RESUMO
OBJECTIVE: Spina bifida (SB) is a congenital birth defect defined as a failure of the neural tube formation during the embryonic development phase. Fetoscopic repair of SB is a novel treatment technique that allows to close spinal defect early and prevent potential neurological and psychomotor complications. CASE REPORT: We present a case report of a 32-year-old-multigravida whose fetus was diagnosed with lumbosacral myelomeningocele at 23rd week. Fetoscopic closure of MMC was performed at 26 weeks. At 32 weeks, due to premature amniorrhexis and placental abruption, an emergency C-section was performed. Newborn's psychomotor development was within normal limits. CONCLUSION: Although intrauterine treatment has an increased risk of premature labor, placental abruption, prenatal closure is associated with improved postnatal psychomotor development. Prenatal surgery decreases the risk of Arnold-Chiari II malformation development and walking disability. Fetoscopic closure of SB is becoming a choice for treatment with beneficial outcomes for mother and fetus.
Assuntos
Fetoscopia/métodos , Região Lombossacral/cirurgia , Meningomielocele/cirurgia , Segundo Trimestre da Gravidez , Disrafismo Espinal/cirurgia , Descolamento Prematuro da Placenta/etiologia , Descolamento Prematuro da Placenta/cirurgia , Adulto , Cesárea , Feminino , Ruptura Prematura de Membranas Fetais/etiologia , Ruptura Prematura de Membranas Fetais/cirurgia , Humanos , Recém-Nascido , Região Lombossacral/embriologia , Meningomielocele/diagnóstico , Meningomielocele/embriologia , Gravidez , Disrafismo Espinal/diagnóstico , Disrafismo Espinal/embriologiaRESUMO
OBJECTIVE: To investigate cerebral anomalies other than Chiari type 2 malformation in fetuses with myelomeningocele (MMC). DESIGN: A retrospective cohort study in a single tertiary centre. SETTING: A review of associated cerebral anomalies in cases with prenatal diagnosis of myelomeningocele. POPULATION: Seventy cases of fetal myelomeningocele. METHODS: Ultrasound and MRI images were blindly reviewed. Postnatal imaging and results of the postmortem results were also reviewed. The association between cerebral anomalies and the following ultrasound findings was measured: level of the defect, ventriculomegaly, microcephaly and fetal talipes. MAIN OUTCOME MEASURES: A microcephaly was observed in 32/70 cases (46%) and a ventriculomegaly was observed in 39/70 cases (56%). Other cerebral anomalies were diagnosed in 47/70 (67%). RESULTS: Other cerebral anomalies were represented by 42/70 cases with abnormal CC (60%), 8/70 cases with perinodular heterotopia (PNH; 11%), 2/70 cases with abnormal gyration (3%). MRI performed only in fetal surgery cases confirmed the ulltrasound findings in all cases and provided additional findings in two cases (PNH). Risk ratios of fetal cerebral anomalies associated with MMC did not reach significance for microcephaly, ventriculomegaly, talipes or the level of the defect There was an overall good correlation between pre- and postnatal findings with a Kappa value of 0.79 [95% CI 0.57-1] and 82% agreement. CONCLUSION: Fetal brain anomalies other than Chiari type 2 malformation are frequently observed in fetuses with myelomeningocele, predominantly represented by CC anomalies. Whether these associated cerebral anomalies have an impact on selecting cases eligible for fetal surgery needs further evaluation. TWEETABLE ABSTRACT: Fetal cerebral anomalies other than Chiari type 2 malformation, microcephaly, and ventriculomegaly may be associated with MMC in up to 67% of the cases.
