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Background: We aimed to conduct a systematic review and meta-analysis to evaluate the fetoscopic tracheal occlusion in patients with isolated severe and left-sided diaphragmatic hernia. Methods: Cochrane Library, Embase, and PubMed (Medline) databases were searched from inception to February 2024 with no filters or language restrictions. We included studies evaluating the outcomes of fetoscopic intervention compared to expectant management among patients with severe congenital diaphragmatic hernia exclusively on the left side. A random-effects pairwise meta-analysis was performed using RStudio version 4.3.1. Results: In this study, we included 540 patients from three randomized trials and five cohorts. We found an increased likelihood of neonatal survival associated with fetoscopic tracheal occlusion (Odds Ratio, 5.07; 95% Confidence Intervals, 1.91 to 13.44; p < 0.01) across general and subgroup analyses. Nevertheless, there were higher rates of preterm birth (OR, 5.62; 95% CI, 3.47-9.11; p < 0.01) and preterm premature rupture of membranes (OR, 7.13; 95% CI, 3.76-13.54; p < 0.01) in fetal endoscopic tracheal occlusion group compared to the expectant management. Conclusions: Our systematic review and meta-analysis demonstrated the benefit of fetoscopic tracheal occlusion in improving neonatal and six-month postnatal survival in fetuses with severe left-sided CDH. Further studies are still necessary to evaluate the efficacy of tracheal occlusion for isolated right-sided CDH, as well as the optimal timing to perform the intervention.
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INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases. CASE PRESENTATION: We present a case with both cervicothoracic and lumbosacral myelomeningocele, Arnold-Chiari malformation type II and bilateral ventriculomegaly, that was treated successfully at 25 weeks with open micro-neurosurgery. Double myelomeningocele was successfully treated through a single 2-cm micro-hysterotomy, by performing external versions to sequentially expose and repair both defects. Weekly postoperative follow-up showed no progression of ventriculomegaly or complications attributable to the procedure. Preterm rupture of membranes prompted a conventional cesarean delivery at 32 weeks of gestation. Neurodevelopmental outcome at 20 months was within normal ranges, having achieved ambulation without orthopedic support and with no need for ventriculoperitoneal shunting. CONCLUSION: This report demonstrates for the first time the feasibility of double OSB repair through a single 2-cm micro-hysterotomy, suggesting that selected isolated cases of double myelomeningocele could be candidates for fetal intervention. Further prospective studies should be carried out to assess the potential benefit of double OSB intrauterine open repair.
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Histerotomía , Meningomielocele , Humanos , Meningomielocele/cirugía , Meningomielocele/diagnóstico por imagen , Femenino , Histerotomía/métodos , Embarazo , Recién Nacido , Malformación de Arnold-Chiari/cirugía , Malformación de Arnold-Chiari/diagnóstico por imagen , Adulto , Terapias Fetales/métodosRESUMEN
INTRODUCTION: Large congenital neck tumors can cause neonatal death due to airway obstruction. The aim of this study was to report outcomes of the first cohort of fetuses with neck masses and suspected airway obstruction managed with fetal laryngoscopy (FL) and fetal endoscopic tracheal intubation (FETI) to secure fetal airways and avoid ex utero intrapartum treatment (EXIT) procedure. METHODS: A prospective observational cohort of consecutive fetuses with neck masses that were candidates for an EXIT procedure due to suspicion of laryngeal and/or tracheal occlusion on ultrasonographic (US) or magnetic resonance imaging (MRI) examination were recruited for FL in a tertiary referral center in Queretaro, Mexico. FETI was performed if the obstruction was confirmed by FL. Maternal and perinatal outcomes were evaluated. RESULTS: Between January 2012 and March 2023, 35 cases with neck masses were evaluated. Airway obstruction was suspected in 12/35 (34.3%), either by US in 10/35 (28.6%) or by fetal MRI in 2/35 (5.7%). In all cases, FL was successfully performed at the first attempt at a median gestational age (GA) of 36+5 (range, 33+5-39+6) weeks+days, with a median surgical time of 22.5 (12-35) min. In 4 cases, airway patency was confirmed during FL and an EXIT procedure was avoided. In 8/12 cases (66.7%), airway obstruction was confirmed during fetoscopy and FETI was successfully performed at a median GA of 36+3 (33+2-38+5) weeks+days, with a median surgical time of 25.0 (range, 12-45) min. No case required an EXIT procedure. All patients underwent conventional cesarean delivery with no maternal complications and all neonates were admitted to the neonatal intensive care unit with a correctly positioned endotracheal tube (ETT) immediately after delivery. Three neonatal deaths (37.5%) were reported due to postnatal unplanned extubation, failed ETT replacement, and tumoral bleeding. CONCLUSION: In fetuses with neck masses and suspected airway obstruction, FL and FETI are feasible and could replace EXIT procedures with good maternal and perinatal outcomes.
