Vein of Galen aneurysmal malformation (VGAM) in the fetus: retrospective analysis of perinatal prognostic indicators in a two-center series of 49 cases.

OBJECTIVE: Vein of Galen aneurysmal malformation (VGAM) is a rare fetal anomaly, the neurological outcome of which can be good with appropriate perinatal management. However, most fetal series are too small to allow reliable statistical assessment of potential prognostic indicators. Our aim was to assess, in a two-center series of 49 cases, the prognostic value of several prenatal variables, in order to identify possible prenatal indicators of poor outcome, in terms of mortality and cerebral disability. METHODS: This was a retrospective study involving 49 cases of VGAM diagnosed prenatally and managed at two centers over a 17-year period (1999-2015). All cases had undergone detailed prenatal cerebral and cardiac assessment by grayscale ultrasound, color and pulsed-wave Doppler and magnetic resonance imaging (MRI). Ultrasound and MRI examination reports and images were reviewed and outcome information was obtained from medical reports. Volume of the VGAM (on ultrasound and MRI) was calculated and development of straight-sinus dilatation, ventriculomegaly and other major brain abnormalities was noted. Cardiothoracic ratio, tricuspid regurgitation and reversed blood flow across the aortic isthmus were evaluated on fetal echocardiography. Major brain lesions were considered by definition to be associated with poor outcome in all cases. Pregnancy and fetoneonatal outcome were known in all cases. Fetoneonatal outcome and brain damage were considered as dependent variables in the statistical evaluation. Poor outcome was defined as death, late termination of pregnancy due to association with related severe brain anomalies or severe neurological impairment. RESULTS: At a mean follow-up time of 20 (range, 0-72) months, 36.7% of the whole series and 52.9% of the cases which did not undergo late termination were alive and free of adverse sequelae. Five (10.2%) cases showed progression of the lesion between diagnosis and delivery. On univariate analysis, dilatation of the straight sinus, VGAM volume ≥ 20 000 mm3 and tricuspid regurgitation were all significantly related to poor outcome. However, on logistic regression analysis, the only variables associated significantly with poor outcome were tricuspid regurgitation and, to a lesser extent, VGAM volume ≥ 20 000 mm3 . The former was also the only variable associated with brain damage. CONCLUSIONS: Major brain lesions, tricuspid regurgitation and, to a lesser extent, VGAM volume ≥ 20 000 mm3 are the only prenatal variables associated with poor outcome in fetal VGAM. Prenatal multidisciplinary counseling should be based on these variables. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Pulmonary stenosis in complicated monochorionic twin pregnancies: prevalence, management and outcome.

OBJECTIVE: To review prevalence, management and prognostic factors of pulmonary stenosis (PS) in monochorionic diamniotic (MCDA) pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). METHODS: Retrospective study over the last 10 years in a single referral center. We reviewed fetal echocardiography data of all MC twin cases with diagnosis of isolated PS. We assessed fetoscopy characteristics of those that underwent laser coagulation. We collected data regarding perinatal outcome, neonatal echocardiography and cardiac management. RESULTS: We found 24 cases of isolated PS among 2091 MCDA pregnancies. Among 1052 complicated MCDA that underwent fetal laser surgery, 22 (2.09%) developed PS of which 20 were diagnosed prenatally. Two cases were diagnosed in uncomplicated MCDA pregnancies (0.2%). Four of 22 (18.18%) cases with TTTS showed in utero regression after laser treatment. Thirteen newborns (65%) required valvular dilatation. Peak systolic velocities in the pulmonary artery trunk (PSV-PA) at diagnosis and the interval between the diagnosis of TTTS and that of PS were significantly different (p < 0.001 and p = 0.05 respectively) between PS requiring cardiac intervention and those who did not. CONCLUSION: An elevated PSV-PA at the time of PS diagnosis and a short time-interval between fetoscopic laser surgery and PS diagnosis are predictive of the need for interventional treatment after birth.

Placental elastography in a murine intrauterine growth restriction model.

