The orthopaedic history of spinal dysraphism. I: The early history.
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History, 17th Century↗
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Results for “Spinal Dysraphism”
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The surgery of occult spinal dysraphism.
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Adolescent↗
Spinal dysraphism. A case report.
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Ankle↗
Ultrastructural analysis of the midaxial extracellular matrix in spinal dysraphism.
Ultrastructural aspects of the extracellular matrix (ECM) in the midaxial region of dysraphic embryos of the loop-tail (Lp) mutant mouse were analyzed by means of electron microscopy. In 17-23 somite embryos, ultrastructural differences in the ECM occurred with respect to the presence of a pair of long trailing basal laminar strands extending continuously from the ventral notochordal cells to the gut in abnormal (Lp/Lp) embryos, in contrast to short, ragged, discontinuous strands in normal (+/+; Lp/+) embryos. The ultrastructural localization and configuration of fibronectin (FN) and laminin (L) associated with these strands, however, were similar in normals and abnormals. In addition, FN occurred over interstitial bodies, fibrils, and sporadically along the basal laminae of the neural tube (or folds), notochord, gut, and vessels, whereas L was largely confined to the basal laminae. The results indicate that although the ultrastructural pattern of FN and L reactivity are similar in normal and abnormal embryos, a disturbance in the manner whereby the notochord detaches from the gut in dysraphic embryos may be of causal significance in the etiology of dysraphism in this mutant.
Animals↗
Intradural spinal lipoma of the conus medullaris without spinal dysraphism.
A 42-year-old man suffering from progressive left radicular sensory motor loss (L4 level) underwent neurosurgical repair. Neuroimaging (RMI) had led to the diagnosis of schwannoma of the filum terminale with lipomatous component. Histological examination visualized a true mature lipoma associated with numerous bundles of more or less dystrophic nerve fibers. This histological benign tumor raised the problem of the genesis of intradural lipomas of spinal cord.
Adult↗
Spinal dysraphism in achondroplasia.
The neurological complications in achondroplasia are commonly due to spinal canal stenosis. A case with rare coexistence of achondroplasia and dysraphic condition of the spine is presented wherein the neurological problem was presumably due to the latter.
Achondroplasia↗
Variable expressivity of congenital longitudinal radial deficiency and spinal dysraphism in monozygotic twins.
We report monozygotic twins with longitudinal radial hypoplasia and low (L5) spina bifida occulta, but with differing severity. There is only one previous report of similar twins. We report these identical twins with the expression of longitudinal radial hypoplasia with mirror image pattern on contralateral sides, and the association of low spina bifida occulta.
Humans↗
Primary lumbosacral Wilms tumor associated with diastematomyelia and occult spinal dysraphism. A report of a rare case and a short review of literature.
BACKGROUND: The occurrence of an extrarenal Wilms tumor in the lumbosacral region is an extremely uncommon condition. CASE REPORT: We report a case of Wilms tumor in the lumbosacral region that was associated with diastematomyelia and occult spina bifida. An 18-month-old girl presented with a swelling over the lower back with a tuft of hair on it, which she had had since birth. Imaging of the spine revealed spina bifida, bony diastematomyelia, and tethered cord. Excision of the bony spur and detethering of the cord was done. After a year, she had recurrence of swelling at the same site, weakness of both lower limbs, and incontinence of bladder and bowel. Excision of the mass and bony spur and detethering of the spinal cord were done. Histopathological examination showed features of a Wilms tumor.
Female↗
Neurourologic evaluation and urologic management of spinal dysraphism.
The urologic management of the spina bifida child has evolved considerably over the past 10 years. Clinicians now more fully understand the nature of the bladder dysfunction and are therefore able to modify this behavior by either conservative or surgical measures. The spina bifida child and parents can now be confident that the bladder can be appropriately rehabilitated with significant improvement in quality of life.
Adolescent↗
[3-dimensional post-myelographic CT reconstruction in diagnosis and therapy planning of spinal dysraphism].
Dysrhaphia is often associated with severe osseous aberrations of the spine such as, for example scoliosis, hemivertebra, and synostosis. With the advanced possibilities of the post-processing of CT-data (segmentation, three-dimensional reconstruction), post-myelo-CT is an excellent method for the evaluation of osseous structures and the myelon in preoperative planning.
Adolescent↗
Complex colon duplication mimicking an obstructed, non-functioning kidney in a newborn with imperforate anus and spinal dysraphism.
Gastrointestinal (GI) duplications contain tissue resembling several portions of the GI tract and are associated with vertebral and genitourinary (GU) abnormalities [1-4]. We report a newborn with low, imperforate anus and lumbosacral dysraphism, who presented with a large cystic mass in the left renal fossa and pelvis. The flank mass (felt initially to be a dysplastic kidney and ureter) proved to be a complex GI duplication with histologic evidence of gastric, small bowel, and colonic mucosa, as well as respiratory epithelium and pancreatic tissue.
Abnormalities, Multiple↗
[Neurosurgical treatment of spinal dysraphism in children].
