ABSTRACT
BACKGROUND: Almost 20% of parvovirus B19 foetal infections require intrauterine transfusions. In addition, myocardial dysfunction has been observed in severe parvovirus B19 infections. One objective of an intrauterine exchange transfusion (IUET) is to avoid an overload during the transfusion. Our aim was to study the obstetrical and neonatal outcomes in cases of IUETs performed for foetal parvovirus infections and to compare our survival rate to those studies in which simple in utero transfusions were chosen. STUDY DESIGN AND METHODS: This was a retrospective monocentre study of all patients followed up for parvovirus B19 infections in which IUETs were performed. An IUET was indicated when foetal hydrops was observed and/or when severe foetal anaemia was diagnosed though an elevation in the middle cerebral artery peak systolic velocity. The characteristics of each pregnancy and the neonatal outcomes were studied until hospital discharge. RESULTS: Thirty-five IUETs were performed in 26 foetuses. The median gestational age of the first IUET was 22.6 weeks. Only one foetal bradycardia incidence was recorded during the procedure. Three medical pregnancy terminations were observed in our series, secondary to severe cerebral anomalies confirmed in the magnetic resonance imaging. Five in utero deaths occurred, in which 2 of the foetuses underwent multiple IUETs. All the neonates had normal haemoglobin levels at birth, and none were transferred to the neonatal intensive care unit. The overall survival rate was 70%. CONCLUSION: IUETs exhibit a survival rate similar to that of simple intrauterine transfusions in foetal parvovirus infection cases.
Subject(s)
Blood Transfusion, Intrauterine/methods , Parvoviridae Infections/pathology , Parvoviridae Infections/therapy , Adult , Female , Fetal Diseases/mortality , Fetal Diseases/pathology , Fetal Diseases/therapy , Humans , Male , Parvoviridae Infections/mortality , Prenatal Care , Prognosis , Retrospective Studies , Young AdultABSTRACT
OBJECTIVE: Congenital diaphragmatic hernia (CDH) is a severe anomaly. The observed-to-expected lung-area-to-head-circumference ratio (o/e-LHR) has been shown to provide a useful prediction of subsequent survival of fetuses with CDH in referral centers with expertise and a large caseload. However, the accuracy of o/e-LHR measurements in general fetal medicine units with less expertise is not well known. The aim of this study was to evaluate the capacity of o/e-LHR to provide a useful prediction of mortality of fetuses with CDH when the measurement is performed in fetal medicine units with different levels of expertise. METHODS: Between January 2008 and November 2013, 305 live births with expectantly managed left-sided isolated CDH were recorded in the database of the French National Center for Rare Diseases (31 centers) and followed up after birth. Centers were grouped into two categories according to their mean annual CDH caseload over the study period: large centers with an average of ≥ 14 cases and smaller centers with < 14 cases per year. The relationship between o/e-LHR and 28-day and 6-month mortality was modeled using fractional polynomials and the predictive value of o/e-LHR was quantified using the area under the receiver-operating characteristics curve. Comparisons between the two center categories were carried out. Analyses were adjusted for potential confounders such as thoracic herniation of the liver and gestational age at birth and at diagnosis. RESULTS: During the study period, two large centers managed a total of 82 CDH cases and 29 smaller centers a total of 223 CDH cases. Overall, there was a significant inverse relationship between 28-day mortality rate and o/e-LHR, which decreased from 54% when o/e-LHR was 20% to 6% when o/e-LHR was 75% (P < 0.01). When the category of center was considered, adjusted associations between o/e-LHR and 28-day mortality were significantly different (P = 0.032) between large and smaller centers. The ability to predict survival at 28 days postpartum based on o/e-LHR was better in large centers; for a specificity of 0.30, the sensitivity was 0.71 in large centers and 0.55 in smaller ones. The results were similar for 6-month mortality. CONCLUSIONS: Our results show that o/e-LHR measured on two-dimensional ultrasound is a good indicator of neonatal prognosis in cases of CDH that may be used even in fetal medicine centers with a small caseload. However, our results also suggest that LHR measurement may be difficult to perform correctly. Therefore, appropriate training should be offered to professionals. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.
