Perinatal Outcomes after Fetal Endoscopic Tracheal Occlusion for Isolated Congenital Diaphragmatic Hernia: Rapid Review / Resultados perinatais após oclusão traqueal endoscópica fetal por hérnia diafragmática congênita isolada: Revisão rápida

Abstract Objective To compare the perinatal outcomes of fetuses with isolated congenital diaphragmatic hernia after fetal endoscopic tracheal occlusion (FETO) and antenatal expectant management. Data sources In this rapid review, searches were conducted in the MEDLINE, PMC, EMBASE and CENTRAL databases between August 10th and September 4th, 2020. Randomized controlled trials (RCTs), quasi-RCTs or cluster-RCTs published in English in the past ten years were included. Study selection We retrieved 203 publications; 180 studies were screened by abstract. Full-text selection was performed for eight studies, and 1 single center RCTmet the inclusion criteria (41 randomized women; 20 in the FETO group, and 21 in the control group). Data collection Data collection was performed independently, by both authors, in two steps (title and abstract and full-text reading). Data synthesis There were no cases of maternal mortality. The mean gestational age at delivery was of 35.6±2.4 weeks in the intervention group, and of 37.4±1.9 weeks among the controls (p<0.01). Survival until 6 months of age was reported in 50% of the intervention group, and in 5.8% of the controls (p<0.01; relative risk: 10.5; 95% confidence interval [95%CI]: 1.5-74.7). Severe postnatal pulmonary hypertension was found in 50% of the infants in the intervention group, and in 85.7% of controls (p=0.02; relative risk: 0.6; 95%CI: 0.4-0.9). An analysis of the study indicated some concerns of risk of bias. The quality of evidence was considered moderate to low. Conclusion Current evidence is limited but suggests that FETO may be an effective intervention to improve perinatal outcomes.

Resumo Objetivo Comparar os resultados perinatais de fetos com hérnia diafragmática congênita após oclusão traqueal endoscópica fetal (OTEF) e conduta expectante pré-natal. Fontes dos dados Nesta revisão rápida, pesquisas foram conduzidas nas bases de dados MEDLINE, PMC, EMBASE e CENTRAL entre 10 de agosto de 2020 e 4 de setembro de 2020. Ensaios clínicos randomizados (ECRs), quase-ECRs e ECRs em cluster publicados em inglês nos últimos dez anos foram incluídos. Seleção dos estudos Foram recuperadas 203 publicações; 180 destas foram triadas pelo resumo. Fez-se a leitura do texto completo de 8 estudos, e 1 ECR cumpriu os critérios de inclusão (41 mulheres aleatorizadas; 20 no grupo OTEF e 21 no grupo de controle). Coleta de dados A coleta de dados realizada independentemente pelos dois autores, em duas etapas (título e resumo, e leitura do texto completo). Síntese dos dados Não houve casos de mortematerna. A idade gestacionalmédia no parto foi de 35,6±2,4 semanas no grupo de intervenção, e de 37,4±1,9 semanas entre os controles (p<0,01). A sobrevida até 6 meses de idade foi relatada em 50% do grupo de intervenção, e em 5,8% dos controles (p<0,01; risco relativo: 10,5; intervalo de confiança de 95% [IC95%]: 1,5-74,7). Hipertensão pulmonar grave ocorreu em 50% dos lactentes do grupo de intervenção, e em 85,7% dos controles (p = 0.02; risco relativo: 0,6; IC95%: 0,4-0,9). Uma análise do estudo indicou algumas preocupações quanto ao risco de viés. A qualidade da evidência foi considerada de moderada a baixa. Conclusão As evidências atuais são limitadas,mas sugeremque a OTEF pode ser uma intervenção eficaz para melhorar resultados perinatais.

Seguridad de la anestesia neuroaxial en mujeres con embarazo gemelar y síndrome de transfusión feto-fetal, sometidas a fotocoagulación láser. Estudio de cohorte retrospectiva / Safety of neuraxial anesthesia in patients twin pregnancy and twin-to-twin transfusion syndrome taken to laser photocoagulation. Retrospective cohort study

Objetivo: describir las características clínicas y la frecuencia de complicaciones maternas, fetales y neonatales, según técnica de anestesia neuroaxial (AN) en mujeres con síndrome de transfusión feto-fetal (STFF) tratadas con fotocoagulación láser (FL). Materiales y métodos: estudio de cohorte retrospectiva descriptivo. Se incluyeron gestantes con STFF tratadas con FL y AN en la Fundación Valle del Lili, Cali (Colombia) entre 2013-2017. Se excluyeron pacientes con STFF estadio-V de Quintero. Se usó estadística descriptiva. El protocolo fue aprobado por el Comité de Ética de la institución. Resultados: 32 participantes cumplieron con los criterios de inclusión y de exclusión. La población estuvo constituida por mujeres jóvenes, multíparas. En el 87,5% de los casos se realizó intervención de urgencia. El 43,7% presentaba el estadio-III de Quintero y en el 56,2 % de las gestantes se utilizó anestesia epidural. Las variables hemodinámicas maternas exhibieron un comportamiento similar, acorde al momento de la cirugía y la técnica neuoraxial implementada. El 65,6 % de las gestantes presentó hipotensión sostenida y el 9,3 % desarrolló edema pulmonar. El 65,6 % de las pacientes experimentó parto pretérmino y el 18,7 % ruptura prematura de membranas. Se registraron 14 muertes fetales y cinco neonatales. No se registraron casos de mortalidad materna. Conclusiones: en pacientes con STFF que requieren FL, el uso de la anestesia epidural, espinal o combinada probablemente se asocia con un comportamiento similar al de las variables hemodinámicas maternas, durante los momentos de la cirugía. Los profesionales que brindan atención a estas gestantes deben estar alerta ante la frecuente aparición de complicaciones maternas, fetales y neonatales. Se requieren estudios prospectivos que evalúen la seguridad y la efectividad de las diferentes técnicas de anestesia neuroaxial en pacientes con STFF.

