pregnancies involving a monochorionic (MC) twin pair are at an especially high risk. The presence of vascular anastomoses within a MC placenta creates risk for discordant malformations, twin reversed arterial perfusion sequence, twin-to-twin transfusion syndrome, or severe selective intrauterine growth restriction. While selective reduction of higher order multiple pregnancies is performed to reduce pregnancy-related risks and improve overall outcomes, selective reduction is also an important management strategy in situations where MC pregnancies develop complex complications. This procedure can be lifesaving for the cotwin by preventing intrauterine demise or extreme prematurity. There are several techniques that can be considered, each with its own advantages and disadvantages.

Laser ablation of placental anastomoses in twin-to-twin transfusion syndrome: preoperative predictors of death by recursive partitioning.

OBJECTIVE: The aim of this study was to develop a simple clinical algorithm for prediction of donor and recipient death using 'yes'or 'no' questions through the process of recursive partitioning for patients undergoing laser therapy for twin to twin transfusion syndrome (TTTS). The intent was to identify a subset of patients with very high specificity to whom clinical decisions would be simplified. METHOD: Secondary analysis of data retrospectively collected from laser procedures was performed for TTTS at NAFTNet centers from 2002 to 2009. Preoperative factors associated with donor and recipient death were identified by recursive partitioning regression analysis. Classification And Regression Trees (CARTs) were developed to refine specificity for prediction of death. RESULTS: There were 466 TTTS patients from eight centers. CARTs were obtained for prediction of donor death. Improved specificity was achieved through recursive partitioning as demonstrated in receiver operator characteristic curves for prediction of death of the donor. There was less than optimal predictive ability for prediction of death in the recipient, as demonstrated by lack of generation of CARTs. CONCLUSION: Recursive partitioning improves the specificity and refines the prediction of donor fetal and neonatal demise in TTTS treated with laser therapy. This has the potential to improve therapeutic choices and refine counseling regarding outcomes.

Twin reversed arterial perfusion sequence: a new method of parabiotic twin mass estimation correlated with pump twin compromise.

OBJECTIVES: The purpose of this study was to test the hypothesis that using the formula of a prolate ellipsoid to estimate parabiotic twin mass correlates better with findings of pump twin compromise than using the sonographic method of Moore et al (Am J Obstet Gynecol 1990; 163:907-912). METHODS: A 10-year retrospective review was performed to identify all cases of the suspected twin reversed arterial perfusion (TRAP) sequence. Parabiotic twin mass was estimated by summing body and extremity volumes calculated using the prolate ellipsoid formula (width × height × length × 0.523). Parabiotic twin mass was also estimated using the sonographic Moore method [1.21 × length(2) - (1.66 × length)]. Parabiotic twin mass estimated by both methods was correlated with sonographic findings associated with increased risk of pump twin compromise. RESULTS: Fifty-nine pregnancies complicated by TRAP were identified. Using the prolate ellipsoid formula, the parabiotic twin mean sizes ± SD (as a percentage of pump twin weight) were 103.0% ± 52.0% and 56.9% ± 44.3% in cases with and without pump twin compromise (P = .0005), respectively. Using the sonographic Moore method, the mean parabiotic twin sizes were 122.9% ± 54.3% and 99.6% ± 62.8% in cases with and without pump twin compromise (P = .14). The median estimated masses of the parabiotic twin were 197 ± 219 g using the prolate ellipsoid formula and 310 ± 212 g using the sonographic Moore method (P = .0001). A parabiotic twin size greater than 70% of the pump twin correlated with findings associated with increased risk of pump twin compromise when using the prolate ellipsoid formula (P = .002) but not the sonographic Moore method (P = .09). CONCLUSIONS: Sonographic findings associated with increased risk of pump twin compromise correlate better with prolate ellipsoid estimates of parabiotic twin mass. The median estimated mass of the parabiotic twin was statistically larger when calculated by the sonographic Moore method than by the prolate ellipsoid formula.

The North American Fetal Therapy Network Registry data on outcomes of radiofrequency ablation for twin-reversed arterial perfusion sequence.

