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Congenital Diaphragmatic Hernia (congenital + diaphragmatic_hernia)
Selected AbstractsCongenital Diaphragmatic Hernia: Update and ReviewJOURNAL OF OBSTETRIC, GYNECOLOGIC & NEONATAL NURSING, Issue 3 2001Linda J. Juretschke MSN Newborns with congenital diaphragmatic hernia (CDH) present challenges for the neonatal intensive-care nurse. Although CDH has been a known condition for almost 200 years, the treatment strategy for newborns with CDH has changed during the past decade. Despite these improvements, the mortality rate for this condition remains high. An understanding of the anatomical basis and new treatment modalities for this condition will prepare nurses to care for these newborns. [source] Expression and function of phosphodiesterases in nitrofen-induced congenital diaphragmatic hernia in ratsPEDIATRIC PULMONOLOGY, Issue 4 2010Irene W.J.M. van der Horst MD Abstract Background Congenital diaphragmatic hernia (CDH) is an anomaly associated with pulmonary hypoplasia and pulmonary hypertension (PH). The limited efficacy of current approaches to treat PH in CDH, including inhaled nitric oxide (NO), drives the search for other therapies. Phosphodiesterases (PDEs) degrade cyclic nucleotide second messenger cAMP and cGMP downstream of NO thereby limiting the vasodilatory response to NO. Objective To identify therapeutic targets by cataloguing the expression and function of PDE isoforms in the pulmonary vasculature in nitrofen-induced CDH in fetal rats. Methods/Results Quantitative RT-PCR revealed PDE1,5 and PDE9 mRNA expression in pulmonary arteries (PAs) of control and nitrofen-induced CDH term fetal rats. In this order of potency, the PDE inhibitors Sildenafil (PDE5),>,EHNA (PDE2),>,Rolipram (PDE4),>,Cilostamide (PDE3) all dilated isolated third generation PA after pre-constriction with the thromboxane analog U46619. Hyperoxic pre-incubation of PAs significantly attenuated vasodilatation induced by the PDE5 inhibitor Sildenafil (65% vs. 33%, P,<,0.004). CDH PAs dilated significantly less to PDE2 inhibitor EHNA compared to control (51% vs. 72%, P,<,0.05). Subsequently PDE2 protein expression was higher in PAs of CDH animals. Conclusion Most PDE isoforms exist in the PAs of fetal rats and their inhibition causes pulmonary vasodilatation. PDE5 inhibition was the most potent vasodilator, however, there were no differences between groups. PDE5-induced vasodilatation was attenuated by hyperoxic pre-incubation. PDE inhibitors might be considered therapeutic targets in combination with iNO in neonates with CDH. Pediatr Pulmonol. 2010; 45:320,325. © 2010 Wiley-Liss, Inc. [source] Surfactant protein expression is increased in the ipsilateral but not contralateral lungs of fetal sheep with left-sided diaphragmatic hernia,PEDIATRIC PULMONOLOGY, Issue 4 2005Marcus G. Davey PhD Abstract Congenital diaphragmatic hernia (CDH) impairs fetal lung growth and increases the density of alveolar epithelial type 2 (AE2) cells. There is controversy whether surfactant protein (SP) expression is altered in CDH. The primary aim of this study was to assess SP expression (mRNA and protein) in the left and right lungs of fetal sheep with and without a diaphragmatic hernia (DH). Left-sided DH was created in four fetal sheep at 65 days of gestational age (g.a.). Sham-operated animals were used as controls. At 138 days g.a., lungs were harvested and the following parameters were measured: SP-A, -B, and -C mRNA expression (Northern blot), SP-A and -B expression (Western blot), and AE2 cell density (immunohistochemistry). The lung weight-to-body weight ratio was reduced by 42% in DH animals. The left-to-right lung weight ratio was lower in DH animals (0.47,±,0.03 vs. 0.69,±,0.03), indicative of asymmetric lung growth. SP-A, -B, and -C mRNA expression were increased by 61.7%, 32.9%, and 75.5%, respectively, in the left lungs of DH animals. SP-A and SP-B were also increased in DH. In the right lung, SP expression (mRNA and protein) was not different between groups. AE2 cell density was higher (by 67%) in the left but not right lungs of DH animals. Although DH in fetal sheep results in significant lung hypoplasia, SP expression is not reduced. On the contrary, SP expression was increased in the ipsilateral lung of fetuses with left-sided DH. Furthermore, AE2 cell density is increased in DH, suggesting that the increase in SP mRNA and protein levels is due to increases AE2 cell number. Our data further support the premise that fetal lung hypoplasia favors an AE2 phenotype. Pediatr Pulmonol. 