Manejo del síndrome de transfusión feto fetal en pandemia COVID-19: reporte de caso

El síndrome de transfusión feto-fetal (STFF) es una complicación propia de los embarazos gemelares monocoriales, con mortalidad perinatal del 80 a 100%. Ocurre por un desequilibrio en el flujo sanguíneo placentario entre ambos gemelos. El diagnóstico se basa en la discordancia del líquido amniótico y luego se clasifica en estadios según los criterios de Quintero. El tratamiento principal es la fetoscopia y fotoablación con energía láser (FFL) de las anastomosis placentarias. Presentamos un caso de gestación gemelar monocorial biamniótica tratado con FFL en plena cuarentena e inmovilización de la pandemia COVID-19 en Perú, que incluyó diagnóstico oportuno y referencia rápida para recibir el tratamiento especializado.

The twin-to-twin transfusion syndrome (TTTS) is a potentially lethal complication that can occur in monochorionic twin pregnancies due to an imbalance in placental blood flow. Diagnosis is based on amniotic fluid discordance and classified using the Quintero staging system. The primary treatment is fetoscopic laser photocoagulation (FLP) of placental anastomoses. A successful case of FLP treatment in a monochorionic diamniotic twin pregnancy with TTTS during COVID-19 pandemic in Peru is presented.

Prenatal repair of gastroschisis using partial carbon dioxide insufflation fetoscopy: lessons learned

ABSTRACT We report the long-term outcomes of a case of prenatal gastroschisis repair using a fully percutaneous fetoscopic approach with partial carbon dioxide insufflation. Surgery was performed as an experimental procedure before the scheduled elective birth. The fetal intestines were successfully returned to the abdominal cavity without any fetal or maternal complications. Ultrasonography performed 24 hours later revealed bowel peristalsis and no signs of fetal distress. After 48 hours, partial extrusion of the small bowel was observed, and the fetus was delivered. Gastroschisis repair was immediately performed upon delivery using the EXIT-like procedure as per our institutional protocol. The newborn did not require assisted mechanical ventilation, was discharged at 14 days of age and was then exclusively breastfed. At 3-year follow-up, the patient had no associated gastroschisis-related complications. This is the first case of prenatal repair of gastroschisis, which provides baseline knowledge for future researchers on the potential hurdles and management of prenatal repair.

Neurodevelopmental outcomes in twin-to-twin transfusion syndrome survivors at 12 months old after fetoscopic laser occlusion of chorioangiopagous vessels / 中华围产医学杂志