OBJECTIVE: To report the North American Fetal Therapy Network (NAFTNet) Registry data on the outcomes of using radiofrequency ablation to treat twin-reversed arterial perfusion (TRAP). METHODS: This was a retrospective review of the records of all patients who underwent percutaneous radiofrequency ablation of an acardiac twin after referral to 12 NAFTNet institutions between 1998 and 2008. Maternal, fetal and neonatal data were analyzed. The primary outcome was neonatal survival to 30 days of age. All participating sites conducted this study under institutional review board approval. RESULTS: Of the 98 patients identified, there were no maternal deaths and no women required blood transfusions. Most women (76 of 98; 78%) stayed in the hospital for ≤1 day after the procedure. Mean gestational age at delivery was 33.4 weeks overall and 36.0 weeks for survivors. Median gestational age at delivery was 37.0 weeks. Survival of the pump twin to 30 days was 80% in the overall cohort. CONCLUSION: The NAFTNet registry data suggest that radiofrequency ablation of the acardiac twin is an effective treatment for TRAP sequence.

Open and endoscopic fetal myelomeningocele surgeries display similar in-hospital safety profiles in a large, multi-institutional database.

BACKGROUND: Open intrauterine fetal myelomeningocele repair has demonstrated decreased ventriculoperitoneal shunting and improved motor outcomes despite maternal and fetal risks. Few data directly compare the safety of open vs endoscopic approaches. OBJECTIVE: This study aimed to analyze in-hospital maternal and fetal outcomes of pregnant patients undergoing open vs endoscopic fetal myelomeningocele repair using a large, multi-center database. STUDY DESIGN: This was a review of the Pediatric Health Information System database from October 1, 2015, to December 31, 2021. All patients who underwent open or endoscopic fetal myelomeningocele repair according to the International Classification of Diseases, Tenth Revision, were identified. Demographics, gestational age, and outcomes were analyzed. Descriptive and univariate statistics were used. RESULTS: A total of 378 pregnant patients underwent fetal myelomeningocele repair. The approach was endoscopic in 143 cases (37.8%) and open in 235 cases (62.2%). Overall postprocedural outcomes included no maternal in-hospital mortalities or intensive care unit admissions, a median length of stay of 4 days (interquartile range, 4-5), 14 cases (3.7%) of surgical and postoperative complications, 6 cases (1.6%) of intrauterine infections, 12 cases (3.2%) of obstetrical complications (including preterm premature rupture of membranes), 3 cases (0.8%) of intrauterine fetal demise, and 16 cases (4.2%) of preterm delivery. Compared with an open approach, the endoscopic approach occurred at a later gestational age (25 weeks [interquartile range, 24-25] vs 24 weeks [interquartile range, 24-25]; P<.001) and had an increased rate of intrauterine infection (6 [4.2%] cases vs 0 [0%] case; P=.002). There was no difference between approaches in the rates of surgical complications, obstetrical complications, intrauterine fetal demise, or preterm deliveries. CONCLUSION: Compared with an open approach, endoscopic fetal myelomeningocele repair displays a comparable rate of fetal complications, including intrauterine fetal demise and preterm delivery, and a similar in-hospital maternal safety profile despite an association with increased intrauterine infection.

Cerclage for cervical shortening at fetoscopic laser photocoagulation in twin-twin transfusion syndrome.

OBJECTIVE: The purpose of this study was to evaluate the benefit of cervical cerclage for cervical length ≤ 25 mm at the time of fetoscopic laser photocoagulation (FLP) for twin-twin transfusion syndrome. STUDY DESIGN: A multicenter, retrospective cohort study was conducted with 163 patients with a short cervix before FLP for twin-twin transfusion syndrome. Seventy-nine of the patients (48%) had cerclage placement at the surgeon's discretion. The outcome measures that were compared were gestational age at delivery and perinatal mortality rates for patients with cerclage and those who were treated conservatively. Outcomes were evaluated with the use of comparative statistics. RESULTS: There were no differences in the preoperative variables, except cerclage was performed more frequently for a cervical length of ≤ 15 mm (P < .001). There were no differences in the gestational age at delivery (28.8 ± 5.4 vs 29.1 ± 5.6 weeks with and without cerclage, respectively; P = .15); perinatal mortality rates were similar between the 2 groups. CONCLUSION: The benefit of cerclage for patients with short cervix before FLP remains questionable.