Subject(s)
Cephalometry/methods , Fetal Diseases/diagnostic imaging , Head/diagnostic imaging , Hernias, Diaphragmatic, Congenital/diagnostic imaging , Lung/diagnostic imaging , Ultrasonography, Prenatal , Adult , Female , Fetal Diseases/mortality , France , Gestational Age , Head/embryology , Head/pathology , Hernias, Diaphragmatic, Congenital/embryology , Hernias, Diaphragmatic, Congenital/mortality , Hernias, Diaphragmatic, Congenital/pathology , Humans , Lung/embryology , Lung/pathology , Predictive Value of Tests , Pregnancy , Reproducibility of Results , Retrospective Studies , Survival AnalysisABSTRACT
The French Rare Disease Reference Center for congenital diaphragmatic hernia (CDH) was created in 2008, to implement a national protocol for foetuses and children with this serious condition. Neonatal mortality from CDH is 30-40%, mainly due to pulmonary hypoplasia and persistent pulmonary hypertension, and half of those who live have high respiratory, nutritional and digestive morbidity. CDH management requires long-term and specialised multidisciplinary care. It has been well established that a standardised management protocol improves the prognosis of children with CDH. CONCLUSION: Organising health care and implementing a nationwide French protocol were key factors for reducing mortality and morbidity from CDH.
Subject(s)
Hernias, Diaphragmatic, Congenital/therapy , Clinical Protocols , France/epidemiology , Hernias, Diaphragmatic, Congenital/complications , Hernias, Diaphragmatic, Congenital/mortality , Humans , Perinatal Care , Prenatal Care , Respiratory Insufficiency/etiologyABSTRACT
UNLABELLED: In-utero transfusion is now well under control and improves the survival of foetuses monitored for fetal anemia with a survival rate of more than 80 %. The aim was to evaluate short-term neonatal outcome after fetal severe anemia managed by intrauterine transfusions. We did a retrospective study of all neonates born after management of severe fetal anemia (n = 93) between January 1999 and January 2013 in our regional center. The two main causes of anemia were maternal red blood cell alloimmunization (N = 81, 87 %) and Parvovirus B19 infection (N = 10, 10.8 %). In the alloimmunization group, phototherapy was implemented in 85.2 % of cases with a maximum level of bilirubin of 114.4 ± 60.7 (mg/dl). Transfusion and exchange transfusion were, respectively, required in 51.9 % and in 34.6 % of cases. One neonate presented a convulsive episode, and we observed three neonatal deaths. In the parvovirus group, none of the child had anemia at birth and no management was necessary. CONCLUSION: Contemporary management of Rhesus disease is associated with encouraging neonatal outcomes. In case of Parvovirus infection, no specific management is necessary at. But, in all cases of fetal anemia, children should be followed up with particular attention to neurologic development. WHAT IS KNOWN: ⢠In-utero transfusion is now well under control and improves the survival of fetuses monitored for fetal anemia. ⢠Limited studies are available on the effect of IUT on postnatal outcome in infants with a history of fetal anemia. What is New: ⢠Contemporary management of severe Rhesus disease is associated with encouraging neonatal outcomes. ⢠The majority of infants can be managed with phototherapy and a limited number of top-up transfusions and exchange transfusions. In case of Parvovirus infection, the short-term neonatal outcome is excellent.