2005; 39:359,367. © 2005 Wiley-Liss, Inc. [source] Patent ductus arteriosus flow patterns in the treatment of congenital diaphragmatic herniaPEDIATRICS INTERNATIONAL, Issue 4 2009Shinya Okamoto Abstract Background:, Congenital diaphragmatic hernia (CDH) mortality still remains high, due to lung hypoplasia and persistent pulmonary hypertension of the neonate (PPHN). Effective management of PPHN and time of operation are quite important to the improvement of CDH treatment. In order to determine the optimal time for operation, we monitored PPHN with cardiac ultrasound. Methods:, PPHN was assessed with three parameters: patent ductus arteriosus flow patterns (PDAFP), %left ventricular diameter at diastole, and left ventricular fraction of shortening (LVFS). Four patients with an antenatal diagnosis were treated under this protocol. Diaphragm repair was performed when PDAFP became left to right shunt dominant and the pre- and postoperative course was analyzed with regular chart reviews. Results:, The alveolar-arterial oxygen difference levels of four patients were 590, 335, 613 and 530 mmHg, and operations were carried out when the patients were 2, 2, 3 and 2 days old, respectively. In three of the four patients (all except case 3) the PDAFP changed from right to left shunt dominant or bidirectional (BD), to left to right shunt dominant within 48 h. The %left ventricular diameter at diastole was relatively stable around the time of operation. The LVFS of all patients decreased after the operation. Only the LVFS of case 3 decreased temporarily to less than 30% (which indicates poor left ventricular function) but recovered. No patients needed extracorporeal membrane oxygenation support. All patients survived the procedure and were extubated. Case 3, who took 10 days to become left to right shunt dominant after the operation, needed home oxygenation therapy for 10 months. Conclusions:, PDAFP was a reliable marker of PPHN on a high-frequency oscillatory ventilator to determine the optimal time for the operation for CDH. The optimal time for operation is supposed to be the time when PDAFP become left to right shunt dominant. [source] Congenital diaphragmatic hernia and pulmonary hypoplasia: New insights from developmental biology and genetics,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 2 2007Kate G. Ackerman First page of article [source] Congenital diaphragmatic hernia: prenatal diagnosis permits immediate intensive care with high survival rate in isolated cases.PRENATAL DIAGNOSIS, Issue 7 2004A population-based study Abstract Objectives To estimate the prognosis of prenatally diagnosed isolated congenital diaphragmatic hernia (PDICDH) treated with ,immediate planned care' (IPC) between 1999 and 2003 in Eastern Brittany. Methods The prognosis of PDICDH was compared with the prognosis of the other live-born CDH, either prenatally undiagnosed or not having had IPC. IPC consisted in prenatal lung maturation with corticosteroids, elective caesarean section at 37 weeks, immediate intubation, surfactant, high- frequency ventilation or oscillation, nitric oxide, intravenous prostacyclin, anaesthesia and haemodynamic support. Surgical repair was performed in the NICU 34 h after birth. Results The incidence of CDH was 0.8, with a prenatal diagnosis rate of 27/30 (90%), leading to a termination of pregnancy in nine cases. Ten CDH were associated with other malformations. IPC in PDICDH was performed in 12 cases. The survival rate of PDICDH with IPC was 11/12 versus 1/9 in CDH with no IPC or no prenatal diagnosis (p < 0.01). Logistic regression analysis showed that IPC was determinant for survival (p < 0.01). Conclusion Prenatal diagnosis of isolated CDH treated with immediate planned care is associated with a high survival rate. This suggests that prenatal diagnosis associated with specifically adapted postnatal procedure may improve the prognosis of isolated CDH. Copyright © 2004 John Wiley & Sons, Ltd. [source] A review of Donnai-Barrow and facio-oculo-acoustico-renal (DB/FOAR) syndrome: Clinical features and differential diagnosisBIRTH DEFECTS RESEARCH, Issue 1 2009Barbara R. Pober Abstract Mutations in the gene LRP2 have recently been identified as the cause of Donnai-Barrow and Facio-oculo-acoustico-renal (DB/FOAR) syndrome. More than two dozen cases, the first reported more than 30 years ago by Holmes, have been published. Summarizing available information, we highlight the cardinal features of the disorder found in ,90% of published cases. These features include: agenesis of the corpus callosum, developmental delay, enlarged anterior fontanelle, high myopia, hypertelorism, proteinuria, and sensorineural hearing loss. Congenital diaphragmatic hernia and omphalocele are reported in only half of the patients. There is no evidence for genotype-phenotype correlation, though the sample size is too small to preclude this with certainty. Although several conditions to consider in the differential diagnosis are highlighted, the diagnosis of DB/FOAR syndrome should not be difficult to establish as its constellation of findings is strikingly characteristic. Birth Defects Research (Part A), 2009. © 2008 Wiley-Liss, Inc. [source] Nutrient intakes in women and congenital diaphragmatic hernia in their offspring,BIRTH DEFECTS RESEARCH, Issue 3 2008Wei Yang Abstract BACKGROUND: Congenital diaphragmatic hernia (CDH) is a severe birth defect where there is an opening in the diaphragm through which a portion of the abdominal contents protrudes into the thoracic cavity. The etiologies of CDH remain unknown, although experimental animal data suggest dietary factors might play a role. This study examined whether maternal