Objective:To investigate the risk factors for cerebral injury in survivors of twin-to-twin transfusion syndrome (TTTS) after fetoscopic laser occlusion of chorioangiopagous vessels(FLOC) and to analyze the neurodevelopmental outcomes at 12 months of corrected age.Methods:A total of 136 cases of TTTS receiving FLOC in the Third Affiliated Hospital of Zhengzhou University from May 2018 to August 2021 were retrospectively selected as the FLOC group, and the survivors were followed up. Neurological development at 12 months of corrected age was assessed using the Griffiths mental development scales-Chinese (GDS-C) from five dimensions with locomotor, personal-social, hearing and language, hand-eye coordination and performance subscales. Eighty-eight fetuses of TTTS pregnancies receiving expectant treatment or amniotic fluid reduction were selected as the non-FLOC group. The perinatal mortality and the incidence of cerebral injury in the two groups were compared, as well as the incidence of cerebral injury between patients undergoing Solomon surgery and selective laser surgery in the FLOC group. Generalized estimating equations were used to analyze the risk factors for neonatal cerebral injury after FLOC and the factors influencing general developmental quotient score at the corrected age of 12 months. Chi-square test, t-test, and Mann-Whitney U test were used for statistical analysis. Results:(1) The perinatal mortality rate in the FLOC group was lower than that in the non-FLOC group [14.7% (20/136) vs 26.1% (23/88), χ 2=4.50, P=0.034]. There was no statistical significance in the incidence of neonatal cerebral injury between the two groups [18.7% (23/123) vs 21.8% (17/78), χ 2=0.29, P=0.592], but the incidence of severe cerebral injury in the FLOC group was lower than that in the non-FLOC group [6.5% (8/123) vs 15.4% (12/78), χ 2=4.20, P=0.040]. (2) In the FLOC group, there was no significant difference in the incidence of cerebral injury between donors and recipients, or between Solomon surgery and selective laser surgery [16.4% (10/61) vs 21.0% (13/62), χ 2=0.42; 20.0% (9/45) vs 17.9% (14/78), χ 2=0.08; both P>0.05]. (3) Multivariate analysis showed that neonatal asphyxia ( OR=7.04, 95% CI: 1.45-34.20, P=0.016) and higher preoperative TTTS stage ( OR=2.05, 95% CI: 1.10-3.82, P=0.023) were risk factors for neonatal cerebral injury. (4) Fifty-two cases were successfully followed up at the corrected age of 12 months, and the incidence of developmental delay in at least one dimension was 34.6% (18/52). Developmental delay was mainly manifested in locomotor skills and language, accounting for 26.9% (14/52) and 11.5% (6/52). No significant difference in Z value was found between recipients and donors in each dimension (all P>0.05). Solomon surgery, larger gestational age at operation and low birth weight were related to low general developmental quotient score (95% CI:－11.71 to－0.23，－1.99 to－0.47，0.00-0.01，respectively，all P<0.05). Conclusions:The occurrence of cerebral injury in TTTS survivors after FLOC is related to preoperative TTTS staging and intrapartum neonatal asphyxia. Neurodevelopment of survivors is related to birth weight and gestational age at surgery, and there is a higher incidence of mild developmental delay at corrected age of 12 months.

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

Pregnancy outcome and placental characteristics of twin-to-twin transfusion syndrome with different vascular patterns following fetoscopic laser surgery / 中华围产医学杂志

Objective:To evaluate the effect of different superficial vascular patterns on pregnancy outcome and residual anastomosis following laser coagulation in placentae with twin-to-twin transfusion syndrome (TTTS).Methods:This study retrospectively collected and analyzed the clinical data and postnatal placenta perfusion characteristics of 57 cases of TTTS who received fetoscopic laser occlusion of chorioangiopagous vessels (FLOC) and delivered at Peking University Third Hospital from April 2014 to April 2019. According to the vascular pattern, all the cases are divided into four groups, which were parallel, staggered, mixed, and monoamniotic groups. Differences in the operation time and method, pregnancy outcome, and residual vascular anastomosis between the four groups were compared using analysis of variance, non-parametric tests, and Chi-square (or Fisher's exact) tests. Results:Among the 57 cases, the staggered, mixed, parallel, and monoamniotic types were accounted for 68.4%(39/57), 15.8%(19/57), 14.0%(8/57), and 1.7%(1/57), respectively. After exclusion of one case of monoamniotic type, gestational weeks at onset and surgery in the parallel group were both later than the staggered and mixed groups [23.0 (22.0-26.3) weeks vs 21.0 (17.0-24.7) weeks and 22.6 (21.3-23.9) weeks, H=10.306, P=0.006; 25.0 (22.6-26.3) weeks vs 22.0 (17.4-24.9) weeks and 23.2 (22.4-24.0) weeks, H=9.926, P=0.007; all P'<0.016 7]. There was no statistical significance in the differences in operation time and method, gestational age at the end of the pregnancy, neonatal birth weight, or birth weight discordance between the three groups. The diameter of residual vascular anastomosis of women in the staggered group was less than that in the mixed group [0.6 (0.1-5.0) mm vs 1.4 (0.3-5.1) mm, P'<0.016 7], but no significant difference was observed in the parallel-group [0.9 (0.2-3.6) mm] neither with the mixed or staggered group. The staggered group was noted for an increased distance ratio of umbilical cord insertion compared with the parallel and the mixed group [0.66 (0.59-1.00) vs 0.49 (0.25-0.55) and 0.48 (0.42-0.53); P'<0.016 7]. There was no significant difference between the parallel and the mixed groups. Conclusions:Placental superficial vascular patterns may affect the residual vascular anastomosis in women with TTTS following FLOC, which should be thoroughly evaluated before the operation to develop an individual management plan to reduce residual anastomosis incidence.

