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 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The type of first aid given to patients requiring emergency pediatric surgery is decisive for the prognosis in many cases. With this aspect in mind, individual disease pictures from the group of connatal deformities (esophageal atresia, intestinal atresia, gastroschisis, diaphragmatic hernia and defect, myelomeningocele), from emergency surgical situations beyond the neonatal stage (acute abdomen and ileus, esophageal varices, pneumothorax), and accident injuries (blunt abdominal trauma, cranio-cerebral trauma, burns) are selected and the most important first aid measures described. But for all diseases, the general rule for the treatment of all seriously ill children applies: provision of a safe venous access, readiness to intubate, adequate oxygenation and control of the acid-base, water and electrolyte balances.
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PMID:[First aid measures in emergency pediatric surgery (author's transl)]. 41 88

Limb deficiencies (LDs) are rarely reported in anencephalic infants. A review of 662 patients in the literature on non-neural defects in anencephaly only showed five patients with LDs. We report on eight patients with various LDs from the records of 141 necropsies of the anencephalic infants found among 495,830 births. Compared with another group of anencephalic infants reported in the literature, the patients in this group of anencephalic infants with LDs were predominantly male, their mean gestational age was younger by approximately 5 weeks, their mean birth-weight was approximately 1,400 g less, and they presented with a higher incidence of polyhydramnios during gestational development. The association of this pair of anomalies, which was 100 times more frequent than expected, seems not due to chance. Since all eight patients had other multiple congenital anomalies (MCA), in addition to anencephaly and LDs, the postmortem study should be mandatory in anencephalic infants with LDs. The most common associated anomalies were cardiovascular and renal defects. Oral clefts, diaphragmatic hernia, esophageal atresia, and imperforate anus were also observed in these infants. The recognition of LDs in anencephalic infants indicates severe and extensive disturbance of the early embryogenesis (blastogenesis), which affects the midline of the embryo.
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PMID:Anencephaly and limb deficiencies. 151 54

Many patients with illnesses that once were fatal at birth or during childhood now survive into adult life. This article considers four respiratory illnesses of early life in which long-term survival now occurs frequently: cystic fibrosis, diaphragmatic hernia, esophageal atresia-tracheoesophageal fistula, and bronchopulmonary dysplasia. In cystic fibrosis, although the median age at death is now 25 years, chronic pulmonary infection due ultimately to the abnormal composition and clearance of airway mucus is still the usual cause of death. Earlier survivors of congenital diaphragmatic hernia had only minor diminution of perfusion and ventilation of the lung on the side of the hernia as adolescents or young adults; however, as infants with greater degrees of pulmonary hypoplasia have successful repair of their hernias, more long-term respiratory impairment will probably be found. The esophageal atresia tracheoesophageal fistula complex leaves all esophagi and many tracheas permanently abnormal; recurrent aspiration, repeated pneumonia, and an unduly collapsible trachea are the result, although symptoms may be few. Survivors of bronchopulmonary dysplasia have decreased exercise capacity, wheezing, and recurrent pneumonia, although their chest radiographs may become normal or almost normal.
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PMID:Caldwell Lecture. Respiratory problems of early life now allowing survival into adulthood: concepts for radiologists. 835 26

To investigate the heterogeneity of congenital malformations, we analyzed the distribution of 14 selected anomalies among 11,421 children with isolated defects and with different patterns of multiple congenital anomalies (MCA). Our study showed a marked variability in the distribution of each of these malformations. For example, although anophthalmia/microphthalmia, cleft palate, and limb deficiency were observed in all etiological categories of syndromes, no case with anencephaly was identified among the 1,244 children with different syndromes. Diaphragmatic hernia, esophageal atresia +/- tracheoesophageal fistula, and gastroschisis were not found in any monogenic syndrome in this sample. These observations may be of help to the clinician in the evaluation of individual children with MCA.
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PMID:Clinical/epidemiological analysis of malformations. 230 61

In newborns with surgically correctable malformations the quality of primary care has considerable influence on the results of the eventual surgical repair. This is particularly true for extensive diaphragmatic and abdominal wall defects as well as for esophageal and intestinal atresia, exstrophy of the bladder and urogenital malformations. The neonatal management comprises measures generally valid for all diseased newborns and measures specific for the particular malformation. The general care concerns heat regulation, fluid balance and other vital functions. Specific regimens are necessary as primary care for diaphragmatic hernia, esophageal atresia, abdominal wall defects and neonatal bowel obstruction. Furthermore this primary management must be extended to include the care of the pregnant mother. Already in utero infants with diaphragmatic and esophageal malformations should be referred to the gynecological-neonatal center.
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PMID:[Initial measures in newborn infants with surgically correctable abnormalities]. 257 73

