RESUMEN
OBJECTIVES: In gastroschisis, the intestines are exposed to amniotic fluid during pregnancy. The defect in the abdominal wall may also compress the mesentery and impair the intestinal blood supply. There is a varying degree of intestinal damage at birth. Complex gastroschisis is defined as gastroschisis with severe complications such as intestinal atresia, volvulus, necrosis and perforation. The aim of this study was to investigate long-term gastrointestinal morbidity and compare simple and complex cases. METHODS: A nation-wide retrospective cohort study with data from national registers was conducted. All children born with gastroschisis in Sweden from 1 January 1997 to 31 December 2016 were included in the study. Exposure was complex gastroschisis and the primary outcomes were intestinal failure and bowel obstruction. RESULTS: The study included 315 cases with gastroschisis, 260 classifieds as simple gastroschisis and 55 as complex. The median time to follow was 8 years. A significantly higher risk of developing intestinal failure (hazard ratio: 11.7) was found in complex cases. Nine percent of the complex cases underwent autologous gastrointestinal reconstructive surgery for intestinal failure, none of the simple cases did. The complex cases had a higher risk for bowel obstruction (hazard ratio: 4.3) with a higher proportion requiring surgery (18.2% vs. 6.9%) compared to simple cases. CONCLUSIONS: This nationwide study showed that the risk for intestinal failure and bowel obstruction is significantly higher for children with complex gastroschisis compared to simple gastroschisis. Most of the events occurred during the first 2 years of life.
RESUMEN
Introduction: The role of cardiac left ventricle (LV) dysfunction in children with congenital diaphragmatic hernia (CDH) has gained increasing attention. The hernia allows abdominal mass to enter thorax and subsequently both dislocating and compressing the heart. The pressure on vessels and myocardium alters blood flow and may interfere with normal development of the LV. A dysfunctional LV is concerning and impacts the complex pathophysiology of CDH. Hence, assessing both the systolic and diastolic LV function in the newborn with CDH is important, and it may add value for medical treatment and prognostic factors as length of stay (LOS) in pediatric intensive care unit (PICU). LV strain is considered an early marker of systolic dysfunction used in the pediatric population. Left atrial (LA) strain is an echocardiographic marker of LV diastolic dysfunction used in the adult population. When filling pressure of the LV increases, the strain of the atrial wall is decreased. We hypothesized that reduced LA strain and LV strain are correlated with the LOS in the PICU of newborns with CDH. Methods: This retrospective observational cohort study included data of 55 children born with CDH between 2018 and 2020 and treated at Karolinska University Hospital, Sweden. Overall, 46 parents provided consent. Echocardiograms were performed in 35 children <72â h after birth. The LA reservoir strain (LASr), LV global longitudinal strain, LV dimensions, and direction of blood flow through the patent foramen ovale (PFO) were retrospectively assessed using the echocardiograms. Results: Children with LASr <33% (n = 27) had longer stays in the PICU than children with LA strain ≥33% (n = 8) (mean: 20.8 vs. 8.6 days; p < 0.002). The LASr was correlated with the LOS in the PICU (correlation coefficient: -0.378; p = 0.025). The LV dimension was correlated with the LOS (correlation coefficient: -0.546; p = 0.01). However, LV strain was not correlated to LOS. Conclusion: Newborns with CDH and a lower LASr (<33%) had longer stays in the PICU than children with LASr ≥33%. LASr is a feasible echocardiographic marker of diastolic LV dysfunction in newborns with CDH and may indicate the severity of the condition.
RESUMEN
OBJECTIVE: Congenital Diaphragmatic Hernia (CDH) associated with hydrops is rare. The aim of this study was to describe the incidence of this combination of anomalies and the postnatal outcomes from a large database for CDH. STUDY DESIGN: Data from the multicenter, multinational database on infants with prenatally diagnosed CDH (CDHSG Registry) born from 2015 to 2021 were analyzed. RESULTS: A total of 3985 patients were entered in the registry during the study period, 3156 were prenatally diagnosed and 88 were reported to have associated fluid in at least 1 compartment, representing 2.8% of all prenatally diagnosed CDH cases in the registry. The overall survival to discharge for CDH patients with hydrops was 43%. The hydropic CDH group had lower birth weight and gestational age at birth, and increased incidence of right-sided CDH (55%), and rate of non-repair (45%). However, the survival rate for hydropic infants with CDH undergoing surgical repair was 80%. Other associated anomalies were more common in hydropic CDH (50% vs 37%, p = 0.001). CONCLUSION: Hydropic CDH is rare, only 2.8% of all prenatally diagnosed cases, and more commonly occurring in right-sided CDH. Survival rates are low, with higher rates of non-repair. However, decision-making regarding goals of care and an aggressive surgical approach in selected cases may result in survival rates comparable to non-hydropic cases.
