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Query: UMLS:C0019270 (hernia)
 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe an infant with severe eventration of the right diaphragm and pulmonary hypoplasia who presented like a newborn with congenital diaphragmatic hernia complicated by persistent pulmonary hypertension. Surgical correction while on extracorporeal life support was unsuccessful due to attachments of the liver which prevented reduction into the abdominal cavity and our inability to distinguish the true defect from complete agencies of the right hemidiaphragm. At autopsy the pulmonary remnant and the fibrous membrane separating it from the liver were identified.
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PMID:Right diaphragmatic eventration simulating a congenital diaphragmatic hernia. 872 27

Extracorporeal membrane oxygenation (ECMO) has been successful in rescuing near term or term infants in cardio-respiratory failure that results from a reversible disease process. In most cases, only one course of ECMO is needed to save these infants. However, a second course of ECMO may be beneficial in a select group of infants when recurrent persistent pulmonary hypertension develops. Other than abstract form, this is the first report of the use of a second course of ECMO in the literature. The authors report on three infants, two with recurrent persistent pulmonary hypertension secondary to congenital diaphragmatic hernia and one with necrotizing tracheobronchitis after Group B streptococcal sepsis who were treated at their institution with a second course of ECMO. Technical considerations in using a second course of ECMO depend upon the initial vessel cannulation site, time elapsed between cannulations, and the condition of the original artery and vein. By adopting a stenting procedure in those infants whose initial trial off was equivocal, a second cannulation may be prevented in neonatal patients with recurrent persistent pulmonary hypertension.
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PMID:The use of a second course of extracorporeal membrane oxygenation in neonatal patients. 872 96

Pulmonary hypoplasia(PH) commonly occurs in association with oligohydramnios and other congenital anomalies, especially congenital diaphragmatic hernia (CDH). Pulmonary hypoplasia is an important factor, as persistent pulmonary hypertension, in the prognosis of CDH. In some reports, there is a decrement of pulmonary surfactant in PH accompanying CDH. Recently, there are some reports that exogenous pulmonary surfactant therapy is effective in experimental animal model and neonatal respiratory distress with PH. We report a case of a 5 day-old male neonate, who had shown dyspnea and diagnosed as left pulmonary hypoplasia accompanying CDH. The CDH was surgically treated and the ipsilateral PH, with intratracheal administration of exogenous pulmonary surfactant postoperatively. After exogenous pulmonary surfactant application, the left lung volume was increased on chest roentgenogram and lung perfusion scan findings, and there was an improvement in oxygenation and clinical manifestations. We suggest that postoperative exogenous pulmonary surfactant replacement therapy is effective in the case of PH and further trials are needed to clarify the optimal dose and timing of supplementation of surfactant for treatment of infants with PH accompanying CDH.
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PMID:Exogenous pulmonary surfactant replacement therapy in a neonate with pulmonary hypoplasia accompanying congenital diaphragmatic hernia--a case report. 884 10

Because nitric oxide (NO) dilates vascular smooth muscle cells, a deficiency of endogenous pulmonary nitric oxide production by nitric oxide synthase (NOS) has been suggested to be involved in the pathophysiology of pulmonary hypertension in congenital diaphragmatic hernia (CDH). Our aim was to determine whether experimentally induced CDH in rats results in a decrease in the synthesis of NO in the lungs. Adult Sprague-Dawley rats were fed 300 mg/kg of nitrofen at 10.5 days' gestation. CDH, control, and sham (dosed with nitrofen, but without CDH) lungs were homogenized at full term (22 days' gestation) for measurement of NOS activity using the 14C-L-arginine to 14C-L-citrulline conversion assay. Western blot analysis with anti-endothelial cell NOS (EC-NOS) monoclonal antibody (mAb) was performed, and NOS expression was measured by densitometry. NOS activity was highest in the pulmonary parenchyma of control rat lungs (0.420 +/- 0.20 fmol/min/mg lung; n = 11), intermediate in sham lungs (0.370 +/- 0.010 fmol/min/mg lung; n = 14), and lowest in CDH lungs (0.300 +/- 0.04 fmol/min/mg lung; n = 12). NOS activity in the CDH and sham lungs was significantly lower than that of control lungs (P < .05). There was no difference in pulmonary NOS activity between sham and CDH lungs. NOS protein expression by Western blot analysis paralleled the observation for NOS activity in all groups, with the highest concentrations in controls, intermediate expression in sham lungs, and lowest expression in CDH lungs. Both NOS expression and NOS activity are significantly decreased in CDH rat lungs. Pulmonary hypertension in this model may be attributable to a deficiency of endogenous NO. This is the first reported study to suggest that decreased NOS activity may result in pulmonary hypertension in CDH.
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PMID:Decreased pulmonary nitric oxide synthase activity in the rat model of congenital diaphragmatic hernia. 886 23

