Fetal neurosurgery.

Among fetal surgical procedures, neurosurgery stands out due to the number of cases and the possibility of developing new procedures that can be performed in the fetal period. To perform fetal neurosurgical procedures, there is a need for specialized centers that have experts in the diagnosis of fetal pathologies and a highly complex obstetrics service with specialized maternal-fetal teams associated with a pediatric neurosurgery center with expertise in the diverse pathologies of the fetus and the central nervous system that offers multidisciplinary follow-up during postnatal life. Services that do not have these characteristics should refer their patients to these centers to obtain better treatment results. It is essential that the fetal neurosurgical procedure be performed by a pediatric neurosurgeon with extensive experience, as he will be responsible for monitoring these patients in the postnatal period and for several years. The objective of this manuscript is to demonstrate the diagnostic and treatment possibilities, in the fetal period, of some neurosurgical diseases such as hydrocephalus, tumors, occipital encephalocele, and myelomeningocele.

Training model for the fetal myelomeningocele correction with multiportal endoscopic technique.

PURPOSE: The recent history of myelomeningocele has shown that treatment during the fetal life may reduce the risk of developing hydrocephalus in individuals by approximately 50%. Thus, a significant advancement involves fetal surgery performed through an endoscopic technique in which portals are placed to introduce the forceps and laparoscopic instruments. However, the development of this technique requires training; therefore, this study aimed to develop a training model for fetal myelomeningocele repair technique with multi-portal endoscopy. METHODS: Two stages of endoscopic technique development were performed. The first stage consisted of exercises in order to familiarize the surgeon with 2D-vision endoscopic surgery, associated with the application of exercises focused on surgical skills, such as the development of laparoscopic knots in a synthetic model. The second stage involved the creation and application of the stages of myelomeningocele closure with a non-living animal model consisting of a chicken breast to simulate the myelomeningocele and a basketball to simulate the gravid uterus, in which perforations were made to introduce vascular introducers (portals) that, as in vivo, are used as portals (trocars) for the introduction of laparoscopic instruments. Overall, two different scenarios with three portals and two portals were tested. RESULTS: In three-portal simulator, the triangular apex trocar was used for the introduction of 4-mm 0° or 30° optics or even Minop type neurodoscope (Aesculap®, Germany) that was operated by the assistant surgeon; the other two portals are used for the introduction of laparoscopic instruments. Thus, the surgeon is able to perform maneuvers bimanually since dissection to laparoscopic sutures. In two-portal simulator, the surgeon and assistant stay side by side and one of the portals is used for the optic and the other for the laparoscopic instruments. There is no possibility of bimanual dissection in this method. CONCLUSION: Realistic simulation models for endoscopic fetal surgery for myelomeningocele correction are easily performed and help develop the necessary skills for fetal surgery teams.

Assessment of lower limb trophism in fetuses with open spina bifida using fractional thigh volume of three-dimensional ultrasound.

OBJECTIVE: To assess the trophism of the lower limbs of fetuses with open spina bifida using fractional thigh volume (TVOL) of three-dimensional (3D) ultrasound. METHODS: A prospective cross-sectional study was carried out with normal fetuses and with open spina bifida (myelomeningocele and rachischisis) at 26 weeks' gestation. The TVOL (delimitation of five cross-sectional areas of the middle portion of the limb) was evaluated, as well as the subjective assessment of hypotrophy and lower limb movement. RESULTS: Thirty-one fetuses with open spina bifida, 21 with myelomeningocele and 10 with rachischisis, and 51 normal fetuses were included. There were no significant differences in the TVOL between normal and spina bifida fetuses (p = 0.623), as well as between normal fetuses, with myelomeningocele and with rachischisis (p = 0.148). There was no significant difference in the TVOL of fetuses with spina bifida with or without lower limb hypotrophy (p = 0.148). Fetuses with spina bifida and with lower limb movement had higher TVOL values than fetuses without lower limb movement (p = 0.002). CONCLUSION: There were no significant differences in the TVOL measurement of normal and spina bifida fetuses (rachischisis and myelomeningocele). Fetuses with spina bifida without spontaneous movement of the lower limbs had lower TVOL values.