RESUMEN
Congenital diaphragmatic hernia (CDH) is a severe birth defect frequently associated with pulmonary hypoplasia, pulmonary hypertension, and heart failure. Since amniotic fluid comprises proteins of both fetal and maternal origin, its analysis could provide insights on mechanisms underlying CDH and provide biomarkers for early diagnosis, severity of pulmonary changes and treatment response. The study objective was to identify proteomic changes in amniotic fluid consistently associated with CDH. Amniotic fluid was obtained at term (37-39 weeks) from women with normal pregnancies (n = 5) or carrying fetuses with CDH (n = 5). After immuno-depletion of the highest abundance proteins, off-line fractionation and high-resolution tandem mass spectrometry were performed and quantitative differences between the proteomes of the groups were determined. Of 1036 proteins identified, 218 were differentially abundant. Bioinformatics analysis showed significant changes in GP6 signaling, in the MSP-RON signaling in macrophages pathway and in networks associated with cardiovascular system development and function, connective tissue disorders and dermatological conditions. Differences in selected proteins, namely pulmonary surfactant protein B, osteopontin, kallikrein 5 and galectin-3 were validated by orthogonal testing using ELISA in larger cohorts and showed statistically significant differences aiding in the diagnosis and prediction of CDH. The findings provide potential tools for clinical management of CDH.
Asunto(s)
Hernias Diafragmáticas Congénitas , Embarazo , Humanos , Femenino , Líquido Amniótico , Proteómica , Proteoma , BiomarcadoresRESUMEN
AIM OF THE STUDY: Children with omphalocele have an increased prevalence of Beckwith Wiedemann syndrome (BWS) and thus a suspected increased risk of developing embryonal tumors, e.g. Wilms tumor, hepatoblastoma, neuroblastoma and rhabdomyosarcoma. The aim of this study was to examine the prevalence of BWS and the risk of embryonal tumors amongst patients born with omphalocele. METHODS: A population-based cohort was used, including all children born in Sweden 1/1 1997-31/12 2016. Patients with omphalocele were identified through the Swedish National Patient Register and the Swedish Medical Birth Register. For each case of omphalocele ten age and sex matched individuals unexposed for omphalocele were randomly selected for comparison. Data on BWS and embryonal tumors were collected from the Swedish National Patient Register and the Swedish National Cancer Register. MAIN RESULTS: Out of 207 cases of omphalocele, 15 (7.2%) were diagnosed with BWS. None of the children with omphalocele had yet developed any kind of embryonal tumor (median follow-up time 8 years). None of the 2070 controls were diagnosed with BWS but 3 (0.1%) of them had developed embryonal tumors during a median follow-up time of 10 years. CONCLUSIONS: In this study the prevalence of BWS amongst children born with omphalocele is in the lower range of previously reported figures. Also, the prevalence of embryonal tumors amongst children with BWS is lower than expected and the risk of embryonal tumors in children with omphalocele and BWS might not be as high as previously stated. This must be taken into consideration when counseling parents prenatally. TYPE OF STUDY: National register cohort study. LEVEL OF EVIDENCE: II.
