Fotocoagulación láser guiada por ultrasonido para el tratamiento del secuestro broncopulmonar fetal. Reporte de dos casos exitosos en Venezuela / Ultrasound-guided laser photocoagulation for the treatment of fetal bronchopulmonary sequestration. Report of two successful cases in Venezuela

El secuestro broncopulmonar es una malformación del aparato respiratorio que consiste en tejido bronquial y pulmonar no funcionante, separado del árbol tráqueo-bronquial y alimentado por un vaso sanguíneo proveniente de la circulación sistémica. La incidencia es de 1 por cada 5000 nacimientos, comprende entre 0,15 % y 6,45 % de las patologías pulmonares. El pronóstico es, generalmente, favorable, reportándose regresión espontánea de la lesión en 50 % a 75 % de los pacientes. Puede ocasionar efecto de masa, comprimiendo el corazón y el pulmón hasta generar cambios hemodinámicos y falla cardíaca. Hay múltiples procedimientos para el tratamiento y manejo, principalmente en casos de gran tamaño y fetos hidrópicos, para mejorar el pronóstico perinatal. Se presentan los dos primeros casos de secuestro broncopulmonar tratados en Venezuela mediante fotocoagulación láser del vaso nutricio y su evolución perinatal, con sobrevida del 100 % y sin ninguna complicación registrada en el periodo perinatal(AU)

Bronchopulmonary sequestration is a malformation of the respiratory system consisting of non-functioning bronchial and pulmonary tissue, separated from the tracheo-bronchial tree and fed by a blood vessel from the systemic circulation. The incidence is 1 in 5000 births, ranging from 0.15% to 6.45% of pulmonary pathologies. The prognosis is generally favorable, with spontaneous regression of the lesion reported in 50% to 75% of patients. It can cause mass effect, compressing the heart and lung to the point of generating hemodynamic changes and heart failure. There are multiple procedures for treatment and management, mainly in large cases and hydropic fetuses, to improve perinatal prognosis. We present the first two cases of bronchopulmonary sequestration treated in Venezuela by laser photocoagulation of the nutrient vessel and their perinatal evolution, with 100% survival and without any complications recorded in the perinatal period(AU)

Intrauterine Correction of Fetal Myelomeningocele Through Minihysterotomy.

Spina bifida is the most common congenital central nervous system anomaly, resulting in lifelong neurologic, urinary, motor, and bowel disability.1 Its most frequent form is myelomeningocele, characterized by spinal cord extrusion into a sac filled with cerebrospinal fluid.1 We report the case of a 28-year-old pregnant female with no comorbidities. At 16 weeks of pregnancy, fetal ultrasound presented ventriculomegaly, cerebellar herniation, and lumbar myelomeningocele. At 22 weeks, intrauterine surgical correction was performed (Video 1). A minihysterotomy spanning approximately 3 cm was performed. The defect was opened, and the neural placode was dissected and released. This was followed by the isolation of the peripheric dura, which was molded into a tube and closed with watertight suture. Finally, the minihysterotomy was sutured and the skin was closed. The pregnancy followed its course with no complications, and the child was born at term with the lesion closed and no necessity of intensive care. Recent studies have demonstrated that infants who undergo open in utero myelomeningocele repair have better neurologic outcomes than those who are treated after birth.1,2 However, maternal morbidity is nonnegligible with the classical open surgery.2 Peralta et al2 propose a modification of the classic 6.0- to 8.0-cm hysterotomy in which the same multilayer correction of the spinal defect is performed through a 2.5- to 3.5-cm hysterotomy. This modification, called minihysterotomy, has been successfully performed outside of its creation center and was associated with reduced risks of preterm delivery and maternal, fetal, and neonatal complications.2,3.

The Management of Acardiac Twinning: Twin Reverse Arterial Perfusion Sequence - An International Survey.

INTRODUCTION: The optimal approach and therapy method for the acardiac twin with a reverse arterial perfusion sequence has not yet been established. The aim of this study was to determine the clinical practice patterns among international fetal therapy units in their management of these cases. METHODS: A survey was sent to fetal centers across the world via email between December 2020 and December 2021. RESULTS: Responses were obtained from 77% contacted centers. The most frequent ultrasound variables used in the evaluation of twin reverse arterial perfusion sequence include echocardiographic assessment of the pump twin and umbilical artery Doppler waveforms in the acardiac and pump twins, in 90% and 80% of the centers, respectively. Most centers in Europe and Latin America propose an in utero intervention in all cases. Most centers in Europe and Latin America prefer interstitial laser ablation, whereas radiofrequency ablation (RFA) is preferred in North America. The earliest gestational age for an intervention is on mean 13 weeks in Europe, which is earlier than the other geographic areas (p = 0.001). CONCLUSIONS: Most centers agreed that antenatal evaluation should include echocardiography along with the UA Doppler waveform measurements, and the most frequently used interventions were interstitial laser ablation or RFA at a median between 14 and 26 weeks.