Objective: To describe the clinical characteristics and the frequency of maternal, fetal and neonatal complications in accordance with the neuraxial anesthesia (NA) technique in women with twin-to- twin transfusion syndrome (TTTS) treated with laser photocoagulation. Materials and Methods: Descriptive retrospective cohort study of pregnant patients with TTTS treated with laser photocoagulation under NA at Fundación Valle del Lili, Cali (Colombia), between 2013-2017. Patients with Quintero stage VTTTS were excluded. The protocol was approved by the institutional ethics committee. Results: Of the participants, 32 met the inclusion and exclusion criteria. The study population consisted of young, multiparous women. Urgent interventions were performed in 87.5% of cases; 43.7% were Quintero stage III and epidural anesthesia was used in 56.2% of the women. Maternal hemodynamic variables were similar, in accordance with the timing of surgery and the neuraxial technique used. Sustained hypotension occurred in 65.6% of the pregnant women and 9.3% developed pulmonary edema. Pre-term delivery occurred in 65.6% of the patients and 18.7% had premature rupture of membranes. There were 14 fetal demises and five neonatal deaths. There were no cases of maternal mortality. Conclusions: In patients with TTTS requiring laser photocoagulation, the use of epidural, spinal or combined anesthesia is likely associated with similar maternal hemodynamic variables at the time of surgery. Practitioners providing care to these pregnant women must be aware of the frequent occurrence of maternal, fetal and neonatal complications. Prospective studies to assess the safety and effectiveness of the different neuraxial anesthesia techniques in patients with TTTS are required.

Actualización del diagnóstico prenatal y cirugía fetal del mielomeningocele / Update on prenatal diagnosis and fetal surgery for myelomeningocele

A partir del estudio seminal Management of Myelomeningocele Study en el año 2011, el cual demostró que la reparación prenatal del defecto del mielomeningocele antes de la semana 26 mejoraba los resultados neurológicos, la cirugía fetal fue incorporada dentro de las opciones de estándar de cuidado. Así, el diagnóstico prenatal del mielomeningocele dentro de la ventana terapéutica se convirtió en un objetivo obligatorio y, por ello, se intensificó la investigación de estrategias de tamizaje, sobre todo, en el primer trimestre. Además, se desarrollaron distintas técnicas de cirugía fetal para mejorar los resultados neurológicos y disminuir los riesgos maternos. El objetivo de la siguiente revisión es actualizar los avances en tamizaje y diagnóstico prenatal en el primer y segundo trimestre, y en cirugía fetal abierta y fetoscópica del mielomeningocel

A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved neurological outcomes; based on this study, fetal surgery was introduced as a standard of care alternative. Thus, prenatal myelomeningocele diagnosis within the therapeutic window became a mandatory goal; therefore, research efforts on screening strategies were intensified, especially in the first trimester. In addition, different fetal surgery techniques were developed to improve neurological outcomes and reduce maternal risks. The objective of this review is to provide an update on the advances in prenatal screening and diagnosis during the first and second trimesters, and in open and fetoscopic fetal surgery for myelomeningocele

Twin-twin transfusion syndrome - a University Hospital experience with intrauterine treatment / Síndrome da transfusão feto-fetal - experiência de um hospital universitário com tratamento intrauterino

ABSTRACT Introduction: twin-to-twin transfusion syndrome (TTTS), defined by combination of polyhydramnios-oligohydramnios, is the most prevalent (5%-35%) of the abnormalities due to placental vascular anastomoses and the most lethal (80%-100% mortality) if untreated. Fetoscopic laser ablation of abnormal vasculature using the Solomon technique is the gold standard approach. It consists of interrupting the intertwin blood flow. Objectives: to present our initial experience at the Fetal Surgery Service of the Hospital de Clinicas of the Federal University of Parana (HC-UFPR) and to compare our results with those reported in the literature. Methods: we conducted a retrospective analysis of pregnancies who had undergone laser ablation, assessing data on Quintero's staging, gestational age at diagnosis and at the time of the procedure, placental position, immediate post-procedure survival, and survival after the neonatal period. We then compared these data with the most recent data available in the literature. Results: we analyzed ten TTTS cases. The diagnosis was performed before the 26th week of pregnancy (median 20.8 weeks) and treatment occurred in a median of 9.5 days later. The distribution by the Quintero's staging was of three cases in stage II, five in stage III, and two in stage IV. In 50% of the gestations, at least one of the fetuses survived through the neonatal period. Conclusion: the treatment of TTTS in the HC-UFPR had a positive impact in the survival of the affected fetuses, although the results were worse than the ones reported in the literature, probably due to the delay in referencing the patients to our service, leading to a prolonged interval between diagnosis and treatment.

RESUMO Introdução: a síndrome de transfusão feto-fetal (STFF), definida pela combinação polidrâmnio-oligohidrâmnio, é a mais prevalente (5 a 35%) das anormalidades associadas às anastomoses vasculares placentárias e tem a maior letalidade (80 a 100%) se não tratada. A ablação a laser destes vasos por via fetoscópica com a técnica de Solomon é o tratamento de escolha atual para a interrupção das anastomoses vasculares. Objetivo: apresentar a experiência inicial do Serviço de Cirurgia Fetal do Hospital de Clínicas da Universidade Federal do Paraná (HC-UFPR) e comparar nossos resultados com os da literatura. Métodos: foram revisados os prontuários de todas as pacientes submetidas ao procedimento de ablação a laser, sendo analisados os dados referentes ao estadiamento de Quintero, à idade gestacional ao diagnóstico e ao procedimento, à posição placentária, à sobrevida imediata pós-procedimento e sobrevida após o período neonatal. Os dados foram então comparados com os mais recentes disponíveis na literatura. Resultados: dez casos de STFF foram analisados. Todos diagnosticados antes da 26a semana (mediana 20,79) e o intervalo diagnóstico-tratamento teve mediana de 9,5 dias. A distribuição pelo estadiamento de Quintero foi: três casos no estádio II, cinco casos no III e dois casos no IV. Em 50% das gestações pelo menos um dos fetos sobreviveu ao período neonatal. Conclusão: o tratamento da STFF no HC-UFPR impactou positivamente a sobrevida dos fetos acometidos. Entretanto, nossos resultados estão aquém dos reportados na literatura, possivelmente pelo demora no referenciamento das pacientes, com intervalo aumentado entre diagnóstico e tratamento.