Assuntos
Encéfalo/anormalidades , Doenças Fetais/diagnóstico por imagem , Meningomielocele/complicações , Meningomielocele/diagnóstico por imagem , Ultrassonografia Pré-Natal , Adulto , Encéfalo/diagnóstico por imagem , Encéfalo/embriologia , Feminino , Doenças Fetais/etiologia , Humanos , Imageamento por Ressonância Magnética , Meningomielocele/embriologia , Gravidez , Resultado da Gravidez , Estudos RetrospectivosRESUMO
OBJECTIVE: This observational study reports on the postnatal mortality and 30-month outcome of children who underwent fully percutaneous fetoscopic repair of myelomeningocele (MMC) at a single center in Giessen, Germany. METHODS: Between October 2010 and August 2014, a total of 72 patients underwent fully percutaneous fetoscopic MMC closure at 21 + 0 to 29 + 1 (mean, 23 + 5) weeks' gestation. Of these, 52 (72%) participated in this study; however, 30-month mortality data are available for all 72 children. Children were examined at four timepoints: shortly after birth and at 3 months, 12 months and 30 months of corrected age. The patients underwent age-specific standardized neurological examinations and assessment of leg movements and ambulation at all timepoints. Cognitive and motor development were assessed using the Bayley Scales of Infant Development, second edition (BSID-II), at 30 months. RESULTS: All 72 children survived the intrauterine procedure, however, four (5.6%) infants died postnatally (including two of the 52 comprising the study cohort). Of the 52 patients included in the study, 11.5% were delivered before the 30th week of gestation (mean, 33 + 1 weeks) and, of the survivors, 48.1% had ventriculoperitoneal shunt placement. Of the 50 infants that were alive at 30 months, independent ambulation, without orthosis, was feasible for 46%. At 30 months of follow-up, 46% of children presented with a functional level that was at least two segments better than the anatomical level of the lesion. At 30 months, 70% of the children presented with BSID-II psychomotor development index score of ≥ 70 and 80% with BSID-II mental development index score of ≥ 70. CONCLUSION: Intrauterine repair of MMC by percutaneous fetoscopy shows largely similar outcomes to those reported for open repair, with respect to mortality, prematurity, shunt-placement rates, motor and mental development and free ambulation. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
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Doenças Fetais/cirurgia , Fetoscopia/mortalidade , Meningomielocele/cirurgia , Pré-Escolar , Fetoscopia/métodos , Seguimentos , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Meningomielocele/embriologia , Transtornos do Neurodesenvolvimento/prevenção & controle , Desempenho Físico Funcional , Derivação Ventriculoperitoneal/métodosRESUMO
BACKGROUND: Abnormal intracranial findings are often detected at mid-trimester ultrasound (US) in fetuses with myelomeningocele (MMC). It is unclear whether these findings constitute a spectrum of the disease or are an independent finding, which should contraindicate fetal surgery. OBJECTIVE: To ascertain the spectrum and frequency of US-detected cranial findings in fetuses with MMC. SEARCH STRATEGY: MEDLINE, Embase, Web of Science and CENTRAL were searched from January 2000 to June 2020. SELECTION CRITERIA: Study reporting incidence of cranial US findings in consecutive cases of second-trimester fetuses with MMC. DATA COLLECTION AND ANALYSIS: Publication quality was assessed by Newcastle-Ottawa Scale (NOS) and modified NOS. Meta-analysis could not be performed as a result of high clinical diversity and study heterogeneity. MAIN RESULTS: Fourteen cranial US findings were reported in 15 studies. Findings in classic Chiari II malformation (CIIM) spectrum included posterior fossa funnelling (96%), small transcerebellar diameter (82-96%), 'banana' sign (50-100%), beaked tectum (65%) and 'lemon' sign (53-100%). Additional cranial findings were small biparietal diameter (BPD) and head circumference (HC) (<5th centile; 53 and 71%, respectively), ventriculomegaly (45-89%), abnormal pointed shape of the occipital horn (77-78%), thinning of the posterior cerebrum, perinodular heterotopia (11%), abnormal gyration (3%), corpus callosum disorders (60%) and midline interhemispheric cyst (42%). CONCLUSIONS: We identified 14 cranial findings by second-trimester US in fetuses with MMC. The relatively high incidence of these findings and their unclear prognostic significance might not contraindicate fetal surgery in the case of normal fetal genetic testing. Some cranial findings may independently affect postnatal outcome, however. Long-term detailed follow-up is required to investigate this. TWEETABLE ABSTRACT: A high rate of cranial abnormalities found on second-trimester ultrasound in fetuses with myelomeningocele.
Assuntos
Doenças Fetais/diagnóstico por imagem , Meningomielocele/diagnóstico por imagem , Crânio/anormalidades , Crânio/diagnóstico por imagem , Ultrassonografia Pré-Natal , Feminino , Humanos , Meningomielocele/embriologia , Gravidez , Segundo Trimestre da Gravidez , Crânio/embriologiaRESUMO
Continuous fetal hemodynamic monitoring during in-utero surgery is desirable, but it is often not feasible without intermittent interruption. We report the use of a fetal spiral electrode for continuous heart rate monitoring during fetal myelomeningocele repair. Fetal echocardiography and a fetal spiral electrode were used to monitor fetal heart rate during in-utero repair at 25 weeks' gestation. We observed good agreement between echocardiographic and spiral electrode heart rate measurements. Using the Bland-Altman approach, the mean (SD) difference between measurements was 1.8 (3.5) beats per minute with limits of agreement of -5.3 to 8.8 beats per minute. This case illuminates a potential role for a fetal spiral electrode as a real-time adjunct in fetal interventions.
Assuntos
Ecocardiografia/métodos , Monitorização Fetal/instrumentação , Monitorização Fetal/métodos , Frequência Cardíaca Fetal/fisiologia , Meningomielocele/embriologia , Meningomielocele/cirurgia , Adulto , Eletrodos , Feminino , Humanos , Meningomielocele/diagnóstico por imagem , Gravidez , Ultrassonografia Pré-Natal/métodosRESUMO
Linked Comment: Ultrasound Obstet Gynecol 2020; 55:730-739.
