Prenatal Course and Sonographic Features of Congenital Mesoblastic Nephroma.

Background: Congenital mesoblastic nephroma (CMN) is the most common renal tumor among fetuses and infants before the age of 6 months. It usually behaves as a benign tumor. The prenatal features and outcomes of pregnancies with fetal CMN have never been systematically reviewed and analyzed, whereas neonatal or pediatric series have been published several times. The aims of this study are to (1) describe the prenatal natural course and prenatal sonographic char-acteristics of CMN; (2) determine the outcomes of pregnancies with fetal CMN; and (3) demonstrate typical sonographic images together with video clips of prenatal CMN, as an educational example based on our index case presented here. Methods: Studies focused on fetal CMN, including those consecutively published on PubMed from 1980 to June 2022 as well as the index case presented here, were identified and validated to perform a systematic review. The data of fetal imaging and the prenatal course of pregnancies were extracted for analysis. Results: The findings derived from 41 cases of review are as follows: (1) No single case has been diagnosed in the first half of pregnancy. No cases were detected during routine anomaly screening at mid-pregnancy. All cases were de-tected in the third trimester or late second trimester. (2) Polyhydramnios is very common and is the first clinical manifestation in most cases, leading to detailed ultrasound in the second half of pregnancy. (3) Preterm birth and low birth weight are the most common adverse pregnancy out-comes, resulting in neonatal morbidity. (4) Hydrops fetalis, though relatively rare, can be associated with CMN and is a grave sign. (5) Prenatal diagnosis is essential since it is critical for the antenatal plan, comprising either referral to a tertiary care center or proper surveillance to prevent serious obstetric complications, especially preterm birth. (6) Ultrasound is the primary tool for prenatal diagnosis of CMN, whereas MRI can be used as an adjunct if some other tumors are suspicious or sonographic features are not typical for CMN. Conclusion: In contrast to CMN in neonates, fetal CMN is much more serious since it significantly impacts adverse pregnancy outcomes and perinatal morbidity and mortality. The typical prenatal course and the sonographic features of CMN are described.

Comparison of the Performances of Middle Cerebral Artery Peak Systolic Velocity and Cardiothoracic Diameter Ratio in Predicting Fetal Anemia: Using Fetal Hemoglobin Bart's Disease as a Study Model.

OBJECTIVE: The aim of the study was to compare the performances of cardiothoracic diameter ratio (CTR) and middle cerebral artery peak systolic velocity (MCA-PSV) in predicting fetal hemoglobin (Hb) Bart's disease and identify the best CTR cut-off for each gestational period. METHODS: Pregnancies at risk of fetal Hb Bart's disease (gestational ages of 12-36 weeks) were prospectively recruited to undergo ultrasound examination. The measurements of CTR and MCA-PSV were performed and recorded before invasive diagnosis. RESULTS: During the study period (2005-2019), a total of 1,717 pregnancies at risk of fetal Hb Bart's disease met the inclusion criteria and were available for analysis, including 329 (19.2%) fetuses with Hb Bart's disease. The mean gestational age at the time of diagnosis was 19.30 ± 5.6 weeks, ranging from 12 to 36 weeks. The overall performance of CTR Z-scores is superior to that of MCA-PSV multiple of median (MoM) values; area under curve of 0.866 versus 0.711, p value <0.001. The diagnostic indices of CTR and MCA-PSV are increased with gestational age. Based on receiver operating characteristic curves of CTR Z-scores, the best cut-off points of CTR at 12-14, 15-17, 18-20, 21-23, and ≥24 weeks are 0.48, 0.49, 0.50, 0.51, and 0.54, respectively. The best cut-off of MCA-PSV is 1.3 MoM, giving the best performance at 21-23 weeks with a sensitivity of 91.8% and specificity of 85.5%. CONCLUSION: The performance of CTR is much better than MCA-PSV in predicting fetal anemia caused by Hb Bart's disease. Nevertheless, whether this can be reproduced in anemia due to other causes, like isoimmunization, is yet to be explored.

New genetic and clinical evidence associated with fetal Beckwith-Wiedemann syndrome.

Early detection of Beckwith-Wiedemann syndrome (BWS) is very important since it is very useful regarding counseling of parents concerning the risk of developing embryonic tumors, selection of the mode of delivery due to potential adrenal cysts that might bleed during labor, prevention of neonatal hypoglycemia and even options of pregnancy termination in non-viable fetuses. This report describes the prenatal classic sonographic triad of fetal BWS (omphalocele, macrosomia, macroglossia) and other supporting findings (hepatomegaly, adrenal enlargement) as well as additional postnatal evidence. Also, it demonstrates new molecular genetic evidence potentially associated with the disease (the presence of a novel heterozygous c.358G>T variant of the CDKN1C gene). Importantly, we provide new evidence indicating that elevated levels of the four serum biomarkers (alpha-fetoprotein, beta-human gonadotropin, unconjugated estriol, and inhibin-A) in late first or early second trimester might be strongly suggestive of BWS which may facilitate early detection especially in cases of no obvious anomaly. In conclusion, this study emphasizes on early detection of BWS as early as at 14 weeks of gestation, based on the abnormal rise of the four serum biomarkers together with omphalocele. To the best of our knowledge, this is the earliest prenatal detection of BWS ever reported. Finally, we provide new molecular genetic evidence that is, potentially associated with BWS.

