RESUMEN
INTRODUCTION: Unilateral congenital high airway obstruction syndrome (CHAOS) is caused by a complete obstruction of a mainstem bronchus with resulting hyperinflation and accelerated growth of one lung, severe mediastinal shift, and hydrops. Spontaneous perforation of the atresia has been observed in CHAOS which allows hydrops to resolve but hyperinflation, mediastinal shift and a critical airway obstruction persists as the perforation is usually pinhole-sized. CASE PRESENTATION: We present a case of unilateral CHAOS presenting at 26 2/7 weeks with observed-to-expected total lung volume (O/E TLV) of 203% with spontaneous perforation occurring at 28 weeks with resolution of hydrops but persistence of hyperinflation and mediastinal shift with an O/E TLV of 60.5% on 34 5/7 weeks' magnetic resonance imaging (MRI), successfully managed in a 35 5/7 weeks, 1,670 gm, growth restricted baby, by venoarterial extracorporeal membrane oxygenation (VA ECMO) and resection of the tracheobronchial atresia and tracheobronchoplasty on day of life 5. The baby was separated from ECMO on post-op day 12, required tracheostomy for positive end expiratory pressure for tracheomalacia at 4 months. CONCLUSION: At 2 years of age, she has met all developmental milestones, has been weaned to room air tracheostomy collar, and has been anticipating tracheal decannulation. There is persistent bronchiectasis in the hyperinflated right lung but no malacia. This is the first reported survivor of mainstem bronchial atresia suggesting the importance of preservation of the hyperplastic lung and airway reconstruction to normal long-term outcome.
Asunto(s)
Obstrucción de las Vías Aéreas , Bronquios , Humanos , Femenino , Obstrucción de las Vías Aéreas/cirugía , Obstrucción de las Vías Aéreas/diagnóstico por imagen , Obstrucción de las Vías Aéreas/etiología , Bronquios/cirugía , Bronquios/diagnóstico por imagen , Bronquios/anomalías , Recién Nacido , Tráquea/cirugía , Tráquea/diagnóstico por imagen , Tráquea/anomalías , Oxigenación por Membrana Extracorpórea , Síndrome , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVE: To report the outcomes of fetoscopic endoluminal tracheal occlusion in a multicenter North American cohort of patients with isolated, left-sided congenital diaphragmatic hernia (CDH) and to compare neonatal mortality and morbidity in patients with severe left-sided congenital diaphragmatic hernia who underwent fetoscopic endoluminal tracheal occlusion with those expectantly managed. METHODS: We analyzed data from 10 centers in the NAFTNet (North American Fetal Therapy Network) FETO (Fetoscopic Endoluminal Tracheal Occlusion) Consortium registry, collected between November 1, 2008, and December 31, 2020. In addition to reporting procedure-related surgical outcomes of fetoscopic endoluminal tracheal occlusion, we performed a comparative analysis of fetoscopic endoluminal tracheal occlusion compared with contemporaneous expectantly managed patients. RESULTS: Fetoscopic endoluminal tracheal occlusion was successfully performed in 87 of 89 patients (97.8%). Six-month survival in patients with severe left-sided congenital diaphragmatic hernia did not differ significantly between patients who underwent fetoscopic endoluminal tracheal occlusion and those managed expectantly (69.8% vs 58.1%, P =.30). Patients who underwent fetoscopic endoluminal tracheal occlusion had higher rates of preterm prelabor rupture of membranes (54.0% vs 14.3%, P <.001), earlier gestational age at delivery (median 35.0 weeks vs 38.3 weeks, P <.001), and lower birth weights (mean 2,487 g vs 2,857 g, P =.001). On subanalysis, in patients for whom all recorded observed-to-expected lung/head ratio measurements were below 25%, patients with fetoscopic endoluminal tracheal occlusion required fewer days of extracorporeal membrane oxygenation (ECMO) (median 9.0 days vs 17.0 days, P =.014). CONCLUSION: In this cohort, fetoscopic endoluminal tracheal occlusion was successfully implemented across several North American fetal therapy centers. Although survival was similar among patients undergoing fetoscopic endoluminal tracheal occlusion and those expectantly managed, fetoscopic endoluminal tracheal occlusion in North American centers may reduce morbidity, as suggested by fewer days of ECMO in those patients with persistently reduced lung volumes (observed-to-expected lung/head ratio below 25%).
