1/147. 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.- - - - - - - - - - ranking = 1keywords = spina, spinal (Clic here for more details about this article) |
2/147. 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.- - - - - - - - - - ranking = 0.83333333333333keywords = spina, spinal (Clic here for more details about this article) |
3/147. The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention.OBJECTIVE: To determine how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and to determine if surgical intervention influences outcome in these patients. methods: A chart review of the 220 patients who attend a clinic for adults with spina bifida and hydrocephalus (CASBAH), and follow-up of the five cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. RESULTS: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. CONCLUSION: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.- - - - - - - - - - ranking = 1.8486925310287keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
4/147. Segmental costovertebral malformations: association with neural tube defects. Report of 3 cases and review of the literature.patients with spondylocostal dysostosis (SCD) have vertebral abnormalities and numerical or structural rib anomalies that produce thoracic asymmetry. Rib anomalies and dysmorphism are the typical features that differentiate this syndrome from spondylothoracic dysostosis (STD). Jarcho-Levin syndrome is a severe form with involvement of the whole vertebral column. Other associated findings such as congenital heart defects, abdominal wall malformations, genitourinary malformations and upper limb anomalies may be found; in addition, neural tube defects (NTDs) have been associated with this malformation. SCD is transmitted both in a recessive form and as a dominant defect. We report on 3 children with SCD; 2 also had NTDs. All of them were studied with x-rays and spinal magnetic resonance (MR), and over the same period they underwent multidisciplinary clinical functional evaluation. One of our cases with NTD also presented polythelia, which has not previously been described in patients with SCD. The common association of segmental costovertebral malformations with NTDs could be related to an early gastrulation genomic defect, or one after gastrulation, when there are two independent somitic columns. The latter sometimes progresses and then involves primary and secondary neurulation. Also, the association of SCD with NTDs could be related to the interaction of different genes, resulting in this complex phenotype. Therefore, additional genetical and embryological studies are necessary to provide evidence of an etiological link between SCD and NTD.- - - - - - - - - - ranking = 0.16666666666667keywords = spina, spinal (Clic here for more details about this article) |
5/147. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = spina, spinal (Clic here for more details about this article) |
6/147. Myelocystocele-cloacal exstrophy in a pedigree with a mitochondrial 12S rRNA mutation, aminoglycoside-induced deafness, pigmentary disturbances, and spinal anomalies.A large Filipino-American family with progressive matrilineal hearing loss, premature graying, depigmented patches, and digital anomalies was ascertained through a survey of a spina bifida clinic for neural crest disorders. deafness followed a matrilineal pattern of inheritance and was associated with the A1555G mutation in the 12S rRNA gene (MTRNR1) in affected individuals as well as unaffected maternal relatives. Several other malformations were found in carriers of the mutation. The proband had a myelocystocele, Arnold-Chiari type I malformation, cloacal exstrophy, and severe early-onset hearing loss. Several family members had premature graying, white forelock, congenital leukoderma with or without telecanthus, somewhat suggestive of a waardenburg syndrome variant. In addition to the patient with myelocystocele, two individuals had scoliosis and one had segmentation defects of spinal vertebrae. The syndromic characteristics reported here are novel for the mitochondrial A1555G substitution, and may result from dysfunction of mitochondrial genes during early development. However, the mitochondrial A1555G mutation is only rarely associated with neural tube defects as it was not found in a screen of 218 additional individuals with spina bifida, four of whom had congenital hearing loss.- - - - - - - - - - ranking = 4.5307183953908keywords = spina bifida, bifida, spina, spinal (Clic here for more details about this article) |
7/147. Detection of skin over cysts with Spina bifida may be useful not only for preventing neurological damage during labor but also for predicting fetal prognosis.Spina bifida is one of the most common open neural tube defects. There are two common types of spina bifida cystica, myelomeningocele and meningocele. Special attention to the thickness of the cystic sac (presence of intact skin and subcutaneous tissue) on magnetic resonance imaging is advantageous for determination of whether the child will profit from cesarean section in order to prevent neurological change (infection and drying of nerve tissue) and for management of spina bifida (most meningocele) during the perinatal period. Furthermore, skin detection may help to predict the prognosis of spina bifida after birth. meningocele, with intact skin over the cyst, has a better clinical course than myelomeningocele. Some myelomeningoceles with neural tube defects in a lower position, also frequently having an intact skin over the cyst, have almost the same clinical course as a meningocele. From this, we hypothesize that a baby with spina bifida who has intact skin over the cyst might have a good prognosis neurologically. In this report, we concentrate attention on the skin over cysts in 3 cases (1 meningocele and 2 myelomeningoceles).- - - - - - - - - - ranking = 11.276658509678keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
8/147. Terminal Myelocystocele:an unusual presentation.Terminal myelocystocele is an unusual form of occult spinal dysraphism. It consists of a cystic dilatation of a low-lying terminal cord herniated posteriorly through a skin-covered lumbosacral spina bifida. An arachnoid-lined meningocele, continuous with the spinal subarachnoid space, is traversed by the hydromyelic cord. Clinically, this presents with a skin-covered lumbosacral mass, but often no neurological deficit is present. We present a case of terminal myelocystocele in a child born without deficit and without an obvious back mass. diagnosis was delayed until sphincter disturbance and lower limb inequalities developed. We discuss the presentation, imaging and operative findings in this case.- - - - - - - - - - ranking = 2.1820258643621keywords = spina bifida, bifida, spina, spinal (Clic here for more details about this article) |
9/147. gait analysis in myelomeningocele: possibilities and applications.gait analysis with a fully integrated laboratory is a relatively new instrument in the armamentarium of the pediatric orthopedic surgeon. The introduction of it has been especially successful in neuromuscular pathology and, particularly, in cerebral palsy. In spina bifida, however, it also enhances substantially the possibilities of detailed analysis of the locomotion problem. It is, furthermore, a very useful instrument in evaluating treatment and in follow-up. With a few examples, this paper tries to show the possibilities and advantages of a gait laboratory in the evaluation of patients with ambulatory spina bifida.- - - - - - - - - - ranking = 3.6973850620575keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
10/147. Delayed CSF pseudocyst following shunt malfunction after myelomeningocele repair.We report a case of delayed cerebrospinal fluid (CSF) pseudocyst following shunt malfunction in a 20-year-old patient with myelomeningocele. Magnetic resonance (MR) images and a radioimmunoassay shunt-gram detected the CSF fistula at the old scar of the myelomeningocele repaired 20 years before. Since the revision of the shunt system failed to keep the pseudocyst, the lesion was successfully directly repaired. Treatment of the delayed CSF pseudocyst following shunt malfunction thus requires a radical operation of the lesion.- - - - - - - - - - ranking = 0.16666666666667keywords = spina, spinal (Clic here for more details about this article) |
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