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OBJECTIVE Available data on management of sacral arteriovenous fistulas (sAVFs) are limited to individual case reports and small series. Management includes observation, endovascular embolization, or surgical ligation, with no clear guidelines on the optimal treatment modality. The authors’ objective was to report their multiinstitutional experience with management of sAVF patients, including clinical and radiographic characteristics and postprocedural outcomes. METHODS The electronic medical records of patients with a diagnosis of spinal arteriovenous fistula treated from January 2004 to December 2019 at the authors’ institutions were reviewed, and data were summarized using descriptive statistics, including percentage and count for categorical data, median as a measure of central tendency for continuous variables, and interquartile range (IQR) as a measure of dispersion. RESULTS A total of 26 patients with sAVFs were included. The median (IQR) age was 65 (57–73) years, and 73% (n = 19) of patients were male. Lower-extremity weakness was the most common presenting symptom (n = 24 [92%]), and half the patients (n = 13 [50%]) reported bowel and bladder sphincter dysfunction. The median (IQR) time from symptom onset to treatment was 12 (5.25–26.25) months. Radiographically, all patients had T2 hyperintensity at the level of the conus medullaris (CM) (n = 26 [100%]). Intradural flow voids were identified in 85% (n = 22) of patients. The majority of the lesions had a single identifiable arterial feeder (n = 19 [73%]). The fistula was located most commonly at the S1 level (n = 13 [50%]). The site where the draining vein connects to the pial venous plexus was seen predominantly at the lumbar level (n = 16 [62%]). In total, 29 procedures were performed: 10 open surgeries and 19 endovascular embolization procedures. Complete occlusion was achieved in 90% (n = 9) of patients after open surgery and 79% (n = 15) after endovascular embolization. Motor improvement was seen in 68% of patients (n = 15), and bladder and bowel function improved in 9 patients (41%). At last follow-up, 73% (n = 16) of patients had either resolution or improvement of the pretreatment intramedullary T2 signal hyperintensity. CONCLUSIONS T2 hyperintensity of the CM and a dilated filum terminale vein are consistent radiographic signs of sAVF, and delayed presentation is common. Complete occlusion was achieved in almost all patients after surgery, and endovascular embolization was effective in 70% of the patients. Further studies are needed to determine the best treatment modality based on case-specific characteristics.

OBJECTIVE Lipoma of the conus medullaris (LCM) causes neurological symptoms known as tethered cord syndrome (TCS). The symptoms can be seen at diagnosis and during long-term follow-up. In this report, pediatric patients with LCMs who underwent untethering surgery, under the policy of performing surgery if diagnosed regardless of symptoms, were retrospectively reviewed to evaluate long-term surgical outcomes. Possible risk factors for retethered cord syndrome (ReTCS) were evaluated in the long-term follow-up period. METHODS A total of 51 consecutive pediatric patients with LCMs who underwent a first untethering surgery and were followed for > 100 months were retrospectively analyzed. The surgery was performed with the partial removal technique. Pre- and postoperative clinical and radiological data were reviewed to analyze the outcomes of surgery and identify potential risk factors for ReTCS. RESULTS During follow-up, 12 patients experienced neurological deterioration due to ReTCS. The overall 10-year and 15-year progression-free survival rates were 82.3% and 75.1%, respectively. On univariate analysis, a lipoma type of lipomyelomeningocele (OR 11, 95% CI 2.50–48.4; p = 0.0014), patient age at the time of surgery (OR 0.41, 95% CI 0.14–1.18; p = 0.0070), and the mean patient growth rate after surgery (OR 2.00, 95% CI 1.12–3.41; p = 0.0040) were significant factors associated with ReTCS. Cox proportional hazard models showed that a lipoma type of lipomyelomeningocele (HR 5.16, 95% CI 1.54–20.1; p = 0.010) and the mean growth rate after surgery (HR 1.88, 95% CI 1.00–3.50; p = 0.040) were significantly associated with the occurrence of ReTCS. CONCLUSIONS More complex lesions and a high patient growth rate after surgery seemed to indicate increased risk of ReTCS. Larger prospective studies and registries are needed to define the risks of ReTCS more adequately.

OBJECTIVE This study aimed to explore the migration process of the conus medullaris (CM) in early infancy using infant MRI and to evaluate the application of MRI for locating the infant CM level. METHODS The authors retrospectively analyzed the CM level on the lumbosacral MR images of 26 term infants aged < 3 months who were classified into three groups according to age. The authors numbered the CM level in each patient and analyzed the range and average of the CM level of the cohort. The authors studied the linear correlation between CM level and postnatal days with linear regression analysis, 1-way ANOVA, and the least significant difference test. RESULTS The CM level ranged from the superior border of the L1 vertebra to the top third of the L3 vertebra. About 96.2% of infants had CM higher than the superior border of the L3 vertebra. On average, CM was located between the L1–2 intervertebral disc and the inferior border of the L2 vertebra (mean ± SD score 1.64 ± 1.14). The three groups had no significant statistical difference in CM level (F = 1.071 and p = 0.359; groups 1 and 2, p = 0.408; groups 1 and 3, p = 0.170; groups 2 and 3, p = 0.755). CM level had no linear regression correlation with postnatal days within the first month (r2 = 0.061, F = 0.654, p = 0.438) or within the first 3 months (r2 = 0.002, F = 0.056, p = 0.816). CONCLUSIONS The CM level reaches the normal adult level by birth in term infants and does not ascend during childhood. On average, the CM was between the L1–2 intervertebral disc and the inferior border of the L2 vertebra in term infants. Considering the possibility of physiologically low-lying CM, the authors agree that normal CM is located above the L3 level in term infants and CM at the L3 level could be equivocal and should be investigated with other clinical data. The study data suggest that MRI is an accurate and valuable method for determining the CM level in term infants.

