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

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a significant mortality rate. Despite widespread prenatal diagnosis, few parameters have been well defined to aid in prediction of outcome of these infants. Antenatal maternal steroid administration and foetal surgery are not proven interventions. Postnatal treatment has changed over the last 10 years, with avoidance of hyperventilation and ventilator-induced lung injury resulting in improved survival. Therapies such as inhaled nitric oxide, exogenous surfactant administration and extracorporeal membrane oxygenation (ECMO) have undergone limited study, but show no clear benefit in this population. With improved outcome, principally due to avoidance of barotrauma, greater opportunity exists for long-term evaluation of survivors. To date, continuing problems with pulmonary function, nutrition and growth, effects of right ventricular hypertension and developmental issues have been identified. Through co-ordinated, multidisciplinary evaluation of CDH survivors, improved long-term outcome for these challenging patients can be attained.
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PMID:Current therapy of infants with congenital diaphragmatic hernia. 1500 Nov 40

Persistent pulmonary hypertension of the newborn infant (PPHN), is a clinical syndrome characterized by elevated pulmonary vascular resistance, resulting from reactive vasoconstriction or structural remodeling of the pulmonary vasculature. Although inhaled nitric oxide (iNO) has emerged as a novel selective treatment of PPHN, responses to iNO are variable according to the etiologies or the clinical situation. A retrospective chart review of 51 newborn infants with PPHN and treated with iNO, was undertaken to evaluate the factors affecting response to iNO. Response to iNO was defined as a reduction in the oxygenation index (OI) of more than 20%, or disappearance of the difference in oxygen saturation between preductal and postductal circulation after iNO therapy. The patients were divided into two groups; the responder group and the non- responder group. Respiratory distress syndrome (RDS) was more commonly associated with PPHN in the responder group than in the non-responder group (p < 0.05), while there were many more patients with congenital diaphragmatic hernia (CDH) in the non-responder group than in the responder group (p < 0.05). Infants with meconium aspiration syndrome (MAS) were similar in both of the two groups. Initial OI, initial mean airway pressure (MAP), and initial and peak NO concentration were significantly lower in the responder group compared to the non-responder group (p < 0.05). Rapid response (response to iNO within the first hour) was shown in 74% of the responder group and 33% of the nonresponder group (p < 0.05). There was no significant differences in the initial chest radiographic findings, such as normal, focal or bilateral diffuse infiltration, with the exception of CDH, between each group. Lower initial OI, lower initial MAP and significant response within the first hour were shown to be favourable factors in response to iNO therapy. Patients with RDS associated with PPHN responded much better to iNO than those with other diseases.
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PMID:Factors affecting the response to inhaled nitric oxide therapy in persistent pulmonary hypertension of the newborn infants. 1500 68

There is no consensus on the treatment of congenital diaphragmatic hernia (CDH), and practice seems to vary between centres. The main purpose of the present study was to survey current practice in Scandinavia. Thirteen paediatric surgical centres serving a population of about 22 million were invited, and all participated. One questionnaire was completed at each centre. The questionnaire evaluated management following prenatal diagnosis, intensive care strategies, operative treatment, and long-term follow-up. Survival data (1995-1998) were available from 12 of 13 centres. Following prenatal diagnosis of CDH, vaginal delivery and maternal steroids were used at eight and six centres, respectively. All centres used high-frequency oscillation ventilation (HFOV), nitric oxide (NO), and surfactant comparatively often. Five centres had extracorporeal membrane oxygenation (ECMO) facilities, and four centres transferred ECMO candidates. The majority of centres (7/9) always tried HFOV before ECMO was instituted. Surgery was performed when the neonate was clinically stable (11/13) and when no signs of pulmonary hypertension were detected by echo-Doppler (6/13). The repair was performed by laparotomy at all centres and most commonly with nonabsorbable sutures (8/13). Thoracic drain was used routinely at seven centres. Long-term follow-up at a paediatric surgical centre was uncommon (3/13). Only three centres treated more than five CDH patients per year. Comparing survival in centres treating more than five with those treating five or fewer CDH patients per year, there was a tendency towards better survival in the higher-volume centres (72.4%) than in the centres with lower volume (58.7%), p =0.065.
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PMID:Congenital diaphragmatic hernia: a survey of practice in Scandinavia. 1515 35

A number of new techniques have been studied for managing newborns with congenital diaphragmatic hernia and respiratory insufficiency. Among these have been the techniques of delayed approach to the repair of the diaphragmatic hernia; permissive hypercapnia; nitric oxide and surfactant administration; intratracheal pulmonary ventilation; liquid ventilation; perfluorocarbon-induced lung growth; and lung transplantation. These interventions are at various stages of development and evaluation of effectiveness. All, however, are being explored in the hopes of improving outcome in patients with congenital diaphragmatic hernia who continue to have significant morbidity and mortality in the newborn period.
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PMID:New approaches to managing congenital diaphragmatic hernia. 1528 98

Congenital diaphragmatic hernia is a cardiopulmonary anomaly that causes severe respiratory disorder. Traditionally, inhalational anesthetics with mechanical hyperventilation, opioids, and muscle relaxants are used in anesthesia for repair surgery. In this case, we used total intravenous anesthesia combined with high-frequency oscillatory ventilation and inhaled nitric oxide for surgical repair of the diaphragm. After surgery, the patient recovered well and was discharged from hospital 1 month later.
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PMID:Total intravenous anesthesia for repair of congenitral diaphragmatic hernia: a case report. 1550 61

