Subamniotic haemorrhage, a possible new presentation of fetal and neonatal alloimmune thrombocytopenia

Background: Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a rare fetal disease in which maternal antibodies directed towards fetal human platelet antigens (HPA) are formed during pregnancy and cause fetal thrombocytopenia. The diagnosis FNAIT is suspected when a fetus or neonate presents with signs of bleeding. Case: We describe a pregnancy complicated by a placental hematoma in the 20th week of gestation as the first manifestation of FNAIT. Further evaluation showed signs of germinal matrix haemorrhage and HPA-5b allo-antibodies. After the diagnosis, intravenous immunoglobulin was administered weekly and a healthy daughter was born at 37 weeks. Histopathological analysis revealed that the hematoma was caused by a subamniotic haemorrhage of fetal origin. Conclusion: A subamniotic hematoma appears to be the first manifestation of FNAIT.

Effectiveness of ultrasound screening for placenta accreta spectrum using standard ultrasound criteria in a secondary care setting

Objectives: Ultrasound detection of placenta accreta spectrum (PAS) among women at risk is a key goal to reduce obstetric morbidity, but there is scarce information of its performance in real clinical settings. We report the effectiveness of a standardized ultrasound protocol to detect PAS in women with placenta previa in a secondary-level hospital. Methods: A retrospective analysis, including a cohort of 126 women with persistent placenta previa among 27,975 pregnancies between 2008 and 2020. All 126 women underwent standardized transabdominal and transvaginal ultrasound assessing 5 criteria: 1) loss of hypoechoic retroplacental zone and/or myometrial thinning <1 mm; 2) lacunar images with flow >15 cm/sec; 3) thick and bulging placenta; 4) thinning or interruption of the uterine-bladder serous interface; and 5) placental or uterovesical hypervascularity. The presence of at least one criterion was considered a high-risk for PAS. Diagnosis of PAS was confirmed during caesarean section and by histopathological analysis. Results: Among 126 women with placenta previa, 11 (8.7%) cases of PAS were diagnosed, of which 10 were detected prenatally by ultrasound. This resulted in a sensitivity of 90,9%, a specificity of 98,3%, a positive predictive value (PPV) of 83,3%, and negative predictive value (NPV) of 99,1%. Histopathological assessment showed six placenta increta (54.5%), four percreta (36.4%) and one accreta (9.1%). All 10 cases of invasive placenta presented more than three ultrasound criteria. Conclusions: Standardized ultrasound screening protocol in women at risk due to placenta previa in the third trimester was highly effective in detecting PAS in a secondary-level hospital setting.

Fetoscopic balloon dilation and stent placement of congenital high airway obstruction syndrome leading to successful Caesarian delivery

Introduction: Without fetal or perinatal intervention, congenital high airway obstruction syndrome (CHAOS) is a fatal anomaly. The ex utero intrapartum treatment (EXIT) procedure has been used to secure the fetal airway and minimize neonatal hypoxia, but is associated with increased maternal morbidity. Case Presentation: A 16-year-old woman (gravida 1, para 0) was referred to our hospital at 31 weeks gestation with fetal anomalies, including echogenic lungs, tracheobronchial dilation and flattened diaphragms. At 32 weeks, fetoscopic evaluation identified laryngeal stenosis, which was subsequently treated with balloon dilation and stent placement. The patient developed symptomatic and regular preterm contractions at post-operative day 7 with persistent sonographic signs of CHAOS, which prompted a repeat fetoscopy with confirmation of a patent fetal airway followed by Cesarean delivery under neuraxial anesthesia. Attempts to intubate through the tracheal stent were limited and resulted in removal of the stent. A neonatal airway was successfully established with rigid bronchoscopy. Direct laryngoscopy and bronchoscopy confirmed laryngeal stenosis with a small tracheoesophageal fistula immediately inferior to the laryngeal stenosis and significant tracheomalacia. A tracheostomy was then immediately performed for anticipated long term airway and pulmonary management. The procedures were well tolerated by both mom and baby. The baby demonstrated spontaneous healing of the tracheoesophageal fistula by day of life 7 with discharge home with ventilator support at three months of life. Conclusion: Use of repeated fetoscopy in order to relieve fetal upper airway obstruction offers the potential to minimize neonatal hypoxia, while concurrently decreasing maternal morbidity by avoiding an EXIT procedure. Use of the tracheal stent in CHAOS requires further investigation. The long-term reconstruction and respiratory support of children with CHAOS remain challenging

