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PubMed1976

Gas myelography in the investigation of occult spinal dysraphism.

In patients presenting with suspected spinal dysraphism of the occult type, particularly children, cisternal gas myelography is coming to be accepted as the radiological investigation of choice. The technique of the examination as performed on 40 such patients is described in detail. There have been no complications. Twenty-five patients showed abnormalities of the cord together with low termination and abnormal fixation of the conus. The visualization was excellent in terms both of reliability and detail. The reasons for the difficulties in demonstrating these lesions using positive contrast and for preferring gas myelography are discussed.

Adolescent↗
PubMed2005

Dwarfism, occult spinal dysraphism, and presacral myxopapillary ependymoma with an epidermoid cyst in a child.

The authors present a case of a child with dwarfism that was noted to be developmentally delayed. Imaging revealed atlantoaxial instability, occult spinal dysraphism, and a presacral mass. Histopathology of the presacral lesion was that of a myxopapillary ependymoma with epidermoid cyst. We believe this to be the first report in the extant medical literature of this constellation of findings in the same patient. However, there are rare reports indicating a possible association of occult spinal dysraphism and the simultaneous occurrence of spinal ependymomas. Further case reports are necessary to discern whether these pathological entities are true low rate associations that the clinician should consider in their evaluation of these patients.

Cauda Equina↗
PubMed1983

Occult spinal dysraphism in infants: screening with high-resolution real-time ultrasound.

Spinal dysraphism refers to incomplete embryologic development of the dorsal midline. Frequently it is not clinically obvious, and lack of treatment can lead to progressive neurological damage. Early diagnosis of occult defects can allow appropriate surgical intervention and prevent neurological deficits. The authors have used high-resolution real-time ultrasound to screen infants with various cutaneous lumbosacral markers, all of whom were at risk of occult spinal dysraphism. Preliminary results suggest that ultrasound is a reliable screening procedure and could obviate the need for more costly and invasive tests.

Humans↗
PubMed1988

The management of children with spinal dysraphism.

Improvements in technology have dramatically increased the survival of children with spinal dysraphism. Because this complex condition affects multiple organ systems as well as the psychosocial functioning of the child and family, these children require care from a host of specialists in order to achieve optimum functioning. This article reviews the pathophysiology and discusses the current management of the medical and psychosocial effects of spinal dysraphism. It also briefly discusses strategies for intervention, long-term outcomes, and some controversies regarding care and screening.

Child↗
PubMed1998

Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release.

OBJECTIVE: To investigate the influence of neurosurgical intervention on the appearance of upper motor neuron (UMN) signs in newborns diagnosed with occult spinal dysraphism and tethered cord (TC) during the first month of life. METHODS: A prospective study (1990 to 1996) of 22 consecutive newborns with occult spinal dysraphism monitored for the appearance of UMN signs. Untethering was performed when neurologic or urodynamic investigation indicated the presence of UMN dysfunction. RESULTS: Of 22 patients, 10 remained free of UMN symptoms during follow-up (mean, 67+/-22 months). Untethering was performed in 12 of 22 patients because of the presence of UMN symptoms. In 7 of these 12 patients, there was a documented asymptomatic period of 13+/-11 months before the onset of UMN symptoms. Untethering at a mean age of 18+/-17 months restored normal neurologic and urinary function in all patients (mean postoperative follow-up, 25+/-16 months). Of the 12 children, 5 presented with UMN signs at birth. In these children, untethering was performed at a mean age of 9+/-5 months. In two of these five patients, UMN symptoms did not resolve after surgery, and ongoing conservative bladder treatment was required (mean follow-up, 37+/-14 months). In none of the 12 operated children did signs of retethering occur. CONCLUSIONS: A significant number (10/22) of children born with occult spinal dysraphism and TC did not develop UMN symptoms during follow-up; neurosurgical correction after the appearance of an UMN sign restored normal neurologic and urinary function in all children; and untethering in children presenting at birth with UMN symptoms resulted in poorer outcome.

Child, Preschool↗
PubMed2002

Magnetic resonance urography in patients with neurogenic bladder dysfunction and spinal dysraphism.

OBJECTIVE: To investigate the reliability of magnetic resonance urography (MRU) using heavily T2-weighted very fast spin-echo sequences in imaging the upper urinary tract in patients with spinal dysraphism and neuropathic bladder dysfunction. PATIENTS AND METHODS: Patients with neuropathic bladder dysfunction secondary to spinal dysraphism may have gross spinal deformity, which makes assessing the urinary tract extremely difficult by ultrasonography or intravenous urography. The study included 50 patients (median age 33 years, range 19-52) with a median (range) Cobb's angle of 60 (0-240) degrees. No contrast medium was given but all patients received 20 mg of intramuscular frusemide 20-30 min before MRU. RESULTS: Thirty-five patients had previous ultrasonography, during which only 42 kidneys and no ureters were visible. Of the 94 renal tracts, MRU visualized all kidneys and 86 (89%) ureters. There was good agreement amongst observers on assessing the kidneys and pelvicalyceal systems (kappa > or = 0.61) but only moderate agreement on evaluating the ureters (kappa < or = 0.61). There was disagreement on normality vs renal parenchymal scarring in 14 (15%) kidneys, about no dilatation vs dilatation of the pelvicalyceal system in 14 (15%) systems and about no dilatation vs dilatation of the ureter in 20 (22%) ureters. The overall quality of the MRU images was rated highly, with a mean (range) score of 4.6 (3-5). CONCLUSION: MRU provides a reliable noninvasive technique for imaging the upper tract in patients who were previously difficult to investigate.

