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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) and its attendant lack of abdominal domain can create major technical challenges with respect to diaphragmatic and abdominal wall reconstruction, especially in seriously ill infants who require extracorporeal membrane oxygenation (ECMO). The authors reviewed the medical records of all infants with CDH repaired on ECMO at their institution (group 1, 15 patients), and compared them with infants having CDH repair before ECMO (group 2, 20 patients) and with those who had CDH repair but did not require ECMO (group 3, 15 patients). Thirty-seven of 50 patients survived (74%): 10 in group 1, 12 in group 2, and all 15 in group 3. There was a statistically significant difference (P < .001) with respect to the requirement of a polytetrafluoroethylene (PTFE) diaphragmatic patch for patients in group 1 versus those in both groups 2 and 3. There was also a significant difference in the number of patients in whom the abdomen could not be closed (P < .001 for group 1 v groups 2 and 3). Infants who require ECMO before CDH repair are more likely to have large diaphragmatic defects that require prosthetic reconstruction, and abdominal wall closure problems resulting from loss of abdominal domain, which further complicate the management of the physiological derangements from pulmonary hypoplasia and persistent pulmonary hypertension.
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PMID:Experience with abdominal wall closure for patients with congenital diaphragmatic hernia repaired on ECMO. 772 21

Lung hypoplasia (LH) and pulmonary hypertension are responsible for the high mortality rate in congenital diaphragmatic hernia (CDH) patients. Angiotensin-converting enzyme (ACE) plays a role in the regulation of pulmonary vascular resistance in the postnatal period and might be involved in the development of pulmonary hypertension of the newborn. A study was made of the development of ACE activity spectrophotometrically in a rat model of LH and CDH. It was previously shown that the lungs in this model are hypoplastic and the muscularization of the pulmonary vascular bed is increased. CDH was induced in fetal rats by oral administration of 115 mg/kg Nitrofen to the mother on day 10.5 of pregnancy. Fetuses were delivered by hysterotomy on days 19, 20, 21, and 22. Nitrofen-exposed rats showed significantly lower lung weights and not statistically significant lower total ACE activities than in controls. ACE activity expressed per milligram lung wet weight and per milligram protein was significantly increased compared to controls. ACE converts angiotensin I to the vasoconstrictor angiotensin II, and it inactivates the vasodilator bradykinin. Increased ACE activity may therefore contribute to pulmonary hypertension. Whether ACE and angiotensin II levels are increased in human newborns with a diaphragmatic defect and whether they contribute to the development of persistent pulmonary hypertension has not been studied up till now.
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PMID:Angiotensin-converting enzyme activity is increased in lungs of rats with pulmonary hypoplasia and congenital diaphragmatic hernia. 772 78

Stabilization and delayed operation for patients with congenital diaphragmatic hernia (CDH) is now widely accepted. When preoperative extracorporeal membrane oxygenation (ECMO) is needed, most centers have CDH repaired on ECMO to minimize the risk of postoperative deterioration. The authors adopted a policy of weaning from ECMO before repair in an effort to avoid hemorrhagic risks. They reviewed their experience with CDH patients who required ECMO for stabilization before repair but for whom post-ECMO repair was planned. The records of all high-risk CDH patients with a gestational age of at least 34 weeks were reviewed. Eighteen patients were identified. None of the eight who were stabilized and operated on without ECMO required bypass postoperatively; all survived. Ten were placed on bypass, nine for stabilization before repair. Of the nine, seven (78%) were weaned from ECMO to conventional ventilation. Repair of the diaphragmatic defect was performed an average of 3.8 days later; none of these patients had severe pulmonary hypertension postoperatively, and all survived. Two could not be weaned before repair, one of whom had a complex congenital heart defect. This patient died. The other patient had repair on ECMO because of intrathoracic gastric volvulus. Severe blood loss prompted decannulation, and the patient died. One patient who was placed on bypass was transferred 10 days after having had repair elsewhere (at 4 hours of age). Pulmonary hypertension did not resolve, and the postmortem examination showed alveolar capillary dysplasia, with focal misalignment of the pulmonary vessels.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Repair of congenital diaphragmatic hernia after weaning from extracorporeal membrane oxygenation. 773 64