Asunto(s)
Obstrucción de las Vías Aéreas , Oclusión con Balón , Hernias Diafragmáticas Congénitas , Embarazo , Recién Nacido , Femenino , Humanos , Lactante , Hernias Diafragmáticas Congénitas/cirugía , Fetoscopía/efectos adversos , Pulmón , Feto , Obstrucción de las Vías Aéreas/etiología , América del Norte , Tráquea/cirugía , Oclusión con Balón/efectos adversosRESUMEN
Endothelial progenitor cells (EPCs) contribute to de novo angiogenesis, tissue regeneration, and remodeling. Interleukin 10 (IL-10), an anti-inflammatory cytokine that primarily signals via STAT3, has been shown to drive EPC recruitment to injured tissues. Our previous work demonstrated that overexpression of IL-10 in dermal wounds promotes regenerative tissue repair via STAT3-dependent regulation of fibroblast-specific hyaluronan synthesis. However, IL-10's role and specific mode of action on EPC recruitment, particularly in dermal wound healing and neovascularization in both normal and diabetic wounds, remain to be defined. Therefore, inducible skin-specific STAT3 knockdown mice were studied to determine IL-10's impact on EPCs, dermal wound neovascularization and healing, and whether it is STAT3-dependent. We show that IL-10 overexpression significantly elevated EPC counts in the granulating wound bed, which was associated with robust capillary lumen density and enhanced re-epithelialization of both control and diabetic (db/db) wounds at day 7. We noted increased VEGF and high C-X-C motif chemokine 12 (CXCL12) levels in wounds and a favorable CXCL12 gradient at day 3 that may support EPC mobilization and infiltration from bone marrow to wounds, an effect that was abrogated in STAT3 knockdown wounds. These findings were supported in vitro. IL-10 promoted VEGF and CXCL12 synthesis in primary murine dermal fibroblasts, with blunted VEGF expression upon blocking CXCL12 in the media by antibody binding. IL-10-conditioned fibroblast media also significantly promoted endothelial sprouting and network formation. In conclusion, these studies demonstrate that overexpression of IL-10 in dermal wounds recruits EPCs and leads to increased vascular structures and faster re-epithelialization.
Asunto(s)
Diabetes Mellitus , Células Progenitoras Endoteliales , Interleucina-10/metabolismo , Animales , Medios de Cultivo Condicionados/metabolismo , Diabetes Mellitus/metabolismo , Células Progenitoras Endoteliales/metabolismo , Interleucina-10/genética , Ratones , Neovascularización Fisiológica/fisiología , Factor de Transcripción STAT3/metabolismo , Factor A de Crecimiento Endotelial Vascular/metabolismo , Cicatrización de Heridas/fisiologíaRESUMEN
Twin-twin transfusion syndrome (TTTS) is a rare but serious cause of fetal cardiomyopathy with poorly understood pathophysiology and challenging prognostication. This study sought a nonbiased, comprehensive assessment of amniotic fluid (AF) microRNAs from TTTS pregnancies and associations of these miRNAs with clinical characteristics. For the discovery cohort, AF from ten fetuses with severe TTTS cardiomyopathy were selected and compared to ten normal singleton AF. Array panels assessing 384 microRNAs were performed on the discovery cohort and controls. Using a stringent q < 0.0025, arrays identified 32 miRNAs with differential expression. Top three microRNAs were miR-99b, miR-370 and miR-375. Forty distinct TTTS subjects were selected for a validation cohort. RT-PCR targeted six differentially-expressed microRNAs in the discovery and validation cohorts. Expression differences by array were confirmed by RT-PCR with high fidelity. The ability of these miRNAs to predict clinical differences, such as cardiac findings and later demise, was evaluated on TTTS subjects. Down-regulation of miRNA-127-3p, miRNA-375-3p and miRNA-886 were associated with demise. Our results indicate AF microRNAs have potential as a diagnostic and prognostic biomarker in TTTS. The top microRNAs have previously demonstrated roles in angiogenesis, cardiomyocyte stress response and hypertrophy. Further studies of the mechanism of actions and potential targets is warranted.
