Pathological Findings in Fetuses Terminated for Suspected Lower Urinary Tract Obstruction: Experience From a High-Risk Fetal Center in Canada.

PURPOSE: Pregnancies complicated by prenatally suspected lower urinary tract obstruction (LUTO) can be associated with high rates of terminations due to potentially poor outcomes. Herein, we assessed autopsy findings of fetuses terminated for suspected LUTO to evaluate the prenatal diagnostic accuracy and spectrum of underlying pathologies. MATERIALS AND METHODS: We performed a retrospective review of all pregnancies referred to a high-risk fetal center in a universal access to care health care system for suspected LUTO that opted for termination of pregnancy between 2009 and 2022. Ultrasound features, genetic investigations, placental findings, and distribution of postmortem diagnoses were assessed. RESULTS: Of a total of 190 pregnancies with suspected LUTO evaluated during the study period, 79 (42%) were terminated. We excluded 35 fetuses with incomplete data, resulting in 44 available for analysis. Pregnancies were terminated at a mean gestation of 22 ± 5 weeks. A LUTO diagnosis was confirmed in 37 (84.1%) fetuses (35 males, 2 females), and the remaining 7 showed other pathologies. Pulmonary hypoplasia was found in 62.2% (n = 23) and placental pathologies in 56.8% of confirmed LUTO compared to 33.4% and 71.4% in non-LUTO cases, respectively. Overall, a total of 31 fetuses underwent additional prenatal investigations with genetic anomalies detected only in fetuses with a confirmed LUTO diagnosis (13.6%). CONCLUSIONS: In our health care system, almost half of prenatally suspected LUTO pregnancies are terminated. The sonographic diagnostic accuracy for LUTO is reasonable at 84%. However, the remaining 16% still had significant pathologies. Genetic abnormalities are uncommon and rarely the trigger for pregnancy terminations.

Deep-learning computer vision can identify increased nuchal translucency in the first trimester of pregnancy.

OBJECTIVE: Many fetal anomalies can already be diagnosed by ultrasound in the first trimester of pregnancy. Unfortunately, in clinical practice, detection rates for anomalies in early pregnancy remain low. Our aim was to use an automated image segmentation algorithm to detect one of the most common fetal anomalies: a thickened nuchal translucency (NT), which is a marker for genetic and structural anomalies. METHODS: Standardized mid-sagittal ultrasound images of the fetal head and chest were collected for 560 fetuses between 11 and 13 weeks and 6 days of gestation, 88 (15.7%) of whom had an NT thicker than 3.5 mm. Image quality was graded as high or low by two fetal medicine experts. Images were divided into a training-set (n = 451, 55 thick NT) and a test-set (n = 109, 33 thick NT). We then trained a U-Net convolutional neural network to segment the fetus and the NT region and computed the NT:fetus ratio of these regions. The ability of this ratio to separate thick (anomalous) NT regions from healthy, typical NT regions was first evaluated in ground-truth segmentation to validate the metric and then with predicted segmentation to validate our algorithm, both using the area under the receiver operator curve (AUROC). RESULTS: The ground-truth NT:fetus ratio detected thick NTs with 0.97 AUROC in both the training and test sets. The fetus and NT regions were detected with a Dice score of 0.94 in the test set. The NT:fetus ratio based on model segmentation detected thick NTs with an AUROC of 0.96 relative to clinician labels. At a 91% specificity, 94% of thick NT cases were detected (sensitivity) in the test set. The detection rate was statistically higher (p = 0.003) in high versus low-quality images (AUROC 0.98 vs. 0.90, respectively). CONCLUSION: Our model provides an explainable deep-learning method for detecting increased NT. This technique can be used to screen for other fetal anomalies in the first trimester of pregnancy.

The Toronto nomogram: A Bayesian meta-regression derived prenatal ultrasound index to predict lower urinary tract obstruction and prune belly syndrome.

