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PubMedDate not supplied

Spinal dysraphism: a comprehensive diagnostic approach.

Twelve patients with the clinical findings of spinal dysraphism form the basis for this report. In eight patients, physical findings, plain x-rays, and unenhanced spinal computed tomography allowed for precise diagnosis and subsequent therapy, without contrast myelography. All patients in the study had intravenous pyelography, and this revealed evidence of renal deterioration in two. The other 10 patients had radiographically normal upper urinary tracts. Urodynamic assessment was performed in seven; three were normal and four were abnormal. The pattern of the abnormal studies (three "flaccid type" with an adequate urethral pressure profile and one with detrusor-sphincter dyssynergia) allowed for appropriate therapy to be employed for bladder emptying and continence. Both spinal computed tomography and urodynamic testing serve as noninvasive studies that can be employed in the follow-up and management of patients with spinal dysraphism.

Adolescent↗
PubMed2005

Spinal hamartoma associated with spinal dysraphism.

OBJECTS: The aims of the study were (1) to review the pathological findings of spinal lipomatous masses associated with congenital spinal dysraphism and (2) to discuss the pathological diagnosis. METHODS: The pathological records of 47 patients at our institution were reviewed, and three illustrative cases were presented. CONCLUSION: Spinal tumorous lesions associated with spinal dysraphism have been traditionally described as lipoma since they are composed mostly of fatty tissue. However, they are different from lipomas arising in other part of the body in that they often contain various tissues of ecto- and mesodermal origin. In our study, we detected such heterotopic components in 24 out of 47 cases. Although they are also similar to teratoma, it is generally accepted that they are malformative lesions which lack neoplastic potential. We therefore should diagnose them as hamartoma rather than lipoma or teratoma.

Child↗
PubMed2002

Callosal anomalies in patients with spinal dysraphism: correlation of clinical and neuroimaging features with hemispheric abnormalities.

Dysgenesis of the corpus callosum can occur in association with spinal dysraphic lesions. Clinical and neuroimaging features were reviewed in 23 patients (12 male, 11 female; mean age 11.3 years) with caudal spinal dysraphism (myeloschisis in eight, meningomyelocele in 10, and lumbosacral lipoma in five) to characterize types and degrees of callosal and other cerebral anomalies. T1- and T2-weighted magnetic resonance images were obtained, and the total midsagittal cross-sectional area of the corpus callosum was determined. The corpus callosum appeared normal in nine patients and was abnormal in 14. In five patients the corpus callosum was narrow, with all regions present; the cerebral hemispheres were hypoplastic. Two patients with dysgenesis of frontal, parietal, and occipital lobes had a small, partly agenetic corpus callosum. In the remaining seven patients the posterior third of the corpus callosum was absent or hypoplastic; six of them had ventriculomegaly that selectively affected the occipital horns (colpocephaly). All callosal anomalies were accompanied by hemispheric ones. This supports a disordered developmental relationship between the corpus callosum and the hemispheres as a cause. Spinal dysraphism can no longer be considered a single developmental abnormality, given the frequent association of other defects.

Adolescent↗
PubMed2002

Value of electrophysiological assessment after surgical treatment of spinal dysraphism.