Fetoscopic cord laser therapy in management of monochorionic monoamniotic twin pregnancies / 中华围产医学杂志

Objective:To analyze fetoscopic cord laser therapy for management of monochorionic monoamniotic (MCMA) twin pregnancies.Methods:The clinical data of fetoscopic cord laser therapy, including cord occlusion, transection, and disentanglement in three pairs of MCMA twins from January 2020 to January 2021 in Peking University Third Hospital were summarized. Literature on cord occlusion and/or transection in MCMA twins were retrieved from Cochrane Library, PubMed, EMBASE, CBM, WanFang, and CNKI from the time at establishment to December 2020. The clinical conditions, surgical indications and methods, disease progression, and maternal and infant prognosis were analyzed.Results:Three cases of MCMA twins in this study period received fetoscopic cord laser therapy between 17-24 weeks, among which two cases gave birth at full-term without any maternal or infant complications, and one was terminated due to fetal malformation. Seven English articles including 29 MCMA twin pregnancies were retrieved. In addition to the three cases reported in this article, a total of 32 cases were analyzed. The indication of cord occlusion and/or transection included twin-reversed arterial perfusion sequence (21.9%, 7/32), fetal malformation (46.9%, 15/32), selective fetal growth restriction (sFGR) (21.9%, 7/32), twin-to-twin transfusion syndrome (TTTS) (3.1%, 1/32), TTTS combined with sFGR (3.1%, 1/32), single intrauterine death (3.1%, 1/32). Gestational age at surgery was between 14 +1 to 27 +3 weeks. No maternal complication due to the operation was reported. After exclusion of two cases who did not receive cord transection and one case was terminated due to fetal malformation, all the other 29 co-twins were born alive at the gestational age between 24 +3 to 40 weeks and birth weight between 800-3 800 g. Among the 29 live born babies, four died soon after birth with unclarified reasons in the literature and one was born with multiple malformations which were detected prenatally, and the other 24 neonates were healthy during the follow-up from 1 month to 9 years old. Conclusions:For MCMA twin pregnant women with umbilical cord entanglement or other indications for fetal reduction, cord occlusion, transection, and disentanglement using fetoscopic cord laser is safe and effective for protecting the surviving fetus.

Fetal therapies as standard prenatal care in Japan

With recent advances in fetal medicine, various attempts have been made to save fetuses facing perinatal death or devastating consequences despite optimal management after birth. The concept of the fetus as a patient has been established through the application of in utero treatments. This paper reviews fetal therapies in order to highlight the role of perinatal medicine as standard prenatal care. Fetal therapies consist of medical therapy, percutaneous ultrasound-guided surgery, fetoscopic surgery, and open fetal surgery. In the 1980s, with advances in ultrasound imaging, percutaneous ultrasound-guided surgeries such as vesicoamniotic shunting for lower urinary tract obstruction and thoracoamniotic shunting (TAS) for fetal hydrothorax (FHT) were started. In the 1990s, fetoscopic laser surgery (FLS) for twin-twin transfusion syndrome (TTTS) was introduced, and later, a fetoscopic approach for congenital diaphragmatic hernia was also established. The revival of open fetal surgery, introduced in the 1980s by pediatric surgeons, began in the 2010s after a successful clinical study for myelomeningocele. Although many fetal therapies are still considered experimental, some have proven effective, such as FLS for TTTS, TAS for primary FHT, and radiofrequency ablation (RFA) for twin reversed arterial perfusion (TRAP) sequence. These three fetal therapies have been approved for coverage by Japan National Health Insurance as a result of clinical studies performed in Japan. FLS for TTTS, TAS for primary FHT, and RFA for TRAP sequence have become standard prenatal care approaches in Japan. These three minimally invasive fetal therapies will help improve the perinatal outcomes of fetuses with these disorders.