INTRODUCTION: A nomogram for predicting the diagnosis of lower urinary tract obstruction (LUTO) based on an antenatal ultrasound index generated from a Bayesian Meta-regression analysis has been in development and noted with superior diagnostic accuracy compared to the keyhole sign (KHS). We aim to assess the accuracy of the nomogram in expanded diagnostic utilization to predict LUTO. METHODOLOGY: The validation of the nomogram for expanded diagnostic utilization was based on data from a prospective institutional antenatal clinic database between January 2020 and June 2022. Diagnostic accuracy indices were determined for confirmed postnatal diagnosis of LUTO or prune belly syndrome (PBS). Receiver operating characteristics (ROC) curves were generated to compare the area under the curve (AUC) of the nomogram versus KHS. RESULTS: Based on 84 male fetuses with antenatal ultrasound of moderate-severe hydronephrosis (PUV n = 15, PBS n = 4), the KHS had 26.3% (95%CI 9.1-51.2) sensitivity and 100% (95%CI 94.4%-100%) specificity, with 14 false-negatives. The nomogram showed a 84.2 (95%CI 60.4%-96.6%) sensitivity and 95.4 (95%CI 87.1%-99%) specificity with three false-positives. The nomogram also had a superior AUC compared to KHS (0.98 vs. 0.63). CONCLUSION: The nomogram can be used as a valuable tool to trigger further postnatal screening and provide individualized risk assessments to families during prenatal counseling.

Contemporary Outcomes of a National Fetal Spina Bifida Surgery Service.

OBJECTIVE: To assess contemporary outcomes of fetuses who underwent open fetal spina bifida surgery in Canada. METHODS: Our clinical program prospectively collected outcomes of all consecutive fetuses who underwent open fetal spina bifida closure at the Ontario Fetal Center in Toronto and who were at least 1 year of age at the time of postnatal follow-up. We gathered information on the need for hydrocephalus treatment, motor function, bladder function, as well as neurodevelopment (Ages and Stages Questionnaire and Bayley's scales of infant development). Developmental outcomes were categorized as "Typical Development," "Possible Delay," or "Significantly Delayed." RESULTS: Between 2017 and 2022, 41 fetuses underwent open fetal spina bifida closure. Twenty-four patients (58.5%) responded to the questionnaire at a median age of 46.5 months. Eight children (33.3%) required CSF diversion procedures. Bladder management included clean intermittent catheterization (43.5%), spontaneous voiding (34.8%), or both (21.7%), with 43.5% needing medication for overactive bladder. All patients could sit independently, with 50% walking outside and 50% crawling indoors. Among those walking outdoors (50%), 25% did so without orthotics or aid, 58.3% with orthotics, and 16.7% required additional walking aids. Most children demonstrated typical communication and problem-solving skills (79.2%), while gross motor development was significantly delayed in 91.7% of cases. Fine motor skills varied, with 56.5% showing typical development and 34.8% possibly experiencing delays. CONCLUSIONS: This study showed a mixed developmental profile among patients who underwent open fetal spina bifida repair, consistent with the MOMs trial findings.

Maternal "mirror" syndrome: Evaluating the benefits of fetal therapy.

OBJECTIVE: To evaluate maternal and perinatal outcomes following fetal intervention in the context of maternal "mirror" syndrome. STUDY DESIGN: A multicenter retrospective study of all cases of fetal hydrops complicated by maternal "mirror" syndrome and treated by any form of fetal therapy between 1995 and 2022. Medical records and ultrasound images of all cases were reviewed. "Mirror" syndrome was defined as fetal hydrops and/or placentomegaly associated with the maternal development of pronounced edema, with or without pre-eclampsia. Fetal hydrops was defined as the presence of abnormal fluid collections in ≥2 body cavities. RESULTS: Twenty-one pregnancies met the inclusion criteria. Causes of fetal hydrops and/or placentomegaly included fetal lung lesions (n = 9), twin-twin transfusion syndrome (n = 6), severe fetal anemia (n = 4), and others (n = 2). Mean gestational age at "mirror" presentation was 27.0 ± 3.8 weeks. Maternal "mirror" syndrome was identified following fetal therapeutic intervention in 14 cases (66.6%). "Mirror" symptoms resolved or significantly improved before delivery in 8 (38.1%) cases with a mean interval from fetal intervention to maternal recovery of 13.1 days (range 4-35). Three women needed to be delivered because of worsening "mirror" syndrome. Of the 21 pregnancies treated (27 fetuses), there were 15 (55.5%) livebirths, 7 (25.9%) neonatal deaths and 5 (18.5%) intra-uterine deaths. CONCLUSION: Following successful treatment and resolution of fetal hydrops, maternal "mirror" syndrome can improve or sometimes completely resolve before delivery. Furthermore, the recognition that "mirror" syndrome may arise only after fetal intervention necessitates hightened patient maternal surveillance in cases of fetal hydrops.

