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

A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in Mainz from 1977 to 1996. IND was associated with Hirschsprung's disease in 47 cases; 22 children with IND had other abdominal malformations, including anal atresia, rectal stenosis, sigmoidal stenosis, ileal atresia, pyloric stenosis, and esophageal atresia. A cystic bowel duplication, a choledochal cyst, and a persisting urachus were also found. Extra-abdominal malformations such as Down's syndrome, congenital diaphragmatic hernia, aortic stenosis, and malformations of vertebral bodies were seen. Twin siblings of children with IND were either healthy (n=3) or died in utero (n=1). Seventeen children with IND developed severe intra-abdominal complications during the perinatal period such as necrotizing enterocolitis (NEC), meconium ileus, or bowel perforations. NEC was frequently associated with preterm birth. Bowel perforations were seen in mature and preterm newborns with IND. Taken together, IND is found in a variety of obstructive bowel diseases. This may support the hypothesis that IND is a secondary phenomenon or that congenital atresias and stenoses of the digestive tract have a pathogenesis similar to that of intestinal innervation disturbances. IND may also be a part of complex malformation patterns since it occurs with a number of extraintestinal and non-obstructive intestinal malformations.
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PMID:Congenital malformations and perinatal morbidity associated with intestinal neuronal dysplasia. 971 73

A new case report of laparoscopic repair of a diaphragmatic hernia through the foramen of Morgagni in a 11-year-old boy with Down's syndrome is described. The review of 11 other cases in the adult literature illustrated the various aspects of this laparoscopic approach.
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PMID:[Laparoscopic treatment of a diaphragmatic hernia through the foramen of Morgagni in children. A case report and review of eleven cases reported in the adult literature]. 995 Nov 12

This chapter presents the American Academy of Pediatrics guidelines for participation in competitive sports, and discusses several conditions that may require exclusion. These conditions include absence of paired organs, hernia, Down syndrome, hypertension, cardiac and respiratory diseases, diabetes mellitus, dermatologic infections, and neurologic disorders.
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PMID:Medical Exclusion from Sport. 1035 Jul 37

Nuchal translucency refers to the normal subcutaneous space, observed on first trimester ultrasound examination, between the skin and the cervical spine in the fetus. Increased nuchal translucency is known to be associated with an increased risk of aneuploidy, particularly Down syndrome. In addition to this association with aneuploidy, multiple studies have now identified increased nuchal translucency as a nonspecific marker of a wide range of fetal structural abnormalities, to include congenital diaphragmatic hernia, cardiac defects, and various genetic syndromes. The degree of nuchal translucency is directly related to the prevalence of fetal anomalies and may have prognostic significance, especially when found in association with other anomalies. The pathophysiology of increased nuchal translucency is uncertain but may be the result of cardiac failure or alterations in lymphatic drainage. Increased nuchal translucency may identify pregnancies that require further assessment, to include additional sonographic evaluation and possible fetal echocardiography. Further evaluation is required to assess the role of nuchal translucency screening in the general population.
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PMID:First trimester screening for structural fetal abnormalities: nuchal translucency sonography. 1055 91

A retrospective clinical study was performed to evaluate the hernias encountered in the anterior part of the diaphragm. Twenty patients (14 males, 6 females; aged 7 days-7 years) with hernias located in the anterior part of the diaphragm who were treated surgically formed the study group. The exact locations, contents and additional malformations were evaluated. The locations were parasternal in 14 and retrosternal in six. Parasternal locations were the right side in 11, left side in two and bilateral in one patient. Three patients had trisomy 21 syndrome. A sac was presented in all cases and included the colon in 12 patients. A patient with retrosternal location also had trisomy 21 syndrome. The patients with retrosternal hernias also presented with sacs, and the colon was the most commonly included viscus. In the presented series, no intrapericardial herniations or anteromedial defects were encountered. Comparison of previously reported patients and the present series suggests that the anterior part of the diaphragm hosts various hernias of congenital origin in its different locations. According to the exact location and the presence or absence of sacs, four different types of hernias occur in this area: retrosternal hernias with a sac, intrapericardial herniation, and parasternal and anteromedial hernias with either unilateral or bilateral involvements. Since four different hernias were distinguished, the term Morgagni hernia does not include or define all the hernias of the anterior part of the diaphragm. We believe they should, therefore be designated according to the location and presence or absence of a sac.
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PMID:Four different hernias are encountered in the anterior part of the diaphragm. 1077 Jan 16