nutrient intakes were associated with delivering infants with CDH. METHODS: We analyzed infants with isolated CDH who were born from 1997 to 2003 and recruited into the National Birth Defects Prevention Study (NBDPS), a multisite, population-based case-control study. Exposure data were obtained from telephone interviews, which were completed within 24 months after delivery, and were available for 377 case mothers and 5,008 control mothers. A food frequency questionnaire was used to derive nutrient intakes during the year before pregnancy. RESULTS: A crude OR of 0.6 (95% CI: 0.3,1.0) was observed for higher intake of choline. Elevated ORs (1.4 to 1.7) were found for lower intakes of choline, cysteine, methionine, and protein. Among women who took vitamin supplements, higher intakes of B vitamins (i.e., folate, vitamin B1, B2, B6, and B12), minerals (i.e., calcium, iron, magnesium, and zinc), and vitamin E were inversely associated with CDH (ORs from 0.7,0.3). Moreover, among women who did not take vitamin supplements, lower intakes of calcium, retinol, selenium, vitamin B12, and vitamin E had positive associations with CDH (ORs from 1.4 to 2.1). CONCLUSIONS: Our observations contribute to a limited body of evidence suggesting a woman's periconceptional diet might be associated with CDH in her offspring. Birth Defects Research (Part A), 2008. © 2007 Wiley-Liss, Inc. [source] Linking animal models to human congenital diaphragmatic herniaBIRTH DEFECTS RESEARCH, Issue 8 2007Niels Beurskens Abstract BACKGROUND:Congenital diaphragmatic hernia (CDH) is a major life-threatening malformation, occurring in approximately 1 in 3,000 live births. Over the years, different animal models have been used to gain insight into the etiology of this complex congenital anomaly and to develop treatment strategies. However, to date the pathogenic mechanism is still not understood, and treatment remains difficult because of the associated pulmonary hypoplasia and pulmonary hypertension. METHODS: In this review, data available from several animal models will be discussed. The retinoic acid signaling pathway (RA pathway, retinoid pathway) will be addressed as a developmental pathway that is potentially disrupted in the pathogenesis of CDH. Furthermore, genetic factors involved in diaphragm and lung development will be discussed. CONCLUSIONS: With this review article, we aim to provide a concise overview of the current most important experimental genetic data available in the field of CDH. Birth Defects Research (Part A), 2007. © 2007 Wiley-Liss, Inc. [source] Congenital diaphragmatic hernia: review of the literature in reflection of unresolved dilemmasACTA PAEDIATRICA, Issue 12 2009S Grisaru-Granovsky Abstract Background:, Congenital diaphragmatic hernia (CDH) is a rare but clinically and scientifically challenging condition. The introduction of ultrasound has enabled early prenatal detection and consequently, hope of early therapeutic intervention. Aim:, We undertook the task to review the recent developments in understanding the pathology of CDH as well as the history and current management strategies to aid perinatologists in consultations with parents of CDH-affected foetuses. Study design:, A Medline search was undertaken of all reports and reviews published between 1980 and 2008 using MeSH search terms ,diaphragmatic hernia', ,congenital' and ,newborn'. Results:, The true incidence of CDH is still difficult to estimate because of the high incidence of hidden mortality of CDH. Complete case ascertainment also poses difficulties in assessment of the impact of new therapeutic modalities on overall survival. Recent improvements in prenatal detection are a milestone in affording time for re-assessments and parental counselling. The true benefit of antenatal therapy is circumscribed and should be offered only in selected cases of isolated severe CDH as defined by existing guidelines. Postnatal intensive respiratory supportive therapy and innovative surgical techniques within specialized tertiary centres has had a major impact on survival of babies with CDH. Conclusion:, The high survival of ,selected cases' that are live births and benefit from optimal care will be difficult to improve by antenatal interventions. The multidisciplinary approach to basic research and randomized clinical trials will further define the best approach to the foetus and neonate with CDH. [source] Congenital diaphragmatic hernia: an overview of the etiology and current managementACTA PAEDIATRICA, Issue 4 2009Alejandra Gaxiola Abstract Aim: To review provide an overview of the etiology and current strategies in the management of congenital diaphragmatic hernia (CDH). Methods: We did a comprehensive review of research trends, evidence based studies and epidemiologic studies. Results: CDH is a life-threatening pathology in infants, and a major cause of death due to the pulmonary hypoplasia and pulmonary hypertension. There is much research related to elucidating the etiology of CDH and developing management strategies to improve the outcomes in these infants. Conclusion: An early diagnosis with increased