OBJECTIVE Although evaluating tissue elasticity has various clinical applications, spinal cord elasticity (SCE) in humans has never been well documented. In this study, the authors aimed to evaluate the impact of compression on human SCE in vivo. METHODS The authors prospectively assessed SCE using intraoperative shear wave elastography (SWE). All consecutive patients undergoing spinal cord (SC) decompression (laminectomy or corpectomy) between June 2018 and June 2019 were included. After intraoperative exposure of the patient’s dura mater, at least three SWE measurements of the SC and its coverings were performed. Intraoperative neurological monitoring in the form of motor and somatosensory evoked potentials was utilized. Cases were divided into two groups based on the state of SC compression following bone removal (laminectomy or corpectomy): patients with adequate decompression (the decompressed SC group [DCG]) following bone removal and patients with remining compression, e.g., compressing tumor or instability (the compressed SC group [COG]). RESULTS A total of 25 patients were included (8 females and 17 males) with a mean age of 48.28 ± 21.47 years. Most cases were degenerative diseases (10 cases) followed by tumors (6 cases), and the compression was observed at cervical (n = 14), thoracic (n = 9), and conus medullaris (n = 2) levels. The COG (6 cases) expressed significantly higher elasticity values, i.e., greater stiffness (median 93.84, IQR 75.27–121.75 kPa) than the decompressed SC in DCG (median 9.35, IQR 6.95–11.22 kPa, p < 0.001). Similarly, the compressed dura mater in the COG was significantly stiffer (mean ± SD 121.83 ± 70.63 kPa) than that in the DCG (29.78 ± 18.31 kPa, p = 0.042). Following SC decompression in COG, SCE values were significantly reduced (p = 0.006; adjusted for multiple comparisons). Intraoperative monitoring demonstrated no worsening from the baseline. CONCLUSIONS The current study is to the authors’ knowledge the first to quantitatively demonstrate increased stiffness (i.e., elasticity value) of the human SC and dura mater in response to external compression in vivo. It appears that SCE is a dynamic phenomenon and is reduced following decompression. Moreover, the evaluation of human SCE using the SWE technique is feasible and safe. Information from future studies aiming to further define SCE could be valuable in the early and accurate diagnosis of the compressed SC.

Introduction To the best of our knowledge, here we present two post-COVID19 longitudinally extensive transverse myelitis (LETM) with atypical presentations CASE PRESENTATIONS: A 44-year-old male who did not have any previous medical condition and a 73-year-old male foreigner who did not have any disease other than type 2 diabetes mellitus were admitted to our neurology clinic in the same period with similar clinical presentations of transverse myelitis. Upon admission, paraplegia and urinary-fecal incontinence were observed in their neurological examination. Neurological complaints had started within approximately 3-4 weeks following the resolution of the COVID-19 infection. Thoracic lower segment LETM was observed on spinal magnetic resonance imaging (MRI) in one of the patients, and long segment myelitis extending from the lower thoracic segment to the conus medullaris was observed in the other one. No significant diagnostic positivity was present in their diagnostic evaluation. In both cases, we assume a post-infectious etiology in terms of secondary immunogenic overreaction following COVID-19. Conclusion Our patients improved with multiple treatments such as methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Whether post-infectious myelitis behaves differently from other viral infections after COVID-19 is currently unclear. Long lag times appear to be a post-infectious neurological complication resulting from the host response to the virus.

OBJECTIVE To explore the migration process of the conus medullaris (CM) and propose a normal range of CM levels during the third trimester. METHOD We retrospectively collected the ultrasonographic and clinical data of 588 fetuses during the third trimester. We located the CM and assigned scores. One-way analysis of variance and linear regression analyses were used to statistically analyze CM migration. Statistical significance was set at p

The H3K27M-mutant spinal cord gliomas are very aggressive with a dismal prognosis, very few cases have been reported in the thoracic spinal cord and conus medullaris, and it is extremely rare with morphological features of pilocytic astrocytoma.A 20-year-old man presented with thoracolumbar pain, progressive paraparesis, and urinary incontinence. Magnetic resonance imaging revealed an intramedullary solid-cystic lesion from D9 to conus medullaris. Subtotal resection was performed, restricted by the indistinct margins and the decline of the motor evoked potential during the surgery. Pathologic findings revealed a pilocytic astrocytoma with anaplastic features. However, a further assessment determined a diffuse midline glioma H3K27M-mutant, and adjuvant chemoradiotherapy was administered. After seven months of progression-free survival, the paraparesis worsened; at twelve months of follow-up, the patient developed paraplegia, and at 24 months the patient remains alive without any neurologic functions distal to the tumor and he is still under adjuvant treatment.The H3K27M-mutant spinal cord glioma is a very infrequent tumor with a wide variety of histological presentations even as indolent as pilocytic astrocytoma, which should be considered in spinal cord tumors, especially if there are clinical, histological, or radiological data that suggest aggressiveness. On the other hand, the fast progression led to the loss of complete neurological function distal to the tumor, in spinal tumors could explain a not so poor prognosis as it is in functionally and vital structures.

A 65-year-old right-handed retired male firefighter presented following a fall. He had a 3-month history of scrotal paraesthesia that had insidiously evolved to bilateral leg weakness. Following hospital admission, he developed acute urinary retention requiring catheterisation. He had a history of ulcerative colitis, which was well controlled with mesalazine. He was an ex-smoker and did not drink alcohol. Cranial nerve and upper limb examinations were unremarkable. On lower limb examination, tone was normal. He had mild (Medical Research Council grade 4/5) bilateral and symmetrical weakness involving hip and knee flexion, and ankle dorsiflexion. There was a sensory level to T7. Joint position was impaired in the lower limbs, and vibration sense was absent below the xiphisternum. Knee and ankle jerks were absent, and plantar responses were mute. Although there were no obvious upper motor neurone signs, three clinical signs on lower limb neurological examination suggested localisation to the spinal cord. Firstly, the presence of a sensory level at T7 implied involvement of the thoracic cord. Secondly, the pyramidal pattern of weakness (flexors preferentially affected more than extensors in the lower limbs) suggested corticospinal tract dysfunction, and finally, the acute sphincter dysfunction in the context of lower limb weakness pointed to involvement of the lower cord. It is important to keep in mind that peripheral lesions affecting the cauda equina may also present with a sphincter disturbance, but this would not explain the sensory level. Cord pathologies can present without upper motor neurone signs particularly in the acute phase (spinal shock). However, this patient’s symptoms had started 3 months before admission. Conus medullaris lesions often have a mixture of upper and lower motor neurone signs with early sphincter dysfunction. Nevertheless, if this patient has a lesion involving the conus medullaris it would have to extend upwards to T7 to explain the …