Adult↗
PubMed2003

Chiari II malformation and occult spinal dysraphism. Case reports and a review of the literature.

We report two cases of children with occult spinal dysraphism who were also found to have many associated brain anomalies seen in the Chiari II malformation. No previous report has commented on the possible association between the Chiari II malformation and the 'closed' form of neural tube defect. One child had symptoms referable to pathology at the craniocervical junction. Neither child had cutaneous stigmata associated with occult spinal dysraphism over the caudal midline spine. These cases, although seemingly rare, lend support to the theories that the association between the Chiari II malformation and patients with myelomeningoceles is due to dysgenesis of the rostral and caudal neural tube. These cases are also important as refutation of earlier theories that link these two entities by proposing that the Chiari II malformation is due to overdrainage of cerebrospinal fluid at the site of myelomeningocele.

Arnold-Chiari Malformation↗
PubMed1991

The human tail and spinal dysraphism.

Recent publications have endeavoured to differentiate between the true, or vestigial tail, and the pseudotail by clinical and pathological examination, and have indicated the benign nature of the true tail. The true tail arises from the most distal remnant of the embryonic tail, contains adipose, connective, muscle, and nerve tissue, and is covered by skin. Pseudotails represent a variety of lesions having in common a lumbosacral protrusion and a superficial resemblance to vestigial tails. A review of the case reports indicates spina bifida to be the most frequent coexisting anomaly with both. A review of occult spinal dysraphism shows it to be associated with cutaneous signs in more than 50% of instances. Three cases of spinal dysraphism with tail-like cutaneous structures are described and their radiological, operative, and pathological findings presented. The classification of each of the appendages into true tail or pseudotail remains obscure. Although the finding of these three tails was the subject of much curiosity, surgical treatment was clearly designed to adequately deal with the associated dysraphic state. The presence of a tail-like appendage in the lumbosacral region should alert the clinician to the possibility of underlying spinal dysraphism. Preoperative assessment must include a complete neurological history and examination as well as computed tomographic or magnetic resonance imaging.

Bone Neoplasms↗
PubMed1995

Intradural spinal lipomas not associated with spinal dysraphism: a report of four cases.

Intradural spinal lipomas that are not associated with spinal dysraphism are rare. We report four cases of adult patients with intradural spinal lipomas. All of the patients were young adults, three women and one man, who ranged in age from 22 to 37 years (mean, 30.3 yr). The clinical presentation of these lesions varied, depending upon the size and location of each lipoma. It is interesting that the symptoms in the women gradually deteriorated during pregnancy or after delivery. Magnetic resonance imaging was very useful in determining the exact location of lipoma. In three of four patients, neurological improvement was obtained after decompressive laminectomy and partial removal of the lipoma.

Adult↗
PubMed1984

[Clinical, sonographic and radiologic findings in spinal dysraphism of the lumbosacral region].

Demonstrating the clinical, sonographic and radiologic findings in three pediatric patients suffering from caudal spinal dysraphism the various diagnostic and therapeutic problems of neural tube defects are discussed. Important conclusions are as follows: (1) Early diagnosed and operated malformations of the caudal spine may cause neurologic, neurosurgical, urogenital, gastrointestinal and orthopedic complications even after uneventful years. Mostly the reason is a prevented ascensus of the conus medullaris. Therefore a longstanding follow-up of these patients is mandatory. (2) The patient's examiners have to look for cutaneous manifestations of spinal dysraphism even in the sacral region to avoid secondary damage. (3) Sonography today is a valuable diagnostic method not only applied for screening examinations but also to establish definite diagnoses in many cases. The application of other methods is therefore guided by sonographic findings.

Child↗
PubMed1980

Intramedullary abscess--a rare complication of spinal dysraphism.

Two cases are reported of patients with spinal dysraphism who developed abscesses within the spinal cord. In one case the infection had spread to the cord through a dermal sinus, as in the six cases previously recorded in the literature. In the other patient the sepsis developed within an intramedullary epidermoid tumour, but the route of infection was not clear. Each patient made a virtually full neurological recovery after open drainage of the abscess.

Abscess↗
PubMed2006

Primary spinal intramedullary adrenal cortical adenoma associated with spinal dysraphism: case report.