Ultrasound evaluation of uterine scar thickness after open fetal surgery for myelomeningocele.

PURPOSE: This study aimed to analyse the evolution of uterine scar thickness after open fetal surgery for myelomeningocele (MMC) by ultrasonography, and to establish a cut-off point for uterine scar thickness associated with high-risk of uterine rupture. METHODS: A prospective longitudinal study was conducted with 77 pregnant women who underwent open fetal surgery for MMC between 24 and 27 weeks of gestation. After fetal surgery, ultrasound follow-up was performed once a week, and the scar on the uterine wall was evaluated and its thickness was measured by transabdominal ultrasound. At least five measurements of the uterine scar thickness were performed during pregnancy. A receiver operating characteristics (ROC) curve was constructed to obtain a cut-off point for the thickness of the scar capable of detecting the absence of thinning. Kaplan-Meier curves were constructed to evaluate the probability of thinning during pregnancy follow-up. RESULTS: The mean ± standard deviation of maternal age (years), gestational age at surgery (weeks), gestational age at delivery (weeks), and birth weight (g) were 30.6 ± 4.5, 26.1 ± 0.8, 34.3 ± 1.2 and 2287.4 ± 334.4, respectively. Thinning was observed in 23 patients (29.9%). Pregnant women with no thinning had an average of 17.1 ± 5.2 min longer surgery time than pregnant women with thinning. A decrease of 1.0 mm in the thickness of the uterine scar was associated with an increased likelihood of thinning by 1.81-fold (95% confidence interval [CI]: 1.32-2.47; p < 0.001). The area below the ROC curve was 0.899 (95% CI: 0.806-0.954; p < 0.001), and the cut-off point was ≤ 3.0 mm, which simultaneously presented greater sensitivity and specificity. After 63 days of surgery, the probability of uterine scarring was 50% (95% CI: 58-69). CONCLUSION: A cut-off point of ≤ 3.0 mm in the thickness of the uterine scar after open fetal surgery for MMC may be used during ultrasonography monitoring for decision-making regarding the risk of uterine rupture and indication of caesarean section.

Measurement of the Area and Circumference of the Leg: Preliminary Results of a New Method for Estimating Leg Muscle Trophism in Fetuses With Open Lumbosacral Spina Bifida.

OBJECTIVE: To standardize a new technique to evaluate the fetal leg muscle trophism by measuring the area and circumference, and comparing this technique between normal and fetuses with open lumbosacral spina bifida (SB). METHODS: Observational cross-sectional study was carried out on pregnant women with 26 weeks who were divided into two groups: Group I-fetuses with diagnosis of open lumbosacral SB; Group II-normal fetuses (control). In fetuses with SB, subjective evaluation of the lower limbs was performed (muscle echogenicity and leg movements). To estimate the leg muscle trophism, the measurement of the area and circumference of the leg were standardized, and the reproducibility of this method was performed. RESULTS: Thirty-one fetuses with open lumbosacral SB and 51 normal fetuses were evaluated. The measurements of the area and circumference of the leg proved to be highly reproducible (intraclass correlation coefficient-ICC > 0.95). The leg area and circumference measurements were statistically lower in the SB group than in the control group (p < .001). When subjective ultrasound assessment demonstrated hypotrophy of the lower limbs, the measurements of the area and circumference of the leg were statistically lower when compared to normal fetuses (p < .001). Fetuses with open SB with abnormal lower limb movements had lower measurements of the area and circumference of the leg than fetuses with normal movements (p < .001). CONCLUSION: A new technique for estimating fetal leg muscle trophism was standardized, which proved to be highly reproducible and was able to show the difference between normal and fetuses with SB.

Hemorrhage due to attempted excision of a frontal exophytic lesion: A cautionary tale.

