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PubMedDate not supplied

Intramedullary mass lesion of the spinal cord in children of a developing milieu.

A retrospective study of 22 children with intramedullary mass lesions (neoplastic and nonneoplastic) was performed to see the frequency of different histotypes of tumors/lesions and the functional outcome in Indian children. The age of the children varied from 1.5 to 16 years with a median of 8 years. The male to female ratio was 2.7:1. The children having a minimum follow-up of 3 months and a complete clinicopathological record were entered into this study. McCormick functional grading was applied retrospectively to assess the preoperative grade and postoperative functional outcome of the children. Histopathologically, these 22 cases were astrocytomas (n = 6, 27.3%), ependymomas (n = 3, 13.6%), dermoids (n = 3, 13.6%), epidermoids (n = 2, 9.1%), ependymal cysts (n = 2, 9.1%), and myxopapillary ependymoma, neurenteric cyst, ganglioglioma, teratoma, tuberculoma and anteriovenous malformation one each (4.55%). Near-total or gross-total resection was achieved in 13 (59%) and subtotal resection in 7 patients (32%), while complete excision of tuberculoma was achieved in 1 patient. Coagulation of perimedullary vessels was done in another single patient with anteriovenous malformations. Fourteen of the 22 cases having had functional grade IV or V preoperatively, improved to grade I or II (n = 7) and to grade III (n = 4, all astrocytomas) following surgery. Two patients died (1 within 2 days and 1 after 3 months due to recurrence of the tumor) and 1 case deteriorated at the follow-up of 4 months. Eight out of the 22 patients who had been admitted with functional grade II or III improved to grade I or II following surgery. The following conclusions were drawn after evaluation of these cases: (1) astrocytomas and ependymomas constituted only 50% of intramedullary tumors; (2) nonneoplastic, malformative tumors like dermoid and epidermoid showed a higher incidence (23%) in comparison with the Western World where all congenital intramedullary tumors (including dermoids and epidermoids) account for 5-8% only; (3) extremely rare cystic lesions like ependymal cyst and inflammatory granuloma may manifest as an intramedullary lesion; (4) even neurenteric cyst, though rare, may manifest as a pure intramedullary lesion without the stigmata of spinal dysraphism, and (5) delayed presentation was the cause of unfavorable outcome in a developing milieu.

Adolescent↗
PubMed1994

Adult diastematomyelia.

With modern imaging techniques, various types of spinal dysraphism are being diagnosed in adults with increasing frequency. We report a 42-year-old woman with diastematomyelia whose symptoms characteristically were precipitated by trauma. She exhibited other typical features such as a posterior midline hair patch and vertebral malformations. Metrizamide myelography with computerized tomography showed a bony septum at T11 with a cleft at T9-T11 separating two dural tubes each of which contained a single spinal hemicord. The septum was excised with complete relief of symptoms.

Adult↗
PubMed1990

Magnetic resonance imaging in neurological disorders.

To investigate the role of magnetic resonance imaging (MRI) in neurological disorders, 115 children were studied in two groups. Group A (78 patients) was studied by paired computed tomography and MRI cranial scans. Group B (37 patients) was studied by paired computed tomography assisted myelography (CTM) and MRI spinal scans. In group A, the scans were generally equivalent for supratentorial tumours and for investigating fits, hydrocephalus, benign intracranial hypertension, and cerebral atrophies, but MRI scanning was superior for posterior fossa tumours and cysts. In group B, MRI scans were superior for intramedullary spinal tumours, spinal dysraphic problems with tethering or syrinx, and were complementary to CTM in diastematomyelia.

Adolescent↗
PubMed1981

Sacral intraspinal lipoma associated with congenital iliac anomaly.

2 cases of a peculiar unilateral protuberance near the iliac bone with intraspinal abnormalities are reported. 8 cases have been described in the literature with this characteristic congenital association; they indicate the frequency of an occult spinal dysraphism which must be proved by myelography. In each case, surgical treatment of bone abnormality or of intraspinal lesions, or of both, should be discussed.