Prevalencia de patologías fetales que pueden beneficiarse de intervenciones intrauterinas en un hospital de tercer nivel del Occidente de México / Prevalence of fetal pathologies that can benefit from intrauterine interventions in a third level hospital in western Mexico

Resumen OBJETIVO: Determinar la prevalencia de afecciones susceptibles de ser tratadas mediante un procedimiento intrauterino en una unidad de Medicina Materno Fetal de un hospital de tercer nivel del Occidente de México, en un periodo de nueve años. MATERIALES Y MÉTODOS: Estudio transversal y descriptivo, con revisión de los reportes de ultrasonidos obstétricos practicados en la Unidad de Medicina Materno Fetal del Hospital Civil de Guadalajara Dr. Juan I. Menchaca del 2013 al 2021, con selección de los casos de pacientes con diagnóstico de alguna afectación susceptible de ser intervenida de manera intrauterina. RESULTADOS: Durante el periodo de estudio se practicaron y registraron 103,721 ultrasonidos obstétricos, de éstos se integraron al estudio aquí publicado 257 pacientes con diagnóstico de alguna afectación susceptible de ser intervenida in útero. La prevalencia de afectaciones con posibilidad de ser intervenidas por vía intrauterina fue del 0.47% de la totalidad de pacientes valoradas. La media de edad de las pacientes fue de 24.6 años; 162 (63%) multigestas y 95 (37%) primigestas. Embarazos únicos 193 (75%) y 64 (25%) múltiples. Las semanas promedio de gestación al diagnóstico de la afectación fueron 25.6. CONCLUSIONES: En este ensayo se estimó una prevalencia de 0.47 padecimientos que pueden ser intervenidos in útero, dejando de lado muchas otros en los que los estudios no han demostrado beneficio de una cirugía fetal, ni los beneficios superan los riesgos, si se practica la cirugía de manera prenatal o posnatal.

Abstract OBJECTIVE: To determine the prevalence of conditions amenable to treatment by an intrauterine procedure in a Maternal-Fetal Medicine unit of a tertiary hospital in western Mexico over a nine-year period. MATERIALS AND METHODS: Cross-sectional and descriptive study, with review of obstetric ultrasound reports performed in the Maternal-Fetal Medicine Unit of the Civil Hospital of Guadalajara Dr. Juan I. Menchaca from 2013 to 2021, with selection of cases of patients diagnosed with any condition susceptible to intrauterine intervention. RESULTS: During the study period 103,721 obstetric ultrasounds were performed and recorded, of which 257 patients were included in the study published here with a diagnosis of a condition that could be treated in utero. The prevalence of conditions that could be treated in utero was 0.47% of all patients assessed. The mean age of the patients was 24.6 years; 162 (63%) were multigestational and 95 (37%) primigravid. Singleton pregnancies 193 (75%) and 64 (25%) multiple pregnancies. Mean weeks of gestation at diagnosis of involvement was 25.6. CONCLUSIONS: This trial estimated a prevalence of 0.47% of conditions that can be intervened in utero, leaving aside many others where studies have not demonstrated benefit of fetal surgery, nor do the benefits outweigh the risks, whether surgery is performed prenatally or postnatally.

Intrauterine transfusion of a hydropic fetus with anemia due to a giant chorioangioma: A case report.

Giant chorioangiomas are a potentially life-threatening condition that may require intrauterine therapy. We describe a case of a large chorioangioma (>4cm) diagnosed at 30 weeks of gestation causing severe fetal anemia and hydrops. An intrauterine blood transfusion was performed at 31 weeks with reversal of the anemia and hydrops. The neonate was born at 37 weeks showing respiratory distress syndrome that required neonatal intensive care unit admission but was discharged at 30 days of life. Further evaluation at two months of age showed no signs of abnormal neurodevelopment. When timely indicated, intrauterine transfusion of a hydropic fetus with anemia due to a giant chorioangioma is a potentially life-saving therapy that shows good neurodevelopment of the surviving fetus.

Critical Ebstein's anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique.

We report an innovative treatment strategy for fetal Ebstein's anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.

Prenatal Repair of Spina Bifida: A 2-Center Experience with Open Intrauterine Neurosurgery in Chile.

OBJECTIVE: To report the experience with prenatal repair of open spina bifida (OSB) from 2 centers in Chile. METHODS: Women with a second-trimester fetus with OSB were offered intrauterine neurosurgical repair following the protocol from the Management of Myelomeningocele Study (MOMS) trial. Pediatric follow-up with infants reaching 12 and 30 months of life was also reviewed. RESULTS: Fifty-eight fetuses with OSB underwent intrauterine repair at an average (±SD) gestational age of 24.8 ± 0.9 weeks. There were 3 (5.1%) intrauterine deaths. The average gestational age at delivery of the remaining 55 cases was 33.3 ± 3.6 weeks, and the average birth weight was 2,172 ± 751 g. Delivery before 30 weeks occurred in 11 cases (20.0%). Two (3.6%) neonatal deaths (<28 days) occurred. At 12 months, a ventriculoperitoneal shunt or an endoscopic third ventriculostomy was required in 25% of the cases. At 30 months, 72.4% of the infants were able to walk. DISCUSSION: Prenatal neurosurgical repair of OSB is a complex and challenging intervention. Major complications include perinatal death and severe prematurity. No major maternal complications occurred in our series. A reduction in the need for cerebrospinal fluid diversion and an improved ability to walk seem to be the greatest long-term advantages of this procedure.