BACKGROUND: Despite mainly benign, exophytic subcutaneous cranial masses present with a myriad of differential diagnosis possibilities, ranging from simple, superficial lesions to complex lesions involving the central nervous system. Although the gold standard imaging modality for the diagnosis of these lesions is magnetic resonance imaging, Doppler Ultrasonography can be a useful, inexpensive, and available tool for evaluation of lesions that could potentially be safely treated in the primary care setting, and lesions that would demand advanced neurosurgical care. CASE DESCRIPTION: This patient presented with a complex exophytic plasmocytoma that was first diagnosed and erroneously approached as a subcutaneous lipoma with surgical resection in an outpatient surgical setting. This interpretive approach resulted in the failure of the procedure due to significant hemorrhage. The patient was immediately referred to neurosurgical care and transferred to our center. Admission doppler ultrasound imaging revealed absence of the frontal bone, the enriched and profuse vascularization, allowing further and proper diagnostic approach and treatment. CONCLUSION: Ultrasound could be a reliable, fast, and simple imaging method aiding practitioners to perform a better workup for patients with exophytic subcutaneous cranial masses.

Hydrocephalus in myelomeningocele.

PURPOSE: To investigate certain aspects of hydrocephalus in patients with myelomeningocele. METHODS: We retrospectively analyzed data of 1050 patients with myelomeningocele who underwent surgical treatment between June 1991 and June 2021. These patients were divided into three groups: group 1 consisted of patients who underwent surgery after the first 6 h of life, group 2 consisted of patients who underwent surgery within the first 6 h, and group 3 consisted of patients who underwent surgery during the fetal period and before 26 6/7 weeks of gestation. RESULTS: There were 125, 590, and 335 patients in groups 1, 2, and 3, respectively. In groups 1 and 2, 593 (83%) patients developed hydrocephalus after birth and required ventriculoperitoneal shunt placement in the maternity ward, mainly within the first 4 days of life. In contrast, in group 3, 24 (7.2%) patients required surgery to treat hydrocephalus after birth. Hydrocephalus was the primary cause of mortality in groups 1 and 2, with mortality rates of 35% and 10%, respectively. In group 3, the mortality rate was 0.8% and was not related to hydrocephalus. CONCLUSION: The onset of hydrocephalus is directly related to myelomeningocele closure in neurosurgery.

Functional Motor Skills in Children Who Underwent Fetal Myelomeningocele Repair: Does Anatomic Level Matter?

BACKGROUND: In this study, we evaluated children who underwent prenatal myelomeningocele (MMC) repair to investigate the influence of the anatomical level of the lesion on functional motor skills and congenital orthopedic malformations. METHODS: This cross-sectional study evaluated children who underwent prenatal correction. The anatomical level of the lesion was classified by observing the magnetic resonance of the spine. The sensory/motor assessment was performed by physical examination to classify the status of ambulation, functional level, and functional performance according to chronological age using the Pediatric Evaluation of Disability Inventory (PEDI-CAT) scale. RESULTS: One hundred cases were evaluated; for 60%, lesions were located in the upper lumbar region and for 40%, they were located in the lower lumbar and sacral regions. The functionality, measured by the PEDI-CAT scale, showed a normal average according to age (mean 50), with 71% of children being community ambulators. Congenital orthopedic malformations were also unrelated to the injury levels, except for knee dislocation in relation to upper lumbar injury. At the functional level, we observed that for the majority, the levels of function of at least 2 vertebrae were below the anatomical level. CONCLUSIONS: There were no differences in functional motor skills, walking pattern, or congenital orthopedic malformation when compared with the anatomical level of injury in patients who underwent prenatal repair of MMC, except for congenital knee dislocation.

Fetal surgery for occipital encephalocele.

OBJECTIVE: In 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. METHODS: This 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. RESULTS: The 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. CONCLUSIONS: The 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.

Alterations in skull base anatomy in intrauterine and postnatal repaired myelomeningoceles.