Adolescent↗
PubMedDate not supplied

Patterns and progression in congenital scoliosis.

Sixty cases of congenital scoliosis over a 13-year period were studied. The female:male ratio was 1.4:1. The deformity usually presented itself first either at 2 or 8-13 years of age. A positive family history was present in five cases, and parental consanguinity was observed in 40% of patients. Five patterns of deformity were recognized. Hemivertebra was the most common type, and unilateral unsegmented bar with contralateral hemivertebra was the most severe and most progressive pattern of deformity. Thoracic curves were more prevalent while larger curves were more commonly seen in the thoracolumbar area. The curves measured >40 degrees in 70% of the patients who had reached maturity. The curve progression index was 9 degrees for unilateral unsegmented bar with contralateral hemivertebra, and 6 degrees without contralateral hemivertebra. This index was 1.5 degrees for hemivertebra and complex type of deformity, and 0.5 degrees for block vertebra. Unbalanced fully segmented hemivertebra was next after the two types of unsegmented bars in terms of potential for progression. Presence of fused ribs on concave side of lower thoracic curves increased the rate of curve progression. Spinal dysraphism, diagnosed in 20% of cases, was the most commonly associated anomaly.

Adolescent↗
PubMed2006

The patient with symptoms following resection of a lipomyelomeningocele: do increases in the lumbosacral angle indicate a tethered spinal cord?

OBJECT: The angulation of the sacrum is easily measured. The authors have previously reported on patients who were symptomatic with a diagnosis of myelomeningocele who were found to have changes in their lumbosacral angle (LSA) corresponding to the onset of symptoms indicative of a tethered spinal cord. The aim of this study was to verify this same finding in a group of patients with occult spinal dysraphism (that is, closed neural tube defect). METHODS: A retrospective analysis of 50 consecutive lipomyelomeningocele repair procedures was performed. Data pertaining to 30 age-matched control patients were also analyzed. Measurements were made of the LSA over time in all studied patients harboring lipomyelomeningoceles. Appropriate imaging was available for 25 cases of lipomyelomeningocele (that is, radiographs of the lumbosacral junction were available from the patient's perinatal period and at presentation of symptoms of a tethered spinal cord). Roughly one third of these patients suffered symptoms from a tethered spinal cord at the most recent follow-up examination. Nine patients (36%) were found to have corresponding LSA measurements greater than 70 degrees, and seven (28%) of these patients presented with signs and symptoms of a tethered spinal cord, such as decreased lower-extremity function (two patients), urinary bladder incontinence (three patients), back pain (one patient), and lower-extremity paresthesias (one patient). The LSA measurements were statistically greater (p < 0.05) in the symptomatic patient population than in age-matched control patients and asymptomatic patients. Changes in the LSA in these symptomatic patients ranged from 5 to 24 degrees (mean 13 degrees). Changes in the LSA were noted in only two asymptomatic patients. No single sign or symptom of a tethered spinal cord appeared to correlate to any degree of increase in the LSA. Of the 25 patients excluded from this study because perinatal radiographic images were not available, none was symptomatic at the most recent follow-up examination and none had a grossly exaggerated LSA. CONCLUSIONS: Signs and symptoms indicative of a tethered spinal cord appear to correspond to increases in the LSA. Although the authors do not advocate the use of LSA measurement as the sole indicator of a tethered spinal cord, this imaging finding may prove useful to the clinician as an indication of the tethered spinal cord or as an adjunct in verifying symptomatology in patients harboring a lipomyelomeningocele.

Adolescent↗
PubMedDate not supplied

The effect of tethered cord release on scoliosis in myelomeningocele.