understanding of this disease is a crucial factor for a timely approach to managing the critically ill infant, and to offer the potential for improved outcomes and substantial reductions in morbidity. [source] Three-dimensional sonographic measurement of contralateral lung volume in fetuses with isolated congenital diaphragmatic hernia,JOURNAL OF CLINICAL ULTRASOUND, Issue 5 2008Rodrigo Ruano MD Abstract Purpose To use 3-dimensional sonography (3DUS) to measure contralateral lung volume and evaluate the potential of this measurement to predict neonatal outcome in isolated congenital diaphragmatic hernia (CDH). Methods Between January 2002 and December 2004, the contralateral lung volumes of 39 fetuses with isolated CDH were measured via 3DUS using rotational multiplanar imaging. The observed/expected contralateral fetal lung volume ratios (o/e-ContFLVR) were compared with the lung/head ratio (LHR), observed/expected total fetal lung volume ratio (o/e-TotFLVR), and postnatal outcome. Results Contralateral lung volumes are less reduced than total lung volumes in CDH. The bias and precision of 3DUS in estimating contralateral lung volumes were 0.99 cm3 and 1.11 cm3, respectively, with absolute limits of agreement ranging from ,1.19 cm3 to +3.17 cm3. The o/e-ContFLVR was significantly lower in neonatal death cases (median, 0.49 cm3; range, 0.22,0.99 cm3) than in survival cases (median, 0.58 cm3; range, 0.42,0.92 cm3 [p < 0.01]). Overall accuracy of the o/e-ContFLVR, o/e-TotFLVR, and LHR in predicting neonatal death were 67.7% (21/31), 80.7% (25/31), and 77.4% (24/31), respectively. Conclusion Although o/e-ContFLVR can be precisely measured with 3DUS and can be used to predict neonatal death in CDH, it is less accurate than LHR and o/e-TotFLVR for that purpose. © 2007 Wiley Periodicals, Inc. J Clin Ultrasound, 2008 [source] Congenital lung malformations in the second trimester: Prenatal ultrasound diagnosis and pathologic findingsJOURNAL OF CLINICAL ULTRASOUND, Issue 5 2007Ágnes Harmath MD Abstract Purpose. To correlate prenatal sonographic diagnosis of cystic lung malformations with fetopathologic findings after termination of pregnancy. Methods. We retrospectively analyzed the data of 16 terminated cases in which a cystic lung lesion was diagnosed pre- or postnatally. Results. On average, prenatal diagnosis was established on the 21st gestational week (range, 19,26 weeks). The cause of termination was severe polyhydramnios in 4 cases, nonimmune fetal hydrops in 4 cases, other congenital malformation in 5 cases (renal malformation, 2 cases; congenital diaphragmatic hernia, 3 cases), and obstetrical conditions (intrauterine death, placental abruption, spontaneous abortion) in 3 cases. In 11 cases, congenital cystic adenomatoid malformation (CCAM) was the presumptive prenatal diagnosis. Autopsy confirmed the prenatal diagnosis in 6 of them, while in the other 5 cases, an enteric cyst, a laryngeal atresia, an unidentified tumor, a pulmonary hypoplasia, and an extralobar pulmonary sequestration were found on histologic examination. On the other hand, the autopsy revealed CCAM in those 5 cases in which other malformations were suggested prenatally. Conclusion. The prenatal sonographic diagnosis of CCAM is difficult. Our cases emphasize the important role of fetopathology even today in the verification of prenatal diagnosis based on sonographic examinations. © 2007 Wiley Periodicals, Inc. J Clin Ultrasound, 2007 [source] Congenital Diaphragmatic Hernia: Update and ReviewJOURNAL OF OBSTETRIC, GYNECOLOGIC & NEONATAL NURSING, Issue 3 2001Linda J. Juretschke MSN Newborns with congenital diaphragmatic hernia (CDH) present challenges for the neonatal intensive-care nurse. Although CDH has been a known condition for almost 200 years, the treatment strategy for newborns with CDH has changed during the past decade. Despite these improvements, the mortality rate for this condition remains high. An understanding of the anatomical basis and new treatment modalities for this condition will prepare nurses to care for these newborns. [source] Neurodevelopmental outcomes and surgery in neonatesJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 12 2006Karen Walker Abstract: A neonate requiring major surgery in 2006 has a greater prospect of survival than ever before. Increasingly, however, there is awareness that critical illness may affect later neurodevelopment. Pre-existing conditions in addition to the physiologic stresses associated with cardiac and general surgery are implicated but remain unavoidable in the case of significant structural abnormalities such as transposition of the great arteries or congenital diaphragmatic hernia. For those affected by neurodevelopmental impairment, there is a significant cost to the child, family and society. Current research focuses on the preventable causes of brain injury, before, during and after the intervention, and the rate of impairment in apparently uncomplicated procedures. In contrast to the quantity of neurodevelopmental outcome data following cardiac surgery, there remain few outcome studies dealing with non-cardiac