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PubMed1983

Clinical indications for computer-assisted myelography.

Computer-assisted myelography (CAM) is a technique in which computed tomography (CT) is combined with the intrathecal administration of metrizamide to demonstrate the spinal cord and surrounding structures. This retrospective study of 139 CAMs performed at Emory University Hospital included 125 CAMs that were preceded by routine metrizamide myelography (secondary CAM). The remaining 14 CAMs were primary studies without concomitant myelography. These CAMs and conventional myelograms were reviewed to provide indications for the use of CAM as a replacement for other radiodiagnostic studies or as an adjunct to radiological diagnosis. Eighty-one CAMs (58%) were positive for some pathological process. Of the 69 secondary CAMs demonstrating a pathological condition, 46 (67%) revealed some aspect of the lesion not apparent on routine metrizamide myelography. In no case with positive myelography was CAM negative. However, conventional myelography often added valuable diagnostic information. Although high resolution CT has allowed limited visualization of the spinal cord, CAM is often indicated for cases in which diagnosis requires more definition of cervicomedullary, extradural, intradural, extramedullary, and intramedullary lesions. Our clinical review found CAM to be extremely useful in the diagnostic evaluation of pathological conditions involving the spine and spinal cord and suggests clinical indications for the use of CAM based upon selected illustrative cases. Furthermore, CAM seemed to be superior to other radiological procedures in certain instances, such as in cases of spinal dysraphism and in the evaluation of lesions at the foramen magnum. Until more experience is obtained using spinal CT and CAM, CAM should not replace myelography altogether. The present study indicates that, rather than replacing conventional CT and myelography, CAM should be used as a primary study in situations where it has been shown to be superior and as a complementary study when other neurodiagnostic examinations are equivocal or nondiagnostic.

Adult↗

PubMedDate not supplied

[Cervicodorsal diastematomyelia in an adult].

A 44-year-old male had paraparesis which had began at age 20 years; a cleft spinal cord (diastematomyelia) was discovered at cervicodorsal level. The patient had nevus and hypertrichosis in the skin overlying the dysraphic spinal cord malformation. Both computer-assisted myelography and magnetic resonance scan showed the spinal cord segmentation and other associated disorders: bone spicula projecting between the 2 cordal halves, vertebral fusion defect and syringomyelic cavity. We emphasize the rarity of this abnormality in a so high spinal cord level and the onset of symptoms at adult age.

Adult↗

PubMed1994

Prevalence of incidental intraspinal lipoma of the lumbosacral spine as determined by MRI.

STUDY DESIGN: The prevalence and appearance on MRI of lipomas of the filum terminale was studied in a random population referred to MRI for evaluation of the lumbosacral spine. The MRI scans of 100 patients selected at random were retrospectively reviewed. OBJECTIVES: The study sought to determine the frequency and MRI appearance of incidental filum lipomas in a random population. SUMMARY OF BACKGROUND DATA: Postmortem studies have reported a 4%-6% incidence of occult fibrolipomas of the filum terminale in what were thought to be otherwise normal spinal cords. The improved resolution of MRI imaging allows the detection of small amounts of fat associated with the filum terminale that heretofore had not been demonstrated on radiologic imaging examinations. METHODS: The lumbosacral spine unenhanced MRI scans of 100 patients not previously operated upon were selected at random and were retrospectively reviewed by two of the authors (EB and JCM). RESULTS: Of the 100 MRI examinations reviewed, four patients were found to have small lipomas of the filum terminale. No spinal dysraphism or cord tethering were present in these four patients. Their clinical symptoms were related to disc herniation in two patients, spinal stenosis in one, and discitis in one. The lipomatous tissue was hyperintense to cerebrospinal fluid (CSF) on T1-weighted images and hypointense to CSF on T2-weighted images. CONCLUSIONS: Incidental lipomas of the filum terminale were present in 4% of 100 lumbosacral spine MRI examinations in a random population.

Adult↗

PubMed1992

[Cervicobulbar intramedullary lipoma. Apropos of a case with review of the literature].