Assuntos
Competência Clínica , Fetoscopia/educação , Meningomielocele/cirurgia , Feminino , Fetoscopia/normas , Humanos , Curva de Aprendizado , Meningomielocele/embriologia , GravidezRESUMO
OBJECTIVE: To assess our initial experience with prenatal restoration of hindbrain herniation following in utero repair of myelomeningocele (MMC). PATIENTS AND METHODS: Three consecutive patients with prenatally diagnosed MMC (between January 1, 2018 and September 30, 2018) were managed with open in utero surgery. As per institutional review board approval and following a protocol designed at the Mayo Clinic Maternal & Fetal Center, fetal intervention was offered between 19 0/7 and 25 6/7 weeks of gestation. Prenatal improvement of hindbrain herniation was the declared restorative end point. Obstetrical and perinatal outcomes were also assessed. RESULTS: Diagnosis of MMC was confirmed upon referral between 20 and 21 weeks' gestation by using fetal ultrasound and magnetic resonance imaging. In all cases reported here, the spinal defect was lumbosacral with evidence of hindbrain herniation. Open in utero MMC repair was performed between 24 and 25 weeks' gestation with no notable perioperative complications. Postprocedure fetal magnetic resonance imaging performed 6 weeks after in utero repair documented improvement of hindbrain herniation. Deliveries were at 37 weeks by cesarean section without complications. Most recent postnatal follow-ups were unremarkable at both 11 months (baby 1) and 3 months of age (baby 2), with mild ventriculomegaly. Antenatal and postnatal follow-up of baby 3 at 1 month of age was also unremarkable. CONCLUSION: Our study highlights the prenatal restoration of hindbrain herniation following in utero MMC repair in all cases presented here as an example of a prenatal regenerative therapy program in our institution.
Assuntos
Encefalocele/embriologia , Meningomielocele/embriologia , Medicina Regenerativa/métodos , Rombencéfalo/embriologia , Adulto , Encefalocele/cirurgia , Feminino , Feto/anormalidades , Feto/cirurgia , Humanos , Meningomielocele/cirurgia , Gravidez , Cuidado Pré-Natal/métodos , Medicina Regenerativa/classificação , Rombencéfalo/anormalidades , Rombencéfalo/cirurgiaRESUMO
BACKGROUND: Despite undisputable benefits, midtrimester prenatal surgery is not a cure for myelomeningocele (MMC): residual intracranial and motor deficits leading to lifelong handicap question the timing of prenatal surgery. Indeed, the timing and intensity of intrauterine spinal cord injury remains ill defined. OBJECTIVE: We aimed to describe the natural history of neuronal loss in MMC in utero based on postmortem pathology. STUDY DESIGN: Pathology findings were analyzed in 186 cases of myelomeningocele with lesion level between S1 and T1. Using a case-control, cross-sectional design, we investigated the timewise progression and topographic extension of neuronal loss between 13 and 39 weeks. Motor neurons were counted on histology at several spinal levels in 54 isolated MMC meeting quality criteria for cell counting. These were expressed as observed-to-expected ratios, after matching for gestational age and spinal level with 41 controls. RESULTS: Chiari II malformation increased from 30.7% to 91.6% after 16 weeks. The exposed spinal cord displayed early, severe, and progressive neuronal loss: the observed-to-expected count dropped from 17% to ≤2% after 16 weeks. Neuronal loss extended beyond the lesion to the upper levels: in cases <16 weeks, the observed-to-expected motor neuron count was 60% in the adjacent spinal cord, decreasing at a rate of 16% per week. Progressive loss was also found in the upper thoracic cord, but in much smaller proportions. The observed-over-expected ratio of motor neurons was not correlated with the level of myelomeningocele. CONCLUSIONS: Significant neuronal loss is present ≤16 weeks in the exposed cord and progressively extends cranially. Earlier prenatal repair (<16 weeks) could prevent Chiari II malformation in 69.3% of cases, rescue the 17% remaining motor neurons in the exposed cord, and prevent the extension to the upper spinal cord.