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Obstrucción de las Vías Aéreas , Laringoscopía , Embarazo , Femenino , Recién Nacido , Humanos , Laringoscopía/efectos adversos , Intubación Intratraqueal/efectos adversos , Intubación Intratraqueal/métodos , Feto , Atención Prenatal , Obstrucción de las Vías Aéreas/diagnóstico por imagen , Obstrucción de las Vías Aéreas/cirugía , Obstrucción de las Vías Aéreas/etiologíaRESUMEN
INTRODUCTION: A proportion of monochorionic diamniotic (MCDA) twin pregnancies complicated by twin-to-twin transfusion syndrome (TTTS) can present after 26 weeks of gestation. The aim of this study was to compare perinatal outcomes of late TTTS treated by fetoscopic laser coagulation versus traditional management with amniodrainage and/or emergency preterm cesarean delivery (CD). METHODS: Retrospective cohort from January 2012 to January 2023 of consecutive MCDA twin pregnancies complicated by TTTS after 26 weeks and evaluated in our referring centers. We analyzed perinatal outcomes of cases treated with fetoscopic laser surgery at our national referral fetal surgery center in Queretaro, Mexico, and compared them with those managed with traditional management (amniodrainage and/or emergency preterm CD). The primary outcome was survival at discharge and the secondary outcome was gestational age (GA) at birth. RESULTS: Among the study population, 46 TTTS cases were treated by fetoscopy at 27+6 (26+0-31+0) weeks+days and were compared with a group of 39 cases who underwent emergency preterm CD. In comparison to the group who underwent traditional management, the group treated by laser fetoscopy showed a significantly higher GA at birth (32+3 vs. 29+1 weeks+days, p < 0.001), lower frequency of preterm delivery below 37 weeks (91.3% vs. 100%, p = 0.06), 34 weeks (63.0% vs. 100%, p < 0.001), 32 weeks (50% vs. 74.4%, p = 0.02), or 30 weeks (28.3% vs. 53.8%, p = 0.01), and significantly higher perinatal survival (89.1% vs. 71.8%, p < 0.05 of at least one twin; and 65.2% vs. 38.5%, p = 0.01 of both twins, respectively). CONCLUSION: MCDA twins complicated with TTTS can be treated with fetoscopic laser surgery between 26 and 31 weeks of gestation, which is a feasible and safe option, and such cases are associated with a higher GA at birth and better perinatal survival than those managed with amniodrainage and/or emergency preterm CD.
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Transfusión Feto-Fetal , Terapia por Láser , Embarazo , Recién Nacido , Femenino , Humanos , Fetoscopía , Resultado del Embarazo , Estudios Retrospectivos , Terapia por Láser/efectos adversos , Embarazo Gemelar , Coagulación con Láser , Edad GestacionalRESUMEN
INTRODUCTION: Identification of intertwin anastomosis may be challenging during fetoscopy in cases with complete anterior placenta. The aim of this study was to describe the technique, feasibility, and outcomes of flexible video fetoscopy for laser coagulation in monochorionic (MC) twin pregnancies with twin-to-twin transfusion syndrome (TTTS) presenting with inaccessible anterior placenta. METHODS: From April 2021 to March 2022, a prospective cohort of consecutive MC twin pregnancies complicated with TTTS presenting with anterior placenta after 20 weeks was recruited. Cases with inaccessible anterior placenta during standard technique were converted into flexible video fetoscopy for completion of laser coagulation of placental anastomoses using a 270° flexible video endoscope through the same uterine port. Descriptive analysis includes feasibility, remaining anastomoses requiring laser photocoagulation, and perinatal outcomes. RESULTS: A total of 45 pregnancies with TTTS were treated with fetoscopic laser therapy during the 1-year study period. Twenty-one pregnancies presented with anterior placenta after 20 weeks, in which an inaccessible vascular equator was observed in 33.3% (7/21). Flexible video fetoscopy was successfully performed in all 7 cases at a median gestational age of 22+2 (20+0-27+1) weeks+days. Visualization of the entire placental surface, coagulation of selected vessels, and exploration of the entire vascular equator were achieved in all cases. Six cases (85.7%) required additional laser coagulation due to either vascular patency despite initial coagulation with conventional fetoscopy (1/6, 16.7%) and/or remaining noncoagulated anastomoses (5/6, 83.3%). Perinatal survival of at least one twin and both twins was achieved in 85.7% and 57.1%, respectively. DISCUSSION: Flexible video fetoscopy for completion of laser coagulation of placental anastomoses is feasible and represents a good option for TTTS cases presenting after 20 weeks with inaccessible anterior placenta.