Lung hypoplasia and persistent pulmonary hypertension are the principal causes of high mortality and morbidity in infants with congenital diaphragmatic hernia (CDH). Amine- and peptide-producing pulmonary neuroendocrine cells (PNEC), widely distributed throughout the airway mucosa, are thought to play an important role in both pulmonary development and regulation of pulmonary vascular tone. Furthermore, recent studies show increased levels of calcitonin gene-related peptide (CGRP), a pulmonary vasodilator produced by PNEC, during chronic hypoxia. The article reports data on morphometric analysis of CGRP immunoreactive PNEC clusters (neuroepithelial bodies, NEB) in a rat model of CDH. CDH was induced in neonatal Sprague Dawley rats by oral administration of 2,4-dichloro-phenyl-p-nitrophenylether (Nitrofen; Rohm Haas, Philadelphia, PA) to the mother at 10 days of gestation. Sections of lungs from term neonatal rats with and without CDH and controls were immunostained for CGRP (marker of NEB) with specific antibody against rat CGRP. NEB size and number of NEB/area of lung were assessed using a semiautomatic image analysis system. In lungs of neonatal rats with CDH, the number of NEB per surface area of lung parenchyma was significantly increased compared with the age-matched controls. Although the mean size of NEB was larger in CDH, the differences were not significant. This is the first study of PNEC in CDH. Whether the phenomenon observed in this study results in altered NEB function including imbalance in vasoactive mediators requires further studies, especially in the human being.
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PMID:Pulmonary neuroendocrine cells in neonatal rats with congenital diaphragmatic hernia. 776 Feb 32

Persistent pulmonary hypertension (PPH) together with pulmonary hypoplasia accounts for high mortality among patients with congenital diaphragmatic hernia (CDH). Endothelin (ET) is a recently described endothelium-derived vasoconstrictor peptide with a potent and sustained effect. The authors examined plasma ET levels in eight patients with CDH who had respiratory distress at birth and in 15 age-matched controls. The plasma ET levels were measured using a highly sensitive and specific enzyme immunometeric assay (EIA) preoperatively, intraoperatively, and 24 and 48 hours postoperatively. The mean preoperative plasma ET level (53.9 +/- 21.1 pg/mL) of the patients was significantly (P < .01) higher than that of controls (5.9 +/- 4.5 pg/mL). Two patients who died before operation had plasma ET levels of 60 pg/mL and 82 pg/mL, respectively. Three patients who had PPH postoperatively continued to have high ET levels, and at 48 hours, the mean plasma ET level in these patients was 33 pg/mL. In contrast, in the three patients who survived after repair of CDH, the mean plasma ET level at 48 hours was 1.3 pg/mL. The authors further examined the lungs of two patients who died before operation for the expression of ET, using immunocytochemistry. Pulmonary endothelial cells had strong ET staining compared with control tissues. These findings suggest that ET may be a pathophysiological mediator of pulmonary hypertension in patients with CDH.
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PMID:Plasma endothelin levels in congenital diaphragmatic hernia. 780 62

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment for neonates who have severe respiratory failure that does not respond to maximal conventional therapy. A consequence of venoarterial ECMO is the sacrifice of the right common carotid artery. Evaluation of the impact of a single carotid artery in babies treated with ECMO concerns mostly long-term neurodevelopmental outcome. The authors encountered a peculiar problem caused by a single carotid artery in a post-ECMO patient during the surgical correction of aortic coarctation with hypoplastic distal aortic arch. For patients with a confirmed cardiac malformation that necessitates future surgical repair and for whom ECMO support is required, reconstruction of the right common carotid artery should be considered. Veno-venous ECMO is an alternative solution if this approach is not contraindicated because of the patient's clinical condition. Patients with congenital diaphragmatic hernia have a higher incidence of cardiac malformations; therefore, careful cardiological attention is required. Anomalies masked by pulmonary hypertension also must be considered.
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PMID:Surgical repair of an aortic coarctation in a patient after treatment with extracorporeal membrane oxygenation. 787 18

The mortality rate for infants severely affected with congenital diaphragmatic hernia (CDH) remains high despite significant advances in surgical and neonatal intensive care including delayed repair and extracorporeal membrane oxygenation (ECMO). Because of the increasingly successful experience with single-lung transplantation in adults; this approach has been suggested as a potential treatment for CDH infants with unsalvageable pulmonary hypoplasia. The authors report on a newborn female infant who was the product of a pregnancy complicated by polyhydramnios. At birth, she was found to have a right-sided CDH and initially was treated with preoperative ECMO, followed by delayed surgical repair. Despite the CDH repair and apparent resolution of pulmonary hypertension, the infant's condition deteriorated gradually after decannulation, and escalating ventilator settings were required as well as neuromuscular paralysis and pressor support because of progressive hypoxemia and hypercarbia. A lung transplant was performed 8 days after decannulation, using the right lung obtained from a 6-week-old donor. The right middle lobe was excised because of the size discrepancy between the donor and recipient. After transplantation, the patient was found to have duodenal stenosis and gastroesophageal reflux, which required duodenoduodenostomy and fundoplication. The patient was discharged from the hospital 90 days posttransplantation, at 3 1/2 months of age. Currently she is 24 months old and doing well except for poor growth. This case shows the feasibility of single-lung transplantation for infants with CDH, and the potential use of ECMO as a temporary bridge to transplantation. Lobar lung transplantation allowed for less stringent size constraints for the donor lung.
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PMID:Lobar lung transplantation as a treatment for congenital diaphragmatic hernia. 787 27