Subject(s)
Anemia, Hemolytic/therapy , Blood Transfusion, Intrauterine/methods , Erythroblastosis, Fetal/therapy , Parvoviridae Infections/therapy , Rh Isoimmunization/therapy , Adult , Anemia, Hemolytic/virology , Erythroblastosis, Fetal/virology , Female , Humans , Infant , Infant, Newborn , Male , Middle Aged , Parvovirus B19, Human/isolation & purification , Pregnancy , Retrospective Studies , Survival Rate , Young AdultABSTRACT
OBJECTIVE: Prenatal diagnosis of esophageal atresia (EA) remains a challenge. Our objective was to evaluate the combination of sonography, magnetic resonance imaging (MRI), and amniotic fluid biochemical markers in prenatal diagnosis of EA. STUDY DESIGN: A retrospective study of all cases with prenatal suspicion of EA from January 2008 to May 2013 in our regional reference center was carried out. Patients were included if all the three tests were performed. For each test, sensitivity (Se), specificity (Sp), positive predictive value (PPV), and negative predictive value (NPV) were evaluated. Each test was compared using Fisher's exact test. RESULTS: Fifteen patients were referred at a median gestational age of 28(+5) weeks (24-36) for suspicion of EA on the basis of small or non-visualized fetal stomach bubble and/or polyhydramnios. Se, Sp, PPV, and NPV for sonographic pouch sign/MRI/biochemical amniotic fluid were respectively 40/100/100/45.5%, 80/100/100/71.4%, and 90/60/81.8/75%. MRI was the best predictive test (p = 0.007). CONCLUSION: In case of ultrasound prenatal suspicion of EA (with or without visualization of the pouch sign), an MRI at 30-32 weeks using fast imaging employing steady-state acquisition should be proposed. Biochemical amniotic fluid may be helpful and should be evaluated in a larger study.
Subject(s)
Amniocentesis , Amniotic Fluid/metabolism , Esophageal Atresia/diagnosis , Magnetic Resonance Imaging , Ultrasonography, Prenatal , Biomarkers/metabolism , Esophageal Atresia/metabolism , Female , Humans , Predictive Value of Tests , Pregnancy , Pregnancy Trimester, Second , Pregnancy Trimester, Third , Retrospective Studies , Sensitivity and SpecificityABSTRACT
OBJECTIVE: The objective of this study is to highlight the factors that may affect prenatal diagnosis of transposition of the great arteries (TGA) in order to improve it. METHODS: This is a retrospective study performed between 2004 and 2009 in the maternity units from North of France. We identified a total of 68 cases of TGA (isolated or associated with only VSD or coarctation of aorta), of which 32 (47.1%) had prenatal diagnosis (PND+) and 36 did not (PND-). Maternal characteristics and ultrasound factors were studied in relation to PND. RESULTS: Maternal weight and body mass index were significantly higher in the PND- group (70.4 kg and 26.5 kg/m(2) vs 63.6 kg and 23.6 kg/m(2) , respectively). Maternal obesity (body mass index >30) was significantly more frequent in the PND- group (27.8% vs 12.5%). More than a quarter of TGA (28.1%) were diagnosed during the third trimester. CONCLUSION: Obesity is the main cause of missed PND of TGA. Obese patients with suboptimal prenatal scans may benefit from reassessment of fetal cardiac anatomy and/or from referral for fetal echocardiography.
Subject(s)
Transposition of Great Vessels/diagnostic imaging , Ultrasonography, Prenatal/statistics & numerical data , Adult , Body Mass Index , Echocardiography/statistics & numerical data , Female , Fetal Heart/diagnostic imaging , France/epidemiology , Humans , Middle Aged , Obesity/diagnostic imaging , Obesity/epidemiology , Pregnancy , Pregnancy Complications/diagnostic imaging , Pregnancy Complications/epidemiology , Retrospective Studies , Transposition of Great Vessels/epidemiology , Young AdultABSTRACT
BACKGROUND: The vast majority of prenatally diagnosed congenital pulmonary malformations (CPM) remain asymptomatic at birth. The maximal value of the CPM volume ratio (CVRmax) predicts the risk of neonatal respiratory distress (NRD), and should allow for better assessment of the level of expertise needed at the delivery site. AIM: This study evaluated the level of maternity units currently chosen for the delivery of CPMs, and determined the impact of the choice of delivery site based on the CVRmax, with a threshold of 0.4 cm2. METHODS: Data were extracted from the French prospective MALFPULM cohort, with inclusion between March 2015 and June 2018. RESULTS: The final study population consisted of 383 women. Deliveries in level 1 or 2 maternity units (n = 98, 25%) involved CPMs with lower CVRmax (p<0.001), causing fewer signs of prenatal compression (p = 0.025). Among the 62 children (16%) who presented with NRD, only seven (11%) were born in level 1 or 2 units (p = 0.0078). Choosing the maternity level according to the CVRmax would have increased the number of births in level 1 or 2 maternity hospitals by 70%. In these maternity units, the percentage of children with NRD would have increased from 8% in the actual distribution to 10% in the new strategy. CONCLUSION: Our results showed an overuse of level 3 maternity hospitals for the delivery of newborns with a prenatal diagnosis of CPM. The use of CVRmax should enable a reduction in the use of expertise centers without an adverse impact on newborns.