Implementation of Multiple Marker Screening for Preterm Preeclampsia in a Single Tertiary Obstetric Centre.

OBJECTIVES: Early assessment of pregnant individuals for risk of preterm preeclampsia (PE) is possible at the 11-14 week ultrasound visit using a validated multiple marker algorithm, allowing timely use of preventative low-dose acetylsalicylic acid (LDA) in high-risk patients. With no established early screening program for preterm PE in Canada, our objectives were to assess the acceptability and operational impact of routine screening for preterm PE during the 11-14 week ultrasound visit, evaluate uptake and adherence to LDA when recommended, and assess screening performance. METHODS: A prospective implementation study of preterm PE screening among pregnant patients at the ultrasound unit of a tertiary obstetric centre in Toronto, Canada. RESULTS: A total of 1057 patients were screened, with an acceptance rate of 87.1%. First-trimester ultrasound appointment time increased by a median time of 7 minutes (Interquartile range 6-9). By 16 weeks gestation, 88.7% of high-risk patients had started LDA, with adherence of 88.7%‒94.6% from 16‒36 weeks. Satisfaction with counselling was ≥7/10 in more than 95% of patients. There were 7 cases of preterm PE (0.73%), 3 in the low-risk group (0.35%), and 4 in the high-risk group (4.1%). When accounting for LDA use, the treatment-adjusted detection rate was 78.6%. CONCLUSIONS: We demonstrate successful implementation of a validated, effective screening and prevention program for preterm PE as a first step in the implementation of a broader program adaptable for cultural, access/equity considerations, and marker availability.

Revisiting the Implications of a Wide or Narrow Fetal Cavum Septi Pellucidi.

OBJECTIVES: To investigate short-term neonatal developmental outcomes in fetuses with an isolated wide or narrow cavum septi pellucidi (CSP) using new reference ranges. METHODS: A cross-sectional study on fetuses at 16 + 0 to 36 + 6 weeks of gestation between December 2020 and January 2022. CSP width reference ranges were constructed from low-risk pregnancies. Wide and narrow CSPs were defined as measurements above the 95th percentile and below the 5th percentile, respectively. For the primary outcome fetuses with normal neurosonograms were included. Neonatal developmental outcomes were assessed using the Survey of Well-being of Young Children (SWYC). RESULTS: A total of 352 fetuses were included in this study, of whom 138 were healthy and had uncomplicated neonatal outcomes. These fetuses constituted the control group and were used to construct the CSP width reference ranges. Of 185 fetuses in the neurosonography group, 9.7% had wide and 7.6% had narrow CSPs, of whom 33.3% and 22.2%, respectively, scored below the SWYC threshold for expected developmental milestones, a rate similar to that reported in the general population. CONCLUSIONS: The presence of a prenatally isolated wide or narrow CSP does not appear to increase the risk of neonatal neurodevelopmental delay.

Elevated Middle Cerebral Artery Peak Systolic Velocity in Non-Anemic Fetuses: Providing a Better Understanding of Enigmatic Middle Cerebral Artery Peak Systolic Velocity.

INTRODUCTION: Our aim was to investigate the incidence, comorbidities, and outcomes of fetuses with an elevated middle cerebral artery peak systolic velocity (MCA-PSV) >1.5 multiples of median (MoM), despite normal hemoglobin (Hgb) levels on fetal blood sampling (FBS). METHODS: A single-center observational retrospective cohort study of all fetuses undergoing FBS and MCA-PSV >1.5 MoM. Only those fetuses with no or mild anemia were included. Indications for Doppler assessment, associated anomalies, and neonatal outcomes were collected. RESULTS: Overall, 383 fetuses had an MCA-PSV >1.5 MoM and underwent FBS. Twenty-three (6%) fetuses met our inclusion criteria and had no or only mild anemia. Associations with elevated MCA-PSV were elucidated in 12 of the 23 cases (52.2%) and included mild anemia (n = 2), intracranial hemorrhage (n = 3), genetic disease (n = 1), idiopathic nonimmune hydrops (NIH, n = 1), hypoxic-ischemic encephalopathy (n = 1), maternal and or fetal acidosis (n = 3), and fetal growth restriction (n = 1). Favorable perinatal outcomes were observed in truly unexplained 11 cases with no additional anomalies (47.8%). CONCLUSION: Elevated MCA-PSV >1.5 MoM with normal Hgb levels is seen in 6% of pregnancies undergoing FBS and is often associated with other significant maternal or fetal problems. Those with unexplained and isolated MCA-PSV elevation have normal outcomes.