Assuntos
Malformação de Arnold-Chiari/patologia , Idade Gestacional , Meningomielocele/patologia , Neurônios Motores/patologia , Medula Espinal/patologia , Aborto Induzido , Malformação de Arnold-Chiari/embriologia , Autopsia , Progressão da Doença , Feminino , Terapias Fetais , Humanos , Vértebras Lombares , Meningomielocele/embriologia , Meningomielocele/cirurgia , Procedimentos Neurocirúrgicos , Gravidez , Primeiro Trimestre da Gravidez , Segundo Trimestre da Gravidez , Estudos Retrospectivos , Sacro , Vértebras TorácicasRESUMO
INTRODUCTION: The ovine model is the gold standard large animal model of myelomeningocele (MMC); however, it has a high rate of fetal loss. We reviewed our experience with the model to determine risk factors for fetal loss. METHODS: We performed a retrospective review from 2009 to 2018 to identify operative factors associated with fetal loss (early fetal demise, abortion, or stillbirth). Operative risk factors included gestational age at operation, operative time, reduction of multiple gestations, amount of replaced amniotic fluid, ambient temperature, and method of delivery. RESULTS: MMC defects were created in 232 lambs with an overall survival rate of 43%. Of the 128 fetuses that died, 53 (42%) had demise prior to repair, 61 (48%) aborted, and 14 (11%) were stillborn. Selective reduction of multiple gestations in the same uterine horn was associated with increased fetal demise (OR 3.03 [95% CI 1.29-7.05], p = 0.01). Later gestational age at MMC repair and Cesarean delivery were associated with decreased abortion/stillbirth (OR 0.90 [95% CI 0.83-0.90], p = 0.03, and OR 0.37 [95% CI 0.16-0.31], p = 0.02), respectively. CONCLUSION: Avoiding selective reduction, repairing MMC later in gestation, and performing Cesarean delivery decreases the rate of fetal loss in the ovine MMC model.
Assuntos
Modelos Animais de Doenças , Morte Fetal/etiologia , Meningomielocele/embriologia , Meningomielocele/cirurgia , Ovinos , Aborto Espontâneo/epidemiologia , Animais , Cesárea , Feminino , Morte Fetal/prevenção & controle , Idade Gestacional , Meningomielocele/mortalidade , Gravidez , Estudos Retrospectivos , Fatores de Risco , Natimorto/epidemiologiaRESUMO
PURPOSE OF REVIEW: To review the advance of maternal--fetal surgery, the research of stem cell transplantation and tissue engineering in prenatal management of fetal meningomyelocele (fMMC). RECENT FINDINGS: Advance in the imaging study provides more accurate assessment of fMMC in utero. Prenatal maternal--fetal surgery in fMMC demonstrates favourable postnatal outcome. Minimally invasive fetal surgery minimizes uterine wall disruption. Endoscopic fetal surgery is performed via laparotomy-assisted or entirely percutaneous approach. The postnatal outcome for open and endoscopic fetal surgery shares no difference. Single layer closure during repair of fMMC is preferred to reduce postnatal surgical intervention. All maternal--fetal surgeries impose anesthetic and obstetric risk to pregnant woman. Ruptured of membrane and preterm delivery are common complications. Trans-amniotic stem cell therapy (TRASCET) showed potential tissue regeneration in animal models. Fetal tissue engineering with growth factors and dura substitutes with biosynthetic materials promote spinal cord regeneration. This will overcome the challenge of closure in large fMMC. Planning of the maternal--fetal surgery should adhere to ethical framework to minimize morbidity to both fetus and mother. SUMMARY: Combination of endoscopic fetal surgery with TRASCET or tissue engineering will be a new vision to achieve to improve the outcome of prenatal intervention in fMMC.
Assuntos
Fetoscopia/métodos , Meningomielocele/cirurgia , Transplante de Células-Tronco/métodos , Feminino , Fetoscopia/efeitos adversos , Humanos , Meningomielocele/embriologia , Procedimentos Cirúrgicos Minimamente Invasivos/efeitos adversos , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Gravidez , Engenharia Tecidual/métodosRESUMO
OBJECTIVES: Prenatal myelomeningocele (MMC) repair has been shown to provide significant benefits to the infant, decreasing the postnatal need for ventriculoperitoneal shunt and improving motor outcome. Chorioamniotic membrane separation (CAS) is a potential complication following prenatal MMC repair and may increase the risk of preterm prelabor rupture of membranes (PPROM) and preterm birth. The objectives of this study were: (1) to evaluate the incidence of CAS after prenatal MMC repair; (2) to determine risk factors associated with its occurrence; and (3) to assess its association with adverse perinatal outcomes. METHODS: This was a retrospective cohort study of patients who underwent fetal MMC repair between November 2011 and December 2018. Surgery was performed using either a fetoscopic (laparotomy or exteriorized uterus) approach or an open-hysterotomy approach. Eligibility criteria were those reported in the Management of Myelomeningocele Study. If CAS was detected on ultrasound (US), its severity