RESUMEN
INTRODUCTION: Hepatopulmonary fusion (HPF) is a rare anomaly specifically associated with right-sided congenital diaphragmatic hernia (CDH). Fewer than 50 cases of HPF have been reported, most at the time of surgery or postmortem with an associated high morbidity and mortality rate. Prenatal diagnosis and optimal management of these rare cases have not been established. CASE PRESENTATION: We present a case of HPF diagnosed prenatally by fetal ultrasound and magnetic resonance imaging allowing postnatal and intraoperative planning leading to successful HPF separation and repair of the CDH. DISCUSSION: The prenatal recognition allowed the use of preoperative computed tomography angiogram and right cardiac catheterization to plan the surgical approach. Intraoperative ultrasound confirmed abnormal vasculature associated with the HPF facilitating a successful complete HPF separation and CDH repair was performed. The patient survived and continues to do well on long-term follow-up. Prenatal recognition may help reduce the high morbidity and mortality associated with HPF.
Asunto(s)
Anomalías del Sistema Digestivo , Hernias Diafragmáticas Congénitas , Embarazo , Femenino , Humanos , Hernias Diafragmáticas Congénitas/cirugía , Diagnóstico Prenatal , Ultrasonografía Prenatal , Estudios RetrospectivosRESUMEN
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that is often accompanied by other anomalies. Although the role of genetics in the pathogenesis of CDH has been established, only a small number of disease-associated genes have been identified. To further investigate the genetics of CDH, we analyzed de novo coding variants in 827 proband-parent trios and confirmed an overall significant enrichment of damaging de novo variants, especially in constrained genes. We identified LONP1 (lon peptidase 1, mitochondrial) and ALYREF (Aly/REF export factor) as candidate CDH-associated genes on the basis of de novo variants at a false discovery rate below 0.05. We also performed ultra-rare variant association analyses in 748 affected individuals and 11,220 ancestry-matched population control individuals and identified LONP1 as a risk gene contributing to CDH through both de novo and ultra-rare inherited largely heterozygous variants clustered in the core of the domains and segregating with CDH in affected familial individuals. Approximately 3% of our CDH cohort who are heterozygous with ultra-rare predicted damaging variants in LONP1 have a range of clinical phenotypes, including other anomalies in some individuals and higher mortality and requirement for extracorporeal membrane oxygenation. Mice with lung epithelium-specific deletion of Lonp1 die immediately after birth, most likely because of the observed severe reduction of lung growth, a known contributor to the high mortality in humans. Our findings of both de novo and inherited rare variants in the same gene may have implications in the design and analysis for other genetic studies of congenital anomalies.