Intravenous immunoglobulin in the management of severe early onset red blood cell alloimmunisation.

Our objective was to assess the effect of maternal intravenous immunoglobulin (IVIG) administration for severe red blood cell (RBC) alloimmunisation on fetal outcomes. This is a case-control study. Women with a history of severe early onset alloimmunisation resulting in fetal loss in a previous pregnancy and high anti-D or anti-K antibody titres received IVIG in a subsequent pregnancy. We assessed gestational age at first transfusion and fetal outcomes in the subsequent pregnancy and compared these with the outcomes in the previous pregnancy. The most responsible antibody was anti-D in 17 women and anti-K in two others, whilst seven had more than one antibody. In all, 19 women received IVIG in 22 pregnancies, two of which did not even need an intrauterine transfusion (IUT). For previous early losses despite transfusion, IVIG was associated with a relative increase in fetal haemoglobin between treated and untreated pregnancies of 36.5 g/L (95% confidence interval 19.8-53.2, p = 0.0013) and improved perinatal survival (eight of eight vs. none of six, p = 0.001). For previous losses at <20 weeks, it enabled first transfusion deferral in subsequent pregnancies to at least 19.9 weeks (mean 23.2 weeks). Overall, IVIG decreases the severity of haemolytic disease of the fetus and newborn and allows deferral of the first IUT to a safer gestation in severe early-onset RBC alloimmunisation and rarely may even avoid the need for IUT entirely.

National and international guidelines on the management of twin pregnancies: a comparative review.

Twin gestations are associated with increased risk of pregnancy complications. However, high-quality evidence regarding the management of twin pregnancies is limited, often resulting in inconsistencies in the recommendations of various national and international professional societies. In addition, some recommendations related to the management of twin gestations are often missing from the clinical guidelines dedicated to twin pregnancies and are instead included in the practice guidelines on specific pregnancy complications (eg, preterm birth) of the same professional society. This can make it challenging for care providers to easily identify and compare recommendations for the management of twin pregnancies. This study aimed to identify, summarize, and compare the recommendations of selected professional societies from high-income countries on the management of twin pregnancies, highlighting areas of both consensus and controversy. We reviewed clinical practice guidelines of selected major professional societies that were either specific to twin pregnancies or were focused on pregnancy complications or aspects of antenatal care that may be relevant for twin pregnancies. We decided a priori to include clinical guidelines from 7 high-income countries (United States, Canada, United Kingdom, France, Germany, and Australia and New Zealand grouped together) and from 2 international societies (International Society of Ultrasound in Obstetrics and Gynecology and the International Federation of Gynecology and Obstetrics). We identified recommendations regarding the following care areas: first-trimester care, antenatal surveillance, preterm birth and other pregnancy complications (preeclampsia, fetal growth restriction, and gestational diabetes mellitus), and timing and mode of delivery. We identified 28 guidelines published by 11 professional societies from the 7 countries and 2 international societies. Thirteen of these guidelines focus on twin pregnancies, whereas the other 16 focus on specific pregnancy complications predominantly in singletons but also include some recommendations for twin pregnancies. Most of the guidelines are recent, with 15 of the 29 guidelines published over the past 3 years. We identified considerable disagreement among guidelines, primarily in 4 key areas: screening and prevention of preterm birth, using aspirin to prevent preeclampsia, defining fetal growth restriction, and the timing of delivery. In addition, there is limited guidance on several important areas, including the implications of the "vanishing twin" phenomenon, technical aspects and risks of invasive procedures, nutrition and weight gain, physical and sexual activity, the optimal growth chart to be used in twin pregnancies, the diagnosis and management of gestational diabetes mellitus, and intrapartum care.This consolidation of key recommendations across several clinical practice guidelines can assist healthcare providers in accessing and comparing recommendations on the management of twin pregnancies and identifies high-priority areas for future research based on either continued disagreement among societies or limited current evidence to guide care.

International Society for Prenatal Diagnosis 2022 DEBATE: There should be formal accreditation and ongoing quality assurance/review for units offering fetal therapy that includes public reporting of outcomes.