Fetal-maternal surgery for spina bifida in a HIV-infected mother

INTRODUCTION: In select cases, in utero surgery for MMC leads to better outcomes than postnatal repair. However, maternal HIV infection constitutes a formal exclusion criterion due to the potential of vertical HIV transmission. Encouraged by a previous case of a successful fetal spina bifida repair in a Hepatitis Bs antigen positive woman, a plan was devised allowing for fetal surgery. CASE REPORT: In utero MMC repair was performed although the mother was HIV-infected. To minimize the risk of in utero HIV transmission, the mother was treated by HAART throughout gestation as well as intravenous zidovudine administration during maternal-fetal surgery. The mother tolerated all procedures very well without any sequelae. The currently 20 month-old toddler, is HIV negative and has significantly benefitted from fetal surgery. DISCUSSION/CONCLUSION: This case shows that maternal HIV is not a priori a diagnosis that excludes fetal surgery. Rather, it might be a surrogate for moving towards personalized medicine and away from applying too rigorous exclusion criteria in the selection of candidates for maternal-fetal surgery.

Severe fetal symptomatic infection from human cytomegalovirus following non-primary maternal infection: report of two cases

Introduction Human cytomegalovirus (HCMV) is the most common congenital infection, expecially severe after a maternal primary infection; sequelae in neonates born to mothers experiencing a non-primary infection have been already reported. Hereby, two cases of severe fetal HCMV disease in seroimmune gravidas referred to our Unit are described. Cases presentation Case 1 A fetus at 21 weeks’ gestation with signs of anemia and brain abnormalities at ultrasound (US), described at magnetic resonance (MR) imaging as ependymal irregularity and bilateral asymmetric parenchimal thinning; amniotic fluid sample was positive for HCMV although the woman had a previous immunity; after termination of pregnancy, autopsy demonstrated a thicken layer of disorganized neurons on the right cortical plate, while on the left there was a morphological pattern coherent with polymicrogyria. Case 2 A fetus at 20 weeks’ gestation with anemia, moderate atrio-ventricular insufficiency, hepatosplenomegaly but no major cerebral lesions. Fetal blood was positive for HCMV, although unexpected for pre-pregnancy maternal immunity, and intrauterine transfusion was needed. A cesarean section at 34 weeks gestation was performed due to worsening condition of the fetus, who had a birthweight of 2210 grams, needed platelet transfusions but MR examination and clinical evaluation were normal. Conclusion The impact of non-primary maternal infection on pregnancy outcome is unknown and fetal brain damage in HCMV seroimmune transmitter-mothers can occur as a consequence of maternal re-infection or reactivation for a hypotetic different role of HCMV-primed CD4+ or CD8+ T-cells in fetal brain, with progressive brain lesions coexistent in the first case and with severe unexpected anemia in the second case. A previous maternal HCMV immunity should not exempt to test anemic fetuses for such infection, nor to consider a potential transplacental transmission.

Outcome of Monochorionic Monoamniotic Twin Reversed Arterial Perfusion Sequence Diagnosed in the First Trimester

