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

Despite surgical treatment, congenital foramen of Bochdalek diaphragmatic hernia continues to carry a high mortality rate due to associated respiratory insufficiency. We studied the pathologic and hemodynamic changes that result from this condition in lambs. Surgical creation of diaphragmatic hernias in fetal lambs was performed in utero with subsequent delivery by cesarean section near term. Cardiac catheterization was performed on these newborn lambs immediately after delivery, before and after surgical repair of the defect. Data from five of these lambs were compared to data obtained from seven normal lambs. Pulmonary hypertension was found only in lambs with hernias. Pulmonary artery pressures and the mean ratio of pulmonary to systemic resistance were higher in experimental lambs. Oxygen saturation and average cardiac index were higher in normals. Lambs in the experimental group, but not in the control group, had large left-to-right shunts at the level of the ductus arteriosus in the early neonatal period. Arterial oxygen saturations were consistently low with no differences in pre- and post-ductal samples, which suggests right-to-left shunting at the atrial level or physiologic shunting across the unexpanded lungs. No further expansion of the hypoplastic lungs occurred following decompression by surgical hernia repair, but transient hemodynamic improvement was noted in some cases. Surgical ligation of the ductus arteriosus did not significantly alter the clinical condition of the lambs. Lungs in lambs with hernias were grossly abnormal, the left appearing more hypoplastic than the right.
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PMID:Hemodynamics of congenital diaphragmatic hernia in lambs. 35 42

The authors present four new born with severe anoxemia after congenital diaphragmatic hernia repair. In three babies' hypoxemia was present from birth. Cardiac catheterization revealed pulmonary hypertension, resulting in a state of fetal circulation. Tolazoline produced an improvement in oxygenation, but became secondary ineffective. In one case ductus arteriosus was occluded during cardiac catheterization, after which immediate improvement in peripheral oxygenation was seen. Ligation of the patient ductus arteriosus was proposed in that case. In the fourth infant, hypoxemia developed secondary and was successfully treated with tolazoline. It is suggested that ligation of the patient ductus arteriosus and administration of pulmonary vasodilatators are both effective in improving oxygenation, in patients who may die an anoxic death after repair of a severe congenital diaphragmatic hernia.
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PMID:[Pulmonary hypertension and fetal circulation after severe congenital diaphragmatic hernia (author's transl)]. 43 90

The abnormal pulmonary vasculature of the infant with congenital diaphragmatic hernia combined with a single expanded lung creates persistent pulmonary hypertension. Survival depends on the ability of the myocardium to withstand this increased work load until the second lung expands and pulmonary hypertension ceases.
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PMID:Congenital diaphragmatic hernia: hypoplastic lung and pulmonary vasoconstriction. 65 9

We report our experience from May 1985 to January 1991 with surgical complications and procedures performed in neonates on extracorporeal membrane oxygenation (ECMO) (218 venoarterial and 7 venovenous bypass). Eleven children older than 1 month were excluded. Total complications were 96 in 67 patients and included: bleeding (37), problems with initial cannula placement (17), thrombus formation (15), hemothorax, pneumothorax, or effusions (11), mechanical problems (11), and miscellaneous (5). Forty-eight procedures were performed in 37 patients while on ECMO. These were recannulation or reposition of cannulas (14), tube thoracostomy (11), cardiac surgery (6), cardiac catheterization (4), repair of congenital diaphragmatic hernia (5), thoracotomy (4), and others. Twenty-eight complications occurred in 15 of the 27 patients who died. Mortality rate was 12% for the entire group. Primary causes of death were hypoplastic lung (11), cardiac (8), sepsis (4), intraventricular hemorrhage (2), and pulmonary hypertension (2). No deaths were due solely to complications except for the two patients with intraventricular hemorrhage. Mortality in neonates who had complications while on ECMO was significantly higher (P less than .005) than in patients without complications. Hemorrhagic and thoracic complications were associated with higher mortality (P less than .001). Mortality was not affected by mechanical problems, thrombus formation, or catheter-related problems. While on ECMO cardiac defects, diaphragmatic hernia, lobar emphysema, and other conditions can be safely corrected. The use of echocardiography to position the cannulas, better control of coagulation factors and improvement in equipment may ultimately decrease complications.
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PMID:Surgical complications and procedures in neonates on extracorporeal membrane oxygenation. 140 45

One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [PCO2] < 50; partial pressure of oxygen [PO2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.
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PMID:Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia. 141 95

Extracorporeal membrane oxygenation (ECMO) has been used at the Prince of Wales Children's Hospital, Sydney for the treatment of newborn infants with life-threatening respiratory or cardiac failure since August 1989. The main indications are that the disease is reversible, the surviving infant is likely to be normal and there is an 80% likelihood of death without ECMO. Eighteen of 19 newborn infants have survived at least 2 months after ECMO. The 15 infants receiving ECMO (nine with meconium aspiration, six with persisting pulmonary hypertension) who did not have a congenital diaphragmatic hernia were normal survivors. One death occurred at 5 months of age from chronic lung disease. Three of four infants with congenital diaphragmatic hernia were discharged following ECMO and appeared normal at 6, 9 and 18 months of age. These results are similar to results from other centres internationally. It appears that ECMO is a useful therapy for near-term newborn infants with otherwise fatal cardiorespiratory failure.
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PMID:ECMO in newborn infants: the New South Wales experience. 146 36

