Concept: Conus medullaris
The spinal canal is frequently a source of difficulties, traps and diagnostic errors. Pitfalls related to artifacts are resolved by using appropriate sequences. Good knowledge of the appearance of certain particular anatomical structures (the cauda equina roots, the radicular veins of the lumbar spine and conus medullaris, the dorsal root ganglion) and of frequent variants (fibrolipoma of the filum terminale, common root sheaths, root cysts) will avoid a good many errors. Dilatation of epidural veins in intracranial hypotension can simulate the contrast enhancement of a tumour. An increase in epidural fat can induce pathogenic stenosis of the dural sheath.
Myxopapillary ependymoma (MPE) is a rare variant of ependymoma which usually occurs in the conus medullaris or the filum terminale. It is usually a single encapsulated lesion.
Spinal cord arteriovenous shunts (scAVSs) are a group of lesions located in the spinal cord itself or in the surrounding structures. The most common scAVSs are spinal dural arteriovenous fistulas (sDAVFs), which are acquired lesions. The pathogenesis of sDAVFs involves thrombosis and venous hypertension as trigger factors. Intradural scAVSs such as spinal cord arteriovenous nidus type malformations (AVMs) and pial arteriovenous fistulas are less common than sDAVFs and are considered to have a so-called ‘congenital’ origin. The association between different concomitant scAVSs is very rare and the association of sDAVFs with intradural scAVSs has been described in only a few case reports.
OBJECTIVE Tethered cord syndrome (TCS) is a neurosurgical disorder with varied clinical manifestations believed to result from vascular compromise due to stretch forces on the spinal cord. Conventional supine MRI findings may include a low-lying conus medullaris, thickened or fat-infiltrated filum terminale, or lipoma; however, imaging sensitivity and specificity for tethered cord can be low. The purpose of this study was to evaluate the utility of prone MRI in the diagnosis of tethered and retethered spinal cord. METHODS Medical records were reviewed in 41 patients who underwent surgical release of tethered cord and in whom preoperative prone MRI sequences were available. Patients were divided into Group 1 (new TCS diagnosis) and Group 2 (recurrent TCS after previous untethering). Absolute conus ventral motion and motion as a percentage of canal width between supine and prone positions was measured in these 2 groups via sagittal T2-weighted sequences; these groups were compared with 30 consecutive patients (Group 3) who were classified as the normal control group. RESULTS The mean ventral motion was as follows: Group 1 (absolute: 0.5 ± 0.5 mm [range 0-2.4 mm]; canal percentage: 3.7% ± 3.9% [range 0%-16.3%]); Group 2 (absolute: 0.4 ± 0.7 mm [range 0-2.6 mm]; canal percentage: 2.2% ± 3.7% [range 0%-14.0%]); and Group 3 (absolute: 3.4 ± 1.3 mm [range 1.4-5.6 mm]; canal percentage: 22.0% ± 7.2% [range 10.5%-36.1%]). Whereas 38/41 surgically treated patients with TCS had diminished (< 10% canal width) ventral motion on preoperative MRI, 30/30 controls had > 10% canal width motion. Sensitivity and specificity were thereby calculated as 92.7% and 100%, respectively. CONCLUSIONS In the present series, prone imaging is found to be a sensitive and specific tool, and the authors believe it may have a role as supportive evidence in the diagnosis of tethered and retethered spinal cord.
Filum terminale arteriovenous fistulae are a rare type of arteriovenous shunt generally characterized by a single direct communication between the artery of the filum terminale and a single draining vein. These intradural arteriovenous shunts are three times more common in men than women (mean age 55 years). Symptoms are related to venous congestion, vascular hypertension, and a putative chronic steal phenomenon which result in spinal cord ischemia and myelopathy. Interestingly, hemorrhage has never been reported as a mode of presentation. MRI demonstrates increased flow voids and T2 changes involving the conus and the lower spinal cord, and these findings are not dissimilar from those seen with the more common type 1 spinal dural arteriovenous fistulae. Thus conventional spinal angiography is necessary for a definitive diagnosis and to localize exactly the site of the fistula. Both surgical interruption of the fistula and endovascular embolization are safe and effective therapeutic modalities. However, because of the very small caliber of the feeding artery, endovascular therapy is often not feasible; and thus, surgery remains the method more commonly utilized for their treatment. Definitive treatment consists of obliteration of the direct arteriovenous shunt. In this review, we describe the anatomy, pathophysiology, clinical presentation, imaging, and treatment options of these less common intradural arteriovenous shunts.
