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

Neonatal surgery is the most specialized and sophisticated field of pediatric surgery. I herein review esophageal atresia, abdominal wall defects, gastrointestinal perforation, and congenital diaphragmatic hernia (CDH) as representative types of neonatal surgery. The clinical results of esophageal atresia have been considered to reflect the level of medicine of an individual country. Owing to an early diagnosis, improved operative techniques, and better perioperative management, the mortality rate has now become almost 0%. In addition, a minimally invasive thoracotomy is considered to improve long-term quality of life. The overall mortality rate of neonatal surgical disease has markedly decreased and is now less than 10%. However, abdominal wall defects, gastrointestinal perforation, and CDH still show a high mortality rate. A high incidence of chromosomal anomalies results in a poor outcome for abdominal wall defect. A gastrointestinal perforation in an infant complicated with an extremely how birth weight shows a high mortality rate. In CDH, pulmonary hypoplasia as well as pulmonary hypertension often causes an acute respiratory and circulatory deterioration after birth. Neither intensive care for pulmonary hypertension, including ECMO, nor fetal intervention has yet achieved a satisfactory outcome. Permissive hypercapnea and a delayed operation aiming at circulatory stabilization have resulted in a good survival rate. However, CDH survivors may be at risk for long-term morbidities. The method to induce pulmonary development is considered to be mandatory to achieve a good quality of life for severe CDH.
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PMID:Current progress in neonatal surgery. 1856 Sep 58

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and congenital diaphragmatic hernia (CDH) are two relatively common neonatal conditions. The coexistence of the two conditions in the same newborn is extremely rare. This is a case report of a newborn with CDH and concurrent EA and TEF. Aspects of diagnosis and the literature on the subject are also reviewed.
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PMID:Coexisting congenital diaphragmatic hernia, esophageal atresia, and tracheoesophageal fistula: a case report and review of the literature. 1882 68

Esophageal atresia, congenital diaphragmatic hernia, bronchopulmonary malformations and cystic lung diseases are the common neonatal thoracic surgical lesions encountered in practice. The availability of antenatal ultrasonography has lead to these lesions being detected before birth. Antenatal diagnosis can be made with a fair degree of accuracy in tertiary fetal medicine centres. Antenatal intervention is limited in a very few centres in the western world and not being done in India at present. The outcome of these babies with antenatal diagnosis of thoracic lesions has changed in the last decade. Earlier intervention is now possible in cystic lung disease before infectious complication has set in. All these lesions are managed exclusively in well developed neonatal surgery units with excellent outcome in the western world. The present study reviews the antenatal detection, clinical presentation, interventional/surgical procedures [antenatally and postnatally] and outcome of these common neonatal thoracic surgical lesions.
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PMID:Perinatal management of common neonatal thoracic lesions. 1901 6

A newborn female infant born to a woman on immunosuppressive medications including mycophenolate mofetil (MMF) for a renal graft secondary to lupus nephritis presented with congenital diaphragmatic hernia (CDH) and additional findings of microtia, esophageal atresia with tracheoesophageal fistula, cleft palate, congenital heart defect, digital anomalies, and dysmorphic facial features. Pulmonary hypoplasia resulted in death at day 2 of life. She was presumed to have Fryns syndrome based on diagnostic criteria established for this recessive disorder with prominent features including CDH, facial anomalies, and nail hypoplasia. In retrospect, this infant's findings are more likely the result of teratogenic exposure to MMF, as more recent data have emerged linking aural atresia, digital anomalies, and dysmorphic features to this drug. To date, this is the only human report of CDH in an infant with prenatal exposure to MMF, although the manufacturer's package insert alludes to animal studies with a broad spectrum of malformations, including CDH. Thus, a teratogenic exposure can mimic a known Mendelian genetic syndrome, and caution is urged in presuming a genetic etiology for infants with potential teratogenic exposure to relatively new drugs with limited published animal data.
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PMID:Congenital diaphragmatic hernia and microtia in a newborn with mycophenolate mofetil (MMF) exposure: phenocopy for Fryns syndrome or broad spectrum of teratogenic effects? 1944 4

