Vesicoamniotic shunting for fetal megacystis in the first trimester with a Somatex® intrauterine shunt.

PURPOSE: The objective was to evaluate the feasibility of vesicoamniotic shunting (VAS) in the first trimester with the Somatex® intrauterine shunt and report on complications and neonatal outcome. METHODS: Retrospective cohort study of all VAS before 14 weeks at two tertiary fetal medicine centres from 2015 to 2018 using a Somatex® intrauterine shunt. All patients with a first trimester diagnosis of megacystis in male fetuses with a longitudinal bladder diameter of at least 15 mm were offered VAS. All patients that opted for VAS after counselling by prenatal medicine specialists, neonatologists and pediatric nephrologists were included in the study. Charts were reviewed for complications, obstetric and neonatal outcomes. RESULTS: Ten VAS were performed during the study period in male fetuses at a median GA of 13.3 (12.6-13.9) weeks. There were two terminations of pregnancy (TOP) due to additional malformations and one IUFD. Overall there were four shunt dislocations (40%); three of those between 25-30 weeks GA. Seven neonates were born alive at a median GA of 35.1 weeks (31.0-38.9). There was one neonatal death due to pulmonary hypoplasia. Neonatal kidney function was normal in the six neonates surviving the neonatal period. After exclusion of TOP, perinatal survival was 75%, and 85.7% if only live-born children were considered. CONCLUSION: VAS in the first trimester is feasible with the Somatex® Intrauterine shunt with low fetal and maternal complication rates. Neonatal survival rates are high due to a reduction in pulmonary hypoplasia and the rate of renal failure at birth is very low. VAS can be safely offered from the late first trimester using the Somatex® intrauterine shunt.

First-trimester intervention in twin reversed arterial perfusion sequence: does size matter?

OBJECTIVE: To evaluate the outcome of first-trimester intervention (12 + 0 to 14 + 0 weeks of gestation) in pregnancies complicated by twin reversed arterial perfusion (TRAP) sequence. METHODS: All monochorionic diamniotic twin pregnancies diagnosed with TRAP sequence that underwent intrafetal laser ablation (IFL) of the feeding vessels before 14 + 0 weeks of gestation at the University of Bonn between 2010 and 2015 were analyzed retrospectively for intrauterine course and outcome. RESULTS: In the study period, 12 pregnancies with TRAP sequence were treated by IFL. Median gestational age at intervention was 13.2 (interquartile range (IQR), 12.6-13.6) weeks. In all cases, one intervention sufficed to disrupt the perfusion of the TRAP twin. There was no case of miscarriage, preterm prelabor rupture of membranes or hemorrhage. In five (41.7%) pregnancies, intrauterine death of the pump twin occurred at a median of 72.0 (IQR, 54.0-90.0; range, 48-96) h after intervention. The remaining seven pregnancies continued uneventfully resulting in birth of a healthy infant at term. A comparison of survivors and non-survivors identified a significant difference in median discordance between crown-rump length (CRL) of the pump twin and upper pole-rump length (URL) of the TRAP twin ((CRL - URL)/CRL ratio, 0.56 vs 0.31; P < 0.05 and URL/CRL ratio, 0.44 vs 0.68; P < 0.05). Survivors were treated at a significantly later gestational age than were non-survivors (median, 13.4 (IQR, 12.9-14.1) vs 12.6 (IQR, 12.5-13.1); P < 0.05). However, none of these parameters independently predicted survival. CONCLUSIONS: Although technically feasible, IFL performed in the first trimester for TRAP sequence is associated with a significant fetal loss rate. Gestational age at intervention, (CRL - URL)/CRL ratio and URL/CRL ratio are potential predictors of pregnancy outcome. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Prenatal diagnosis of absent pulmonary valve syndrome from first trimester onwards: novel insights into pathophysiology, associated conditions and outcome.