was graded as 'mild' if amnion detachment involved < 25% of the uterine cavity, 'moderate' if it involved 25-50% and 'severe' if it involved > 50%. Evolution of CAS was classified as stable, increasing or decreasing based on the difference in severity grading between the time at first diagnosis and the last US scan before delivery. Logistic regression analysis was performed to identify pre- or perisurgical factors associated with the development of CAS and to determine the risk of adverse perinatal outcome associated with CAS. RESULTS: In total, 91 cases were included. Fetoscopic or open-hysterotomy repair of MMC was performed in 52/91 (57.1%) and 39/91 (42.9%) cases, at a median gestational age (GA) of 25.0 weeks (range, 22.9-26.0 weeks) and 25.0 weeks (range, 21.3-25.9 weeks), respectively. CAS was diagnosed in 31/91 (34.1%) patients, at a median GA of 28.1 weeks (range, 24.4-37.6 weeks). Anterior placenta was identified as a risk factor for the postoperative development of CAS (odds ratio (OR), 3.72 (95% CI, 1.46-9.5); P < 0.01). This risk was dependent on the repair technique. An anterior placenta significantly increased the risk of CAS after fetoscopic repair (OR, 3.94 (95% CI, 1.14-13.6); P = 0.03) but not after open repair (OR, 2.8 (95% CI, 0.6-12.5); P = 0.16). There was no significant difference in the rate of CAS after fetoscopic repair (21/52 (40.4%)) vs open-hysterotomy repair (10/39 (25.6%)) (P = 0.14), nor were there any differences in GA at diagnosis of CAS, interval between surgery and diagnosis, distribution of CAS severity or progression of CAS between the two groups. CAS increased the risk of PPROM (50% in those with vs 12% in those without CAS) (OR, 7.6 (95% CI, 2.5-21.9); P < 0.01) and preterm delivery (70% vs 38%) (OR, 3.2 (95% CI, 1.3-8.1); P < 0.01). Fetoscopically repaired cases with CAS had a higher rate of PPROM (12/20 (60.0%) vs 2/31 (6.5%); P < 0.01) and preterm delivery (13/20 (65.0%) vs 5/31 (16.1%); P < 0.01) than those that did not develop CAS, while the differences were not significant in cases with open-hysterotomy repair. Early detection of CAS (before 30 weeks' gestation) was a risk factor for preterm delivery (90% before 30 weeks vs 36% at or after 30 weeks) (OR, 15.7 (95% CI, 2.3-106.3); P < 0.01). There was no association between PPROM or preterm delivery and the severity or progression of CAS. CONCLUSIONS: The presence of an anterior placenta was the only factor that increased the risk for CAS after fetoscopic MMC repair. Detection of CAS after fetoscopic MMC repair significantly increases the risk for PPROM and preterm delivery. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
Assuntos
Ruptura Prematura de Membranas Fetais/epidemiologia , Fetoscopia/efeitos adversos , Histerotomia/efeitos adversos , Meningomielocele/cirurgia , Resultado da Gravidez/epidemiologia , Adulto , Âmnio/patologia , Âmnio/cirurgia , Feminino , Ruptura Prematura de Membranas Fetais/etiologia , Fetoscopia/métodos , Idade Gestacional , Humanos , Histerotomia/métodos , Incidência , Recém-Nascido , Meningomielocele/embriologia , Meningomielocele/patologia , Placenta/patologia , Placenta/cirurgia , Período Pós-Operatório , Gravidez , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/etiologia , Período Pré-Operatório , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Resultado do Tratamento , Ultrassonografia Pré-NatalRESUMO
OBJECTIVE: To determine whether the presence of a myelomeningocele (MMC) sac and sac size correlate with compromised lower-extremity function in fetuses with open spinal dysraphism. METHODS: A radiology database search was performed to identify cases of MMC and myeloschisis (MS) diagnosed prenatally in a single center from 2013 to 2017. All cases were evaluated between 18 and 25 weeks. Ultrasound reports were reviewed for talipes and impaired lower-extremity motion. In MMC cases, sac volume was calculated from ultrasound measurements. Magnetic resonance imaging reports were reviewed for hindbrain herniation. The association of presence of a MMC sac and sac size with talipes and impaired lower-extremity motion was assessed. Post-hoc analysis of data from the multicenter Management of Myelomeningocele Study (MOMS) randomized controlled trial was performed to confirm the study findings. RESULTS: In total, 283 MMC and 121 MS cases were identified. MMC was associated with a lower incidence of hindbrain herniation than was MS (80.9% vs 100%; P < 0.001). Compared with MS cases, MMC cases with hindbrain herniation had a higher rate of talipes (28.4% vs 16.5%, P = 0.02) and of talipes or lower-extremity impairment (34.9% vs 19.0%, P = 0.002). Although there was a higher rate of impaired lower-extremity motion alone in MMC cases with hindbrain herniation than in MS cases, the difference was not statistically significant (6.6% vs 2.5%; P = 0.13). Among MMC cases with hindbrain herniation, mean sac volume was higher in those associated with talipes compared with those without talipes (4.7 ± 4.2 vs 3.0 ± 2.6 mL; P = 0.002). Review of the MOMS data demonstrated similar findings; cases with a sac on baseline imaging had a higher incidence of talipes than did those without a sac (28.2% vs 7.5%; P = 0.007). CONCLUSIONS: In fetuses with open spinal dysraphism, the presence of a MMC sac was associated with fetal talipes, and this effect was correlated with sac size. The presence of a larger sac in fetuses with open spinal dysraphism may result in additional injury through mechanical stretching of the nerves, suggesting another acquired mechanism of injury to the exposed spinal tissue. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
