Data to guide evidence-based management of pregnant people with sickle cell disease (SCD) are limited. This international Delphi panel aimed to identify consensus among multidisciplinary experts for SCD management during pregnancy. The 2-round Delphi process used questionnaires exploring 7 topics (antenatal care, hydroxyurea use, transfusion, prevention of complications, treatment of complications, delivery and follow-up, and bottlenecks and knowledge gaps) developed by a steering committee. Thirteen panelists (hematologists, physiologists, obstetricians, maternal fetal medicine, and transfusion medicine physicians) from the United States, the United Kingdom, Turkey, and France completed the first survey; 12 panelists completed the second round. Anonymized responses were collected and summarized by a contract research organization (Akkodis Belgium). Consensus and strong consensus were predefined as 75% to 90% (9-10 of 12) and >90% (≥11 of 12) of panelists, respectively, agreeing or disagreeing on a response to a predefined clinical scenario or statement. In several areas of SCD management, consensus was achieved: experts recommended performing at least monthly multidisciplinary antenatal follow-up, administering prophylactic aspirin for preeclampsia prevention between gestational weeks 12 and 36, initiating prophylactic transfusion therapy in certain cases, or choosing automated red blood cell exchange over other transfusion methods for patients with iron overload or severe acute chest syndrome. No consensus was reached on several topics including the prophylactic aspirin dose, indications for starting infection prophylaxis, routine use of prophylactic transfusions, or use of prophylactic transfusions for preventing fetal complications. These recommendations could inform clinical care for patients with SCD who are pregnant in the absence of large clinical trials involving this population; the identified knowledge gaps can orient future research.

Introduction: To evaluate the role of three-dimensional (3D) sonography in the prenatal detection of the upper pole in fetuses with spina bifida. Material and Methods: Women admitted to a tertiary center with pregnancies with isolated open spina bifida were enrolled in the study. All fetuses had 3D sonography to predict the lesion level. The exact lesion level was ascertained using radiography and/or autopsy following the delivery at term or abortion. Results: Forty-eight cases were eligible for this study. Twenty-eight fetuses were diagnosed in the second trimester and the lesion level was precisely predicted in 24 (86%) of them. In 14 (70%) of the 20 fetuses diagnosed in the last trimester, the level was accurately determined. The remaining 10 fetuses, from both the second and third trimesters, were found to have a lesion level that was within one segment of the predicted lesion level. The correct matching rate was 38/48 (79%), and agreement within one segment was achieved in all cases. Discussion: According to our data, 3D ultrasound is useful for the prenatal determination of lesion level in spina bifida, which is an important prognostic factor.

To evaluate persistent left superior vena cava (PLSVC) cases according to associated cardiac, extracardiac, and chromosomal anomalies in the prenatal period and to review their outcomes. The data of patients with a prenatal diagnosis of PLSVC between January 2013 and December 2017 were reviewed retrospectively. Data of 32 cases were reviewed. Nineteen (60%) cases were associated with cardiac defects, 5 (15%) were associated with both cardiac and extracardiac defects, and 8 (25%) had no associated anomalies. Two fetuses had karyotype anomalies. All patients with isolated PLSVC survived. Among the cases associated with extracardiac anomalies, cardiac anomalies, and with both extracardiac and cardiac anomalies, the survival rate was 40%, 40%, and 25%, respectively. Outcome was more favorable in cases with isolated PLSVC (100% vs. 40%). Prenatally diagnosed PLSVC is associated with cardiac and extracardiac anomalies in the majority of cases. The prognosis is good in isolated cases, but worsens when accompanied by cardiac or extracardiac anomalies.