Fetoscopia y fotocoagulación láser en el manejo del síndrome de transfusión feto fetal: serie de los primeros casos tratados en el Perú / Fetoscopy and laser photocoagulation in the treatment of twin-to-twin transfusion syndrome: series of cases treated in Peru

Twin-to-twin transfusion syndrome is a severe complication of twin monochorial pregnancies manifested with one hypovolemic fetus (donor) and another hypervolemic fetus (receiver). Perinatal mortality is about 90 to 100% if untreated. The accepted treatment is fetoscopy and selective laser photocoagulation of the placental shunts that connect both twins. Currently, this specialized procedure is only performed in our institution. We present the first cases of monochorionic twin pregnancy complicated with twin-to-twin transfusion syndrome that underwent laser photocoagulation and fetoscopy.

El síndrome de transfusión feto fetal es una complicación severa de los embarazos gemelares monocoriónicos, que fisiopatológicamente se manifiesta con un feto hipovolémico (donante) y un feto hipervolémico (receptor). Conlleva una mortalidad perinatal de 90 a 100% si no es tratado. El tratamiento aceptado es la fetoscopia y fotocoagulación láser selectiva de las anastomosis placentarias que conectan ambos gemelos, procedimiento especializado que se viene realizando solo en nuestra institución. Se presenta los primeros casos de gestación gemelar monocoriónica complicado con síndrome de transfusión feto fetal sometidos a fetoscopia y fotocoagulación laser.

Cirurgia fetal em Brasília

Brasilia is the capital of Brazil. There are currently two public hospitals in the city that are able to perform fetal surgery: Hospital Materno-Infantil de Brasília (HMIB) and Hospital Universitário de Brasília (HUB). In this paper, we present the data from HUB. Ten patients underwent invasive procedures during pregnancy in the last five years. There was one intrauterine transfusion (cordocentesis) and zero laser ablation for monochorionic diamniotic pregnancy or fetal endotracheal occlusion for diaphragmatic hernia. The paucity of cases is mainly due to a failure in the diagnosis and a delay in the reference to tertiary centers.

Brasilia es la capital de Brasil. Existen en la actualidad dos hospitales públicos en la ciudad que tienen la posibilidad de realizar cirugía fetal: el Hospital Materno-Infantil de Brasília (HMIB) y el Hospital Universitário de Brasília (HUB). En el presente artículo presentamos la información del HUB. En los últimos cinco años, diez pacientes fueron sometidos a procedimientos invasivos durante la gestación. Hubo una transfusión intrauterina (cordocentesis) y ninguna ablación láser por embarazo diamniótico monocoriónico u oclusión endotraqueal del feto por hernia diafragmática. La escasez de casos se debe principalmente a la falla en los diagnósticos y la demora en la referencia a los centros terciarios.

Cirugía fetal en Costa Rica

Fetal surgery is an alternative to improve patient outcomes for diseases such as twin to twin transfusion syndrome, myelomeningocele and many others. This review highlights an issue unknown in our country, even when these procedures have been successfully performed in our setting. The application of ultrasound has allowed the correct, timely identification of patients potentially treatable with those techniques. We reviewed the available evidence supporting the benefits of those procedures on both the fetuses and their mothers. We conclude that fetal surgery represents a multidisciplinary specialty that requires the participation of the obstetrician, neonatologist and anesthesiologist, among many other specialists.

La cirugía fetal es una alternativa para mejorar los resultados del paciente para enfermedades como el síndrome de transfusión feto-feto y el mielomeningocele, entre muchas otras. Esta revisión pretende dar a conocer un tema que en nuestro país era desconocido, a pesar de que estos procedimientos ya se han empezado a realizar con éxito en nuestro medio. La aplicación del ultrasonido ha permitido la identificación prenatal oportuna y adecuada del paciente potencialmente tratable mediante estas técnicas. Se revisó la evidencia existente de los beneficios que estas terapias aportan tanto al feto como a la madre. Se concluye que la cirugía fetal es una especialidad multidisciplinaria donde participan el perinatólogo, el obstetra, el neonatólogo, el anestesiólogo, entre otros especialistas.