The authors report a case of cervical intramedullary lipoma extending into the bulbomedullary junction. 99 cases of cervical and/or dorsal intramedullary lipomas without spinal dysraphism, reported since 1884, are reviewed. There is poor correlation between lipoma's length and age of onset. Only lipoma extended to all the spinal cord begin in the first years of life. Clinical presentation is in 58% of cases a slowly compressive myelopathy, a syringomyelic syndrome in 9.5% of cases, a Brown Sequard syndrome in 6.5% of cases or atypical clinical features in 26% of patients. Finding of a subcutaneous lipoma at the level of the lesion helps for diagnosis. C.T. scan and magnetic resonance imaging (M.R.I.) precise lipomatous constitution of the tumor, its limits and relations with neighbouring tissues. Surgery is necessary when clinical features are advanced. Surgery indication is debatable when neurological manifestations are poor or absent. Post operative course is generally good and is not related with type of intervention.

Brain Neoplasms↗

PubMed1990

Unique anomalies in cephalothoracopagus janiceps conjoined twins with implications for multiple mechanisms in the abnormal embryogenesis.

The anatomic features of female conjoined twins with the Janiceps type of cephalothoracopagus are described. Abnormalities included bilateral clefts of the alveolar arches, shared rudimentary mandible, high, arched clavicles, multiple rib deformities, single shared foregut and small intestine, absent large intestines, omphalocele, multicystic kidneys, hypoplastic lungs, interconnected aortas and neck vessels, single ovary with elongated uterus in each twin, displaced labia, abnormal segmentation of the vertebrae, spinal dysraphism, diastasis of the symphysis pubis, malrotated lower extremity, bilateral posterior dislocation of the hips, and club feet. There were two hearts with internal anomalies. Both spinal cords had a myelocele in the lumbar region. The abnormalities noted in previous reports of conjoined twins of this type are reviewed and compared. We propose that factors associated with conjoining, dysgenetic (developmental) defects, and deformations resulting from crowding in utero all may have been important in the abnormal development in this case.

Digestive System Abnormalities↗

PubMed2000

Intramedullary spinal epidermoid cyst.

Intramedullary epidermoid cysts of the spinal cord are rare tumours, especially those not associated with spinal dysraphism. Around 50 cases have been reported in the literature. Of these, only seven cases have had magnetic resonance imaging studies. We report two cases of spinal intramedullary epidermoid cysts with MR imaging. Both were not associated with spina bifida. In one patient the tumour was located at D4 vertebral level, while in other within the conus medullaris. The clinical features, MR imaging characteristics and surgical treatment of such rare intramedullary benign tumours are discussed, and the relevant literature reviewed.

Adolescent↗

PubMedDate not supplied

[A rare cause of occult neuropathic bladder in children: the tethered cord syndrome].

The tethered cord syndrome is a form of spinal dysraphism in which a short, thickened filum terminale prevents the ascent of the conus medullaris and intraspinal lipoma causes compression upon the caudal part of the spinal cord. In pediatric age the first symptoms are urological: urinary incontinence and infection. Diagnosis is performed with urodynamic studies followed by nuclear magnetic resonance. Tethered cord syndrome is no obvious cause of a neurogenic bladder. When this pathological conditions is recognized properly and an operation is performed early the likelihood of improvement is rather high. The authors report a case of tethered cord syndrome in a girl seven years old.

Child↗

PubMedDate not supplied

[Neurosurgical treatment of children with spina bifida associated with congenital spinal lipoma in the lumbosacral region].

This paper presents twelve neurosurgically treated children with spina bifida associated with congenital spinal lumbosacral lipoma over a 3-year period. The introduction deals with various types of lipoma, their development, clinical presentation, local findings in children with spinal lipoma, as well as with the significance of neurosurgical treatment. The age span of children with occult spina bifida and spinal lumbosacral lipoma was from 3 months to 2 years, and there were seven girls and five boys. In all the twelve children, a smaller or bigger fatty mass in the lumbosacral area was noticed immediately after birth, gradually increasing in size. Neurologic examination revealed weakness of lower extremities, with somewhat atonic sphincters, in only two of the children. Spinal dysraphism was demonstrated by plain roentgenograms in all the twelve children, while CT-scans, CT-myelography and recently MR-scans, confirmed the diagnosis of spinal lipoma. In all the twelve children the spinal lipoma and the adherences between the lipoma and the low-lying conus or an enlarged filum terminale were removed by the neurosurgical excision. Follow-up of children 6 and more months following the surgery showed improvement of their condition. Spina bifida associated with congenital spinal lumbosacral lipoma is a condition that although benign in histology, can have devastating effects if not surgically corrected early. Children who are asymptomatic at the time of repair will tend to remain asymptomatic. Those who are not repaired early will develop and show subsequent deterioration as time progresses.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

PubMed2000

Split cord malformations in myelomeningocele patients.