This article presents a case study of an infant (JG) with an antenatal diagnosis of a left diaphragmatic hernia and an extralobar sequestration of his right lung, which was noted postnatally. JG's course was complicated by persistent pulmonary hypertension of the newborn (PPHN) and suspected pulmonary hypoplasia, and he required support with extracorporeal life support (ECLS). JG's case was unusual in his presentation of extreme PPHN that was unresponsive to inhaled nitric oxide and ECLS. His PPHN was nearly intractable, requiring treatment with vasodilators combined with intravenous sildenafil, which had never been tried in our institution before this case. The article concludes with a discussion of the etiology, diagnosis, and management of congenital diaphragmatic hernia and extralobar sequestration, singly and in combination.
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PMID:Extralobar sequestration with congenital diaphragmatic hernia: a complicated case study. 1561 17

When NO became available for clinical use, it seemed to hold enormous promise for newborn infants with lung disease. With regard to the term and near-term infant, the role of NO seems satisfactorily defined: Near-term and term infants with hypoxic respiratory failure unresponsive to current therapy, excluding infants with diaphragmatic hernia, should have a trial of inhaled nitric oxide. In the preterm infant, the situation is less clear with, as yet, no specific indication identified.
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PMID:Nitric oxide-still no consensus. 1570 9

The high mortality in patients with congenital diaphragmatic hernia (CDH) has been attributed to pulmonary hypoplasia and persistent pulmonary hypertension (PPH). Endothelin-1 (ET-1), nitric oxide (NO), and calcitonin gene-related peptide (CGRP) have been reported to be important vasoactive mediators in the perinatal pulmonary circulation. The exact mechanism by which these vasoactive mediators interact to regulate the perinatal pulmonary vascular tone in CDH with PPH is not fully understood. We hypothesized that the altered pulmonary vascular reactivity in CDH is due to imbalance in vasoactive mediators. This study was designed to investigate mRNA expression of ET-1, eNOS, and CGRP in CDH lung in the perinatal period. A CDH model was induced in pregnant rats following administration of nitrofen. In control animals, the same dose of olive oil was given without nitrofen. Cesarean section was performed on day 21 of gestation. The newborn rats were intubated and ventilated, and ventilation was continued for 1-6 h. Left lungs were collected from both groups at 0, 1, and 6 h after ventilation (n=8 in each group). Reverse transcriptase-polymerase chain reaction on lung tissue was performed to evaluate the relative level of ET-1, eNOS, and CGRP mRNA expression. The results showed a significant increase in ET-1 mRNA in CDH lung at 1 and 6 h after ventilation compared with controls. In CDH lung, eNOS mRNA and CGRP mRNA levels were significantly increased at 1 h but were similar to control values at 6 h after ventilation. The increased expression of vasoconstrictor ET-1 mRNA and vasodilators eNOS mRNA and CGRP mRNA in the CDH lung at 1 h after ventilation suggests that pulmonary vascular tone is rapidly changing after birth. An imbalance in the production of vasoconstrictors and vasodilators by the CDH lung may contribute to high pulmonary vascular resistance.
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PMID:Expression of vasoactive mediators during mechanical ventilation in nitrofen-induced diaphragmatic hernia in rats. 1575 63

The aim of this paper is to review the role of extracorporeal membrane oxygenation (ECMO) in neonates with severe acute hypoxemic respiratory failure secondary to congenital diaphragmatic hernia (CDH). The difficulties in identifying patients with fatal lung hypoplasia are highlighted and the role of adjunctive therapies on ECMO (surfactant, inhaled nitric oxide, high-frequency ventilation and liquid lung distension) as well as the timing of surgical repair is discussed. Survivors of severe CDH who have been supported on ECMO have significant late mortality and morbidity. There remains a need for a randomized controlled trial of the role of ECMO in neonates with severe CDH.
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PMID:The role of extracorporeal membrane oxygenation in congenital diaphragmatic hernia. 1577 May 91

Persistent pulmonary hypertension (PPHN) and subsequent hypoxic respiratory failure is seen in association with numerous diseases and conditions in the neonate. This includes infections such as group B streptococcus, meconium aspiration syndrome, perinatal asphyxia, congenital diaphragmatic hernia, congenital heart disease, and as an idiopathic phenomenon. Conventional therapy of persistent pulmonary hypertension is discussed, as well as integrated with current treatment modalities such as surfactant replacement therapy and high frequency ventilation. The molecular action of nitric oxide including its relationship to neonatal cardiopulmonary transition at birth and the human neonatal clinical experience with term infants from 1992 to the present is explored. Also, the current use of inhaled nitric oxide in preterm infants is reviewed. Additionally, the follow-up of infants treated with inhaled nitric oxide is summarized, and novel therapies including inhaled prostacyclin and other pulmonary vasodilators such as sildenafil are introduced.
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PMID:Inhaled nitric oxide in the treatment of persistent pulmonary hypertension/ hypoxic respiratory failure in neonates: an update. 1585 81


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