Asunto(s)
Proteasas ATP-Dependientes/genética , Proteasas ATP-Dependientes/fisiología , Anomalías Craneofaciales/genética , Variaciones en el Número de Copia de ADN , Anomalías del Ojo/genética , Trastornos del Crecimiento/genética , Hernias Diafragmáticas Congénitas/genética , Luxación Congénita de la Cadera/genética , Proteínas Mitocondriales/genética , Proteínas Mitocondriales/fisiología , Mutación Missense , Osteocondrodisplasias/genética , Anomalías Dentarias/genética , Animales , Estudios de Casos y Controles , Estudios de Cohortes , Anomalías Craneofaciales/patología , Anomalías del Ojo/patología , Femenino , Trastornos del Crecimiento/patología , Hernias Diafragmáticas Congénitas/patología , Luxación Congénita de la Cadera/patología , Humanos , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Osteocondrodisplasias/patología , Linaje , Anomalías Dentarias/patologíaRESUMEN
BACKGROUND: Mediastinal masses in children may present with compression of the great vessels and airway. An interdisciplinary plan for rapid diagnosis, acute management, and treatment prevents devastating outcomes and optimizes care. Emergency pretreatment with steroids or radiation is more likely to be administered when care is variable, which may delay and complicate diagnosis and treatment. Strategies to standardize care and expedite diagnosis may improve acute patient safety and long-term outcomes. AIMS: The aim of this quality improvement project was to decrease time from presentation to diagnostic biopsy for children with an anterior mediastinal mass by 50% over 3 years within a tertiary healthcare system. METHODS: This quality improvement project involved a single center with data collected and analyzed retrospectively and prospectively for 71 patients presenting with anterior mediastinal mass between February 2008 and January 2018. The Model for Improvement was utilized for project design and development of a driver diagram and smart aim. An algorithm was implemented to facilitate communication between teams and standardize initial care of patients with mediastinal masses. The algorithm underwent multiple Plan-Do-Study-Act (PDSA) cycles. Data were collected before and after algorithm implementation and between each PDSA cycle. The primary outcome measure included time from presentation to biopsy, which was monitored with a statistical process control chart. Several process measures were evaluated with Student's t-tests including administration of emergency pretreatment. RESULTS: Nineteen patients preintervention and 52 patients postintervention were included in the analysis. Time from presentation to biopsy significantly decreased from 48 h at baseline to 24 h postimplementation. Although not statistically significant, emergency pretreatment decreased from a baseline of 26.3% to 6.7% postimplementation. CONCLUSION: Implementation of a diagnostic and management algorithm coordinating care among multidisciplinary teams significantly reduced time to biopsy for children presenting with mediastinal mass and may result in decreased use of emergent pretreatment.
Asunto(s)
Seguridad del Paciente , Mejoramiento de la Calidad , Algoritmos , Biopsia , Niño , Humanos , Estudios RetrospectivosRESUMEN
PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with significant mortality and long-term morbidity in some but not all individuals. We hypothesize monogenic factors that cause CDH are likely to have pleiotropic effects and be associated with worse clinical outcomes. METHODS: We enrolled and prospectively followed 647 newborns with CDH and performed genomic sequencing on 462 trios to identify de novo variants. We grouped cases into those with and without likely damaging (LD) variants and systematically assessed CDH clinical outcomes between the genetic groups. RESULTS: Complex cases with additional congenital anomalies had higher mortality than isolated cases (P = 8 × 10-6). Isolated cases with LD variants had similar mortality to complex cases and much higher mortality than isolated cases without LD (P = 3 × 10-3). The trend was similar with pulmonary hypertension at 1 month. Cases with LD variants had an estimated 12-17 points lower scores on neurodevelopmental assessments at 2 years compared with cases without LD variants, and this difference is similar in isolated and complex cases. CONCLUSION: We found that the LD genetic variants are associated with higher mortality, worse pulmonary hypertension, and worse neurodevelopment outcomes compared with non-LD variants. Our results have important implications for prognosis, potential intervention and long-term follow up for children with CDH.
Asunto(s)
Hernias Diafragmáticas Congénitas , Niño , Hernias Diafragmáticas Congénitas/genética , Humanos , Recién Nacido , Estudios RetrospectivosAsunto(s)
COVID-19 , Enfermedades Fetales , Terapias Fetales , Control de Infecciones/métodos , Procedimientos Quirúrgicos Obstétricos/tendencias , Perinatología , COVID-19/epidemiología , COVID-19/prevención & control , Femenino , Enfermedades Fetales/diagnóstico , Enfermedades Fetales/epidemiología , Enfermedades Fetales/cirugía , Terapias Fetales/métodos , Terapias Fetales/tendencias , Transfusión Feto-Fetal/cirugía , Humanos , Innovación Organizacional , Perinatología/organización & administración , Perinatología/tendencias , Embarazo , Tiempo de TratamientoRESUMEN
BACKGROUND: Congenital diaphragmatic hernia (CDH) occurs in 1 out of 2500-3000 live births. Right-sided CDHs (R-CDHs) comprise 25% of all CDH cases, and data are conflicting on outcomes of these patients. The aim of our study was to compare outcomes in patients with right versus left CDH (L-CDH). METHODS: We analyzed a multicenter prospectively enrolled database to compare baseline characteristics and outcomes of neonates enrolled from January 2005 to January 2019 with R-CDH vs. L-CDH. RESULTS: A total of 588, 495 L-CDH, and 93 R-CDH patients with CDH were analyzed. L-CDHs were more frequently diagnosed prenatally (p=0.011). Lung-to-head ratio was similar in both cohorts. R-CDHs had a lower frequency of primary repair (p=0.022) and a higher frequency of need for oxygen at discharge (p=0.013). However, in a multivariate analysis, need for oxygen at discharge was no longer significantly different. There were no differences in long-term neurodevelopmental outcomes assessed at two year follow up. There was no difference in mortality, need for ECMO, pulmonary hypertension, or hernia recurrence. CONCLUSION: In this large series comparing R to L-CDH patients, we found no significant difference in mortality, use of ECMO, or pulmonary complications. Our study supports prior studies that R-CDHs are relatively larger and more often require a patch or muscle flap for repair. TYPE OF STUDY: Prognosis study LEVEL OF EVIDENCE: Level II.