Cardiopatías congénitas, diagnóstico y manejo prenatal / Congenital heart disease, prenatal diagnosis and management

Dentro de las malformaciones congénitas, las cardiopatías son las anomalías más frecuentes y se asocian a una elevada morbimortalidad perinatal y a largo plazo. El objetivo de esta actualización es revisar la tasa de detección prenatal, las características del tamizaje a lo largo del embarazo, tanto en el primero como en el segundo trimestre, las indicaciones de ecocardiografía avanzada, y establecer un algoritmo de manejo ante el diagnóstico prenatal de una cardiopatía congénita. Se discutirán los estudios invasivos y no invasivos que pueden realizarse y el seguimiento obstétrico. Finalmente, se revisarán las características principales de la terapia fetal en anomalías cardíacas, tanto intervencionismo cardíaco como el tratamiento intrauterino de las arritmias.

Among congenital malformations, heart defects are the most common type of anomaly, and these are associated with a high perinatal, long-term morbidity and mortality. The objective of this update was to review the rate of prenatal detection, screening characteristics throughout the pregnancy, in both the first and second trimesters, indications for advanced echocardiography, and to establish a management algorithm in case of prenatal diagnosis of a congenital heart disease. Potential invasive and non-invasive tests and obstetric follow-up will be discussed here. Finally, the main characteristics of fetal therapy in heart anomalies will be reviewed, both cardiac interventions and intrauterine treatment of arrhythmias.

Congenital heart disease, prenatal diagnosis and management. / Cardiopatías congénitas, diagnóstico y manejo prenatal.

Among congenital malformations, heart defects are the most common type of anomaly, and these are associated with a high perinatal, longterm morbidity and mortality. The objective of this update was to review the rate of prenatal detection, screening characteristics throughout the pregnancy, in both the first and second trimesters, indications for advanced echocardiography, and to establish a management algorithm in case of prenatal diagnosis of a congenital heart disease. Potential invasive and non-invasive tests and obstetric follow-up will be discussed here. Finally, the main characteristics of fetal therapy in heart anomalies will be reviewed, both cardiac interventions and intrauterine treatment of arrhythmias.

Dentro de las malformaciones congénitas, las cardiopatías son las anomalías más frecuentes y se asocian a una elevada morbimortalidad perinatal y a largo plazo. El objetivo de esta actualización es revisar la tasa de detección prenatal, las características del tamizaje a lo largo del embarazo, tanto en el primero como en el segundo trimestre, las indicaciones de ecocardiografía avanzada, y establecer un algoritmo de manejo ante el diagnóstico prenatal de una cardiopatía congénita. Se discutirán los estudios invasivos y no invasivos que pueden realizarse y el seguimiento obstétrico. Finalmente, se revisarán las características principales de la terapia fetal en anomalías cardíacas, tanto intervencionismo cardíaco como el tratamiento intrauterino de las arritmias.

EXIT (ex utero intrapartum treatment) surgery for the management of fetal airway obstruction: A systematic review of the literature.

PURPOSE: To provide a comprehensive overview of the perinatal and maternal outcomes of fetuses undergoing EXIT surgery for the management of fetal airway obstruction secondary to cervical or oral tumors. METHODS: A comprehensive search from inception to September 2018 was conducted on databases including MEDLINE, EMBASE, Cochrane Library and LILACS. All studies that reported an EXIT surgery in singleton were considered eligible. A descriptive analysis was performed. RESULTS: Out of the 250 full-text study reports, 120 articles reporting 235 cases of EXIT surgery were included. EXIT surgery was performed at 35.1 weeks of gestation on average. The most frequent diagnosis was teratoma (46.4%, n = 109/235). There were 13 adverse maternal events, and the most frequent one was postpartum hemorrhage (4.7%, n = 11/235). No maternal death was reported. Fetal and neonatal death occurred in 17% (40/235) of the cases. There were 29 adverse fetal events (12.2%), and the most frequent one was the failure of intubation or tracheostomy (3.4%, n = 8/235). CONCLUSION: EXIT surgery could be considered for the management of an oral or cervical tumor that's highly suspicious of blocking the fetal airway. This systematic review reports that EXIT surgery poses substantial risks of maternal and fetal adverse events, including neonatal death. LEVEL OF EVIDENCE: IV case series with no comparison group.

North American Fetal Therapy Network: Timing of and indications for delivery following laser ablation for twin-twin transfusion syndrome.