OBJECTIVE: To assess the spectrum of associated anomalies, intrauterine course and outcome in fetuses with absent pulmonary valve syndrome (APVS). METHODS: All cases with a prenatal diagnosis of APVS at two centers over a period of 13 years were analyzed retrospectively. APVS was diagnosed in the presence of rudimentary or dysplastic pulmonary valve leaflets with to-and-fro blood flow in the pulmonary trunk on color and pulsed-wave Doppler ultrasound. Data on demographic characteristics, presence of associated conditions, Doppler studies and pregnancy outcome were reviewed. RESULTS: During the study period, 40 cases of APVS were diagnosed prenatally. Thirty-seven (92.5%) cases were associated with tetralogy of Fallot (TOF) and three (7.5%) had an intact ventricular septum. Patency of the ductus arteriosus (DA) was found in 17/37 (45.9%) TOF cases and in all three cases with an intact ventricular septum. Mean gestational age at diagnosis was 19.7 (range, 12-34) weeks with 10 (25.0%) cases (all with TOF) diagnosed in the first trimester. TOF was an isolated finding in 15 (37.5%) cases. Chromosomal anomalies, cardiac defects and extracardiac anomalies were present in 18 (45.0%), four (10.0%) and three (7.5%) cases, respectively. Among the 40 cases, there were 19 (47.5%) terminations of pregnancy, six (15.0%) intrauterine deaths, four (10.0%) neonatal deaths and 11 (27.5%) survivors. Patency of the DA, reversed flow during atrial contraction in the ductus venosus, umbilical artery or fetal middle cerebral artery, and hydrops/increased nuchal translucency thickness were significantly associated with non-survival. All 10 cases diagnosed in the first trimester had a patent DA and abnormal Doppler parameters, eight had hydrops and/or increased nuchal translucency, six were associated with trisomy 13 or 18 and none survived. CONCLUSION: APVS diagnosed in the first trimester is significantly associated with TOF, patency of the DA, abnormal Doppler parameters, lethal trisomies and intrauterine mortality. Cases of APVS with isolated TOF and agenesis of the DA have a better outcome than those with additional anomalies, with > 80% survival. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Extracardiac anomalies in prenatally diagnosed heterotaxy syndrome.

OBJECTIVE: To assess the incidence and impact of extracardiac anomalies on the prognosis of fetuses with heterotaxy syndrome. METHODS: All fetuses diagnosed with heterotaxy syndrome by three experienced examiners over a period of 14 years (1999-2013) were reviewed retrospectively. RESULTS: In total, 165 fetuses with heterotaxy syndrome were diagnosed in the study period. One hundred and fifty (90.9%) had cardiac defects; extracardiac anomalies that did not involve the spleen were present in 26/165 (15.8%) cases. Of the total study cohort, termination of pregnancy was performed in 49 (29.7%) cases, intrauterine death occurred in 11 (6.7%), postnatal death occurred in 38 (23.0%) and 67 (40.6%) were alive at the latest follow-up, resulting in a total perinatal and pediatric mortality of 59.4%. Among the 105 liveborn neonates, 15 (14.3%) had extracardiac anomalies with significant impact on the postnatal course: one neonate died following repair of an encephalocele, six had successful treatment for various types of intestinal malrotation and/or atresia and one underwent hiatal hernia repair; the remaining seven had biliary atresia, of which five died and the two survivors are awaiting liver transplantation. The status of the spleen was assessed in 93/105 liveborn children and was found to be abnormal in 84/93 (90.3%). There were three cases of lethal sepsis, all associated with asplenia. Of the 38 postnatal deaths, 29 (76.3%) had a cardiac cause, seven (18.4%) had an extracardiac cause and in two (5.2%) the reason was uncertain. CONCLUSIONS: Although the leading causes of death in fetuses and children with heterotaxy syndrome are cardiac, a small subset of fetuses have extracardiac anomalies with significant impact on outcome. These anomalies often escape prenatal detection, and therefore neonates at risk should be monitored for bowel obstruction, biliary atresia and immune dysfunction in order to allow timely intervention through a multidisciplinary approach. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Intrauterine vesicoamniotic shunting for fetal megacystis.

OBJECTIVE: To examine the outcome of fetuses with megacystis treated with vesicoamniotic shunting (VAS) from 14 weeks onward. METHODS: Retrospective review of all fetuses that received VAS at two centres from 2004 to 2012. RESULTS: 53 fetuses with megacystis were included in the study. Mean gestational age at diagnosis was 16.4 weeks. Mean gestational age at first shunt placement was 17.8 weeks. The first shunt placement was performed before 16 weeks in 18 (34 %) cases. The mean number of shunts was 1.38. Dislocation occurred in 35 (66 %). TOP was performed in 21 (39.6 %), intrauterine death occurred in two (3.8 %) and spontaneous abortion in three cases (5.7 %). Of the 27 (50.9 %) live births, 17 (32.1 %) infants survived. Normal renal function was present in 10 cases, 4 have compensated renal failure and 3 infants had renal transplantation. Oligohydramnios was significantly associated with non-survival and renal insufficiency. The gestational age at VAS was neither correlated with renal function after birth nor with the survival in our cohort. Conversely, the interval between first shunt placement and delivery was positively correlated with survival and normal renal function. The gestational age at delivery was significantly higher in survivors and those born with normal renal function. CONCLUSION: Despite intervention, the morbidity and mortality of megacystis is still high. We failed to demonstrate that early intervention is associated with an improved rate of normal renal function after birth. Oligohydramnios was the only parameter identifying fetuses with unfavourable outcome, while all other parameters were inconclusive.

