Cases reported "Meningomyelocele"

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1/240. Intraspinal epidermoid cyst occurring 15 years after lipomyelomeningocele repair. Case report.

    The authors report the case of a spinal epidermoid cyst that developed in a patient who had undergone surgery for lipomyelomeningocele repair 15 years earlier. The patient presented with symptoms of retethering. magnetic resonance imaging revealed a cystic intraspinal mass that extended from L-2 to L-5. The mass proved to be an epidermoid cyst. Spinal epidermoid cysts can cause retethering after a repair of lipomyelomeningocele, and the risk of this development can be present for decades.
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keywords = myelomeningocele
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2/240. A case of cerebrospinal fluid eosinophilia associated with shunt malfunction.

    A 3-month-old female patient presented with a meningomyelocele at the lumber region associated with congenital hydrocephalus. She underwent ventriculoperitoneal (V-P) shunt surgery using the Sophy system. The shunt system was replaced due to a malformation. Following replacement, the patient presented with cerebrospinal fluid (CSF) eosinophilia at the age of 8 months. The eosinophilic granulocytosis of the CSF improved dramatically following systemic prednisolone administration. CSF eosinophilia without accompanying inflammation or pyrexia in the present case may have resulted from an allergic response to a foreign material such as the silicone tube pressure valve of the Sophy system or the sutures rather than bacterial or fungal infection. Based on our results, we believe that some patients may experience CSF eosinophilia following postoperative V-P shunt due to an allergic reaction to the shunt equipment. Prompt steroid treatment can produce spontaneous regression in such cases.
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ranking = 0.45718231157094
keywords = meningomyelocele
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3/240. Migration of the abdominal catheter of a ventriculoperitoneal shunt into the scrotum--case report.

    A 3-day-old male neonate presented with migration of the ventriculoperitoneal (VP) shunt tip through the patent processus vaginalis resulting in scrotal hydrocele. The association of myelomeningocele with hydrocephalus may have been a predisposing factor in this rare complication. Development of scrotal swelling or hydrocele in a child with VP shunt should be recognized as a possible shunt complication.
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ranking = 0.16666666666667
keywords = myelomeningocele
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4/240. An unexpected complicationof the remote-controlled intraurethral valve pump for urinary incontinence.

    We report an unexpected complication of the remote-controlled intraurethral valve pump in a patient with neurogenic bladder. A meningomyelocele patient with an atonic bladder received an intraurethral valve pump. Acute urinary retention was caused by a mucus clot obstructing the pump. Prior to extended clinical use, the remote-controlled intraurethral valve pump should be evaluated in prospective multicenter studies.
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ranking = 0.45718231157094
keywords = meningomyelocele
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5/240. Anomalous ependyma inducing split cord and meningomyelocele?

    The case is that of a female fetus of 17 to 18 weeks' gestation with major defects of the central nervous system: (1) The thoracic vertebrae demonstrated rachischisis, with segmental diplomyelia; the duplicated cords were dissimilar in size and lay side by side within a single meningeal sheath lacking a dividing septum or spur. Cranially to the divided cord lay an unsplit segment of "open cord" lacking the posterior elements and exposing the centrally placed ependyma of the central canal flanked by glial and epidermal lining, respectively; it could be regarded as an example of a meningomyelocele. (2) Heterotopic massed ependymal cells, some of which were actively proliferating, were associated with the choroid plexus in the brain. Minor anomalies included cerebellar heterotopia and the malpositioning of dorsal root ganglia outside the meningeal sheath. Because the ependyma is such a powerful inducer of the development of neighboring tissue, the findings could be united by a common pathogenic theme, viz problematic ependymal development and migration within both the brain and spinal cord. The causative agent responsible for these abnormalities remains unidentified, but the balance of evidence suggests that its effect was felt during the second week of postconceptual age.
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ranking = 2.2859115578547
keywords = meningomyelocele
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6/240. Once a shunt, always a shunt?

    Over a period of 15 years, during which some 1000 operations were performed, CSF-drainage systems were able to be removed in 40 of a total of 444 children with communicating hydrocephalus or hydrocephalus associated with myelomeningocele. Shunts were removed from 26 children in the former group, of whom 17 continued to be compensated; in the latter group there were 14 removals, all of whom remained compensated. It is stressed that true compensation can be proven only by continuous measurement of ventricular fluid pressure over periods of several hours or at intervals of days or weeks, and four cases are discussed in which decompensation occurred, in two cases with fatal results.
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ranking = 0.16666666666667
keywords = myelomeningocele
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7/240. Combined anomaly of intramedullary arteriovenous malformation and lipomyelomeningocele.

    We report a rare situation in which a lipomyelomeningocele and an intramedullary arteriovenous malformation (AVM) occurred together at the T11-L1 level in a 44-year-old man. MR images showed a hypervascular lesion intradurally and a fatty component extradurally. Spinal angiography revealed this lesion to be an intramedullary AVM with multiple feeding arteries from the right T12 and left T10 intercostal artery and the left L1 lumbar artery, drained by tortuous, dilated, perimedullary veins.
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ranking = 0.83333333333333
keywords = myelomeningocele
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8/240. Vesicostomy in adult meningomyelocele patients. Reappraisal of an old technique.

    Intermittent catheterization is the standard treatment of neurogenic bladder dysfunction. In adult meningomyelocele patients, self-catheterization is not always possible or is not accepted. In these patients, urinary diversion is frequently hampered by anatomical abnormalities, preventing the use of bowel. Vesicostomy as a safe procedure in selected adult patients was employed at our department.
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ranking = 2.2859115578547
keywords = meningomyelocele
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9/240. Inverted duplication of the distal short arm of chromosome 3 associated with lobar holoprosencephaly and lumbosacral meningomyelocele.

    A fetus with lobar holoprosencephaly and lumbosacral meningomyelocele associated with duplication of the short arm of chromosome 3 is reported. The anomalies were detected on fetal ultrasound at 20 weeks' gestation and the autopsy findings correlated well with the prenatal findings. The fetal karyotype was 46,XY,der(3)del(3)(p26) dup(3)(p26p21.3). The association of holoprosencephaly with duplication 3p is well known, but to the best of our knowledge this is the first reported association of meningomyelocele with 3p duplication. These findings suggest that a gene or genes with a crucial role in central nervous system development are located on the short arm of chromosome 3.
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ranking = 2.7430938694256
keywords = meningomyelocele
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10/240. neural tube defects and the 13q deletion syndrome: evidence for a critical region in 13q33-34.

    neural tube defects (NTD) are common findings in the 13q deletion syndrome, but the relationship between the 13q- syndrome and NTDs is poorly understood. We present a child with a 13q deletion and lumbosacral myelomeningocele. This was a boy with microcephaly, telecanthus, minor facial anomalies, and ambiguous genitalia. Cytogenetic and fluorescence in situ hybridization analysis showed a de novo 46,XY,del(13)(q33.2-->qter) with no visible translocation. By using microsatellite markers, the deletion breakpoint was mapped to a 350-kb region between D13S274 and D13S1311 and was paternal in origin. An analysis of 13q deletions with NTDs, including the present case, suggests that a deletion in 13q33-34 is sufficient to cause an NTD. The deletions associated with NTDs are distal to and nonoverlapping with the previously defined critical region in 13q32 for the major malformation syndrome [Brown et al., 1999: Am J Hum Genet 57: 859-866]. Our analysis also suggests that one or more genes in 13q33-34 produces NTDs by haploinsufficiency.
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ranking = 0.16666666666667
keywords = myelomeningocele
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