surgery despite such intervention being two to three times more common. There appear to be compelling clinical and economic arguments for the instigation of formalised population-based developmental assessments for all infants undergoing major surgery. [source] Intrathoracic gastric volvulus mimicking pyloric stenosisJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 2 2003JJ Munoz Abstract: A 5-week-old-infant presented to hospital following the acute onset of non-bilious vomiting with clinical and acid,base features suggestive of pyloric stenosis. A chest radiograph obtained because of intercurrent infection unexpectedly revealed a left-sided congenital diaphragmatic hernia. A barium meal demonstrated the presence of an intrathoracic gastric volvulus, requiring urgent surgical management. We discuss the presentation and management of this rare surgical cause of non-bilious vomiting in infancy. [source] Repair of a large congenital diaphragmatic defect with a reverse latissimus dorsi muscle flapMICROSURGERY, Issue 2 2008Rui F. Barbosa M.D. Advances in neonatal care have led to an increase in the survival rate of children with large congenital diaphragmatic defects. Reconstruction by direct closure is not usually possible and surgical correction in the newborn consists of synthetic patch closure. Recurrence and complications are high with this technique and a latter reconstruction with living tissue is now advocated. We report a case of congenital diaphragmatic hernia treated initially by synthetic patch closure and 10 months latter, after a recurrence, with a reinnervated reverse latissimus dorsi (RLD) flap. We used an end-to-side neural coaptation of the thoracodorsal nerve to the phrenic nerve. The result was satisfactory, with remission of symptoms and improvement in growth and development, with permanent pleuro-peritoneal separation, normal lung growth, and apparent neodiaphragmatic function. © 2008 Wiley-Liss, Inc. Microsurgery, 2008. [source] Evolving management of congenital diaphragmatic herniaPEDIATRIC ANESTHESIA, Issue 8 2007ROBIN A. BROWN MBChB DCH FRCS FCS MPhil First page of article [source] Expression and function of phosphodiesterases in nitrofen-induced congenital diaphragmatic hernia in ratsPEDIATRIC PULMONOLOGY, Issue 4 2010Irene W.J.M. van der Horst MD Abstract Background Congenital diaphragmatic hernia (CDH) is an anomaly associated with pulmonary hypoplasia and pulmonary hypertension (PH). The limited efficacy of current approaches to treat PH in CDH, including inhaled nitric oxide (NO), drives the search for other therapies. Phosphodiesterases (PDEs) degrade cyclic nucleotide second messenger cAMP and cGMP downstream of NO thereby limiting the vasodilatory response to NO. Objective To identify therapeutic targets by cataloguing the expression and function of PDE isoforms in the pulmonary vasculature in nitrofen-induced CDH in fetal rats. Methods/Results Quantitative RT-PCR revealed PDE1,5 and PDE9 mRNA expression in pulmonary arteries (PAs) of control and nitrofen-induced CDH term fetal rats. In this order of potency, the PDE inhibitors Sildenafil (PDE5),>,EHNA (PDE2),>,Rolipram (PDE4),>,Cilostamide (PDE3) all dilated isolated third generation PA after pre-constriction with the thromboxane analog U46619. Hyperoxic pre-incubation of PAs significantly attenuated vasodilatation induced by the PDE5 inhibitor Sildenafil (65% vs. 33%, P,<,0.004). CDH PAs dilated significantly less to PDE2 inhibitor EHNA compared to control (51% vs. 72%, P,<,0.05). Subsequently PDE2 protein expression was higher in PAs of CDH animals. Conclusion Most PDE isoforms exist in the PAs of fetal rats and their inhibition causes pulmonary vasodilatation. PDE5 inhibition was the most potent vasodilator, however, there were no differences between groups. PDE5-induced vasodilatation was attenuated by hyperoxic pre-incubation. PDE inhibitors might be considered therapeutic targets in combination with iNO in neonates with CDH. Pediatr Pulmonol. 2010; 45:320,325. © 2010 Wiley-Liss, Inc. [source] The effect of fetal tracheal occlusion on lung tissue mechanics and tissue composition,PEDIATRIC PULMONOLOGY, Issue 2 2009Jacques C. Jani MD Abstract Fetal tracheal occlusion (TO) is currently used to treat severe cases of congenital diaphragmatic hernia (DH). Clinical and experimental studies suggest an improved postnatal outcome, but lung tissue mechanics after TO have not been studied. We determined the effect of TO on mechanical impedance and lung tissue components in a rabbit model for DH. At 23 days of gestation (term,=,31 days) either a sham thoracotomy or a diaphragmatic defect was induced. DH fetuses were randomly assigned to undergo 5 days later TO. Fetuses were delivered by term cesarean section to determine lung to body weight ratio (LBWR), dynamic lung mechanics and lung impedance. Airway resistance (Raw), elastance (HL), tissue damping (GL) and hysteresivity (GL/HL) were calculated from impedance data. Collagen I and III and elastin were quantified histologically. LBWR was significantly increased by TO compared to DH (P,<,0.001) and resistance and compliance of the respiratory system (Rrs, Crs) were improved as well. TO resulted in a significant decrease of Raw comparable to observations in sham-fetuses, without effect on lung tissue mechanics HL, GL and hysteresivity. This coincides with a significant decrease of collagen I, III and elastin in comparison to DH fetuses. In this first report on lung tissue mechanics in a rabbit model of DH, TO had a substantial effect on tissue morphology yet this was not mirrored in lung mechanics. We conclude that the effect of TO on lung mechanics without in utero reversal of occlusion, is dominated by airway remodeling. Pediatr Pulmonol. 