The patient born with congenital diaphragmatic hernia (CDH) presents with a multitude of problems. The pulmonary hypoplasia associated with CDH results in an inadequate surface area for gas exchange, decreased cross sectional area of the pulmonary vascular tree, precocious muscularization of the pulmonary vessels, and probable surfactant deficiency. Elevated levels of circulating vasoactive mediators, as well as a heightened sensitivity to these mediators, further compounds the pulmonary hypertension associated with CDH. We have reviewed the pathophysiology of CDH, with specific reference to how this knowledge has affected clinical management.
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PMID:Pathophysiology of congenital diaphragmatic hernia. 893 52

Congenital diaphragmatic hernia (CDH) with severe respiratory failure is still associated with significant mortality. Modern treatment of CDH is now widely accepted to be delayed repair after stabilization. Availability of Extracorporeal Membrane Oxygenation (ECMO) led up to real improvement in survival. Several others modalities have been recently used in attempting to reduce the need for ECMO or, otherwise, to improve outcome. Multicenter controlled trial of high-frequency oscillatory ventilation (HFOV), exogenous surfactant replacement, nitric oxide (NO) inhalation and, more recently, liquid ventilation have been reported. We describe four cases of CDH treated in our ECMO-centre from 1993 to date, 25% surviving. One patient died by pulmonary hypertension and multiorgan failure while on ECMO; one by pulmonary hypertension and cardiac failure and one by sepsis, both ones far from effective ECMO weaning. All patients underwent extracorporeal bypass because of Oxygenation Index (OI) ranging 65-215. Venovenous has been always made but one patient needed early switching on venoarterial. Several trials with surfactant and nitric oxide were performed during extracorporeal bypass. In survived patient, diaphragmatic defect was repaired out of ECMO. Patients survived to the weaning underwent vascular reconstruction. Our ECMO data confirm worse prognosis for CDH rather than other ECMO requiring diseases (we report 66.7% surviving in overall ECMO application); we underline real improvement by using alternative therapies together with extracorporeal bypass and primary role of OI as predicting index for ECMO.
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PMID:[Congenital diaphragmatic hernia: the use of ECMO and other modern therapeutic strategies]. 896 31

Persistent pulmonary hypertension poses a significant problem to the surgeon managing an infant with congenital diaphragmatic hernia. It is likely that a number of abnormalities contribute to this pathophysiologic entity. These include: (1) in the hypoplastic lung the overall cross-sectional area of the pulmonary vascular bed is reduced, (2) the muscular arteries are hypertrophied and extend more peripherally than normal, (3) the pulmonary vessels are more labile than normal and are overly sensitive to the normal stimuli of vasoconstriction, and (4) the immature surfactant-deficient lung is predisposed to barotrauma and atelectasis, resulting in alveolar hypoxemia which contributes to pulmonary hypertension. All of these interfere with the ability of the lung to accept the increase in pulmonary blood flow required by the transitional circulation. If this impairment reaches a level such that the lung cannot accept the right ventricular output then pulmonary hypertension will ensue and a poor outcome can be anticipated.
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PMID:Pulmonary vascular abnormalities in congenital diaphragmatic hernia. 898 71