Cirurgias intrauterinas: alternativa de intervenção para as cardiopatias congênitas / Intrauterine surgeries: intervetion alternative for congenital cardiopathies

As cardiopatias congênitas são anormalidades estruturais ou funcionais do sistema cardiovascular, advindas desde o nascimento, mesmo que diagnosticadas posteriormente. Entre as malformações congênitas, as cardiopatias são as principais causas de mortalidade infantil nos Estados Unidos e em outros países desenvolvidos. Com o intuito de reduzir os efeitos progressivos dessas patologias, a intervenção intrauterina tem se destacado como opção terapêutica diante de resultados iniciais satisfatórios. O presente artigo teve por objetivo demonstrar a evolução da cirurgia intrauterina para correção cardíaca, a partir da avaliação dos benefícios ao feto e visando a riscos mínimos e aceitáveis para a mãe. Foi realizada uma pesquisa na base de dados Publisher Medline (PubMed), Scientific Electronic Library Online (SciELO) e Biblioteca Virtual da Saúde (BVS), incluindo artigos publicados entre 2008 e 2018; além da pesquisa efetuada no Manual de Medicina Fetal da SOGIMIG ­ 2018. Os critérios de elegibilidade são amplos. São necessários equipe multidisciplinar, equipamentos sofisticados e aperfeiçoamento da técnica, dessa forma dificulta-se a realização das cirurgias. Entretanto, diante dos resultados já demonstrados, a cirurgia intrauterina apresenta-se como alternativa terapêutica promissora.(AU)

The congenital cardiopathies are structural or functional abnormalities of the cardiovascular system, originated from birth, even when previously diagnosed. Among the congenic malformations, the cardiopathies are the main causes of infant mortality in the United States and in other developed countries. In order to reduce the progressive effects of these pathologies, intrauterine intervention has been highlighted as a therapeutic option in contempt of satisfactory initial results. This article has as goal to demonstrate the evolution of the intrauterine surgery for cardiac correction, based on the evaluation of the benefits to the fetus and aiming at minimum and acceptable risks to the mother. A research was made based on the Publisher Medline (PubMed) data base, Scientific Electronic Library Online (SciELO) and Biblioteca Virtual da Saúde (BVS), including published articles between 2008 and 2018. Beyond the research made on The fetal medicine manual from SOGIMIG ­ 2018. The eligibility criteria are broad. It is necessary a multidisciplinary team, sophisticated equipments and technique improvement, therefore interfering in the performance of surgeries. However, in contempt of the already demonstrated results the intrauterine surgery presents itself as a therapeutic promising alternative.(AU)

Hernia diafragmática congénita / Congenital diaphragmatic hernia

The congenital diaphragmatic hernia (CDH) is a congenital defect of the formation and /or closure of the diaphragm that permits the herniation of abdominal contents into the thorax. It occurs when the diaphragmatic muscle fails to close during the prenatal development, and the contain of the abdomen migrate into the chest through this hole. When the abdominal organs are in the chest, there is limited room for the lungs to grow. This prevents the lungs for developing normally, resulting in pulmonary hypoplasia (or underdeveloped lungs). This can cause reduced blood flow to the lungs and pulmonary hypertension, as well as gastrointestinal reflux, feeding disorders and developmental delays. CDH can occur on the left side, right side or, very rarely, on both sides, and it can be life-threatening. The etiology is usually unknown. The incidence of CDH may be as high as 1 in 2000 to 1 in 5000 newborns alive. The sex relationship male/female is 1:1.8. Because of associated persistent pulmonary hypertension of the newborn and pulmonary hypoplasia, medical therapy in patients with CDH is directed toward optimizing oxygenation while avoiding definitive therapy. No time for repair of CDH is ideal, but it is suggested that the opportunity is 24-48 hours after birth to achieve pulmonary development. The key to survival lies in prompt diagnosis and treatment. Over the past two decades antenatal diagnosis rates have increased the knowledge of the pathophysiology of CDH and has become better understand with advances in clinical care including prenatal surgery, with a reported mortality of almost 35 % in live-born patients and a higher mortality when in utero deaths are conected. All these considerations are described in the article, with special reference to pre and post-natal treatment, complications management, diagnosis and prognosis

Síndrome de Banda Amniótica / Amniotic Band Syndrome

Amniotic band sequence or syndrome, is the term applied to a wide range of congenital anomalies, as a group of congenital birth defects caused by entrapment of fetal parts (usually a limb or digits) in fibrous amniotic bands while in utero. Before the baby was born, the body parts shows signs of arm, fingers, etc, that were caught and estrangled. Amniotic band syndrome can cause a number of different birth defects depending on which body part(s) is affected. Amniotic band sequence (ABS) is a rare condition caused by strands of the amniotic sac that separate and entrangle digits, limbs or other parts of the fetus. This constriction can cause a variety of problems depending on where the strands are located and how trightly they are wrapped. ABS can cause a broad spectrum of anomalies ranging from simple band constrictions to major craniofacialand visceral defects. This causes deformations, malformation and disruption, that results in incapacity or death. The aims of the present report, were to present a review of the literature concerning with this pathology, describing the clinical characteristics, etiology, diagnosis and prognosis, in order to improve the efficacy of the prenatal management

Secuencia de bandas amnióticas, una actualización / An update on amniotic bands sequence

La secuencia de bandas amnióticas es un desorden congénito caracterizado por anomalías craneofaciales, de la pared corporal y de las extremidades que pueden asociarse con bandas fibrosas fetoplacentarias. Su prevalencia ha sido reportada entre 0,19 y 8,1 por 10 000 nacimientos. Diversas teorías han tratado de explicar su etiología, sin embargo, ninguna, en forma individual, sustenta todas y cada una de las anomalías observadas, por lo que se ha considerado una entidad multifactorial. La identificación de anomalías (pre-yposnatalmente) sugestivas de secuencia de bandas amnióticas permite el abordaje diagnóstico para efectuar intervenciones terapéuticas oportunas que posibiliten la liberación de bandas amnióticas mediante fetoscopia con recuperación de la perfusión de la porción distal de la extremidad involucrada o bien la reparación quirúrgica posnatal y para otorgar asesoramiento genético. Este artículo ofrece una actualización sobre aspectos epidemiológicos, teorías etiológicas, factores de riesgo, características clínicas, diagnóstico (que incluye el diagnóstico prenatal), asesoramiento genético, abordaje terapéutico y pronóstico de esta entidad.