The field of fetal therapy has so far escaped from formal accreditation and quality control. Despite that, current published evidence shows that outcomes of interventions in younger fetal therapy centers are similar to what is achieved in more experienced centers and outcomes of interventions have improved over time. The question however remains what is not being published and what should be the standard of care, given the lack of level 1 evidence from randomized controlled trials for many interventions. Formal collaborative networks such as NAFTnet and others allow for anonymized benchmarking of center outcomes, without publicly shaming (and financially punishing) underperforming centers. Large registries also allow for tracking of rare complications and may result in improved patient outcomes over time. Core outcome sets, which could serve as a basis for outcome reporting, are available for some conditions, but certainly not for all, resulting in communication difficulties between centers. Formal accreditation, quality control, and outcome reporting are hard to implement, expensive, and may result in decreasing access to care by pushing smaller centers out of the market. Despite the existing difficulties, international societies have committed to quality improvement, and fetal therapy programs are strongly recommended to participate in voluntary outcome tracking.

Performance of comprehensive first trimester fetal anatomy assessment.

OBJECTIVE: Ultrasound assessment of the fetal anatomy and fetal echocardiography are feasible in the first trimester of pregnancy. This study was designed to assess the performance of a comprehensive fetal anatomy assessment in a high-risk population at a tertiary fetal medicine unit. METHODS: A retrospective review of high-risk patients undergoing comprehensive fetal anatomy ultrasound assessment between 11 weeks and 13 + 6 weeks of gestation was conducted. Findings of the early anatomy ultrasound scan were compared with those of the second trimester anatomy scan, and birth outcomes or post-mortem results. RESULTS: Early anatomy ultrasounds were performed in 765 patients. The sensitivity of the scan for detecting fetal anomalies compared to the birth outcome was 80.5% (95% CI 73.5-86.3) and specificity was 93.1% (95%CI 90.6-95.2). Positive and negative predictive values were 78.5% (95% CI 71.4-84.6) and 93.9% (95% CI 91.4-95.8), respectively. The most missed and overdiagnosed abnormalities were ventricular septal defects. The second trimester ultrasound had sensitivity of 69.0% (95% CI 55.5-80.5) and specificity of 87.5% (95% CI 84.3-90.2). CONCLUSIONS: In a high-risk population, early assessments had similar performance metrics as the second trimester anatomy ultrasound. We advocate for a comprehensive fetal assessment in the care of high-risk pregnancies.

Simple prenatal imaging predictors for postnatal cerebrospinal fluid diversion surgery in fetuses undergoing in utero surgery for spina bifida.

OBJECTIVES: To determine simple prenatal imaging parameters that can easily be acquired to predict the need for postnatal CSF diversion (PCD) surgery in fetuses undergoing open fetal surgery for open spina bifida (OSB). METHODS: All fetuses with OSB that underwent open fetal surgery between June 2017 and June 2021 with available follow-up outcomes were included. Imaging parameters including clivus-supraocciput angle (CSA) on magnetic resonance imaging, transcerebellar diameter (TCD) and lateral ventricle size (Vp) on ultrasound (US), were collected pre- and postoperatively. The requirement for PCD surgery was determined at 1 year of age. The predictive strength of each parameter was determined by Receiver Operating Characteristic curve analysis. RESULTS: Among 36 babies eligible for the analyses, 41.7% required PCD by one year of age. Pre-operative Vp (AUC 0.71; 95% confidence interval [CI] 0.54-0.88; p = 0.03), TCD (AUC 0.72; 95% CI 0.55-0.89; p = 0.02) and CSA (AUC 0.72; 95% CI 0.51-0.93; p = 0.04) were fair predictors for PCD surgery. After fetal surgery, TCD (AUC 0.93; 95% CI 0.83-1.00; p < 0.0001) and CSA (AUC 0.94; 95% CI 0.83-1.00; p = 0.0005) were outstanding predictors of PCD, whereas post-operative Vp was a fair predictor (AUC 0.71, 95% CI 0.54-0.88, p = 0.03). CONCLUSION: Post-operative CSA and TCD were outstanding predictors for the need for PCD surgery.

Guideline No. 440: Management of Monochorionic Twin Pregnancies.