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Transfusión Feto-Fetal , Terapia por Láser , Embarazo , Femenino , Humanos , Lactante , Transfusión Feto-Fetal/diagnóstico por imagen , Transfusión Feto-Fetal/cirugía , Placenta/cirugía , Placenta/irrigación sanguínea , Fetoscopía/métodos , Estudios Prospectivos , Estudios de Factibilidad , Coagulación con Láser/métodos , Edad GestacionalRESUMEN
OBJECTIVE: To evaluate maternal and perinatal outcomes of late open fetal repair for open spina bifida (OSB) between 26+0 -27+6 weeks. METHODS: A cohort of fetuses with OSB who underwent open surgery in two fetal surgery centers (Argentina and Mexico). Two groups were defined based on the gestational age (GA) at intervention: Management of Myelomeningocele Study (MOMS) time window group: GA 19+0 -25+6 , and late intervention group: GA 26+0 -27+6 . RESULTS: Intrauterine OSB repair was successfully performed in 140 cases, either before (n = 57) or after (n = 83) 26 weeks, at on average 25+0 (22+6 -25+6 ) and 26+5 (26+0 -27+6 ) weeks + days, respectively. There were no significant differences in the rate of premature rupture of membranes, chorioamnionitis, oligohydramnios, preterm delivery, perinatal death and maternal complications. The late intervention group showed a significantly lower surgical times (112.6 vs. 124.2 min, p = 0.01), lower interval between fetal surgery and delivery (7.9 vs. 9.2 weeks, p < 0.01) and similar rate of hydrocephalus requiring treatment (30.6% vs. 23.3%, p = 0.44) than the MOMS time window group. CONCLUSION: Late fetal surgery for OSB repair between 26+0 -27+6 weeks is feasible and was associated with similar outcomes than that performed before 26 weeks. These findings may allow an extension of the proposed time window for cases with late diagnosis or referral.
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Feto , Meningomielocele , Espina Bífida Quística , Femenino , Feto/cirugía , Edad Gestacional , Humanos , Recién Nacido , Meningomielocele/cirugía , Embarazo , Espina Bífida Quística/cirugía , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVE: To assess the effect of Fetal Endoscopic Tracheal Occlusion (FETO) on neonatal survival in fetuses with left congenital diaphragmatic hernia (CDH) and moderate lung hypoplasia. STUDY DESIGN: CDH fetuses with moderate pulmonary hypoplasia (observed/expected lung area to head ratio between 26% and 35%, or between 36% and 45% with liver herniation) were prospectively recruited. Included patients were matched to a control group who were ineligible for FETO. Primary outcomes were survival at 28 days, at discharge, and at 6 months of age, respectively. RESULTS: 58 cases were recruited, 29 treated with FETO and 29 matched controls. Median gestational age (GA) at balloon placement and removal were 29.6 and 33.6 weeks, respectively. FETO group showed significantly lower GA at delivery (35.2 vs. 37.1 weeks, respectively, p < 0.01), higher survival at 28 days (51.7 vs. 24.1%, respectively, p = 0.03), at discharge (48.3 vs. 24.1%, respectively, p = 0.06), and at six months of age (41.4 vs. 24.1%, respectively, p = 0.16), and significantly lower length of ventilatory support (17.8 vs. 32.3 days, p = 0.01) and NICU stay (34.2 vs. 58.3 days, p = <0.01) compared to controls. CONCLUSION: FETO was associated with a non-significant increase in survival and significantly lower neonatal respiratory morbidity among CDH fetuses with moderate lung hypoplasia.
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Obstrucción de las Vías Aéreas , Oclusión con Balón , Hernias Diafragmáticas Congénitas , Anomalías del Sistema Respiratorio , Femenino , Fetoscopía , Feto , Edad Gestacional , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Lactante , Recién Nacido , Pulmón/anomalías , Pulmón/diagnóstico por imagen , Embarazo , Tráquea/cirugía , Resultado del Tratamiento , Ultrasonografía PrenatalRESUMEN
BACKGROUND/OBJECTIVES: Prenatal myelomeningocele (MMC) repair has been shown to improve neurological outcomes. It has been suggested that decreases in the hysterotomy diameter during surgery can improve perinatal outcomes without altering neurologic outcomes. The objective of this study is to describe and compare the main maternal and fetal outcomes of fetuses undergoing open surgery for MMC repair, through the different modifications (standard-classical, mini-hysterotomy, and microneurosurgery). DATA SOURCE: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Ovid, SciELO, LILACS, PROSPERO. RESULTS: From a total of 112 studies, seven case series were selected including 399 fetuses with open fetal surgery, five studies using the classical technique (n = 181), one with mini-hysterotomy (n = 176), and one with the microneurosurgery technique (n = 42). The mini-hysterotomy and microneurosurgery techniques presented a lower risk of preterm delivery (21.4% and 30%, respectively) compared to the classic technique (47.3%), premature rupture of membranes (78%, 62%, and 72.5 %, respectively), oligohydramnios (0% and 72.5%, respectively), dehiscence of hysterotomy, maintaining the same frequency of Chiari reversion (78%, 62%, and 72.5%, respectively), postnatal correction requirement (0%, 4.8%, and 5.8%, respectively), and lower frequency of requirement for a ventriculoperitoneal shunt placement (13.0%, 7.5%, and 29.1%, respectively). CONCLUSION: The least invasive techniques (minihysterotomy-microneurosurgery) are possible and reproduceable, as they are associated with better maternal and perinatal outcomes.
