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

Fifty-eight patients with occipital encephalocoeles were retrospectively examined. These comprised about one-half of the cases seen and evaluated for treatment at the University College Hospital, Ibadan, Nigeria, between January 1973 and December 1987. There was a female-to-male preponderance of 2:1. Of the patients 91% were treated during infancy. Only one patient was precluded from surgery because his large ulcerated lesion was associated with severe microcephaly and neonatal sepsis, to which he succumbed. About four-fifths of the lesions exceeded 5 cm in diameter. The operative mortality was 6%, all deaths occurring in patients who were neonates at the time of surgery and whose hernia sacs contained brain substance. Clinically apparent hydrocephalus was more frequently encountered postoperatively, than preoperatively. Developmental delay was apparent in 5 of the 13 patients in whom developmental milestones were assessed during follow-up. For most patients, the follow-up period was short, possibly a reflection of the poor prognosis of the disease.
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PMID:Occipital encephalocoeles in 57 Nigerian children: a retrospective analysis. 234 May 37

Three large and comparable series of births were used to test the working hypothesis that if there is a real seasonal variation in the frequency of a given congenital malformation; it would have to be shown by adequate analysis; to be more overt in non-tropical areas; and to be six months out of phase in northern and southern hemispheres. The data set were hospital births from tropical (287,165 births) and non-tropical (582,585 births) South America, and from Italy (508,536 births). Sixteen well-defined malformation types were tested: anencephaly, spina bifida, cephalocoele, hydrocephaly, microtia, cleft palate, cleft lip, oesophageal atresia, anal atresia, hypospadias, pes equino-varus, pes talovalgus, postaxial polydactylyl, pre-axial polydactylyl, diaphragmatic hernia, and Down's syndrome. No seasonal variation was proven (p less than 0.01) for any malformation type in any of the three series of data by means of Walter and Elwood's test, or Hewitt et al's non-parametric test2 applied to seven instances with sample sizes smaller than 50 cases. Variations of borderline significance (p less than 0.05) included oesophageal atresia in tropical South America, none in non-tropical South America, and anencephaly in Italy. It is concluded that seasonal variation in the occurrence of congenital malformations is a rare phenomenon when tests are strictly used within their recommended limitations.
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PMID:Monthly and seasonal variations in the frequency of congenital anomalies. 237 54

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

The records of the B.C. Health Surveillance Registry were used to analyze all live births with spina bifida and hydrocephalus (SBHC) in British Columbia between 1952 and 1986 inclusive. A total of 479 cases (218 males and 261 females) occurred during this period in 1,298,267 consecutive live births, for an incidence of 3.7/10,000. There were more females, with the sex ratio being significantly different from that of the general population born over this period. No significant seasonal differences were observed over the time period. A comparison of life expectancy for individuals born 1962-1970 and 1970-1986 showed significant improvement in the probability of surviving to the age of 1 year for the latter group. There was also a small but statistically significant increased chance of surviving to age 7 years in the latter group but no difference in the probability of surviving from 7 years to 16 years. Life expectancy figures are shown in a format practical for counseling with regard to prognosis for affected individuals. Additional malformations not attributable to SBHC were observed in 6% (27 cases). These included renal anomalies, cleft lip and/or palate, tracheoesophageal fistula, and diaphragmatic hernia. The incidence of each defect was significantly greater than in the general population of births.
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PMID:Spina bifida and hydrocephalus: a population study over a 35-year period. 264 14

Accurate fetal diagnosis became possible by the steadily increasingly complex techniques of amniocentesis, ultrasound, and ultrasound-guided fetal blood sampling and chorion villous sampling. A high degree of diagnostic accuracy for a wide variety of structural and metabolic anomalies is required. The field of fetal diagnosis has been extended to the point that a journal dedicated to this subject alone is a viable proposition. It is becoming apparent, however, that lesions that were well known and well understood when recognized in neonatal life appear in general to have a worse prognosis if the lesion is diagnosed in utero. Fetal surgery began with attempts to perform in utero transfusions for babies with erythroblastosis fetalis. For a while, there was competition between open surgical procedures and the percutaneous placement of blood through catheters introduced into the fetal peritoneal cavity from outside the mother's abdomen. For fetal transfusion, closed techniques proved far safer and just as efficacious. There has been a worldwide interest in shunting of hydrocephalus and obstructive uropathy. The results of shunting hydrocephalus have been disappointing, with most of the patients surviving, but most of the survivors being severely handicapped. The results of shunting obstructive uropathy were that only about 50 per cent of the babies survived, but it appeared that those that did survive did well. Other lesions that have been shunted have been hydrothoraces or fetal ascites. A limited number of open procedures have been carried out in the last few years in San Francisco, and it may well be that diaphragmatic hernia (in appropriately selected patients) will be a lesion that can be corrected by in utero surgery. The future of this field is exciting, but before this form of treatment becomes routine, the ethical implications of the possibility of fetal surgery must be defined much more clearly than is currently the case.
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PMID:Fetal diagnosis and fetal surgery. 265 57