PURPOSE: The aim of this study was to analyze the skull base anatomy of patients who underwent intrauterine or postnatal myelomeningocele repair and to determine its relationship with hydrocephalus. METHODS: This was a retrospective cross-sectional study that analyzed three groups: the postnatal group, 57 patients who underwent myelomeningocele repair up to 48 h after birth; the fetal group, 70 patients who underwent myelomeningocele repair between 19 and 27 weeks of gestation; and a control group (65). We compared the rate of hydrocephalus treatment, the clivus-supraocciput angle (CSA), and the Welcher angle. RESULTS: The mean CSA in the fetal group was 87.6°, and the postnatal group was significantly different at 78.3° (p < 0.0001). The control group (89.1°) was significantly different from the postnatal group but not from the fetal group. The mean Welcher angle was not significantly different between the groups. There was an 8.5% rate of surgical treatment for hydrocephalus in the fetal group, compared with 73.6% in the postnatal group. CONCLUSIONS: The CSA in the fetal group was larger than that in the postnatal group, which may explain the decrease in the prevalence of hydrocephalus in the fetal group.

Fetoscopic laser photocoagulation for twin-to-twin transfusion syndrome treatment: initial experience in tertiary reference center in Brazil.

OBJECTIVE: To evaluate the initial maternal and perinatal outcomes of fetoscopic laser photocoagulation for the treatment of twin-to-twin transfusion syndrome (TTTS) in a referral center in Brazil. METHODS: This prospective observational study analyzed 24 fetoscopic laser photocoagulation procedures at 18-26 weeks of gestation. TTTS severity was determined using the Quintero classification. Blood vessels that crossed the interamniotic membrane were nonselectively photocoagulated. The χ2 test and Mann-Whitney U test were used for the statistical analysis. RESULTS: The mean (±standard deviation) age of pregnant women, gestational age at surgery, surgical time, gestational age at birth, and newborn weight were 32.2±4.1 years, 20.7±2.9 weeks, 51.8±16.7 minutes, 30.5±4.1 weeks, and 1,531.0±773.1 g, respectively. Using the Quintero classification, there was a higher percentage of cases in stage III (54.2%), followed by stages IV (20.8%), II (16.7%), and I (8.3%). Ten (41.7%) donor fetuses died and 14 (58.3%) donor fetuses survived until the end of gestation. Placental insertion location (anterior vs. posterior) did not affect the incidence of iatrogenic septostomy, surface bleeding, and premature rupture of membranes until the end of gestation. The death rate of donor and recipient fetuses before 24th gestational week increased with severity of TTTS. CONCLUSION: The maternal and perinatal outcomes resulting from the implementation of a new minimally invasive surgical technique are in line with those obtained in major centers worldwide, considering the learning curves and infrastructures.

Fetal modified left myocardial performance index monitoring during open surgery for myelomeningocele repair.

OBJECTIVE: Fetal hemodynamic changes have already been described during open myelomeningocele repair. This study aimed to access fetal myocardial performance index (MPI) during this high-complexity surgery. METHODS: Open myelomeningocele repair was performed in 37 fetuses between the 24th and 27th week of gestation. MPI was calculated at specific periods: pre-anesthesia, postanesthesia, neurosurgery (early skin manipulation, spinal cord releasing, and sintesis), and end of surgery. Mean ± standard deviation (SD) of MPI and its related times-isovolumetric contraction time (ICT), isovolumetric relaxation time (IRT), and ejection time (ET)-was determined for each period. Analysis of variance (ANOVA) with repeated measures was used to assess differences among these periods. Tukey multiple comparison times test compared global surgery stages. RESULTS: The mean of MPI in the specific time points was 0.32, 0.32, 0.34, 0.48, 0.36, and 0.32, respectively (P < .001). In the two-tailed comparison times, neurosurgery stage presents MPI highest levels, especially on stage 3b (early skin manipulation and spinal cord releasing) related to ICT and IRT rising and ET decreased levels. CONCLUSION: Fetal global cardiac function is altered during the open myelomeningocele repair. The neurosurgery stage represents the critical phase of the procedure.