OBJECTIVE: This guideline reviews the evidence-based management of normal and complicated monochorionic twin pregnancies. TARGET POPULATION: Women with monochorionic twin or higher order multiple pregnancies. BENEFITS, HARMS, AND COSTS: Implementation of these recommendations should improve the management of both complicated and uncomplicated monochorionic (and higher order multiple) twin pregnancies. They will help users monitor monochorionic twin pregnancies appropriately and identify and manage monochorionic twin complications optimally in a timely manner, thereby reducing perinatal morbidity and mortality. These recommendations entail more frequent ultrasound monitoring of monochorionic twins compared to dichorionic twins. EVIDENCE: Published literature was retrieved through searches of PubMed and the Cochrane Library using appropriate MeSH headings (Twins, Monozygotic; Ultrasonography, Prenatal; Placenta; Fetofetal Transfusion; Fetal Death; Fetal Growth Retardation). Results were restricted to systematic reviews, randomized controlled clinical trials, and observational studies. There were no date limits, but results were limited to English or French language materials. VALIDATION METHODS: The content and recommendations were drafted and agreed upon by the principal authors. The Board of the SOGC approved the final draft for publication. The authors rated the quality of evidence and strength of recommendations using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. See online Appendix A (Tables A1 for definitions and A2 for interpretations of strong and conditional [weak] recommendations). INTENDED AUDIENCE: Maternal-fetal medicine specialists, obstetricians, radiologists, sonographers, family physicians, nurses, midwives, residents, and other health care providers who care for women with monochorionic twin or higher order multiple pregnancies. TWEETABLE ABSTRACT: Canadian (SOGC) guidelines for the diagnosis, ultrasound surveillance and management of monochorionic twin pregnancy complications, including TTTS, TAPS, sFGR (sIUGR), acardiac (TRAP), monoamniotic twins and intrauterine death of one MC twin. SUMMARY STATEMENTS: RECOMMENDATIONS.

Directive clinique no 440 : Prise en charge de la grossesse gémellaire monochoriale.

OBJECTIF: Cette directive clinique passe en revue les données probantes sur la prise en charge de la grossesse gémellaire monochoriale normale et compliquée. POPULATION CIBLE: Les femmes menant une grossesse gémellaire ou multiple de haut rang. BéNéFICES, RISQUES ET COûTS: L'application des recommandations de cette directive devrait améliorer la prise en charge des grossesses gémellaires (ou multiples de haut rang) monochoriales compliquées et non compliquées. Ces recommandations aideront les fournisseurs de soins à surveiller adéquatement les grossesses gémellaires monochoriales ainsi qu'à détecter et prendre en charge rapidement les complications associées de façon optimale afin de réduire les risques de morbidité et mortalité périnatales. Ces recommandations impliquent une surveillance échographique plus fréquente en cas de grossesse monochoriale qu'en cas de grossesse bichoriale. DONNéES PROBANTES: La littérature publiée a été colligée par des recherches dans les bases de données PubMed et Cochrane Library au moyen de termes MeSH pertinents (Twins, Monozygotic; Ultrasonography, Prenatal; Placenta; Fetofetal Transfusion; Fetal Death; Fetal Growth Retardation). Les résultats ont été restreints aux revues systématiques, aux essais cliniques randomisés et aux études observationnelles. Aucune date limite n'a été appliquée, mais les résultats ont été limités aux contenus en anglais ou en français. MéTHODES DE VALIDATION: Les auteurs principaux ont rédigé le contenu et les recommandations et ils se sont entendus sur ces derniers. Le conseil d'administration de la SOGC a approuvé la version définitive aux fins de publication. Les auteurs ont évalué la qualité des données probantes et la force des recommandations en utilisant le cadre méthodologique GRADE (Grading of Recommendations Assessment, Development and Evaluation). Voir l'annexe A en ligne (tableau A1 pour les définitions et tableau A2 pour l'interprétation des recommandations fortes et conditionnelles [faibles]). PROFESSIONNELS CONCERNéS: Spécialistes en médecine fœto-maternelle, obstétriciens, radiologues, échographistes, médecins de famille, infirmières, sages-femmes, résidents et autres fournisseurs de soins de santé qui s'occupent de femmes menant une grossesse gémellaire ou multiple de haut rang. RéSUMé POUR TWITTER: Directive canadienne (SOGC) pour le diagnostic, la surveillance échographique et la prise en charge des complications de la grossesse gémellaire monochoriale (p. ex., STT, TAPS, retard de croissance sélectif, cojumeau acardiaque, monoamnionicité et mort d'un jumeau). DÉCLARATIONS SOMMAIRES: RECOMMANDATIONS.

Outcome predictors for maternal red blood cell alloimmunisation with anti-K and anti-D managed with intrauterine blood transfusion.