Subject(s)
Cystic Adenomatoid Malformation of Lung, Congenital , Lung Diseases , Respiratory Distress Syndrome , Respiratory Insufficiency , Child , Female , Humans , Infant, Newborn , Pregnancy , Cystic Adenomatoid Malformation of Lung, Congenital/diagnosis , Prospective Studies , Lung/diagnostic imaging , Retrospective Studies , Ultrasonography, Prenatal/methodsABSTRACT
Esophageal atresia (EA) is prenatally diagnosed in less than one third of the cases and is usually only suspected. Recently, magnetic resonance imaging (MRI) with dynamic sequence and biochemistry of the amniotic fluid have been proposed to enhance prenatal diagnosis of EA. We report the case of a triple negative screening (ultrasound, MRI with dynamic sequence and biochemistry of the amniotic fluid) with a postnatal diagnosis of EA type III with a small defect. Even using second line tests, prenatal diagnosis of EA remains a challenge.
Subject(s)
Esophageal Atresia/diagnosis , Prenatal Diagnosis/standards , Adult , Amniocentesis/standards , Esophageal Atresia/diagnostic imaging , Esophageal Atresia/metabolism , Female , Humans , Infant, Newborn , Magnetic Resonance Imaging/standards , Pregnancy , Ultrasonography, Prenatal/standardsABSTRACT
OBJECTIVE: To quantify the degree of intrathoracic liver herniation by magnetic resonance imaging (MRI) and evaluate its effect on postnatal survival in fetuses with isolated congenital diaphragmatic hernia (CDH). METHODS: Forty fetuses that were expectantly managed and that were delivered after 32 weeks' gestation were included in this study. On axial T2 weighted MR images the degree of intrathoracic liver herniation was measured by volumetry, using the xyphoid process and thoracic apex as landmarks. The ratio of the volume of the liver that was herniated into the thoracic cavity to the volume of the thoracic cavity was calculated (LiTR). All the fetuses also underwent lung volumetry, and the ratio of the observed/expected total fetal lung volume (o/e TFLV) was calculated. Regression analysis was used to investigate the effect on survival of side of occurrence of CDH, o/e TFLV, LiTR and gestational age at delivery. Receiver-operating characteristics (ROC) curves were constructed to examine the prediction of survival by o/e TFLV or LiTR alone and o/e TFLV and LiTR together. RESULTS: Univariate regression analysis demonstrated that significant predictors of survival were o/e TFLV and LiTR. Multiple regression analysis demonstrated that o/e TFLV and LiTR provided independent prediction of survival. The area under the ROC curve (AUC) for the prediction of postnatal survival from o/e TFLV alone was 0.846 (P < 0.001; SE = 0.062) and the AUC from LiTR alone was 0.875 (P = 0.001; SE = 0.072). The AUC for the prediction of postnatal survival from o/e TFLV and LiTR combined was 0.912 (P < 0.001; SE = 0.045), however it was not statistically significantly different from that of o/e TFLV alone. CONCLUSION: In expectantly managed CDH fetuses, assessment of LiTR using MRI provided prediction of postnatal survival independently from o/e TFLV.