Amniotic bands sequence is a congenital disorder characterized by craniofacial, body wall, and limb anomalies that may be associated with fetal-placental fibrous bands. Its prevalence has been reported to range from 0.19 to 8.1 per 10 000births. Different theories have attempted to explain the etiology of amniotic band sequence; however, none has individually been able to support each and every defect observed, so it has been considered to be a multifactorial condition. The (pre- and post-natal) identification of anomalies suggestive of amniotic band sequence is useful for the diagnostic approach and implementation of timely therapeutic interventions favoring the release of the amniotic bands using fetoscopy with recovery of the involved distal limb perfusion, or else the possibility of performing a post-natal surgical repair. It is also helpful to provide genetic counseling. This article offers an update on the epidemiological aspects, etiological theories, risk factors, clinical characteristics, diagnosis (including antenatal diagnosis), genetic counseling, therapeutic approach, and prognosis of amniotic bands sequence.

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.

Fetoscopic tracheal occlusion for severe congenital diaphragmatic hernia: retrospective study / Oclusão traqueal por fetoscopia em hérnia diafragmática congênita grave: estudo retrospectivo

Abstract Background and objectives: The temporary fetal tracheal occlusion performed by fetoscopy accelerates lung development and reduces neonatal mortality. The aim of this paper is to present an anesthetic experience in pregnant women, whose fetuses have diaphragmatic hernia, undergoing fetoscopic tracheal occlusion (FETO). Method: Retrospective, descriptive study, approved by the Institutional Ethics Committee. Data were obtained from medical and anesthetic records. Results: FETO was performed in 28 pregnant women. Demographic characteristics: age 29.8 ± 6.5; weight 68.64 ± 12.26; ASA I and II. Obstetric: IG 26.1 ± 1.10 weeks (in FETO); 32.86 ± 1.58 (reversal of occlusion); 34.96 ± 2.78 (delivery). Delivery: cesarean section, vaginal delivery. Fetal data: Weight (g) in the occlusion and delivery times, respectively (1045.82 ± 222.2 and 2294 ± 553); RPC in FETO and reversal of occlusion: 0.7 ± 0.15 and 1.32 ± 0.34, respectively. Preoperative maternal anesthesia included ranitidine and metoclopramide, nifedipine (VO) and indomethacin (rectal). Preanesthetic medication with midazolam IV. Anesthetic techniques: combination of 0.5% hyperbaric bupivacaine (5-10 mg) and sufentanil; continuous epidural predominantly with 0.5% bupivacaine associated with sufentanil, fentanyl, or morphine; general. In 8 cases, there was need to complement via catheter, with 5 submitted to PC and 3 to BC. Thirteen patients required intraoperative sedation; ephedrine was used in 15 patients. Fetal anesthesia: fentanyl 10-20 mg.kg-1 and pancuronium 0.1-0.2 mg.kg-1 (IM). Neonatal survival rate was 60.7%. Conclusion: FETO is a minimally invasive technique for severe congenital diaphragmatic hernia repair. Combined blockade associated with sedation and fetal anesthesia proved safe and effective for tracheal occlusion.

Resumo Justificativa e objetivos: A oclusão traqueal fetal temporária feita por meio da fetoscopia acelera o desenvolvimento pulmonar e reduz a mortalidade neonatal. O objetivo deste trabalho é apresentar experiência anestésica em gestantes cujos fetos eram portadores de hérnia diafragmática e foram submetidos à oclusão traqueal por fetoscopia (FETO). Método: Estudo retrospectivo, descritivo, aprovado pelo Comitê de Ética da Instituição. Os dados foram obtidos das fichas anestésicas e dos prontuários. Resultados: A FETO foi feita em 28 gestantes. Características demográficos: idade 29,8 ± 6,5; peso 68,64 ± 12,26; ASA I e II. Obstétricas: IG 26,1 ± 1,10 semana (na FETO); 32,86 ± 1,58 (desoclusão); 34,96 ± 2,78 (parto). Via de parto: cesárea, parto vaginal. Dados fetais: peso (g) nos momentos da oclusão e nascimento, respectivamente (1.045,82 ± 222,2 e 2294 ± 553); RPC na FETO e desoclusão: 0,7 ± 0,15 e 1,32 ± 0,34, respectivamente. Anestesia materna: pré-operatório incluiu ranitidina e metoclopramida; nifedipina (VO) e indometacina (retal). Medicação pré-anestésica com midazolam EV. Técnicas anestésicas: bloqueio combinado com bupivacaína 0,5% hiperbárica 5-10 mg associada ao sufentanil; peridural contínua predominantemente com bupivacaína 0,5% associada a sufentanil, fentanil ou morfina; geral. Em oito casos houve necessidade de complementação pelo cateter, cinco nas submetidas a PC e três a BC. No intraoperatório 13 pacientes necessitaram de sedação; efedrina foi usada em 15 pacientes. Anestesia fetal: fentanil 10 a 20 mg.kg-1 e pancurônio 0,1-0,2 mg.kg-1 (IM). A taxa de sobrevida neonatal foi de 60,7%. Conclusão: A FETO constitui técnica minimamente invasiva para correção de hérnia diafragmática congênita grave. O bloqueio combinado associado à sedação e anestesia fetal se mostrou seguro e eficaz para a oclusão traqueal.