Red blood cell (RBC) alloimmunisation with anti-D and anti-K comprise the majority of cases of fetal haemolytic disease requiring intrauterine red cell transfusion (IUT). Few studies have investigated which haematological parameters can predict adverse fetal or neonatal outcomes. The aim of the present study was to identify predictors of adverse outcome, including preterm birth, intrauterine fetal demise (IUFD), neonatal death (NND) and/or neonatal transfusion. We reviewed the records of all pregnancies alloimmunised with anti-K and anti-D, requiring IUT over 27 years at a quaternary fetal centre. We reviewed data for 128 pregnancies in 116 women undergoing 425 IUTs. The median gestational age (GA) at first IUT was significantly earlier for anti-K than for anti-D (24·3 vs. 28·7 weeks, P = 0·004). Women with anti-K required more IUTs than women with anti-D (3·84 vs. 3·12 mean IUTs, P = 0·036) and the fetal haemoglobin (Hb) at first IUT was significantly lower (51.0 vs. 70.5 g/l, P = 0·001). The mean estimated daily decrease in Hb did not differ between the two groups. A greater number of IUTs and a slower daily decrease in Hb (g/l/day) between first and second IUTs were predictive of a longer period in utero. Earlier GA at first IUT and a shorter interval from the first IUT until delivery predicted IUFD/NND. Earlier GA and lower Hb at first IUT significantly predicted need for phototherapy and/or blood product use in the neonate. In the anti-K group, a greater number of IUTs was required in women with a higher titre. Furthermore, the higher the titre, the earlier the GA at which an IUT was required in both groups. The rate of fall in fetal Hb between IUTs decreased, as the number of transfusions increased. Our present study identified pregnancies at considerable risk of an unfavourable outcome with anti-D and anti-K RBC alloimmunisation. Identifying such patients can guide pregnancy management, facilitates patient counselling, and can optimise resource use. Prospective studies can also incorporate these characteristics, in addition to laboratory markers, to further identify and improve the outcomes of these pregnancies.

The randomized Tracheal Occlusion To Accelerate Lung growth (TOTAL)-trials on fetal surgery for congenital diaphragmatic hernia: reanalysis using pooled data.

BACKGROUND: Two randomized controlled trials compared the neonatal and infant outcomes after fetoscopic endoluminal tracheal occlusion with expectant prenatal management in fetuses with severe and moderate isolated congenital diaphragmatic hernia, respectively. Fetoscopic endoluminal tracheal occlusion was carried out at 27+0 to 29+6 weeks' gestation (referred to as "early") for severe and at 30+0 to 31+6 weeks ("late") for moderate hypoplasia. The reported absolute increase in the survival to discharge was 13% (95% confidence interval, -1 to 28; P=.059) and 25% (95% confidence interval, 6-46; P=.0091) for moderate and severe hypoplasia. OBJECTIVE: Data from the 2 trials were pooled to study the heterogeneity of the treatment effect by observed over expected lung-to-head ratio and explore the effect of gestational age at balloon insertion. STUDY DESIGN: Individual participant data from the 2 trials were reanalyzed. Women were assessed between 2008 and 2020 at 14 experienced fetoscopic endoluminal tracheal occlusion centers and were randomized in a 1:1 ratio to either expectant management or fetoscopic endoluminal tracheal occlusion. All received standardized postnatal management. The combined data involved 287 patients (196 with moderate hypoplasia and 91 with severe hypoplasia). The primary endpoint was survival to discharge from the neonatal intensive care unit. The secondary endpoints were survival to 6 months of age, survival to 6 months without oxygen supplementation, and gestational age at live birth. Penalized regression was used with the following covariates: intervention (fetoscopic endoluminal tracheal occlusion vs expectant), early balloon insertion (yes vs no), observed over expected lung-to-head ratio, liver herniation (yes vs no), and trial (severe vs moderate). The interaction between intervention and the observed over expected lung-to-head ratio was evaluated to study treatment effect heterogeneity. RESULTS: For survival to discharge, the adjusted odds ratio of fetoscopic endoluminal tracheal occlusion was 1.78 (95% confidence interval, 1.05-3.01; P=.031). The additional effect of early balloon insertion was highly uncertain (adjusted odds ratio, 1.53; 95% confidence interval, 0.60-3.91; P=.370). When combining these 2 effects, the adjusted odds ratio of fetoscopic endoluminal tracheal occlusion with early balloon insertion was 2.73 (95% confidence interval, 1.15-6.49). The results for survival to 6 months and survival to 6 months without oxygen dependence were comparable. The gestational age at delivery was on average 1.7 weeks earlier (95% confidence interval, 1.1-2.3) following fetoscopic endoluminal tracheal occlusion with late insertion and 3.2 weeks earlier (95% confidence interval, 2.3-4.1) following fetoscopic endoluminal tracheal occlusion with early insertion compared with expectant management. There was no evidence that the effect of fetoscopic endoluminal tracheal occlusion depended on the observed over expected lung-to-head ratio for any of the endpoints. CONCLUSION: This analysis suggests that fetoscopic endoluminal tracheal occlusion increases survival for both moderate and severe lung hypoplasia. The difference between the results for the Tracheal Occlusion To Accelerate Lung growth trials, when considered apart, may be because of the difference in the time point of balloon insertion. However, the effect of the time point of balloon insertion could not be robustly assessed because of a small sample size and the confounding effect of disease severity. Fetoscopic endoluminal tracheal occlusion with early balloon insertion in particular strongly increases the risk for preterm delivery.