Background Spinal schwannomas that arise from spinal nerve root sheaths are the most common intradural extramedullary spinal tumours and are often accompanied by nerve roots or spinal cord irritation symptoms. The phenomenon of spinal schwannoma causing subarachnoid haemorrhage (SAH) is rare, with ependymoma of the conus medullaris accounting for most cases. Case report A 45-year-old man was admitted to our hospital due to progressive lower limb weakness and sudden back pain after hard physical work. The patient had not been able to walk for 2 hours upon admission. An emergency magnetic resonance imaging (MRI) scan showed that the spinal cord at the C6-T4 level was severely compressed by a subdural mass. During the emergency operation, exploration of the dura and arachnoid mater revealed a fresh blood clot covering a tumour located on the ventral side of the spinal cord. The size of the tumour was approximately 3 × 2 × 1 cm without adhesion to the surrounding tissue, but the drainage vein was ruptured. Postoperative pathology showed that the tumour was a schwannoma with areas of fresh haemorrhage and focal necrosis. Conclusions Spinal schwannomas presenting with SAH are rare events. In our opinion, spinal pathology with rapid progression of neurological symptoms requires early diagnosis and emergency management. Complete excision of haemorrhagic tumours is the goal of treatment to prevent recurrence, which can effectively avoid irreversible damage to the spinal cord resulting from spinal cord compression.

Perimedullary arteriovenous fistulas (PMAVFs) of the conus medullaris are rare and usually manifest with progressive myelopathy secondary to venous congestion resulting from retrograde arterialization of the draining vein into the spinal cord. We present a rare case of conus PMAVF presenting with remote intramedullary spinal cord hemorrhage in the thoracic cord. A 37-year-old woman was transferred to our institute due to sudden severe pain in the left lower leg and weakness of the lower extremities following progressive paresthesia of the lower extremities. Magnetic resonance imaging of the thoracic and lumbosacral spine revealed spinal cord congestion extending from the conus medullaris to the level of T6 with intramedullary hemorrhage at the level of T8–9 on the left side of the spinal cord. There were abnormal serpiginous intradural flow voids along the anterior surface of the spinal cord extending from the level of L2 to the lower cervical with venous varix at the level of T8–9, probably being the source of hemorrhage. Spinal angiography confirmed conus PMAVF at the distal end of the conus medullaris supplied by the sulco-commissural artery arising from the enlarged anterior spinal artery originating from the left T11 intercostal artery with cranial drainage through the dilated anterior spinal vein into the tortuous perimedullary veins up to the lower cervical level. The patient underwent successful endovascular treatment with N-butyl cyanoacrylate and had gradually improved until being ability to walk independently without residual pain of the left lower leg. We speculated that an increased venous flow into a varix may be considered an important risk factor of hemorrhage.

We report a case of a 40-year-old female patient admitted to the hospital due to lumbar pain that spread to both legs and was associated with weakness of the lower extremities. Magnetic resonance imaging revealed an intradural - extramedullary tumor at the level of the T12 - L2 vertebra. The lesion was over 7 cm in greatest diameter and compressed the conus medullaris. The patient underwent surgery to remove the entire tumor. Postoperative pathology confirmed the diagnosis of schwannoma. The symptoms resolved almost completely without significant complications.

Spinal epidermoid cysts (ECs) are benign slow-growing spinal tumors. The account for

Spinal Cord (SC) Anatomy is often assimilated to a morphologically encapsulated neural entity, but its functional anatomy remains only partially understood. We hypothesized that it could be possible to reexplore SC neural networks by performing live electrostimulation mapping, based on “super-selective” Spinal Cord Stimulation (SCS), originally designed as a therapeutical tool to address chronic refractory pain. As a starting point, we initiated a systematic SCS lead programming approach using live electrostimulation mapping on a chronic refractory perineal pain patient, previously implanted with multicolumn SCS at the level of the conus medullaris (T12-L1). It appeared possible to (re)explore the classical anatomy of the conus medullaris using statistical correlations of paresthesia coverage mappings, resulting from 165 different electrical configurations tested. We highlighted that sacral dermatomes were not only located more medially but also deeper than lumbar dermatomes at the level of the conus medullaris, in contrast with classical anatomical descriptions of SC somatotopical organization. As we were finally able to find a morpho-functional description of ‘Philippe-Gombault’s triangle’, in 19th century historical textbooks of neuroanatomy, matching remarkably with these conclusions, the concept of “Neuro-Fiber-Mapping” was introduced.

OBJECTIVE The background for this investigation was the dramatic course of a 14-year-old girl with a spontaneous hemorrhage in the area of the conus medullaris resulting in a complete cross-sectional syndrome with bladder and bowel dysfunction. Despite immediate surgical treatment, the patient showed close to no postoperative improvement. Subsequent histopathological examination of the removed masses revealed a cavernoma. To better understand the link between the site and symptoms of conus medullaris lesions, the authors performed a literature search and then histological examination of the conus medullaris of 18 cadaveric specimens from body donors. METHODS After a literature search regarding the histological features of the structure of the conus medullaris did not lead to satisfying results, the authors performed histological examination of the conus medullaris in 18 cadaveric specimens from body donors. The largest (a) and smallest (b) diameters of the conus medullaris were measured, noting individual variations in the distance from the caudal ending of the gray matter to the macroscopically visible end of the conus medullaris. Correlations of these differences with sex, body height, gray matter transverse diameter, and cross-sectional area at the end of the gray matter were analyzed. RESULTS Gray matter displayed in the form of a butterfly figure was found along almost the entire length of the conus medullaris. The specific slide containing the end of the gray matter was noted. The distance between the caudal ending of the gray matter in the conus and the macroscopical end of the conus medullaris was defined as the gray matter to cone termination (GMCT) distance. There were great individual variations in the distance from the caudal ending of the gray matter to the macroscopically visible end of the conus medullaris. Analysis of the correlations of these differences with sex, body height, gray matter transverse diameter, and cross-sectional area at the end of the gray matter showed no significant sex-specific differences in the GMCT distance. Patient body height and transverse diameter at the end of the gray matter were found to be correlated positively with the GMCT distance. Moreover, greater height also correlated positively with the cross-sectional area at the end of the gray matter. CONCLUSIONS This report is, to the authors’ knowledge, the first published description of the histological structure of the conus medullaris and can serve as the basis for a better understanding of neurological deficits in patients with a conus medullaris syndrome. Findings that gray matter can be detected far into the conus medullaris, with large individual differences in the endpoint of the gray matter, are important for operative care of intramedullary masses and vascular malformations in this area. It is therefore important to use electrophysiological monitoring during these operations.