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Terapias Fetales/normas , Edad Gestacional , Disrafia Espinal/cirugía , Derivación Ventriculoperitoneal/métodos , Adulto , Femenino , Terapias Fetales/métodos , Fetoscopía/métodos , Humanos , Embarazo , Derivación Ventriculoperitoneal/tendenciasRESUMEN
OBJECTIVE: To determine the historical aspects, current availability, and clinical outcomes of open intrauterine repair of spina bifida aperta (IRSBA) in Spanish-speaking Latin American countries. METHODS: Cases were collected from centers with at least 2 years of experience and a minimum of 10 open IRSBA interventions by December 2020. Clinical variables were compared to the results of the Management of Myelomeningocele Study (MOMS) trial. RESULTS: Clinical experience with 314 cases from seven centers was reviewed. Most cases (n = 189, 60.2%) were performed between 24 and 25.9 weeks' gestation. Delivery at less than 30 weeks' gestation occurred in 36 cases (11.5%) and the overall perinatal mortality rate was 5.4% (17 of 314). The rate of maternal complications was low, including the need for blood transfusion (n = 3, 0.9%) and dehiscence or a thin uterine scar (n = 4, 1.3%). No cases of maternal death were recorded. Fifteen neonates required additional surgical repair of the spinal defect (4.8%) and 63 of 167 infants (37.7%) required a cerebrospinal fluid diversion procedure. Only two of the seven centers reported preliminary experience with fetoscopic IRSBA. CONCLUSIONS: Clinical experience and outcomes were within the expected results reported by the MOMS trial. There is still very limited experience with fetoscopic IRSBA in this part of the world.
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Feto/cirugía , Accesibilidad a los Servicios de Salud/normas , Evaluación de Resultado en la Atención de Salud/estadística & datos numéricos , Espina Bífida Quística/cirugía , Adulto , Femenino , Edad Gestacional , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Humanos , América Latina/epidemiología , Evaluación de Resultado en la Atención de Salud/métodos , Embarazo , Espina Bífida Quística/complicaciones , Espina Bífida Quística/epidemiologíaRESUMEN
BACKGROUND: To describe the perinatal outcomes of fetoscopic urethral meatotomy (FUM) in fetuses with lower urinary tract obstruction (LUTO) by congenital megalourethra. STUDY DESIGN: Between 2012 and 2020, 226 cases with LUTO were referred to our fetal surgery center in Queretaro, Mexico. We report the perinatal outcome of cases with LUTO by congenital megalourethra that were selected for FUM in an attempt to release the penile urethral obstruction. RESULTS: Congenital megalourethra was diagnosed in 10 cases (4.4%) but only 3 cases (30%) with obstructive megalourethra and megacystis were selected for fetal surgery. Fetoscopic urethral metatotomy was successfully performed in all three cases at a median gestational age (GA) of 21.4 (18.0-26.7) weeks and with a median surgical time of 27 (12-43) min. A resolution of urethral dilatation and subsequent reduction of the penile length and normalization of both the bladder size and amniotic fluid were observed in all cases. The median GA at delivery was 35.2 (range: 30.6-38.0) weeks. There were no fetal deaths but one neonatal death (33%) secondary to renal failure and preterm delivery. CONCLUSION: In fetuses with LUTO by congenital obstructive megalourethra, FUM is feasible and is associated with good perinatal outcomes.
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Fetoscopía/métodos , Obstrucción Uretral/congénito , Femenino , Fetoscopía/tendencias , Edad Gestacional , Humanos , Lactante , Recién Nacido , Masculino , México/epidemiología , Pene/anomalías , Pene/cirugía , Embarazo , Ultrasonografía Prenatal/métodos , Ultrasonografía Prenatal/estadística & datos numéricos , Uretra/anomalías , Obstrucción Uretral/epidemiología , Obstrucción Uretral/cirugíaRESUMEN
OBJECTIVE: The objective of this study was to assess the predictive performance of preoperative cervical length (CL) for delivery within 1 week after pleuroamniotic shunting (PAS) in fetuses with severe hydrothorax. METHODS: A prospective cohort of fetuses with severe hydrothorax referred to our fetal surgery center in Querétaro, Mexico from January 2012 to July 2020. Severe fetal hydrothorax was diagnosed as an accumulation of fluid within the fetal pleural space accompanied with severe bilateral lung compression, mediastinal shift, polyhydramnios, and/or hydrops. Transvaginal CL was measured immediately before PAS, and a short cervix was defined as that <25 mm. The interval from fetal intervention to delivery, prevalence of preterm prelabor rupture of membranes (PPROMs), and associations with delivery within the first week after PAS according to a short or a normal CL, were evaluated. RESULTS: Thirty-five pregnancies with severe fetal hydrothorax treated with PAS were evaluated. Median gestational age at PAS was (weeks + days) 31+2 (range, 26+0-36+1). Two (5.7%) and 7 (20.0%) cases delivered within the first 24 h and 1 week after PAS, respectively. Ten (28.6%) women had a short cervix before PAS, while 25 (71.4%) had normal preoperative CL. Women with a short cervix showed lower mean interval between fetal intervention and delivery (2.4 vs. 5.5 weeks, p = 0.01), and higher prevalence of PPROM (50 vs. 12%, p = 0.01), as compared to women with a nonshort cervix. Preoperative short cervix was associated with significantly higher risk of delivery within the first 24 h (20.0 vs. 0%, respectively, p < 0.05) and 1 week after PAS (50.0 vs. 8.0%, respectively, p < 0.01) compared with pregnancies with normal preoperative CL. CONCLUSION: In pregnancies with severe fetal hydrothorax candidates for pleuroamniotic shunt, identification of a short cervix before fetal intervention can predict delivery within 1 week after the surgical procedure.