Split cord malformations (SCMs) may occur in conjunction with myelomeningoceles, and are often ignored or misdiagnosed, potentially causing spinal cord tethering. In this paper, we study the incidence and clinical significance of such an association. We have retrospectively reviewed the medical records and radiographs of 20 myelomeningocele patients who had a SCM. These comprised at least 6% of our myelomeningocele patients. Five of the 20 had simultaneous repair of both lesions at birth. The other 15 were diagnosed with the SCM in a delayed fashion (mean age 4.4 years). Clinical presentations that prompted a diagnostic investigation included hypertrichosis (1), pain (2), routine radiographic follow-up (2), neurourological deterioration (10) and progressive scoliosis (5). In 17 of the 20 patients, the SCM involved the placode or was within one level of it. Fifteen of these were in the area of the placode or one segment above it. Five of the 20 patients had hypertrichosis and 15 of the 20 patients had a bony midline septum (i.e. type I SCM). Several accompanying spinal dysraphic lesions also contributed to the tethering: thickened (previously inconspicuous) filum terminale (6), syringohydromyelia (5) and a neurenteric cyst with a benign teratoma (1). Arachnoiditis secondary to the prior myelomeningocele repair was found, as expected, in all 15 patients. However, in virtually all patients, there was also evidence of tethering at the level of the SCM. Long-term follow-up showed stabilization of preoperative symptoms and signs, whereas complications of the operation and clinical evidence of retethering were uncommon. Myelomeningocele patients should be screened with clinical examinations looking for hypertrichosis and with spinal radiographs preoperatively looking for evidence of SCM (bony midline septum and/or interpedicular widening not due to the myelomeningocele). Intraoperatively, the placode and the rostral spinal cord segment should be carefully inspected for an SCM and other dysraphic lesions.

Adolescent↗

PubMed1999

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.

Arteriovenous Fistula↗

PubMed1987

Short latency somatosensory-evoked potentials in children--Part 2. Effects of spinovertebral disorders.

Surface-recorded spinal and cortical somatosensory-evoked potentials (SEPs) following tibial or median nerve stimulation were studied in 26 children aged from 12 months to 13 years. Fifteen patients had caudal or dysraphic spinal malformations, often with a tethered cord syndrome, while the remaining patients had spinal tumors (4), syringomyelia (3), spinal canal stenosis (2), or demyelinating syndromes (2). Somatosensory-evoked potential abnormalities were found in 13 patients. Loss of a SEP at or rostral to the site of spinal pathology was the most common finding. Prolongation of central conduction times or delay in SEP peak latencies was documented in only four patients. Correlations between clinical and electrophysiological findings revealed that all patients with sensory dysfunction had abnormal SEPs, whereas patients with motor or sphincteric dysfunction, but intact sensation, usually had normal SEPs. These relationships between SEPs and clinical findings were constant irrespective of the etiology and location of the spinal pathology. The results from this study suggest that although SEP studies provide information regarding the somatosensory pathways, their clinical utility as a method of assessing spinal cord function in children with spinal disorders is limited by technical, clinical, and anatomical factors.

Adolescent↗

PubMed1999

Neurogenic bladder in infants born with anorectal malformations: comparison with spinal and urologic status.

BACKGROUND/PURPOSE: Spinal dysraphism and neurovesical dysfunction (NVD) frequently are associated in children with anorectal malformations (ARM). This study compares the urodynamic data from a selected group of patients with the results of their spinal and urologic imaging studies. METHODS: Twenty-six children (20 with isolated imperforate anus and six with persistent cloacal malformations) were investigated. All patients were evaluated with leak point pressures (LPP), renal ultrasound scan, and voiding cystourethrography. Eight children had urodynamics performed before and after posterior sagittal anorectoplasty (PSARP). The spinal cord was assessed using ultrasonography or magnetic resonance imaging. Current urologic status was obtained to provide long-term follow-up. RESULTS: Twenty-one of 26 children demonstrated elevated LPPs above the established normal value of 40 cm H2O, and 15 of these children had normal spinal imaging study findings. Uroradiographic studies findings showed that 12 of 21 children with elevated LPPs had hydronephrosis or vesicoureteral reflux with seven of these patients having normal spinal cords. LPPs in the eight patients with pre- and postoperative studies were 74 +/- 14.7 cm H2O and 68 +/- 31.8 cm H2O (mean +/- SD), respectively. CONCLUSIONS: These urodynamic and radiographic data confirm that NVD (elevated LPP) is common in patients with anorectal malformations despite normal spinal cords. Bladder dysfunction does not appear to be a sequelae of a properly performed PSARP. Patients with ARM and any uroradiographic or clinical urologic abnormality should undergo urodynamic testing even though the spinal studies are normal.