Prenatal diagnosis of Aicardi syndrome based on a suggestive imaging pattern: A multicenter case-series.

OBJECTIVES: To characterize a suggestive prenatal imaging pattern of Aicardi syndrome using ultrasound and MR imaging. METHODS: Based on a retrospective international series of Aicardi syndrome cases from tertiary centers encountered over a 20-year period (2000-2020), we investigated the frequencies of the imaging features in order to characterize an imaging pattern highly suggestive of the diagnosis. RESULTS: Among 20 cases included, arachnoid cysts associated with a distortion of the interhemispheric fissure were constantly encountered associated with complete or partial agenesis of the corpus callosum (19/20, 95%). This triad in the presence of other CNS disorganization, such as polymicrogyria (16/17, 94%), heterotopias (15/17, 88%), ventriculomegaly (14/20, 70%), cerebral asymmetry [14/20, 70%]) and less frequently extra-CNS anomaly (ocular anomalies [7/11, 64%], costal/vertebral segmentation defect [4/20, 20%]) represent a highly suggestive pattern of Aicardi syndrome in a female patient. CONCLUSION: Despite absence of genetic test to confirm prenatal diagnosis of AS, this combination of CNS and extra-CNS fetal findings allows delineation of a characteristic imaging pattern of AS, especially when facing dysgenesis of the corpus callosum.

Impact of Planned Delivery on the Perinatal Outcome of Term Fetuses with Isolated Heart Defects.

OBJECTIVE: Pregnancies complicated by fetal heart defects often undergo a planned delivery prior to term by either induction of labour or cesarean delivery to ensure optimal availability of neonatal care. We aimed to assess whether such planned deliveries achieve their goal of better perinatal care. METHODS: We conducted a retrospective case-control study of pregnancies complicated by isolated fetal cardiac defects, without other fetal comorbidities, managed at a single fetal medicine unit over a 10-year period. Only pregnancies delivered past 37 weeks gestation were included. Patients undergoing elective delivery for care planning reasons only were compared with patients in whom planned delivery was clinically indicated and patients who laboured spontaneously. Obstetric and perinatal outcomes were recorded. RESULTS: Of the 180 pregnancies included in the study, 59 (32.8%) were in the elective group, 49 (27.2%), in the indicated group, and 72 (40%), in the spontaneous group. Mean gestational age at delivery was 39.0 ± 1.1 weeks overall and did not differ between the groups. For the elective group, only 35.6% of deliveries occurred during office hours, which was similar to the 2 other groups. The rate of adverse obstetric or postnatal outcomes was not statistically significantly different between groups. CONCLUSION: Timed delivery at term does not seem to be associated with an increased risk of poor perinatal outcomes. It may improve perinatal care by providing proximity to a neonatal intensive care unit and convenience for patients and providers.

Development of a Training Model for Teaching Intrauterine Fetal Blood Transfusion.

This article describes an inexpensive simulator developed for teaching intrauterine blood transfusion. The model is constructed from a boneless chicken thigh folded over a Penrose drain placed in a water-filled snap-lock lid container and covered by melted ballistic gel to simulate the fetal intrahepatic vessel. Participants valued this educational tool and reported feeling the model was practical and realistic. This low-cost, high-fidelity model provides realistic tissue resistance and represents a sonographically accurate intrahepatic fetal blood transfusion training tool.