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Diaphragmatic Hernia (diaphragmatic + hernia)
Kinds of 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] Diaphragmatic hernia in horses: 44 cases (1986,2006)JOURNAL OF VETERINARY EMERGENCY AND CRITICAL CARE, Issue 4 2009Samantha K. Hart BVMS (Hons) Abstract Objectives , To present a case series of horses diagnosed with diaphragmatic hernia, and to determine the significance of (1) historical information, examination findings, and laboratory data; and (2) exploratory laparotomy or necropsy findings on short- and long-term outcome. Setting , University Referral Hospital. Design , Retrospective study. Animals , Forty-four horses/foals admitted between 1986 and 2006 with a diagnosis of diaphragmatic hernia made either at exploratory laparotomy or necropsy. Interventions , None. Measurements and Main Results , Information from the medical records included history, clinical examination findings at presentation, and findings of exploratory laparotomy or necropsy. Logistic regression or the Fisher exact test was used to determine factors associated with survival. Outcome was defined as survival to discharge (short-term survival), and long-term survival was defined as horses alive at least 1-year post surgery. Of the 44 horses, 18 died or were euthanized before surgery. Twenty-six were taken to surgery, 17 were euthanized. Nine horses recovered from anesthesia, 7 of which survived to hospital discharge. Of these, 5 were alive at long-term follow-up. Survival was significantly associated with the age of the horse (,2 y old) at presentation, presence of normal peritoneal fluid at presentation, amount of compromised viscera at surgery (<50% small intestine), and the size (<10 cm) and location (ventral) of the diaphragmatic tear. Conclusion , This study confirms that size and location of the lesion do play a significant role in prognosis. And, although the prognosis for horses with diaphragmatic hernia is poor, if horses have operable lesions there is a fair prognosis for long-term survival. [source] Teratogenic effect of bis-diamine on embryonic rat heartCONGENITAL ANOMALIES, Issue 3 2000Masao Nakagawa ABSTRACT, Bis-diamine induces conotruncal anomalies including persistent truncus arteriosus, tetralogy of Fallot, interruption of the aortic arch, and ventricular septal defect in rat embryos when administered to the mother. Bis-diamine also induces extracardiac malformations including thymic hypoplasia, facial dysmorphism, forelimb anomalies and diaphragmatic hernia. However, the teratogenic mechanisms of this chemical in early developing rat hearts have not been fully established. Chimeric studies in chick and quail embryos demonstrated that the cranial neural crest cells reached the cardiac outflow tract, contributing to aorticopulmonary and truncal septation. Since an ablation of the cranial neural crest also produced the conotruncal anomalies, bis-diamine is proposed to disturb the normal migration of cardiac neural crest cells to the heart. Based on our data concerning cardiac anomalies induced by bis-diamine, we reviewed how the cardiac malformations were morphologically established in early developing rat hearts. Our data showed that 1) cardiovascular anomalies induced by bis-diamine are time- and species or strain- dependent. 2) bis-diamine reduces the number of neural crest cells migrating to participate in the conotruncal septation, 3) bis-diamine induces anomalous coronary arteries, thin ventricular walls and epicardial defects, and 4) some embryos cultured in the medium containing bis-diamine had extra-cardiac abnormalities including abnormal location of the otic placodes and delay in mid brain closure. Conclusively, bis-diamine does not appear to merely affect the cardiac development, but rather disturbs normal development of all the organs contributed to by neural crest cells. [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] Diaphragmatic hernia in horses: 44 cases (1986,2006)JOURNAL OF VETERINARY EMERGENCY AND CRITICAL CARE, Issue 4 2009Samantha K. Hart BVMS (Hons) Abstract Objectives , To present a case series of horses diagnosed with diaphragmatic hernia, and to determine the significance of (1) historical information, examination findings, and laboratory data; and (2) exploratory laparotomy or necropsy findings on short- and long-term outcome. Setting , University Referral Hospital. Design , Retrospective study. Animals , Forty-four horses/foals admitted between 1986 and 2006 with a diagnosis of diaphragmatic hernia made either at exploratory laparotomy or necropsy. Interventions , None. Measurements and Main Results , Information from the medical records included history, clinical examination findings at presentation, and findings of exploratory laparotomy or necropsy. Logistic regression or the Fisher exact test was used to determine factors associated with survival. Outcome was defined as survival to discharge (short-term survival), and long-term survival was defined as horses alive at least 1-year post surgery. Of the 44 horses, 18 died or were euthanized before surgery. Twenty-six were taken to surgery, 17 were euthanized. Nine horses recovered from anesthesia, 7 of which survived to hospital discharge. Of these, 5 were alive at long-term follow-up. Survival was significantly associated with the age of the horse (,2 y old) at presentation, presence of normal peritoneal fluid at presentation, amount of compromised viscera at surgery (<50% small intestine), and the size (<10 cm) and location (ventral) of the diaphragmatic tear. Conclusion , This study confirms that size and location of the lesion do play a significant role in prognosis. And, although the prognosis for horses with diaphragmatic hernia is poor, if horses have operable lesions there is a fair prognosis for long-term survival. [source] Surgical repair of a peritoneopericardial diaphragmatic hernia in a pregnant dogJOURNAL OF VETERINARY EMERGENCY AND CRITICAL CARE, Issue 1 2007Gretchen D. Statz DVM Abstract Objective: To describe the surgical repair and pre- and postoperative management of