RESUMEN
OBJECTIVE: To evaluate the impact of repeat extracorporeal life support (ECLS) on survival and in-hospital outcomes in the congenital diaphragmatic hernia (CDH) neonates. BACKGROUND: Despite the widespread use of ECLS, investigations on multiple ECLS courses for CDH neonates are limited. METHODS: This is a retrospective cohort study of all ECLS-eligible CDH neonates enrolled in the Congenital Diaphragmatic Hernia Study Group registry between 1995 and 2019. CDH infants with estimated gestational age at birth <32 weeks and a birth weight <1.8 kg and/or with major cardiac or chromosomal anomalies were excluded. The primary outcomes were survival and morbidities during the index hospitalization. RESULTS: Of 10,089 ECLS-eligible CDH infants, 3025 (30%) received 1 ECLS course, and 160 (1.6%) received multiple courses. The overall survival rate for patients who underwent no ECLS, 1 ECLS course, and multicourse ECLS were 86.9±0.8%, 53.8±1.8%, and 43.1±7.7%, respectively. Overall ECLS survival rate is increased by 5.1±4.6% ( P =0.03) for CDH neonates treated at centers that conduct repeat ECLS compared with those that do not offer repeat ECLS. This suggests that there would be an overall survival benefit from increased use of multiple ECLS courses. Infants who did not receive ECLS support had the lowest morbidity risk, while survivors of multicourse ECLS had the highest rates of morbidities during the index hospitalization. CONCLUSIONS: Although survival is lower for repeat ECLS, the use of multiple ECLS courses has the potential to increase overall survival for CDH neonates. Increased use of repeat ECLS might be associated with improved survival. The potential survival advantage of repeat ECLS must be balanced against the increased risk of morbidities during the index hospitalization.
Asunto(s)
Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas , Recién Nacido , Humanos , Hernias Diafragmáticas Congénitas/terapia , Estudios Retrospectivos , Tasa de Supervivencia , HospitalesRESUMEN
INTRODUCTION: The optimal timing of delivery for pregnancies complicated by prenatally diagnosed gastroschisis remains controversial. Therefore, the aim of this study was to find whether elective or expectant delivery is associated with improved neonatal outcome. MATERIALS AND METHODS: MEDLINE and Embase databases were searched for studies up to 2021 that reported timing of delivery for prenatally diagnosed gastroschisis. A systematic review and meta-analysis were then performed in group 1: moderately preterm (gestational age [GA]: 34-35 weeks) elective delivery versus expectant management after GA 34-35 weeks; and group 2: near-term (GA: 36-37 weeks) elective delivery versus expectant management after GA 36-37 weeks. The following clinical outcomes were evaluated: length of stay (LOS), total parenteral nutrition (TPN) days, bowel morbidity (atresia, perforation, and volvulus), sepsis, time of first feeding, short gut syndrome and respirator days, and mortality. RESULTS: Two randomized controlled trials (RCT)s and eight retrospective cohort studies were included, comprising 629 participants. Moderately preterm elective delivery failed to improve clinical outcomes. However, near-term elective delivery significantly reduced bowel morbidity (7.4 vs. 15.4%, relative risk = 0.37; 95% confidence interval [CI]: 0.18, 0.74; p = 0.005; I2 = 0%) and TPN days (mean difference =-13.44 days; 95% CI: -26.68, -0.20; p = 0.05; I2 = 45%) compared to expectant delivery. The mean LOS was 39.2 days after near-term delivery and 48.7 days in the expectant group (p = 0.06). CONCLUSION: Based on the data analyzed, near-term elective delivery (GA 36-37 weeks) appears to be the optimal timing for delivery of pregnancies complicated by gastroschisis as it is associated with less bowel morbidity and shorter TPN days. However, more RCTs are necessary to better validate these findings.
Asunto(s)
Gastrosquisis , Embarazo , Recién Nacido , Femenino , Humanos , Lactante , Gastrosquisis/cirugía , Gastrosquisis/complicaciones , Espera Vigilante , Edad GestacionalRESUMEN
INTRODUCTION: The global increase of individuals born by cesarean section with reported levels up to 20% of all deliveries, makes it important to study cesarean section and possible associations that can increase risk of subsequent diseases in children. The aim of the study was to evaluate if cesarean section is associated with increased risk of gastrointestinal disease later in life in a large population-based cohort. MATERIAL AND METHODS: In this national population-based cohort study including all full-term individuals registered in the Medical Birth Register in Sweden between 1990 and 2000, type of delivery (exposure) was collected from the Medical Birth Register. The study population was followed until 2017 with regards to the outcomes: inflammatory bowel disease (Crohn's disease or ulcerative colitis), appendicitis, cholecystitis, or diverticulitis registered in the Swedish National Patient Register. Cox proportional-hazards models compared disease-free survival time between exposed and unexposed. RESULTS: The final study population consisted of 1 102 468 individuals of whom 11.6% were delivered by cesarean section and 88.4% were vaginally delivered. In univariate analysis, cesarean section was associated with Crohn's disease (hazard ratio [HR] 1.13, 95% confidence interval [CI] 1.02-1.25), diverticulosis (HR 1.57, 95% CI 1.13-2.18), and cholecystitis (HR 1.16, 95% CI 1.05-1.28). However, the increased risk only remained for Crohn's disease after adjustment for confounders (HR 1.14, 95% CI 1.02-1.27). No associations between delivery mode and appendicitis, ulcerative colitis, cholecystitis, or diverticulosis were found in the multivariate analysis. CONCLUSIONS: Cesarean section is associated with Crohn's disease later in life, but no other association between delivery mode and gastrointestinal disorders later in life could be found.