Background: Spinal hamartoma is an extremely rare lesion for symptomatic spinal cord compression in adult population. Without any association of spinal dysraphism and neurofibromatosis, only 3 cases have been reported in the literature. Case description: A 40-year-old man presented with lumbago femorica for 6 months. Gradually the symptoms worsened and he developed features of Cauda equina syndrome. Magnetic Resonence Imagimg demonstrated a heterogeneously hyperintense intradural extramedullary mass in both T1 and T2WI, involving conus medullaris and cauda equina, opposite to L1. Patient underwent near total resection of lesion through posterior midline approach. Histopathological features were consistent with hamartoma. Patient neurologically improved and follow up MRI at 6 months showed static small nodular tumor residue. Conclusion: To the best of our knowledge, current report is the fourth case of spinal hamartoma in adult male, without any association of spinal dysraphism and neurofibromatosis .This may be the first reported case from Bangladesh.

Filar cysts (FCs) can be detected using ultrasound before or after delivery. They usually present as anechoic structures with a clear boundary just caudal to the lower end of the conus medullaris. They are generally considered a physiological variation and do not affect health. Some studies have pointed out that FCs in children can disappear spontaneously, as identified by ultrasound or magnetic resonance imaging. Three cases of FCs diagnosed by prenatal transabdominal ultrasound were reported in this study, and it was observed that FCs could disappear spontaneously in utero. The shortest time of disappearance was 4 weeks.

BACKGROUND: Unsuccessful primary neurulation is usually considered to be the main origin of spinal lipomatous malformations. Yet, we are not able to clarify many of its characteristics using this hypothesis. A new system of spinal lipomatous malformations categorization based on embryological development observed in the course of the first and second stages of neurulation process may be more realistic. OBJECT: The aim of this study was to introduce our experience in the surgical treatment of spinal lipoma correlated with the most recent classification by Morota. METHODS: This study is a retrospective clinical study of 39 cases of spinal lipoma who have been subjected to surgery from August 2007 to May 2019 in neurosurgery department in our institution. The authors classified them to four categories depending on neural tube evolution in the course of embryological growth. Category one is characterized by failing in the first stage of neurulation process; category two includes failing between the first and second stages of neurulation process; category three is characterized as failing in the beginning of the second stage of neurulation process; and category four is characterized as failing in the end of the second stage of neurulation process. We will evaluate the application of this new categorization system. RESULTS: Twenty-one cases of category one, ten cases of category two, six cases of category three and two cases of category four were included in the study. Filar spinal lipomatous malformations were included in category four. Concomitant anomalous disorders were detected only for categorizes 2–4 lipomatous malformations involving failing in the second stage of neurulation process. Only cases with category one lipomatous malformations underwent complete excision. CONCLUSION: Conus medullaris and filum terminale are usually affected in spinal lipomatous malformations and they are the products of the second stage of neurulation process. Evolution of spinal lipomatous malformations looks as an ongoing mechanism involving the first and the second stages of neurulation in some cases. Total excision was feasible for category one only.

The aim of the present systematic review and meta-analysis was to identify when the ascent of the conus medullaris occurs in human foetuses considering differences in evaluation methods and sample characteristics. Five databases were searched for relevant articles using different combinations of keywords. Article selection and data extraction were performed independently by two reviewers. Disagreements were resolved by a third reviewer. The variables were distributed into four groups according to the gestational age of the specimens: I (13 to 18 weeks); II (19 to 25 weeks); III (26 to 32 weeks); IV (33 weeks to the probable date of birth). Eighteen articles were included. The majority used imaging exams as the evaluation method. Cadaveric dissections were reported in the remaining articles. Only morphological studies were included in the meta-analysis. Significant ascent occurs between groups I and III as well as groups II and IV. Despite the considerable heterogeneity among the studies included in the present review, the findings enabled the determination that the conus medullaris reaches its normal birth level by the 26th week. Further analyses should be performed based on nationality and ethnicity to diminish the heterogeneity of the data.

Background "Diffuse midline glioma, H3 K27M-mutant" (DMG) mainly arises within the pontine, thalamic, and spinal cord regions. Because of the rarity of spinal cord gliomas, the general knowledge surrounding DMGs is mainly based on pontine and thalamic gliomas, whereas tumor location tends to influence the clinicopathological features and prognosis. Objective To determine the clinicopathological characteristics and molecular profiles of DMGs located in the spinal cord. Methods The clinical and molecular pathologic features and prognosis were comprehensively analyzed in a series of 44 patients with spinal cord DMGs. Results The median age was 36 yr, and 88.7% of patients (39/44) were adults (≥18 yr). Histopathologically, malignant grades included grade II (16 cases), grade III (20 cases), and grade IV (8 cases). Compared with patients with histological grade IV, patients with lower histological grade (grade II/III) were older (37 vs 24 yr, P = .020) and were associated with longer overall survival (24.1 vs 8.6 mo, P = .007). All 30 tested tumors were isocitrate dehydrogenase (IDH) wild type, and 96% of cases (22/23) presented with unmethylated O6-methylguanine-DNA methyltransferase. Univariate and multivariate analyses showed that histological grade and presurgery McCormick Scale scores were independent prognostic factors for overall survival, whereas extensive surgical resection and chemoradiotherapy were not significantly associated with improved survival. The most frequent anatomic locations were the cervical enlargement (C4-T1, n = 16) and conus medullaris (T12-L1, n = 13), which exhibited distinctive clinical characteristics and molecular features. Conclusion The findings provide guidelines for the evidence-based practice of the specialized management of spinal cord DMGs.