Subject(s)
Fetal Diseases/diagnosis , Hernias, Diaphragmatic, Congenital , Liver Diseases/congenital , Prenatal Diagnosis/methods , Epidemiologic Methods , Female , Fetal Diseases/mortality , Fetal Organ Maturity , Hernia, Diaphragmatic/diagnosis , Hernia, Diaphragmatic/mortality , Humans , Liver Diseases/diagnosis , Liver Diseases/mortality , Lung/embryology , Lung Volume Measurements/methods , Magnetic Resonance Imaging , PregnancyABSTRACT
OBJECTIVES: To investigate the potential value of antenatally determined total fetal lung volume (TFLV) by magnetic resonance imaging (MRI) in the prediction of the postnatal survival in congenital diaphragmatic hernia (CDH). METHODS: We examined fetuses with isolated CDH, in which MRI was used at 22-38 weeks of gestation to measure TFLV and assess intrathoracic herniation of abdominal viscera, that were liveborn after 30 weeks of gestation and had postnatal follow-up until death or discharge from hospital. Regression analysis was used to investigate the effect on survival of gestational age at diagnosis, observed to expected (o/e) TFLV, intrathoracic herniation of the liver, side of CDH, gestational age at MRI, institution, year and gestational age at delivery. In 76 fetuses measurements of o/e TFLV and the lung area to head circumference ratio (LHR) were performed within 2 weeks of each other; in these cases o/e TFLV and o/e LHR were compared for their prediction of postnatal survival. RESULTS: In the 148 cases that fulfilled the entry criteria, multiple regression analysis demonstrated that significant predictors of survival were the presence or absence of intrathoracic herniation of the liver and o/e TFLV. The area under the receiver-operating characteristics curves for prediction of postnatal survival from o/e TFLV was 0.786 (standard error, 0.059; P < 0.001) and that from o/e LHR was 0.743 (standard error, 0.069; P = 0.001). CONCLUSIONS: In the assessment of fetuses with CDH, MRI-based o/e TFLV is useful in the prediction of postnatal survival.
Subject(s)
Hernia, Diaphragmatic/diagnosis , Hernias, Diaphragmatic, Congenital , Hypertension, Pulmonary/diagnosis , Magnetic Resonance Imaging , Biometry , Cephalometry , Female , Gestational Age , Hernia, Diaphragmatic/mortality , Humans , Hypertension, Pulmonary/congenital , Hypertension, Pulmonary/mortality , Infant, Newborn , Lung Volume Measurements , Male , Predictive Value of Tests , Pregnancy , ROC Curve , Regression Analysis , Retrospective Studies , Ultrasonography, PrenatalABSTRACT
OBJECTIVES: Exclusive hepatocele is defined as a hernia containing in majority the liver with possibly some intestinal loops. This study was undertaken to evaluate neonatal morbidity and mortality in this series of exclusive hepatoceles. MATERIALS AND METHODS: We reviewed 11 cases of exclusive hepatoceles with delivery at the hospital Jeanne-de-Flandre in the CHRU of Lille, in France. RESULTS: The mean gestational age of diagnosis was 14.5+/-3.4 weeks of gestation. Karyotype determination was performed in 100% of cases: it was abnormal in one case of 11. One termination of pregnancy was performed because of trisomy 13. The mean gestational age at delivery was 38+/-1.8 weeks of gestation. Cesarean deliveries were performed in nine cases. Morbidity was important with: one case of fetal growth retardation on total hepatocele, three cases of severe respiratory distress, two cases of severe digestive complications. The mean length of stay was 42.8 days. The mean length of parenteral feeding was 14.4 days. Postnatal mortality concerned one child, which died because of a severe respiratory distress due to pulmonary hypoplasia. CONCLUSION: In this series, morbidity is thus important, making of exclusive hepatoceles a full entity among the omphaloceles. The multidisciplinary take care is more complex but conceivable.
Subject(s)
Fetal Diseases/diagnosis , Hernia, Diaphragmatic/diagnosis , Liver Diseases/diagnosis , Adult , Cesarean Section , Female , Gestational Age , Humans , Pregnancy , Pregnancy Outcome , Prognosis , Retrospective StudiesABSTRACT
INTRODUCTION: Intrauterine transfusion (IUT) has changed fetal anemia prognosis. However, long-term neurodevelopmental outcome is altered in 5% of children. Our objective was to study the contribution of fetal MRI to diagnosis brain lesions in case of fetal anemia. MATERIAL AND METHODS: Retrospective monocentric descriptive study from 2005 to 2016, including all patients followed for fetal anemia requiring IUT. The indications for MRI were: hydrops fetalis and / or hemoglobin <5â¯g / dL and / or more than 3 IUTs and / or acute severe anemia and / or ultrasound abnormality. Fetal and neonatal outcome and pediatric neurological monitoring were studied. RESULTS: 89 patients were followed for fetal anemia with IUT and 28 (29.1%) had fetal MRI, 12 of which were abnormal. Two out of twelve had abnormal ultrasound. Seven out of twelve had poor neurological prognosis: 2 medical terminations of pregnancy were performed; 2 children had severe developmental delay and 3 children had schooling difficulties. Five out of twelve children had favorable neurological prognosis. CONCLUSION: MRI of the fetal brain makes it possible to better detect brain lesions than ultrasound does in the management of severe fetal anemia and seems particularly appropriate in cases of acute anemia.