Asunto(s)
Apendicitis , Colecistitis , Colitis Ulcerosa , Enfermedad de Crohn , Divertículo , Cesárea/efectos adversos , Niño , Colecistitis/epidemiología , Colecistitis/etiología , Estudios de Cohortes , Colitis Ulcerosa/complicaciones , Colitis Ulcerosa/epidemiología , Enfermedad de Crohn/complicaciones , Divertículo/complicaciones , Femenino , Humanos , Embarazo , Factores de RiesgoRESUMEN
Consensus on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) is lacking, and comparison between studies remains difficult due to a large variety in outcome measures. We aimed to define a core outcome set (COS) for pediatric patients with an asymptomatic CPAM. An online, three-round Delphi survey was conducted in two stakeholder groups of specialized caregivers (surgeons and non-surgeons) in various European centers. Proposed outcome parameters were scored according to level of importance, and the final COS was established through consensus. A total of 55 participants (33 surgeons, 22 non-surgeons) from 28 centers in 13 European countries completed the three rounds and rated 43 outcome parameters. The final COS comprises seven outcome parameters: respiratory insufficiency, surgical complications, mass effect/mediastinal shift (at three time-points) and multifocal disease (at two time-points). The seven outcome parameters included in the final COS reflect the diversity in priorities among this large group of European participants. However, we recommend the incorporation of these outcome parameters in the design of future studies, as they describe measurable and validated outcomes as well as the accepted age at measurement.
RESUMEN
PURPOSE: The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim of this study was to examine the national Swedish birth prevalence and survival rates among these patients. METHODS: This study is based on a nationwide population-based cohort of all children born in Sweden between 1/1/1997 and 31/12/2016. All omphalocele cases were identified though the Swedish National Patient Register and the Swedish Medical Birth Register. Outcome of malformations and deaths were retrieved from the Swedish Birth Defects Register and the Swedish Causes of Death Register. RESULTS: The study included 207 cases of omphalocele (42% females). The birth prevalence for omphalocele was 1/10,000 live births. About 62% of the cases had associated malformations and/or genetic disorders; most common was ventricular septal defect. The mortality within the first year was 13%. The rate of termination of pregnancy was 59%. CONCLUSION: The national birth prevalence for omphalocele in Sweden is 1/10,000 newborn, with high termination rates. Over half of the pregnancies with prenatally diagnosed omphalocele will be terminated. Among those who continue the pregnancy, 1-year survival rates are high. TYPE OF STUDY: National register study LEVEL OF EVIDENCE: III.
Asunto(s)
Hernia Umbilical , Niño , Femenino , Hernia Umbilical/epidemiología , Humanos , Recién Nacido , Masculino , Tamizaje Masivo , Embarazo , Diagnóstico Prenatal , Prevalencia , Suecia/epidemiologíaRESUMEN
AIM: An increased incidence has been reported for the congenital abdominal wall defect gastroschisis. The reason for this increasing trend is not known, nor the aetiology. The aim of this study was to examine the national birth prevalence in Sweden, the termination rate, associated anomalies and the mortality of gastroschisis within the cohort. METHODS: A nationwide, population-based descriptive study of children born with gastroschisis in Sweden between 1/1 1997 and 31/12 2016 was conducted. The cohort was collected from the Swedish Medical Birth Register and the Swedish National Patient Register. Several other national registers were then interlinked to identify outcome data. RESULTS: The study included 361 cases of gastroschisis, 54% female. The birth prevalence was 1.52 in 10,000 live births. The termination rate was 21%. The mortality within the cohort was 4.4% with a 1-year mortality of 3.9%. Most frequent associated anomalies were gastrointestinal (11.4%), musculoskeletal (9.8%) and cardiovascular anomalies (7.9%). CONCLUSION: During the 20-year study period, a stable birth prevalence of 1.52 per 10 000 live births was seen in Sweden. The mortality was low, 4.4%, but the termination of pregnancies was high, 21%. Almost one-third had associated congenital anomalies where gastrointestinal anomalies were the most common.