OBJECTIVE: To compare placental elasticity in normal versus intrauterine growth restriction (IUGR) murine pregnancies using shear wave elastography (SWE). METHODS: Intrauterine growth restriction was created by ligation of the left uterine artery of Sprague-Dawley rats on E17. Ultrasonography (US) and elastography were performed 2 days later on exteriorized horns after laparotomy. Biparietal diameter (BPD) and abdominal diameter (AD) were measured and compared in each horn. Placental elasticity of each placenta was compared in the right and left horns, respectively, using the Young's modulus, which increases with increasing stiffness of the tissue. RESULTS: Two hundred seventeen feto-placental units from 18 rats were included. Fetuses in the left ligated horn had smaller biometric measurements than those in the right horn (6.7 vs 7.2 mm, p < 0.001, and 9.2 vs 11.2 mm, p < 0.001 for BPD and AD, respectively). Mean fetal weight was lower in the pups from the left than the right horn (1.65 vs 2.11 g; p < 0.001). Mean (SD) Young's modulus was higher for placentas from the left than the right horn (11.7 ± 1.5 kPa vs 8.01 ± 3.8 kPa, respectively; p < 0.001), indicating increased stiffness in placentas from the left than the right horn. There was an inverse relationship between fetal weight and placental elasticity (r = 0.42; p < 0.001). CONCLUSION: Shear wave elastography may be used to provide quantitative elasticity measurements of the placenta. In our model, placentas from IUGR fetuses demonstrated greater stiffness, which correlated with the degree of fetal growth restriction.

Active management of selective intrauterine growth restriction with abnormal Doppler in monochorionic diamniotic twin pregnancies diagnosed in the second trimester of pregnancy.

OBJECTIVE: This study aims to compare outcomes of active management of monochorionic diamniotic twin pregnancies complicated with severe intrauterine growth restriction (IUGR) of one twin before 24 weeks with continuous or intermittent absent or reversed end-diastolic flow (AREDF) in the umbilical artery, with or without twin-to-twin transfusion syndrome (TTTS). METHOD: This study is a retrospective comparison of 45 consecutive cases of severe selective IUGR (s-IUGR) defined as an estimated fetal weight at or below the fifth centile with a >25% weight discordance and AREDF in the umbilical artery before 24 weeks and 166 consecutive cases of TTTS stage III, with AREDF in the donor (TTTS3D) and also with s-IUGR. These were treated by either selective laser photocoagulation of chorionic vessels (SLPCV) or cord coagulation (CC). RESULTS: The 166 cases of TTTS3D were treated by SLPCV, whereas 23 and 22 cases of s-IUGR were treated by SLPCV and CC, respectively. Overall survival was 52.17% or 45.45% in s-IUGR treated by SLPCV or CC, respectively, and 48.49% in TTTS3D. The survival of appropriately grown for gestational age (AGA) twins following CC (90.9%) was higher than that following SLPCV in s-IUGR (74%) or in recipient twins of TTTS3D (55.42%) (p = 0.001). Survival of the IUGR twin was 30% and 41.56% with SLPCV in s-IUGR and TTTS3D, respectively. CONCLUSIONS: Active management of severe IUGR with AREDF in the umbilical artery seems beneficial. Survival rates with SLPCV were similar in s-IUGR and TTTS3D. However, there was a trend for higher survival rates in the AGA twin for CC. The choice of the technique should be driven by objective counseling on survival of both IUGR and AGA twins and therefore by the utility-based ethical values expressed by the pregnant woman.

Hidden mortality of prenatally diagnosed vein of Galen aneurysmal malformation: retrospective study and review of the literature.

OBJECTIVE: To evaluate the prognosis of prenatally diagnosed vein of Galen aneurysmal malformation (VGAM) in a large cohort with this condition and to review the literature on prenatally diagnosed VGAM. METHODS: This was a retrospective study of all cases of prenatally diagnosed VGAM managed in our referral center during a 12-year period. VGAM was categorized as being either isolated or associated with any other abnormality, based on fetal ultrasound and magnetic resonance imaging findings. Poor outcomes comprised termination of pregnancy with confirmation of antenatal findings, perinatal death and severe cardiac and/or neurological impairment in survivors. The literature was also reviewed for similar cases. RESULTS: Twenty-one cases of prenatally diagnosed VGAM were managed in our center. Four (19.0%) cases were isolated and 17 (81.0%) were associated with other anomalies. There were nine terminations (42.9%) and six neonatal deaths (28.6%). Six children (28.6%) were still alive at last follow-up, of whom three had abnormal neurological development. VGAM associated with other anomalies was strongly associated with a poor outcome compared with isolated forms (P < 0.0001). One hundred and nine cases from the literature were also reviewed. CONCLUSION: Fetuses with prenatally diagnosed VGAM have unexpectedly poor outcomes in the presence of cardiac or cerebral anomalies, while those with strictly isolated VGAM tend to have more favorable outcomes. Our literature review corroborates these findings.

Secondary bladder herniation in isolated gastroschisis justifies increased surveillance.

