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Query: UMLS:C0019270 (hernia)
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The perinatal autopsy is frequently viewed by pathologists as being scientifically unrewarding and contributing little to patient care. To emphasize its importance in patient management, genetic counselling and specific research, a ten-year review (1979-88) was made of the perinatal autopsy experience at Loyola University Medical Center. The 657 deaths included late fetal deaths (22%), early neonatal deaths (51%), late neonatal deaths (10%) and deaths between 29 days and one year (17%). Comparison of the principal causes of death in various groups categorized by birth weight and age revealed significant trends. There has been a steady decline in mortality from immediate complications of immaturity, while the mortality rate from long-term complications of immaturity has not increased. There were increased frequencies of congenital diaphragmatic hernia and cardiac malformations, while the frequency of renal malformations decreased; the etiological significance of these changes requires further evaluation. A correlation of clinical observations with post-mortem findings indicates that newer diagnostic procedures, such as ultrasound, echocardiography and cardiac catheterization, are of limited value for accurate diagnosis of complex anomalies, cystic renal disease and chromosomal anomalies. A substudy included cases up to 18 years of age. The frequency of childhood neoplasia was low (17 cases), and among these cases there was a predominance of haematological malignancies (11 cases). Even with these small numbers, a shift in cause of death from disseminated malignancy to overwhelming infection was apparent. This paper includes the essential elements of a protocol for perinatal autopsies, with illustrations of specific applications. The perinatal autopsy is clearly an undervalued source of information and discovery. Little or no information is available from developing countries, where autopsies could provide information on causes of paediatric mortality and permit recognition of disease patterns, which is so critical to the planning of health services.
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PMID:The perinatal autopsy: a neglected source of discovery. 185 33

Trends are changing in the management of infants and children with indirect inguinal hernias. Advances in neonatal intensive care have resulted in the survival of many small premature infants who have a high incidence of inguinal hernia. The rate of incarceration, strangulation, and gonadal infarction in these babies is twice that of the general pediatric age group. Respiratory immaturity, apnea, bradycardia, and associated neonatal conditions require special management at the time of hernia repair, usually performed just before discharge from the neonatal intensive care unit. New information concerning volume loss and depletion of germ cells beginning at 6 months of age in boys with undescended testes has stimulated the performance of orchiopexy when the patient is 1 year of age. More than 90% of boys with cryptorchid testes at the age of 1 year have an associated hernia that requires concomitant repair at the time of orchiopexy. The use of the peritoneal cavity for fluid absorptive purposes in hydrocephalus treated by venticuloperitoneal shunts or of peritoneal dialysis for renal failure and metabolic diseases such as hyperammonemia and lactic acidosis causes increased intraabdominal pressure and results in the appearance of a previously unrecognized hernia. Recognition of these and other conditions associated with a high incidence of hernial occurrence should allow early diagnosis and treatment before the development of complications. Most elective repairs of hernias are safely performed in the outpatient setting; however, some infants and children with concurrent illnesses are best managed in a "morning admissions" program, in which hospital admission occurs postoperatively.
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PMID:Current concepts in inguinal hernia in infants and children. 257

Congenital diaphragmatic hernia, a highly lethal condition, displays at term the pulmonary biochemical and morphologic immaturity characteristic of premature delivery. We hypothesized that antenatal glucocorticoid, now the standard treatment to prevent hyaline membrane disease in premature human beings, might correct the parameters of the pulmonary biochemical and morphologic immaturity in severe congenital diaphragmatic hernia. A total of 112 fetal rats with or without nitrofen-induced congenital diaphragmatic hernias from 34 pregnancies were treated antenatally with either saline or dexamethasone. Antenatal dexamethasone increased the lung disaturated phosphatidylcholine content, reduced the lung glycogen concentration, reduced the saccular septal thickness, and increased the mean saccular size and volume fraction of saccules in the lungs of rats with large congenital diaphragmatic hernia in comparison with similar rats not so treated. All differences were statistically significant. Antenatal glucocorticoid therapy was efficacious in treating rats with nitrofen-induced congenital diaphragmatic hernia. This encouraging finding warrants further investigation in a large animal model with surgically created congenital diaphragmatic hernia.
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PMID:Antenatal glucocorticoid corrects pulmonary immaturity in experimentally induced congenital diaphragmatic hernia in rats. 752 May 65