Asunto(s)
Hernias Diafragmáticas Congénitas , Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/epidemiología , Hernias Diafragmáticas Congénitas/mortalidad , Hernias Diafragmáticas Congénitas/terapia , Humanos , Hipertensión Pulmonar , Recién Nacido , Estudios RetrospectivosRESUMEN
The growth of the field of fetal surgery over the last two decades driven by new indications and data from prospective randomized trials supporting prenatal intervention has resulted in techniques protocols and methodologies that have gained confidence by insuring good outcomes. Error traps are methods or techniques that usually work well in most of the cases, but which are apt to fail under certain specific circumstances. The very confidence the surgeon develops in these techniques or methodologies makes them a trap for the unwary surgeon. The purpose of this article is to discuss common error traps in fetal interventions, including ultrasound guided procedures, fetoscopic surgery, open fetal surgery and EXIT procedures. Awareness of these error traps and approaches to avoid them may enhance fetal surgical outcomes and reduce complications rates.
Asunto(s)
Enfermedades Fetales/cirugía , Fetoscopía/normas , Coagulación con Láser/normas , Errores Médicos , Ablación por Radiofrecuencia/normas , Procedimientos Quirúrgicos Operativos/normas , Ultrasonografía Prenatal/normas , Femenino , Fetoscopía/métodos , Humanos , Coagulación con Láser/métodos , Embarazo , Ablación por Radiofrecuencia/métodos , Procedimientos Quirúrgicos Operativos/métodos , Ultrasonografía Prenatal/métodosRESUMEN
INTRODUCTION: The use of perioperative tocolytic agents in fetal surgery is imperative to prevent preterm labor. Indomethacin, a well-known tocolytic agent, can cause ductus arteriosus (DA) constriction. We sought to determine whether a relationship exists between preoperative indomethacin dosing and fetal DA constriction. MATERIALS AND METHODS: This is an IRB-approved, single-center retrospective observational case series of 42 pregnant mothers who underwent open fetal myelomeningocele repair. Preoperatively, mothers received either 1 (QD) or 2 (BID) indomethacin doses. Maternal anesthetic drug exposures and fetal cardiac dysfunction measures were collected from surgical and anesthesia records and intraoperative fetal echocardiography. Pulsatility Index was used to calculate DA constriction severity. Comparative testing between groups was performed using t- and chi-square testing. RESULTS: DA constriction was observed in all fetuses receiving BID indomethacin and in 71.4% of those receiving QD dosing (p = 0.0002). Severe DA constriction was observed only in the BID group (35.7%). QD indomethacin group received more intraoperative magnesium sulfate (p < 0.0001). Minimal fetal cardiac dysfunction (9.5%) and bradycardia (9.5%) were observed in all groups independent of indomethacin dosing. CONCLUSIONS: DA constriction was the most frequent and severe in the BID indomethacin group. QD indomethacin and greater magnesium sulfate dosing was associated with reduced DA constriction.