Morgagni hernia presenting with massive pericardial effusion and ascites: prenatal management by thoraco-amniotic shunting and fetal endoscopic tracheal occlusion (FETO) and review of the literature.

BACKGROUND: Morgagni hernia presents a rare type of congenital diaphragmatic hernia (CDH, about 2-5 %) (Herman, J Perinatol 21:343-344, 2001), which is characterized by an anterior mainly right-sided defect of the diaphragm. Infrequently, this is combined with a herniation of the liver into the pericardial cavity (Aké, Prenat Diagn 11:719-724, 1991; Stevens, Pediatr Radiol 26:791-793, 1996). This may cause massive pericardial effusion and subsequently lung hypoplasia (Pober et al., Congenital diaphragmatic hernia overview, University of Washington, Seattle, 2015; Ikeda, J Perinat Med 30:336-340, 2002; Hara, J Obstet Gynaecol Res 33:561-565, 2007). So far only few cases have been reported in fetal life. CASE: We report a case of Morgagni hernia with pericardiodiaphragmatic aplasia, complicated by two-compartment effusions (massive pericardial effusion and mild ascites), diagnosed in the second trimester. The case was successfully managed in utero with thoraco-amniotic shunting and late tracheal occlusion, followed by corrective surgery after birth. DISCUSSION: A review of the literature was performed, identifying 13 cases of prenatally diagnosed Morgagni hernia. The diagnosis was established by the sonographic findings of pericardial effusion und intrathoracic herniation of the liver. In only two cases a prenatal intervention was carried out. All neonates were operated postnatally with excellent final outcome.

Quality of analgesic care in labor: A cross-sectional study of the first national register-based benchmarking system.

OBJECTIVE: Unlike other types of acute pain, labor pain is considered physiological. Due to the heterogeneous management during labor, there is a lack of intention to define quality of care of peripartal analgesia. This study presents the first results of the national register for this evaluation. METHODS: This prospective cross-sectional study, conducted in five different German level-three hospitals, included women after vaginal childbirth between January 2020 and January 2022. A validated questionnaire was completed 24 h postpartum, including information about labor pain, satisfaction, and expectations regarding analgesia. Data were centrally recorded with obstetric records using the database of the QUIPS (Quality Improvement in Postoperative Pain Management) Project. RESULTS: A total of 514 women were included. On an 11-point Numerical Rating Scale, pain intensity during labor was severe (8.68 ± 1.8) while postpartal pain was 3.9 (±2.1). The second stage of labor was considered the most painful period. Only 62.6% of the parturients obtained pharmacological support, with epidural being the most effective (reduction of 3.8 ± 2.8 points). Only epidural (odds ratio [OR] 0.22) and inhalation of nitrous oxide (OR 0.33) were protective for severe pain. In benchmarking, a relation between satisfaction, pain intensity, and the use of epidural was found; 40.7% of the women wished they had received more analgesic support during labor. CONCLUSION: This study highlights deficiencies in analgesic management in high-level perinatal centers, with more than 40% of parturients considering actual practices as insufficient and wishing they had received more analgesic support, despite the availability of analgesic options. Using patient-reported outcomes can guarantee qualitative tailored analgesic care in women.

Arabin cervical pessary in women at high risk of preterm birth: a magnetic resonance imaging observational follow-up study.

OBJECTIVE: To help elucidate the mechanism of action of the Arabin cervical pessary in pregnancies at high risk for preterm delivery. METHODS: Cervical length and uterocervical angle were evaluated in relation to gestational age in 198 pregnancies not at high risk for preterm birth that underwent clinical fetal magnetic resonance imaging (MRI). Additionally, in 73 singleton pregnancies at high risk for preterm birth, an Arabin cervical pessary was placed at 14-33 weeks' gestation. We performed MRI of the cervix immediately before and after placement and at monthly follow-up until removal of the pessary. In a subgroup of 54 pregnancies with a short cervix and pessary placement at 17-31 weeks' gestation, the uterocervical angle and cervical length at follow-up were compared with the preplacement values. RESULTS: In pregnancies not at high risk for preterm birth, the uterocervical angle did not vary, but cervical length showed a significant decrease with gestational age (r = -0.15, P < 0.05). Among the high-risk patients, the cervical pessary was successfully placed at first attempt in 60 (82.2%) and by the second attempt in 66 (90.4%), remaining well positioned until removal. In five patients we failed to place the pessary after two attempts, in one patient the pessary dislodged during follow-up and in one case the pessary was partly placed in the external cervical canal and triggered labor the next day. Among the subgroup of 54 patients, the median uterocervical angle immediately after pessary placement was significantly more acute than that prior to placement in the 46 (85.2%) who delivered after 34 weeks (132° vs 146°, P < 0.01), but was unchanged in the eight patients who delivered before 34 weeks (143° vs 152°, P > 0.05). CONCLUSION: In patients at high risk for preterm delivery, correct placement of the Arabin cervical pessary should be checked immediately; this can be performed quickly and easily using MRI. This study provides some evidence that, in singleton pregnancies with a short cervix, a cervical pessary delays birth through a mechanical effect on the uterocervical angle.