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Terapias Fetales , Hidrotórax , Cuello del Útero/diagnóstico por imagen , Cuello del Útero/cirugía , Femenino , Feto , Humanos , Hidrotórax/diagnóstico por imagen , Hidrotórax/cirugía , Recién Nacido , Embarazo , Estudios ProspectivosRESUMEN
OBJECTIVE: The purpose of this article was to describe our experience with the prenatal diagnosis of CHD in patients referred to our Fetal Cardiology Unit. METHODS: Prospective cohort study of consecutive fetuses referred for advanced fetal echocardiography to our Fetal Cardiology Unit during a 3-year period (September 2015-September 2018). RESULTS: Totally 809 fetuses were evaluated, with 1263 fetal advanced echocardiographies performed. Suspected cardiac abnormality was the most common indication for referral (62.2%). Only 7.3% of patients had known morbidities or risk factors for CHD. Mean gestational age at first examination was 25.6 ± 6.4 weeks. A total of 528 (65.3%) fetuses were found to have a cardiac defect: 40.7% had isolated CHD while 24.6% had associated anomalies. The most common defects found were ventricular septal defects (20.3%), followed by conotruncal defects (9.7%), hypoplastic left heart syndrome (9.3%), fetal arrhythmias (8.9%), and venous anomalies (8.7%). 31.6% presented abnormal genetic studies, the most frequent being Down syndrome (23/212, 10.8%), followed by DiGeorge syndrome (11/212, 5.2%). CONCLUSIONS: Prenatal screening and diagnosis of CHD in Mexico are feasible, with suspected cardiac abnormality being the main reason for referral to a specialized Fetal Cardiology Unit. Efforts must be made to make screening available to the general population in the first and second trimesters of pregnancy by fetal medicine or trained specialists, in order to identify fetal CHD and offer advanced echocardiography, genetic studies, timely fetal cardiac intervention in selected cases, and delivery in tertiary centers, to improve overall survival.
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Cardiología , Cardiopatías Congénitas , Femenino , Corazón Fetal/diagnóstico por imagen , Feto , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/epidemiología , Humanos , México/epidemiología , Embarazo , Diagnóstico Prenatal , Estudios Prospectivos , Ultrasonografía PrenatalRESUMEN
BACKGROUND: To evaluate the incidence of laryngeal ultrasound (US) abnormalities in fetuses with congenital diaphragmatic hernia (CDH) and to assess the utility of fetal laryngoesophagoscopy for prenatal diagnosis of laryngo-tracheo-esophageal anomalies. STUDY DESIGN: A cohort of CDH fetuses with laryngeal ultrasound abnormalities were selected for diagnostic fetal laringoesophagoscopy in a single fetal surgery center at Queretaro, Mexico. RESULTS: During the study period, 210 CDH fetuses were evaluated. US examination of the vocal cords was successfully performed in all fetuses, and abnormal ultrasound findings were observed in four cases (1.9%). Fetal laringoesophagoscopy was successfully performed in all four cases at a median gestational age of 29.5 (range, 28.1-30.6) weeks. During fetal intervention, a laryngo-tracheo-esophageal cleft (TEC) extending from the larynx to the carina (type IV) was endoscopically visualized in three cases, and laryngeal atresia coexisting with TEC was confirmed in the remaining case. Fetal karyotype was normal in all cases, but abnormal chromosomal microarray analysis was reported in two cases (50%). All cases were delivered liveborn with severe respiratory failure presenting cardiac arrest and immediate neonatal death. CONCLUSIONS: Laryngeal anomalies in CDH fetuses can be presumed by ultrasound evaluation of the vocal cords and confirmed by fetal laryngoesophagoscopy during pregnancy.
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Esofagoscopía , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Laringoscopía , Ultrasonografía Prenatal , Pliegues Vocales/diagnóstico por imagen , Adolescente , Adulto , Obstrucción de las Vías Aéreas/congénito , Obstrucción de las Vías Aéreas/diagnóstico por imagen , Esófago/anomalías , Femenino , Humanos , Embarazo , Estudios Prospectivos , Tráquea/anomalías , Pliegues Vocales/anomalíasRESUMEN
BACKGROUND: Fetal aortic stenosis (AoS) may progress to hypoplastic left heart syndrome (HLHS) in utero. There are currently no data, prenatal or postnatal, describing survival of fetuses or neonates with AoS or HLHS in a country with suboptimal postnatal management. STUDY DESIGN: Prospective cohort study performed in Mexico, including cases diagnosed with AoS and HLHS within a 6-year period. AoS patients fulfilling previously published criteria for evolving HLHS (eHLHS) were offered fetal aortic valvuloplasty. Outcome variables were perinatal mortality, postnatal management, type of postnatal circulation, and overall survival. RESULTS: Fifty-four patients were included: 16 AoS and 38 HLHS. Eighteen patients had associated anomalies and/or an abnormal karyotype. Seventy-four percent of HLHS received comfort measures, with only three cases reporting an attempt at surgical palliation, and one survivor of the first stage. Fetal aortic valvuloplasty was performed successfully in nine cases of eHLHS. Overall postnatal survival was 44% in AoS with fetal aortic valvuloplasty, and one case (ongoing) in the HLHS group. CONCLUSIONS: HLHS in Mexico carries more than a 95% risk of postnatal death, with little or no experience at surgical palliation in most centers. Fetal aortic valvuloplasty in AoS may prevent progression to HLHS and in this small cohort was associated with ≈50% survival.