Prenatal diagnosis of concomitant duodenal atresia (DA) and esophageal atresia (EA) without tracheoesophageal fistula (TEF) (Gross type A) is very rare. We describe prenatal findings of one such case. Sonographic examination of a 26-week fetus showed a double cystic structure and an intrathoracic cyst. MRI and ultrasound at 26 weeks and 2 days' gestation showed shrinkage of the stomach and duodenum, massive ascites, and the presence of dilated pouch-like structure in the thoracic inlet level, consistent with an upper pouch sign. Polyhydramnios was detected at 30 weeks' gestation. Prenatal diagnosis was concomitant DA and an intrathoracic anomaly such as congenital hiatal hernia, diaphragmatic hernia, esophageal duplication or EA. A boy was delivered at 38 weeks' gestation. Physical examination showed a markedly distended abdomen and imperforate anus. Emergency surgery revealed existing DA and EA without TEF (Gross type A).
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PMID:Prenatal findings of concomitant duodenal and esophageal atresia without tracheoesophageal fistula (Gross type A). 1957 91

Congenital diaphragmatic hernia is known to be associated with esophageal atresia, which is a rare association. We report a rare occurrence of congenital diaphragmatic hernia and lower esophageal atresia.
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PMID:A rare association of congenital diaphragmatic hernia with lower esophageal atresia and perforation. 2070 57

NIRS has been used as a key device with the aim to evaluate the impact of surgery and anesthesia on cerebral and splanchnic oxygenation in neonatal population. The main applications has been in cardiac surgery, congenital diaphragmatic hernia and esophageal atresia. In this report we summarized the results published on the application of NIRS in neonatal surgery with particular respect to cerebral and splanchnic oxygenation, presenting also some future prospective.
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PMID:Near infrared spectroscopy in newborns with surgical disease. 2194 93

The occurrence of phenotypic differences between monozygotic (MZ) twins is commonly attributed to environmental factors, assuming that MZ twins have a complete identical genetic make-up. Yet, recently several lines of evidence showed that both genetic and epigenetic factors could have a role in phenotypic discordance after all. A high occurrence of copy number variation (CNV) differences was observed within MZ twin pairs discordant for Parkinson's disease, thereby stressing on the importance of post-zygotic mutations as disease-predisposing events. In this study, the prevalence of discrepant CNVs was analyzed in discordant MZ twins of the Esophageal Atresia (EA) and Congenital Diaphragmatic Hernia (CDH) cohort in the Netherlands. Blood-derived DNA from 11 pairs (7 EA and 4 CDH) was screened using high-resolution SNP arrays. Results showed an identical copy number profile in each twin pair. Mosaic chromosome gain or losses could not be detected either with a detection threshold of 20%. Some of the germ-line structural events demonstrated in five out of eleven twin pairs could function as a susceptible genetic background. For example, the 177-Kb loss of chromosome 10q26 in CDH pair-3 harbors the TCF7L2 gene (Tcf4 protein), which is implicated in the regulation of muscle fiber type development and maturation. In conclusion, discrepant CNVs are not a common cause of twin discordancy in these investigated congenital anomaly cohorts.
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PMID:Copy number detection in discordant monozygotic twins of Congenital Diaphragmatic Hernia (CDH) and Esophageal Atresia (EA) cohorts. 2207 87

Intrathoracic congenital malformations may be associated with long-term pulmonary morbidity. This certainly is the case for congenital diaphragmatic hernia, esophageal atresia and cardiac and aortic arch abnormalities. These conditions have variable degrees of impaired development of both the airways and lung vasculature, with a postnatal impact on lung function and bronchial reactivity. Pulmonary complications are themselves frequently associated to non-pulmonary morbidities, including gastrointestinal and orthopaedic complications. These are best recognized in a structured multidisciplinary follow-up clinic so that they can be actively managed.
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PMID:Long term respiratory outcomes of congenital diaphragmatic hernia, esophageal atresia, and cardiovascular anomalies. 2229 25

This report describes an infant born with esophageal atresia, tracheoesophageal fistula, right-sided diaphragmatic hernia, and an interrupted inferior vena cava (IVC) that underwent successful repair. The report emphasizes the importance of diagnosing an interrupted IVC prior to tracheoesophageal repair.
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PMID:Preserving the azygos vein when repairing esophageal atresia and tracheoesophageal fistula accompanied by interrupted inferior vena cava. 2412 74


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