A tethered cord syndrome includes in its meaning all types of dysraphism in children. The syndrome is described in regard to its etiology, pathophysiology and clinical outcome. Next, the most prevalent types of dysraphism in children are presented. A surgical approach in myelocele--the most frequent defect--is discussed in detail. Reasons for taking a difficult decision not to operate are considered. The most important operative problems are also reviewed. It is reminded that myelocele often coexists with Arnold-Chiari malformation type II and hydrocephalus. Problems in selecting an appropriate surgical approach in this syndrome are shortly discussed. A rare syndrome of sacral agenesis, poorly amenable to surgical intervention, is presented. There are also some less frequent syndromes described, including:-sinus dermalis, potentially life-threatening in the case of recurrent meningitis;-lipomyelocele, needing early identification and prophylactic surgical treatment;-diastematomyelia, in half of the cases requiring surgery due to a bony spur;-retethered cord syndrome, an iatrogenic complication of neurosurgical interventions done on the spinal cord.
Abnormalities, Multiple↗
The benefits of team treatment and control of adult patients with spinal dysraphism.
We investigated the outcome of newly referred patients to our spina bifida team in the period 1989-1994. 12 of 42 patients had spina bifida occulta, 8 had a meningocele and 22 a myelomeningocele. More than half of the patients underwent an operation which was beneficial to them. 13 operations for a tethered cord syndrome were performed with good results. In 15 patients referral delay caused unnecessary and mostly irreversible harm. Life-long regular control by members of a spina bifida team is, therefore, highly recommended.
Adult↗
Unusual MR presentation of occult spinal dysraphism with lumbosacral lipoma and dermoid cyst.
CASE REPORT: We describe a case with tethered cord syndrome due to the association of intradural lipoma and a dermoid cyst at the filum terminale that had an unusual magnetic resonance (MR) appearance. DISCUSSION: Although both pathologies usually demonstrate a similar intensity on MR images, the involvement of a capsule filled with hair in the dermoid cyst, which presented hypointensity, may be a clue to be differentiated from lipoma on preoperative MR images.
Abnormalities, Multiple↗
Spinal dysraphism associated with congenital heart disorder in a girl with MELAS syndrome and point mutation at mitochondrial DNA nucleotide 3271.
We describe a case of mitochondrial encephalopathy, lactacidosis, and stroke-like episode (MELAS syndrome) associated with ventricular septal defect and meningocele at the L3 level in a 5-year-old girl. Mitochondrial DNA analysis showed point mutation at nucleotide 3271--> TC. The occurrence of heart and neural tube defects in association with usual features of the MELAS syndrome might be explained by either defective high-energy metabolism during early embryogenesis or a common genetic cause.
Child↗
[Magnetic resonance imaging in spinal dysraphisms].
We studied by magnetic resonance imaging the incidence of associated malformations in a group of 38 patients with diagnosis of spina bifida followed in our outpatient clinic. Twenty-two children were born with spina aperta and 16 with spina bifida occulta. Tethered cord was found in the majority of patients of both groups. Syringomyelia was found in 13 patients. The craniocervical region was studied in 11 patients with the diagnosis of spina bifida aperta; Chiari malformation was found in 8. The results point to the fact that asymptomatic malformations detected by MRI techniques are frequent in patients with spina bifida.
Adolescent↗
Cushing's experience with the surgical treatment of spinal dysraphism.
Although Harvey Cushing is best known for his role in developing surgical treatments for tumors of the central nervous system, he performed diverse neurosurgical procedures throughout his career, both at The Johns Hopkins Hospital (1886--1912) and at the Peter Bent Brigham Hospital (1912--1932). His unique and innovative approach to the treatment of myelomeningoceles associated with hydrocephalus, displayed early in his career, is characteristic of his attempts to circumvent the technical limitations of his time in the management of neurosurgical problems. In this report, the authors discuss the evolution of Cushing's technique in the treatment of myelomeningoceles through two illustrative patient records.
Female↗
Third ventriculostomy for hydrocephalus associated with spinal dysraphism: indications and contraindications.
Twenty-five patients have undergone this procedure at our unit. These constitute 25% of our total experience with endoscopic third ventriculostomy (4). In the patients under 6 months of age, only one out of 11 patients has had a successful long-term result despite initial good fenestration of the floor of the third ventricle. These patients were selected on the basis of their adequate third ventricular size and a relatively slowly progressive hydrocephalus. Fourteen patients had a ventriculostomy performed instead of shunt revision. In 13 patients this has been a success long term. All of these patients had a Heyer-Schulte valve with antisiphon device installed for months, or more often, years, prior to the third ventriculestomy. We believe that the difference in these two groups is due to a very poor cerebrospinal fluid(CSF)-resorptive capability in patients immediately after back closure due to the prior venting of CSF into the amniotic sac. The absorptive capacity seems to improve with the passage of time in our patients who have had a shunt system that maintains a relatively normal intracranial pressure and thus contributes to the development of the patient's CSF-absorptive system.
Cerebrospinal Fluid Shunts↗