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Surfactant Maturation Is Not Delayed in Human Fetuses with Diaphragmatic Hernia  [PDF]
Olivier Boucherat,Alexandra Benachi,Bernadette Chailley-Heu,Marie-Laure Franco-Montoya,Caroline Elie,Jelena Martinovic,Jacques R Bourbon
PLOS Medicine , 2007, DOI: 10.1371/journal.pmed.0040237
Abstract: Background Pulmonary hypoplasia and persistent pulmonary hypertension account for significant mortality and morbidity in neonates with congenital diaphragmatic hernia (CDH). Global lung immaturity and studies in animal models suggest the presence of surfactant deficiency that may further complicate the pathophysiology of CDH. However, data about surfactant status in human fetuses with CDH at birth are contradictory. The lack of a chronological study of surfactant content in late pregnancy has been a significant limitation. The appropriateness of administering surfactant supplements to neonates with CDH is therefore a debated question. Methods and Findings We investigated surfactant content in human fetuses with CDH compared to age-matched fetuses with nonpulmonary diseases used as controls. Concentrations of disaturated phosphatidylcholine and surfactant proteins were found to be similar at a given stage of pregnancy, with both components showing a similar pattern of increase with progressing pregnancy in fetuses with CDH and in control fetuses. Thyroid transcription factor 1, a critical regulator of surfactant protein transcription, similarly displayed no difference in abundance. Finally, we examined the expression of three glucocorticoid-regulated diffusible mediators involved in lung epithelial maturation, namely: keratinocyte growth factor (KGF), leptin, and neuregulin 1 beta 1 (NRG1-β1). KGF expression decreased slightly with time in control fetuses, but remained unchanged in fetuses with CDH. Leptin and NRG1-β1 similarly increased in late pregnancy in control and CDH lungs. These maturation factors were also determined in the sheep fetus with surgical diaphragmatic hernia, in which surfactant deficiency has been reported previously. In contrast to the findings in humans, surgical diaphragmatic hernia in the sheep fetus was associated with decreased KGF and neuregulin expression. Fetoscopic endoluminal tracheal occlusion performed in the sheep model to correct lung hypoplasia increased leptin expression, partially restored KGF expression, and fully restored neuregulin expression. Conclusions Our results indicate that CDH does not impair surfactant storage in human fetuses. CDH lungs exhibited no trend toward a decrease in contents, or a delay in developmental changes for any of the studied surfactant components and surfactant maturation factors. Surfactant amounts are likely to be appropriate to lung size. These findings therefore do not support the use of surfactant therapy for infants with CDH. Moreover, they raise the question of the relevance
Bilateral Congenital Diaphragmatic Hernia  [cached]
Anjan K Dhua,Satish K Aggarwal,NB Mathur,GR Sethi
APSP Journal of Case Reports , 2012,
Abstract: Bilateral congenital diaphragmatic hernia (CDH) is a rare birth defect, with a poor prognosis. We describe a case of bilateral CDH discovered while repairing the right sided CDH. Diaphragmatic defect was repaired and a silo was applied on the abdominal wound to avoid abdominal compartment syndrome. The patient however died postoperatively due to severe pulmonary hypertension.
CONGENITAL DIAPHRAGMATIC HERNIA
MEHRSIMA ABDOLLAHZADEH,FARNOUSH FARZI,MOHAMMAD HOSSEIN GHORBANI
The Professional Medical Journal , 2010,
Abstract: We report a case of late-presenting congenital diaphragmatic hernia (CDH) in a three-month-old male infant presenting to the pediatric hospital with attacks of cough and cyanosis .Although CDH is usually manifested on the first day of life, there are a number of casereports of late-presenting CDH usually presenting with respiratory or gastrointestinal symptoms. In this case report, we have focused on theanesthetic management of late-presenting CDH. Text: Congenital diaphragmatic hernia is usually manifested on the first days of life as respiratory distress and a scaphoid abdomen. The abnormality is herniation of the abdominal viscera through a defect in the diaphragm, most commonly the foramen of Bochdalek on the left side1. These infants often have a dramatic presentation3, in contrast, late onset or late-presenting CDHs present outside the neonatal period with variable signs and symptoms4. We describe a case of late-presenting CDH that referred to the pediatric hospital with attacks of cough and cyanosis.