<b><i>Introduction:</i></b> The aim of this study is to evaluate the outcome of pregnancies complicated by monochorionic monoamniotic twin reversed arterial perfusion sequence (MOMA TRAP) diagnosed in the first trimester. <b><i>Methods:</i></b> All patients diagnosed with MOMA TRAP sequence &#x3c;14.0 weeks of gestation in a 10-year study period were retrospectively analyzed for intrauterine course and outcome. All patients were offered either expectant management or intrauterine intervention. Adverse outcome was defined as either intrauterine death (IUD), neonatal death or preterm birth &#x3c;34.0 weeks of gestation. <b><i>Results:</i></b> In the study period, 17 cases with MOMA TRAP sequence were diagnosed. Of these, 2 couples opted for termination of pregnancy. The remaining 15 were divided into 2 groups depending on the management: group A (<i>n</i> = 8) with expectant management and group B (<i>n</i> = 7) with intrauterine intervention. All fetuses in group A died before 20 weeks. Survival in group B was significantly better with 4/7 (57.1%) life births at a median of 39.6 weeks of gestation (<i>p</i> = 0.0256). The reasons for IUD in the 3 cases in group B were hemodynamic, strangulation, and bleeding complications during intervention. <b><i>Conclusions:</i></b> Intrauterine intervention in MOMA TRAP pregnancies significantly improves neonatal survival, although it is still associated with a substantial risk for IUD by hemodynamic complications or entanglement.

Assessment of the Size and Shape of the 4-Chamber View and the Right and Left Ventricles using Fetal Speckle Tracking in Normal Fetuses at 17-24 Gestational Weeks

Introduction: The aim of the study was to establish normal reference values obtained by fetal speckle tracking analysis of the fetal heart between 17-24 weeks of gestation among Thai fetuses and compare the nomograms with previous studies. Methods: The 4-chamber view of the fetal heart in 79 normal fetuses was analyzed by speckle tracking analysis to determine the best-fit regression model. The 95% reference intervals and Z-score equations of fetal cardiac parameters were computed. Results: The end-diastolic length, width, area, and circumference of the 4-chamber view (4CV) as well as the ventricular end-diastolic length, 24-segment widths, and area were all increased as a function of gestational age (GA) and 5 fetal biometric parameters. In contrast, the global sphericity index (SI), 24-segment SI, and right ventricle/left ventricle width and area ratios did not change with GA or fetal biometric measurements. There were few differences in Z-score reference ranges of fetal cardiac measurements between the current study and previous studies conducted in different patient populations. Conclusion: Our study provided z-score and corresponding centile calculators, 5th and 95th centile reference tables, and corresponding graphs for evaluating the size and shape of the 4CV and the right and left ventricles using 6 independent variables between 17 and 24 weeks of gestation. These results provide normal reference ranges for future studies of fetuses with pathologies that may alter the size and shape of the 4-chamber view and ventricles.

Placental Location in Maternal-Fetal Surgery for Myelomeningocele

Introduction: Uterine incision based on placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regards to maternal or fetal outcomes. Objective: To investigate whether an anterior placenta was associated with increased rates of intraoperative, perioperative, antepartum, obstetric, or neonatal complications in mothers and babies who underwent OMFS for myelomeningocele (fMMC) closure. Methods: Data from the international multi-center prospective registry of patients who underwent OMFS for fMMC closure (fMMC Consortium Registry, 12/15/2010-7/31/2019) was used to compare fetal and maternal outcomes between anterior and posterior placental locations. Results: Placental location for 623 patients was evenly distributed between anterior (51%) or posterior (49%). Intraoperative fetal bradycardia (8.3% vs 3.0%, p=0.005) and performance of fetal resuscitation (3.6% vs 1.0%, p=0.034) occurred more frequently in cases with an anterior placenta when compared to those with a posterior placenta. Obstetric outcomes including membrane separation, placental abruption, and spontaneous rupture of membranes were not different among the two groups. However, thinning of the hysterotomy site (27.7% vs 17.7%, p=0.008) occurred more frequently in cases of anterior placenta. Gestational age at delivery (p=0.583) and length of stay in the neonatal intensive care unit (p=0.655) were similar between the two groups. Fetal incision dehiscence and wound revision were not significantly different between groups. Critical clinical outcomes including fetal demise, perinatal death, and neonatal death were all infrequent occurrences and not associated with placental location. Conclusions: Anterior placental location is associated with increased risk of intraoperative fetal resuscitation and increased thinning at the hysterotomy closure site. Individual institutional experiences may have varied but the aggregate data from the fMMC Consortium did not show a significant impact on the gestational age at delivery or maternal or fetal clinical outcomes.