Asunto(s)
Antiinflamatorios no Esteroideos/administración & dosificación , Conducto Arterial/cirugía , Terapias Fetales/métodos , Indometacina/administración & dosificación , Meningomielocele/cirugía , Tocolíticos/administración & dosificación , Constricción , Relación Dosis-Respuesta a Droga , Conducto Arterial/diagnóstico por imagen , Conducto Arterial/efectos de los fármacos , Femenino , Humanos , Meningomielocele/diagnóstico por imagen , Meningomielocele/tratamiento farmacológico , Embarazo , Estudios Retrospectivos , Ultrasonografía Prenatal/métodosRESUMEN
Congenital diaphragmatic hernia (CDH) is a severe birth defect that is often accompanied by other congenital anomalies. Previous exome sequencing studies for CDH have supported a role of de novo damaging variants but did not identify any recurrently mutated genes. To investigate further the genetics of CDH, we analyzed de novo coding variants in 362 proband-parent trios including 271 new trios reported in this study. We identified four unrelated individuals with damaging de novo variants in MYRF (P = 5.3x10(-8)), including one likely gene-disrupting (LGD) and three deleterious missense (D-mis) variants. Eight additional individuals with de novo LGD or missense variants were identified from our other genetic studies or from the literature. Common phenotypes of MYRF de novo variant carriers include CDH, congenital heart disease and genitourinary abnormalities, suggesting that it represents a novel syndrome. MYRF is a membrane associated transcriptional factor highly expressed in developing diaphragm and is depleted of LGD variants in the general population. All de novo missense variants aggregated in two functional protein domains. Analyzing the transcriptome of patient-derived diaphragm fibroblast cells suggest that disease associated variants abolish the transcription factor activity. Furthermore, we showed that the remaining genes with damaging variants in CDH significantly overlap with genes implicated in other developmental disorders. Gene expression patterns and patient phenotypes support pleiotropic effects of damaging variants in these genes on CDH and other developmental disorders. Finally, functional enrichment analysis implicates the disruption of regulation of gene expression, kinase activities, intra-cellular signaling, and cytoskeleton organization as pathogenic mechanisms in CDH.
Asunto(s)
Variación Genética , Hernias Diafragmáticas Congénitas/genética , Proteínas de la Membrana/genética , Mutación , Factores de Transcripción/genética , Preescolar , Variaciones en el Número de Copia de ADN , Discapacidades del Desarrollo/genética , Femenino , Cardiopatías Congénitas/genética , Hernias Diafragmáticas Congénitas/metabolismo , Humanos , Recién Nacido , Estudios Longitudinales , Masculino , Proteínas de la Membrana/metabolismo , Mutación Missense , Fenotipo , Análisis de Secuencia de ARN , Síndrome , Factores de Transcripción/metabolismo , Secuenciación del Exoma , Secuenciación Completa del GenomaRESUMEN
Intrauterine growth restriction (IUGR) in premature newborns increases the risk for bronchopulmonary dysplasia, a chronic lung disease characterized by disrupted pulmonary angiogenesis and alveolarization. We previously showed that experimental IUGR impairs angiogenesis; however, mechanisms that impair pulmonary artery endothelial cell (PAEC) function are uncertain. The NF-κB pathway promotes vascular growth in the developing mouse lung, and we hypothesized that IUGR disrupts NF-κB-regulated proangiogenic targets in fetal PAEC. PAECs were isolated from the lungs of control fetal sheep and sheep with experimental IUGR from an established model of chronic placental insufficiency. Microarray analysis identified suppression of NF-κB signaling and significant alterations in extracellular matrix (ECM) pathways in IUGR PAEC, including decreases in collagen 4α1 and laminin α4, components of the basement membrane and putative NF-κB targets. In comparison with controls, immunostaining of active NF-κB complexes, NF-κB-DNA binding, baseline expression of NF-κB subunits p65 and p50, and LPS-mediated inducible activation of NF-κB signaling were decreased in IUGR PAEC. Although pharmacological NF-κB inhibition did not affect angiogenic function in IUGR PAEC, angiogenic function of control PAEC was reduced to a similar degree as that observed in IUGR PAEC. These data identify reductions in endothelial NF-κB signaling as central to the disrupted angiogenesis observed in IUGR, likely by impairing both intrinsic PAEC angiogenic function and NF-κB-mediated regulation of ECM components necessary for vascular development. These data further suggest that strategies that preserve endothelial NF-κB activation may be useful in lung diseases marked by disrupted angiogenesis such as IUGR.