May-Thurner Syndrome Is Aggravated by Pregnancy.

This study aims to emphasize that asymptomatic patients with undiagnosed and asymptomatic May-Thurner syndrome (MTS) may firstly develop severe compression during pregnancy. A 40-year-old woman, G1P0, at 22 weeks of twin gestation presented with left lower extremity edema and pain. One twin was structurally normal while the other had bilateral renal agenesis with oligohydramnios. Magnetic resonance venography (MRV) revealed severe compression of the left iliac vein by the right iliac artery without evidence of deep vein thrombosis (DVT). Conservative treatment with anticoagulant prophylaxis was instituted throughout the rest of pregnancy and postpartum period. She was also complicated with severe pre-eclampsia, a cesarean section was performed due to a prolapsed cord at 27 weeks of gestation, and she gave birth to a surviving baby weighing 1100 g. In conclusion, this case report provides evidence that pregnancy can disclose a subtle May-Thurner anatomy to be symptomatic without DVT. Successful pregnancy outcomes could be achieved with conservative treatment and anticoagulant prophylaxis.

Prenatal Sonographic Features of CHARGE Syndrome.

CHARGE syndrome is a rare autosomal dominant disorder, associated with coloboma (C), heart defects (H), choanal atresia (A), retardation of growth and/or central nervous system (R), genitourinary anomalies (G) and ear abnormalities (E). Prenatal diagnosis of the syndrome is very rare but may be suspected when a combination of such abnormalities is identified. We describe a prenatally suspected case of CHARGE syndrome due to unique findings of cardiac defects (DORV) in combination with minor clues, including a structurally malformed ear with persistent non-response to an acoustic stimulation (which has never been prenatally described elsewhere), renal malrotation and growth restriction. Postnatal diagnosis was made based on confirmation of the prenatal findings and additional specific findings of bilateral coloboma, choanal atresia and ear canal stenosis. Finally, molecular genetic testing by whole exome sequencing of the neonate and her parents revealed a novel de novo heterozygous frameshift c.3506_3509dup variant in the CHD7 gene, confirming the clinical diagnosis of CHARGE syndrome. In conclusion, we describe unique prenatal features of CHARGE syndrome. Educationally, this is one of the rare examples of CHARGE syndrome, comprising all of the six specific anomalies as originally described; it is also supported by the identification of a specific genetic mutation. The identified genetic variant has never been previously reported, thereby expanding the mutational spectrum of CHD7. Finally, this case can inspire prenatal sonographers to increase awareness of subtle or minor abnormalities as genetic sonomarkers.

Effect of elastic abdominal binder on pain and functional recovery after caesarean delivery: a randomised controlled trial.

The Elastic abdominal binder has been widely employed by clinicians for pain relief, wound complications prevention, improved pulmonary function, and stabilisation. However, these proposed benefits have not been properly examined in women following caesarean delivery. We aimed to examine the effects of post-caesarean elastic abdominal binder use on recovery by comparing post-operative pain, mobility and quality of life. Pregnant women undergoing caesarean delivery were randomly assigned into two groups: abdominal binder (90 patients) and control (90 patients). The primary outcomes included the daily visual analogue scale pain scores and the distance from the six-minute walk test. Baseline characteristics were similar between the groups. There was no significant difference in pain scores and six-minute walking distance between the study groups. There was no significant between-group difference in quality-of-life dimensions, overall health status, and post-operative complication. The positive effects of elastic abdominal binder use following caesarean delivery could not be demonstrated in this study.Impact statementWhat is already known on this subject? Elastic abdominal binder is commonly used after laparotomy to support incision. There was evidence to support the benefit of abdominal binder in reducing psychological distress during the first five days following laparotomy for other indications. From limited number of studies addressing caesarean section, the evidence for the benefits of the binder on pain, symptom distress, and change in haemoglobin level is conflicting.What do the results of this study add? In contrast to the results of the previous study, the beneficial effects of abdominal binder on pain reduction, functional recovery, and quality of life following caesarean delivery could not be demonstrated in this study.What are the implications of these findings for clinical practice and/or further research? The use of elastic abdominal binder after caesarean delivery is not associated with reduction of postoperative pain, faster functional recovery, and improved quality of life in our population. Further studies in other population with different characteristics may be worthwhile.