Subject(s)
Anemia/diagnostic imaging , Brain/diagnostic imaging , Fetal Diseases/diagnostic imaging , Anemia/etiology , Brain/abnormalities , Female , Fetal Diseases/etiology , Humans , Magnetic Resonance Imaging , Neuroimaging , Pregnancy , Retrospective StudiesABSTRACT
The main cause of fetal anemia is maternal red blood cell alloimmunization (AI). The search of maternal antibodies by indirect antiglobulin test allows screening for AI during pregnancy. In case of AI, fetal genotyping (for Rh-D, Rh-c, Rh-E and Kell), quantification (for anti-rhesus antibodies) and antibody titration, as well as ultrasound monitoring, are performed. This surveillance aims at screening for severe anemia before hydrops fetalis occurs. Management of severe anemia is based on intrauterine transfusion (IUT) or labor induction depending on gestational age. After intrauterine transfusion, follow-up will focus on detecting recurrence of anemia and detecting fetal brain injury. With IUT, survival of fetuses with alloimmunization is greater than 90% but 4.8% of children with at least one IUT have neurodevelopmental impairment.
Subject(s)
Anemia/therapy , Blood Transfusion, Intrauterine/methods , Erythroblastosis, Fetal/therapy , Erythrocytes/immunology , Fetal Diseases/therapy , Rh Isoimmunization/therapy , Female , Humans , PregnancyABSTRACT
OBJECTIVES: Our purpose was to study a non-invasive management of fetomaternal alloimmune thrombocytopenia (FMAIT). PATIENTS AND METHODS: Between 1996 and 2005, 18 women were treated. The population was divided into 2 groups: patients with a history of intracranial haemorrhage (ICH) in the older sibling received weekly intravenous immunoglobulin (IVIG) therapy to the mother (1 g/kg per week) without initial cordocentesis whereas patients with a history of neonatal thrombocytopenia did not undergo any treatment. RESULTS: All pregnancies with a previous FMAIT were monitored with serial ultrasound scans without cordecentesis. 15 patients had HPA-1, 2 HPA-3 and 1 HPA-5 immunizations. Weekly intravenous immunoglobulin therapy was administered in 5 patients with a history of ICH in the older sibling. Two of these delivered thrombocytopenic children; one had a platelet count < 50 x 10(9)/l. For the 13 women (one twin) who had a sibling with neonatal thrombocytopenia, 11/14 newborns had a platelet count < 50 x 10(9)/l. Predelivery fetal blood sampling were performed in 8/18 pregnancies. The neonatal periods of the 19 children were uncomplicated and no ICHs were observed. DISCUSSION AND CONCLUSION: Our results suggest that a non-invasive strategy avoiding serial cordocentesis may be an effective therapy in patients who are at risk of fetal and neonatal alloimmune thrombocytopenia.
Subject(s)
Fetal Diseases/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Thrombocytopenia/drug therapy , Adult , Cordocentesis , Female , Fetal Blood/cytology , Fetal Diseases/immunology , Humans , Infant, Newborn , Intracranial Hemorrhages/etiology , Intracranial Hemorrhages/prevention & control , Male , Maternal-Fetal Exchange , Pregnancy , Pregnancy Outcome , Risk Factors , Thrombocytopenia/complications , Thrombocytopenia/immunology , Treatment OutcomeABSTRACT
The ex utero intrapartum treatment (EXIT) procedure is a surgical procedure maintaining utero-placental circulation during caesarean section. Anaesthetic implications are described: foetal transplacental anaesthesia to avoid first breathing and to permit surgical procedure on obstructed foetal airway, deep maternal haemodynamically stable anaesthesia to relax uterine smooth muscle during a long caesarean procedure but avoiding post-partum haemorrhage. Volatile anaesthesia with sevoflurane seems to be adequate for these aims. Two cases are described.
