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

Eleven patients with congenital, traumatic and functional extrahiatal diaphragmatic lesions are reported. Since 1991 two patients with acute, two patients with old ruptures of the diaphragm and one patient with a Morgagni-Larrey-hernia were successfully treated by laparoscopic direct suturing. In two other patients with Morgagni hernias we used a polypropylene mesh for closure of the defect. One procedure was performed in a patient with symptomatic congenital dysplasia of the diaphragm with aplasia of the pericard. Laparoscopic plication of the diaphragm was performed in three symptomatic patients with phrenic nerve palsy after cardiac surgery. The intra- and postoperative course was uneventful in all cases. During a median follow-up of 60 months there was no recurrence. Therefore the laparoscopic technique is an effective and attractive alternative for treatment of these diseases.
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PMID:[Indications, techniques and results of laparoscopic surgery for diaphragmatic diseases]. 1212 88

Interstitial proliferation of striated muscle in the lung is extremely rare. Most cases are associated with other congenital malformations, such as lung sequestration, diaphragmatic hernia, or cardiac malformations. We describe a newborn with rhabdomyomatous dysplasia of the lung associated with multiple congenital malformations of the heart and great vessels. The female neonate was born at 37 weeks of gestation as the second child to a 31-year-old woman without relevant previous medical or family history. In week 26 of gestation, a complex heart malformation and polyhydramnion were diagnosed by ultrasound. Postnatally, right lung hypoplasia, a bilobar right and left lung, anomalous drainage of the pulmonary veins, atrial and ventricular septal defects, and double-outlet right-ventricle and multiple aortopulmonary collaterals were described. Histological examination of a biopsy of the right lung demonstrated the presence of numerous bundles of striated muscle fibers arranged randomly in the pulmonary interstitium. Unilateral resection of the right lung was not a therapeutic option, because the left lung had developed bronchopulmonary dysplasia with severe reduction in gas exchange as a consequence of long-term mechanical ventilation. Symptomatic relief and palliative cardiac surgery were offered. At age 5 months, the infant died of a pulmonary hemorrhage following cardiac surgery.
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PMID:Rhabdomyomatous dysplasia of the newborn lung associated with multiple congenital malformations of the heart and great vessels. 1220 52

Although most lesions that occur in the chest have a nonspecific soft-tissue appearance, fat-containing lesions are occasionally encountered at cross-sectional computed tomography (CT) or magnetic resonance imaging. The various fat-containing lesions of the chest include parenchymal and endobronchial lesions such as hamartoma, lipoid pneumonia, and lipoma. Endobronchial hamartoma usually appears at CT as a lesion with a smooth edge, focal collections of fat, or fat collections that alternate with foci of calcification. Mediastinal fat-containing lesions include germ cell neoplasms, thymolipomas, lipomas, and liposarcomas. The most frequent CT manifestation of the germ cell neoplasm teratoma is a heterogeneous mass with soft-tissue, fluid, fat, and calcium attenuation. Cardiac lesions with fat content include lipomatous hypertrophy of the interatrial septum and arrhythmogenic right ventricular dysplasia. Diagnosis of the former is made with CT when a smooth, nonenhancing, well-marginated fat-containing lesion is identified in the interatrial septum. Finally, fat may herniate into the chest at several characteristic locations. When such a lesion is identified, the time required for differential diagnosis is significantly reduced, often allowing a definitive radiologic diagnosis. Sagittal and coronal reformatted images can add valuable information by showing diaphragmatic defects and hernia contents.
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PMID:Fat-containing lesions of the chest. 1237 1

Pulmonary hypoplasia is a rare cause of pulmonary insufficiency, and has a significant rate of morbidity and mortality among affected infants. In most cases, pulmonary hypoplasia is secondary to underlying abnormalities. These may include space occupying lesions, as in infants with congenital diaphragmatic hernia; malformation of chest wall resulting in a small thoracic cavity; severe and prolonged oligohydramnios; and neuromuscular disorders, which prevent normal fetal chest expansion. All lead to poor lung development. Primary pulmonary hypoplasia as a result of congenital acinar dysplasia is exceedingly rare and is diagnosed by exclusion of all known etiologies of secondary pulmonary hypoplasia.
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PMID:Congenital acinar dysplasia. Familial cause of a fatal respiratory failure in a neonate. 1259 Feb 84

The aim of this study was to evaluate the role of MR imaging of the fetus to improve sonographic prenatal diagnosis of congenital anomalies. In 40 fetuses (not consecutive cases) with an abnormality diagnosed with ultrasound, additional MR imaging was performed. The basic sequence was a T2-weighted single-shot half Fourier (HASTE) technique. Head, neck, spinal, thoracic, urogenital, and abdominal fetal pathologies were found. This retrospective, observational study compared MR imaging findings with ultrasonographic findings regarding detection, topography, and etiology of the pathology. The MR findings were evaluated as superior, equal to, or inferior compared with US, in consent with the referring gynecologists. The role of these findings in relation to pregnancy management was studied and compared with postnatal follow-up in 30 of 40 babies. Fetal MRI technique was successful in 36 of 39 examinations and provided additional information in 21 of 40 fetuses (one twin pregnancy with two members to evaluate). More precise anatomy and location of fetal pathology (20 of 40 cases) and additional etiologic information (8 of 40 cases) were substantial advantages in cerebrospinal abnormalities [ventriculomegaly, encephalocele, vein of Galen malformation, callosal malformations, meningo(myelo)cele], in retroperitoneal abnormalities (lymphangioma, renal agenesis, multicystic renal dysplasia), and in neck/thoracic pathology [cervical cystic teratoma, congenital hernia diaphragmatica, congenital cystic adenomatoid lung malformation (CCAM)]. This improved parental counseling and pregnancy management in 15 pregnancies. In 3 cases, prenatal MRI findings did not correlate with prenatal ultrasonographic findings or neonatal diagnosis. The MRI provided a more detailed description and insight into fetal anatomy, pathology, and etiology in the vast majority of these selected cases. This improved prenatal parental counseling and postnatal therapeutic planning.
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PMID:The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis. 1269 20

