RESUMO
Congenital adrenal hyperplasia (CAH) is a term that encompasses a wide range of conditions that affect the adrenals. Diagnosis and treatment before birth are important as irreparable birth defects can be avoided, decreasing the need for surgical intervention later in life, especially regarding genitalia anomalies. Although early implementation of dexamethasone in the prenatal treatment of CAH has been controversial, there is recent evidence that this treatment can reduce long-term complications.
Assuntos
Hiperplasia Suprarrenal Congênita , Dexametasona , Diagnóstico Pré-Natal , Humanos , Hiperplasia Suprarrenal Congênita/diagnóstico , Hiperplasia Suprarrenal Congênita/terapia , Feminino , Gravidez , Diagnóstico Pré-Natal/métodos , Dexametasona/uso terapêutico , Dexametasona/administração & dosagem , Terapias Fetais/métodos , Glucocorticoides/uso terapêuticoRESUMO
The purpose of this review is to provide an overview of the perinatal outcomes of fetuses who underwent fetal surgery for the management of Amniotic Band Syndrome (ABS). A systematic review of studies reporting on the perinatal outcome of fetuses undergoing fetoscopic release of amniotic bands according to the (PRISMA) guidelines was performed. The MEDLINE, Embase, Scopus, and Cochrane Library databases were systematically searched. In total, 17 studies reporting 37 cases of ABS that underwent amniotic band release by fetoscopy were included. The median gestational age at which fetal surgery was performed was 22 weeks (range 18-29 weeks). PPROM occurred in 51.3%, while fetal survival reached 89.2%. The success of fetal surgery was 75.7% in preserving and maintaining the functionality of the affected limb. Fetoscopic release of amniotic bands can preserve the affected limb and its function in cases of ABS and prevent fetal death in cases of ABS involving the umbilical cord. Further studies are needed to determine the optimal criteria for selecting patients who can benefit from fetal surgery, considering that it is an intervention that is not free of perinatal complications.
RESUMO
OBJECTIVES: We aimed to perform a systematic review and network meta-analysis to evaluate the preventive strategies for preterm birth in twin-to-twin transfusion syndrome. METHODS: PubMed, Embase and Cochrane Central were searched from inception to December 2023 with no filters. Additionally, the reference lists of the included studies were manually examined to identify any supplementary studies. We selected randomized controlled trials and cohorts comparing interventions to prevent preterm birth in twin pregnancies complicated by twin-to-twin transfusion syndrome. A random-effects frequentist network meta-analysis was performed using RStudio version 4.3.1. Randomized controlled trials and cohorts were assessed respectively using the Risk of Bias in Non-randomized Studies of interventions tool and Cochrane Collaboration's tool for assessing risk of bias in randomized trials. RESULTS: In this systematic review and meta-analysis, we included eight studies comprising a total of 719 patients. Compared with expectant management, cerclage stood out as the only intervention associated with an increase in the survival of at least one twin (risk ratio 1.12; 95â¯% confidence interval 1.01-1.23). Our subgroup analysis based on different thresholds for short cervix demonstrated a significant reduction in the risk of preterm birth before 32 weeks with ultrasound-indicated cerclage using a 15â¯mm criterion (risk ratio 0.65; 95â¯% confidence interval 0.47-0.92). CONCLUSIONS: Our study suggests the potential benefit of cerclage as a preventive strategy for preterm birth in pregnancies complicated by twin-to-twin transfusion syndrome. These findings highlight the necessity for further investigation to corroborate our results and address the optimal threshold for ultrasound-indicated cerclage.