Subject(s)
Airway Obstruction/surgery , Anesthesia, Inhalation , Anesthesia, Obstetrical , Cesarean Section , Fetal Diseases/surgery , Adult , Airway Obstruction/etiology , Anesthetics, Inhalation/administration & dosage , Female , Fetus/surgery , Goiter/complications , Goiter/surgery , Humans , Hysterotomy , Intubation, Intratracheal , Laryngostenosis/etiology , Maternal-Fetal Exchange , Methyl Ethers/administration & dosage , Pregnancy , Sevoflurane , Teratoma/complications , Teratoma/surgery , Tongue Neoplasms/complications , Tongue Neoplasms/surgery , Tracheal Stenosis/etiologyABSTRACT
Congenital diaphragmatic hernia has a physiopathology unfully understood, and is the cause of an important morbimortality. We report the case of a fetus suffering from a diaphragmatic hernia associated with a EDNRA gene triplication, coding for the endothelin 1 receptor. High-resolution genetic techniques were able to find the possible origin of this pathology, and showed that it was an isolated form with a good prognostic. ET-A receptor over-expression in lung vessels may cause a vascular remodeling and a lung arterial high blood pressure. This lung abnormality would have occurred before the diaphragmatic defect.
Subject(s)
Endothelin-1/genetics , Hernias, Diaphragmatic, Congenital/diagnostic imaging , Hernias, Diaphragmatic, Congenital/genetics , Receptor, Endothelin A/metabolism , Chromosomes, Human, Pair 4 , Female , Humans , Hypertension/etiology , Lung/abnormalities , Lung/diagnostic imaging , Pregnancy , Trisomy , Ultrasonography, PrenatalABSTRACT
OBJECTIVES: In case of hyperechogenic fetal bowel (HFB), invasive procedures such as amniocentesis are often proposed to detect an underlying cause. Our goal is to study etiologies and prognosis of HFB according to antenatal sonographic findings in order to evaluate the relevance of antenatal assessment. MATERIALS AND METHODS: It is a retrospective monocentric study lead from 2008 to 2012, including all patients with a suspicion of HFB on routine sonography. We analysed the antenatal and neonatal results, distinguishing four situations: isolated HFB, HFB+other digestive anomalies, HFB+vascular pathology, HFB+other associated anomalies. RESULTS: For 149 patients, HBF was confirmed. Sixty-nine were isolated HFB, 24 associated with other digestive anomalies, 16 with vascular pathology and 40 with other anomalies. Pregnancy outcomes were different with 92.8, 41.7, 0 and 45.0% of healthy newborns. In the case of isolated HBF, we noted 2.9% cystic fibrosis and 2.9% congenital infection. CONCLUSION: Isolated HBF seems to have a better prognosis than associated forms. However, prenatal investigations to eliminate cystic fibrosis or congenital infection should be offered and may be initially non-invasive, if a larger series confirmed the absence of dyschromosomy in this population.
