1/170. Extraspinal dural arteriovenous fistula in a patient with lipomyelodysplasia: value of MRI and MRA.Spinal dural arteriovenous fistulae are extremely rare in spinal dysraphism. A fistulous malformation within a lipomyelomeningocele has not been reported previously. A 50-year-old man presented with progressive paraparesis and bladder dysfunction. MRI revealed a large lumbar lipomyelomeningocele. A vascular malformation was indicated by abnormal signal in the thoracolumbar spinal cord and dilated perimedullary veins. Phase-contrast MRA demonstrated only the slow-flow veins of the fistula and an intradural ascending vein. Contrast-enhanced ultra-fast MRA gave excellent delineation of all parts of the fistula within the dysraphic lesion.- - - - - - - - - - ranking = 1keywords = spina, spinal (Clic here for more details about this article) |
2/170. 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) |
3/170. spinal cord tethering associated with amniotic band syndrome.amniotic band syndrome (ABS) comprises fetal morphological abnormalities that may be associated with fibrous amniotic bands that damage developing fetal parts resulting in cutaneous scars, erosions and ulcerations, digital constricting bands, craniofacial and visceral anomalies. Multiple asymmetric encephaloceles and anencephaly are neural-tube-like defects previously reported with ABS. This is the first report of spinal dysraphism with dorsal spinal cord tethering associated with ABS. We examine the pathogenetic theories of ABS in light of this report.- - - - - - - - - - ranking = 0.33333333333333keywords = spina, spinal (Clic here for more details about this article) |
4/170. A case report of caudal regression syndrome associated with an intraspinal arachnoid cyst.We report here a rare case of caudal regression syndrome associated with an intraspinal arachnoid cyst. The patient was a 6-month-old baby girl with multicomplex congenital abnormalities: sacrococcygeal dysgenesis and ventral curvature, large terminal cyst (myelocystocele), spinal arachnoid cyst, cerebellar hypertrophy (suspected), high imperforate anus, partial dysgenesis of the large intestine, omphalocele, atresia of the vagina, bilateral incomplete ureter duplication, incomplete pseudoduplicated bladder and bilateral talipes equinovarus. We performed plastic repair of the myelocystocele and perineal lesion for caudal regression syndrome and partial removal of the cyst wall for the intraspinal arachnoid cyst. She has been well for 3 years postoperatively, and her mental development is normal.- - - - - - - - - - ranking = 1.1666666666667keywords = spina, spinal (Clic here for more details about this article) |
5/170. Myolipoma in a tethered cord. Case report and review of the literature.The intradural myolipoma is a very rare tumor, consisting of fully differentiated striated muscle fibers mingled with fat. Only four previous cases have been identified. The authors present a case in which this tumor was associated with a symptomatic tethered spinal cord in an 18-year-old man.- - - - - - - - - - ranking = 0.16666666666667keywords = spina, spinal (Clic here for more details about this article) |
6/170. Prenatal ultrasound evaluation of fetal diastematomyelia: two cases of type I split cord malformation.Isolated diastematomyelia is a rare form of spinal dysraphism characterized by a sagittal cleft in the spinal cord, conus medullaris and/or filum terminale with splaying of the posterior vertebral elements. This condition is the result of the presence of an osseous or fibrocartilaginous septum producing a complete or incomplete sagittal division of the spinal cord into two hemicords. It may be isolated or associated with other segmental anomalies of the vertebral bodies. prenatal diagnosis of this anomaly is possible in the early midtrimester by sonography, thus allowing for early surgical intervention and a favorable prognosis. Two cases of fetal diastematomyelia diagnosed by prenatal sonography are presented, each demonstrating the typical sonographic features diagnostic of this condition. The first case, detected at 28 weeks' gestation, presented with disorganization of the bony processes of the vertebral column with a midline echogenic focus. The second fetus, diagnosed at 17 weeks' gestation, had a similar appearance with widening of the posterior elements and the presence of a midline echogenic bony spur. Postnatally, both infants underwent magnetic resonance imaging for a definitive diagnosis. Surgical repair of the defect was performed in the neonatal period in both cases.- - - - - - - - - - ranking = 0.5keywords = spina, spinal (Clic here for more details about this article) |
7/170. Spinal neurenteric cyst presenting in infancy with chronic fever and acute myelopathy.The authors describe the clinical, radiologic, and pathologic features of a neonatal spinal neurenteric cyst (NC) presenting with long-lasting fever and acute myelopathy, and compare this observation with other infants reported in the literature. This observation shows that NC must be considered in the differential diagnosis of acute myelopathy with persistent fever in infancy. fever is attributed to degenerative changes in the NC, triggering inflammatory cell infiltration and tumor necrosis factor alpha secretion.- - - - - - - - - - ranking = 0.16666666666667keywords = spina, spinal (Clic here for more details about this article) |
8/170. Craniocervical neurenteric cyst without associated abnormalities.Spinal neurenteric (NE) cyst is an uncommon congenital cyst and frequently found in the cervical region. The clinical symptoms associated with this entity depend on the site of the lesion and are not typical for all such cysts. A definitive diagnosis can only be made by biopsy and histological examination. MRI can confirm these cystic masses and is the method of choice for their imaging investigation. They are often connected by a fibrous tract, fistula or cleft to structures derived from the primitive gut in the thoracic or abdominal cavities and are commonly associated with anterior spina bifida or other vertebral anomalies. We report a case of craniocervical NE cyst without associated abnormalities and discuss the implications for clinical diagnosis and management by a thorough review of the literature.- - - - - - - - - - ranking = 1.5534442985812keywords = spina bifida, bifida, spina (Clic here for more details about this article) |
9/170. Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report.STUDY DESIGN: This is a report of a rare presentation of a split cord malformation with diastometamyelia. OBJECTIVES: This report draws attention to the fact that the only manifestation of diastmetamyelia in the adult patient may be neurogenic claudication. SUMMARY OF BACKGROUND DATA: patients with split cord malformations and diastometamyelia rarely have symptomatic onset in adulthood. When present, a traumatic event leading to an acute neurologic change is the usual presentation. methods: An adult patient presented with symptoms of neurogenic claudication in the left leg. magnetic resonance imaging examination showed a split cord malformation and diastometamylia at L3-L4 with spinal stenosis of the left hemicord. Decompressive laminectomy and subtotal resection of the bony spur were performed. RESULTS: Two years after decompression, the patient has complete resolution of his leg symptoms and is back to work. CONCLUSIONS: Neurogenic claudication without any objective neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may be the only presentation in the adult with diastometamyelia. decompression to relieve both clinical and radiologic evidence of spinal stenosis obtained excellent outcome.- - - - - - - - - - ranking = 0.5keywords = spina, spinal (Clic here for more details about this article) |
10/170. Primary tethered cord syndrome: diagnosis and treatment of an insidious defect.Failure to recognize the signs and symptoms of tethered cord syndrome in patients with spina bifida occulta can result in tragic consequences. Of patients with tethered spinal cord, 35% have bowel and/or bladder dysfunction. scoliosis, foot or leg length discrepancies, pes cavus, and varus or valgus deformities also can occur. Early assessment and intervention by the neuroscience nurse who is familiar with these clinical signs can help prevent significant deformities and irreversible neurological deficits.- - - - - - - - - - ranking = 20.333317183076keywords = spina bifida occulta, bifida occulta, occulta, spina bifida, bifida, spina, spinal (Clic here for more details about this article) |
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