Central nervous system (CNS) toxoplasmosis is a well-known opportunistic infection in patients with AIDS. Spinal cord involvement and brain hemorrhage are very infrequent manifestations of CNS toxoplasmosis. In this report, we describe a patient with unknown AIDS presenting with bilateral headaches and new onset of bilateral leg weakness, diagnosed with CNS toxoplasmosis with a CSF polymerase chain reaction (PCR) for T. gondii, associated with spinal cord involvement (a central lesion in the conus medullaris) and multiple brain lesions with hemorrhage. The patient required therapy with pyrimethamine, sulfadiazine, and dexamethasone, with favorable outcomes after 30 days of therapy. Our case re-emphasizes the diagnosis of CNS toxoplasmosis in patients with advance HIV disease and spinal cord involvement. A high index of suspicion is needed, especially in HIV/AIDS patients not receiving HAART, who are seropositive for T. gondii, have a CD4+ count less than 100 cells/mm3, and present with symptoms consistent with spinal disease and even more with suggestive coincident brain lesions.

The authors describe a case of filum terminale arteriovenous fistula (FTAVF) in association with a large L2–L3 disc sequestration and diffuse lumbar arachnoiditis. A 64-year-old male manifested with chronic back pain and gait difficulty. Magnetic resonance imaging (MRI) of the thoracic and lumbosacral spine revealed spinal cord congestion extending from the conus medullaris to the level of T9. There was a large disc sequestration came from L2–L3 disc herniation. In addition, thickening, clumping, and enhancement of the entire cauda equina were noted, probably representing arachnoiditis. MR angiography (MRA) and spinal angiography confirmed FTAVF at the level of L5. The patient underwent laminectomy with lysis adhesions and obliteration of the fistula. His postoperative course was uneventful. MRI and MRA of the thoracolumbar spine obtained 4 months after surgery revealed complete obliteration of the fistula and significant resolution of spinal cord congestion. Enhancement of the cauda equina roots was no longer visible. Interestingly, the significant resorption of the sequestrated disc was documented on MRI. The formation of the FTAVF in the present study may result from severe spinal canal stenosis caused by a large disc sequestration blocking the rostral venous drainage of the fistula, or chronic inflammation, and adhesions of the caudal nerve roots from lumbar arachnoiditis. It seems that FTAVF may be of acquired origin by this evidence.

Giant cell ependymoma (GCE) is a slow-growing tumor that accounts for 9–13% of all ependymoma and occurs preferably at conus medullaris, cauda equina, and filum terminate. Squash cytology is a fast, simple, and reliable technique for intraoperative diagnosis. We present a case of GCE arising at the L5-S2 level and suprasellar region in a 17-year-old male who initially presented with low backache followed by headache and double vision. Intraoperative squash smears of the lumbosacral lesion were suggestive of high-grade glioma. Histomorphological and immunohistochemical profile favors the diagnosis of giant cell variant of myxopapillary ependymoma. He received craniospinal irradiation followed by a boost to the sellar-suprasellar and lumbosacral region through IGMT. After sometime, he developed papilledema and decreased vision, the suprasellar lesion was decompressed which on histopathology revealed similar features. GCE remains a diagnostic challenge on intraoperative squash smears due to its rarity. It has a good prognosis with a 5-year survival of 98.4% after total resection; however, our patient succumbed to illness due to post-operative residual lumbosacral mass and effects of the suprasellar lesion.

Objective: Neural tube defects are a heterogeneous and complex group of congenital central nervous system anomalies involving that involve the failed closure of the vertebral column. The spinal cord is frequently affected, and neural tube defects substantially increase the risk of disability and death within the first year of life. Neural malformations are often associated with abnormalities in other organ systems. This study presents our clinical experiences regarding the development and comorbidities of infants with neural tube defect and compares them with the literature. Materials and Methods: This retrospective study included 47 patients (24 females, 23 males) who underwent surgical treatment for neural tube defect in the neurosurgery department of Sivas Cumhuriyet University Faculty of Medicine Training and Research Hospital between 2000 and 2020. Results: Of the 47 patients, 34 (72.34%) had myelomeningocele and 13 (27.65%) had meningocele. Lesion location was thoracolumbar in 38 patients (80.85%), lumbar in 5 (10.63%), lumbosacral in 3 (6.38%), and cervical in 1 patient (2.12%). Twenty-seven patients (57.44%) underwent ventriculoperitoneal shunting due to hydrocephalus. Thirty patients (63.82%) had Chiari malformation type II, 13 (27.65%) had cranial anomalies, and 1 (2.12%) had Down syndrome. In 9 patients, the conus medullaris terminated at or below the L3 vertebral body (19.14%). Two patients (4.25%) died. Conclusion: Because of their multidimensional nature, neural tube defects have various effects on individuals, families, society, and the field of medicine. This study represents a small group of patients with neural tube defects. Cases should be included from other centers to compile more comprehensive data about patients with neural tube defects in Turkey.

Objective: To modify the current neural tube defect (NTD) classification for fetal medicine specialists, and to investigate the impact of prenatal ultrasound conus medullaris position screening on the detection rate of closed spinal dysraphism and pregnancy outcomes. Materials and methods: The clinical data of 112 patients prenatally diagnosed with neural tube defects in Taiji clinic from 2008 to 2018 were retrospectively analyzed. All cases were classified following the modified classification. We compared the detection rate before and after introducing the conus medullaris screening and pregnancy outcomes for NTD types. Results: Closed spinal dysraphism type prevailed in our sample (43.8%). The median gestational age at the time of detection for cranial dysraphism was 13.3 weeks, open spinal dysraphism was 22.0 weeks, and closed spinal dysraphism was 22.6 weeks. All cranial dysraphism (n = 43) and open spinal dysraphism cases (n = 20) had pregnancies terminated. For closed spinal dysraphism Class 1, the live-birth rate was 100.0% in the cases without other anomalies and 33.3% in the cases with other anomalies, respectively (X2 = 17.25, p

