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

Acute gastric volvulus occurred in nine infants and one older child during the past 19 years; all patients had an associated left diaphragmatic anomaly. There were seven examples of eventration of the diaphragm, two of giant hiatal hernia and one Bochdalek hernia. Nine of the ten patients presented with vomiting and one with acute respiratory distress. The gastric volvulus was mesenteroaxial in eight patients and organoaxial in two. Operative treatment consisted of repair of the diaphragmatic anomaly and gastric fixation in eight patients. Gastric fixation alone was performed in one patient. A single patient died preoperatively and had gastric necrosis at postmortem examination. Of the nine patients treated operatively, all remain alive and well without recurrence. Acute gastric volvulus should be considered in any infant presenting with unexplained vomiting in association with a left diaphragm anomaly, and once diagnosed, immediate operation is mandatory.
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PMID:The pediatric diaphragm in acute gastric volvulus. 814 14

The lungs of patients born with severe congenital diaphragmatic hernia (CDH) are biochemically and morphologically immature. Because antenatal glucocorticoid therapy can accelerate pulmonary maturation in premature neonates who have respiratory distress syndrome, we hypothesized that it may correct the pulmonary biochemical and morphological immaturity associated with CDH. We showed in previous experimental studies that antenatal low-dose dexamethasone improved the biochemical and morphological parameters of pulmonary immaturity in rats that had severe CDH. Somatic and pulmonary growth were inhibited with high doses of dexamethasone. In the present study, we examined the effects of antenatal low-dose dexamethasone and thyrotropin-releasing hormone (TRH), alone or in combination, on the pulmonary maturation in CDH. Combined antenatal low-dose dexamethasone and TRH significantly reduced mean lung glycogen concentration (P = .001), and increased mean disaturated phosphatidylcholine content (P < .005) to better than that observed with either therapy alone, without changing mean body or lung weight. Combined TRH and low-dose glucocorticoid as an antenatal therapy may reduce the morbidity and mortality of CDH.
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PMID:Combined antenatal thyrotropin-releasing hormone and low-dose glucocorticoid therapy improves the pulmonary biochemical immaturity in congenital diaphragmatic hernia. 817 20

Three preterm infants are described who had respiratory failure immediately after birth. For each, historical factors (extrauterine abdominal pregnancy; ruptured membranes with oligohydramnios for 14 weeks; congenital diaphragmatic hernia) and the initial postnatal course were suggestive of pulmonary hypoplasia. However, with vigorous resuscitation each infant showed rapid improvement in pulmonary status for several hours. Two infants survived with no apparent pulmonary sequelae; the third had almost complete resolution of pulmonary disease but died from gastrointestinal complications. Thus some infants with historical and clinical indicators of severe pulmonary hypoplasia do not have hypoplastic lungs and respond favorably to resuscitation. The causes of the respiratory distress and of its rapid resolution are not known. On the basis of this experience, even infants with historical factors suggesting pulmonary hypoplasia and with postnatal respiratory distress should receive vigorous resuscitation.
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PMID:Transient severe respiratory distress mimicking pulmonary hypoplasia in preterm infants. 822 32

In a retrospective review we analysed alveolar-arterial oxygen difference (AaDO2) as an entry criterion for extracorporeal membrane oxygenation (ECMO) in neonates with several forms of acute respiratory insufficiency. Although for meconium aspiration syndrome, respiratory distress syndrome, sepsis, and idiopathic pulmonary hypertension of the newborn we found values in accordance with the literature, patients with congenital diaphragmatic hernia (CDH) met 80% mortality criteria with significant lower AaDO2 values. Several patients died before ever reaching usual entry criteria for ECMO, because serious lung deterioration makes AaDO2 values unreliable. Awaiting classical ECMO entry criteria for patients with CDH may at least partially explain the lower survival rate for ECMO in CDH.
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PMID:Do we use the right entry criteria for extracorporeal membrane oxygenation in congenital diaphragmatic hernia? 822 83

During a period of 5 years, 33 newborns with congenital diaphragmatic hernia were treated. Three groups presenting with respiratory distress in the delivery room were identified. These included 8 newborns with agenesis (group 1) and 4 newborns with nonagenesis (group 2), all of whom died. There were 19 nonagenesis survivors (group 3), giving an overall survival rate of 61%. Two newborns who presented beyond 6 hours of life were excluded. No one specific arterial blood gas value or ventilation parameter obtained preoperatively could predict survival. Postmortem right and left lung weights, lung/body weight ratio, and radial alveolar counts demonstrate that agenesis is a unique subgroup with profound pulmonary hypoplasia and a dismal prognosis.
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PMID:An association of pulmonary hypoplasia with unilateral agenesis of the diaphragm. 822 7

Bochdalek hernia is a posterolateral defect in the embryogenesis of the diaphragm. Abdominal contents enter the thorax during fetal development and result in pulmonary hypoplasia. A cause of respiratory distress in the newborn, the management of this disease has undergone dramatic changes. The defect requires surgical repair, but success depends more on preoperative and postoperative management of the associated physiologic derangements.
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PMID:Newborn surgical emergencies. Congenital diaphragmatic hernia and extracorporeal membrane oxygenation. 825 27

