ObjectiveDescribe outcomes of open fetal surgery for myelomeningocele (MMC) repair in two Brazilian hospitals and the impact of surgical experience on outcome.DesignRetrospective cohort study.SettingSao Paulo, Brazil.Population237 pregnant women carrying a fetus with an open spinal defect.MethodsSurgical details, and maternal and fetal outcomes collected from all patients.Main outcome measuresAnalysis of surgical and perinatal outcome parameters.ResultsTotal surgical time was 119 ± 7.6 minutes. Preterm labour occurred in 24.2%, premature rupture of membranes in 26.7%, placental abruption in 0.8%, need for a blood transfusion at delivery in 2.1%, and dehiscence at the repair site in 2.5%. Reversal of hindbrain herniation at birth occurred in 71.4%. There were no maternal deaths or severe maternal morbidities. The failure rate with the patient anaesthetised was 0.42% and perinatal mortality was 2.1% (three intrauterine demises and two neonatal deaths). Comparing results from our study in the first 3 years with the last 3 years demonstrated improvement in the total surgical time (121.2 ± 6.4 versus 118.5 ± 8.2 minutes, P = 0.005) and an increase in reversal of hindbrain herniation at birth (64.0 versus 77.1%, P = 0.042).ConclusionOur open fetal surgical approach for MMC was effective and results were comparable to past studies. Improvements in surgical performance and perinatal outcome increased as the surgical team became more familiar with the procedure.FundingThe study was funded solely by institutional funds.Tweetable abstractBrazilian experience of in utero open surgery for myelomeningocele repair.

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.

OBJECTIVEIn this study, the authors retrospectively reviewed two cohorts of patients with occipital encephalocele (OE) and demonstrate the technical aspects of an innovative and unprecedented method for fetal OE correction.METHODSThis was a cross-sectional retrospective study of 22 patients who underwent surgery from July 2012 to July 2018. The inclusion criteria for participants were a gestational age between 19 weeks and 27 weeks, a maternal age ≥ 18 years, a normal fetal karyotype, the presence of microcephaly, and a cystic hernial sac with the cystic component accounting for more than 80% of the total volume, which was larger than 10 ml. The fetuses with OE and microcephaly underwent intrauterine repair. The exclusion criteria were the presence of a fetal anomaly not associated with OE, chromosomopathy, presence of the brainstem inside the hernial sac or venous sinuses inside the herniated content, the risk of premature birth, placenta previa, and maternal conditions that would constitute an additional risk for fetal and maternal health, as well as refusal for fetal surgery. The authors reviewed the potential for microcephaly reversal and the long-term neurocognitive development.RESULTSThe authors report the cases of 9 fetuses with OE and microcephaly who underwent intrauterine OE repair at gestational ages of less than 27 weeks (fetal group). One case selected for fetal surgery also presented with placental abruption. All patients who underwent the fetal operation had microcephaly reversal, and 3 patients required ventriculoperitoneal shunting in the 1st year of life. The authors reviewed the cases of 12 patients who underwent postnatal repair. In 10 cases, fetal surgery was refused (postnatal group), and in 2 cases, the inclusion criteria for fetal surgery were not met. The authors evaluated the neurocognitive development of the patients with the Bayley Scales of Infant Development II. The median score for the fetal group was 98.7, and that for the postnatal group was 27.8.CONCLUSIONSThe intrauterine repair of OE may stop the progression of encephalocele sac herniation and result in microcephaly reversal. The fetal group had a better cognitive outcome than the postnatal group. The technique required to correct this defect is feasible for those with previous experience in the correction of fetal myelomeningocele. However, more studies are needed to ensure the efficacy of this procedure.

Objective. The purpose of this study was to assess the reliability and validity of in vitro volume calculations by 3‐dimensional ultrasonography. Methods. This observational study was performed by 2 examiners to obtain volumes of 3 objects of different shapes and sizes filled with ultrasound gel and immersed in water. The examiners used the multiplanar (5‐mm interval), virtual organ computer‐aided analysis (VOCAL, 30°) and extended imaging (XI) VOCAL (5, 10, 15, and 20 planes) methods to estimate the volumes of each object. A paired Student t test (P) and intraclass correlation coefficients (ICCs) were used to assess reproducibility of the methods. Validity was assessed comparing the percent differences between the estimated and the real volumes using the P value, mean differences, and ICC for each method. Results. All methods were highly reliable and valid. There were no significant differences in interobserver variability; there was a strong interobserver correlation. There were no significant differences in the percent differences between the estimated and real volumes of the objects using the 3 methods. The XI VOCAL method was superior to the multiplanar and VOCAL methods in the measurement of irregularly shaped objects. The XI VOCAL method with 10 planes estimated volumes closest to the real volumes. Conclusions. All 3 methods were reliable and valid; however, XI VOCAL was superior to the other methods in the measurement of irregularly shaped objects.

Objective: The aim of this study was to assess fetal hemodynamics during intrauterine open surgery for myelomeningocele (MMC) repair by describing fetal heart rate (FHR) monitoring in detail related to each part of the procedure. Methods: A study was performed with 57 fetuses submitted to intrauterine MMC repair between the 24th and 27th week of gestation. Evaluations of FHR were made in specific periods: before anesthesia, after anesthesia, at the beginning of laparotomy, during uterus abdominal withdrawal, hysterotomy, neurosurgery (before incision, during early skin manipulation, spinal cord releasing, and at the end of neurosurgery), abdominal cavity reintroduction, and abdominal closure, and at the end of surgery. Means ± standard deviations of FHR were established for each period, and analysis of variance with repeated measures was used to assess differences between these periods. The mean differences were assessed with 95% confidence intervals and were analyzed by Tukey's multiple comparison test. Results: The mean FHR during the specific periods mentioned above was 140.2, 140, 139.2, 138.8, 135.1, 133.9, 123.1, 134.0, 134.5, 137.9, and 139.9 bpm, respectively (p < 0.0001). Comparing the different periods, the highest frequencies were observed in the initial and final moments. The neurosurgery stage presents lower frequencies, especially during the release of the spinal cord. Conclusion: FHR monitoring revealed interesting findings in terms of physiological fetal changes during MMC repair, especially during neurosurgery, which was the most critical period.

Three-dimensional volumetry of fetal LVs by VOCAL method has good agreement with fetal MRI in fetuses with ventriculomegaly and may be used as an additional tool in patient counseling and prognosis prediction.

Central nervous system malformations constitute the second most common group of anomalies in fetuses. Such malformations have assumed clinical importance because of their association with high rates of perinatal morbidity and mortality. Therefore, it is extremely important to assess the fetal central nervous system during the prenatal period, in order to identify any changes in its development and thereby gain sufficient information to advise parents about pregnancy follow-up, options for fetal therapy, and the timing/type of delivery, as well as the postnatal treatment and prognosis. The objective of this review was to describe the ultrasonographic evaluation of the fetal central nervous system as per the guidelines of the International Society of Ultrasound in Obstetrics and Gynecology.

During prenatal evaluation, detailed ultrasonographic assessment of the entire spine with the identification of the position and morphology of the conus medullaris and absence of cranial signs of spinal dysraphism are the most valuable sonographic clues for the diagnostic of the CSB.