Use of magnetic resonance imaging in prenatal prognosis of the fetus with isolated left congenital diaphragmatic hernia.

OBJECTIVES: To investigate the prognostic value of magnetic resonance-calculated fetal lung volumes (FLV) in fetuses with isolated left congenital diaphragmatic hernia (L-CDH) who receive standardized prenatal and postnatal care at a single institution. MATERIALS AND METHODS: A retrospective review was undertaken to identify fetuses with isolated L-CDH between 2001 and 2010. RESULTS: Eighty-five cases of isolated L-CDH were identified. The overall survival was 65% (55/85). Survival was 45% if there was 'liver up' (23/51) and 94% if there was 'liver down' (32/34). Univariate statistical analysis showed that 'liver up' (p=0.001), lung-to-head ratio (LHR) at diagnosis (p=0.009), observed/expected (O/E) LHR (p=0.01), total FLV (p=0.03), right LV (p=0.04), magnetic resonance imaging (MRI) observed versus expected (O/E) FLV (p=0.002), intrathoracic versus intraabdominal stomach (p=0.002), percentage of herniated liver (p=0.004), and postnatal extracorporeal membrane oxygenation use (p=0.001) are predictive of postnatal survival. Multivariate analysis of only prenatal factors showed that the most important determinants of postnatal outcome are percentage of herniated liver, presence of liver up, and MRI O/E FLV. CONCLUSION: Herniated intrathoracic liver expressed as 'liver up' or as percent herniated liver and MRI measurement of FLV expressed as a O/E ratio are strong prenatal indicators of postnatal survival.

Low-dose fetal CT for evaluation of severe congenital skeletal anomalies: preliminary experience.

Congenital skeletal abnormalities compose a heterogeneous and complex group of conditions that affect bone growth and development and result in various anomalies in shape and size of the skeleton. Prenatal sonographic diagnosis of these anomalies is challenging because of the relative rarity of each skeletal dysplasia, the multitude of differential diagnoses encountered when the bony abnormalities are identified, lack of precise molecular diagnosis and the fact that many of these disorders have overlapping features and marked phenotypic variability. The following review is a preliminary summary of our experience at the Children's Hospital of Philadelphia (CHOP) using low-dose fetal CT in the evaluation of severe fetal osseous abnormalities.

Speckle tracking-derived myocardial tissue deformation imaging in twin-twin transfusion syndrome: differences in strain and strain rate between donor and recipient twins.

OBJECTIVES: Twin-twin transfusion syndrome (TTTS) is a complex disorder with altered cardiovascular loading conditions that affects both donors and recipients. Myocardial tissue deformation analysis using vector velocity imaging is an angle-independent, speckle-tracking technique which can assess myocardial mechanics and may provide insight into cardiac dysfunction in TTTS. METHODS: Digital dynamic two-dimensional four-chamber views were interrogated offline. Images were acquired utilizing standard video frame rates (30 frames/s). The global longitudinal strain, systolic strain rate, and diastolic strain rate were measured in the left (LV) and right ventricles (RV) of 25 fetal pairs with TTTS and compared to 25 gestational age-matched normal controls. Pulsatility indices for the umbilical artery and middle cerebral artery were measured. RESULTS: The gestational age at evaluation was 20.5 ± 1.3 weeks. The donor LV systolic strain rate was higher, while the donor RV diastolic strain rate was significantly lower, than control values. The recipient longitudinal strain, systolic strain rate, and diastolic strain rate were significantly lower for both LV and RV in comparison to controls. The donor umbilical artery pulsatility index was higher than control values (1.92 ± 0.45 vs. 1.41 ± 0.25, p < 0.001), while the donor middle cerebral artery pulsatility index was lower (1.46 ± 0.28 vs. 1.87 ± 0.21). Recipient umbilical artery and middle cerebral artery pulsatility indices were no different than control values. CONCLUSIONS: In TTTS, both the donor and the recipient exhibit abnormalities of myocardial tissue deformation with ventricle-specific changes evident based on loading conditions. Donor LV systolic function is hyperdynamic due to hypovolemia and selective ejection into a low-resistance cerebrovascular circuit while the donor RV selectively ejects into a high-resistance placental circuit. Recipient RV and LV are both globally depressed with systolic and diastolic dysfunction. Further prospective validation of our findings using high frame rate analysis is indicated.