We report on two cases (male twins), and a female sib terminated at 20 weeks, whose autopsy revealed features of spondylothoracic dysplasia (STD) and also a diaphragmatic hernia and preaxial polydactyly. We present the findings and review STD and the closely related spondylocostal dysostosis. On the basis of the discussion we suggest that our cases are possibly the first report of preaxial polydactyly in spondylothoracic dysplasia and that STD and spondylocostal dysostosis may be allelic.
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PMID:Diaphragmatic hernia and preaxial polydactyly in spondylothoracic dysplasia. 1456 19

Although adrenomedullin (ADM) is a potent vasodilating peptide reported to play a possible role in the mechanisms of fetal lung differentiation and maturation, the ADM blood level in fetuses and in neonates with persistent pulmonary hypertension (PPHN) and pulmonary hypoplasia is not known. Therefore, we examined 15 patients with PPHN: 10 with congenital diaphragmatic hernia, four with congenital cystic adenomatoid malformation of the lung, and one with misalignment of pulmonary vessels with alveolar capillary dysplasia. Eight surgical patients with neonatal conditions such as intestinal atresia served as controls. Blood samples were drawn from the umbilical artery and vein at birth, and arterial blood was drawn from patients with PPHN on the 3rd and 6th days after birth. Plasma levels of ADM were measured by radiometric assay. Plasma levels of ADM in the umbilical artery and vein were elevated in patients with PPHN compared with controls, and in all groups the levels in the umbilical vein were higher than those in the umbilical artery. The arterial levels in patients with poor prognoses were elevated on the 3rd and 6th days after birth compared with those in survivors. These results indicate that ADM may be involved in the pathophysiology of PPHN and in the mechanisms of lung differentiation and/or maturation.
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PMID:Is adrenomedullin involved in the pathophysiology of persistent pulmonary hypertension of the newborn? 1469 36

Fryns syndrome (FS) is the commonest autosomal recessive syndrome associated with congenital diaphragmatic hernia (CDH) and comprises CDH, pulmonary hypoplasia, craniofacial anomalies, distal limb hypoplasia, and internal malformations. Although there have been more than 50 case reports on probands with FS, the diagnostic guidelines were formulated from a review of eight patients and modifications to the guidelines have only once been suggested. Recently, several case reports have described new anomalies in FS and other papers have highlighted the variation in expressivity found in FS. This paper examines the medical literature on FS to define the phenotype and to review the diagnostic guidelines. We conclude that CDH with brachytelephalangy and/or nail hypoplasia is strongly suggestive of the diagnosis and that pulmonary hypoplasia, craniofacial dysmorphism, orofacial clefting, and polyhydramnios are sufficiently frequent to be diagnostically useful. Other distinctive malformations that are consistent with FS include ventricular dilatation or hydrocephalus, agenesis of the corpus callosum, neuronal or cerebellar heterotopias, abnormalities of the aorta, renal cysts, dilatation of the ureters, bicornuate uterus, renal dysplasia, proximal thumbs, and broad clavicles.
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PMID:Fryns syndrome: a review of the phenotype and diagnostic guidelines. 1473 97

Our understanding of lung development in the past two decades has moved from an anatomical to a histological basis and, most recently, to a molecular basis. Tissue interactions specify tracheal and lung primordia formation, program branching morphogenesis of the airway epithelium and regulate epithelial differentiation. In addition, lung development is influenced by mechanical and humoral factors. The regulatory molecules involved in morphogenetic signaling include growth and transcription factors and extracellular matrix molecules. These morphogenetic signals are responsible for lung patterning and differentiation. We will provide a brief overview of molecular signaling during early respiratory formation, airway branching, pulmonary vascularization and epithelial differentiation. We will then review aberrant morphogenetic signaling in human lung abnormalities, such as tracheoesophageal fistula, congenital diaphragmatic hernia, pulmonary hyperplasia, alveolar capillary dysplasia, congenital cystic adenomatoid malformation and bronchopulmonary dysplasia.
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PMID:The molecular basis for abnormal human lung development. 1559 17

Neonatal extracorporeal support is most often required for neonatal hypoxemic respiratory failure, usually accompanied by persistent pulmonary hypertension of the newborn (PPHN). PPHN is a clinical syndrome that results from the failure of pulmonary vascular transition to extrauterine life. Infants typically present shortly after birth with respiratory distress and cyanosis, but a structurally normal heart. The incidence of PPHN is estimated at 0.2% of live-born term infants. Respiratory failure and hypoxemia in the term newborn result from a heterogeneous group of disorders, and the therapeutic approach and response often depend on the underlying disease. PPHN can largely be thought of as one of three types: (1) the abnormally constricted pulmonary vasculature which is the most common type and includes diagnoses such as meconium aspiration syndrome, respiratory distress syndrome, and sepsis; (2) the structurally abnormal vasculature, which is often termed idiopathic PPHN; or (3) the hypoplastic vasculature such as is seen in congenital diaphragmatic hernia, or alveolar capillary dysplasia, a rare malformation of lung development. The pathophysiology of each type is dependent on the point in gestation when the normal transition to extrauterine life fails. This article will discuss the known pathophysiology in PPHN and new treatment modalities.
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PMID:The diseases treated with ECMO: focus on PPHN. 1592 Nov 47


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