PURPOSE: This study was conducted in children treated surgically for spinal dysraphism between 1994 and 2000 to determine the value of electrophysiological parameters for followup in comparison with clinical, radiological and urodynamic findings. MATERIALS AND METHODS: Electrophysiological examination was performed based on electromyographic detection of perineal muscle activity with evaluation of the sacral reflexes and measurement of lower limb and perineal evoked potentials. Clinical and radiological assessment consisted of physical examination, kidney ultrasonography and retrograde cystouretrography. Urodynamic assessment included determination of urethral pressure profile, bladder activity, compliance, voiding pressure and sphincter synergy. A total of 45 children were included in the study. In 28 cases electrophysiological assessment was performed routinely in 20 children (group 1) before and after (6 months and annually thereafter) surgical treatment (mean followup 30 months) and in 8 children (group 2) only after surgical treatment (mean followup 65 months). Electrophysiological findings were correlated with the urodynamic and clinical findings. Sensitivity, specificity and positive and negative predictive values were calculated. In 17 children (group 3) electrophysiological assessment was performed only once either before or after surgery. RESULTS: Electrophysiological assessment with combined electromyographic detection and perineal evoked potentials was highly sensitive in predicting urodynamic impairment, particularly vesico-sphincter dyssynergia (sensitivity 100%) or bladder dysmotility (sensitivity 86%). Specificity of perineal evoked potentials for detecting urodynamic dysfunction was 90%. Electromyographic detection and perineal evoked potentials also demonstrated satisfactory sensitivity in confirming urological impairment (sensitivity 79%, negative predictive value 90%). CONCLUSIONS: Electromyographic detection and perineal evoked potentials are valuable for assessment of patients after surgical treatment of spinal dysraphism. Because these techniques are minimally invasive and highly reproducible, they are suitable for baseline assessment in most cases (tethered cord, lipoma, syringomyelic cavities). Urodynamic assessment should be reserved for patients at high risk for secondary neurogenic bladder dysfunction such as myelomeningocele or sacral agenesis.

Child↗
PubMed2004

Lumbar meningeal hamartoma and epidermoid cyst associated with spinal dysraphism in an elderly patient.

Meningeal hamartoma associated with spinal dysraphism in an elderly patient is an extremely rare clinical presentation. A 62-year-old woman who progressively developed gait disturbance and urinary retention was admitted to our hospital. Magnetic resonance imaging (MRI) revealed a large cystic mass associated with a dermal sinus at the L4-L5 level. MRI was useful in the diagnosis and determination of this lesion. However, preoperative differential diagnosis of the tumor was not possible based on neuroimaging evidence. The diagnosis of meningeal hamartoma was based on the histological and immunohistological findings of the specimen obtained from the tumor. Meningeal hamartoma associated with spinal dysraphism should be kept in mind, even in adult cases. In this article, we also discuss the radiological and pathological appearance of this rare clinical entity.

Epidermal Cyst↗
PubMed1992

The value of urodynamic studies in infants less than 1 year old with congenital spinal dysraphism.

The role and timing of urodynamic studies in infants with congenital spinal dysraphism are controversial. We reviewed 64 consecutive infants with a mean followup of 28 months who underwent video urodynamic study when they were less than 1 year old (50 of the 64 patients were less than 3 months old). A previously reported video urodynamic "hostility score" from 0 (best) to 10 (worst) was given to each study and compared with the outcome of the upper urinary tract and subsequent urodynamic studies. A larger fraction of these children with high hostility scores (5 or greater) had upper tract deterioration (39%) compared to those with low hostility scores (9%). Moreover, 6 of 14 patients in the low hostility group who underwent repeat video urodynamic studies after age 1 year had high hostility lower urinary tracts. We conclude that urodynamic studies during and after the first year of life are useful in the appropriate urological evaluation and management of infants with spinal dysraphism.

Female↗
PubMed1991

Investigation of children with suspected spinal dysraphism by magnetic resonance imaging.

Findings on Magnetic Resonance Imaging (MRI) of 52 children with suspected spinal dysraphism have been reviewed. In 24, no significant spinal abnormality was demonstrated. Seven patients had scoliosis or vertebral segmentation anomalies without demonstrable abnormality of the underlying soft tissues and one had an isolated subcutaneous haemangioma. In 20 children with spinal dysraphism, a low tethered cord was the most frequent finding, occurring in 80%. Other manifestations included myelo- or meningocoele (60%), syringomyelia (30%), lipoma (25%), congenital tumour (20%), diastematomyelia (15%) and thickened filum terminale (5%). The relationship between the clinical reason for requesting MRI and the scan results are discussed.