Background: Despite improvements in fetal survival for pregnancies affected by twin-twin transfusion syndrome since the introduction of laser photocoagulation, prematurity remains a major source of neonatal morbidity and mortality. Objective: To investigate the indications and factors influencing the timing of delivery following laser treatment, we collected delivery information regarding twin-twin transfusion syndrome cases in a large multicenter cohort. Study Design: Eleven North American Fetal Therapy Network (NAFTNet) centers conducted a retrospective review of twin-twin transfusion syndrome patients who underwent laser photocoagulation. Clinical, demographic and ultrasound variables including twin-twin transfusion syndrome stage, and gestational age at treatment and delivery were recorded. Primary and secondary maternal and fetal indications for delivery were identified. Univariate analysis was used to select candidate variables with significant correlation with latency and GA at delivery. Multivariable Cox regression with competing risk analysis was utilized to determine the independent associations. Results: A total of 847 pregnancies were analyzed. After laser, the average latency to delivery was 10.11 ± 4.8 weeks and the mean gestational age at delivery was 30.7 ± 4.5 weeks. Primary maternal indications for delivery comprised 79% of cases. The leading indications included spontaneous labor (46.8%), premature rupture of membranes (17.1%), and placental abruption (8.4%). Primary fetal indications accounted for 21% of cases and the most frequent indications included donor non-reassuring status (20.5%), abnormal donor Dopplers (15.1%), and donor growth restriction (14.5%). The most common secondary indications for delivery were premature rupture of membranes, spontaneous labor and donor growth restriction. Multivariate modeling found gestational age at diagnosis, stage, history of prior amnioreduction, cerclage, interwin membrane disruption, procedure complications and chorioamniotic membrane separation as predictors for both gestational age at delivery and latency. Conclusion: Premature delivery after laser therapy for twin-twin transfusion syndrome is primarily due to spontaneous labor, preterm premature rupture of membranes and non-reassuring status of the donor fetus. Placental abruption was found to be a frequent complication resulting in early delivery. Future research should be directed toward the goal of prolonging gestation after laser photocoagulation to further reduce morbidity and mortality associated with twin-twin transfusion syndrome.

Polidocanol versus alcohol absoluto como sustancias esclerosantes en modelo experimental animal / Polidocanol versus absolute alcohol as sclerosing substances in experimental animal model

Introducción: La esclerosis se caracteriza por la destrucción celular epitelial con apoptosis, inflamación estéril, fibrosis irreversible y disminución de la masa tisular por retracción con subsecuente obliteración de la luz de los quistes y/o vasos sanguíneos. Existen diferentes sustancias esclerosantes que pueden ser utilizados. Aun así, actualmente no se han definido pautas claras en el manejo con sustancias esclerosantes. Esto puede considerarse como un tratamiento poco invasivo y efectivo. Objetivo: Determinar la eficacia y seguridad del polidocanol versus alcohol absoluto como sustancias esclerosantes en modelo experimental animal. Materiales y Métodos: El estudio se realizó con 34 ratas, distribuidas en forma aleatoria en 3 grupos. Grupo A con 16 ratas a las cuales se les introdujo 1 ml de polidocanol intravesical. Grupo B con 16 ratas a las que se les introdujo 1 ml de alcohol absoluto intravesical. Grupo C (control) con 2 ratas sin intervención. Las 2 ratas del grupo control fueron sacrificadas con sobredosis de halotano. Igualmente las ratas de cada grupo experimental, que fueron sacrificadas en pares, escogidos al azar, a las 12, 24, 48, 72, 96 y 120 horas post aplicación respectivamente. Luego se realizó el estudio anatomopatológico. Se verificó la profundidad y extensión de la esclerosis y se adjudicó puntaje según escala numérica previamente diseñada. Se determinó y comparó la eficacia y seguridad de ambas sustancias dependiendo de la extensión y profundidad de la esclerosis según el tiempo de acción con regresión logística y test exacto de Fisher. Resultados: Ambas sustancias mostraron similar efectividad al producir esclerosis con retracción de la pared vesical en 100% de los casos después de 24 horas de aplicación. El polidocanol mostró acción biológica progresiva encontrándose correlación entre el tiempo de acción y la profundidad de la esclerosis mediante el análisis de regresión logística con coeficiente de correlación de 0.75 e índice de probabilidad: 0.00183 (p < 0.05). El alcohol absoluto produjo esclerosis profunda inmediata, sin correlación con el tiempo de acción, con coeficiente de correlación de - 0.0465 con un índice de probabilidad de 0.864 (p > 0.05). Se estimó la seguridad de las sustancias según el hallazgo de esclerosis transmural y lesión de órganos vecinos. En ningún caso con polidocanol (0%) hubo paso transmural ni lesión de órganos vecinos mientras que en 83,33% de los casos del alcohol absoluto se presentó esclerosis transmural con lesión de órganos adyacentes en 50% de los casos (Test de Fisher's p < 0.0001 extremadamente significativo). Ninguna de las 2 sustancias produjo lesiones de órganos a distancia. Conclusiones: Tanto el alcohol como el polidocanol son efectivos para generar esclerosis. La esclerosis con polidocanol impresiona ser segura por quedar limitada a la vejiga. La escleroterapia con alcohol no parece ser segura porque genera esclerosis transmural y lesión de órganos contiguos. La diseminación de las sustancias a través de vasos sanguíneos o linfáticos con lesiones de órganos distantes no ocurrió en ningún caso y parece improbable ya que los esclerosantes producen la oclusión de estos vasos.