Spinal dysraphism often causes neurological impairment from direct involvement of lesions or from cord tethering. The conus medullaris and lumbosacral roots are most vulnerable. Surgical intervention such as untethering surgery is indicated to minimize or prevent further neurological deficits. Because untethering surgery itself imposes risk of neural injury, intraoperative neurophysiological monitoring (IONM) is indicated to help surgeons to be guided during surgery and to improve functional outcome. Monitoring of electromyography (EMG), motor evoked potential, and bulbocavernosus reflex (BCR) is essential modalities in IONM for untethering. Sensory evoked potential can be also employed to further interpretation. In specific, free-running EMG and triggered EMG is of most utility to identify lumbosacral roots within the field of surgery and filum terminale or non-functioning cord can be also confirmed by absence of responses at higher intensity of stimulation. The sacral nervous system should be vigilantly monitored as pathophysiology of tethered cord syndrome affects the sacral function most and earliest. BCR monitoring can be readily applicable for sacral monitoring and has been shown to be useful for prediction of postoperative sacral dysfunction. Further research is guaranteed because current IONM methodology in spinal dysraphism is still deficient of quantitative and objective evaluation and fails to directly measure the sacral autonomic nervous system.

Diastematomyelia or Split cord syndrome is a rare form of spinal dysraphism characterized by longitudinal splitting of spinal cord, conus medullaris or filum terminale to a variable extent. Presence of SCM is suggested by certain superficial markers like skin pigmentation, hemangioma, lipoma, dermal sinus and hypertrichosis. Meningocele or myelomenigocele may also be present. Affected children usually present with progressive sensorimotor symptoms and bowel and bladder dysfunction. Development of sensorimotor symptoms and progressive loss of function emphasis the need for antenatal diagnosis of the spinal deformities which paves way for early intervention and management thus minimizing the morbidity. Keywords: Diastematomyelia, Syrinx, Tethered cord, Newborn

A 67-year-old man presented with headache, middle back pain that radiated to both legs, and paresthesia in the right leg for 1 day. He had eaten raw shrimp 1 week previously. Over the next week after admission, he developed urinary retention and weakness in both legs. The numbness in his right leg expanded to below the umbilicus. Magnetic resonance imaging of the spinal cord showed myelopathy with minimal cord swelling at T9 to the conus medullaris and a hemorrhagic lesion from T10 to T11. A complete blood count on day 28 after the onset of symptoms revealed leukocytosis without eosinophilia and no white blood cells in his cerebrospinal fluid. Results of an immunochromatographic test kit were positive for Angiostrongylus cantonesis but negative for Gnathostoma spinigerum. After a 4-week course of albendazole combined with a tapering dose of dexamethasone, he achieved nearly complete recovery.

Lumbosacral lipomas (LSL) of the ‘dorsal’ type have been associated with more favourable outcomes compared with other conus region lipomas. We hypothesised that integrity of the conus on MRI underpins the improved prognosis in this subgroup of LSL patients. The definition of ‘dorsal lipomas’ included lipomas with attachment to the conus, but where the conus could be delineated on MRI (Morota type 1) as reported by Morota et al. (J Neurosurg Pediatr 19:428–439, 2017). Additional inclusion criteria included asymptomatic status at presentation, age >3 years at follow-up, and neurological and urological evaluation at presentation and at last follow-up. Lipoma extent and conus level were recorded. Outcome measures were the need for untethering surgery and neuro-urological status at last follow-up. Urological outcomes were defined by continence and efficacy of bladder emptying. Twenty-six children were included (median age 8.7 years). Conus level was low (at or below L2) in 92%. Nine required untethering surgery: 5 prophylactic, 4 because of clinical deterioration. Twenty-five children were continent at last follow-up, one had stress incontinence, and none required catheterisation. One had persisting ankle weakness after surgery requiring orthotic support. In LSL of the conus, visualisation of the conus on MRI is associated with good urological and motor outcomes. The integrity of the conus appears to be a more important prognostic factor than anatomical level. An observational approach to this group of LSL patients does not appear to compromise outcomes. These findings support a selective approach to untethering surgery.

Intramedullary spinal cord metastasis (ISCM) is a rare event in the course of advanced malignancy. Management of these lesions remains controversial. Recently, surgery for ISCM has been advocated for selected patients. We performed a retrospective analysis of the clinical course, complications, and outcome of 30 patients surgically treated for ISCM. Patients' age, histopathological diagnoses of primary cancer, tumor size, spinal location, and extramedullary tumor dissemination were collected. Preoperative functional status, pre- and postoperative neurological status, and extent of the tumor resection were also analyzed. Predominant tumor location was thoracic, followed by cervical and conus medullaris. Lung cancer constituted the majority of primary malignancies. In 9 cases, one of the indications for spinal surgery was to obtain a histopathological diagnosis. On admission, all patients presented with neurological symptoms suggestive of myelopathy. After surgery, 18 patients exhibited improvement of symptoms in terms of pain relief and partial recovery of motor and/or sensory deficits; 6 patients were unchanged, while 6 patients exhibited postoperative deterioration. Median survival time after surgery was 9.9 months. Age > 70 years old, presence of systemic metastases, preoperative neurological non functional status, and lung cancer as primary tumor were all factors associated with a worse survival prognosis. This study did not show a clear survival difference between gross total and subtotal ISCM tumor resection. Patients who underwent gross total resection had a worse functional outcome with respect to patients with only partial resection. Gross total resection with low morbidity must be the surgical target, but when not possible, subtotal resection and adjuvant therapy are a valid therapeutic option.

Spinal pial arteriovenous fistulas (spAVFs) are believed to be congenital lesions, and the development of a de novo spAVF has not been previously described. A 49-year-old female with a childhood history of vascular malformation–induced right lower-extremity hypertrophy presented in 2004 with progressive pain in her right posterior thigh and outer foot. Workup revealed 3 separate type IV spAVFs, which were treated by combined embolization and resection, with final conventional angiography showing no residual spinal vascular lesion in 2005. Ten years later, the patient returned with new right lower-extremity weakness, perineal pain, and left plantar foot numbness. Repeat spinal angiography demonstrated 2 de novo intertwined conus medullaris spAVFs.