Adolescent↗
PubMed2004

Pre- and post-operative hydromyelia in spinal dysraphism.

BACKGROUND AND PURPOSE: There has been limited description of hydromyelia after surgery for spinal dysraphism. The opportunity to compare pre- and post-operative hydromyelia in patients with spinal dysraphism has been possible in two groups of patients using MRI. Post-operative assessment and possible relationship of hydromyelia to clinical findings has been made during a 7- to 14-year period. METHODS: A total of 38 patients with congenital lumbar or sacral lipomas and 20 with diastematomyelia were studied pre-operatively with a 1.5-T MRI. Most patients in each group were examined with surveillance MRI (1.5 T) post-operatively. Clinical correlations were carried out with each examination. RESULTS: Of the 38 dorsal or terminal lipoma post-operative patients, hydromyelia increased in 3 and was a new finding in 4. One symptomatic patient in the latter group had extensive septated lumbar hydromyelia. In 8 of 20 diastematomyelia patients, pre-operative hydromyelia was unchanged post-operatively. Hemicord hydromyelia developed in 1. CONCLUSION: Hydromyelia of varying degree was found in almost one-third of post-operative dorsal or terminal lipoma patients and nearly one-half of diastematomyelia patients. In five post-operative lipoma and two diastematomyelia patients, significant neurological findings remained. One of the six post-operative lipomas had new extensive lumbar hydromyelia that may have been responsible for the patient's symptoms. In the remaining symptomatic patients, hydromyelia was absent or modest.

Child↗
PubMed1978

Cutaneous signs of spinal dysraphism. Report of a patient with a tail-like lipoma and review of 200 cases in the literature.

The term "spinal dysraphism" was coined in 1940 by Dr Lichtenstein to designate incomplete fusion or malformations of structures in the dorsal midline of the back, particularly congenital abnormalities of the vertebral column and spinal cord. Raphes develop on the face and head, brancheal arches, sternum, and spinal column. When dysraphism occurs in these sites, failure of closure of fontanelles, cleft lip and palate, brancheal cysts, and abnormalities of the ribs and spine result. A review of 200 cases of occult spinal dysraphism showed the condition to be more common in female patient and to be associated with cutaneous signs in more than 50% of instances. The age at which neurologic symptoms appeared in recorded cases is from birth to 76 years, the average being three years. A case of spinal dysraphism with a tail-like cutaneous structure is presented. The cutaneous manifestations accompanying spinal dysraphism that may lead to early recognition of this syndrome and early institution of treatment are discussed.

Adult↗
PubMed2005

Minor skin lesions as markers of occult spinal dysraphisms--prospective study.

BACKGROUND: Neonates with occult spinal dysraphisms (OSDs) may not present any clinical manifestations, but may be associated to cutaneous stigmas that indicate dysraphism. Association of minor cutaneous stigmas (discreet skin lesions, most of which are not assessed) with OSD was investigated in this study, as well as the use of ultrasonography (US) as a screening method for those patients. METHODS: Two thousand ten neonates were evaluated trough active search. Both the pediatrician and the neurosurgeon performed the search for the presence of cutaneous stigmas on the midline of the dorsal region. For all of them, the gestational age, type of delivery, gender, ethnicity, age of mother, and whether it was a high-risk gestation were recorded. Patients with skin lesions comprised the case group. For each case, another neonate of the same gender, gestational age, and ethnicity was selected as control. Both groups were evaluated with respect to personal, social, and medical information. Spinal US was performed in all case and control patients-if altered or inconclusive, it was complemented with magnetic resonance imaging (MRI). RESULTS: Of the 2010 patients, 144 presented cutaneous stigmas. Of these, 8 had alterations to US (5.5%) and 6 to MRI. There were no alterations to US in the control group. The most frequent lesions were tufts of hair and dimples; through US, the most frequent findings were dermal sinuses. CONCLUSIONS: The so-called minor skin lesions were not markers of OSD in the evaluated population. However, in 4 patients, US was decisive for the surgical decision. From the statistical point of view, there is no indication for complementary examinations in patients with minor cutaneous stigmas. However, because of the feasibility, simplicity, and low cost of the spinal US, the examination is justified in the benefits of early diagnosis, regardless of the need of immediate surgical treatment.