Implementation of a piritramide based patient-controlled analgesia (PCA) as a standard of care for pain control in late abortion induction: A prospective cohort study from a patient perspective.

Objective: To assess whether the implementation of patient-controlled analgesia (PCA) with piritramide using an automatic pump system under routine conditions is effective to reduce pain in late abortion inductions. Study design: Prospective observational cohort study. Setting: Patients requiring medically indicated abortion induction from 14 weeks of pregnancy onwards between July 2019 and July 2020 at the department of Obstetrics and Prenatal Medicine of the Bonn University Hospital in Germany. Methods: Evaluation of pain management after implementation of a PCA system compared with previous nurse-controlled tramadol-based standard under routine conditions. Patients answered a validated pain questionnaire and requirement of rescue analgesics was assessed. Pain intensity and satisfaction were measured on a ten-point numeric rating scale. Main Outcome Measure Maximal pain intensity. Results: Forty patients were included. Patients using Piritramide-PCA complained of higher pain sores than those in the standard group (6.90 (± 2.34) vs. 4.83 (± 2.87), (p < 0.05)). In both groups the level of satisfaction with the analgesia received was comparable (8.00 (± 2.45) vs 7.67 (± 2.62), (p = 0.7)). Patients in the PCA group suffered more nausea (63.2 % vs 30 % respectively, OR 4.0, 95 % CI 1.05-15.20, p < 0.05) and expressed more the desire for more analgesic support compared to the control group (OR 5.7 (1-33.25), p = 0.05). Conclusion: Women with abortion induction after 14 weeks of gestation suffer from relevant severe pain, which requires adequate therapy. However, addition of PCA does not seem to bring any advantage in patients undergoing this procedure.

First trimester fetal echocardiography: where are we now?

The detailed study of the fetal cardiac anatomy in the first trimester of pregnancy by means of ultrasound is feasible whether using a transvaginal or a transabdominal approach. There is nowadays enough evidence that ultrasound in the first trimester of pregnancy is a safe procedure provided thermal and mechanical indices are taken into account. The best timing for successful imaging of the four chambers and great arteries in early gestation appears to be between around 13 to 14 weeks rather than 11 to 12 weeks. In experienced hands, first-trimester fetal echocardiography is quite sensitive for the detection of major structural cardiac abnormalities. Besides the nasal bone, markers for first trimester screening of chromosomal abnormalities such as nuchal translucency thickness, the flow in the ductus venosus and the flow through the tricuspid valve constitute also markers for cardiac abnormalities. The finding of an increased nuchal translucency, an abnormal flow in the ductus venosus or a tricuspid regurgitation constitutes an indication for more detailed fetal cardiac assessment. Other indication for a detailed cardiac assessment is the finding of an aberrant right subclavian artery and vascular anomalies. The emerging importance of these markers has caused renewed interest in the early study of the fetal heart.

Cytokines and parturition: investigating adiponectin, TNF-α, and IL-6 in mother-newborn pairs.

OBJECTIVE: Cytokines are reported to be associated with various pregnancy complications and health outcomes of the offspring. However, reference values during pregnancy have not been established, effects of clinical and obstetric factors have not been examined, and interactions between different cytokines have not yet been reported. METHODS: In this cross-sectional study, we recruited 120 mother-newborn pairs. Maternal venous blood samples (6 mL) were taken on admission to the labor ward; newborn venous blood samples (6 mL) were drawn from the placental part of the umbilical cord (UC). Adiponectin, TNF-α, and IL-6 serum concentrations were measured by commercial immunoassays. Clinical and obstetric variables were analyzed for their association with maternal and UC cytokine concentrations. RESULTS: Forty-six adiponectin pairs, 55 TNF-α pairs, and 14 IL-6 pairs were available for analysis. Correlation between UC and maternal adiponectin-, IL-6-, and TNF-α levels was low. We found a significant correlation of UC adiponectin with maternal brain-derived neurotrophic factor (BDNF) and maternal adiponectin, and between maternal leptin and maternal TNF-α. CONCLUSIONS: Clinical and obstetric variables as well as interactions between cytokines may have an impact on serum concentrations of the respective cytokines in maternal and UC blood. Further studies are required to confirm our findings.