Asunto(s)
Gastrosquisis , Niño , Femenino , Gastrosquisis/epidemiología , Humanos , Incidencia , Masculino , Parto , Embarazo , Prevalencia , Suecia/epidemiologíaRESUMEN
INTRODUCTION: A worldwide lack of consensus exists on the optimal management of asymptomatic congenital pulmonary airway malformation (CPAM) even though the incidence is increasing. Either a surgical resection is performed or a wait-and-see policy is employed, depending on the treating physician. Management is largely based on expert opinion and scientific evidence is scarce. Wide variations in outcome measures are seen between studies making comparison difficult thus highlighting the lack of universal consensus in outcome measures as well. We aim to define a core outcome set which will include the most important core outcome parameters for paediatric patients with an asymptomatic CPAM. METHODS AND ANALYSIS: This study will include a critical appraisal of the current literature followed by a three-stage Delphi process with two stakeholder groups. One surgical group including paediatric as well as thoracic surgeons, and a non-surgeon group including paediatric pulmonologists, intensive care and neonatal specialists. All participants will score outcome parameters according to their level of importance and the most important parameters will be determined by consensus. ETHICS AND DISSEMINATION: Electronic informed consent will be obtained from all participants. Ethical approval is not required. After the core outcome set has been defined, we intend to design an international randomised controlled trial: the COllaborative Neonatal NEtwork for the first CPAM Trial, which will be aimed at determining the optimal management of patients with asymptomatic CPAM.
Asunto(s)
Evaluación de Resultado en la Atención de Salud , Investigadores , Niño , Consenso , Técnica Delphi , Humanos , Recién Nacido , Proyectos de Investigación , Encuestas y CuestionariosRESUMEN
AIM: To evaluate the results in CDH patients subjected to a second course of ECMO at a single institution. MATERIAL AND METHODS: Retrospective review of medical charts of patients treated for CDH and ECMO in our center since 1990 to December 2018 was performed. For patients subjected to a second course of ECMO and who survived to hospital discharge charts from follow up visits were also reviewed. RESULTS AND DISCUSSION: From Jan 1990 until December 2018, 311 patients with CDH were treated in the department. 267 of these (86%) were discharged alive from the hospital and 81% (237/293) of the Swedish patients were alive by December 2018. 101 patients (32%) were subjected to ECMO treatment of whom 71 survived (70%). 22 patients underwent a second ECMO run and 13 of these survived to hospital discharge. Seven of the Swedish patients [19] were long-term survivors (37%). The vast majority was on V-A ECMO. CONCLUSIONS: It is possible to recannulate the right common carotid artery and internal jugular vein for a second course of venoarterial ECMO in CDH patients, who deteriorate severely after decannulation. Previous research has shown that long-term survivors subjected to ECMO twice reported similar frequencies of pulmonary, gastrointestinal, neurological and musculoskeletal sequelae as the long-term survivors, who needed ECMO support only once, and similar health-related quality of life. Regarding their psychosocial function, they scored within normal range in the behavioral, emotional and social scales domains. A second ECMO run may contribute to a higher survival and that the long-term morbidity among survivors is not more pronounced than among survivors after a single course of ECMO. It is therefore suggested that a second course of ECMO should be offered on the same indications as the first course. LEVEL OF EVIDENCE: III Case series.