Introduction: Sclerosis is characterized by epithelial cell destruction with apoptosis, sterile inflammation, irreversible fibrosis and decreased tissue mass by retraction with subsequent obliteration of the lumen of the cysts and / or blood vessels. There are different sclerosing substances that can be used. Even so, clear guidelines have not yet been defined in the management of sclerosing substances. This can be considered as a minimally invasive and effective treatment. Objective: To determine the efficacy and safety of polidocanol versus absolute alcohol as sclerosing substances in an experimental model. Materials and Methods: The study was conducted with 34 rats, randomly distributed in 3 groups. Group A with 16 rats to which 1 ml of intravesical polidocanol was introduced. Group B with 16 rats to which 1 ml of intravesical absolute alcohol was introduced. Group C (control) with 2 rats without intervention. The 2 rats of the control group were sacrificed with halothane overdose. Also the rats of each experimental group, which were sacrificed in pairs, chosen at random, at 12, 24, 48, 72, 96 and 120 hours after application respectively. Then the anatomopathological study was carried out. The depth and extension of the sclerosis was verified and a score was awarded according to a previously designed numerical scale. The efficacy and safety of both substances were determined and compared depending on the extension and depth of the sclerosis according to the time of action with logistic regression and Fisher's exact test. Results: Both substances showed similar effectiveness in producing sclerosis with retraction of the bladder wall in 100% of the cases after 24 hours of application. The polidocanol showed progressive biological action, finding a correlation between the time of action and the depth of the sclerosis by means of the logistic regression analysis with a correlation coefficient of 0.75 and a probability index of 0.00183 (p <0.05). Absolute alcohol produced immediate deep sclerosis, without correlation with the time of action, with a correlation coefficient of - 0.0465 with a probability index of 0.864 (p> 0.05). The safety of the substances was estimated according to the finding of transmural sclerosis and injury to close organs. In no case with polidocanol (0%) there was transmural passage or injury of close organs, while in 83.33% of cases of absolute alcohol, transmural sclerosis with lesion of adjacent organs occurred in 50% of the cases (Fisher's Test p <0.0001 extremely significant). None of the 2 substances produced remote organ injuries. Conclusions: Both alcohol and polidocanol are effective in generating sclerosis. Sclerosis with polidocanol is safe because it is limited to the bladder. Sclerotherapy with alcohol does not appear to be safe because it generates transmural sclerosis and injury to contiguous organs. The dissemination of the substances through blood or lymphatic vessels with lesions of distant organs did not occur in any case and seems unlikely since the sclerosants produce the occlusion of these vessels

Balloon removal after fetoscopic endoluminal tracheal occlusion for congenital diaphragmatic hernia.

BACKGROUND: Isolated congenital diaphragmatic hernia defect allows viscera to herniate into the chest, competing for space with the developing lungs. At birth, pulmonary hypoplasia leads to respiratory insufficiency and persistent pulmonary hypertension that is lethal in up to 30% of patients. Antenatal measurement of lung size and liver herniation can predict survival after birth. Prenatal intervention aims at stimulating lung development, clinically achieved by percutaneous fetal endoscopic tracheal occlusion under local anesthesia. This in utero treatment requires a second intervention to reestablish the airway, either before birth or at delivery. OBJECTIVE: To describe our experience with in utero endotracheal balloon removal. MATERIALS AND METHODS: This is a retrospective analysis of prospectively collected data on consecutive patients with congenital diaphragmatic hernia treated in utero by fetal endoscopic tracheal occlusion from 3 centers. Maternal and pregnancy-associated variables were retrieved. Balloon removal attempts were categorized as elective or emergency and by technique (in utero: ultrasound-guided puncture; fetoscopy; ex utero: on placental circulation or postnatal tracheoscopy). RESULTS: We performed 351 balloon insertions during a 144-month period. In 9 cases removal was attempted outside fetal endoscopic tracheal occlusion centers, 3 of which were deemed impossible and led to neonatal death. We attempted 302 in-house balloon removals in 292 fetuses (217 elective [71.8%], 85 emergency [28.2%]) at 33.4 ± 0.1 weeks (range: 28.9-37.1), with a mean interval to delivery of 16.6 ± 0.8 days (0-85). Primary attempt was by fetoscopy in 196 (67.1%), by ultrasound-guided puncture in 62 (21.2%), by tracheoscopy on placental circulation in 30 (10.3%), and postnatal tracheoscopy in 4 cases (1.4%); a second attempt was required in 10 (3.4%) cases. Each center had different preferences for primary technique selection. In elective removals, we found no differences in the interval to delivery between fetoscopic and ultrasound-guided puncture removals. Difficulties during fetoscopic removal led to the development of a stylet to puncture the balloon, leading to shorter operating time and easier reestablishment of airways. CONCLUSION: In these fetal treatment centers, the balloon could always be removed successfully. In 90% this was in utero, with the use of fetoscopy preferred over ultrasound-guided puncture. Ex utero removal was a fall-back procedure. In utero removal does not seem to precipitate immediate membrane rupture, labor, or delivery, although the design of the study did not allow for a formal conclusion. For fetoscopic removals, the introduction of a stylet facilitated retrieval. Successful removal may rely on a permanently prepared team with expertise in all possible techniques.