Pulmonary hypertension is a major complication of congenital diaphragmatic hernia (CDH). Inhaled nitric oxide (NO) is a selective pulmonary vasodilator because it produces vasodilatation of the pulmonary vasculature without systemic hypotension. In experimental and clinical studies, inhaled NO ameliorates pulmonary hypertension and improves gas exchange. The goal of the present study was to determine the extent to which infants with CDH respond to inhaled NO. Four newborn infants with CDH complicated by severe respiratory insufficiency and right-to-left shunting received inhaled NO. In three patients, postductal oxygenation improved in response to small concentrations of NO (5 to 10 ppm); two received NO after operative repair, and the third both before and after repair. However, tachyphylaxis developed in all cases within 1 to 6 days. A fourth patient received inhaled NO in an attempt at weaning from ECMO. He did not respond, remaining hypoxic despite 80 ppm NO, and continued to require ECMO. In the three patients who responded to inhaled NO, plasma nitrites and nitrates (stable oxidative end products of NO) accumulated over time, but not in the patient who did not respond. The accumulation of nitrite and nitrate in plasma may reflect alveolar-capillary NO absorption, and may identify patients who will respond to continued inhaled NO. Methemoglobin remained below 1.9% in all four babies. Selected infants with CDH may respond to NO, but the benefit may be temporary.
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PMID:Inhaled nitric oxide in congenital diaphragmatic hernia. 796 97

To evaluate left ventricular (LV) mass in children with left-sided congenital diaphragmatic hernia (CDH), we retrospectively examined the echocardiographic data available on all newborn infants with a diagnosis of CDH between April 1989 and May 1993. Adequate data for evaluation were available for 20 of 31 patients with left-sided CDH and no significant congenital heart disease. Left ventricular mass was determined from two-dimensional echocardiograms by an area-length method. Findings were compared with a control group that consisted of neonates with other causes of pulmonary hypertension. Patients with left-sided CDH had a significantly lower indexed LV mass than control subjects (1.96 gm/kg +/- 0.59 vs 2.84 gm/kg +/- 0.41; p = 0.0001). Additionally, children with left-sided CDH who required extracorporeal membrane oxygenation before repair (n = 7) had a significantly lower indexed LV mass than those patients who did not require extracorporeal membrane oxygenation before repair (1.53 gm/kg +/- 0.50 vs 2.20 gm/kg +/- 0.52; (p = 0.007). Infants who survived (n = 13) had an indexed LV mass of 2.09 gm/kg +/- 0.58 vs 1.64 gm/kg +/- 0.58 in those who died (p = 0.07). We conclude that the LV mass index in children with left-sided CDH is significantly lower than in children with other causes of pulmonary hypertension in the newborn period. Evaluation of LV mass in neonates with left-sided CDH may help predict the need for extracorporeal support before surgical repair, and may help indicate overall prognosis.
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PMID:Evaluation of left ventricular mass in children with left-sided congenital diaphragmatic hernia. 807 56

Infants with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO) can have initial lung atelectasis which, in survivors, gradually improves over time. To test the hypothesis that these patients could benefit from surfactant therapy, infants with CDH (born at > 34 weeks' gestation) on ECMO received either four doses of modified bovine lung surfactant extract (beractant) (surfactant group, n = 9) or an equal volume of air (control group, n = 8). Tracheal aspirate surfactant protein-A (SP-A) concentrations were initially low, and then increased over time in both CDH groups (P = .0021); however, levels remained low when compared with those of infants on ECMO who had other diagnoses (P = .04). Lung compliance (CL), time to extubation, time on oxygen, and total no. of hospital days were not different between the two groups. Infants with CDH had persistently elevated right ventricular pressure (RVP) at cessation of bypass when compared with non-CDH infants on ECMO (RVP = 53.25 mm Hg +/- 19.52 in the CDH group, 32.90 +/- 10.63 in the non-CDH group; P = .0121). The findings suggest that the postnatal surfactant deficiency may be more persistent in CDH infants than in non-CDH infants on ECMO. However, CDH remains a multifactorial condition, with delayed improvement, because of persistence of pulmonary hypertension, difficulties with vascular remodeling, degree of lung hypoplasia, or compromised respiratory mechanics.
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PMID:Surfactant (beractant) therapy for infants with congenital diaphragmatic hernia on ECMO: evidence of persistent surfactant deficiency. 787 59


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