Magnetic resonance imaging (MRI) has been used sparingly in obstetrics. The reasons for this relate mainly to cost, availability, difficulty obtaining clear images because of fetal movement, and the convenience and utility of ultrasound. However, MRI use is expanding and has the potential to play an important role in selected problem pregnancies. We studied the pregnancies of five women whose fetuses showed anomalies by ultrasound. These included cases of a large body wall defect, a diaphragmatic hernia, hydrocephalus, Meckel-Gruber syndrome, and iniencephaly with a diaphragmatic hernia (the iniencephaly sequence). Three of these examinations followed fetal neuromuscular blockade, and two were associated with oligohydramnios without fetal paralysis. Paralysis provided superior images. The fetal central nervous system, subcutaneous tissue, and liver imaged particularly well. This study illustrates that MRI can enhance and even clarify certain information provided by ultrasound.
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PMID:Magnetic resonance imaging of anomalous fetuses. 265 26

Analysis of skin fibroblast cultures from a deformed stillborn female Alaskan Malamute pup revealed a 3n = 117,XXX (normal, 2n = 78,XX) chromosome count. The triploid pup was delivered by cesarean section 5 days after estimated date of parturition, because the bitch failed to start labor. The bitch had been inseminated with thawed frozen semen deposited into the lumen of the uterus approximately 4 days after ovulation. Gross anatomic abnormalities of the pup included omphalocele, diaphragmatic hernia, persistent right aortic arch, atresia ani, and no right forelimb distal to the scapula. The pup also had arthrogryposis of the left carpus, kyphosis of the thoracic vertebrae, widely separated cranial sutures, open fontanelles, hydrocephalus, and cleft palate. Suspected cause of the triploidy was dispermy of an aged oocyte after intrauterine deposition of the thawed frozen semen. Numeric chromosome abnormalities may be causes of fetal deformity or death that can be detected by fetal karyotype.
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PMID:Triploidy (117,XXX) in a stillborn canine pup conceived with frozen semen. 272 39

A case of giant left inguinal hernia in a 5-year-old boy is presented. The patient had multiple neurosurgical procedures performed in the neonatal period for spina bifida and hydrocephalus, including the placement of a ventriculoperitoneal shunt. The hernia was first noted during this period but was not repaired, and the child was lost to follow-up until age 5. The hernia underwent progressive enlargement over this interval, and the eventual development of gastrointestinal symptoms prompted the "rediscovery" of the defect. The majority of the child's intestines were within the hernia, with at least partial loss of domain. The unique preoperative and postoperative management of this difficult problem is described.
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PMID:Giant inguinal hernia in a 5-year-old boy with hydrocephalus: a case report. 280 99

Syringomyelia management is showing some progressive improvements following surgical methods of investigation and treatment. Investigation of simultaneous pressure changes in the cerebrospinal fluid pathways has illustrated the importance of craniospinal pressure dissociation in impacting the cerebellar and medullary tissues in the foramen magnum in hindbrain related syringomyelia. Such pressure differences may be referred to as 'suck' and similar changes are to be found in non-hindbrain related forms of syringomyelia such as those associated with spinal arachnoiditis. When cavities have formed then impulsive movements may occur with them and enlargement of the cavities may be continued by sloshing of the fluid within them. Investigations have been improved following the widespread use of water soluble contrast media and CT scanning with reconstructions after myelography. A definite relationship between birth injury and hindbrain related syringomyelia has been established especially with cases showing arachnoiditis. The nature of the relationship to hindbrain hernia and basilar invagination remains unclear. Magnetic resonance imaging holds great promise particularly in showing hindbrain deformation in new-born babies, showing whether or not a communication commonly exists between the fourth ventricle and the cavities within the spinal cord in early childhood and also in outlining the changes in the spinal cord in the presence of acute traumatic paraplegia. Treatment still relies upon valved ventricular to extrathecal shunts for hydrocephalus, cranio-vertebral decompression to prevent suck and drainage of the syrinx in appropriate cases. Syrinx to extrathecal shunting may be preferred to shunts to the subarachnoid space. The peritoneum and the pleura are favoured sites and a valve is not necessary. The advances for the future may depend on earlier diagnosis and greater understanding of the mechanisms of pathogenesis in which MRI seems likely to play an increasingly important part.
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PMID:Progress in syringomyelia. 287 6

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


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