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

Bony anomalies encountered in the 'no neck' form of Klippel-Feil syndrome (KFS) are a wide, short, fused, bifid, retroflexed spinal canal; craniolacunia, cranium bifidum, and acrania. The only symptom may be mirror movement (MM). The CNS anomalies are hindbrain hernia, hydrocephalus, hydromyelia, syringomyelia, meningocele, myelocele, encephalocele, and anencephalus. In severe KFS, i.e. iniencephalus (IN) and in anencephalus (AN), the inion is in contact with the back. In both there is hindbrain hernia and the left thorax may contain the stomach tethered to an anterior spina bifida. KFS results from distortion of somites by an overdistended neural tube. A neural tube that fails to close cannot overdistend.
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PMID:Klippel-Feil syndrome, iniencephalus, anencephalus, hindbrain hernia and mirror movements: overdistention of the neural tube. 45 10

Definitions are offered for the concepts of sterility and infertility. The notion of infertility is expanded to cover not only habitual abortions (three or more miscarriages) but, as well, deliveries of defective children. --Five-hundred and fifty-five pregnancies so far have occurred to 240 infertile probands, among them 78 with habitual abortions and 162 with damaged children. Only one in ten of those pregnancies resulted in clinically intact live birth. --Abortion was the result of 95 per cent of all pregnancies of the women with habitual abortion. The same applied to 25 per cent of all pregnancies of the above women with defective children (e.g. Down's syndrome, neural tube defects, diaphragmatic hernia, hydrocephalus, and progressive muscular dystrophy). A damaged child was born in more than 50 per cent of the latter pregnancy cases. --The number of children born by 162 women has been 181, with only 96 of them alive. The causality relationship between abortion and birth of defective children is discussed. --Infertile women should be given special attention, before conception takes place, and they should be kept under intensive care to the end of pregnancy.
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PMID:[Infertility--a risk factor in obstetrics (author's transl)]. 53 55

The use of computer tomography and myelography with amipaque is of considerable value for establishing the diagnosis of the Arnold-Chiari syndrome. The cerebral lesion (hydrocephalus) can be clearly demonstrated, together with a hernia of the amygdalae, IVth ventricle abnormalities and the size and contents of any myelomeningocele present.
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PMID:[Neuroradiological investigations in the Arnold-Chiari syndrome. Value of computer tomography and myelography with amipaque (author's transl)]. 53 61

A case of asplenia with the assoicated congential defects of a diaphragmatic hernia, hydrocephalus, anophthalmia, and dysostosis of the frontal skull bones is reported. A small ventricular septal defect was also present. The case is discussed with particular emphasis on the embryologic malformations.
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PMID:Asplenia associated with a congenital diaphragmatic defect and neurologic anomalies. 71 99

Ten infants with spina bifida cystica were investigated during life by simultaneous ventricular and intraspinal CSF pressure recordings, and 11 post-mortem specimens were studied by means of crystic resin castings of the ventricles. The results indicate marked pressure differentials between the lateral ventricles and intraspinal CSF pathways, which contribute to the moulding of the Arnold-Chiari malformation as a sliding hernia. Similar pressure differentials may exist at the incisura, with moulding of tissues producing blockage of the aqueduct and the subarachnoid pathways. A valvular action was demonstrated, in which baseline pressure differences were exaggerated by the infant's straining (e.g. crying, sucking), and it seems that complete dissociation between the two pressures may develop as a result of periods of partial dissociation. Removal of the meningocele sac increased the peaks in recordings of intraspinal pressure produced by straining. If it is carried out when there is a valvular effect, it seems that it will hasten the onset of established ventriculo-spinal pressure dissociation and uncompensated hydrocephalus by leading to impaction of the Arnold-Chiari malformation in the foramen magnum. The results indicate that even after birth the CSF pathways of babies with spina bifida cystica are in a plastic and changeable condition, with competition between constricting and distending forces. The demonstration that pressures tend to be low in the spine and to become lower before exacerbation of hydrocephalus suggests that all the intracranial manifestations of spina bifida cystica may be due to low intraspinal pressure, which results from initial failure of the neural tube to close during embryogenesis.
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PMID:Cerebrospinal fluid pressure-gradients in spina bifida cystica, with special reference to the Arnold-Chiari malformation and aqueductal stenosis. 110 12

The concept that the fetus may be a patient is very recent. Modern ultrasonography can detect fetal abnormalities early in utero, and lead to prenatal correction of such malformations. At present three anatomic defects can benefit from fetal surgery: diaphragmatic hernia, urethral obstructions and obstructive hydrocephalus. The feasibility of fetal surgery has been proved both experimentally and clinically. Microsurgery can be of great help in this type of surgery. At this stage, fetal surgery should be pursued only in very qualified centers.
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PMID:[Fetal microsurgery. The past and present of a frontier of surgery]. 148 43