Using data from the population-based Metropolitan Atlanta Congenital Defects Program, the association of seven relatively common and easily ascertainable groups of midline defects was studied. These defects were neural tube defects (575 patients), oral clefts (633 patients), omphalocele (141 patients), esophageal atresia/tracheoesophageal fistula (88 patients), imperforate anus (151 patients), conotruncal heart defects (289 patients), and diaphragmatic hernia (75 patients). Known syndromes were excluded from the analysis. Of 1743 infants with at least one midline defect, 86 (4.9%) had at least a second midline defect, and 9 (0.5%) had two additional midline defects. Pairwise analysis of the seven defects shows that, although most midline defects tend to be statistically associated with other midline defects, specific combinations of midline defects are seen. For example, neural tube defects are more strongly associated with cleft lip with or without cleft palate than with cleft palate alone; imperforate anus is more strongly associated with spina bifida than with anencephaly or encephalocele. Moreover, some combinations of defects are not observed (eg, neural tube defect and conotruncal heart defect, clefts and diaphragmatic hernia, omphalocele and esophageal atresia/tracheoesophageal fistula). These data point to the need for further refinement in the study of the association of midline defects in terms of embryologic and pathogenetic mechanisms because most midline defects tend to occur as an isolated defect, some midline defects occur with nonmidline defects (such as limb defects), and specific associations among midline defects are observed.
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PMID:Selected midline defect associations: a population study. 274 54

Fetal echocardiography is a well-established technique for the prenatal identification of congenital heart disease. One of the indications for its use is the presence of extracardiac anomalies, as such coexistent defects may have important implications for obstetric and neonatal management. We have reviewed the obstetric and pediatric literature to examine reported associations. If a fetus is suspected to have hydrocephalus, microcephaly, holoprosencephaly, agenesis of the corpus callosum, Meckel-Gruber syndrome, esophageal atresia, duodenal atresia, diaphragmatic hernia, omphalocele, or renal dysplasia, cardiac evaluation should be pursued. Furthermore, echocardiography may be of help in differential diagnosis of some anomalies (for instance, skeletal dysplasias). Maternal diabetes and phenylketonuria, as well as exposure to phenytoin, trimethadione, or isotretinoin, may result in multiple systemic defects, including congenital heart disease.
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PMID:Congenital heart disease and extracardiac anomalies: associations and indications for fetal echocardiography. 293 23

A screening program for latent malformations in infants born with surgically correctable anomalies was reviewed to determine its cost effectiveness. Two hundred and seventy six infants with esophageal atresia, imperforate anus, omphalocele, gastroschisis, or diaphragmatic hernia were screened for latent congenital anomalies not detected by the routine history, physical examination, and roentgenograms. While additional malformations were detected, many congenital defects were missed only to become evident later in the infant's course. Routine screening for latent malformations is not cost effective in all infants with surgically correctable anomalies, but directed screening is indicated in selected neonates. Screening IVP's are indicated in patients with esophageal atresia, high pouch imperforate anus and possibly diaphragmatic hernia. Screening IVP's are not indicated in infants with gastroschisis, omphalocele, or females with low pouch imperforate anus who have normal sacral spine films.
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PMID:Screening for latent malformations: cost effectiveness in neonates with correctable anomalies. 680 16

The occurrence in an infant of coexisting congenital diaphragmatic hernia, proximal esophageal atresia, and distal tracheoesophageal fistula is extremely rare, and the literature contains only anecdotal reports of treatment. Because these infants often have challenging clinical presentations and may deteriorate rapidly, physicians should be aware of manifestations of this combination of conditions and how to manage them.
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PMID:Simultaneous Bochdalek hernia and type C tracheoesophageal fistula. 765 82

We present comparative epidemiologic characteristics of five congenital abnormalities that have been suggested to result from midline abnormal developmental disturbances: esophageal atresia with or without tracheoesophageal fistula (EA/TEF), imperforate anus with or without fistula (IA/F), omphalocele (OM), bladder exstrophy (BE), and diaphragmatic hernia (DH). The purpose was to assess the extent of epidemiologic similarities among these five defects. Data were collected as part of a population-based case-control study of infants with these defects born to mothers residing in Maryland, Washington, D.C., or Northern Virginia from 1980 through 1987. The estimated annual birth prevalences (per 10,000 live births) and 95% confidence intervals (CI) of these five defects were 0.40 (0.26-0.61) for BE, 1.34 (1.08-1.67) for OM, 1.59 (1.29-1.95) for DH, 2.11 (1.76-2.53) for EA/TEF, and 2.97 (2.55-3.46) for IA/F. The birth prevalence of IA/F and DH increased between 1980 and 1987. In contrast to the other four defects, DH showed a significant male preponderance (rate ratio 1.57, 95% CI 1.03-2.47), a significant white excess (rate ratio white:other, 1.56, 95% CI 1.00-2.48), and a lower proportion of multiple associated defects (30% vs. 46-61%). We concluded from this study that the descriptive epidemiology of diaphragmatic hernia is different from that of the other four defects. This finding may imply differences in etiologic and pathogenetic mechanisms underlying DH.
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PMID:Comparative epidemiology of selected midline congenital abnormalities. 801 95


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