Secuencia de perfusion arterial reversa (TRAP) / Twin Reversed Arterial Perfusion, TRAP

Resumen:Considerada como una complicación exclusiva de gestaciones múltiples monocoriales, la secuencia de perfusión arterial reversa se caracteriza por la presencia de un feto malformado que tiene un corazón rudimentario o ausente, con una perfusión desde el gemelo estructuralmente normal hacia el feto acárdico y una mortalidad del 100% en el feto acárdico y el 50% en el feto sano. Se reporta un caso clínico de una paciente de 15 años con embarazo gemelar monocorial de 27 semanas, referida al Hospital San Juan de Dios, en donde se le dio seguimiento diario con ultrasonido y se documentó el patrón ultrasonógrafico anormal de sumación-colisión. A los 7 días de ingreso se decide la interrupción del embarazo por sospecha de coriamnioitis, naciendo un producto viable y otro polimalformado.

Abstract:Considered an exclusive complication of monochorionic gestations, the TRAP sequence is characterize by the presence of a malformed fetus with a rudimentary or absent heart, in which there is an perfusion from the structurally normal twin to the acardiac fetus and a 100% mortality in the acardiac fetus and 50% in the healthy one. We present a clinical case of a 15 years old patient with monochorionic twin pregnancy of 27 weeks, referred to Hospital San Juan de Dios, where daily monitoring with ultrasound was done and an abnormal collision-summantion pattern was documented and reported. After 7 days of admission chorioamnionitis was suspected and termination of the pregnancy was decided resulting in a viable product and another with multiple malformations.

Terapéutica fetal no invasiva / Non-invasive fetal therapy

La terapia farmacológica fetal puede definirse como cualquier tratamiento prenatal administrado a la madre con la indicación primaria para mejorar los resultados perinatales o a largo plazo para el feto o el recién nacido. Esta revisión proporciona una actualización de los tratamientos farmacológicos dirigidos exclusivamente al feto con anomalías. Las anomalías fetales con posibilidad de farmacoterapia prenatal constituyen un grupo heterogéneo de condiciones estructurales, tales como las anomalías del cerebro y de la columna, prevención de la enfermedad hialina del pulmón, anomalías endocrinas y metabólicas, incluyendo la malformación adenomatoidea quística congénita (CCAM), hiperplasia suprarrenal congénita, bloqueo congénito de corazón, taquiarritmias fetales, errores innatos del metabolismo, trastornos de la tiroides fetal y polihidramnios. Hasta la fecha, la mayor parte de la farmacoterapia para anomalías fetales ha sido evaluada en estudios retrospectivos, no controlados. El camino a seguir será con un enfoque basado en la evidencia para intervenciones farmacológicas prenatales.

Fetal pharmacologic therapy can be defined as any prenatal treatment administered to the mother with the primary indication of improving perinatal or long-term outcomes for the fetus and the newborn. This review provides an update on the pharmacological therapies for the fetus with anomalies. Fetal anomalies targeted with prenatal pharmacotherapy are a heterogeneous group of structural, endocrine, and metabolic conditions, including brain and spine anomaly, hyaline membrane disease prevention, congenital cystic adenomatoid malformation (CCAM), congenital adrenal hyperplasia, congenital heart block, fetal tachyarrhythmia, inborn errors of metabolism, fetal thyroid disorders, and polyhydramnios. To date, most of the pharmacotherapy for fetal anomalies has been evaluated only in retrospective, uncontrolled studies. Future evaluation will be determined on evidence-based approach to prenatal pharmacological interventions.

Chorioamniotic membrane separation and preterm premature rupture of membranes complicating in utero myelomeningocele repair.