Cerebellar herniation demonstrated by the occipitum-dens line: Ultrasonography assessment of normal fetuses, fetuses with myelomeningocele, and fetuses that underwent antenatal myelomeningocele surgery.

OBJECTIVE: To establish a method to quantify the position of the cerebellum by ultrasonography in normal fetuses, fetuses with myelomeningocele (MMC), and fetuses that underwent in utero MMC repair. METHODS: Reference points identifiable on ultrasound were established. The basilar portion of the occipital bone and upper portion of the odontoid process were considered as the external and internal limits, respectively, of the level zero of a line that was designated the occipitum-dens line (ODL). Eighty-three normal fetuses were assessed 1 occasion (cross-sectional study) in addition to 25 fetuses with MMC and 25 fetuses that underwent in utero MMC repair. The groups were compared using analysis of variance or the Kruskal-Wallis test. The intraclass correlation coefficient (ICC) was used to determine intra- and interobserver reproducibility. RESULTS: The analysis of ODL indicated that all normal fetuses had the cerebellar tonsil above level zero at a mean distance of 2.8 ± 1.1 mm (P = 0.125). The fetuses with MMC had the cerebellar tonsil below level zero, except in 2 cases of myelocystocele. All the fetuses that underwent in utero MMC repair exhibited regression of the cerebellar herniation (0.5 mm per week, P < 0.005). Herniation increased gradually (1.0 mm per week, P < 0.005) in fetuses that did not undergo MMC repair. ICC indicated good intra- and interobserver reproducibility (0.996 and 0.983, respectively). CONCLUSION: The reference points for ODL may be used to assess cerebellar height. ODL allowed the demonstration of the regression of cerebellar herniation in fetuses that underwent in utero MMC repair.

Antenatal management of fetal neurosurgical diseases.

The advance in the imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth. Myelomeningocele is the most recognized disease that can be treated during pregnancy with a high rate of success. Additionally, this field can be extended to other conditions such as hydrocephalus and encephaloceles. However, each one of these diseases has nuances in the diagnostic evaluation that should fit the requirements to perform the fetal procedure and overbalance the benefits to the patients. In this article, the authors aim to review the neurosurgical aspects of the antenatal management of neurosurgical conditions based on the experience of a pediatric neurosurgery center.

Fetal Heart Rate Monitoring during Intrauterine Open Surgery for Myelomeningocele Repair.

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.

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.

Ductus venosus versus cerebral transverse sinus Doppler velocimetry for predicting acidemia at birth in pregnancies complicated by placental insufficiency.

OBJECTIVES: The objectives of this study is to compare ductus venosus (DV) and cerebral transverse sinus (CTS) Doppler velocimetry for predicting acidemia at birth in pregnancies complicated by placental insufficiency. METHODS: A prospective cross-sectional study involving 69 cases. Doppler assessment of the DV and CTS was carried out in the last 24 hours prior to delivery. The sensitivity, specificity, positive and negative predictive values, and the accuracy and false-positive and false-negative rates were calculated for those parameters considered to be good predictors of acidemia. The McNemar test was used to compare the various parameters. RESULTS: The DV pulsatility index(PI), S/A, and (S - A)/S ratios as well as the CTS PI and the (S - A)/S ratio were good predictors of acidemia. The comparison between DV and CTS showed that for pulsatility index for veins, the sensitivity was 52.4% versus 66.7%, p = 0.508; the specificity was 81.2% versus 77.1%, p = 0.774; and the accuracy was 72.5% versus 73.9%, p = 1.00. For the (S - A)/S ratio the sensitivity was 52.4% versus 52.4%, p = 1.00; the specificity was 85.4% versus 79.2%, p = 0.508; and the accuracy was 75.4% versus 71%, p = 0.647. CONCLUSIONS: In pregnancies with placental insufficiency, the PI and the (S - A)/S ratio of both DV and CTS were equally effective in predicting acidemia at birth.