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Valvuloplastia con Balón , Corazón Fetal/cirugía , Fetoscopía , Síndrome del Corazón Izquierdo Hipoplásico/cirugía , Atención Posnatal/estadística & datos numéricos , Resultado del Embarazo/epidemiología , Adulto , Estenosis de la Válvula Aórtica/epidemiología , Estenosis de la Válvula Aórtica/patología , Estenosis de la Válvula Aórtica/cirugía , Valvuloplastia con Balón/métodos , Valvuloplastia con Balón/estadística & datos numéricos , Estudios de Cohortes , Circulación Coronaria/fisiología , Femenino , Corazón Fetal/patología , Fetoscopía/efectos adversos , Fetoscopía/rehabilitación , Fetoscopía/estadística & datos numéricos , Edad Gestacional , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Síndrome del Corazón Izquierdo Hipoplásico/epidemiología , Recién Nacido , Masculino , México/epidemiología , Mortalidad Perinatal , Atención Posnatal/normas , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Embarazo , Atención Prenatal/métodos , Atención Prenatal/estadística & datos numéricos , Estudios Retrospectivos , Análisis de Supervivencia , Adulto JovenRESUMEN
OBJECTIVE: To evaluate natural history of fetuses congenital diaphragmatic hernia (CDH) prenatally diagnosed in countries where termination of pregnancy is not legally allowed and to predict neonatal survival according to lung area and liver herniation. METHODS: Prospective study including antenatally diagnosed CDH cases managed expectantly during pregnancy in six tertiary Latin American centres. The contribution of the observed/expected lung-to-head ratio (O/E-LHR) and liver herniation in predicting neonatal survival was assessed. RESULTS: From the total population of 380 CDH cases, 144 isolated fetuses were selected showing an overall survival rate of 31.9% (46/144). Survivors showed significantly higher O/E-LHR (56.5% vs 34.9%; P < .001), lower proportion of liver herniation (34.8% vs 80.6%, P < .001), and higher gestational age at birth (37.8 vs 36.2 weeks, P < 0.01) than nonsurvivors. Fetuses with an O/E-LHR less than 35% showed a 3.4% of survival; those with an O/E-LHR between 35% and 45% showed 28% of survival with liver up and 50% with liver down; those with an O/E-LHR greater than 45% showed 50% of survival rate with liver up and 76.9% with liver down. CONCLUSIONS: Neonatal mortality in CDH is higher in Latin American countries. The category of lung hypoplasia should be classified according to the survival rates in our Latin American CDH registry.
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Viabilidad Fetal/fisiología , Cabeza/patología , Hernia/diagnóstico , Hernias Diafragmáticas Congénitas/diagnóstico , Hernias Diafragmáticas Congénitas/mortalidad , Hepatopatías/diagnóstico , Pulmón/patología , Adulto , Pesos y Medidas Corporales , Cefalometría/métodos , Femenino , Cabeza/diagnóstico por imagen , Cabeza/embriología , Hernia/congénito , Hernia/mortalidad , Hernia/patología , Hernias Diafragmáticas Congénitas/patología , Humanos , Lactante , Mortalidad Infantil , Recién Nacido , América Latina/epidemiología , Hepatopatías/congénito , Hepatopatías/mortalidad , Hepatopatías/patología , Pulmón/diagnóstico por imagen , Pulmón/embriología , Masculino , Tamaño de los Órganos , Embarazo , Pronóstico , Sistema de Registros/normas , Tasa de Supervivencia , Ultrasonografía Prenatal , Adulto JovenRESUMEN
OBJECTIVES: The objectives of the study are to evaluate longitudinal changes in lung size and intrapulmonary-artery (IPa) Doppler in fetuses with congenital diaphragmatic hernia (CDH) and assess their contribution in predicting neonatal survival. METHODS: The observed/expected lung-to-head ratio (O/E-LHR) and IPa-pulsatility index (PI) and peak early diastolic reversed flow (PEDRF) were evaluated in a cohort of left-sided CDH fetuses managed expectantly during pregnancy. Longitudinal changes were analyzed by multilevel analysis, and their value to predict survival using the multiple logistic regression and decision-tree analysis was assessed. RESULTS: A total of 232 scans were performed on 69 CDH fetuses. The O/E-LHR values remained unchanged during fetal monitoring, whereas IPa-PI and PEDRF showed a progressive increase throughout follow-up, becoming abnormal on average at 30 weeks of gestation. Absent/reversed end-diastolic velocity (EDV) in the IPa was observed in 20.3%. O/E-LHR and IPa Doppler indices were significantly associated with probability of survival (O/E-LHR ≥ 26%, odds ratio [OR] 19.0; IPa-PI <+2.0 z score, OR 3.0; and positive EDV, OR 7.4). All cases with IPa-reversed EDV died after birth. CONCLUSION: While lung size remains stable during pregnancy, CDH fetuses show progressive deterioration in intrapulmonary blood flow. IPa Doppler evaluation may aid in predicting survival of CDH fetuses managed expectantly during pregnancy.