We report an anatomical-based association between conus medullaris pial arteriovenous shunt that drain caudally towards the lumbosacral area with very delayed onset of an acquired lumbar epidural shunt, draining secondarily towards intradural veins and responsible for a venous congestive myelopathy with identical clinical symptoms. These patients require close clinical and imaging follow-ups in order to propose adequate treatments before onset of irreversible neurological deficits. MRA should include the lumbo-sacral area in its field of view.

Lipoma of the filum terminale (FL) is an abnormality in which fat is deposited in the filum terminale. This lipoma is often detected by skin abnormalities in the lumbosacral area such as a sacrococcygeal dimple. Some cases may develop tethered cord and become symptomatic. However, the genetic basis of FL is still unclear. This study aimed to determine whether there was a family history of FL or other forms of spina bifida among 54 families of 56 patients with FL and to examine whether there is a familial predisposition in FL. In addition, sex, age at diagnosis, presence of symptoms, presence of sacrococcygeal dimple, and the level of conus medullaris between familial and spontaneous cases were evaluated. Of the 54 families of FL patients, there were 48 siblings. Among the 48 siblings, 2 had “occult” FL. The frequency of FL among siblings was estimated to be 4.2% (2/48), which was significantly higher than the sum of previously reported cases of spontaneous FL (0.91%; p = 0.017). However, there was no significant difference in sex, age at diagnosis, presence of symptoms, presence of sacrococcygeal dimple, diameter of filum terminale, or level of conus medullaris between familial and spontaneous cases. To our knowledge, this is the first report on familial FL and examination of the frequency of FL among siblings. The high probability of FL among siblings of FL patients suggests that genetic factors may play a role in FL development.

Introduction: Spinal atypical teratoid/rhabdoid tumour (AT/RT) is exquisitely rare and constitutes 2% of all AT/RTs. Case Presentation: A 6-year-old boy presented with low backache for the last 5 months. MRI of the spine showed a 1.5 × 1.5 × 4.7 cm intradural extramedullary mass extending from D10 to D12, causing compression of the conus medullaris. With a preoperative diagnosis of ependymoma, a gross total resection (GTR) of tumour was performed. Post-operative histopathology showed AT/RT. The tumour cells were immunopositive for cytokeratin, epithelial membrane antigen, smooth muscle actin, and p53 and immunonegative for MIC2, desmin, glial fibrillary acidic protein, and INI1. Post-operative neuraxis MRI revealed post-operative changes (D10–D12) with a 9 mm enhancing lesion at L5-S1 junction suggesting drop metastasis. There was no lesion in brain. Cerebrospinal fluid cytology did not show any malignant cell. The metastatic work-up was normal. He received 3 cycles of chemotherapy with ICE regimen (ifosfamide, carboplatin, and etoposide). Subsequently, he received craniospinal irradiation (CSI)-36 Gy/20 fractions/4 weeks followed by focal boost to primary tumour bed and spinal drop metastasis-14.4 Gy/8 fractions/1.5 weeks. Thereafter, he received 3 more cycles of ICE regimen. End-of-treatment MRI spine showed post-op changes (D10–D12) and 38.9% reduction of the L5-S1 lesion suggesting partial response. Six monthly spinal MRI showed serial reduction of the metastatic lesion leading to complete response (CR) 1 year after completion of treatment. On last follow-up (30 months from the initial diagnosis), he was neurologically intact and in CR. Conclusion: Multimodality management comprising GTR of tumour, CSI followed by focal boost, and multiagent chemotherapy (ICE) can lead to successful outcome in patients with this rare and aggressive spinal tumour.

Intramedullary tuberculoma (IMT) of the conus medullaris is extremely rare. We present a case of intramedullary conus medullaris tuberculoma in which the diagnosis was based on there being very high levels of adenosine deaminase (ADA) in the patient’s cerebrospinal fluid (CSF) and improvement with antituberculous therapy. A 78-year-old man presented after having had a dull ache in both thighs and progressive paraparesis. The patient’s medical history included diffuse large B-cell lymphoma, which had undergone remission due to chemotherapy two years earlier, and long-term, well-controlled diabetes. A chest X-ray showed no evidence of tuberculosis. The results of CSF analysis were compatible with Froin’s syndrome. An initial diagnosis was made of an intramedullary tumor of the conus medullaris, based on magnetic resonance imaging (MRI). A myelotomy and multiple punch out biopsy were performed, and histopathology of the tissues revealed mild reactive gliosis. Due to the patient having high levels of CSF-ADA, IMT of the conus medullaris was suspected. The patient was treated with an 18-month course of antituberculous therapy. The dull ache gradually disappeared, and motor power improved slightly. A follow-up MRI of the lumbosacral (LS) spine revealed that the lesion had completely disappeared. Intramedullary tuberculoma of the conus medullaris should be considered in patients with underlying malignancy and no symptoms of systemic tuberculosis. CSF adenosine deaminase levels can be helpful in determining the presence of central nervous system tuberculosis when other systemic signs of disease are lacking.

Background Data:Myxopapillary ependymoma (MPE) is a low-grade variant of ependymoma that typically occurs in conus medullaris and filum terminale of the spinal cord. Only rarely do these tumors present at an extradural location in the sacrococcygeal region and even rarer is the development of these extradural tumors inside the spinal canal. The authors report a case of an intraspinal extradural MPE of the sacrum with relevant literature review on salient features and management. Study Design: Case Report. Purpose: To report a rare entity at an uncommon location. Case Report: A 12-year-old male child presented with chief complaints of low backache and radiation of pain in both lower limbs for six months. The patient also had a history of straining during micturition for one month. On examination, he was found to have mild weakness of bilateral ankle plantar flexion and knee flexion (grade 3). Ankle jerks were absent and there was evidence of sacral hypoaesthesia. Intraoperatively sacral lamina was found to be thinned out. Laminectomy was performed at L5 and sacrum, an extradural, fleshy lesion extending from L5 to S5 was identified, and gross total resection was achieved. There was no intradural expansion of the tumor. Results: Patient had an uneventful course and was discharged home on day 4. Conclusion: Intraspinal extradural MPE is a rare entity and should be kept in mind while diagnosing a midline lower back lesion. Treatment consists of gross total excision wherever possible coupled with radiotherapy. These tumors tend to recur locally and systemically and, thus, patients should be followed up periodically. (2020ESJ226)

