H. Milani

Fetal brain tumors: Prenatal diagnosis by ultrasound and magnetic resonance imaging.

Congenital central nervous system tumors diagnosed during pregnancy are rare, and often have a poor prognosis. The most frequent type is the teratoma. Use of ultrasound and magnetic resonance image allows the suspicion of brain tumors during pregnancy. However, the definitive diagnosis is only confirmed after birth by histology. The purpose of this mini-review article is to describe the general clinical aspects of intracranial tumors and describe the main fetal brain tumors.

Histological evidence of reparative activity in chorioamniotic membrane following open fetal surgery for myelomeningocele

An increased understanding of the reparative process in fetal membrane following surgical techniques may be helpful to decrease the risks to mother and fetus and avoid adverse pregnancy outcomes. The present study discusses histological evaluation of the fetal membrane following open fetal surgery. Chorioamniotic membranes (n=10) were obtained following birth from pregnancies that underwent open fetal surgery for myelomeningocele. The collagen distribution was quantified using picrosirius-polarization method comparing the suture site with non-suture site. The differences between the collagen fiber percentages at the two sites was evaluated by the paired t-test with P<0.05. The mean gestational age of fetal surgery was 26.09±0.3 and 33.81±0.82 weeks at birth. The picrosirius red sign was more intense at the suture site, primarily associated with collagen type 1. Collagen observed in the surgical area was significantly increased (13.22±2.84%) compared with the non-surgical area (6.16±1.09%; P<0.0001). It was observed that the reparative activity at the suture site of the fetal membrane was characterized by a significant increase in collagen fibers. The findings suggest nascent collagen synthesis, tissue remodeling and repair of suture site, a mechanism likely to prevent the amniotic fluid leakage and maintain pregnancy following open fetal surgery.

P09.15: Three‐dimensional sonographic volumetry of phantom objects: comparing rotational method Virtual Organ Computer‐aided AnaLysis (VOCAL) to multi slice method Extended Imaging (XI) VOCAL

obtain the values of EV: XI VOCAL method. Using a sequential section, the contour of each gestational embryo was drawn manually in the fifteen different rotation planes to obtain the 3D volume measurement. To evaluate the relation between EV and gestational age (GA), Person’s correlation coefficient (r) was used. The paired Student’s t-test (P) and ANOVA were used to compare the methods. Results: The study showed there was correlation between EV and GA (r = 0.83 for VOCAL 30 degrees, r = 0.83 for XI VOCAL and r = 0.80 for multiplanar). There was a strong correlation between EV measured by the XI VOCAL e multiplanar methods (RICC = 0,941) and XI VOCAL and VOCAL methods (RICC= 0,965). There weren’t difference statistics between EV measured by the VOCAL, XI VOCAL and the multiplanar by the paired Student’s t-test, but ANOVA showed differences between all methods (p < 0.001). Conclusion: We have demonstrated a strong correlation with the age gestational and embryo volume measurements obtained using the XI VOCAL, VOCAL and multiplanar methods in first trimester gestational. The methods were concordant, but there were differences statistics.

Amniotic fluid lactic acid and matrix metalloproteinase‐8 levels at the time of fetal surgery for a spine defect: association with subsequent preterm prelabour rupture of membranes

In utero fetal surgery to correct incomplete closure of the spinal cord lessens the extent of permanent damage but is associated with preterm prelabour rupture of membranes (PPROM). We determined whether compounds in amniotic fluid collected at the time of surgery predicted subsequent development of PPROM.

P24.11: Fetal neurosonographic aspects of the Zika virus infection

Objectives: Zika virus epidemic occurred in Brazil in 2015 was associated with an increase in the number of cases of microcephaly. Currently, the association between fetal central nervous system (CNS) onset and congenital infection by zika virus is known. The aim of this study was to describe the main neurosonographic manifestations observed in cases of zika virus infection with fetal brain lesions followed up during 2016 in the fetal neurology sector of the Federal University of São Paulo and in the centro paulista de medicina fetal. Methods: 10 pregnant women with suspected changes in fetal brain morphology and diagnosed with zika virus infection were followed up during 2016. Maternal diagnosis was confirmed by serology or urine test. In all cases, a detailed examination of the fetal CNS morphology was performed by neurosonography to describe the main cerebral changes observed. Results: Main neurosonographic findings were: mild ventricumegaly 4 cases (40%); periventricular calcifications 6 cases (60%); calcifications in caudate nucleus 7 (70%); thalamic calcifications 3 cases (30%); polymicrogyria 2 cases (20%); schizencephaly 2 cases (20%); severe ventriculomegaly 6 (60%); cerebellar hypotrophy 5 cases (60%); corpus callosum dysgenesis 10 cases (100%); cerebral sulcation delay 10 (100%); increased subarachnoid space 4 (40%); microcephaly 4 (40%). Conclusions: Zika virus infection can lead to changes in the fetal CNS, although these manifestations are not specific for this infection. However, detailed assessment of brain morphology is very important to correct counselling and follow-up of these cases.