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Edad Gestacional , Hernias Diafragmáticas Congénitas/embriología , Pulmón/diagnóstico por imagen , Pulmón/embriología , Arteria Pulmonar/diagnóstico por imagen , Ultrasonografía Prenatal , Femenino , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/mortalidad , Humanos , Recién Nacido , Pulmón/irrigación sanguínea , Embarazo , Tasa de Supervivencia , Ultrasonografía DopplerRESUMEN
Resumen ANTECEDENTES: El secuestro broncopulmonar suele diagnosticarse antes del nacimiento como una masa sólida intratorácica, homogénea e hiperecogénica, casi siempre localizada en el lóbulo inferior pulmonar. Su signo patognomónico es la demostración con Doppler color de la arteria nutricia sistémica dentro de la masa pulmonar. La historia natural de la enfermedad ha demostrado que durante la vida fetal en la mayoría de los casos la masa puede tener regresión espontánea. Una proporción importante de casos tiene rápido crecimiento de la masa con hidrotórax, compresión pulmonar e hidrops. Estos casos son de mal pronóstico y elevada mortalidad perinatal y, en estas circunstancias, la cirugía fetal es la única opción que puede mejorar el pronóstico. Para este propósito se han intentado varias técnicas de cirugía fetal pero la ideal sigue siendo un tema de controversia. OBJETIVO: Describir los métodos diagnósticos, factores pronóstico y discutir las repercusiones perinatales de cada una de las diferentes técnicas de cirugía fetal descritas en la bibliografía para el tratamiento de fetos complicados con secuestro broncopulmonar. METODOLOGÍA: La búsqueda se efectuó en Medline y PubMed de todos los artículos en inglés y español publicados entre 1990 y 2019 que tuvieran las palabras clave "bronchopulmonary sequestration", "secuestro broncopulmonar", "fetal surgery" y "cirugía fetal". Se eligieron los que describían casos de secuestro broncopulmonar tratados con alguna técnica de cirugía fetal. RESULTADOS: Se encontraron 150 artículos pero solo se seleccionaron 29 estudios que describían casos con diagnóstico prenatal de secuestro broncopulmonar tratados con alguna técnica de cirugía fetal. Para tratar el secuestro broncopulmonar grave se describieron varias técnicas de intervención fetal, entre ellas: cirugía fetal abierta, colocación de catéter de derivación toraco-amniótica, oclusión del vaso nutricio sistémico mediante guía ecográfica con láser, radiofrecuencia, embolización o escleroterapia con inyección de alcohol intravascular e, incluso, broncoscopia fetal. CONCLUSIONES: Al parecer la coagulación láser del vaso nutricio es la técnica con mejores resultados perinatales que evita la muerte fetal, la morbilidad pulmonar y la necesidad de cirugía posnatal en prácticamente todos los casos tratados; se propone como el único tratamiento potencialmente curativo.
Abstract BACKGROUND: The bronchopulmonary sequestration (BPS) is usually diagnosed prenatally as an intrathoracic hyperechoic, homogeneous, solid mass usually located in the lower pulmonary lobe. The pathognomonic sign is the demonstration of its systemic feeding artery into the lung mass by using color Doppler ultrasound. Natural history data reports that a significant proportion of BPS cases usually regress in the intrauterine period. However, a proportion of cases showed a rapid and progressive growth with development of massive pleural effusion and hydrops. Such cases present the poorest prognosis and high perinatal mortality and therefore, fetal intervention should be considered to improve prognosis. Different fetal surgery procedures have been attempted for such purposes. However, the optimal fetal therapeutica strategy remains controversial. OBJECTIVE: In the present manuscript, we describe and discuss the perinatal outcome of the different published fetal interventions in fetuses complicated with BPS. METHODOLOGY: We search in the English and Spanish literature (Medline and PubMed) for cases complicated with BPS and treated with any fetal surgical intervention including the key words "broncopulmonary sequestration" and "fetal surgery" from 1990 to 2019. RESULTS: A total of 150 references were reviewed including only 26 studies describing pregnancies with prenatal diagnosis of bronchopulmonary sequestration that were treated with any fetal surgical intervention. Several fetal surgery procedures have been attempted for the management of complicated fetuses with severe BPS. These include open fetal surgery, placement of thoracoamniotic shunts, and occlusion of the feeding blood vessel by ultrasound-guided intrafetal laser coagulation, radiofrequency ablation, coil embolization, sclerotherapy with intravascular alcohol injection and fetal bronchoscopy. CONCLUSIONS: Laser coagulation of the feeding artery appears to be the best intervention, avoiding fetal death, neonatal pulmonary morbidity and the need for postnatal surgery in virtually all cases postulating itself as the only potential curative management.