Dramatically altered environmental lighting conditions in women with high-risk pregnancy during hospitalization.

The maternal circadian time structure is incredibly important in the entrainment and programing of the fetal and newborn circadian time structure. Natural sunlight is the primary environmental time cue for entrainment of circadian rhythms, but high-risk pregnant women spend most of their time indoors with artificial light sources and extremely low levels of natural light both during the day and night. Because the daily level, timing, duration of light exposure and its spectral properties are important in maintaining the normal circadian physiology in humans, we aimed to evaluate the environmental lighting conditions in high-risk pregnant women admitted to hospital for long-term stay. About 30 patients were included in the study. Exposed illuminance, color temperature and effective circadian radiation dose were measured and recorded every 10 s by light dosimeters attached to the patients' clothing. We documented the illuminance of 29 pregnant women on 235 inpatient days. Median (IQR) measured illuminance was 70 (28-173) lux in the morning, 124 (63-241) lux in the afternoon, 19 (6-53) lux in the evening and 0 (0-0) lux at the night. Median illuminance for the 235 inpatient days of assessment was below the recommended EU standard of 100 lux-60.5% of the mornings and 42.7% of the afternoons. The women confined to indoor locations rarely achieved an illuminances more than 300 lux in the morning and in the afternoon. Compared to women with outdoor mobility, those confined indoors have a significantly lower illuminance and color temperature, both in the morning and in the afternoon. Our study presents the first information about the dramatically altered environmental lighting conditions experienced by high-risk pregnant women during their hospital stay. Their exposure to light while in the hospital is significantly lower than exposure to natural daylight levels and below the recommended EU standard.

Impact of operator experience on the variability of fetal lung volume estimation by 3D-ultrasound (VOCAL) and magnetic resonance imaging in fetuses with congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the impact of operator experience in volumetric measurements on intra- and inter-observer variability of lung volume estimation by 3-dimensional (3D) ultrasound and magnetic resonance imaging (MRI) in fetuses with congenital diaphragmatic hernia (CDH). METHODS: We conducted a retrospective single-center study. Total fetal lung volume (TFLV) was measured twice using 52 stored 3D-ultrasound volumes and 52 corresponding MRI exams by a fetal medicine specialist with no experience in volumetric measurements and two operators experienced in the respective techniques. Bland-Altman analysis was performed to evaluate intra- and inter-observer variability. RESULTS: Measurements of TFLV by 3D-ultrasound showed higher intra-observer variability compared with MRI irrespective of operator experience, with narrower 95% limits of agreement on MRI as compared with ultrasound measurements. Similarly, 3D-ultrasound showed higher inter-observer variability as compared with MRI. Finally the 95% limits of agreement at ultrasound were quite comparable for intra-observer variability between the experienced and the inexperienced operator. CONCLUSION: Our study shows that operator experience has an impact on the variability of TFLV and, in the absence of experience in volumetric measurements, estimation of TFLV in fetuses with CDH by 3D-ultrasound shows higher variability than MRI measurements, but even in experienced hands variability is greater for 3D-ultrasound.

Determination of fetal body volume measurement at term with magnetic resonance imaging: effect of various factors.

PURPOSE: To evaluate various factors that potentially influence the fetal body volume (FBV) measurement using magnetic resonance imaging (MRI) and to analyze whether the technique of measurement could be simplified. MATERIALS AND METHODS: In 20 singleton pregnancies scheduled for a planned delivery, FBV measurements were performed by two independent operators on sagittal, axial and coronal planes and with various slice thickness and intersection gap, totalizing 100 examinations. MR estimation of fetal weight (MR-EFW) was calculated based on the equation developed by Baker. The relative error of MR-EFW was calculated in function of birth weight (BW). Regression analysis was used to investigate the effect on the relative error of MR-EFW of different variables but also to investigate the effect on the measurement time of the FBV of various factors. RESULTS: The mean relative error of MR-EFW was 1.96% and was significantly associated only with patient's BMI but not with the type of MR sequence used or other variables. Type of MR sequence used and BW were significantly associated with the measurement time of FBV. CONCLUSION: Using MRI, the time for FBV measurement can be significantly reduced using thicker slices or intersection gap, with similar accuracy.