RESUMO
OBJECTIVE: To assess the impact of overweight and obesity in the second and third trimesters of pregnancy on fetal cardiac function parameters. METHODS: We performed a prospective cohort study of 374 singleton pregnant women between 20w0d and 36w6d divided into three groups: 154 controls (body mass index - BMI < 25 kg/m2), 140 overweight (BMI 25-30 kg/m2) and 80 obese (BMI ≥ 30 kg/m2). Fetal left ventricular (LV) modified myocardial performance index (Mod-MPI) was calculated according to the following formula: (isovolumetric contraction time + isovolumetric relaxation time)/ejection time. Spectral tissue Doppler was used to determine LV and right ventricular (RV) myocardial performance index (MPI'), peak myocardial velocity during systole (S'), early diastole (E'), and late diastole (A'). RESULTS: We found significant differences between the groups in maternal age (p < 0.001), maternal weight (p < 0.001), BMI (p < 0.001), number of pregnancies (p < 0.001), parity (p < 0.001), gestational age (p = 0.013), and estimated fetal weight (p = 0.003). Overweight pregnant women had higher LV Mod-MPI (0.046 versus 0.044 seconds, p = 0.009) and LV MPI' (0.50 versus 0.47 seconds, p < 0.001) than the control group. Obese pregnant women had higher RV E' than control (6.82 versus 6.33 cm/sec, p = 0.008) and overweight (6.82 versus 6.46 cm/sec, p = 0.047) groups. There were no differences in 5-min APGAR score < 7, neonatal intensive care unit admission, hypoglycemia and hyperglobulinemia between the groups. CONCLUSIONS: We observed fetal myocardial dysfunction in overweight and obese pregnant women with higher LV Mod-MPI, LV MPI' and RV E' compared to fetuses from normal weight pregnant women.
Assuntos
Ecocardiografia Doppler , Coração Fetal , Recém-Nascido , Gravidez , Feminino , Humanos , Terceiro Trimestre da Gravidez , Coração Fetal/diagnóstico por imagem , Estudos Prospectivos , Sobrepeso/complicações , Sobrepeso/epidemiologia , Obesidade/complicações , Obesidade/epidemiologia , Ultrassonografia Pré-NatalRESUMO
INTRODUCTION: Congenital hydrocephalus often results in irreversible and severe damage to the brain despite postnatal interventions. The potential for prenatal intervention to mitigate these deleterious effects underscores the importance of a suitable animal model. We aimed assess the results of an ultrasound guided transuterine approach to replicate the BioGlue injection fetal hydrocephalus model. METHODS: Pregnant ewes were anesthetized at 95 days of gestation and BioGlue was injected into the fetal cisterna magna under ultrasound guidance through the uterus. Ventriculomegaly was assessed by MRI and histology. RESULTS: Nine pregnant ewes were included in the study, and their fetuses were divided into the the BioGlue intervention group (n=9 fetuses) or the control group (n=7 fetuses) who were not injected. Although hydrocephalus was noted in 5 of 9 fetuses in the intervention group, the ability to induce hydrocephalus went from 0% to 100% in the last 3 fetuses following technical modifications. None of the controls developed hydrocephalus. Fetal brains with hydrocephalus demonstrated increased IBA1+ compared to control animals. CONCLUSIONS: While technical challenges were noted, the ultrasound guided transuterine approach to replicate the BioGlue fetal hydrocephalus model in sheep showed consistent and reproducible results. This model offers the advantage of directly visualizing the location of the needle tip and injection of the BioGlue. This technique offers an alternative for testing novel approaches for prenatal congenital hydrocephalus treatment.
RESUMO
BACKGROUND: The aim of this extended review of multicenter case series is to describe the prenatal ultrasound features and pathogenetic mechanisms underlying placental and umbilical cord anomalies and their relationship with adverse perinatal outcome. From an educational point of view, the case series has been divided in three parts; Part 1 is dedicated to placental abnormalities. METHODS: Multicenter case series of women undergoing routine and extended prenatal ultrasound and perinatal obstetric care. RESULTS: Prenatal ultrasound findings, perinatal care, and pathology documentation in cases of placental pathology are presented. CONCLUSIONS: Our case series review and that of the medical literature confirms the ethiopathogenetic role and involvement of placenta abnormalities in a wide variety of obstetrics diseases that may jeopardize the fetal well-being. Some of these specific pathologies are strongly associated with a high risk of poor perinatal outcome.
RESUMO
OBJECTIVE: Fetal surgery has improved neonatal outcomes; however, it is unknown if the intervention contributes to the developmental of inflammatory pathologies in the placenta. Here, an association between fetal surgery and placental pathology was examined. METHOD: This case-control study compared pregnancies with fetal surgery (n = 22), pregnancies with an indication for fetal surgery but without an intervention being done (n = 13), and gestational-age and fetus-number matched controls (n = 36). Data on maternal, infant, and placental outcomes were abstracted. Additionally, immunohistochemistry identified expression of lymphoid and myeloid cells in the placenta on a subset of cases. Comparisons were performed using Kruskal-Wallis or Pearson's chi-squared tests. RESULTS: Maternal characteristics were comparable between groups. Most fetal interventions were for diaphragmatic hernia, spina bifida, or twin-to-twin transfusion syndrome. Fetuses who were operated on before birth were more likely to be born preterm (p = 0.02). There was no increase in the rate of observed placental pathologies or immune cell infiltration in fetal surgery cases compared to controls. CONCLUSION: The data suggest that fetal surgery is not associated with increased inflammatory or morphologic pathology in the placenta. This observation supports the growing field of fetal surgery.