Fetoscopic laser surgery for twin to twin transfusion syndrome

Twin to twin transfusion syndrome (TTTS) is a complication of circa 10% of all monochorionic pregnancies (MC). A predominantly unidirectional, net intertwin blood flow through placental vascular anastomoses triggers a variety of complex renal and cardiovascular disturbances in both fetuses. Left untreated, severe TTTS leads to a mortality rate above 80% and 15-50% morbidity in the survivors. Fetoscopic laser coagulation (FLC) of the placental anastomoses is the first line treatment for severe cases before 27 weeks and it has been shown to improve perinatal outcomes. The risk of preterm premature rupture of membranes (PPROM) and prematurity associated with FLC has to be balanced with the risk of adopting expectant management in every particular case. Since there is no accurate way to predict the evolution of the disease, and no effective method to prevent post-procedure PPROM has been described, the indication of FLC is as challenging as the procedure itself.

El síndrome de transfusión de gemelo a gemelo (TTTS) complica alrededor del 10% de todos los embarazos monocoriónicos (MC). Un flujo sanguíneo intergemelar neto, predominantemente unidireccional, a través de anastomosis vasculares placentarias, desencadena una variedad de alteraciones renales y cardiovasculares complejas en ambos fetos. Sin tratamiento, el TTTS grave conduce a una mortalidad superior al 80% y una morbilidad del 15 a 50% en los sobrevivientes. La coagulación láser fetoscópica (CLF) de las anastomosis placentarias es el tratamiento de elección para casos graves antes de las 27 semanas y ha demostrado mejorar los resultados perinatales. El riesgo de rotura prematura de membranas (RPM) y de prematuridad asociada a CLF se debe sopesar con el riesgo de la conducta expectante en cada caso particular. Dado que no hay una manera precisa de predecir la evolución de la enfermedad, y no se ha descrito aun ningún método eficaz para prevenir la RPM posterior al procedimiento, la indicación de la CLF es tan desafiante como el procedimiento en sí mismo.

Cirugía fetoscópica en mielomeningocele

Myelomeningocele affects 17,8 in 100 000 newborns. It is one of the ten leading causes of death in children under the age of 10 in Mexico and it is associated with high cognitive, sensory and motor morbidity. Studies in animals and, later, the Management of Myelomeningocele Study (MOMS), showed that the repair of prenatal neural tube defects decreases the risk of hydrocephalus and improves motor function at the age of 30 months. Prenatal fetal surgery for myelomeningocele described in MOMS is performed through hysterotomy, and is associated to significant maternal and fetal morbidity. Thus, the therapeutic approach has evolved to fetoscopic techniques with less maternal and fetal complications and better perinatal results. In this section, we describe the different fetoscopic techniques, their evolution, advantages and disadvantages, and the challenges for fetal surgical techniques in the future.

El mielomeningocele se presenta en 17,8:100 000 nacidos vivos. En México se encuentra entre las primeras 10 causas de muerte en niños menores de 10 años y está asociado a alta morbilidad cognitiva, sensorial y motora. Estudios en animales y posteriormente el estudio MOMS (The Management of Myelomeningocele Study) demostró que la reparación prenatal de los defectos del tubo neural disminuye el riesgo de hidrocefalia y mejora la función motora a los 30 meses de edad. La cirugía fetal prenatal descrita en el MOMS para el mielomeningocele se realiza a través de una histerotomía, la cual se asocia con significativa morbilidad materna y fetal. Por lo tanto, se ha evolucionado a técnicas fetoscópicas con menos complicaciones maternas y fetales, obteniendo mejores resultados perinatales. En este apartado se describe las diferentes técnicas fetoscópicas, su evolución, ventajas y desventajas en cada una de ellas, así como los retos en el futuro.