OBJECTIVES: To assess the perinatal outcome of fetuses with gastroschisis complicated by secondary bladder herniation. POPULATION AND MATERIALS: This was a retrospective study of all cases of isolated gastroschisis associated with bladder herniation managed at our institution. Prenatal ultrasound, obstetrical and perinatal information were collected. Pathology reports were also gathered. RESULTS: Out of 105 cases of gastroschisis managed at our institution, six (5.7%) were associated with secondary bladder herniation, two of them being diagnosed postnatally. Median gestational age at diagnosis of bladder herniation was 33.6 weeks (range 31-36) in five female and one male fetuses. Bladder herniation was associated with bowel dilatation in four cases (67%) and with pyelic dilatation in one case (17%). Despite increased surveillance, one male fetus died in utero. In four other cases, cesarean section was performed for fetal distress (three cases) or hyperechogenic bowels (one case). The five survivors had primary abdominal closure (n = 2) or staged repair (n = 3) with uneventful follow-up. CONCLUSION: Bladder herniation was present in 6% of apparently isolated gastroschisis. There was one intrauterine fetal death and four other cases were delivered for fetal distress. Increased surveillance seems justified.

Laser therapy for twin-to-twin transfusion syndrome (TTTS).

Monochorionic twins are subjected to specific complications which originate in either imbalance or abnormality of the single placenta serving two twins including twin-to-twin transfusion syndrome. The diagnosis is well established in overt clinical forms with the association of polyuric polyhydramnios and oliguric oligohydramnios. The best treatment of cases presenting before 26 weeks of gestion is fetoscopic laser ablation of the intertwin anastomoses on the chorionic plate. Although subjected to subtle variations, the core technique follows robust guidelines which could help understanding and acquiring the required skills and experience to perform this procedure. However appropriate and tailored hands-on training and appropriate perinatal set-up are critical not only for surgical management but also for the follow-up and management of related complications.

[Hygiene in endovaginal gynecologic and obstetrical ultrasound in 2008]. / Hygiène en échographie endocavitaire gynécologique et obstétricale en 2008.

Hygiene of ultrasound probes is a hot topic in obstetric and gynecologic ultrasound. The issue concerns mainly the risk of contamination and the need for specific procedures to ensure a high degree of protection against infectious diseases, even if a disposable barrier cover is used. As there are no referenced quality criteria for these disposable probe covers, it is crucial to verify that they are CE marked. The use of condoms as probe covers is not recommended even though they present a lower rate of disruption compared to commercial probe covers. The global rate of probe cover perforation is of 1 to 9%. To date, there are no case reports of contamination by ultrasound examination, be it vaginal or abdominal. Nevertheless, every patient must be regarded as a potential source of infection. The French recommendations consider endovaginal ultrasound probes as semi-critical instruments and thus require intermediate-level disinfection. Because of the risk of disruption, American as well as Canadian and Australian recommendations insist on high-level disinfection of the probe. Every operator should follow reasonable hygiene rules and appropriate precautions should be taken in order to reassure the patients about the risks of contamination during any ultrasound examination.

[Impact of sonography simulation in the training of midwifery students]. / Impact de l'intégration de la simulation en échographie obstétricale dans le cursus des étudiants sages-femmes.

OBJECTIVE: During their studies, French midwives need to acquire basic ultrasound skills to measure the amniotic fluid index, fetal biometry and identify fetal lie and placental positions. They have a clinical ultrasound training during their curriculum to acquire these skills. Assess the contribution that a training on an obstetrical ultrasound simulator (OUS) could make to the competence of midwives and to evaluate the best time to integrate it into their curriculum. METHODS: Forty students in their 4th and final year in midwifery were randomized into 2 groups. Group A learned with the traditional academic course, with a clinical ultrasound training which was followed by an evaluation on an OUS (E1A). Group B was evaluated on a simulator before the clinical ultrasound training (E1B). After these initial assessments, both groups were provided with learning and training sessions on OUS, and re-evaluated in the same exercise (E2A and E2B). Group B then completed its clinical ultrasound training and was evaluated one month later on the simulator (E3B). The evaluation consisted of scoring the biometry images according to previously published quality criteria, and of assessing their competence based on a published objective and structured assessment score of ultrasound skills (OSAUS). RESULTS: Evaluation 1 found comparable OSAUS scores (2.375/5 vs. 2.24/5 P=0.52) between the two groups (E1A vs. E1B) but a significantly higher image quality score for Group A (P=0.02). In Evaluation 2, an improvement was noted for both groups with comparable OSAUS averages between the two groups (P=0.76). After the clinical training of Group B, their OSAUS score (E3B) was significantly better than that of Group A (E1A) after their clinical training (3.45/5 vs. 2.375/5 [P=0.00017]). The same was true for the quality of their images (12.67/16 vs. 9.95/16 [P=0.003]). CONCLUSION: Ultrasound simulation-based training, as an adjunct to ultrasound clinical training, significantly improves obstetrical ultrasound skills. The best time to train on simulators seems to be prior to clinical training on real patients.