Assuntos
Transfusão Feto-Fetal , Placenta , Recém-Nascido , Gravidez , Feminino , Humanos , Placenta/patologia , Estudos de Casos e Controles , Transfusão Feto-Fetal/patologia , Feto/cirurgia , PartoRESUMO
Cleft lip, with or without cleft palate, is the most common congenital craniofacial anomaly and the second most common birth defect worldwide. Micrognathia is a rare facial malformation characterized by small, underdeveloped mandible and frequently associated with retrognathia. Second- and third-trimester prenatal ultrasound is the standard modality for screening and identification of fetal orofacial abnormalities, with a detection rate in the low-risk population ranging from 0% to 73% for all types of cleft. The prenatal ultrasonography detection can also be performed during the first trimester of pregnancy. Given the potential limitations of obstetric ultrasound for examining the fetal face, such as suboptimal fetal position, shadowing from the surrounding bones, reduce amniotic fluid around the face, interposition of fetal limbs, umbilical cord and placenta, and maternal habitus/abdominal scars, the use of adjunct imaging modalities can enhance prenatal diagnosis of craniofacial anomalies in at-risk pregnancies. Fetal magnetic resonance imaging (MRI) is a potentially useful second-line investigation for the prenatal diagnosis of orofacial malformations with a pooled sensitivity of 97%. In this review, we discuss the role of ultrasound and fetal MRI in the prenatal assessment of abnormalities of the upper lip, palate, and mandible.
Assuntos
Fenda Labial , Fissura Palatina , Feminino , Gravidez , Humanos , Fenda Labial/diagnóstico por imagem , Fissura Palatina/diagnóstico por imagem , Diagnóstico Pré-Natal/métodos , Ultrassonografia Pré-Natal/métodos , Imageamento por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Mandíbula/diagnóstico por imagemRESUMO
OBJECTIVE: To describe the ultrasonographic characteristics of congenital porto-systemic venous shunts (CPSS) diagnosed during pregnancy, their outcomes, and their evolution. METHODS: Two independent researchers selected 493 review articles and case reports through the analysis of titles, abstracts, and full text. The PubMed and LILACS databases were searched. Through the application of filters according to the PRISMA protocol, only six articles were used in the research. The following information was collected, when available: gestational age at diagnosis, gender, birth weight, type of shunt, associated anomalies/complications and treatment/progression. RESULTS: The data were obtained from 27 cases, with 22 (82%) fetuses diagnosed with intra-hepatic CPSS and 5 (18%) with extra-hepatic CPSS. The median time of intrauterine diagnosis was 33 weeks. In 12 (57.1%) of the 21 pregnancies evaluated, delivery was preterm. The estimated fetal weight ranged from 1150 to 3760 g, with 4 (25%) cases at <3rd, 3 (18.75%) cases at <10th, 8 (50%) cases at <50th, and 1 (6.25%) case at >97th percentile for gestational age. The most frequent obstetric complication was fetal growth restriction, which occurred in nine (60%) cases. As for postnatal treatment, 19 (70.4%) cases were conservatively treated, and 8 (29.6%) cases required surgical intervention. CONCLUSION: The diagnosis of CPSS still represents a challenge during prenatal care. Its early identification aims to provide guidance to pregnant women and their families, as well as follow-up and anticipation of possible complications, in addition to the evaluation of the mode of delivery and postnatal follow-up, directing the short- and long-term prognosis.