Neonates with congenital diaphragmatic hernia (CDH) often suffer from respiratory insufficiency due to lung hypoplasia and pulmonary hypertension. Artificial ventilation is frequently required, and this leads to a high incidence of bronchopulmonary dysplasia. Long-term follow-up studies have shown persisting airway obstruction. To evaluate the long-term pulmonary sequelae in CDH, we studied 40 CDH patients of age 7 to 18 yr (median 11.7 yr) and 65 age-matched controls without CDH and lung hypoplasia who underwent similar neonatal treatment. Mild airway obstruction was found in both groups with more peripheral airway obstruction in CDH patients than in control subjects. Both groups had normal TLC and single-breath carbon monoxide diffusion capacity (DLCO). CDH patients had increased residual volume (RV) and RV/TLC compared with controls. Increased airway responsiveness to methacholine (MCH) was common but bronchoconstriction to inhaled metabisulfite (MBS) was rare both in CDH and control subjects. We conclude that this group of CDH patients has minor residual lung function impairment. Mild airway obstruction and increased airway responsiveness to inhaled MCH but not to MBS suggest that structural changes in distal airways are involved and not autonomic nerve dysfunction. Both artificial ventilation in the neonatal period and residual lung hypoplasia seem important determinants of persistent lung function abnormalities in CDH patients.
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PMID:Long-term pulmonary sequelae in children with congenital diaphragmatic hernia. 900 8

The first description diaphragmatic hernia appeared in 1575. In 1848, Bochdalek described congenital diaphragmatic hernia (CDH) occurring through a posterolateral defect. Successful surgical treatment of CDH in an infant was first performed in 1902, whereas the first neonate operated within 24 hours of life was reported in 1946. However, early surgery did not improve survival rates and the mortality was in the region of 50%. One reason for this was that more neonates underwent surgery who previously would have died without the repair of the CDH. Pulmonary hypoplasia and pulmonary hypertension were early recognised as important reasons for the high mortality rate. In recent years, an enormous effort has been made by research groups all over the world to describe the pathogenesis and pathophysiology of CDH, and apply these findings to clinical practice. Attempts have been made to define prognostic factors. Extracorporeal membrane oxygenation (ECMO) has produced encouraging results. Fetal surgical therapy remains an option in selected cases despite huge technical and ethical problems. Recently, several new therapeutic methods have been suggested, such as high frequency oscillatory ventilation, partial liquid ventilation, nitric oxide inhalation, surfactant therapy, and fetal tracheal ligation. However, more experience is required before the value of these approaches is clear. Despite these efforts, the mortality remains unacceptably high. The challenge for the future is to continue development of therapeutic approaches in order to improve survival of neonates with CDH.
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PMID:Historical aspects of congenital diaphragmatic hernia 906 5

Primary pulmonary hypertension is characterized by the presence of smooth muscle cells in nonmuscular compartments or segments of the vessel and the abnormal deposition of collagen in both the small muscular arteries and arterioles and the large elastic arteries. Victorian blue van Gieson staining and Immunostaining with anti-alpha smooth muscle actin (ASMA) were performed on lung tissues obtained during autopsy from 21 patients who had congenital diaphragmatic hernia (CDH) complicated by persistent pulmonary hypertension (PPH) and 10 control patients who died of sudden infant death syndrome (SIDS). The degree of medial thickening and adventitial thickening was measured in pulmonary arteries by image analysis and compared statistically. There was a significant increase in adventitial as well as medial thickness in arteries of all sizes in CDH patients compared with control patients (P < .001). The most striking increase occurred in arteries with an external diameter (ED) of less than 75 microns. Calculation of the areas of the various components in the wall of each vessel showed that for smaller vessels (< 75 microns ED), the area of the lumen was smaller and the area of the media and adventitia was larger in CDH patients compared with control patients (P < < .001). In vessels greater than 75 microns ED, the areas of media in CDH was the same as in controls and the area of adventitia in CDH was significantly larger than controls (P < .001). The present study provides evidence that an increase in adventitial thickness and adventitial area occurs in pulmonary arteries in CDH patients complicated by PPH. The structural changes in the adventitia of the pulmonary arteries may be an important factor in the development of PPH in patients with CDH.
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PMID:Pulmonary artery structural changes in pulmonary hypertension complicating congenital diaphragmatic hernia. 909 99


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