Asunto(s)
Displasia Broncopulmonar , Células Endoteliales , Retardo del Crecimiento Fetal , Subunidad p50 de NF-kappa B/metabolismo , Arteria Pulmonar , Transducción de Señal , Factor de Transcripción ReIA/metabolismo , Animales , Displasia Broncopulmonar/inducido químicamente , Displasia Broncopulmonar/embriología , Displasia Broncopulmonar/patología , Displasia Broncopulmonar/fisiopatología , Células Endoteliales/metabolismo , Células Endoteliales/patología , Femenino , Retardo del Crecimiento Fetal/inducido químicamente , Retardo del Crecimiento Fetal/metabolismo , Retardo del Crecimiento Fetal/patología , Retardo del Crecimiento Fetal/fisiopatología , Lipopolisacáridos/toxicidad , Embarazo , Arteria Pulmonar/embriología , Arteria Pulmonar/patología , Arteria Pulmonar/fisiopatología , OvinosRESUMEN
OBJECTIVE: We reviewed our experience with open fetal surgical myelomeningocele repair to assess the efficacy of a new modification of the hysterotomy closure technique regarding hysterotomy complication rates at the time of cesarean delivery. METHODS: A modification of the standard hysterotomy closure was performed on all patients undergoing prenatal myelomeningocele repair. The closure consisted of an interrupted full-thickness #0 polydioxanone (PDS) retention suture as well as a running #0 PDS suture to re-approximate the myometrial edges, and the modification was a third imbricating layer resulting in serosal-to-serosal apposition. A standard omental patch was placed per our routine. Both operative reports and verbal descriptions of hysterotomy from delivering obstetricians were reviewed. RESULTS: A total of 49 patients underwent prenatal repair of myelomeningocele, 43 having adequate follow-up for evaluation. Of those, 95.4% had completely intact hysterotomy closures, with only 1 partial dehiscence (2.3%) and 1 thinned scar (2.3%). There were no instances of uterine rupture. DISCUSSION: In patients undergoing this modified hysterotomy closure technique, a much lower than expected complication rate was observed. This simple modified closure technique may improve hysterotomy healing and reduce obstetric morbidity.
Asunto(s)
Fetoscopía/métodos , Histerotomía/métodos , Meningomielocele/diagnóstico , Meningomielocele/cirugía , Atención Prenatal/métodos , Adulto , Femenino , Estudios de Seguimiento , Humanos , Embarazo , Estudios RetrospectivosRESUMEN
OBJECTIVE: To assess the impact of specific echocardiographic criteria for timing of congenital diaphragmatic hernia repair on the incidence of acute postoperative clinical decompensation from pulmonary hypertensive crisis and/or acute respiratory decompensation, with secondary outcomes including survival to discharge, duration of ventilator support, and length of hospitalization. STUDY DESIGN: The multidisciplinary congenital diaphragmatic hernia management team instituted a protocol in 2012 requiring the specific criterion of echocardiogram-estimated pulmonary artery pressure ≤80% systemic blood pressure before repairing congenital diaphragmatic hernias. A retrospective review of 77 neonatal patients with Bochdalek hernias repaired between 2008 and 2015 were reviewed: group 1 included patients repaired before protocol implementation (n = 25) and group 2 included patients repaired after implementation (n = 52). RESULTS: The groups had similar baseline characteristics. Postoperative decompensation occurred less often in group 2 compared with group 1 (17% vs 48%, P = .01). Adjusted analysis accounting for repair type, liver herniation, and prematurity yielded similar results (15% vs 37%, P = .04). Group 2 displayed a trend toward improved survival to 30 days postoperatively, though this did not reach statistical significance (94% vs 80%, P = .06). Patient survival to discharge, duration of ventilator support, and length of hospitalization were not different between groups. CONCLUSIONS: The implementation of a protocol requiring echocardiogram-estimated pulmonary arterial pressure ≤80% of systemic pressure before congenital diaphragmatic hernia repair may reduce the incidence of acute postoperative decompensation, although there was no difference in longer-term secondary outcomes, including survival to discharge.