The relation between head circumference and mid pelvic circumference: A simple index for cephalo-pelvic disproportion evaluation

Introduction: Cephalo-pelvic-disproportion (CPD) is one of the most common obstetric complications. Since CPD is the disproportion between the fetal head and maternal bony pelvis, evaluation of the head-circumference (HC) relative to maternal bony pelvis may be a useful adjunct to pre-labor CPD evaluation. The aim of the present study was a proof-of-concept evaluation of the ratio between HC to pelvic circumference (PC) as a predictor of CPD. Methods: Of 11,822 deliveries, 104 cases that underwent an abdomino-pelvic CT for any medical indication and who underwent normal vaginal deliveries (NVD) (n=84) or cesarean deliveries (CD) due to CPD (n=20) were included retrospectively. Maternal pelvis dimensions were reconstructed and neonatal HC, as a proxy for fetal HC, were measured. The correlation between cases of CPD and Cephalo-Pelvic Circumference Index (CPCI), which represents the ratio between the HC and PC in percent (HC/PC *100) was evaluated. Results: The mid-pelvis cephalo-pelvic circumference index (MP-CPCI) was larger in CD groups as compared to the NVD group: 103±11 vs. 97±8% respectively (p=0.0003). In logistic regression analysis, the MP-CPCI was found to be independently associated with CD due to CPD: each 1% increase in MP-CPCI increased the likelihood of CD for CPD by 11% (aOR 1.11, CI 95% 1.03-1.19, p=0.004). The adjusted odds ratio for CD due to CPD increased incrementally as the MP-CPCI increased, from 3.56 (95%CI, 1.01-12.6) at MP-CPCI of 100, to 5.6 (95%CI, 1.63-19.45) at 105, 21.44 (95%CI, 3.05-150.84) at 110, and 28.88 (95%CI, 2.3-362.27) at MP-CPCI of 115 Conclusions: The MP-CPCI, representing the relative dimensions of the fetal HC and maternal PC, is a simple tool that can potentially distinguish between parturients at lower and higher risk of CPD. Prospective randomized studies are required to evaluate the feasibility of prenatal pelvimetry and MP-CPCI to predict the risk of CPD during labor.

SIGNIFICANCE OF LOW MATERNAL SERUM Β-HCG LEVELS IN THE ASSESSMENT OF THE RISK OF ATYPICAL CHROMOSOMAL ABNORMALITIES

Introduction: The introduction of prenatal cell-free DNA as a screening test has surpassed traditional combined first-trimester screening (cFTS) in the detection of common trisomies. However, its current limitation in detecting only common trisomies is affecting the diagnostic yield for other clinically significant chromosomal abnormalities. Methods: In efforts to optimize the detection of fetuses with genetic abnormalities, we have analyzed the relationship between the cFTS risk score and biomarkers with atypical chromosomal abnormalities. Furthermore, we have evaluated the impact of prenatal cell-free DNA screening on the detection of chromosomal abnormalities in our population. For these purposes, we performed a retrospective study of 877 singleton pregnancies who underwent chromosomal microarray analysis (CMA) between 2013 to 2020 and for whom first-trimester screening data were available. Results: The results demonstrated that low levels of free beta human chorionic gonadotropin (β-hCG) (≤ 0.37 MoM) and increased fetal nuchal translucency (NT) (≥ 3.5mm) were statistically associated with the presence of atypical chromosomal abnormalities. In fact, the risk of pathogenic CMA results increased from 6% to 10% when fetal NT was increased and from 6% to 20% when a low serum β-hCG level was detected in the high-risk cFTS group. Moreover, our results showed that altered serum levels of β-hCG can have a substantial impact on the early detection of clinically relevant copy number variants. Discussion/Conclusion: Traditional cFTS can potentially identify a substantial proportion of atypical chromosomal aberrations, and women with increased NT or low maternal serum β-hCG levels are at increased risk of having pathogenic CMA results. Our results may help clinicians and women decide whether invasive testing or prenatal cell-free DNA screening testing are more appropriate for each situation.