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

Neonates with congenital diaphragmatic hernia (CDH) continue to have unacceptably high mortality rates. To better understand the associated pulmonary pathology we measured biochemical parameters of lung maturity in neonatal rats with or without congenital diaphragmatic hernia created by maternal feeding of a single dose of nitrofen on day 9.5 or day 11.5 of gestation. Lungs from neonatal rats with large CDH (n = 9, 5 right-sided, 4 left-sided) had a significantly lower lung weight (P = .0001), lung weight/body weight ratio (P = .0001), disaturated phosphatidylcholine (DSPC) per microgram DNA (P < .005), total DSPC (P = .0001), total DNA (P < .05), protein per microgram DNA (P < .05), and total protein content (P < .005) when compared with lungs from the litter mates without congenital diaphragmatic hernia (n = 10). The lungs of rats with hernia also had significantly higher DNA concentrations (P < .05) and glycogen concentrations (P < .05). These data demonstrate that lungs in neonatal rats with large CDH are biochemically immature. Treatment directed toward correcting the pulmonary biochemical immaturity of affected fetuses before birth may improve the prognosis for these babies.
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PMID:Biochemical immaturity of lungs in congenital diaphragmatic hernia. 846 64

We studied the biochemical maturity of the lungs of fetuses born to rats exposed to nitrofen on day 9.5 of gestation. In comparison with controls, nitrofen-treated fetuses had pulmonary hypoplasia (decreased lung/body weight), lung hypocellularity (low DNA content) and cellular atrophy (low protein/DNA and phospholipid/DNA) on gestational days 19 and 21. Treated animals with congenital diaphragmatic hernia (CDH) also had cell atrophy and surfactant immaturity (decreased disaturated phosphatidylcholine/DNA) near term. Our data demonstrate that nitrofen causes lung hypoplasia and some degree of surfactant system immaturity that is particularly prominent in fetuses with CDH.
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PMID:Lung hypoplasia and surfactant system immaturity induced in the fetal rat by prenatal exposure to nitrofen. 871 54

Experimental and clinical findings indicate immaturity of pulmonary surfactant in congenital diaphragmatic hernia (CDH). Lung histology has shown a decreased amount of lamellar bodies. A low lecithin/sphingomyelin ratio in the amniotic fluid, and decreased concentrations of surfactant protein A and disaturated phosphatidylcholine in the pulmonary tissue and the amniotic fluid have been reported. Furthermore, low compliance and high surface tension have also been found. Evidence of clinical and experimental findings of structural, biochemical and functional pulmonary immaturity in CDH is reviewed. Prenatal administration of corticosteroids to accelerate fetal pulmonary maturation, and the use of early surfactant therapy, should be further evaluated in the clinical management of CDH.
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PMID:Pulmonary surfactant dysfunction in congenital diaphragmatic hernia: experimental and clinical findings. 879 Sep 10

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and persistent pulmonary hypertension. Recent experimental studies suggest that surfactant deficiency may also contribute to CDH pathophysiology. In this report, the authors immunohistochemically and morphometrically examined whether or not the hypoplastic lungs of CDH are associated with the immaturity of the surfactant system, especially alveolar type II cell function. Nine autopsy cases with CDH were immunohistochemically examined for the expression of surfactant apoprotein, using anti-IgG against human surfactant apoprotein A (SP-A), and the findings were compared with those in a gestational and postnatal age-matched control group. The lung/body weight ratio in the CDH was less than that in the controls (0.010 +/- 0.005 versus 0.021 +/- 0.013, P < .01). The radial alveolar count (RAC) of the CDH cases were also significantly less than that of the control cases (2.10 +/- 0.52 versus 3.48 +/- 0.39, P < .01). In the CDH cases, the RAC of the lung on the affected side were also significantly less than that of the lung on the unaffected side (1.71 +/- 0.34 versus 2.50 +/- 0.26, P < .01). In the immunohistochemical distribution of SP-A, compared with the control cases, the number of SP-A-positive cells on the alveolar septa of the CDH cases decreased in number, and this immunohistochemical reaction was weak even in positive type II cells. In addition, the immunoreaction observed in the alveolar type II cells of the unaffected side lungs in the four CDH cases was stronger than that of the unaffected side lungs. These results suggest that in the lungs of the CDH cases, especially on the affected side, there is a possible delay in both the structural growth and functional maturation or development of SP-A synthesis by alveolar type II cells, and this retardation of the functional maturation in alveolar type II cells is also considered to play a role in postnatal respiratory insufficiency in CDH patients.
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PMID:Immunohistochemical distribution of surfactant apoprotein-A in congenital diaphragmatic hernia. 916 47