Assuntos
Retardo do Crescimento Fetal , Diagnóstico Pré-Natal , Recém-Nascido , Gravidez , Humanos , Feminino , Idade GestacionalRESUMO
Background: VACTERL association consists of Vertebral, Anorectal, Cardiac, Tracheo-Esophageal, Renal, and Limb defects. The diagnosis depends on the presence of at least three of these structural abnormalities. Methods: The clinical presentation and diagnostic prenatal imaging of VACTERL association are comprehensively reviewed. Results: The most common feature is a vertebral anomaly, found in 60-80% of cases. Tracheo-esophageal fistula is seen in 50-80% of cases and renal malformations in 30% of patients. Limb defects including thumb aplasia/hypoplasia, polydactyly, and radial agenesis/hypoplasia are present in 40-50% of cases. Anorectal defects, like imperforate anus/anal atresia, are challenging to detect prenatally. Conclusion: The diagnosis of VACTERL association mostly relies on imaging techniques such as ultrasound, computed tomography, and magnetic resonance. Differential diagnosis should exclude similar diseases such as CHARGE and Townes-Brocks syndromes and Fanconi anemia. New insights into genetic etiology have led to recommendations of chromosomal breakage investigation for optimal diagnosis and counseling.
Assuntos
Cardiopatias Congênitas , Deformidades Congênitas dos Membros , Deformidades Congênitas das Extremidades Superiores , Humanos , Esôfago/diagnóstico por imagem , Esôfago/anormalidades , Traqueia/diagnóstico por imagem , Traqueia/anormalidades , Deformidades Congênitas dos Membros/diagnóstico por imagem , Coluna Vertebral/anormalidades , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/genética , Canal Anal/diagnóstico por imagem , Canal Anal/anormalidades , Rim/patologia , Deformidades Congênitas das Extremidades Superiores/patologia , Diagnóstico por ImagemRESUMO
During human pregnancy, proinflammatory responses in the placenta can cause severe fetal complications, including growth restriction, preterm birth, and stillbirth. Villitis of unknown etiology (VUE), an inflammatory condition characterized by the infiltration of maternal CD8+ T cells into the placenta, is hypothesized to be secondary to either a tissue rejection response to the haploidentical fetus or from an undiagnosed infection. In this study, we characterized the global TCR ß-chain profile in human T cells isolated from placentae diagnosed with VUE compared with control and infectious villitis-placentae by immunoSEQ. Immunosequencing demonstrated that VUE is driven predominantly by maternal T cell infiltration, which is significantly different from controls and infectious cases; however, these T cell clones show very little overlap between subjects. Mapping TCR clones to common viral epitopes (CMV, EBV, and influenza A) demonstrated that Ag specificity in VUE was equal to controls and significantly lower than CMV-specific clones in infectious villitis. Our data indicate VUE represents an allograft response, not an undetected infection. These observations support the development of screening methods to predict those at risk for VUE and the use of specific immunomodulatory therapies during gestation to improve outcomes in affected fetuses.
Assuntos
Vilosidades Coriônicas/imunologia , Genes Codificadores da Cadeia beta de Receptores de Linfócitos T/genética , Rejeição de Enxerto/imunologia , Inflamação/imunologia , Doenças Placentárias/imunologia , Gravidez/imunologia , Linfócitos T/imunologia , Adulto , Aloenxertos/imunologia , Antígenos Virais/imunologia , Movimento Celular , Estudos de Coortes , Epitopos de Linfócito T/imunologia , Feminino , Feto , Antígenos HLA/imunologia , Humanos , Adulto JovemRESUMO
OBJECTIVE: To evaluate inter-rater agreement for sonographic classification of stomach position (as a surrogate for liver herniation) in fetal left congenital diaphragmatic hernia (LCDH) among: (i) fetal medicine specialists from the North American Fetal Therapy Network (NAFTNet) centers within and without the fetal endoscopic tracheal occlusion (FETO) consortium and in comparison to an expert external reviewer (ER1); and (iii) among two expert ERs (ER1 and ER2). METHODS: Forty-eight physicians from 26 NAFTNet centers and 2 ERs were asked to assess 13 sonographic clips of isolated LCDH and classify stomach position as "intra-abdominal," "anterior left chest," "mid to posterior left chest," or "retro-cardiac" based on the classification published by Basta et al.8 Interrater agreement was assessed by determining proportion of stomach position ratings concordant amongst NAFTNet participants and ER1. Agreement for stomach position between ERs was calculated using kappa statistics. RESULTS: Agreement for stomach position was 69% (39%-85%; n = 19) and 54% (23%-92%; n = 29) among FETO and non-FETO NAFTNet participants, respectively, when compared to ER1. Most disagreement in stomach position was related to a discrepancy of one position. ERs were in agreement for stomach position in 5 of 13 cases (38.5%) and inter-rater agreement was highest for "anterior" stomach position. CONCLUSION: Interrater agreement for stomach position assessment in CDH was poor across NAFTNet and indeed amongst expert reviewers.