EP20.06: Initial experience of a fetal medicine service in the accomplishment of fetoscopy for the treatment of feto-fetal transfusion syndrome

Objectives: To examine the diagnostic indices of fetal middle cerebral artery peak systolic velocity (MCA PSV) above 1.55 multiples of the median(MoM) Doppler velocimetry for the identification of fetal severe anemia in monochorionic diamniotic (MCDA) twin pregnancies. Methods: Study population consisted of 178 MCDA twin fetuses who underwent MCA PSV Doppler velocimetry within 7 days of delivery between gestational age (GA) of 24 and 38 weeks at Seoul National University Hospital between 2005 and February 2017. Fetuses with chromosomal abnormalities, major congenital anomalies or intrauterine demise were excluded. We examined the frequency of severe fetal anemia according to the presence of an increased fetal MCA PSV (≥1.55 MoM). Severe anemia was defined in the presence of hemoglobin concentration < 0.55 MoM in umbilical cord blood at the time of delivery. Results: An increased MCA PSV (≥1.55 MoM) was present in 8.4% of study population and severe anemia was present in 2.2%. Diagnostic indices of an increased MCA PSV (≥1.55 MoM) for the identification of severe anemia were as follows: sensitivity 100% (4/4), specificity 93.7% (163/14), positive predictive value (PPV) 26.7% (4/15) and negative predictive value 100% (163/163). However, PPV at GA 30 weeks or more was only 12.5%, while that at GA less than 30 weeks was 42.9%. Conclusions: The sensitivity and specificity of an increased MCA PSV (≥1.55 MoM) for the identification of severe anemia in MCDA twin pregnancies are very high. However, PPV of that is much lower in cases with GA 30 weeks or more than in those with GA less than less than 30 weeks among MCDA twin pregnancies.

OP07.02: The relationship between the posterior fossa structures and foramen magnum using the occipitum‐dens line (ODL) in following fetal surgery for myelomeningocele

Objectives: Comparing the accuracy of Neurosonography and Magnetic Resonance Imaging (MRI) in the diagnosis of fetal central nervous system (CNS) anomalies and evaluating the positive predictive value (PPV) of both procedures as for short-term and long-term neonatal outcomes. Methods: 80 patients referred to the Fetal Medicine AOUI Verona for a 20 weeks scan positive for a brain anomaly have been studied from 2008-2015. All patients underwent expert ultrasound examination of the fetal CNS, MRI and MRI post-delivery. The accuracy and sensibility of US expert scan and MRI were statistically compared by retrospective analysis using postnatal MRI, necropsy (in two cases of TOP) and postnatal neuropsychiatric outcome as gold standard. Postnatal evaluation was obtained through the analysis of the medical records of Child Neuropsychiatry and Neonatology and through telephone interviews with the children’s mothers. Results: MRI has shown a higher accuracy with 61/80 correct diagnoses, compared to 41/80 of Ultrasound. The difference between them is statistically significant only in the corpus callosum agenesia (ACC) (p-value< 0,0001). Detection Rates of Neurosonography and MRI are similar (63% and 60% respectively) whereas MRI has had a better PPV: 72,5% vs 51%. MRI has contributed to the prenatal diagnosis in general in 30% of cases, whereas its contribution rises to 73% as to the ACC. Postnatal psychiatric outcome has been obtained in 78 cases. 21/78 cases are clinically pathologic. As to long-term postnatal outcomes the PPV is 22% for Ultrasound and 27% for MRI. Conclusions: MRI might play a significant role in the diagnosis of ACC as well as in the cases that have to experience surgery after birth whereas it is less useful in case of isolated ventriculomegalia. MRI has no added value with respect to the neuropsychiatric outcome. Neuropsychiatric troubles are mild for ACC and severe for posterior fossa malformations. Postnatal MRI still might have a role for a long-term follow-up school-age patients.