Cirugía fetal en bandas amnióticas

The amniotic band syndrome (ABS) is characterized by accumulation of fibrous tissue bands or strings that adhere to the fetus and may produce compression in different sites. Most cases present multiple congenital anomalies incompatible with life. A small group of fetuses have isolated constrictions of the limb that may cause severe limb dysfunction or limb amputation if not treated during pregnancy. This rare condition occurs in approximately 1:1 200 to 1:15 000 live births. The exact cause of ABS remains uncertain; however, theories of intrinsic, extrinsic and iatrogenic reasons have been proposed. The evolution of the limb affected by ABS is characterized by progression of distal edema due to venous obstruction, and intrauterine amputation of the limb from vascular insufficiency. Fetoscopic release of the amniotic bands through minimally invasive surgery techniques may allow life preservation as well as limb function. Literature review suggests that fetoscopic release of amniotic bands allows preservation of limb function in 50% of cases.

El síndrome de bandas amnióticas (SBA) se caracteriza por un acúmulo de bandas o cuerdas de tejido fibroso que se adhieren al feto y pueden producir compresión en diferentes partes del mismo. La gran mayoría de los casos presentan múltiples anomalías congénitas incompatibles con la vida. En un pequeño grupo de fetos se evidencian constricciones aisladas de la extremidad, las cuales pueden causar disfunción severa de la extremidad o amputación del miembro si no son tratadas mientras el feto está todavía en el útero. Esta rara condición ocurre en aproximadamente 1:1 200 a 1:15 000 nacidos vivos. La causa exacta de SBA permanece incierta; sin embargo, se plantean teorías de origen intrínseco, extrínseco y iatrogénico. La evolución de la extremidad afectada por SBA se caracteriza por la progresión de edema distal debido a obstrucción venosa, con amputación intrauterina del miembro secundaria a insuficiencia vascular. La liberación fetoscópica de las bandas amnióticas a través de técnicas de cirugía mínimamente invasivas, puede permitir la preservación de la vida, así como la función de los miembros en los casos de fetos con diagnóstico de SBA. Al revisar la literatura se concluye que la liberación fetoscópica de bandas amnióticas permite la preservación de la función de la extremidad en 50% de los casos.

Cirugía fetal para hernia diafragmática congénita en América Latina

Congenital diaphragmatic hernia is an anomaly in the formation of the diaphragm, which is associated with high neonatal mortality secondary to pulmonary hypoplasia and neonatal pulmonary arterial hypertension. Diagnosis is made by ultrasound, usually in the 20th week of gestation, and it is mandatory to request genetic studies (karyotype and microarrays) due to the high risk of chromosomal and monogenic anomalies. In isolated cases, the estimation of lung size in combination with the identification of liver herniation is the best predictive parameter of neonatal survival. In cases with serious pulmonary hypoplasia, fetoscopic tracheal occlusion has improved the survival rate in 30%. In this article, indications, selection criteria, technique and results of fetoscopic tracheal occlusion are reviewed, and differences in neonatal survival rate in Latin America are highlighted.

La hernia diafragmática congénita es una anomalía en la formación del diafragma, asociada a elevada mortalidad neonatal secundaria a hipoplasia pulmonar e hipertensión arterial pulmonar neonatal. El diagnóstico se realiza por ultrasonido, generalmente en la semana 20 de gestación, y es mandatorio solicitar estudios genéticos (cariotipo y microarreglos) debido al alto riesgo de asociación con anomalías cromosómicas y monogénicas. En casos aislados, la estimación del tamaño pulmonar en combinación con la identificación de herniación hepática es el mejor parámetro predictivo de supervivencia neonatal. En los casos con hipoplasia pulmonar grave, la oclusión traqueal fetoscópica ha demostrado una mejoría en la tasa de supervivencia en 30% con respecto a la probabilidad basal. En este artículo, se resume las indicaciones, criterios de selección, técnica y resultados de la oclusión traqueal fetoscópica, destacando las diferencias de tasa de supervivencia neonatal que existen en América Latina.

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.