Assuntos
Terapias Fetais , Hérnias Diafragmáticas Congênitas , Feminino , Fetoscopia , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , América do Norte , Gravidez , Estudos Retrospectivos , Estômago/diagnóstico por imagem , Ultrassonografia Pré-NatalRESUMO
INTRODUCTION: Congenital pulmonary airway malformations (CPAMs) complicated by hydrops portend significant morbidity and mortality, with fetal survival estimates less than 10%. CASE PRESENTATION: We report successful use of ultrasound-guided radiofrequency ablation at 21-week gestation in a hydropic fetus with CPAM, with subsequent resolution of hydrops. Thirty-two-week MRI noted persistent mediastinal shift, and US at 36 weeks and 5 days noted polyhydramnios. Maternal gestational hypertension prompted delivery at 37 weeks, with a cesarean section performed after a failed trial of labor. The infant required CPAP at 100% and weaned to 21%. Tachypnea persisted, and chest CT on day of life 2 demonstrated multiple large cysts in the right lower lobe with anterior pneumothorax. On day of life 3, she successfully underwent a thoracoscopic right lower lobectomy. Adhesions to the chest wall and rib abnormalities were noted. She was extubated to CPAP at the conclusion of the procedure. She was able to wean to 21% on POD2 and transitioned to oral feeds. Her chest tube was removed with resultant ex vacuo pneumothorax noted. She remained asymptomatic and was discharged home on room air POD11. Pathology confirmed a type 1 CPAM. CONCLUSION: In utero radiofrequency ablation may be an adjunct to the management of large CPAM.
Assuntos
Malformação Adenomatoide Cística Congênita do Pulmão , Terapias Fetais , Pneumotórax , Cesárea , Malformação Adenomatoide Cística Congênita do Pulmão/complicações , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Edema , Feminino , Feto/cirurgia , Humanos , Hidropisia Fetal/diagnóstico por imagem , Hidropisia Fetal/cirurgia , Lactente , Pneumotórax/diagnóstico por imagem , Pneumotórax/cirurgia , GravidezRESUMO
INTRODUCTION: Uterine incision based on the placental location in open maternal-fetal surgery (OMFS) has never been evaluated in regard to maternal or fetal outcomes. OBJECTIVE: The aim of this study was to investigate whether an anterior placenta was associated with increased rates of intraoperative, perioperative, antepartum, obstetric, or neonatal complications in mothers and babies who underwent OMFS for fetal myelomeningocele (fMMC) closure. METHODS: Data from the international multicenter prospective registry of patients who underwent OMFS for fMMC closure (fMMC Consortium Registry, December 15, 2010-June 31, 2019) was used to compare fetal and maternal outcomes between anterior and posterior placental locations. RESULTS: The placental location for 623 patients was evenly distributed between anterior (51%) and posterior (49%) locations. Intraoperative fetal bradycardia (8.3% vs. 3.0%, p = 0.005) and performance of fetal resuscitation (3.6% vs. 1.0%, p = 0.034) occurred more frequently in cases with an anterior placenta when compared to those with a posterior placenta. Obstetric outcomes including membrane separation, placental abruption, and spontaneous rupture of membranes were not different among the 2 groups. However, thinning of the hysterotomy site (27.7% vs. 17.7%, p = 0.008) occurred more frequently in cases of an anterior placenta. Gestational age (GA) at delivery (p = 0.583) and length of stay in the neonatal intensive care unit (p = 0.655) were similar between the 2 groups. Fetal incision dehiscence and wound revision were not significantly different between groups. Critical clinical outcomes including fetal demise, perinatal death, and neonatal death were all infrequent occurrences and not associated with the placental location. CONCLUSIONS: An anterior placental location is associated with increased risk of intraoperative fetal resuscitation and increased thinning at the hysterotomy closure site. Individual institutional experiences may have varied, but the aggregate data from the fMMC Consortium did not show a significant impact on the GA at delivery or maternal or fetal clinical outcomes.