Assuntos
Deformidades Congênitas das Extremidades Inferiores/embriologia , Meningomielocele/embriologia , Lesões Pré-Natais/etiologia , Disrafismo Espinal/embriologia , Pé Torto/embriologia , Bases de Dados Factuais , Feminino , Idade Gestacional , Humanos , Deformidades Congênitas das Extremidades Inferiores/diagnóstico por imagem , Meningomielocele/complicações , Meningomielocele/diagnóstico por imagem , Gravidez , Lesões Pré-Natais/diagnóstico por imagem , Disrafismo Espinal/complicações , Disrafismo Espinal/diagnóstico por imagem , Pé Torto/congênito , Pé Torto/diagnóstico por imagem , Ultrassonografia Pré-NatalRESUMO
BACKGROUND: Spina bifida is the most common fetal anomaly of the central nervous system, which affects approximately 1:1000 live births in the United States. Myelomeningocele (MMC) is the most common presentation of spina bifida, representing half of these cases. Given the deformation to the spinal cord and the nerve roots, this defect may result in significant morbidity to infants and major life-long disabilities. In this study we aimed to identify maternal and fetal characteristics associated with expectant management or termination of pregnancy in the setting of antenatally diagnosed MMC. We hypothesized that the level of the defect would correlate with patient's decision to continue the pregnancy. METHODS: A retrospective cohort analysis was performed with patients who had presented to the Cleveland Clinic Fetal Care Center between 2005-2017. RESULTS: Our data showed 36% of patients with antenatal diagnosis of MMC elected for second trimester terminations versus 64% who chose to continue their pregnancy and deliver either by cesarean section or vaginal delivery. Based on ultrasound findings, there were no significant differences between these two groups. Maternal body mass index was significantly higher in those who continued pregnancies (pâ=â0.036). In addition, the fetal diagnostic methods chosen by patients were significantly different. Those who elected to terminate were more likely to pursue amniocentesis (pâ=â0.03) and less likely to opt for MRI characterization of the fetus (pâ=â0.007). CONCLUSION: We conclude, in the setting of fetal MMC diagnosed during pregnancy, patients often rely less on the associated ultrasonographic findings. Personal decisions likely influence the choice of other fetal diagnostic modalities. Other than BMI, we did not see an association between maternal factors and decisions regarding second trimester pregnancy termination.
Assuntos
Aconselhamento Genético/métodos , Meningomielocele/diagnóstico , Pais/psicologia , Espinha Bífida Cística/diagnóstico , Ultrassonografia Pré-Natal , Aborto Induzido/estatística & dados numéricos , Adulto , Cesárea/estatística & dados numéricos , Tomada de Decisão Compartilhada , Parto Obstétrico/estatística & dados numéricos , Feminino , Humanos , Recém-Nascido , Meningomielocele/embriologia , Meningomielocele/terapia , Pais/educação , Gravidez , Estudos Retrospectivos , Espinha Bífida Cística/embriologia , Espinha Bífida Cística/terapia , Estados UnidosRESUMO
OBJECTIVE: Fetal myelomeningocele (fMMC) surgery improves infant outcomes when compared with postnatal surgery. Surgical selection criteria and the option of pregnancy termination, however, limit the number of cases that are eligible for prenatal surgery. We aimed to quantify what proportion of cases could ultimately benefit from fetal therapy. METHODS: We retrospectively reviewed all cases of fMMC referred to a large tertiary care center over a 10-year period and assessed their eligibility for fetal surgery, pregnancy termination rates, and actual uptake of the surgery. RESULTS: Of 158 cases, 67 (42%) were ineligible for fetal surgery based on surgical exclusion criteria. Eleven fetuses (7%) had chromosomal anomalies, 10 of which (91%) had other anomalies on ultrasound. Thirty-four patients had a combination of maternal and fetal contraindications. Of the remaining 91 eligible cases (58%), 45 (49%) pregnancies were terminated, leaving only 46 (29% of initial 158 cases) as potential candidates for fetal repair. Actual uptake of fetal surgery was 15% (n = 14 of 91), but this increased after a national program was started. CONCLUSION: Only a minority of fMMC cases will ultimately undergo fetal surgery. These numbers support the centralization of care in expert centers.