Manejo del síndrome de transfusión feto fetal en gestaciones gemelares monocoriales / Management of twin-twin transfusion syndrome in monochorionic twin gestations

El síndrome de transfusión feto fetal es una complicación grave de las gestaciones gemelares monocoriales. Su etiología se debe a la transfusión crónica de sangre del feto donante, al feto receptor; a través de las anastomosis vasculares entre los territorios placentarios de ambos. El cuadro clínico característico se evidencia en ambos fetos: receptor y donante, lo cual es consecuencia directa de las alteraciones en la volemia que padece cada uno. En el feto receptor, se observa un polihidramnios relacionado con la poliuria derivada de un estado de hipervolemia constante, que evoluciona hacia una insuficiencia cardiaca congestiva. En el feto donante, el cuadro es inverso; caracterizado por oligoamnios, oliguria, crecimiento intrauterino retardado e hipovolemia, con tasas de mortalidad sin tratamiento de entre 80-100 por ciento de los casos, que varían en función del grado de transfusión. El diagnóstico se basa en el estudio ecográfico exhaustivo de ambos fetos, procurando un diagnóstico precoz y un estadiaje correcto, ya que el pronóstico se verá muy influenciado por la actuación temprana. El tratamiento de elección es la coagulación láser de las anastomosis vasculares por fetoscopia, idealmente entre la semana 18 y 26 de embarazo. El pronóstico es variable, en función de la disponibilidad de terapia fetal y de la edad gestacional en el momento del diagnóstico; al ser, en general, de muy mal pronóstico sin tratamiento efectivo(AU)

Twin-twin transfusion syndrome is one of the most serious complications of monochorionic multiple gestations. Its etiology is due to a chronic blood transfusion from de donor twin to the recipient twin through the vascular anastomoses between the placental territories of both fetuses. The characteristic clinical presentation appears in both donor and recipient fetuses, as a direct consequence of altered volemia in each one. Polyhydramnios related to polyuria resulting from a state of constant hypervolemia is observed in the recipient twin, finally evolving into congestive heart failure. The clinical presentation in the donor twin is reversed and characterized by oligoamnios, oliguria, retarded intrauterine growth and hypovolemia, with untreated mortality rates ranging 80-100 percent of all cases, which may vary depending on the severity of the transfusion. The diagnosis is based on exhaustive echographic examination of both fetuses to make an early diagnosis and correct staging, since the prognosis depends very much on early action. The treatment of choice is fetoscopic laser coagulation of vascular anastomoses between 18 and 26 weeks of gestation. The prognosis is variable, depending on the availability of fetal therapy and the gestational age at diagnosis, being prognosis generally very poor without an effective treatment(AU)

Mirror Syndrome after Fetoscopic Laser Treatment - A Case Report / Síndrome do espelho após tratamento laser por fetoscopia - caso clínico

Abstract Mirror syndrome is a rare disease with unknown pathophysiology that can be present in different diseases that can cause fetal hydrops. The prognosis is usually bad with a high perinatal mortality. We report an unusual form of mirror syndrome that manifested itself only after a successful treatment for fetal hydrops (caused by twin-twin transfusion syndrome, in Quinteros stage IV) was performed. This syndrome was controlled by medical treatment, and despite the usually bad prognosis seen in these cases, we could extend the pregnancy from the 23rd to the 34th week of gestation, resulting in the birth of 2 live infants.

Resumo A síndrome do espelho é uma doença rara, de fisiopatologia desconhecida, que se manifesta em situações obstétricas responsáveis pela presença de hidrópsia fetal. Habitualmente o prognóstico é reservado, uma vez que se associa a elevadas taxas de mortalidade perinatal. O presente caso clínico trata de uma situação de síndrome do espelho que se manifestou, atipicamente, após o tratamento eficaz para a hidrópsia fetal associada à síndrome de transfusão feto-fetal. Apesar do mau prognóstico associado a estas situações, conseguiu-se controlar a situação apenas com tratamento médico e, desta forma, prolongar a gravidez durante 12 semanas.

Resultados perinatales en el síndrome de transfusión feto fetal manejados con la técnica de Solomon: revisión de la literatura / Perinatal outcomes in fetal-fetal transfusion syndrome managed with the Solomon technique: literature review

INTRODUCCIÓN: Entre 15-20% de los embarazos gemelares monocoriales biamnióticos se complican con el síndrome de transfusión feto/fetal el cual se asocia con mortalidad superior a 90% y morbilidad significativa en el 50% del gemelo sobreviviente. La técnica láser que coagula la superficie de la placa coriónica entre los principales canales a lo largo del ecuador (técnica de Solomon), se ha sugerido para disminuir la recurrencia, prevenir complicaciones secundarias sin incrementar resultados adversos. Métodos: REVISIÓN de la literatura existente en las bases de datos MEDLINE, EBSCO, OVID, PROQUEST, COCHRANE, Lilacs, SciELO, desde el año 2000 al 2015. Se incluyeron los artículos de revisión e investigaciones originales que compararon la técnica estándar de fotocoagulación secuencial con láser y la técnica de Solomon, el resultado primario fue la reducción de la incidencia Secuencia Anemia Policitemia, recurrencia del síndrome de transfusión feto/fetal, mortalidad perinatal y morbilidad neonatal severa. RESULTADOS: Se encontraron 200 artículos, se seleccionaron seis: 1 ensayo clínico y su análisis secundario, 2 estudios de cohorte retrospectivos, 1 revisión sistemática y un estudio que compara los resultados del neurodesarrollo. Los estudios sugieren una mejoría en la sobrevida de algunos de los fetos con la técnica Solomon, menor recurrencia del síndrome de transfusión feto/fetal y Secuencia Anemia Policitemia, sin la presencia de eventos adversos. CONCLUSIÓN: La técnica de Solomon mejora la sobrevida de algunos gemelos, sin embargo no puede concluirse que haya mejoría en la mortalidad pues los estudios no tienen el suficiente poder para determinarlo.

INTRODUCTION: Between 15 to 20% of monochorionic diamniotic twin pregnancies are complicated by the twin-twin transfusion syndrome. It has a mortality greater than 90% and a significant morbidity, 50% in the surviving twin. The Solomon technique (laser photocoagulation of the main vascular channels of the chorio-nic plate surface along the entire vascular equator) has been suggested to reduce the recurrence, and pre-vent secondary complications without increasing adverse results. METHODS: Systematic review of electronic searches of the literature from 2000 to 2015 (MEDLINE, EBSCO, OVID, PROQUEST, COCHRANE, Lilacs, and SciELO). We included review articles and original investigations comparing the standard photocoagulation technique with laser ablation against the Solomon technique. The primary results were reduction of Anemia Polycythemia Sequence incidence, twin-twin transfusion syndrome recurrence, perinatal mortality and severe neonatal morbidity. RESULTS: Of 200 articles, we selected six: one clinical essay and its secondary analysis, two retrospective cohort studies, one systematic review and a study comparing neurodeve-lopmental outcomes. The studies suggested a survival improvement in some fetuses using the Solomon technique, less twin-twin transfusion syndrome recurrence and Anemia Polycythemia Sequence without the presence of adverse effects. CONCLUSION: Solomon technique improves the survival of some twins, although we cannot conclude there is mortality improvement, because the studies do not have enough power to determine that.