BACKGROUND: Since the results of the Management of Myelomeningocele Study were published, maternal-fetal surgery for the in utero treatment of spina bifida has become accepted as a standard of care alternative. Despite promise with fetal management of myelomeningocele repair, there are significant complications to consider. Chorioamniotic membrane separation and preterm premature rupture of membranes are known complications of invasive fetal procedures. Despite their relative frequency associated with fetal procedures, few data exist regarding risk factors that may be attributed to their occurrence or the natural history of pregnancies that are affected with chorionic membrane separation or preterm premature rupture of membranes related to the procedure. OBJECTIVE: The objective of this study was to review chorioamniotic membrane separation and preterm premature rupture of membranes in a cohort of patients undergoing fetal management of myelomeningocele repair including identification of risk factors and outcomes. STUDY DESIGN: This was a retrospective review of patients undergoing fetal management of myelomeningocele repair and subsequent delivery from January 2011 through December 2013 at 1 institution. Patients were identified through the institutional fetal management of myelomeningocele repair database and chart review was performed. Perioperative factors and outcomes among patients with chorioamniotic membrane separation and preterm premature rupture of membranes were compared to those without. Risk factors associated with the development of chorioamniotic membrane separation and preterm premature rupture of membranes were determined. RESULTS: A total of 88 patients underwent fetal management of myelomeningocele repair and subsequently delivered during the study period. In all, 21 patients (23.9%) were diagnosed with chorioamniotic membrane separation by ultrasound and preterm premature rupture of membranes occurred in 27 (30.7%). Among the chorioamniotic membrane separation patients, 10 (47.6%) were diagnosed with global chorioamniotic membrane separation and 11 (52.4%) with local chorioamniotic membrane separation. Earlier gestational age at the time of fetal surgery was a significant risk factor for the development of chorioamniotic membrane separation (P = .01) and preterm premature rupture of membranes (P < 0.0001). Chorioamniotic membrane separation was significantly associated with preterm premature rupture of membranes (59.1% vs 21.2%, P = .008) and earlier gestational age at delivery (32.1 ± 4.2 vs 34.4 ± 3.5 weeks, P = .01). The average number of days from chorioamniotic membrane separation to preterm premature rupture of membranes was 11.0 ± 10.1 and from chorioamniotic membrane separation to delivery was 31.0 ± 22.5. The mean time interval between fetal management of myelomeningocele repair and preterm premature rupture of membranes was 47.9 days. Mean latency period from preterm premature rupture of membranes to delivery was 25 days. Gestational age at delivery was significantly lower in patients with preterm premature rupture of membranes (31.6 ± 3.4 vs 34.9 ± 3.5 weeks, P = .0001). Using logistic regression analysis, nulliparity, gestational age at fetal management of myelomeningocele repair, and membrane separation remained significant risk factors for preterm premature rupture of membranes. CONCLUSION: Chorioamniotic membrane separation after fetal management of myelomeningocele repair is a significant risk factor for subsequent development of preterm premature rupture of membranes and preterm delivery. Fetal management of myelomeningocele repair <23 weeks is associated with higher rates of preterm premature rupture of membranes and chorioamniotic membrane separation. Therefore fetal management of myelomeningocele repair should be deferred until ≥23 weeks to mitigate these complications. Nulliparity also appears to increase the risk for preterm premature rupture of membranes.

Restricción selectiva del crecimiento intrauterino en la gestación gemelar monocorial: diagnóstico y manejo / Selective intrauterine growth restriction in monochorionic-diamniotic twins: diagnosis and management

La Restricción Selectiva del Crecimiento Intrauterino se produce en 10 a 19% de los gemelos monocoriónicos, y se asocia con un aumento importante de la mortalidad y la morbilidad perinatal. La evolución clínica está dada en gran parte por la presencia de anastomosis vaculares y la distribución placentaria asimétrica. Se propuso una clasificación en tres tipos según el Doppler de la arteria umbilical del gemelo más pequeño, la que sé que se correlaciona con distintos comportamientos clínicos y características de la placenta, lo que ayuda en el asesoramiento y conducta obstétrica. Los del tipo I tienen un pronóstico favorable por el resultado perinatal satisfactorio por lo que se recomienda un seguimiento cercano por ecografía y Doppler. Los de tipo II tienen un mal pronóstico y el tipo III un pronóstico intermedio e impredecible. En estos dos últimos se puede realizar un manejo expectante hasta que se observe deterioro del feto pequeño, considerando la posibilidad de la coagulación con láser o la oclusión del cordón (feticidio selectivo). Ambas conductas aparentemente aumentan las posibilidades de supervivencia del feto de peso normal.

Selective intrauterine growth restriction occurs in 10-19% of monochorionic twins, and is associated with increased mortality important and perinatal morbidity. The clinical course is given largely by the presence of placental vascular anastomosis and asymmetric distribution. It classified into three types according to the proposed Doppler umbilical artery smaller twin, the one that correlates with different clinical behaviors and characteristics of the placenta, which helps in counseling and obstetric conduct. The type I have a favorable prognosis for satisfactory perinatal outcome so closely monitored by ultrasound and Doppler is recommended. The type II has a poor prognosis and type III intermediate and unpredictable prognosis. In these last two you can make an expectant management until deterioration of the small fetus is observed, considering laser coagulation or cord occlusion (selective feticide). Both behaviors appear to increase the chances of survival of the fetus of normal weight.

Fetoscopic single-layer repair of open spina bifida using a cellulose patch: preliminary clinical experience.