To understand better the effects of detethering on scoliosis in persons with myelomeningocele, 21 patients with spinal dysraphism and scoliosis who had undergone a detethering procedure were retrospectively reviewed. Fourteen girls and seven boys were followed for an average of 5 years (range, 2-10). Three patients had thoracic, 12 had lumbar, and six had sacral level myelomeningocele. Three patients (14%) had curves that improved, and six (29%) stabilized. Twelve patients (57%) progressed >10 degrees. Eighty-six percent of patients with initial curves of >40 degrees and 100% of thoracic level patients went on to require spinal fusion. Eight complications occurred in six patients. Although patients with curves <40 degrees may have some improvement after a detethering procedure, those presenting with curves >40 degrees or thoracic neurologic levels had no improvement in their scoliosis.

Adolescent↗
PubMed1992

High cervical intradural lipoma--case report.

A rare high cervical intradural subpial lipoma unassociated with spinal dysraphism manifested by a slowly progressive myelopathy simulating Brown-Sequard syndrome in a 37-year-old male. The diagnosis was based on neuroradiological imaging. Intraoperative recording of somatosensory evoked potentials showed recovery of the prolonged N20 latency, indicating adequate decompression was achieved.

Adult↗
PubMed1995

Intramedullary spinal cord lipomas.

Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.

Adolescent↗
PubMed2005

[Intramedullary rupture of a dermoid cyst of the conus: a case report].

The authors report the case of a 40 year old patient with history of cauda equina syndrome and pyramidal signs due to intramedullary rupture of a congenital dermoid cyst of the conus without associated spinal dysraphism. Diagnosis was suggested at magnetic resonance imaging (MRI) and confirmed by histological examination and percutaneous aspiration of the intramedullary cavity.

Adult↗
PubMed2000

The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation.

PURPOSE: Spinal dysraphism is the most common cause of neurogenic bladder dysfunction in newborns. Urodynamic findings in these patients include uninhibited bladder contractions, bladder areflexia, decreased compliance and detrusor-sphincter dyssynergia. Early urodynamic studies are recommended for spina bifida to help identify bladder characteristics that may cause a risk of upper tract deterioration. We recently evaluated a new early type of intervention involving closure of the neural tube defect during gestation in 25 patients at our institution. We hypothesize that this procedure results in decreased exposure of the spinal cord to amniotic fluid, which may improve neurological function. To date we have evaluated 16 of the 25 patients with video urodynamics. We compared the results to those in the literature on patients with myelomeningocele and without prenatal intervention. MATERIALS AND METHODS: We performed urodynamic testing in 16 patients with a mean age of 6.5 months, including cystometrography, fluoroscopic evaluation of filling and voiding, pelvic floor electromyography and post-void residual urine measurement. In addition, we retrospectively reviewed renal ultrasound, voiding cystourethrography, catheterization need, number of urinary tract infections and medication in these cases. RESULTS: Uninhibited detrusor contractions and an areflexic bladder were identified in 6% and 43% of patients, respectively, while 19% had decreased compliance and 75% had leak point pressure greater than 40 cm. water. Mean bladder capacity was 40 cc and 31% of patients had much lower capacity than expected for age. Previous renal ultrasound and voiding cystourethrography showed evidence of upper tract dilatation and reflux in 2 cases, respectively. Intermittent catheterization and anticholinergic therapy were required by 1 patient each and 1 had a significant urinary tract infection. CONCLUSIONS: Urodynamic findings in this population are comparable to those previously reported in the literature in patients with spina bifida without prenatal closure of the spinal defect. The lower incidence of urinary tract infection and reflux in our study probably represents more aggressive early urological management rather than neurological improvement. These urodynamic studies were performed early in life and future evaluation may ultimately reveal improved bladder function compared with that in others with myelodysplasia. However, at this time we recommend that patients who undergo spinal cord defect closure during gestation be evaluated and treated in the same manner as those with myelomeningocele but without fetal intervention.

Child↗
PubMed1994

Tethered cord syndrome: the low-lying and normally positioned conus.