Congenital diaphragmatic hernia through the foramen of Bochdalek usually presents with severe respiratory symptoms soon after birth. Despite successful repair of the defect the mortality rate still remains high. This may be due to co-existing pulmonary hypoplasia with pulmonary hypertension and concomitant right-to-left shunting. In order to find factors that predispose for good or bad outcome as well as to compare different modalities of treatment being used at our hospital, this twenty-year retrospective study was undertaken. It is confirmed that the extent of pulmonary hypoplasia at birth rather than the promptness of surgical repair and the skill of intensive care appears to be the major mortality factor. Initial experiences from extracorporeal membrane oxygenation (ECMO) are promising.
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PMID:Diaphragmatic hernia in infancy and childhood--20 years experience. 147 57

Pulmonary hypertension is a major source of morbidity and mortality in infants born with congenital diaphragmatic hernia (CDH). Increased pulmonary vascular resistance leads to right-to-left shunting, which is evident as decreases in the PaO2 measured in postductal arterial blood. Thromboxane A2 (TXA2), a vasoconstrictor, and prostacyclin (prostaglandin I2, PGI2), a vasodilator, have been studied as possible mediators of pulmonary hypertension in certain conditions of the newborn, including congenital diaphragmatic hernia (CDH). The goal of our study was to determine the association of TXA2 and PGI2 levels with hypoxemia in infants born with CDH. Eleven newborn infants with severe respiratory insufficiency (birth weight 2.0-4.1 kg; gestational age 32-42 weeks) were studied 0-5 days after surgical repair of CDH. Umbilical artery samples were collected for arterial blood gas determinations and radioimmunoassay of thromboxane B2 (TXB2) and 6-keto prostaglandin F1 alpha (6-keto-PGF1 alpha), stable metabolites of TXA2 and PGI2, respectively. Postductal arterial hypoxemia (reflected by a low a-A ratio, the ratio of oxygen tension in arterial blood to that in the alveolus) was associated with increases in TXB2 (r = -0.71, P = 0.004) and 6-keto-PGF1 (r = -0.65, P = 0.017). The a-A ratio also correlated inversely with TXB2/6-keto-PGF1 alpha (r = -0.50, P = 0.01), suggesting an increased influence of the vasoconstrictor TXA2.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Relation between arterial hypoxemia and plasma eicosanoids in neonates with congenital diaphragmatic hernia. 149 96

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and pulmonary hypertension. We hypothesize that a surfactant deficiency may in part be contributing to the pathophysiology of CDH. This study documents the functional, quantitative, and qualitative aspects of the surfactant status of the alveolar air-liquid interface and the type II pneumocyte in the fetal lamb CDH model. Ten lamb fetuses (gestational age, 80 days) had a CDH created via a left thoracotomy and then were allowed to continue in utero development until term. Three litter mates and three nonoperated time-dated fetuses served as controls. At term, pressure-volume curves were performed to measure pulmonary compliance and total lung capacity. Alveolar lavage was then performed to measure the quantitative and the qualitative aspects of pulmonary surfactant. Finally, isolation of type II pneumocytes allowed quantification of phospholipid synthesis. When compared with controls (N = 6), the CDH lambs (N = 5) had significantly smaller lungs (P = .009), decreased total lung capacity (P less than .001) and compliance (P less than .001), reduced total lavaged phospholipids (P = .006), and decreased percent phosphatidylcholine (P = .02). CDH lambs also had increased total lavaged proteins (P = .05) and higher minimum dynamic surface tension (P less than .001). A surfactant deficiency may be contributing to the pathophysiology of CDH. Surfactant replacement therapy in premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Exogenous surfactant may also benefit infants with CDH.
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PMID:Pathophysiology of congenital diaphragmatic hernia II: the fetal lamb CDH model is surfactant deficient. 830 93

Neonates with congenital diaphragmatic hernia (CDH) are known to be susceptible to stress-induced persistent pulmonary hypertension (PPHN). Congenital cystic adenomatoid malformations (CCAMs) may also present as respiratory distress in the newborn. Intubation and mechanical ventilation cause clinical deterioration because of air trapping within cystic spaces; these patients require prompt lobectomy. PPHN has not been commonly associated with CCAM. Three patients with CCAM were encountered who developed PPHN postlobectomy. Three newborns, 36 to 38 weeks' gestation, presented with respiratory distress. Two had diagnosis of thoracic tumors on fetal ultrasound (22 and 33 weeks). Chest x-ray at birth confirmed cystic intrathoracic tumors in all and they underwent immediate thoracotomy and lobectomy (1 right upper, 1 left lower, 1 left upper). The patients were stable for 4 hours to 5 days postoperatively and then developed findings consistent with PPHN by cardiac echocardiography and required extracorporeal membrane oxygen (ECMO) support. ECMO was required for 66.5 to 120 hours. Each patient was successfully weaned to conventional ventilatory support. The clinical course of these patients was similar to those with CDH who undergo immediate surgery. The stress of surgical intervention combined with hypoxia and hypercarbia stimulates a hyperactive pulmonary vasculature and the development of PPHN. ECMO provides an effective adjunct to support patients with PPHN on the basis of congenital cystic adenomatoid malformations.
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PMID:Persistent pulmonary hypertension complicating cystic adenomatoid malformation in neonates. 155 45


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