Asunto(s)
Oxigenación por Membrana Extracorpórea , Hernias Diafragmáticas Congénitas , Cateterismo , Hernias Diafragmáticas Congénitas/terapia , Humanos , Calidad de Vida , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
BACKGROUND: Neurodevelopmental dysfunction is one of the most disabling outcomes for congenital diaphragmatic hernia (CDH) survivors and may have a long lasting impact in adult life. AIM: To evaluate to which extent being born with CDH has an impact on the educational level and socioeconomic status as a proxy for neurocognitive development. MATERIAL AND METHODS: Nationwide, population-based prospective study of newborn children in Sweden from 1982 to 2015. School grades, highest educational level and income were assessed through Swedish public registries. Children above 15â¯years of age with CDH were compared with randomly selected controls. RESULTS: A significantly higher number of cases (17% vs 10%) did achieve neither a school nor a university degree. Among those who achieved a degree there was no difference in the highest level of education. The qualification points in elementary school did not differ, but in high school female cases had significantly lower qualification points than female controls. There were no differences in individual disposable income between cases and controls. However, males had higher income compared to females. Prematurity and a long hospital stay had a negative impact on educational level. CONCLUSIONS: A higher proportion of children born with CDH compared to controls do not achieve a school degree. Among those who achieved a degree, the school achievements and educational level were similar to controls. Prematurity and a long hospital stay are risk factors for not achieving an educational degree. TYPE OF STUDY: Prognosis study (high-quality prospective cohort study with 99% of patients followed to the study end point). LEVEL OF EVIDENCE: Level I. I for a prognosis study - This is a high-quality, prospective cohort study with 99% of patients followed to the study end point.
Asunto(s)
Escolaridad , Hernias Diafragmáticas Congénitas , Clase Social , Éxito Académico , Adolescente , Estudios de Casos y Controles , Femenino , Hernias Diafragmáticas Congénitas/epidemiología , Humanos , Masculino , Estudios Prospectivos , Suecia/epidemiologíaRESUMEN
BACKGROUND: Congenital Diaphragmatic Hernia (CDH) associated with Omphalocele is a rare condition, and only a few case reports are available in the literature. Both conditions are associated with some degree of pulmonary hypoplasia. We hypothesize that the combination of CDH with Omphalocele might be associated with poorer outcomes. AIM: The aim of this study was to describe the incidence of this association and postnatal outcomes from the largest database available for CDH. METHODS: Data from the multicenter, multinational database on infants with CDH (CDHSG Registry) born from 2007 to 2018 was analyzed. RESULTS: A total of 5730 entries were made into the registry during the study period. The incidence of Omphalocele associated with CDH was 0.63% (36 out of 5730). When comparing posterolateral Bochdalek hernias with Omphalocele (CDHâ¯+â¯O) to CDH without Omphalocele (CDH-), CDHâ¯+â¯O were born at significantly younger gestational ages. They were sicker directly after birth with significantly lower APGARs at all time points, but received ECMO significantly less often. The distribution of left vs right side or the defect size did not differ but CDHâ¯+â¯O required patch in a significantly larger extent. CDHâ¯+â¯O had surgical repair significantly later and had significantly higher rates of non-repairs and significantly lower survival rates. The morbidity was significantly higher with longer hospital stays and higher requirements for O2 at 30 DOL. DISCUSSION: CDH associated with Omphalocele is a rare but more severe condition with higher mortality and morbidity rates. Newborns with these combined conditions can be difficult to stabilize or might pose complicated management problems due to pulmonary hypertension and/or pulmonary hypoplasia. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level I.
Asunto(s)
Hernia Umbilical , Hernias Diafragmáticas Congénitas , Hernia Umbilical/diagnóstico , Hernia Umbilical/epidemiología , Hernia Umbilical/cirugía , Hernias Diafragmáticas Congénitas/diagnóstico , Hernias Diafragmáticas Congénitas/epidemiología , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Recién Nacido , PronósticoRESUMEN
The main, but not sole, indication for an Ex-utero Intrapartum Treatment (EXIT) delivery is an airway obstruction due to either laryngeal atresia or tumors in the head and neck region. Here we present our Institution's experience with eleven cases: three teratomas, four lymphatic malformations, two laryngeal atresias and two dermoid cysts. The EXIT procedure was used to secure the fetal airway while maintaining uteroplacental gas exchange and fetal hemodynamic stability through the umbilical circulation. Five fetuses required tracheostomy. Only one fetal death occurred due to extensive growth of a teratoma preventing us from establishing an airway. No other fetal or major maternal complication occurred. The EXIT procedure is a complex procedure and these rare cases should be referred to a center with a dedicated and experienced multidisciplinary team.