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OBJECTIVE: To evaluate cardiac function by conventional echocardiography and tissue Doppler imaging in fetuses with left congenital diaphragmatic hernia (CDH). METHODS: Conventional echocardiography (myocardial performance index, ventricular filling velocities, and E/A ratios) and tissue Doppler imaging (annular myocardial peak velocities, E/E' and E'/A' ratios) in mitral, septal, and tricuspid annulus were evaluated in a cohort of 31 left-sided CDH fetuses and compared with 75 controls matched for gestational age 2:1. RESULTS: In comparison to controls, CDH fetuses had prolonged isovolumetric time periods (isovolumetric contraction time 35 ms vs 28 ms, P < .001), with higher myocardial performance index (0.49 vs 0.42, P < .001) and tricuspid E/A ratios (0.77 vs 0.72, P = .033). Longitudinal function assessed by tissue Doppler showed signs of impaired relaxation (mitral lateral A' 8.0 vs 10.1 cm/s, P < .001 and an increased mitral lateral E'/A' ratio 0.93 vs 0.78, P < .001) in the CDH fetuses as compared with controls, with preserved systolic function. CONCLUSION: Left CDH fetuses show echocardiographic signs of diastolic dysfunction, probably secondary to fetal heart compression, maintaining a preserved systolic function.
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Corazón Fetal/fisiopatología , Hernias Diafragmáticas Congénitas/fisiopatología , Adulto , Estudios de Casos y Controles , Ecocardiografía , Femenino , Corazón Fetal/diagnóstico por imagen , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Humanos , Embarazo , Estudios Prospectivos , Ultrasonografía PrenatalRESUMEN
OBJECTIVE: To assess longitudinal intrathoracic changes after fetal laser surgery in fetuses with bronchopulmonary sequestration (BPS) with hydrops and/or hydrothorax. STUDY DESIGN: The presence of intrafetal fluid effusions, the lung mass volume ratio (congenital pulmonary airway malformation volume ratio [CVR]), and the observed/expected lung-to-head circumference ratio (O/E-LHR) of both lungs were evaluated in a cohort of BPS fetuses with hydrops and/or hydrothorax treated with full laser ablation of the feeding artery (FLAFA). The longitudinal changes in intrafetal fluid effusions, lung mass volume, and pulmonary growth were analyzed by survival and multilevel analysis against days after FLAFA. RESULTS: FLAFA was successfully performed in 15 cases at a median gestational age of 26.9 weeks. A complete disappearance of the hydrops and hydrothorax was observed a median interval of 7.5 and 21 days after the fetal intervention, respectively. A progressive decrease in the CVR and an increment in the size of both lungs were observed after FLAFA. The O/E-LHR of the lung contralateral and ipsilateral to the side of the BPS became normal on average 8 and 10 weeks after FLAFA, respectively. CONCLUSION: Fetal laser surgery with FLAFA promotes disappearance of all fetal fluid effusions, a lung mass regression sequence, and a normalization of pulmonary growth.
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Secuestro Broncopulmonar/cirugía , Feto/cirugía , Terapia por Láser , Tórax/diagnóstico por imagen , Secuestro Broncopulmonar/diagnóstico por imagen , Femenino , Feto/diagnóstico por imagen , Humanos , Embarazo , Estudios Prospectivos , Resultado del Tratamiento , Ultrasonografía PrenatalRESUMEN
INTRODUCTION: Fetal growth restriction (FGR) affects 5%-10% of all pregnancies, contributing to 30%-50% of stillbirths. Unfortunately, growth restriction often is not detected antenatally. The last weeks of pregnancy are critical for preventing stillbirth among babies with FGR because there is a pronounced increase in stillbirths among growth-restricted fetuses after 37 weeks of pregnancy. Here we present a protocol (V.1, 23 May 2016) for the RATIO37 trial, which evaluates an integrated strategy for accurately selecting at-risk fetuses for delivery at term. The protocol is based on the combination of fetal biometry and cerebroplacental ratio (CPR). The primary objective is to reduce stillbirth rates. The secondary aims are to detect low birth weights and adverse perinatal outcomes. METHODS AND ANALYSIS: The study is designed as multicentre (Spain, Chile, Mexico,Czech Republic and Israel), open-label, randomised trial with parallel groups. Singleton pregnancies will be invited to participate after routine second-trimester ultrasound scan (19+0-22+6 weeks of gestation), and participants will be randomly allocated to receive revealed or concealed CPR evaluation. Then, a routine ultrasound and Doppler scan will be performed at 36+0-37+6 weeks. Sociodemographic and clinical data will be collected at enrolment. Ultrasound and Doppler variables will be recorded at 36+0-37+6 weeks of pregnancy. Perinatal outcomes will be recorded after delivery. Univariate (with estimated effect size and its 95% CI) and multivariate (mixed-effects logistic regression) comparisons between groups will be performed. ETHICS AND DISSEMINATION: The study will be conducted in accordance with the principles of Good Clinical Practice. This study was accepted by the Clinical Research Ethics Committee of Hospital Clinic Barcelona on 23May 2016. Subsequent approval by individual ethical committees and competent authorities was granted. The study results will be published in peer-reviewed journals and disseminated at international conferences. TRIAL REGISTRATION NUMBER: NCT02907242; pre-results.