OC16.04: Intrauterine open fetal surgery for correction of isolated occipital encephalocele

83 to 450 minutes. A total of 42 cases already delivered (4 uneventful vaginal), no uterine dehiscence observed. PPROM occurred in 34/42 (81%) cases. Fetuses remained in utero on average for 6,1w (5d to 13w). Mean GA at delivery was 32,6w, only 16,7% below 30w, mean birthweight was 1906g (890 to 2960g). No intrauterine demise occurred. Two neonatal deaths due to sepsis occurred. VP shunt was placed in 42% cases and two infants died due to shunt related infection. Long-term follow up was obtained in 38 cases, mean 8,9 months. The functional level of the lesion was assessed in total 23 cases and was found to be one or more levels better than the anatomical level in 70%. Conclusions: Entirely percutaneous fetoscopic correction is feasible and safer than open fetal approach; however, it is associated with an increased risk of PPROM. Fetoscopic correction can be used in cases were open surgery cannot. The CECAM technique statistically significantly improves motor outcome, when compared to MOMS trial results, so it is not only safer for mothers but also better for babies.

P20.07: Interhemisferic variant of holoprosencephaly (MIH) or syntelencephaly: the role of fetal brain MRI in differential diagnosis

postnatal or postmortem examination. The frequency of agreement between the ultrasound modalities was estimated and compared using the McNemar-Bower test. Results: Regarding to the ultrasonographic signs, the local and the remote examiners had high correct rates for 2D US and 3D US (80.0%, 84.0% and 76.0% e 86.0%, respectively). Similarly, the diagnosis for congenital anomalies had a better percentage for 3D US compared for 2D US for the remote examiner (92.3 vs. 84.6%) and equal for the local one (96.2% vs. 96.2%), respectively. Regarding to the ultrasonographic signs and the diagnosis of congenital anomalies, comparing the 2D US vs. 3D US performed by the same examiner the local and remote ones did not find any statistically significant difference. Conclusions: In spite of the slightly increased percentage in the ultrasonographic signs of the 3D US compared to 2D US for congenital anomalies from both examiners and in its diagnosis for the remote examiner, it did not reach any statistically significant difference.

P05.10: Septo-optic dysplasia: a differential diagnosis in fetus with absence of the septum pellucidum—case report

septum pellucidum (SP), hypoplasia of the optic chiasma and nerves, and various types of pituitary dysfunction. The etiology of SOD is unknown. Most cases are sporadic, but a familial occurrence has been noted which raises the possibility of autosomal recessive inheritance. Sonographic demonstration in utero of isolated absence of the SP with central fusion of the frontal horns is a clue to the diagnosis of DSO, which is confirmed after birth with findings of the magnetic resonance imaging (MRI), direct ophthalmoscopy and pituitary hormonal deficiency. Case report: A healthy 28 year-old woman, gravida 2 para 1, was referred at 33 weeks’ gestation to the UNIFESP Fetal Medicine Service on suspicion of fetal ventriculomegaly. Sonographic examination revealed absence of the SP and mild ventriculomegaly. A Fetal MRI confirmed the sonographic findings and showed a probable hypoplasia of the optic nerves. The possibility of DSO was raised. A full-term 3240g male infant was delivered by Cesarean. Postnatal investigation confirmed the diagnosis of SOD with the following findings: RMI showed absence of the SP and mild ventriculomegaly, hypoplasia of the optic nerves by direct ophthamoscopy, and pituitary dysfunction. Currently the child has been followed at the UNIFESP endocrinology and neurology service. Discussion: Absence of the SP is a rare congenital brain malformation and occurs in 2-3 : 100.000 individuals in the general population. It may occur without other brain anomalies, or may be associated with holoprosencephaly, schizencephaly, agenesis of the corpus callosum and DSO. Sonographic demonstration in utero of isolated absence of the SP with central fusion of the frontal horns is a clue to the diagnosis of DSO. However, the diagnosis of SOD is only confirmed with postnatal investigation, with findings of the MRI, direct ophthamoscopy and pituitary hormonal deficiency.