Prenatal tracheal ligation or intra-amniotic administration of surfactant or dexamethasone prevents some structural changes in the pulmonary arteries of surgically created diaphragmatic hernia in rabbits
Rodrigues, Consuelo J.;Tannuri, Uenis;Tannuri, Ana Cristina A.;Maksoud-Filho, Jo?o;Rodrigues Junior, Aldo J.;
Revista do Hospital das Clínicas , 2002, DOI: 10.1590/S0041-87812002000100001
Abstract: purpose: characterization of the structural changes occurring in the pulmonary arteries resulting from surgically produced congenital diaphragmatic hernia in rabbits, with particular emphasis on the preventive effects of prenatal tracheal ligation or administration of intra-amniotic dexamethasone or surfactant. methods: twenty rabbit fetuses underwent surgical creation of a left-sided congenital diaphragmatic hernia on the 24th or 25th gestational day. they were divided according to the following procedures: congenital diaphragmatic hernia (n = 5), congenital diaphragmatic hernia plus tracheal ligation (n = 5), congenital diaphragmatic hernia plus intra-amniotic administration of dexamethasone 0.4 mg (n = 5) or surfactant (curosurf 40 mg, n = 5). on gestational day 30, all the fetuses were delivered by caesarean section and killed. a control group consisted of five nonoperated fetuses. histomorphometric analysis of medial thickness, cell nuclei density, and elastic fiber density of pulmonary arterial walls was performed. results: arteries with an external diameter > 100 mm have a decreased medial thickness, lower cell nuclei density, and greater elastic fiber density when compared with arteries with external diameter £ 100 mm. congenital diaphragmatic hernia promoted a significant decrease in medial thickness and an increase in cell nuclei density in artery walls with external diameter > 100 mm. prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes. in arteries with external diameter £ 100 mm, congenital diaphragmatic hernia promoted a significant increase in medial thickness and in cell nuclei density and a decrease in elastic fiber density. the prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes, although no effect was observed in elastic fiber density in the congenital diaphragmatic hernia plus dexamethasone g
Prenatal tracheal ligation or intra-amniotic administration of surfactant or dexamethasone prevents some structural changes in the pulmonary arteries of surgically created diaphragmatic hernia in rabbits  [cached]
Rodrigues Consuelo J.,Tannuri Uenis,Tannuri Ana Cristina A.,Maksoud-Filho Jo?o
Revista do Hospital das Clínicas , 2002,
Abstract: PURPOSE: Characterization of the structural changes occurring in the pulmonary arteries resulting from surgically produced congenital diaphragmatic hernia in rabbits, with particular emphasis on the preventive effects of prenatal tracheal ligation or administration of intra-amniotic dexamethasone or surfactant. METHODS: Twenty rabbit fetuses underwent surgical creation of a left-sided congenital diaphragmatic hernia on the 24th or 25th gestational day. They were divided according to the following procedures: congenital diaphragmatic hernia (n = 5), congenital diaphragmatic hernia plus tracheal ligation (n = 5), congenital diaphragmatic hernia plus intra-amniotic administration of dexamethasone 0.4 mg (n = 5) or surfactant (Curosurf 40 mg, n = 5). On gestational day 30, all the fetuses were delivered by caesarean section and killed. A control group consisted of five nonoperated fetuses. Histomorphometric analysis of medial thickness, cell nuclei density, and elastic fiber density of pulmonary arterial walls was performed. RESULTS: Arteries with an external diameter > 100 mum have a decreased medial thickness, lower cell nuclei density, and greater elastic fiber density when compared with arteries with external diameter <= 100 mum. Congenital diaphragmatic hernia promoted a significant decrease in medial thickness and an increase in cell nuclei density in artery walls with external diameter > 100 mum. Prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes. In arteries with external diameter <= 100 mum, congenital diaphragmatic hernia promoted a significant increase in medial thickness and in cell nuclei density and a decrease in elastic fiber density. The prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes, although no effect was observed in elastic fiber density in the congenital diaphragmatic hernia plus dexamethasone group. CONCLUSIONS: Congenital diaphragmatic hernia promoted different structural changes for large or small arteries. The prenatal intra-amniotic administration of dexamethasone or surfactant had positive effects on the lung structural changes promoted by congenital diaphragmatic hernia, and these effects were comparable to the changes induced by tracheal ligation.