Assuntos
Terapias Fetais/métodos , Meningomielocele/embriologia , Meningomielocele/cirurgia , Aborto Induzido/estatística & dados numéricos , Adulto , Canadá , Aberrações Cromossômicas/estatística & dados numéricos , Definição da Elegibilidade , Feminino , Terapias Fetais/estatística & dados numéricos , Idade Gestacional , Humanos , Meningomielocele/genética , Gravidez , Estudos Retrospectivos , Ultrassonografia Pré-NatalRESUMO
OBJECTIVES: To evaluate the first 100 cases of in utero myelomeningocoele (MMC) repair and urological outcomes in a prospective analysis aiming to define possible improvement in bladder function. PATIENTS AND METHODS: We used a protocol consisting of a detailed medical history, urinary tract ultrasonography, voiding cystourethrography, and urodynamic evaluation. Patients were categorised into four groups: normal, high risk (overactive bladder with a detrusor leak-point pressure >40 cm H2 O and high filling pressures also >40 cm H2 O), incontinent, and underactivity (underactive bladder with post-void residual urine), and patients were treated accordingly. RESULTS: We evaluated 100 patients, at a mean age of 5.8 months (median 4 months), classified as high risk in 52.6%, incontinent in 27.4%, with underactive bladder in 4.2%, and only 14.7% had a normal bladder profile. Clean intermittent catheterisation was initiated in 57.3% of the patients and anticholinergics in 52.6%. Antibiotic prophylaxis was initiated in 19.1% of the patients presenting with vesico-ureteric reflux. CONCLUSION: The high incidence of abnormal bladder patterns suggests little benefit of in utero MMC surgery concerning the urinary tract.
Assuntos
Terapias Fetais/métodos , Meningomielocele/fisiopatologia , Ultrassonografia Pré-Natal , Urodinâmica/fisiologia , Refluxo Vesicoureteral/fisiopatologia , Feminino , Humanos , Lactente , Recém-Nascido , Meningomielocele/diagnóstico por imagem , Meningomielocele/embriologia , Meningomielocele/cirurgia , Estudos Prospectivos , Refluxo Vesicoureteral/diagnóstico por imagem , Refluxo Vesicoureteral/embriologiaRESUMO
BACKGROUND: Prenatal surgery for myelomeningocele (MMC) has been demonstrated to have benefits over postnatal surgery. Nevertheless, prenatal surgery requires a significant emotional, physical, and financial commitment from the entire family. METHODS: Mixed methods study of parents' perceptions regarding provider communication, treatment choices, and the family impact of having a child with MMC. RESULTS: Parents of children with MMC (n= 109) completed questionnaires. Parents were well informed and reported gathering information about prenatal surgery from a wide range of sources. After a fetal diagnosis of MMC, most learned about their options from their obstetrician, although one-third were not told about the option of prenatal surgery. About one-fourth of these parents felt pressure to undergo one particular option. Half of parents said that having a child with MMC has had a positive impact on them and their family, while the other half indicated that having a child with MMC has had both positive and negative impacts. The most commonly noted positive impacts were changes in parental attitudes, as well as having new opportunities and relationships. The most frequently reported negative impacts concerned relational and financial strain. The vast majority of parents indicated that they would still undergo prenatal surgery if they could travel back in time with their present knowledge. CONCLUSIONS: A better understanding of the parental experiences and perspectives following prenatal surgery will play an important role in providing overall support for parents and family members.
Assuntos
Atitude Frente a Saúde , Meningomielocele/psicologia , Meningomielocele/cirurgia , Pais/psicologia , Adulto , Pré-Escolar , Aconselhamento , Feminino , Humanos , Masculino , Meningomielocele/embriologia , Gravidez , Inquéritos e Questionários , Resultado do TratamentoRESUMO
OBJECTIVE: Fetoscopy for closure of open neural tube defects (NTD) remains controversial, as the use of patches or single-layer closure is not considered to meet the standards of good neurosurgical reconstruction. In this study, we describe a fetoscopic two-layer (myofascial and skin) closure technique for the treatment of NTD in five patients and report the preliminary anatomical outcome at birth. METHODS: From February to September 2017, five pregnant women with a fetus with a NTD, including three cases of myelomeningocele and two cases of myelocele, were operated on using a fetoscopic two-layer closure technique. In this technique, with the uterus exteriorized and using three 10-Fr ports, the placode is dissected from the surrounding tissue and detethered, removing the cystic tissue. The skin is undermined by blunt dissection and the defect is sutured to the midline in two layers (myofascial and skin) using a running 4/0 resorbable barbed suture. RESULTS: Median gestational age at the procedure was 24 + 3 (range, 23 + 5 to 27 + 3) weeks. Surgery was successful in all cases, without any intraoperative complications. Median time in surgery was 180 (range, 140-180) min and median time for fetoscopy was 105 (range, 65-120) min. In terms of obstetric complications, three cases of premature rupture of membranes and one case of chorioamnionitis were recorded. Median gestational age at delivery was 34 + 1 (range, 25 + 4 to 37 + 2) weeks and two patients delivered vaginally. The closed defect was watertight with good quality tissue in all cases. CONCLUSION: Fetoscopic two-layer closure of NTD may improve the quality of the tissue covering the defect, diminishing the need for postnatal surgical revision, and preserving the well-documented beneficial effects of prenatal closure on the neural tissue and hindbrain herniation. However, this technique may not be appropriate for those cases with wide diastasis of the myofascial layer or with a low quantity of available tissue. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.