Pulmonary sequestration is a complex anomaly involving the pulmonary parenchyma and its vascularity. From 1975 to 1992, 10 cases have been treated at the UCLA Medical Center. The ages of the seven females and three males were bimodal, the median age of the seven children was 29 days (range 1 day-6 years); it was 32 years (range 28-39) for the three adults. One child was delivered by cesarean section for fetal distress and another was born at 29 weeks gestation. Symptoms included: recurrent pneumonia (5), respiratory distress (5), hemoptysis (2), stridor (1), and pleuritis (1). Chest radiographs were consistent with sequestration in seven patients and diaphragmatic hernia in another, but incorrectly diagnosed one diaphragmatic eventration and one pulmonary varix. Computed tomographs suggested sequestration in five patients, but mistakenly interpreted a pulmonary varix in one case. Angiography, ultrasonography, and magnetic resonance imaging were infrequent investigative studies and yielded variable results. All patients underwent thoracotomy and lobectomy without morbidity or mortality. Five had intralobar and five had extralobar sequestration. All adults had intralobar sequestration. Two had aberrant subdiaphragmatic arterial vessels. Eight had vessels originating from the descending thoracic aorta. Nine patients are asymptomatic at six months to 10 years follow up. The 29-week-old premature infant died 30 days postoperatively due to necrotizing enterocolitis. Pulmonary sequestration remains an uncommon entity. The radiologic investigations that provide the most information are the chest radiography and computed tomography. Definitive diagnosis is made at thoracotomy. Pulmonary sequestrations are resected with excellent results by the trained thoracic surgeon who is aware of the unusual vascular connections.
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PMID:Pulmonary sequestration: 17-year experience at UCLA. 825 32

The main symptom of the congenital diaphragmatic hernia (CDH) is the respiratory distress due to the pulmonary hypoplasia and the persistence of foetal pulmonary circulation. Sometimes the CDH appears outside of the neonatal period with respiratory symptoms, abdominal pain and seldom with intestinal obstruction. A case of CDH presenting with intrathoracic volvulus is reported. This case shows that tendency of delayed repair until the newborn has been recovered, require more attention because the good condition of the patient can be changed not only by the pulmonary hypoplasia and the persistence of the foetal pulmonary circulation but also by the symptoms of the intrathoracic gut complications.
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PMID:Congenital diaphragmatic hernia and intrathoracic intestinal volvulus. 829 83

To study the potential role of endothelin-1, a potent endothelium-derived vasoconstrictor peptide, in the pathophysiology of persistent pulmonary hypertension of the newborn (PPHN), we measured arterial concentrations of immunoreactive endothelin-1 (irET-1) in 24 neonates with PPHN. Secondary diagnoses included meconium aspiration syndrome (13 patients), sepsis (2), congenital diaphragmatic hernia (1), asphyxia (1), pulmonary hemorrhage (1), aspiration of blood (1), and respiratory distress syndrome (1). Compared with irET-1 levels in umbilical cord blood in normal infants (15.1 +/- 4.1 pg/ml; mean +/- SEM) and in newborn infants with hyaline membrane disease who were supported by mechanical ventilation (11.8 +/- 1.2 pg/ml), infants with PPHN had markedly elevated circulating irET-1 levels (27.6 +/- 3.6 pg/ml; p < 0.01 vs cord blood, hyaline membrane disease). Infants with severe PPHN requiring extracorporeal membrane oxygenation (ECMO) therapy had higher irET-1 levels than infants with milder disease (31.0 +/- 4.7 for ECMO-treated infants vs 21.2 +/- 2.0 for non-ECMO-treated infants; p < 0.05). In patients treated without ECMO, irET-1 progressively decreased during the following 3 to 5 days, paralleling clinical improvement. In contrast, irET-1 concentrations remained elevated in infants with severe PPHN during ECMO therapy. We conclude that circulating irET-1 levels are elevated in newborn infants with PPHN, are positively correlated with disease severity, and decline with resolution of disease in patients who do not require ECMO therapy. Whether endothelin-1 contributes directly to the pathophysiology of PPHN or is simply a marker of disease activity remains speculative.
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PMID:Elevated immunoreactive endothelin-1 levels in newborn infants with persistent pulmonary hypertension. 815 68

Three unusual cases of large anterolateral congenital diaphragmatic hernia are described with emphasis on its embryogenesis. This type of diaphragmatic defect is rare and underemphasized. Its clinical spectrum ranges from the asymptomatic case to those with acute respiratory distress. It may present itself as a solitary defect or as part of a more complex association such as the thoracoabdominal syndrome. This defect should be differentiated from Morgagni (and other anterior) diaphragmatic hernias by its size and lateral extension and the presence of left pulmonary hypoplasia. Treatment is by primary operative repair, and prognosis depends on the presence and severity of associated malformations.
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PMID:Anterolateral diaphragmatic hernia: is it a Morgagni hernia? 832 17


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