Congenital Diaphragmatic Hernia
Juan A Tovar
Orphanet Journal of Rare Diseases , 2012, DOI: 10.1186/1750-1172-7-1
Abstract: Congenital Diaphragmatic Hernia (CDH), ORPHA2140, OMIM 142340, 610187, 306950 and 222400CDH consists of a posterolateral defect of the diaphragm, generally located on the left side, that allows passage of the abdominal viscera into the thorax. The mediastinum is displaced to the contralateral side, the lungs are hypoplastic (Figure 1) and their arterioles are abnormal causing pulmonary hypertension. Respiratory and cardiovascular functions are severely compromised at birth and this, together with the frequently associated malformations, cause considerable mortality and morbidity. CDH was described many years ago [1,2] but survival after repair was not achieved until the 20th century. Pioneers of pediatric surgery [3] reported amazingly low mortalities until the actual severity of the condition surfaced when abortions, stillbirths and pre-hospital deaths were considered, adding a "hidden mortality" to operative and postoperative demises [4]. The pathophysiology of lung insufficiency and persistent pulmonary hypertension that threaten survival are currently better understood, but the results remain disappointing since mortalities near 50% are still reported when all deaths are taken into account in population-based series [5]. CDH management indeed remains one of the major challenges of perinatal medicine and surgery and active research on its mechanisms is warranted.CDH is a rare condition that occurs in < 1-5:10000 births [6]. It seems to be slightly more frequent in males and less frequent in blacks [7,8].CDH can be detected during fetal life when screening ultrasonography demonstrates herniation of the intestine and/or the liver into the thorax. Polyhydramnios may lead to antenatal diagnosis in some severe cases [9].Neonatal symptoms of CDH are heralded by respiratory distress with insufficient oxygenation, excavated abdomen with sternal protrusion and displacement of the heart sounds to the contralateral side. In severe cases, APGAR scores at 1 and 5 minutes are
Heart hypoplasia in an animal model of congenital diaphragmatic hernia  [cached]
Tannuri Uenis
Revista do Hospital das Clínicas , 2001,
Abstract: PURPOSE: In previous papers, we described a new experimental model of congenital diaphragmatic hernia in rabbits, and we also reported noninvasive therapeutic strategies for prevention of the functional and structural immaturity of the lungs associated with this defect. In addition to lung hypoplasia, pulmonary hypertension, biochemical, and structural immaturity of the lungs, the hemodynamics of infants and animals with congenital diaphragmatic hernia are markedly altered. Hence, cardiac hypoplasia has been implicated as a possible cause of death in patients with congenital diaphragmatic hernia, and it is hypothesized to be a probable consequence of fetal mediastinal compression by the herniated viscera. Cardiac hypoplasia has also been reported in lamb and rat models of congenital diaphragmatic hernia. The purpose of the present experiment was to verify the occurrence of heart hypoplasia in our new model of surgically produced congenital diaphragmatic hernia in fetal rabbits. METHODS: Twelve pregnant New Zealand rabbits underwent surgery on gestational day 24 or 25 (normal full gestational time - 31 to 32 days) to create left-sided diaphragmatic hernias in 1 or 2 fetuses per each doe. On gestational day 30, all does again underwent surgery, and the delivered fetuses were weighed and divided into 2 groups: control (non-surgically treated fetuses) (n = 12) and congenital diaphragmatic hernia (n = 9). The hearts were collected, weighed, and submitted for histologic and histomorphometric studies. RESULTS: During necropsy, it was noted that in all congenital diaphragmatic hernia fetuses, the left lobe of the liver herniated throughout the surgically created defect and occupied the left side of the thorax, with the deviation of the heart to the right side, compressing the left lung; consequently, this lung was smaller than the right one. The body weights of the animals were not altered by congenital diaphragmatic hernia, but heart weights were decreased in comparison to control fetuses. The histomorphometric analysis demonstrated that congenital diaphragmatic hernia promoted a significant decrease in the ventricular wall thickness and an increase in the interventricular septum thickness. CONCLUSION: Heart hypoplasia occurs in a rabbit experimental model of congenital diaphragmatic hernia. This model may be utilized for investigations in therapeutic strategies that aim towards the prevention or the treatment of heart hypoplasia caused by congenital diaphragmatic hernia.
Congenital diaphragmatic hernia: Misdiagnosis in adolescence  [cached]
Kadian Yogender,Rattan Kamal,Verma Manish,Kajal Pradep
Journal of Indian Association of Pediatric Surgeons , 2009,
Abstract: We report 3 cases of congenital diaphragmatic hernia (CDH) in the second decade of life which were misdiagnosed on initial presentation. The first case had an iatrogenic gastric injury because of intercostal tube drainage for suspected pleural effusion. The second case was treated for pulmonary tuberculosis for 6 months before being diagnosed as a case of CDH. The third case presented as acute chest pain on the left side. It was treated accordingly for 1 month and was diagnosed as a CDH on a CT scan of the chest when seen by a surgeon.
Congenital Bochdalek’s Diaphragmatic Hernia  [PDF]
Baba Usman Ahmadu,Chinda John Yola,Kaleb Abalis Abew
Journal of Nepal Paediatric Society , 2012, DOI: 10.3126/jnps.v32i3.6968
Abstract: Diagnosis of a rare Bochdalek’s congenital diaphragmatic hernia may be challenging. Our patient presented with respiratory and gastrointestinal findings. Chest radiograph revealed the stomach in the left thorax. Diagnosis of recurrent bacterial pneumonia was incorrectly made because of repeated symptoms. Chest radiograph can provide sufficient information for rapid diagnosis. DOI: http://dx.doi.org/10.3126/jnps.v32i3.6968 J. Nepal Paediatr. SocVol.32(3) 2012 254-256
A Rare Association of Congenital Diaphragmatic Hernia with Lower Esophageal Atresia and Perforation  [PDF]
Narendra Kumar Are,K. Nagarjuna,Lavanya Kannaiyan
International Journal of Pediatrics , 2010, DOI: 10.1155/2010/738546
Abstract: Congenital diaphragmatic hernia is known to be associated with esophageal atresia, which is a rare association. We report a rare occurrence of congenital diaphragmatic hernia and lower esophageal atresia.
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