Subject(s)
Cystic Fibrosis/epidemiology , Echogenic Bowel/diagnostic imaging , Echogenic Bowel/epidemiology , Fetal Diseases/epidemiology , Infant, Newborn, Diseases/epidemiology , Pregnancy Outcome/epidemiology , Comorbidity , Female , France , Humans , Infant, Newborn , Pregnancy , Prognosis , Ultrasonography, PrenatalABSTRACT
OBJECTIVES: Analyze factors leading to isolated clubfoot's occurrence, identify clubfeet associated pathologies, and discuss the opportunity of performing an amniocentesis in cases of isolated clubfoot. PATIENTS AND METHODS: Between January 2007 and December 2011, all patients diagnosed with clubfoot in our prenatal diagnostic center were retrospectively included. We then defined and analyzed idiopathic or isolated talipses equinovarus (ITEV) and clubfeet associated with others morphologic abnormalities or syndromic talipses equinovarus (STEV). RESULTS: One hundred and twenty-four clubfeet were analyzed. Forty-seven cases of ITEV, for which 34 caryotypes were performed with a normal result. Risk factors of ITEV in our series were male gender (P=0.0017), a family history of clubfoot (P=0.001) and primiparity (P=0.04). Seventy-seven cases of STEV were diagnosed, 14 of which had chromosomal abnormalities, 18 spina bifida and 10 musculo-skeletal abnormalities. Among the 124 cases of clubfeet, 25 were unilateral and 99 were bilateral. Bilateral talipses equinovarus do not constitute a risk factor of STEV (P=0.8). DISCUSSION AND CONCLUSION: We did not find any chromosomic abnormalities in cases of ITEV. The results of our study could lead to defer systematic amniocentesis in cases of primiparous women diagnosed with an ITEV, with a familial history of clubfoot and a male fetus. A referent echographist in prenatal diagnosis should still perform a systematic morphologic echography.
Subject(s)
Amniocentesis , Clubfoot/diagnostic imaging , Prenatal Diagnosis , Adult , Female , Humans , Male , Pregnancy , Retrospective Studies , Risk FactorsABSTRACT
Twin pregnancies combining complete hydatidiform mole and coexistent fetus are a rare situation (incidence in 1/20,000 in 1/100,000 pregnancies) and a challenge for diagnosis. Their complications can be important - bleeding, preeclampsia, miscarriage - and their management remains complex and controversial. In case of continuing the pregnancy, nearly 40% of women have lives babies. Three quarters of fetal loss occur before 24weeks gestation. We report here three new cases; only one of these cases had a favorable outcome.
Subject(s)
Fetal Viability , Hydatidiform Mole , Pregnancy, Twin , Uterine Neoplasms , Adult , Female , Humans , Hydatidiform Mole/diagnosis , Hydatidiform Mole/surgery , Pregnancy , Uterine Neoplasms/diagnosis , Uterine Neoplasms/surgery , Young AdultABSTRACT
OBJECTIVE: Tocolysis with calcium channel blockers is widespread in France. However, these molecules are off label use for this indication. The objective of this work is to give an update on all the side effects of calcium channel blockers published or reported to the National Bank of Pharmacovigilance. MATERIALS AND METHODS: We conducted a literature review incorporating the animal experimental data on calcium channel blockers, retrospective and prospective studies (randomized or not) comparing different tocolytics, and the published clinical case reports. Finally we inquired the National Bank of Pharmacovigilance for reported cases of adverse effects after use of calcium channel blockers as tocolytics. RESULTS: Adverse effects are found in 2% to 6% of patients after use of nifedipine, of which 0.9% to 1.9% are severe. These are mainly headache, flushes and arterial hypotension. Data on Nicardipine are more limited. For the latter, adverse effects specifically related to the route of administration (induced phlebitis) are described but it seems that other adverse effects reported are not more frequent. Several meta-analyses have demonstrated a decrease in maternal side effects when using calcium channel blockers compared to ß-agonists. Comparison of calcium channel blockers to Atosiban is less documented. More rare serious side effects are reported as clinical cases, consisting almost exclusively of cardiovascular complications (dyspnea, pulmonary edema, myocardial infarction, arterial hypotension). They are more common with Nicardipine than Nifedipine. Similar observations are found by querying the National Bank of Pharmacovigilance. CONCLUSION: The prescription of calcium channel blockers as tocolysis exposes patients to maternal side effects, which are not serious most of the time, and less frequent than with the ß-agonist. Severe maternal complications were nonetheless reported more frequently with Nicardipine than Nifedipine, which justifies avoiding Nicardipine in tocolysis. Nifedipine is the most studied molecule. It is not possible to define a maximum posology from literature data. It seems unreasonable to associate different tocolytics and necessary to closely monitor maternal blood pressure, the occurrence of a skin reaction or hypersensitivity, dyspnea or chest pain during treatment.