Age Factors↗
PubMed1990

Spinal dysraphism: use of magnetic resonance imaging in evaluation.

Three cases of children with spinal dysraphism are reported. Magnetic resonance imaging (MRI) was used as a primary diagnostic examination. The ages of the patients were 2 days, 4 years, and 16 years. In all instances the scan gave a precise diagnosis as well as well as an accurate delineation of the structural abnormalities before surgical treatment. Plain radiographs and ultrasound analysis may not be helpful, and invasive procedures can be associated with morbidity. Technical ease, safety, and anatomic precision suggest that MRI should be performed as a primary radiologic examination in the diagnostic workup of spinal dysraphism.

Adolescent↗
PubMed2001

Newborns with suspected occult spinal dysraphism: a cost-effectiveness analysis of diagnostic strategies.

OBJECTIVE: To assess the clinical and economic consequences of different diagnostic strategies in newborns with suspected occult spinal dysraphism. METHODS: A decision-analytic model was constructed to project the cost and health outcomes of magnetic resonance imaging (MRI), ultrasound (US), plain radiographs, and no imaging in newborns with suspected occult spinal dysraphism. Morbidity and mortality rates of early versus late diagnosis of dysraphism and the sensitivity and specificity of MRI, US, and plain radiographs were obtained from the literature. Cost estimates were obtained from a hospital cost accounting database and from the Medicaid fee schedule. RESULTS: We found that the choice of imaging strategy depends on the underlying risk of occult spinal dysraphism. In low-risk children with intergluteal dimple or newborns of diabetic mothers (pretest probability: 0.3%-0.34%), US was the most effective strategy with an incremental cost-effectiveness ratio of $55 100 per quality-adjusted life year gained. For children with lumbosacral dimples, who have a higher pretest probability of 3.8%, US was less costly and more effective than the other 3 strategies considered. In intermediate-risk newborns with low anorectal malformation (pretest probability: 27%), US was more effective and less costly than radiographs and no imaging. However, MRI was more effective than US at an incremental cost-effectiveness of $1000 per quality-adjusted life year gained. In the high-risk group that included high anorectal malformation, cloacal malformation, and exstrophy (pretest probability: 44%-46%), MRI was actually cost-saving when compared with the other diagnostic strategies. For the intermediate-risk group, we found our analysis to be sensitive to the costs and diagnostic performances (sensitivity and specificity) of MRI and US. Lower MRI cost or greater MRI diagnostic performance improved the cost-effectiveness of the MRI strategy, whereas lower US cost or greater US diagnostic performance worsened the cost-effectiveness of the MRI strategy. Therefore, individual or institutional expertise with a specific diagnostic modality (MRI versus US) may influence the optimal diagnostic strategy. CONCLUSIONS: In newborns with suspected occult dysraphism, appropriate selection of patients and diagnostic strategy may increase quality-adjusted life expectancy and decrease cost of medical work-up.

Cost-Benefit Analysis↗
PubMed1999

Spinal dysraphism in a newborn Holstein-Friesian calf.

Spinal dysraphism, not associated with vertebral defect or arthrogryposis, was found in a 3-day-old Holstein-Friesian calf that was clinically diagnosed as having encephalopathy. The dysraphic lesion occurred in the sixth (C6) and seventh (C7) segments of the cervical spinal cord. Microscopically, the lesion was characterized by hydromyelia, syringomyelia, anomaly of the ventral median fissure, abnormal running of the myelinated nerve fibers in the white column, and absence of the central canal due to a developmental defect of the ependymal cells.

Animals↗
PubMed2004

Use of intraoperative ultrasonography during the surgical repair of pediatric cystic spinal dysraphism--technical note.