Inter-twin placental anastomoses not crossing the dividing membrane: laser photocoagulation of communicating vessels in the twin-to-twin transfusion syndrome / Anastomosis placentarias intergemelares que no cruzan la membrana divisoria: fotocoagulación láser de vasos comunicantes en el síndrome de transfusión intergemelar

Twin-to-twin transfusion syndrome (TTTS) is thought to result from an unbalanced exchange of blood through inter-twin vascular anastomoses. Laser photocoagulation of communicating vessels has been used to prevent the development of the syndrome. Some authors have proposed that vessels crossing the membrane are "suspicious" to participate in the development of the syndrome and the laser photocoagulation of these vessels would reverse the syndrome. We present two cases of TTTS where the communicating vessels did not cross the dividing membrane, which were successfully treated with laser.

El síndrome de transfusión intergemelar (STIG) resulta de un intercambio desbalanceado de sangre a través de anastomosis vasculares entre las circulaciones de ambos fetos en embarazos monocoriales. La fotocoagulación láser de vasos comunicantes se ha utilizado para detener el desarrollo del síndrome. Algunos autores han propuesto que los vasos que cruzan la membrana divisoria son 'sospechosos' de participar en el desarrollo del síndrome y que la fotocoagulación láser de estos es capaz de revertirlo. Presentamos dos casos de STIG tratados exitosamente con fotocoagulación láser de anastomosis intergemelares, en los cuales los vasos comunicantes no cruzaban la membrana divisoria.

Rapid initiation of fetal therapy services with a system of learner-centred training under proctorship: the National University Hospital (Singapore) experience

<p><b>INTRODUCTION</b>Management of complicated monochorionic twins and certain intrauterine structural anomalies is a pressing challenge in communities that still lack advanced fetal therapy. We describe our efforts to rapidly initiate selective feticide using radiofrequency ablation (RFA) and selective fetoscopic laser photocoagulation (SFLP) for twin-to-twin transfusion syndrome (TTTS), and present the latter as a potential model for aspiring fetal therapy units.</p><p><b>METHODS</b>Five pregnancies with fetal complications were identified for RFA. Three pregnancies with Stage II TTTS were selected for SFLP. While RFA techniques utilising ultrasonography skills were quickly mastered, SFLP required stepwise technical learning with an overseas-based proctor, who provided real-time hands-off supervision.</p><p><b>RESULTS</b>All co-twins were live-born following selective feticide; one singleton pregnancy was lost. Fetoscopy techniques were learned in a stepwise manner and procedures were performed by a novice team of surgeons under proctorship. Dichorionisation was completed in only one patient. Five of six twins were live-born near term. One pregnancy developed twin anaemia-polycythaemia sequence, while another was complicated by co-twin demise.</p><p><b>DISCUSSION</b>Proctor-supervised directed learning facilitated the rapid provision of basic fetal therapy services by our unit. While traditional apprenticeship is important for building individual expertise, this system is complementary and may benefit other small units committed to providing these services.</p>

Monochorionic diamniotic twin pregnancy with selective IUGR type 2: fetoscopic and sonographic findings of ominous prognosis / Gestación gemelar monocorial complicada con RCIU selectiva tipo 2: hallazgos ecográficos y fetoscópicos de mal pronóstico

A woman with a monochorionic diamniotic twin pregnancy was referred to our Centre due to a discrepancy in size between the foetuses. She was 17 weeks' pregnant when the diagnosis of selective IUGR type 2 was made and the laser ablation of the placental anastomoses was performed. During the fetoscopy, we identified an arterio-venous anastomosis with bidirectional flow, which is atypical in this type of anastomoses (unidirectionals) and could represent a sign of ominous prognosis for the restricted twin.

Presentamos el caso de una gestante de 17 semanas con una gestación gemelar monocoriónica biamniótica complicada con RCIU selectivo tipo 2 que requirió, de acuerdo a criterios ecográficos, ablación quirúrgica láser de las anastomosis placentarias. Se identificó durante la fetoscopia una anastomosis arterio-venosa que mostraba flujo bidireccional lo cual es sumamente inusual en este tipo de anastomosis, no reportado antes en la literatura, constituyendo así un signo de mal pronóstico para el gemelo pequeño.

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.