Morgagni's hernia is an uncommon type of diaphragmatic hernia in the pediatric age group. Out of 52 children with different types of congenital diaphragmatic hernia that we have treated, 5 (9.6%) had Morgagni's hernia. There were 2 infants and 3 children including one with Down's syndrome. All suffered from repeated attacks of chest infection, and only after a chest X-ray was the diagnosis of Morgagni's hernia suspected. In 2 cases this appeared as an opacity in the anterior mediastinum adjacent to the pericardium; diagnosis was confirmed by barium enema in one and a CT-scan in the other. The remaining 3 cases showed anterior herniation of bowel loops on chest X-ray which was bilateral in one. This bilaterality was confirmed pre-operatively by CT scan. Associated anomalies were present in all cases, including 2 with malrotation. All patients were treated surgically via a transabdominal approach. Our study shows a relative high frequency of Morgagni's hernia in our patients and, although late-presenting Morgagni hernias are relatively benign, it can cause significant morbidity. This calls for early diagnosis and early referral for surgery. Chest X-ray is to be strongly advocated in children with repeated attacks of chest infection.
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PMID:Congenital Morgagni's hernia in infants and children. 1107 35

Prenatal diagnosis has traditionally occurred between the 15th and 20th weeks of gestation. However, the capabilities of screening and diagnostic tools have advanced substantially over the past 10 years. Recent advances in the science of prenatal diagnosis allow for the evaluation of an affected embryo or an abnormal cell line prior to gestation within the womb via preimplantation diagnosis. The technique can be used for any genetic condition which can be detected with a chromosome-specific probe. At the current time, noninvasive second trimester maternal serum screening with either 2 or 3 serum analytes is associated with a 60-70% detection rate of Down syndrome. Although these particular serum markers are not useful during the first trimester, the fetoplacental secretory products-free beta-hCG and pregnancy-associated plasma protein-A (PAPP-A) appear to be meaningful clinically when measured between 8 and 13 weeks of gestation, yielding similar first trimester detection rates as the current second trimester screening programme. The addition of nuchal translucency measurements as an independent predictor of fetal aneuploidy may further increase the detection rate of Down syndrome to 80%. Open fetal surgery is now possible under highly selective circumstances in which the fetal condition is considered life-threatening and the prognosis is extremely poor. Surgical intervention may be appropriate for congenital cystic adenomatoid malformation, bronchopulmonary sequestration, congenital diaphragmatic hernia, and possibly for myelomeningocele.
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PMID:Prenatal diagnosis and fetal therapy--what lies in future? 1126 89

Morgagni hernia is a rare diaphragmatic hernia accounting for only 2% of the congenital diaphragmatic defects. A case of Morgagni hernia was diagnosed radiologically in a 12-months-old male with Down syndrome, with recurrent respiratory distress and chest deformity. The 2-dimensional echocardiography was normal. The diagnosis of Morgagni hernia was confirmed by barium studies. The patient underwent a corrective surgery at 18 months of age following which his symptoms subsided. Literature review revealed only 18 cases of Morgagni hernia with Down syndrome reported till date, with age of presentation varying from neonatal age group to 12 years of age. The mode of presentations varied from asymptomatic detection to severe respiratory distress. The possible mechanism of association and its clinical implication has been discussed. The case emphasises a need for diaphragmatic hernia to be looked for as a possible cause of respiratory distress in Down syndrome.
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PMID:Morgagni hernia with Down syndrome: a rare association -- case report and review of literature. 1183 21

This case report describes the delayed presentation of bilateral Morgagni's hernia in a 13-month-old girl with Down's Syndrome. The report emphasizes the fact that a previously normal chest x-ray should not preclude the diagnosis of Morgagni's hernia even when bilateral. The various presentations and the association between Morgagni's hernia and Down's Syndrome are also discussed.
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PMID:Delayed presentation of bilateral Morgagni's hernia in a child with Down's Syndrome. 1193 6

The majority of congenital diaphragmatic hernias (CDH) occur through the foramen of Bochdalek; herniation through the foramen of Morgagni (MH) is rare. Fifteen children (12 males and 3 females) with congenital MH (7 right, 3 left, 5 bilateral) were treated over a period of 15 years, comprising 11% of a total of 135 children with different types of CDH. The majority (12, 80%) had repeated chest infections. In 1 the hernia was discovered accidentally during evaluation of trauma, and another presented in the neonatal period with acute respiratory distress (ARD). The diagnosis was made on plain lateral chest radiograph when there was anterior herniation of bowel loops, and in these cases the diagnosis was confirmed by barium enema. Computed tomography was useful in preoperative diagnosis when the hernia contents were solid. All patients were operated upon transabdominally except 1, and in all cases there was a hernia sac. Associated anomalies were present in 10 (66.7%) patients: 4 (26.7%) had malrotation, 4 (26.7%) congenital heart disease, and 3 (20%) Down's syndrome. Our study shows a relatively high frequency of MH in our patients. MH rarely presents in the neonatal period, but when it does, it causes ARD. The majority of patients with MH present beyond the neonatal period with repeated attacks of chest infection, and although late-presenting MH is relatively benign, it nevertheless causes significant morbidity. Thus, clinical awareness and early diagnosis and surgical treatment are important factors.
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PMID:Herniation through the foramen of Morgagni: early diagnosis and treatment. 1195 70


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