Twin-twin transfusion syndrome: neurodevelopment of infants treated with laser surgery / Síndrome de transfusão feto-fetal: neurodesenvolvimento de lactentes tratados com cirurgia a laser

ABSTRACT Objective To assess the neurodevelopmental functions of survivors of twin-twin transfusion syndrome (TTTS) treated by fetoscopic laser coagulation (FLC), during the first year of life, comparing them to a control group; and to verify the influence of specific variables on neurodevelopment. Method This was a prospective, longitudinal study. The sample comprised 33 monochorionic diamniotic twins who underwent FLC for treatment of TTTS and 22 full-term infants of single-fetus pregnancies. Bayley Scales of Infant and Toddler Development Screening Test were used for evaluation. Prenatal, perinatal and postnatal information were obtained. Results There was an increased frequency of infants in the TTTS group with inadequate performance compared to the control group. The identified variables (fetal donor, low economic income and cardiorespiratory disease) negatively impacted expressive communication and fine motor skills. Conclusion Although through follow-up is recommended in all TTTS survivors, particular attention is required for the high-risk group as defined in this study.

RESUMO Objetivo Avaliar o desenvolvimento neurológico de sobreviventes da sindrome de transfusão feto-fetal (STFF) submetidos à coagulação a laser por fetoscopia (CLF), durante o primeiro ano de vida, comparando estes ao grupo controle; e verificar a influência de variáveis específicas no desenvolvimento. Método Tratou-se de estudo prospectivo, longitudinal. A amostra foi composta por 33 gêmeos diamnióticos monocoriônicos submetidos à CLF para tratamento da STFF e 22 lactentes a termo de gestação única. Bayley Scales of Infant and Toddler Development Screening Test foram utilizadas para avaliação. Informações pré-natal, perinatal e pós-natal foram coletadas. Resultados Houve maior número de lactentes com desempenho inadequado no grupo STFF do que no controle. As variáveis identificadas (feto doador, baixa renda econômica e doença cárdio-respiratória) influenciaram negativamente a comunicação expressiva e as habilidades motoras finas. Conclusão Embora o acompanhamento seja recomendado para todos lactentes com STFF, especial atenção deve ser dada àqueles que apresentam fatores de risco.

Síndrome de transfusión feto fetal: una mirada global a la enfermedad / Fetofetal transfusion syndrome: a comprehensive look at the disease / Síndrome de transfusão feto no feto: um olhar mais abrangente sobre a doença

Introducción: El síndrome de transfusión feto- feto es una enfermedad compleja producida aproximadamente en 15% de los embarazos monocoriales; a pesar de su baja incidencia, su importancia radica en las graves complicaciones perinatales. Su fisiopatología está basada en el proceso por el cual los gemelos comparten las dos circulaciones a través de vasos sanguíneos anómalos, con posterior alteración hemodinámica del gemelo receptor y donante. El diagnóstico es primordialmente ecográfico y su tratamiento definitivo actual, consiste en estrategias in útero realizadas con el fin de ubicar y sellar los vasos, proporcionando una circulación individual, buscando contrarrestar su mal pronóstico, que puede llegar a una mortalidad de hasta un 100% en aquellos sin tratamiento. Objetivo: Realizar una revisión de la literatura sobre el síndrome de transfusión feto - feto abarcando una actualización en los diferentes componentes que incluyen: epidemiología, fisiopatología, avances en Diagnóstico clínico y tratamiento. Metodología: Se hizo una búsqueda en bases de datos como PubMed, Cochrane, Sciencedirect y Google académico; se encontraron 78 artículos, de los cuales finalmente cumplieron con los criterios de actualización, pertinencia y calidad 32 artículos; se revisó fisiopatología, diagnóstico y tratamiento. Se descartaron artículos que no cumplían con criterios de inclusión. Resultados: Se seleccionó la información más actualizada y completa de las bases de datos encontradas, respecto a clasificación, etiología, fisiopatología, diagnóstico, tratamiento y pronóstico. Conclusiones: El síndrome de Transfusión Feto-Feto consiste en un desbalance hemodinámico de anastomosis placentarias, niveles aumentados de vasopresina y finalmente cambios en el eje renina angiotensina aldosterona, que requiere un diagnóstico temprano con tratamiento oportuno para mejorar el pronóstico y prevenir complicaciones fetales.

Introduction: Fetofetal transfusion syndrome is a complex disease produced in about 15% of monochorionic pregnancies, despite its low incidence, its importance lies in its serious perinatal complications. Its pathophysiology is based on the process in which twins share the two circulations through abnormal blood vessels, with a subsequent hemodynamic alteration of the receptor twin and donor twin. Its diagnosis is primarily with an ultrasound scan and its current definitive treatment consists in-utero strategies performed in order to locate and seal the vessel, providing a single movement, seeking to counteract its poor prognosis, which can lead to a mortality rate of up to 100% in those patients without treatment. Objective: To review the literature about fetofetal transfusion syndrome, covering an update about various components including epidemiology, pathophysiology, clinical advances in its clinical diagnosis and treatment. Methodology: A search was conducted in databases such as PubMed, Cochrane, Sciencedirect and Google scholar and 78 articles were found, but only 32 of them finally achieved the criteria for updating, relevance and quality; also the pathophysiology, diagnosis and treatment were revised in each one. The articles that did not fulfill the inclusion criteria were discarded. Outcomes: The most updated and comprehensive information found in databases was selected according to its classification, etiology, pathophysiology, diagnosis, treatment and prognosis. Conclusions: Fetofetal transfusion Syndrome is a hemodynamic imbalance of placental anastomosis, increased levels of vasopressin and finally, changes in the renin angiotensin aldosterone axis, which requires an early diagnosis and treatment to improve the prognosis and prevent fetal complications.