Asunto(s)
Toma de Decisiones Clínicas/métodos , Ecocardiografía , Hernias Diafragmáticas Congénitas/diagnóstico por imagen , Herniorrafia , Cuidados Preoperatorios/métodos , Presión Arterial , Determinación de la Presión Sanguínea , Protocolos Clínicos , Femenino , Hernias Diafragmáticas Congénitas/fisiopatología , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Recién Nacido , Tiempo de Internación/estadística & datos numéricos , Modelos Logísticos , Masculino , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/prevención & control , Estudios Retrospectivos , Factores de Tiempo , Resultado del TratamientoRESUMEN
Fetuses with anti-SSA-mediated complete atrioventricular block (CAVB) are at high risk for perinatal death if they present at <20 weeks of gestation and develop ventricular rates of <55 beats per minute (bpm), cardiac dysfunction, or hydrops [Izmirly et al.: Circulation 2011;124:1927-1935; Jaeggi et al.: J Am Coll Cardiol 2002;39:130-137; Eliasson et al.: Circulation 2011;124:1919-1926]. After our experience with two such fetuses who died with pulseless electrical activity despite being paced within 30 min of birth, we performed an ex utero intrapartum treatment procedure to ventricular pacing on a 36-week CAVB fetus with cardiac dysfunction, mild hydrops, and a ventricular rate of 46 bpm. While still on placental bypass, temporary epicardial ventricular pacing leads were successfully placed; the infant was delivered and made a successful transition to postnatal life. This approach can improve the 11-fold increase in mortality for the preterm fetus with long-standing CAVB, severe bradycardia, and heart failure.
Asunto(s)
Bloqueo Atrioventricular/cirugía , Bradicardia/cirugía , Enfermedades Fetales/cirugía , Femenino , Humanos , Embarazo , Adulto JovenRESUMEN
Intraperitoneal amniotic fluid leak is a known complication of fetoscopic procedures that usually resolves spontaneously with expectant management. Intraperitoneal amniotic fluid leak may persist after fetoscopic procedures due to a myometrial window as well as to persistent chorioamniotic membrane disruption, which may be amenable to surgical repair.
RESUMEN
Intraocular teratomas are rare neoplasms with only three previously reported cases. We present the fourth case of intraocular teratoma and the second associated with sacrococcygeal teratoma. While the nature of the association between intraocular teratomas and sacrococcygeal teratomas is unclear, it suggests a need for careful ophthalmologic follow-up of infants with congenital sacrococcygeal teratomas.
Asunto(s)
Neoplasias Óseas/diagnóstico , Neoplasias de la Coroides/diagnóstico , Cóccix , Enfermedades del Prematuro/diagnóstico , Sacro , Teratoma/diagnóstico , Neoplasias Óseas/congénito , Neoplasias de la Coroides/congénito , Femenino , Humanos , Recién Nacido , Recien Nacido Prematuro , Teratoma/congénitoRESUMEN
More than 3 decades ago, a small group of physicians and other practitioners active in what they called "fetal treatment" authored an opinion piece outlining the current status and future challenges anticipated in the field. Many advances in maternal, neonatal, and perinatal care and diagnostic and therapeutic modalities have been made in the intervening years, yet a thoughtful reassessment of the basic tenets put forth in 1982 has not been published. The present effort will aim to provide a framework for contemporary redefinition of the field of fetal treatment, with a brief discussion of the necessary minimum expertise and systems base for the provision of different types of interventions for both the mother and fetus. Our goal will be to present an opinion that encourages the advancement of thoughtful practice, ensuring that current and future patients have realistic access to centers with a range of fetal therapies with appropriate expertise, experience, and subspecialty and institutional support while remaining focused on excellence in care, collaborative scientific discovery, and maternal autonomy and safety.