2009; 44:112,121. © 2009 Wiley-Liss, Inc. [source] Neuronal nitric oxide synthase does not contribute to the modulation of pulmonary vascular tone in fetal lambs with congenital diaphragmatic hernia (nNOS in CDH lambs),PEDIATRIC PULMONOLOGY, Issue 4 2008Anthony S. de Buys Roessingh MD Abstract Aim The aim of this study was to determine the presence of the neuronal nitric oxide synthase (nNOS) in near full-term lambs with congenital diaphragmatic hernia (CDH) and its role in the modulation of pulmonary vascular basal tone. Methods We surgically created diaphragmatic hernia on the 85th day of gestation. On the 135th, catheters were used to measure pulmonary pressure and blood flow. We tested the effects of 7-nitroindazole (7-NINA), a specific nNOS antagonist and of N -nitro- l -arginine (l -NNA), a nonspecific nitric oxide synthase antagonist. In vitro, we tested the effects of the same drugs on isolated pulmonary vessels. The presence of nNOS protein in the lungs was detected by Western blot analysis. Results Neither 7-NINA nor l -NNA modified pulmonary vascular basal tone in vivo. After l -NNA injection, acetylcholine (ACh) did not decrease significantly pulmonary vascular resistance (PVR). In vitro, l -NNA increased the cholinergic contractile-response elicited by electric field stimulation (EFS) of vascular rings from lambs with diaphragmatic hernia. Conclusion We conclude that nNOS protein is present in the lungs and pulmonary artery of near full-term lamb fetuses with diaphragmatic hernia, but that it does not contribute to the reduction of pulmonary vascular tone at birth. Pediatr Pulmonol. 2008; 43:313,321. © 2008 Wiley-Liss, Inc. [source] Patent ductus arteriosus flow patterns in the treatment of congenital diaphragmatic herniaPEDIATRICS INTERNATIONAL, Issue 4 2009Shinya Okamoto Abstract Background:, Congenital diaphragmatic hernia (CDH) mortality still remains high, due to lung hypoplasia and persistent pulmonary hypertension of the neonate (PPHN). Effective management of PPHN and time of operation are quite important to the improvement of CDH treatment. In order to determine the optimal time for operation, we monitored PPHN with cardiac ultrasound. Methods:, PPHN was assessed with three parameters: patent ductus arteriosus flow patterns (PDAFP), %left ventricular diameter at diastole, and left ventricular fraction of shortening (LVFS). Four patients with an antenatal diagnosis were treated under this protocol. Diaphragm repair was performed when PDAFP became left to right shunt dominant and the pre- and postoperative course was analyzed with regular chart reviews. Results:, The alveolar-arterial oxygen difference levels of four patients were 590, 335, 613 and 530 mmHg, and operations were carried out when the patients were 2, 2, 3 and 2 days old, respectively. In three of the four patients (all except case 3) the PDAFP changed from right to left shunt dominant or bidirectional (BD), to left to right shunt dominant within 48 h. The %left ventricular diameter at diastole was relatively stable around the time of operation. The LVFS of all patients decreased after the operation. Only the LVFS of case 3 decreased temporarily to less than 30% (which indicates poor left ventricular function) but recovered. No patients needed extracorporeal membrane oxygenation support. All patients survived the procedure and were extubated. Case 3, who took 10 days to become left to right shunt dominant after the operation, needed home oxygenation therapy for 10 months. Conclusions:, PDAFP was a reliable marker of PPHN on a high-frequency oscillatory ventilator to determine the optimal time for the operation for CDH. The optimal time for operation is supposed to be the time when PDAFP become left to right shunt dominant. [source] The making of fetal surgeryPRENATAL DIAGNOSIS, Issue 7 2010Jan A. Deprest Abstract Fetal diagnosis prompts the question for fetal therapy in highly selected cases. Some conditions are suitable for in utero surgical intervention. This paper reviews historically important steps in the development of fetal surgery. The first invasive fetal intervention in 1963 was an intra-uterine blood transfusion. It took another 20 years to understand the pathophysiology of other candidate fetal conditions and to develop safe anaesthetic and surgical techniques before the team at the University of California at San Francisco performed its first urinary diversion through hysterotomy. This procedure would be abandoned as renal and