Asunto(s)
Obstrucción de las Vías Aéreas , Histerotomía/métodos , Laparotomía/métodos , Adulto , Obstrucción de las Vías Aéreas/congénito , Obstrucción de las Vías Aéreas/cirugía , Cesárea , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Neoplasias de Cabeza y Cuello/congénito , Neoplasias de Cabeza y Cuello/cirugía , Hospitales Universitarios , Humanos , Recién Nacido , Intubación Intratraqueal/métodos , Enfermedades de la Laringe/congénito , Enfermedades de la Laringe/cirugía , Laringe/anomalías , Laringe/cirugía , Anomalías Linfáticas/cirugía , Grupo de Atención al Paciente , Embarazo , Resultado del Embarazo , Diagnóstico Prenatal , Estudios Retrospectivos , Región Sacrococcígea/patología , Región Sacrococcígea/cirugía , Suecia , Teratoma/congénito , Teratoma/cirugía , Traqueotomía/métodosRESUMEN
BACKGROUND: The etiology of congenital diaphragmatic hernia (CDH) remains poorly understood. We hypothesize that environmental factors play an important role in the development of CDH. AIM: The objective of this study was to investigate associated maternal risk factors in pregnancies with CDH. MATERIAL AND METHODS: The study was a nationwide, population-based prospective case-control study consisting of a cohort of newborn children entered into the records of pregnant women receiving antenatal care in Sweden, registered in the Medical Birth Registry during the period from January 1, 1982 to December 31, 2015. The study outcome CDH and the different exposures were assessed through linkage to the Swedish National Patient Registry for both cases and mothers. RESULTS: A total of 972 cases of CDH were registered into one of the national registries in Sweden between 1982 and 2015. The incidence of neonates with CDH in Sweden from 1982 to 2015 was 3/10,000 live births. The mortality rate during the study period was 31%. Maternal age, ethnicity, parity, exposure to tobacco, BMI, IVF, previous history of spontaneous abortion or intrauterine fetal demise, and coexisting chronic diseases (urinary tract infection, chronic renal disease, pregestational diabetes, epilepsy, asthma, ulcerative colitis, inflammatory bowel disease, or systemic lupus erythematous) were not associated with an increased risk of CDH in the fetus. There was a significant association between maternal hypertension and the risk of the child being affected by CDH (OR 3.32, 95% CI 1.41-7.79, p = 0.01). No association was found between preeclampsia and CDH. CONCLUSIONS: Pregestational hypertension is associated with an increased risk of giving birth to a baby with CDH, but no association was observed in pregnancies developing preeclampsia and the occurrence of CDH.
Asunto(s)
Hernias Diafragmáticas Congénitas/epidemiología , Hipertensión/epidemiología , Salud Materna , Adulto , Estudios de Casos y Controles , Femenino , Estado de Salud , Hernias Diafragmáticas Congénitas/diagnóstico , Humanos , Hipertensión/diagnóstico , Incidencia , Embarazo , Estudios Prospectivos , Sistema de Registros , Medición de Riesgo , Factores de Riesgo , Suecia/epidemiología , Factores de Tiempo , Adulto JovenRESUMEN
Advances in fetal surgery Fetal surgery is a subspeciality that is evolving rapidly with focus on improving the natural history of congenital malformations and conditions that are either life threatening or cause severe disability. Fetal surgery for myelomeningocele has been shown to improve neurologic outcome, motor function and to reduce the need of ventriculo-peritoneal shunting after birth compared to postnatal care. However, it conveys an increased risk of preterm birth and maternal morbidity. The role of prenatal intervention with endoscopic tracheal occlusion in congenital diaphragmatic hernia is currently the focus of an ongoing multicenter randomized controlled trial. The trial is comparing the effect of fetal surgery as an alternative to standard postnatal management. The main, but not sole, indication for an Ex-utero intrapartum treatment (EXIT) is airway obstruction due to laryngeal atresia and tumors in the head and neck region. It is a complex procedure that should be performed only in experienced centers with a multidisciplinary team.
Asunto(s)
Enfermedades Fetales/cirugía , Terapias Fetales/métodos , Obstrucción de las Vías Aéreas/patología , Obstrucción de las Vías Aéreas/cirugía , Femenino , Terapias Fetales/tendencias , Feto/cirugía , Neoplasias de Cabeza y Cuello/cirugía , Hernias Diafragmáticas Congénitas/patología , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Meningomielocele/cirugía , Embarazo , Resultado del TratamientoRESUMEN
This corrects the article DOI: 10.1038/ncomms15112.