OBJECTIVE: The authors report a primary spinal intramedullary adrenal cortical adenoma in a patient with spinal dysraphism presenting with bilateral leg pain and urinary frequency. METHODS: Magnetic resonance imaging, L2 laminectomy with resection of mass, and pathological and immunohistochemical analysis of resected mass revealed the diagnosis. RESULTS: Microscopic and immunohistochemical findings confirmed the diagnosis as a primary intramedullary tumor of adrenal cortical origin. CONCLUSION: The occurrence of a primary adrenal tumor in the spinal cord is rare and difficult to explain based on our understanding of embryology. A review of the relevant literature and discussion of the pathophysiology and clinical implications is provided.

Adrenal Cortex Neoplasms↗
PubMed1987

Intraoperative sonography in spinal dysraphism and syringohydromyelia.

The use of intraoperative sonography was analyzed in 24 patients with spinal dysraphism and syringohydromyelia in order to determine the role of real-time sonography in the surgical management of these patients. Specific diagnoses included tethered cord (nine), syringohydromyelia (seven), congenital tumor unassociated with a tethered cord (four), diastematomyelia (three), and occult sacral meningocele (one). Intraoperative sonography determined the exact relationship of congenital tumors to the cord before opening the dura, which allowed a more precise approach to the mass. Intraoperative sonography identified the lower end of the syrinx cavities, which allowed optimal catheter placement. Fibroglial scar tissue, which may compartmentalize these syrinx cavities, was clearly shown, and the efficacy of shunt catheter placement was immediately determined. In diastematomyelia, intraoperative sonography identified the relationship of the hemicords to bony, cartilaginous, and/or fibrous septa and demonstrated the effect on the tethered hemicords of removing these septa and constructing a single dural sac from the two dural sacs that had enclosed the hemicords. Since significant surgical decisions are based on these sonographic observations, the authors urge widespread use of intraoperative sonography in patients with spinal dysraphism and syringohydromyelia.

Humans↗
PubMed1990

Magnetic resonance evaluation of spinal dysraphism in children.

Magnetic resonance (MR) imaging of the spine was performed as the initial imaging technique in 20 children when spinal dysraphism was suspected clinically and plain radiographs showed spina bifida. The correlation with surgical findings indicated that MR provided accurate information preoperatively in all the cases. Some unusual observations in cases with spina bifida cystica and occulta are discussed. It is concluded that plain radiograph and MR complete the preoperative radiologic evaluation of cases with spinal dysraphism.

Adolescent↗
PubMed1995

Association of imperforate anus with occult spinal dysraphism.

Neurological deficits may occur in some children with imperforate anus produced by unrecognized spinal cord anomalies amenable to neurosurgical correction. Although this association has been well described, its frequency has not been defined. The authors prospectively studied 45 infants with imperforate anus from 1985 to 1992 to establish the frequency of this association. Eighteen infants (40%) had high imperforate anus, 24 had low imperforate anus (53%), and 3 had anal stenosis (7%). All underwent radiographic spinal cord imaging in early infancy, which consisted of radiographs (45), ultrasound (26), magnetic resonance imaging (29), computed tomography (1), and myelography (1). Thirty-three percent of the patients with imperforate anus had bony sacral abnormalities. Eight infants were discovered to have spinal cord abnormalities. Two of those infants had obvious myelomeningocele at birth, and 6 infants (14%) had occult spinal dysraphism consisting of tethered cord with or without associated lipoma of the cord. There was no correlation between the presence of tethered cord and the gender of the infants, the type of malformation (high versus low imperforate anus), or the presence of sacral dysplasia (Fisher's exact test). Five of the infants who were found to have tethered cord underwent neurosurgical intervention and repair of the defect, and 1 infant was followed up clinically. The mean follow-up time of all the infants was 2.5 +/- 0.9 (+/- SD) years. No neurological sequelae occurred in the infants who had surgical correction. The frequency of occult spinal dysraphism with imperforate anus was 14%. It was independent of the type of imperforate anus or the coexistence of bony sacral anomalies.(ABSTRACT TRUNCATED AT 250 WORDS)

Anus Diseases↗
PubMed2003

Myoclonus in spinal dysraphism.

Two cases of segmental myoclonus occurring in association with spinal dysraphism are described. In one, myoclonus of paralysed legs arose below a region of spinal cord lacking any normal function, illustrating the capacity of the isolated spinal cord to generate and maintain rhythmic activity independent of supraspinal influences.

Adolescent↗
PubMedDate not supplied

Association of wilms tumor with spinal dysraphism.

Previous data suggested an association of vertebral anomalies with Wilms tumor. At the same time, vertebral midline fusion defects are often indicated by dermal anomalies over the spine. In the present study the prevalence of both occult spina bifida and cutaneous signs of spinal dysraphism was significantly higher in 50 Wilms patients than in 180 control children (18.0 versus 4.4%, p <.01, and 35.9 versus 17.5%, p <.02, respectively). Family investigations are needed to answer the question whether signs of spinal dysraphism in parents and sibs of patients may be regarded as indicators of an increased risk of Wilms tumor in the family.

Child↗