Introdução: A doença de transfusão feto-fetal é complexa e aproximadamente 15% das gestações monocoriônicas, padece deste síndrome, embora a incidência seja baixa, sua importância esta nas complicações perinataies. A sua patofisiologia baseia-se no processo pelo qual os gêmeos partilham duas circulações através de vasos sanguíneos anormais, com alteração na hemodinâmica subsequente do gêmeo receptor como do doador. Seu diagnóstico é ultrassonográfico e atualmente o seu tratamento consiste em realizar as estratégias uterinas para localizar e selar os vasos, a fim de cambiar o seu mau prognóstico, caso contrário pode ocasionar a norte até de 100% dos pacientes. Objetivo: Revisar a literatura, sobre el síndrome de transfusão feto – feto, incluindo uma atualização nos diferentes componentes como epidemiologia, fisiopatologia, avanços clínicos no diagnóstico e tratamento. Metodologia: se investigou a base de dados como PubMed, Cochrane, Sciencedirect e Google acadêmico, entre os 78 artigos pesquisados, 32 apresentaram os criterios de atualização, pertinência e qualidade científica, também foi revisto fisiopatologia, diagnóstico e tratamento. Se descartaram, os artigos que não continham os critérios requeridos. Resultados: se escolheu a informação mais atualizada e completa da base de datos pesquisada, com relação à classificação, etiologia, fisiopatologia, diagnóstico, tratamiento e pronóstico.

Advances in fetal surgery / Avanços em cirurgia fetal

ABSTRACT This paper discusses the main advances in fetal surgical therapy aiming to inform health care professionals about the state-of-the-art techniques and future challenges in this field. We discuss the necessary steps of technical evolution from the initial open fetal surgery approach until the development of minimally invasive techniques of fetal endoscopic surgery (fetoscopy).

RESUMO São discutidos os principais avanços na terapia cirúrgica do feto, tendo como objetivo informar profissionais da área da saúde sobre qual o estado atual da arte e quais seus desafios futuros. São debatidos os passos que foram necessários para a evolução da técnica inicial, quando a cirurgia fetal era realizada a céu aberto, até o desenvolvimento de técnicas minimamente invasivas, de cirurgia endoscópica fetal (fetoscopia).

The Impact of Introduction of Fetoscopic Laser Surgery on Twin-to-Twin Transfusion Syndrome

PURPOSE: Fetoscopic laser surgery (FLS) is considered an optimal therapeutic strategy for twin-to-twin transfusion syndrome (TTTS). We aimed to determine the clinical outcomes of TTTS patients since the introduction of FLS. METHODS: A retrospective study of TTTS patients born between January 2005 and December 2015 was conducted. Mortality and morbidity were compared in the FLS and non-FLS groups. The FLS group was divided into selective FLS and Solomon technique group, and subgroup analysis was performed. RESULTS: Of 70 pregnancies diagnosed with TTTS during the study period, FLS was performed for 35 (50%). Survival of at least one infant at discharge was achieved in 100% (35/35) of pregnancies in the FLS group and 91.4% (32/35) in the non-FLS group (P=0.028). Subgroup analysis revealed that the Solomon technique group had a higher dual survival rate than the selective FLS group (81.3% vs. 57.9%, P=0.036). Neonatal mortality was significantly lower in the FLS group than in the non-FLS group (1.7% vs. 16.4%, P=0.005). Severe intraventricular hemorrhage more than grade 3 was less frequent in the FLS group than in the non-FLS group (0% vs. 18.2%, P= 0.001). The FLS group showed smaller inter-twin differences in birth weight (24.4% vs. 33.7%, P= 0.032) and lower incidence of twin anemia-polycythemia sequence (0% vs. 43.8%, P<0.001). CONCLUSION: Since the introduction of FLS, the survival rate of TTTS patients has improved. The Solomon technique resulted in better dual survival rates than selective FLS.

Cirugía fetal: hernia diafragmática congénita presentación de caso / Fetal surgery: congenital diaphragmatic hernia: case presentation

Introducción: la hernia diafragmática congénita es una malformación que resulta de la fusión incompleta de la membrana pleuroperitoneal, ocurre en alrededor de 1 / 2.000-5.000 recién nacidos vivos y es causa por lo general de síntomas severos de insuficiencia respiratoria e hipertensión arterial pulmonar en los niños de este grupo de edad. El tratamiento se basa en mantener las mejores condiciones respiratorias en el niño mediante manejo de terapia intensiva neonatal y una vez que se logra este objetivo, se debe proceder con la corrección quirúrgica del defecto anatómico. Desafortunadamente, a pesar del avance en el manejo respiratorio del recién nacido gravemente enfermo, la mortalidad por este padecimiento se reporta por arriba de 75%. Se han determinado diversos factores pronósticos prenatales que confirman su severidad y la inviabilidad de estos fetos. La oclusión de la tráquea fetal en modelos animales con HDC inducida y en el feto humano evidenció desarrollo y crecimiento pulmonar. La oclusión traqueal fetal ofrece esperanzas vitales para estos casos de HDC severa que con el tratamiento convencional tienen una mortalidad cercana al 100%. Es necesaria una serie más amplia para obtener conclusiones definitivas. El principal enemigo de la cirugía fetal es el trabajo de parto prematuro y la rotura prematura de membranas. El acceso fetoscópico y la tocolisis reducen la incidencia de esta eventualidad. Caso clínico: presentamos dos casos clínicos de recién nacidos con hernia diafragmática de lado izquierdo que permitió el paso de la mayoría de las vísceras abdominales hacia el tórax con síntomas leves de falla respiratoria.

Introduction: congenital diaphragmatic hernia results from the incomplete fusion of the pleuroperitoneal membrane and occurs with a frequency of about ½.000-5.000 live-births. Despite advances in neonatal intensive care and surgery, mortality varies from one institution to another and may be above 75%. There are some prenatal prognostic factors that assess the CDH severity and thus, the fetal viability. Fetal tracheal occlusion (TO) in experimental animal CDH models, and in human fetuses induce lung growth. Fetal TO offers a better outcome for patients with severe CDH that otherwise would have a 100% mortality rate despite the advanced postnatal care. A greater number of cases are needed to obtain stronger conclusions. The major enemies of fetal surgery are the premature rupture of membranes and the preterm labor. Fetoscopic approach and tocolysis could help preventing these eventualities. Case study: we present the cases of two newborn babies with left- sided diaphragmatic hernia that allowed the passage of most of the abdominal viscerae into the thorax with only mild symptoms of respiratory failure. Keywords: diaphragmatic hernia, fetal surgery, intrauterine treatment, fetoscopy, tracheal occlusion.