The present study investigated the benefits of intraoperative ultrasonographic guidance during the surgical repair of congenital cystic spinal dysraphic lesions. Twenty-one children with cystic spinal dysraphism who underwent surgical repair were examined by real-time ultrasonography during the surgical intervention. Five children had meningoceles, six had myelomeningoceles, four had open neural plaques, three had lipomyelomeningoceles, and three had diastematomyelia. Visualization of the cystic compartments, identification of the neural structures, and identification and localization of the associated lesions were all reliably achieved in all cases. Intraoperative ultrasonographic guidance could determine the type of lesion and the associated lipomas, ectopic tissues, dermoid and epidermoid cysts, and doubling of the spinal cord, and locate diastematomyelic spurs, bands, and adhesions. Components filled with cerebrospinal fluid appeared as anechoic areas, and lipomas as hyperechoic. Intraoperative ultrasonographic guidance allowed the surgeon to correlate the complex anatomy identified on preoperative computed tomography and magnetic resonance imaging to the surgical site during the operation. Better orientation to the defect allows appropriate repair of the lesion with optimal preservation of neural tissues.

Child, Preschool↗
PubMedDate not supplied

Magnetic resonance imaging of the spinal cord in spinal dysraphisms.

Magnetic resonance imaging (MRI) was performed 49 times in 42 patients with spinal dysraphism. Scoliosis and a changing neurological picture were the primary indications. Spinal cord anomalies included hydromyelia, diastematomyelia, lipoma, thickened filum terminali, and spinal cord atrophy. All but one patient exhibited Arnold-Chiari malformation. Twenty-two of the 42 patients had computed tomography (CT) scans, myelograms, or operations that corroborated the 41 MRI findings. Three false-positive MRI findings of hydromyelia and no false-negative studies were observed. MRI is a noninvasive investigative technique that provides more information than myelography or CT in defining spinal cord anatomy in spinal dysraphism.

Arnold-Chiari Malformation↗
PubMed1976

Spinal dysraphism: genetic relation to neural tube malformations.

The families of 207 index patients treated for spinal dysraphism at The Hospital for Sick Children were studied to discover whether the condition was aetiologically related to the classical neural tube malformation--spina bifida cystica and anencephaly. The index patients had all had a tethered conus medullaris and one or more of a variety of anomalies of the spinal cord, vertebrae, or skin overlying the vertebral column. Of 364 sibs of index patients, 9 had an encephaly and 6 spina bifida cystica, a pro-proportion of 4.12%. This approximates to the proportion of sibs affected by neural tube malformations in the London region when the index patients themselves have spina bifida or anencephaly. It is, therefore, appropriate that the mothers of children with spinal dysraphism should be offered prenatal screening for neural tube malformations.

Adult↗
PubMed1999

Phase-contrast magnetic resonance imaging study on cord motion in patients with spinal dysraphism: comparison with healthy subjects.

Cord motion at the cervical cord (C-3) level was measured with phase-contrast magnetic resonance imaging (MRI) in 39 healthy young subjects and 34 age-matched patients with spinal dysraphism. Cord velocity curves (composed of waves I, II, and III) were made in both sagittal images and transverse images and classified into 3 types. The measurement in the sagittal images were more reproducible than that in the transverse images. In healthy subjects, the mean cord velocity of wave I was 0.49 +/- 0.11 cm/s in the sagittal image. In the subgroup of patients with spinal dysraphism showing stable clinical courses, cord velocity was comparable to that in the healthy subjects. Patients of the subgroup with symptomatic aggravation showed a significant decrease in cord velocity in wave I, and a characteristic pattern in the cord velocity curve: the velocity change (wave I) was small. This decrease in cord velocity was thought to be due mainly to cord tethering. The measurement of cord motion with phase-contrast MRI could give objective and quantitative information about cord motion and tethering associated with spinal dysraphism.

Adolescent↗