a peritoneopericardial diaphragmatic hernia (PPDH) in a pregnant dog. Case summary: A pregnant dog was presented for vomiting, lethargy, and pale mucous membranes. Pulsus paradoxus was noted on physical examination. The dog was diagnosed with a PPDH via thoracic radiographs, abdominal ultrasound, and an echocardiogram. The hernia was surgically repaired and the dog received supportive medical care until the puppies were old enough to be delivered via cesarean section. The mother and all puppies survived. New or unique information provided: This is the first report that describes the surgical repair and postoperative management of a PPDH in a pregnant dog. [source] Double Chambered Right Ventricle in 9 CatsJOURNAL OF VETERINARY INTERNAL MEDICINE, Issue 1 2007H. Koffas Background: Double-chambered right ventricle (DCRV) is a frequently recognized cardiac congenital abnormality in humans. It has been described in dogs and in 1 cat. However systemic description of clinical and echocardiographic features of the disease in cats is currently lacking from the veterinary literature. Animals: Nine cats with DCRV are described. Results: The cats ranged from 4 months to 10 years of age. Eight cats at presentation were asymptomatic and 1 cat had chylothorax. In all cases echocardiography revealed abnormal fibromuscular bundles obstructing the mid-right ventricle, dividing the chamber into 2 compartments. The proximal right ventricular compartment was markedly hypertrophied, and right atrial dilation was usually present. The mean pressure gradient measured across the stenotic area was 130 ± 50 mm Hg. Concurrent abnormalities included a ventricular septal defect (n = 2); aortic malalignment, aortic insufficiency (n = 1); and congenital peritoneal-pericardial diaphragmatic hernia (n = 1). Two cats had systolic anterior motion of the mitral valve, one of which had concurrent left ventricular hypertrophy. Five cats have remained asymptomatic for a median period of 3.6 years (range, 3.3,5 years) and 3 cats have developed clinical signs associated with congestive heart failure (at 2, 3.3, and 9 years). One cat showed progressive lethargy and exercise intolerance and underwent partial ventriculectomy at the age of 2 years. This cat died during the operation with electromechanical dissociation. Conclusions: DCRV is a congenital cardiac abnormality that may be more common than previously recognized. [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] 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] Five Fanconi anemia patients with unusual organ pathologiesAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2004Selma Unal Abstract Fanconi anemia (FA) is a rare autosomal recessive disorder that presents with variable organ abnormalities, progressive cytopenia, and susceptibility to the development of several malignancies. Although some of the organ pathologies such as microcephaly, microphthalmia, skin dyspigmentation, urogenital system involvement, and radial ray skeletal abnormalities are relatively common, there are some other abnormalities that are rarely associated with the disease [Alter BP. In: Nathan DG, Oski FA, editors. Hematology of infancy and childhood. Philadelphia: Saunders; 2003. p 259,273]. In this paper, five cases of unrelated FA patients with unusual organ pathologies, including chronic obstructive lung disease, lipodystrophy, Sprengel's deformity, diaphragmatic hernia, and inflammatory linear verrucous epidermal nevus (ILVEN) are presented. Recognition of unusual pathologies associated with FA is important in order to improve our understanding of the relationship between the disease and presenting organ pathologies. Am. J. Hematol. 77:50,54, 2004. © 2004 Wiley-Liss, Inc. [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 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] 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] Chronic diaphragmatic hernia: a delayed problem requiring urgent responseANZ JOURNAL OF SURGERY, Issue 10 2009Nitin Babel MD No abstract is available for this article. [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] Birth at hospitals with co-located paediatric units for infants with correctable birth defectsAUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 3 2008Charles S. ALGERT Objectives: To determine the percentage of liveborn infants with selected antenatally identifiable and correctable birth defects who were delivered at hospitals with co-located paediatric surgical units (co-located hospitals). Additionally, to determine the survival rates for these infants. Patients and methods: Data were from linked New South Wales hospital discharge records from 2001 to 2004. Livebirths with one of the selected defects were included if they underwent an appropriate surgical repair, or died during the first year of life. Infants with multiple lethal birth defects were excluded. Deliveries at co-located hospitals were identified, but no data on antenatal diagnosis were available. Results: The study identified 287 eligible livebirths with the selected defects. The highest rates of delivery at co-located hospitals were for gastroschisis (88%), exomphalos (71%), spina bifida (63%) and diaphragmatic hernia (61%), and the lowest for transposition of the great arteries (43%) and oesophageal atresia (40%). Mothers resident outside of metropolitan areas, where the co-located hospitals are located, had a similar rate of delivery at co-located hospitals as metropolitan women. For the non-metropolitan mothers of infants with a birth defect, this represented a 30-fold increase over the baseline delivery rate of 1.8%. Post-surgery survival rates were 87% or higher. Overall survival rates were , 86% except for infants with a diaphragmatic hernia. Conclusions: Delivery rates at co-located hospitals were high for mothers of infants with these correctable birth defects. Regionalised health care appears to work well for these pregnancies, as women living outside metropolitan areas had a similar rate of delivery at co-located hospitals to that of urban women. [source] | ||||||||||||||||||||||||||||