We have previously proposed the existence of the tethered cord syndrome in the presence of a conus medullaris in the normal position. Our 12-year series of 73 patients with the tethered cord syndrome included 13 patients in whom the spinal cord terminated at or above the L1-L2 disc space. We compare the characteristics of these 13 with those of the 60 patients whose spinal cord terminated below the L1-L2 disc space. The parameters for comparison included neurological status at presentation and follow-up, the presence of cutaneous stigmata of occult spinal dysraphism, vertebral anomalies, and others. The frequency of occurrence of each parameter in the normally positioned group was essentially the same as its occurrence in the low-lying group.

Adolescent↗
PubMed1989

Magnetic resonance imaging of lipomyelomeningocele and tethered cord.

The operative and magnetic resonance image (MRI) findings of 25 patients with the diagnosis of lipomyelomeningocele and/or tethered cord were compared. Postoperative MRI scans of eight patients, five of whom were in stable condition, were also compared with the preoperative studies. In this review there was one false negative MRI scan and four MRI scans in which the relationship of the lipoma to the conus or filum was not demonstrated accurately. In six patients, incidental intramedullary cystic lesions at the conus were identified by MRI scan. All eight postoperative (1 month to 2 years) scans demonstrated no change in the level of the conus from the preoperative study. MRI is an accurate screening modality in the initial diagnosis of occult spinal dysraphism. MRI was not useful in the postoperative evaluation of lipomyelomeningocele and the tethered cord, since the caudal, posterior displacement of the conus was unchanged in all studies.

Adolescent↗
PubMedDate not supplied

[The tethered cord syndrome].

The "tethered cord syndrome" as a complication of spinal dysraphism is probably more important than assumed earlier. An abnormally low position of the conus medullaris is caused by different anomalies: e.g. adhesions, lumbosacral lipoma, tight filum terminale. In some patients no skinny changes can be detected. A "tethered cord syndrome" should be considered, if neuromuscular skeletal changes as club-foot, scoliosis, muscular atrophy of disturbances of gait, sensibility or function of bladder and rectum are recognized. It is also a result of a inadequately operated meningomyelocele. For experienced examiners sonography is an interesting non-invasive diagnostic procedure during infancy. The diagnosis should be completed by spinal computerized tomography and myelography. Surgery should be performed prophylactically. The "tethered cord syndrome" is explained by case histories of the University Hospital of Children, Freiburg.

Child, Preschool↗
PubMed1996

Cervical intramedullary lipoma with unusual MRI features: case report.

Intramedullary (subpial) lipomas not associated with spinal dysraphism are very unusual [1]. A confident preoperative diagnosis is usually possible with MRI. We present a case of an isolated cervical intramedullary lipoma with unusual imaging morphology and imaging characteristics (distinctly low-signal on T2-weighted images) on MRI. An explanation for these features is proposed.

Adult↗
PubMed1984

Extradural spinal lipomas. Report of two cases and review of the literature.

The authors present two cases of extradural spinal lipomas causing spinal cord compression, not associated with spinal dysraphism. Sixty-nine cases of such tumours have been reported in the literature. It is important to distinguish between two histological types of these neoplasms: angiolipoma and true lipoma. They have a different distribution in relation to age and site of occurrence. The outcome can be quite variable considering the greater surgical problems that true lipomas can cause.

Adult↗
PubMed2002

Operative nuances of myelomeningocele closure.

ALTHOUGH ADVANCES IN prenatal care and diagnosis have reduced the incidence of spina bifida, repair of neural tube defects remains one of the standard cases encountered by pediatric neurosurgeons. The operative techniques used in closure of these congenital defects have remained essentially unchanged during the past 2 decades; however, the operative nuances for closure often are based on the variability in configuration and size of the open defects, which can present a challenge. Repair of the open spinal dysraphism may require only a simple closure, but extensive dissection and reconstruction of the neural, meningeal, fascial, and epidermal tissues, including the use of specialized vascularized flaps, may be required in some cases. In this article, we briefly describe the preoperative evaluation, decision process, and standard techniques for the repair of myelomeningocele and myeloschisis.

Dermatologic Surgical Procedures↗