Assuntos
Fetoscopia , Procedimentos Neurocirúrgicos , Adulto , Estudos de Viabilidade , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Meningomielocele/diagnóstico por imagem , Meningomielocele/embriologia , Meningomielocele/fisiopatologia , Meningomielocele/cirurgia , Procedimentos Neurocirúrgicos/métodos , Avaliação de Processos e Resultados em Cuidados de Saúde , Gravidez , Resultado da GravidezRESUMO
OBJECTIVE: To present the feasibility of fetal myelomeningocele (MMC) repair through a mini-hysterotomy and to describe the perinatal results from our initial experience. METHODS: A descriptive study of cases of fetal MMC correction via mini-hysterotomy performed between 2014 and 2016. RESULTS: Forty-five women underwent fetal surgery and 87% (39/45) delivered. A complete multilayer correction of the MMC was possible in all cases. There were no maternal, fetal or neonatal deaths. No maternal or fetal complications occurred from fetal MMC correction until maternal hospital discharge. The average gestational age (GA) at surgery was 24.5 weeks (standard deviation, SD: 1.7; range: 20.7-26.9). The median hysterotomy length was 3.05 cm (SD: 0.39; range: 2.50-3.50). One patient (1/39; 2.6%) experienced chorioamniotic separation. Nine patients (9/39; 23.1%) had premature preterm rupture of membranes at a median GA of 34.1 weeks (range: 31.1-36.0). The average GA at delivery was 35.3 weeks (SD: 2.2; range: 27.9-39.1). Ninety-five percent (37/39) of our patients had an intact hysterotomy site at delivery. Ventriculoperitoneal shunt placement was necessary for 7.7% (3/39) of the neonates. CONCLUSION: Fetal MMC repair is feasible through a mini-hysterotomy. This approach appears to be associated with reduced risks of very preterm delivery and maternal, fetal and neonatal complications.
Assuntos
Histerotomia/efeitos adversos , Meningomielocele/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Adulto , Malformação de Arnold-Chiari/etiologia , Malformação de Arnold-Chiari/prevenção & controle , Brasil/epidemiologia , Estudos de Viabilidade , Feminino , Ruptura Prematura de Membranas Fetais/epidemiologia , Ruptura Prematura de Membranas Fetais/etiologia , Ruptura Prematura de Membranas Fetais/prevenção & controle , Seguimentos , Idade Gestacional , Humanos , Hidrocefalia/etiologia , Hidrocefalia/prevenção & controle , Hidrocefalia/cirurgia , Incidência , Recém-Nascido , Masculino , Meningomielocele/embriologia , Meningomielocele/fisiopatologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Gravidez , Segundo Trimestre da Gravidez , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/etiologia , Nascimento Prematuro/prevenção & controle , Risco , Derivação Ventriculoperitoneal/efeitos adversosRESUMO
OBJECTIVES: To report our preliminary experience in the use of prenatal ultrasound examination to assess lower-limb movements in fetuses with myelomeningocele. We aimed to determine the accuracy of this method to establish the segmental level of neurological lesion, as this is the best known predictor of the future ability to walk. METHODS: This was a preliminary, observational study including fetuses with myelomeningocele operated on prenatally. The patterns of movements present and absent in the affected fetuses' lower limbs were evaluated systematically by ultrasound examination. According to the known nerve function associated with each muscle group, the segmental level of neurological lesion was established before birth. The agreement for the segmental levels assigned, between the prenatal ultrasound technique and the classical neurological clinical examination after birth (gold standard), was tested using the weighed kappa (wκ) index. RESULTS: Seventy-one fetuses with myelomeningocele were evaluated at the Hospital Universitari Vall d'Hebron. After counseling, the parents opted for prenatal surgery (26 cases), termination of pregnancy (43 cases) or postnatal repair (two cases). Five patients did not fulfil the inclusion criteria for prenatal surgery and three were excluded after birth. In the 18 fetuses that underwent surgery and were analyzed, the agreement between prenatal and postnatal segmental levels assigned was 91.7% for the right limb (wκ = 0.80) and 88.9% for the left limb (wκ = 0.73). CONCLUSIONS: The agreement found between prenatal and postnatal assignment of level of lesion in this preliminary study suggests that neurological sonographic evaluation is feasible before birth. This may provide accurate individualized information about the motor function and future ambulation prognosis of fetuses with myelomeningocele.