Anus, Imperforate↗

PubMed2003

Lumbar split cord malformation and Klippel-Feil syndrome.

The authors report a patient with type II split cord malformation (SCM) and Klippel-Feil syndrome (KFS). Considering our review of the medical literature and present case report, we believe lumbar SCM and KFS in the same patient not to be coincidental findings but rather embryologically related entities. We propose that KFS in at least one of its phenotypes represents a forme fruste of occult spinal dysraphism.

Adolescent↗

PubMed1990

Intradural lipoma in a child.

We report the case of a 5-year-old Nigerian boy in whom conus medullaris and cauda enquina compression was due to an intradural lipoma, a rare tumour of the spinal canal in the tropics. The literature on the subject is briefly reviewed and the differential diagnoses are discussed. The occurrence of the lesion in this child, without other features of spinal dysraphism, is noteworthy.

Child, Preschool↗

PubMed1986

[Tethered spinal cord syndrome: report of 2 cases].

The tethered spinal cord syndrome is a disease that is part of the group of spinal dysraphisms, that was recently recognized as an individualized nosological entity, yet not frequently diagnosed among us. It is characterized by shortening and thickening of the filum terminale which prevents the ascent of the spinal cord into spinal canal, the conus medularis abnormally remaining in a low place. It is associated in all cases with spina bifida. The diagnosis is simple, once the disease is suspected. It is manifested by progressive motor or sensory deficit in the legs, urinary incontinence, scoliosis and leg or back pain, specially in young children. The plain lumbosacral RX always shows spina bifida. Myelography makes the diagnostic. It shows, basically, the negative image of the thickened filum terminale and the low placed conus medularis. Other exams which can help are the computerized tomography and the ultra-sound of the spinal cord. The surgical treatment is very simple and heals without sequels if done in due time. It consists in a sectioning of the filum terminale through laminectomy. Two cases diagnosed and treated at Hospital da Baleia, from Fundação Benjamin Guimarãcs, Belo Horizonte, are reported in this paper.

Abnormalities, Multiple↗

PubMed1995

Predicting outcome in the tethered cord syndrome: a study of cord motion.

Thirty-two children with spinal dysraphism have been studied with phase MRI to assess longitudinal cord motion. Seventeen children who were asymptomatic and who had normal or slightly decreased motion were not operated. Fifteen had signs and symptoms of spinal cord tethering as well as decreased cord motion and underwent surgery. None of the nonsurgical patients have become symptomatic. The children with markedly decreased cord motion did not improve after surgery. A trend was seen toward a better outcome in younger patients with slight or moderate decrease in cord motion. All children with worse outcome were previously operated meningomyeloceles who had markedly decreased cord motion and who were greater than 10 years old.

Adolescent↗

PubMed1986

Lipomyelomeningocele: prenatal diagnosis and management.

Spina bifida is one of the many congenital malformations possibly detectable in utero with ultrasound. Prenatal diagnosis can be useful in planning prompt, appropriate neonatal care of the infant. Lipomyelomeningocele (lipomyeloschisis) is a common subtype of spinal dysraphism. Prognosis for the infant with this skin-covered lesion is very good if appropriate care is provided early. Neonatal resection of these congenital lipomatous tumors is necessary to prevent tethering of the spinal cord that might lead to irreversible neurologic damage if not released. The prenatal ultrasonic appearance of lipomyelomeningocele is illustrated and the clinical presentation, implications, and neonatal neurosurgical care are reviewed.

Adult↗

PubMed1995

A dumbbell spinal lipoma presenting as a neck mass: CT and MR demonstration.

A case of histologically confirmed spinal lipoma without associated spinal dysraphism is presented. This tumor appeared lobulated, extending through an enlarged neuroforamen to form a so-called dumbbell tumor. In a review of the literature, there were found to be only four case reports of a dumbbell spinal lipoma. Our case was unique in that it manifested as a neck mass without neurological deficits. The fatty nature of the tumor allowed easy detection by computed tomography (CT), as well as by magnetic resonance (MR) imaging. MR is judged to be superior to CT for preoperative planning by virtue of its superb intraspinal resolution and multiplanar capability, and should be the procedure of choice for evaluating spinal lipomas.

Child, Preschool↗