pulmonary function could be just as effectively salvaged by ultrasound-guided insertion of a bladder shunt. Fetoscopy is another method for direct access to the feto-placental unit. It was historically used for fetal visualisation to guide biopsies or for vascular access but was also abandoned following the introduction of high-resolution ultrasound. Miniaturisation revived fetoscopy in the 1990s, since when it has been successfully used to operate on the placenta and umbilical cord. Today, it is also used in fetuses with congenital diaphragmatic hernia (CDH), in whom lung growth is triggered by percutaneous tracheal occlusion. It can also be used to diagnose and treat urinary obstruction. Many fetal interventions remain investigational but for a number of conditions randomised trials have established the role of in utero surgery, making fetal surgery a clinical reality in a number of fetal therapy programmes. The safety of fetal surgery is such that even non-lethal conditions, such as myelomeningocoele repair, are at this moment considered a potential indication. This, as well as fetal intervention for CDH, is currently being investigated in randomised trials. Copyright © 2010 John Wiley & Sons, Ltd. [source] The use of combined ultrasound and magnetic resonance imaging in the detection of fetal anomaliesPRENATAL DIAGNOSIS, Issue 5 2010Xiomara M. Santos Abstract Objective To compare the referral diagnosis based on prenatal ultrasound to diagnoses made following combined ultrasound (US) and magnetic resonance imaging (MRI) evaluation at the Texas Children's Fetal Center (TCFC) and postnatal diagnosis. Methods We performed a retrospective review of patients referred to the TCFC between September 2001 and July 2007 with a fetal structural malformation. Data were abstracted to compare the referral diagnosis to TCFC imaging diagnoses and both were compared to postnatal diagnosis. Results Two hundred and twenty-four patients were referred who had a fetal US and MRI at TCFC. The most frequent indications were for abnormalities of the central nervous system (38%) and lung/thoracic cavity (34%), with congenital diaphragmatic hernia (CDH) the single most common referral diagnosis (n = 39; 17%). In 99 cases (42.7%) the referral diagnosis was concordant with the post-referral diagnosis, however, in 68 cases (29.3%) the post-referral diagnosis changed completely, and in 65 cases (28%) additional findings were discovered. Prenatal diagnoses following imaging at TCFC were concordant with postnatal diagnoses in 94.9% of cases. Conclusions Combined ultrasound and MRI provides additional diagnostic information or a corrected diagnosis in 57% of cases over the referral ultrasound diagnosis. Copyright © 2010 John Wiley & Sons, Ltd. [source] Molecular characterization of a ring chromosome 15 in a fetus with intra uterine growth retardation and diaphragmatic herniaPRENATAL DIAGNOSIS, Issue 5 2007Elghezal Hatem Abstract Objective To improve the phenotype-genotype correlation in terminal 15q deletions and ring chromosome 15 syndrome. Methods Echographic examination of fetus. R-banded chromosome and FISH analysis on cultured amniocytes. Microsatellite analysis to determine parental origin of the ring chromosome 15. Fetal autopsy. Results We report a new case of prenatal diagnosis of congenital diaphragmatic hernia and intrauterine growth retardation in a fetus with ring chromosome 15 involving 15q26.1-qter deletion. Conclusion This case support the evidence that the region 15q26.3 is implicated in intrauterine growth retardation and suggests that the 15q critical region implicated in congenital diaphragmatic hernia is localized in 15q26.1,q26.2. Copyright © 2007 John Wiley & Sons, Ltd. [source] Congenital diaphragmatic hernia: prenatal diagnosis permits immediate intensive care with high survival rate in isolated cases.PRENATAL DIAGNOSIS, Issue 7 2004A population-based study Abstract Objectives To estimate the prognosis of prenatally diagnosed isolated congenital diaphragmatic hernia (PDICDH) treated with ,immediate planned care' (IPC) between 1999 and 2003 in Eastern Brittany. Methods The prognosis of PDICDH was compared with the prognosis of the other live-born CDH, either prenatally undiagnosed or not having had IPC. IPC consisted in prenatal lung maturation with corticosteroids, elective caesarean section at 37 weeks, immediate intubation, surfactant, high- frequency ventilation or oscillation, nitric oxide, intravenous prostacyclin, anaesthesia and haemodynamic support. Surgical repair was performed in the NICU 34 h after birth. Results The incidence of CDH was 0.8, with a prenatal diagnosis rate of 27/30 (90%), leading to a termination of pregnancy in nine cases. Ten CDH were associated with other malformations. IPC in PDICDH was performed in 12 cases. The survival rate of PDICDH with IPC was 11/12 versus 1/9 in CDH with no IPC or no prenatal diagnosis (p < 0.01). Logistic regression analysis showed that IPC was determinant for survival (p < 0.01). Conclusion Prenatal diagnosis of isolated CDH treated with immediate planned care is associated with a high survival