Assuntos
Terapias Fetais , Meningomielocele , Feminino , Terapias Fetais/efeitos adversos , Idade Gestacional , Humanos , Histerotomia/efeitos adversos , Recém-Nascido , Meningomielocele/etiologia , Meningomielocele/cirurgia , Placenta/cirurgia , GravidezRESUMO
PURPOSE: To report the longitudinal lung growth and prognosis of fetuses with severe left sided congenital diaphragmatic hernia (CDH) treated with fetoscopic tracheal occlusion (FETO) in a single institution. METHODS: Fetal lung size (observed-to-expected lung area to head circumference [o/e-LHR]) was measured in seven consecutive fetuses with isolated severe left-sided CDH who underwent FETO. Fetal lung growth was used to prognosticate survival and need for ECMO. RESULTS: Seven consecutive fetuses had a FETO procedure in the timeframe of this study. A total of 44 longitudinal ultrasound were performed to evaluate lung development. FETO was performed at GA 28.5 ± 0.5 weeks. Five (71.4%) infants survived to one-year follow-up and ECMO was needed in three patients (42.8%). Fetal lung response was observed in all fetuses; mean o/e-LHR increased from 22.5% ± 1.4 before FETO to 44.4% ± 9.8 before delivery. Infants who survived had a higher percentage of fetal lung growth (21.8%) than those who died (8.25%). CONCLUSION: Our study supports the hypothesis that FETO promotes fetal lung growth in fetuses with severe left-sided CDH, and the fetal pulmonary response seems to be associated with improved outcomes after the procedure.
Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Fetoscopia , Feto , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/cirurgia , Humanos , Lactente , Pulmão/diagnóstico por imagem , Gravidez , Traqueia/diagnóstico por imagem , Ultrassonografia Pré-NatalRESUMO
We conducted a comprehensive evidence-based review on the epidemiology and current standard of care of gastroschisis management as well as the pathophysiology, rationale and feasibility of fetal therapy as a viable alternative. Gastroschisis is a periumbilical abdominal wall defect characterized by abdominal viscera herniation in utero. It affects 4 in 10 000 live births, but the prevalence has steadily increased in recent years. Gastroschisis is typically diagnosed on routine second-trimester ultrasound. The overall prognosis is favorable, but complex gastroschisis, which accounts for about 10% to 15% of cases, is associated with a higher mortality, significant disease burden and higher healthcare costs due to long- and short-term complications. The current standard of care has yet to be established but generally involves continued fetal surveillance and multidisciplinary perinatal care. Postnatal surgical repair is achieved with primary closure, staged silo closure or sutureless repair. Experimental animal studies have demonstrated the feasibility of in utero closure, antiinflammatory therapy and prenatal regenerative therapy. However, reports of early preterm delivery and amnioinfusion trials have failed to show any benefit in humans. Further experimental studies and human trials are necessary to demonstrate the potential benefit of fetal therapy in gastroschisis.
Assuntos
Terapias Fetais , Gastrosquise/cirurgia , Animais , HumanosRESUMO
INTRODUCTION: Prenatal work-up for congenital diaphragmatic hernia (CDH) is important for risk stratification, standardization, counseling, and optimal therapeutic choice. To determine current practice patterns regarding prenatal CDH work-up, including prenatal ultrasound and magnetic resonance imaging (MRI) use, and to identify areas for standardization of such evaluation between fetal centers. METHODS: A survey regarding prenatal CDH work-up was sent to each member center of the North American Fetal Therapy Network (NAFTNet) (n = 36). RESULTS: All responded. Sonographic measurement of lung-to-head ratio (LHR) was determined by all, 89% (32/36) of which routinely calculate observed-to-expected LHR. The method for measuring LHR varied: 58% (21/36) used a "trace" method, 25% (9/36) used "longest axis," and 17% (6/36) used an "antero-posterior" method. Fetal MRI was routinely used in 78% (28/36) of centers, but there was significant variability in fetal lung volume measurement. Whereas all generated a total fetal lung volume, the planes, methodology and references values varied significantly. All evaluated liver position, 71% (20/28) evaluated stomach position and 54% (15/28) quantified the degree of liver herniation. More consistency in workup was seen between centers offering fetal intervention. CONCLUSION: Prenatal CDH work-up and management differs considerably among North American fetal diagnostic centers, highlighting a need for its standardization.