OBJECTIVE Spinal lipomas are generally thought to occur as a result of failed primary neurulation. However, some clinical features cannot be explained by this theory. The authors propose a novel classification of spinal lipomas based on embryonic changes seen during primary and secondary neurulation. METHODS A total of 677 patients with occult spinal dysraphism underwent 699 surgeries between August 2002 and May 2015 at the National Center for Child Health and Development and Tokyo Metropolitan Children’s Medical Center. This group of patients had 378 spinal lipomas, including 119 conus spinal lipomas, 27 lipomyelomeningoceles, and 232 filum lipomas, which the authors classified into 4 types based on neural tube formation during embryonic development. Type 1 is defined as pure primary neurulation failure; Type 2 ranges from primary to secondary neurulation failure; Type 3 consists of secondary neurulation failure (early phase); and Type 4 is defined as secondary neurulation failure (late phase). The authors also review embryogenesis in secondary neurulation and analyze the clinical utility of the new classification. RESULTS There were 55 Type 1 spinal lipomas, 29 Type 2, 62 Type 3, and 232 Type 4. All filum lipomas fell into the Type 4 spinal lipoma category. Association with anorectal and/or sacral anomalies was seen in none of the Type 1 cases, 15 (52%) of Type 2, 35 (56%) of Type 3, and 31 (13%) of Type 4. Urogenital anomalies were observed in none of the Type 1 or Type 2 cases, 1 (2%) of Type 3, and 28 (12%) of Type 4. Anomaly syndromes were present in none of the Type 1 cases, 6 (21%) of Type 2, 3 (5%) of Type 3, and 16 (7%) of Type 4. Associated anomalies or anomaly syndromes were clearly observed only for Type 2-4 spinal lipomas encompassing failed secondary neurulation. Radical resection was feasible for Type 1 spinal lipomas. CONCLUSIONS Secondary neurulation of the spinal cord gives rise to the conus medullaris and filum terminale, which are often involved in spinal lipomas. Formation of spinal lipomas seems to be a continuous process overlapping primary and secondary neurulation in some cases. Association with other anomalies was higher in Type 2-4 spinal lipomas, which included failed secondary neurulation, than in Type 1 lipomas, with failed primary neurulation. On the other hand, radical resection was indicated for Type 1, but not for Type 2, spinal lipomas. The new classification of spinal lipomas based on embryonic stage has the potential for clinical use and agrees well with both clinical and surgical findings. The classification proposed here is still preliminary. Further studies and verification are necessary to establish its clinical utility.
- Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
- Published over 3 years ago
The filum terminale has oven been overlooked in the literature probably due to its small size and historical lack of research on its true morphology. However, this structure’s roll in the tethered cord syndrome has become more apparent. Therefore, the current comprehensive review seemed timely.
Myxopapillary ependymoma (MPE) is a slow-growing tumor occurring most often in adults. It originates from the filum terminale in the area of the conus medullaris and cauda equina, and is considered a benign lesion. Despite this classification, however, recurrence after both partial and gross total resection is well known. In the pediatric population, primary MPE seeding is well documented and treated through gross total resection, followed by irradiation. In adults, however, primary MPE seeding is not recognized. Only one prior report describes primary drop metastases into multiple spinal locations in an adult before resection of an MPE, and there are only 3 reports of such an occurrence cranially. The reason for this difference between pediatric and adult MPE remains unclear. We present here the case of a 32-year-old man with primary seeding of an MPE into multiple lumbosacral areas. The patient underwent gross total resection of the lesions and had an uneventful postoperative course. Primary seeding could be a sign of aggressive behavior in this tumor. Complete craniospinal magnetic resonance imaging (MRI) studies should be done before and after surgery in patients who present with a multifocal primary MPE. Furthermore, patients with a history of primary tumor seeding of MPE should be thoroughly evaluated radiologically. Unlike in pediatric population, the need for postoperative irradiation in adults is unclear, and further studies, particularly genetic ones, are warranted.
OBJECTIVE Cutaneous stigmata or congenital anomalies often prompt screening for occult spinal dysraphism (OSD) in asymptomatic infants. While a number of studies have examined the results of ultrasonography (US) screening, less is known about the findings when MRI is used as the primary imaging modality. The object of this study was to assess the results of MRI screening for OSD in infants. METHODS The authors undertook a retrospective review of all infants who had undergone MRI of the lumbar spine to screen for OSD over a 6-year period (September 2006-September 2012). All images had been obtained on modern MRI scanners using sequences optimized to detect OSD, which was defined as any fibrolipoma of the filum terminale (FFT), a conus medullaris ending at or below the L2-3 disc space, as well as more complex lesions such as lipomyelomeningocele (LMM). RESULTS Five hundred twenty-two patients with a mean age of 6.2 months at imaging were included in the study. Indications for imaging included isolated dimple in 235 patients (45%), asymmetrically deviated gluteal cleft in 43 (8%), symmetrically deviated (Y-shaped) gluteal cleft in 38 (7%), hemangioma in 28 (5%), other isolated cutaneous stigmata (subcutaneous lipoma, vestigial tail, hairy patch, and dysplastic skin) in 31 (6%), several of the above stigmata in 97 (18%), and congenital anomalies in 50 (10%). Twenty-three percent (122 patients) of the study population had OSD. Lesions in 19% of these 122 patients were complex OSD consisting of LMM, dermal sinus tract extending to the thecal sac, and lipomeningocele. The majority of OSD lesions (99 patients [81%]) were filar abnormalities, a group including FFT and low-lying conus. The rate of OSD ranged from 12% for patients with asymmetrically deviated gluteal crease to 55% for those with other isolated cutaneous stigmata. Isolated midline dimple was the most common indication for imaging. Among this group, 20% (46 of 235) had OSD. There was no difference in the rate of OSD based on dimple location. Those with OSD had a mean dimple position of 15 mm (SD 11.8) above the coccyx. Those without OSD had a mean dimple position of 12.2 mm (SD 19) above the coccyx (p = 0.25). CONCLUSIONS The prevalence of OSD identified with modern high-resolution MRI screening is significantly higher than that reported with US screening, particularly in patients with dimples. The majority of OSD lesions identified are FFT and low conus. The clinical significance of such lesions remains unclear.
Anatomical variations of the filum terminale (FT) have been described in association with split cord malformations (SCM) but they appear to be a rare finding in its absence. We report the first case in literature of a duplicated FT in a patient presenting with tethered cord syndrome (TCS) without any radiological evidence of SCM.