OBJECTIVE: To report our preliminary clinical experience in the antenatal correction of open spina bifida (OSB) using a fetoscopic approach and a simplified closure technique. METHODS: Four fetuses with lumbar-sacral defects were operated in utero from 25 to 27 weeks. Surgeries were performed percutaneously under general anesthesia using three trocars and partial carbon dioxide insufflation. After dissection of the neural placode, the surrounding skin was closed over a cellulose patch using a single continuous stitch. RESULTS: Surgical closure was successful in three of the four cases. All successful cases showed improvement of the hindbrain herniation and no neonatal neurosurgical repair was required in two cases. Delivery occurred between 31 and 33 weeks, and no fetal or neonatal deaths occurred. Ventriculoperitoneal shunting was not needed in two out of the 3 successful cases. CONCLUSIONS: Our preliminary experience suggests that definitive fetoscopic repair of OSB is feasible using our innovative surgical technique. A phase I trial for the fetoscopic correction of OSB with this technique is currently being conducted.

Selective fetoscopic laser photocoagulation of superficial placental anastomoses for the treatment of severe twin-twin transfusion syndrome

OBJECTIVE: To report our initial institutional experience with fetoscopic laser photocoagulation of placental anastomoses in severe twin-twin transfusion syndrome using a 1.0 mm endoscope. METHODS: Between July 2006 and June 2008, 19 monochorionic diamniotic twin pregnancies complicated by severe TTTS (Quintero stages III and IV) underwent fetoscopic laser therapy. Perinatal data were prospectively collected and compared according to the Quintero stages. RESULTS: Nine patients were classified as stage III and ten as stage IV. The Mean gestational ages at diagnosis and procedure were 20 (range: 17-25) and 22.0 (range: 19.0-26.0) weeks, respectively, with no statistical difference between the two groups. Preterm premature rupture of the membranes occurred in two cases (10.5 percent), and spontaneous preterm delivery in eight (42.1 percent). Overall mean gestational age at delivery was 32.1 (range: 26.0-38.0) weeks. Prematurity was more severe in stage IV patients (p<0.01). Among all cases, the overall survival rate was 52.6 percent, and the percentages of pregnancies with survival of both babies and at least one twin were 26.3 percent and 78.9 percent, respectively. In the case of stage III patients, the overall survival rate was 61.1 percent. Of the stage III pregnancies, 33.3 percent resulted in both babies surviving, and 88.9 percent of these pregnancies resulted in at least one surviving twin. For stage IV, as the corresponding statistics were 45.0 percent, 20.0 percent and 70.0 percent respectively. CONCLUSIONS: Our initial institutional experience with 1.0 mm fetoscopic laser therapy for severe TTTS showed results similar to those reported in the literature for larger endoscopes.

To shunt or not to shunt a pulmonary adenomatoid cystic malformation after 33 weeks of gestation: a case report / Derivar ou não uma malformação adenomatóide cística do pulmão após 33 semanas de gestação: relato de caso

CONTEXT: Macrocystic adenomatoid malformation of the lung can cause severe mediastinal shift, hydrops and polyhydramnios, thereby increasing the risk of perinatal deaths. After 33 weeks of gestation, repeated puncturing of the cyst is recommended. We present a case in which a cyst-amniotic shunt was placed instead of performing this procedure. CASE REPORT: A cyst-amniotic shunt was placed at 33 weeks of gestation because of a large macrocystic adenomatoid malformation of the lung associated with severe mediastinal shift and polyhydramnios. Although it was confirmed that the catheter was in the correct place, the cyst increased in size again two weeks later, associated with repetition of polyhydramnios. It was postulated that the catheter was blocked, and we chose to place another catheter instead of performing repeated punctures. The cystic volume, polyhydramnios and mediastinal shift regressed progressively. At 38.5 weeks, a 3,310/g male infant was delivered without presenting any respiratory distress. The infant underwent thoracotomy on the 15th day of life. Thus, in the present study, we discuss the possibility of placing a cyst-amniotic shunt instead of performing repeated cystic punctures, even at a gestational age close to full term.

CONTEXTO: A malformação adenomatóide do pulmão tipo macrocística pode causar compressão mediastinal grave, hidropisia e polihidrâmnio aumentando a chance de óbito perinatal. Após a 33ª semana de gestação, recomenda-se realizar punções repetidas do cisto. Apresentamos um caso em que um dreno cístico-amniótico foi colocado e as punções foram evitadas. RELATO DE CASO: Um dreno cístico-amniótico foi colocado na 33ª semana de gestação devido a grande malfomação adenomatóide cística do pulmão associada a desvio de mediastino grave e polihidrâmnio. Apesar de o cateter ter sido identificado no local correto, o cisto voltou a crescer duas semanas após, repetindo o polihidrâmnio. Postulou-se que o cateter estava obstruído e optamos por colocar um novo cateter ao invés de realizar punções repetidas desse cisto. Foi observada regressão progressiva do volume do cisto, do polihidrâmnio e do desvio de mediastino. Na 38ª semana e meia, um menino de 3.310 g nasceu sem apresentar dificuldade respiratória, sendo submetido a toracotomia no 15º dia de vida. Portanto, no presente estudo, discute-se sobre a possibilidade de colocação do dreno cístico-amniótico no lugar de realizar punções repetidas do cisto mesmo em idades gestacionais próximas do termo.