The paper is concerned with mental disorders in 82 patients aged 3 to 14 years with a cerebrospinal hernia located primarily in the lumbosacral part of the spine. The majority of children and adolescents with relatively preserved mental development (trained in accordance with the routine school program) manifested borderline disorders that correlated in 90% of cases with the symptoms of compensated hydrocephalus and psychogenic (reactive) formations-- astheno-depressive conditions, reactions and others due to the feeling of physical deficiency (motor insufficiency and disorders of pelvic functions).
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PMID:[Mental disorders in children with congenital meningomyelocele]. 166 25

Eighteen patients with a typical clinical picture of syringomyelia were examined by a complex of methods including magnetic resonance tomography (MRT) (T1-suspended tomograms of sagittal and transverse section). MRT made it possible to recognize in 12 patients an occlusive type of the disease with a distinctly visualized expanded cyst against the background of hernia of the cerebellar tonsils and hydrocephalus. A gliomatous variant (with multiple small cysts or without such, without the Arnold-Chiari malformation) was found in 6 patients. The MRT method allows simultaneous determination of the localization and size of the cyst, ectopia of the cerebellar tonsils, and hydrocephalus, which makes it very valuable in considering the indications for and tactics of operative treatment. MRT brings the diagnosis closer to the pathologicoanatomical one and may be used successfully in studying the clinical picture and pathogenesis of various types of syringomyelia.
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PMID:[The magnetic resonance tomographic method in the diagnosis of syringomyelia]. 166 38

The causes of congenital hydrocephalus vary widely and have an important effect in determining the future counseling of affected cases. We analyzed the postmortem findings of 49 autopsies diagnosed as hydrocephalus to find the causes and related conditions. The cases were collected during the last 10 years (1981-1990). The cases consisted of 25 cases associated with various congenital malformations of the central nervous system (CNS), 14 cases of posthemorrhagic hydrocephalus, 4 cases of postinfection hydrocephalus, and 2 cases of hydranencephaly. Four cases were associated with supracerebellar arachnoid cyst (1) and unknown causes (3). Twenty-five cases associated with congenital malformation of the CNS consisted of 10 cases of holotelencephaly, 5 cases of stenosis of the Sylvian aqueduct, 4 cases of agenesis of the corpus callosum, and 3 cases each of Dandy-Walker malformation and Arnold-Chiari malformation. Various malformations of other organs were associated with these cases. Cardiovascular malformations were common, consisting of 11 cases of patent ductus arteriosus (PDA), 7 cases of atrial septal defect (ASD), and 6 cases of patent foramen ovale (PFO). Among the anomalies of the respiratory system, abnormal lobation was commonly associated, as well as hypoplasia of the lung. Gastrointestinal malformations included Meckel's diverticulum, diaphragmatic hernia, mobile intestine, and midline small liver. Cryptorchidism was the most common malformation in the genitourinary system. Holotelencephaly cases showed multiple craniofacial anomalies, as well as other malformations in the central nervous system. Skeletal malformations included polydactyly, simian crease, and flexion deformity. There were no specific constellations of malformations in these different groups of CNS malformations associated with hydrocephalus.
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PMID:Congenital hydrocephalus--analysis of 49 cases. 184 37

Prenatal sonographic findings were reviewed in 30 fetuses with a single umbilical artery (SUA) to determine the reliability of ultrasound for detecting concurrent anomalies. Additional anomalies were identified in 15 fetuses, including 3 fetuses with minor anomalies and 12 fetuses with major or multiple concurrent anomalies. Minor anomalies observed in 3 fetuses included 1 case each of pelvic kidney, unilateral absent kidney, and mild cerebral ventricular dilatation. Major abnormalities detected in 12 fetuses involved a variety of organ systems and included cardiac defects, holoprosencephaly, skeletal dysplasia, hydrocephalus, omphalocele, hydrothorax, enlarged cisterna magna, and diaphragmatic hernia. Clinical and pathologic correlation showed that all fetuses were correctly categorized regarding the presence of other anomalies; none of the 15 fetuses in whom an SUA was considered an isolated finding had a significant anomaly at birth. Chromosome abnormalities were found in 6 of 12 fetuses with major abnormalities but in none of the 18 remaining fetuses. We conclude that prenatal ultrasound can reliably identify major concurrent anomalies in fetuses with SUA. In the absence of additional anomalies, prenatal detection of SUA should not alter obstetric management.
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PMID:Single umbilical artery. Prenatal detection of concurrent anomalies. 205 44


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