rate. This suggests that prenatal diagnosis associated with specifically adapted postnatal procedure may improve the prognosis of isolated CDH. Copyright © 2004 John Wiley & Sons, Ltd. [source] Second-trimester diagnosis of complete trisomy 9 associated with abnormal maternal serum screen results, open sacral spina bifida and congenital diaphragmatic hernia, and review of the literaturePRENATAL DIAGNOSIS, Issue 6 2004Chih-Ping Chen Abstract Objectives To present the prenatal diagnosis of complete trisomy 9 and to review the literature Case A 25-year-old primigravida woman was referred for amniocentesis at 19 weeks' gestation because of abnormal maternal screen results showing an elevated maternal serum alpha-fetoprotein (MSAFP) level and a low maternal serum free ,-human chorionic gonadotrophin (MSfree,-hCG) level. Results Genetic amniocentesis revealed a karyotype of 47,XX,+9 in the amniocytes and an elevated amniotic fluid AFP level. Ultrasonography demonstrated intrauterine growth restriction, left congenital diaphragmatic hernia, fetal ascites, a sacral spina bifida, a horseshoe kidney, and absence of amniotic fluid. Ultrafast magnetic resonance imaging scans further depicted detailed anatomical configurations of the major congenital malformations. The pregnancy was terminated subsequently. The proband postnatally manifested characteristic facial dysmorphism, limb deformities, and an open sacral spina bifida with myelomeningocele. Cytogenetic analysis of the skin fibroblasts revealed a karyotype of 47,XX,+9. Molecular studies of various uncultured fetal tissues using microsatellite markers confirmed a diagnosis of complete trisomy 9 resulting from a meiotic I nondisjunction error of maternal origin. Conclusion Complete trisomy 9 can be identified prenatally with advanced maternal age, sonographically detected fetal structural abnormalities, and abnormal maternal serum screen results. Fetuses with complete trisomy 9 may be associated with congenital diaphragmatic hernia, an open sacral spina bifida, elevated MSAFP, and low MSfree,-hCG. We suggest detailed prenatal imaging investigations and genetic analyses of multiple fetal tissues when a prenatal diagnosis of trisomy 9 is made. Copyright © 2004 John Wiley & Sons, Ltd. [source] Deletion 15q24-26 in prenatally detected diaphragmatic hernia: increasing evidence of a candidate region for diaphragmatic developmentPRENATAL DIAGNOSIS, Issue 4 2001D. Schlembach Abstract Survival of children with congenital diaphragmatic hernia (CDH) is mainly dependent on the extent of lung hypoplasia and the presence of additional congenital anomalies or chromosomal aberrations. A chromosomal deletion 15q25-q26.2 in a fetus with prenatally diagnosed CDH and growth retardation is reported. Despite optimal pre- and neonatal management the baby died shortly after birth. There is increasing evidence that the long arm of chromosome 15, and especially the region 15q24 to 15q26, plays a crucial role in the development of the diaphragm. The finding of a deletion within 15q24-26 in a fetus with CDH has to be considered a predictor of poor prognosis. It is of utmost interest for proper parental counselling to search in fetuses with CDH for subtle chromosomal lesions paying special attention to chromosome 15q. Copyright © 2001 John Wiley & Sons, Ltd. [source] Two new approaches in intrauterine tracheal occlusion using an ultrathin fetoscopeTHE LARYNGOSCOPE, Issue 2 2010Michael Tchirikov MD Abstract Objectives/Hypothesis: To introduce and establish a new approach in minimal invasive fetoscopic surgery in order to reduce access trauma and the iatrogenic preterm premature rupture of the membranes (PPROM) as a major complication of intrauterine treatment of congenital diaphragmatic hernia. Methods: In total, 27 pregnant sheep were operated on using fetoscopes with 1.2 and 1.0 mm optics. We used an elliptic sheath alone with a maximum diameter of 2.6/1.3 mm; in these cases the balloon was placed under ultrasound control. In comparison, we placed the balloon under fetoscopic control using the fetoscopic sheath and a 7F (2.3 mm) introducer. Therefore, the maximum access trauma was not bigger than the diameter of sheath of introducer. Results: With this technique we successfully operated on 22 sheep. The use of real time three-dimensional ultrasound control distinctly facilitates the operation procedure. Conclusions: Our preliminary findings show that fetoscopic tracheal occlusion using ultrathin fetoscopes and reducing the access trauma on the level of 4.2 or even 2.65 mm2 could be seen as a method of reducing the rate of PPROM. Laryngoscope, 2010 [source] Unusual case of Morgagni hernia associated with malrotationANZ JOURNAL OF SURGERY, Issue 9 2003Fiona G . Court Morgagni herniae are rare congenital diaphragmatic hernia, which normally present late in adult life with minimal symptoms. They are always associated with a peritoneal hernial sac, and often contain transverse colon or stomach. We present an unusual case of a Morgagni hernia containing caecum in an 81-year-old woman, post ruptured aortic aneurysm repair. [source] | |||||||||||||