Lung autopsies from four neonates with Ebstein's anomaly or tricuspid valve dysplasia and gross cardiomegaly were examined. The mean cardiothoracic ratio was 92 +/- 0.5%. The degrees of pulmonary hypoplasia and pulmonary artery muscularization were assessed and were compared with those in lungs from four controls and four patients with diaphragmatic hernias. Lung and body weight ratios and radial alveolar counts, which reflect pulmonary hypoplasia and immaturity, were significantly decreased only in patients with diaphragmatic hernia. The thickness of the medical muscle layer in small pulmonary arteries was greater in patients with diaphragmatic hernia; however, in patients with tricuspid valve disease, it was relatively small. Abnormal vascular muscle extension was seen only in patients with diaphragmatic hernia. In the cases of tricuspid valve disease, although cardiomegaly and lung compression were severe, lung hypoplasia and immaturity were not, and neither abnormal medial thickening nor extension were found. Our results suggest that, at least in full-term infants with tricuspid valve disease, surgical relief of lung compression may improve respiratory function, even if the cardiomegaly is severe.
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PMID:The histology of the lung in neonates with tricuspid valve disease and gross cardiomegaly due to severe regurgitation. 956 4

The incidence of congenital diaphragmatic hernia (CDH) is 1:1,207-5,000, and the condition is associated with high mortality and morbidity, attributed principally to associated pulmonary hypoplasia. Repairing the diaphragmatic defect by antenatal surgery has high mortality, mainly due to premature labor. Antenatal tracheal occlusion, which is achievable by less invasive methods, stimulates lung growth (weight and DNA). However, its effectiveness in reversing structural and maturational abnormalities and its optimal timing requires further investigation. We hypothesized that (1) antenatal tracheal occlusion performed in the lamb model of congenital diaphragmatic hernia will stimulate lung growth and structural development and restore lung structure and maturity toward normal levels by term gestation; (2) effects will be detectable by morphometric measurements of the following parameters: lung volume, ratio of parenchyma to nonparenchyma, volume density of connective tissue within nonparenchyma, ratio of gas exchange tissue to airspace in parenchyma, gas exchange surface area, capillary loading, alveolar/airspace density and alveolar perimeter; (3) effects will be seen in all lobes of the lung; and (4) a greater effect will be observed when tracheal occlusion is performed early rather than late in gestation. Fourteen lambs underwent CDH creation at gestation day 72-74 followed by tracheal occlusion at day 101 (n = 7) or 129 (n = 7). They were delivered by Cesarean section at 143 days (term = 145-149). Lungs were obtained at autopsy, inflation fixed, divided into lobes, and sampled; morphometric analysis was performed. Comparisons were made with previously reported results from control lungs of normal lambs and lambs with untreated CDH. In comparison with untreated lungs, antenatal tracheal occlusion at both times resulted in increased volumes for total lung and lobes, increased volume density of parenchyma and of airspace within parenchyma, and increased gas exchange surface areas. Normal values for gas exchange surface area density, and alveolar density and perimeter were attained and the lungs appeared more mature than non-occluded lungs. Tracheal occlusion earlier in gestation produced a greater effect, achieving greater than normal values for lung volumes and volume densities, whereas the capillary loading value was similar to normal lung. Later occlusion achieved less than normal values for lung volumes and volume densities, with a reduced capillary loading value. We conclude that antenatal tracheal occlusion is capable of reversing structural total lung and lobar hypoplasia and immaturity caused by CDH as determined by morphometrically determined parameters. The effect is greater when tracheal occlusion is performed early rather than late in gestation. The results are encouraging for development of treatment methods for humans with antenatally diagnosed CDH.
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PMID:Effect of antenatal tracheal occlusion on lung development in the sheep model of congenital diaphragmatic hernia: a morphometric analysis of pulmonary structure and maturity. 959 Apr 86


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