Assuntos
Hérnias Diafragmáticas Congênitas/diagnóstico , Fígado/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Padrões de Prática Médica , Cuidado Pré-Natal/métodos , Ultrassonografia Pré-Natal/métodos , Aconselhamento , Feminino , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Humanos , Pulmão/embriologia , América do Norte , Tamanho do Órgão , Gravidez , Padrões de Referência , Inquéritos e QuestionáriosRESUMO
Myelomeningocele (MMC), the commonest type of spina bifida (SB), occurs due to abnormal development of the neural tube and manifest as failure of the complete fusion of posterior arches of the spinal column, leading to dysplastic growth of the spinal cord and meninges. It is associated with several degrees of motor and sensory deficits below the level of the lesion, as well as skeletal deformities, bladder and bowel incontinence, and sexual dysfunction. These children might develop varying degrees of neuropsychomotor delay, partly due to the severity of the injuries that affect the nervous system before birth, partly due to the related cerebral malformations (notably hydrocephalus-which may also lead to an increase in intracranial pressure-and Chiari II deformity). Traditionally, MMC was repaired surgically just after birth; however, intrauterine correction of MMC has been shown to have several potential benefits, including better sensorimotor outcomes (since exposure to amniotic fluid and its consequent deleterious effects is shortened) and reduced rates of hydrocephalus, among others. Fetal surgery for myelomeningocele, nevertheless, would not have been made possible without the development of experimental models of this pathological condition. Hence, the aim of the current article is to provide an overview of the animal models of MMC that were used over the years and describe how this knowledge has been translated into the fetal treatment of MMC in humans.
Assuntos
Malformação de Arnold-Chiari , Terapias Fetais , Hidrocefalia , Meningomielocele , Disrafismo Espinal , Animais , Feminino , Humanos , Hidrocefalia/etiologia , Meningomielocele/complicações , Gravidez , Disrafismo Espinal/complicaçõesRESUMO
Amniotic band syndrome is a rare condition. There have been few cases reported of fetoscopic band dissection. The aim of this case series is to report 3 cases of fetoscopic treatment for amniotic band syndrome, including indication for surgery, technical aspects, complications and outcomes. Fetoscopic treatment was performed respectively at 23 5/7 , 26 5/7 and 18 3/7 weeks' gestation. Two procedures were performed with a laser fiber through a single trocar whereas one surgery was performed with scissors. In conclusion, fetoscopic release of the amniotic bands in case of amniotic band syndrome is feasible with encouraging results in order to prevent amputation and dysfunction of the extremities.
Assuntos
Síndrome de Bandas Amnióticas , Transfusão Feto-Fetal , Terapia a Laser , Síndrome de Bandas Amnióticas/diagnóstico por imagem , Síndrome de Bandas Amnióticas/cirurgia , Feminino , Transfusão Feto-Fetal/cirurgia , Fetoscopia , Idade Gestacional , Humanos , Recém-Nascido , GravidezRESUMO
INTRODUCTION: Congenital diaphragmatic hernia (CDH) affects 1 in 3,000 live births and is associated with significant morbidity and mortality. METHODS: A review of fetal magnetic resonance imaging (MRI) examinations was performed for fetuses with left CDH and normal lung controls. Image review and manual tracings were performed by 4 pediatric radiologists; right and left lung volumes in the coronal and axial planes as well as liver volume above and below the diaphragm in the coronal plane were measured. Intra- and interreviewer reproducibility was assessed using intraclass correlation coefficient (ICC) and Bland-Altman analysis. RESULTS: Excellent intra- and interreviewer reproducibility of the right and left lung volume measurements was observed in both axial planes (interreviewer ICC: right lung: 0.97, 95% CI: 0.95-0.99; left lung: 0.97, 95% CI: 0.95-0.98) and coronal planes (interreviewer ICC: right lung: 0.97, 95% CI: 0.95-0.98; left lung: 0.96, 95% CI: 0.93-0.98). Moderate-to-good interreviewer reproducibility was observed for liver volume above the diaphragm (ICC 0.7, 95% CI: 0.59-0.81). Liver volume below the diaphragm had a good-to-excellent interreviewer reproducibility (ICC 0.88, 95% CI: 9.82-0.93). CONCLUSIONS: The present study demonstrated an excellent intra- and interreviewer reproducibility of MRI lung volume measurements and good-to-moderate inter- and intrareviewer reproducibility of liver volume measurements after standardization of the methods at our fetal center.