Fetal MRI-calculated total lung volumes in the prediction of short-term outcome in giant omphalocele: preliminary findings.

OBJECTIVE: To determine the value of fetal MRI-calculated total lung volumes (TLV) in the prediction of short-term outcome in patients with giant omphalocele (GO). MATERIAL AND METHODS: We reviewed all cases of GO undergoing fetal MRI after 21 weeks' gestation and receiving postnatal care at our institution between 2003 and 2010. Observed/expected (O/E) TLV was calculated using age-matched TLV normograms [Radiology 2001;219:236-241]. Postnatal outcomes were stratified based on O/E TLV above or below 50% of expected. RESULTS: Seventeen GO cases fulfilled the entry criteria. The mean age at fetal MRI evaluation was 25.8 ± 4.8 weeks' gestation. The mean GO TLV (21.0 ± 13.2) was lower than age-matched population norms (p < 0.001), resulting in a mean O/E TLV of 52.3 ± 16.8%. The mean gestational age at delivery was 36.8 ± 1.6 weeks. Overall survival was 94%. Fourteen (88%) infants underwent staged reduction, and 2 underwent silver sulfadiazine treatment and delayed repair. Infants with ≤50% of predicted O/E TLV (n = 11, 65%) had lower Apgar scores at birth (p = 0.03), prolonged ventilatory support (p = 0.004), delayed oral intake (p = 0.03), and longer hospitalization (p = 0.03) compared to patients with ≥50% of expected O/E TLV. Two infants (both O/E TLV <50%) required tracheostomy placement. CONCLUSION: In the assessment of GO fetuses, MRI-based O/E TLV of <50% was predictive of increased postnatal morbidity.

Amniotic fluid levels of glial fibrillary acidic protein in fetal rats with retinoic acid induced myelomeningocele: a potential marker for spinal cord injury.

OBJECTIVE: The objective of this study was to determine whether amniotic fluid levels of glial acidic fibrillary protein (GFAP) would reflect myelomeningocele-related neurodegeneration in the rat model of retinoic acid-induced myelomeningocele, which is a model that is very similar to human myelomeningocele and develops the entire spectrum of disease severity including features of the Chiari II malformation. STUDY DESIGN: Time-dated (embryonic day 10) pregnant Sprague-Dawley rats were gavage fed 60 mg/kg/bodyweight retinoic acid that had been dissolved in olive oil or olive oil alone. Myelomeningocele, retinoic acid-exposed no myelomeningocele, and control fetuses were harvested at specific time points throughout gestation. A standard set of pinching tests was performed to interrogate the sensorimotor reflex arc of hindpaws and tails. Amniotic fluid-GFAP levels were analyzed by standard enzyme-linked immunosorbent assay techniques. RESULTS: Amniotic fluid-GFAP levels were similar between groups at embryonic days 14, 16, and 18, respectively. Compared with control fetuses, amniotic fluid GFAP levels were significantly increased in myelomeningocele fetuses at embryonic days 20 and 22 (P < .001). Defect size (P < .001), presence of clubfoot deformity (P = .0004), and absence of sensorimotor function (P < .01) at embryonic day 22 correlated with amniotic fluid-GFAP levels. CONCLUSION: Amniotic fluid-GFAP levels appear to correlate with spinal cord injury as gestation proceeds in fetal rats with myelomeningocele.