Comparação entre duas técnicas cirúrgicas para correção intra-uterina de meningomielocele em feto de ovelha / Comparison between two surgical technique for prenatal correction of meningomyelocele in sheep

Introdução: A incidência de defeitos do tubo neural é de cerca de 1/1000 nascimentos. Na mielomenngocele, o tratamento intra-uterino utilizando a técnica neurocirúrgica clássica, apresenta melhor prognóstico neurológico, do que o tratamento pós natal, mas está associado a complicações maternas e fetais. Novas técnicas de correção estão sendo estudadas para diminuir a morbidade materna e fetal. Objetivo: Comparar os efeitos sobre a medula de duas técnicas cirúrgicas de correção intra-uterina de um defeito semelhante à mielomeningocele , em fetos de ovelha. Métodos: Em 15 fetos foi criado um defeito semelhante à mielomeningocele (laminectomia e excisão de dura-máter) no 90° dia de gestação. O tipo de correção foi randomizado. No grupo 1, o defeito foi corrigido usando a técnica neurocirúrgica clássica, com a sutura de três camadas (dura-máter, músculo e pele), realizada por um neurocirurgião. No grupo 2, um especialista em Medicina Fetal, utilizou uma técnica simplificada, colocando um fragmento de celulose Biosintética sobre a medula e suturando apenas a pele sobre a celulose. Próximo ao termo da gestação (132° dias), os fetos foram sacrificados para análise anatomopatológica. Resultados: Ocorreram 1 morte materna, 3 casos de trabalho de parto precoce e 4 tardios. Um total de 10 casos foram viáveis para avaliação anatomopatológica 10 casos, 6 no grupo 1 e 4 no grupo 2. No grupo 1, todos os casos mostraram aderência da medula à cicatriz (meningoadesão) e perda da arquitetura medular por destruição do funículo posterior e 5 de 6 casos apresentaram perda da visualização da substância cinzenta. No grupo 2, observamos em todos os casos, a formação de uma neoduramater, separando o tecido nervoso do músculo adjacente, sendo que o funículo posterior e a substância cinzenta estavam preservados. Conclusão: A técnica simplificada foi superior à técnica neurocirúrgica, com maior preservação da medula e evitando as aderências do tecido nervoso. Nossos...

Introduction: The incidence of neural tube defects is about 1/1000 births. In myelomeninocele, the intrauterine treatment with the classical neurosurgical technique suggest better neurological prognosis for the fetus, than postnatal treatment, but it is associated with maternal and fetal complications. New correction techniques are being studied to decrease maternal and fetal morbidity. Objective: To compare the effects on the medulla of two surgical techniques for intrauterine correction of myelomeningocele-like defect in sheep. Methods: In 15 pregnant sheep a myelomeningocele -like defect (laminectomy and dural excision) was created in the lumbar region in 90o day gestation. The type of correction was randomized. In group 1 the defect was corrected using the classic neurosurgical technique of three layers suture (dura-mater, muscle and skin closure) performed by a neurosurgeon. In group 2, a fetal medicine specialist used a biosynthetic cellulose patch to protect the medulla and only the skin was sutured above it. Near term (132o day gestation) fetuses were sacrificed for pathological analysis. Results: One maternal death occurred, early preterm labour, and late preterm labour occurred in 3 and 4 cases, respectively. A total of 10 cases were available for pathological analysis, 6 in group 1 and 4 in group 2. In group 1, all the cases showed a adherence of the medulla to the scar (meningoneural adhesion) and a destruction of the normal architecture of nervous tissue, whithout view of posterior funiculus and in 5 of 6 cases without view of grey matter. In group 2, in all the cases were observed a formation of an organized tissue involving the cellulose patch, a neoduramater, separating the nervous tissue of adjacent muscle, preserving the posterior funicullus and grey matter. Conclusion: The simplified new technique was better than the classical neurosurgical technique. It preserved the nervous tissued and avoided the adherence of the medulla to the scar....

Improvement of motor function and decreased need for postnatal shunting in children who had undergone intrauterine myelomeningocele repair / Melhora em função motora e necessidade reduzida de shunts pós-natais em crianças submetidas a cirurgia fetal uterina da mielomeningocele

Objective To compare neuromotor development between patients who did and those who did not undergo intrauterine myelomeningocele repair. Methods Children with myelomeningocele aged between 3.5 and 6 years who did undergo intrauterine repair (Group A, n=6) or not (Group B; n=7) were assessed for neuromotor development at both anatomical and functional levels, need for orthoses, and cognitive function. Results Intrauterine myelomeningocele repair significantly improved motor function. The functional level was higher than the anatomical level by 2 or more spinal segments in all children in Group A and 2 children in Group B, with a significant statistical difference between groups (p<0.05). Five children in Group A and one in Group B were community ambulators. Conclusion Despite the small sample, it was observed that an improvement of motor function and decreased need for postnatal shunting in the 6 children who had undergone intrauterine myelomeningocele repair. .

Objetivo Comparar o desenvolvimento neuromotor de pacientes submetidos à cirurgia fetal intrauterina da mielomeningocele ao de pacientes não submetidos ao procedimento. Métodos Foram avaliados: o desenvolvimento neuromotor (descrevendo o nível anatômico e motor funcional), o tipo de marcha, a necessidade de órteses e o nível cognitivo de crianças com mielomeningocele entre 3,5 e 6 anos de idade, submetidas (Grupo A; n=6) ou não submetidas (Grupo B; n=7) à cirurgia fetal intra-uterina. Resultados A função motora apre-sentou melhora significante, com nível funcional mais elevado em dois ou mais segmentos em relação ao nível anatômico em todas as crianças do Grupo A e em duas crianças do Grupo B, com diferença estatística entre os grupos (p<0.05). Cinco crianças do Grupo A e uma do grupo B eram deambuladoras comunitárias. Conclusão Apesar da pequena amostragem, nos 6 casos de cirurgia prenatal observouse melhora da função motora e menor necessidade de shunts pós-natais. .