Spinal teratoma with associated neuroendocrine tumour (NET) is a rare entity. To date there have been only three such cases reported in the literature. Information available to clinicians regarding clinical presentation, behaviour of this tumour over time, management options and follow-up, are as a consequence very limited. We present a case of a 37-year-old woman who was found to have an incidental lumbar spine teratoma with NET after presenting with a one-week history of acute lower back pain and radiculopathy. Magnetic resonance imaging (MRI) of the lumbosacral spine demonstrated an acute disc prolapse at the L4/5 level as well as incidental lesion at the L1/2 level causing effacement of the conus medullaris. Lumbar laminectomy with gross total resection of the intradural extramedullary lesion at the L1/2 level was performed with histopathology confirming the teratoma with NET. The objective of our case is to report our experience with this unique tumour and potential management implications.

To date, the description of the natural course of concurrent tethered cord syndrome with a low-lying conus medullaris and split cord malformation is lacking in the literature. We report a cohort of adult and pediatric patients with concurrent malformations and long-term follow-up. Patients with concurrent diagnoses of split cord malformation and tethered cord (radiographic evidence supporting clinical symptomatology) were identified between 2000 and 2020. Patients without sufficient documentation or at least 6-month follow-up were excluded. Nine patients were identified with an average of 8.9 years follow-up (range 2–31 years). The most common symptoms were radiating leg pain and lower extremity paresthesias, occurring in 44% of patients; and bladder/bowel dysfunction, worsening scoliosis, and acute motor deterioration were less common. Two patients were successfully treated conservatively for mild leg pain and paresthesias. For those who underwent surgery, all experienced symptomatic relief upon first follow-up. Two had late symptomatic recurrence; one 4 and 8 years after initial surgery; and the other, 11, 26, and 31 years after initial surgery. The rarity of concurrent split cord and tethered cord syndrome with a low-lying conus makes management difficult to formulate. This series supplements our knowledge of the long-term outcomes and lessons learned from the management of these patients. Approximately 25% of patients were managed conservatively and had symptomatic improvement. For surgically managed patients, with intractable pain or worsening neurological function, symptoms can still recur over a decade after intervention. Reoperation, however, can still be beneficial, can provide years of relief, and should be considered.

Introduccion: el ependimoma mixopapilar (EM) es un tumor glial ependimario que afecta predominantemente a los adultos jovenes, se origina en el filum terminale, la cauda equina y el cono medular. Caso clinico: se presenta el caso de una paciente en quien durante el estudio de un dolor radicular se logra detectar ependimoma mixopapilar gigante, el cual comprometia gran parte del hueso sacro, mostrando un comportamiento localmente agresivo con intensa osteolisis. Conclusion: se discutieron las posibles teorias que explican la presencia de estos tumores a nivel sacro, en especial la presencia de remanentes embrionarios de tejido ependimario en los niveles mas caudales del canal vertebral y los posibles mecanismos que explican el comportamiento agresivo de estos tumores histologicamente benignos. Rev.cienc.biomed. 2015;6(2):348-353 PALABRAS CLAVE Columna vertebral; Ependimoma mixopapilar; Neoplasia extradural; Sacro; Region sacrococcigea. SUMMARY Introduction: myxopapillary ependymoma (MP) is a glial ependymal tumor that mainly affects young adults. The tumor originates in the filum terminale, the cauda equina and the conus medullaris. Case record: the case presented in a patient in whom a giant myxopapillary ependymoma was detected during a medical checkup for root pain. The tumor involved the sacral bones showing a locally aggressive behavior with intense osteolysis. Conclusion: possible theories that explain the presence of these tumors in sacral bones were discussed, focusing in the presence of embryonic remnants of ependymal tissue in the most caudal levels of the spinal canal, and the possible mechanisms that explain the aggressive behavior of these histologically benign tumors. Rev.cienc.biomed.2015; 6(2):348-353 KEYWORDS Spine; Myxopapillary ependymoma; Epidural neoplasm; Sacrum;Sacrococcygeal region.

Background Tethered cord syndrome (TCS) is a clinical diagnosis of progressive neurologic aggravation of the lower spinal cord due to a traction on the conus medullaris. Untethering surgery is effective for most TCS; however, when anatomic variations of spinal cord and filum terminale (FT) exist, regular untethering may lead to a failed outcome. Case Description The authors present the case of a 45-year-old patient with TCS caused by duplicated FT with split cord malformation (SCM). Lumbosacral magnetic resonance imaging revealed a type II SCM with a significant low-lying conus medullaris. Laminectomy was performed. Neurophysiologic monitoring was used for nerve root identification and 2 thickened fila, which failed to respond on stimulation, were found during the surgery. Both fila were sectioned, and the diagnosis was finally confirmed by pathologic examination. Postoperatively, the patient's symptoms disappeared immediately and no neurologic sequela was found after surgery. Conclusions This is the first documented adult of duplicated FT with preoperative radiologic evidence and reported in association with SCM as a cause of TCS. When SCM exists, a careful observation for duplicated FT is warranted on preoperative magnetic resonance imaging and during surgery. Complete transection of the double FT under intraoperative neurophysiologic monitoring is the best treatment for this anomaly.

Background Spinal epidural arteriovenous fistulas (SEAVFs) are rare lesions with a low risk of hemorrhage. Most patients with lumbosacral SEAVFs with hemorrhagic events will develop a spinal epidural hematoma from epidural venous pouches. To the best of our knowledge, we have reported the first case of a lumbosacral SEAVF presenting with remote intramedullary hemorrhage in the conus medullaris. Case Description A 56-year-old man presented with sudden-onset severe paraparesis and bowel/bladder dysfunction. Magnetic resonance imaging of the thoracic and lumbosacral spine showed acute intramedullary hemorrhage in the conus medullaris surrounded by spinal cord congestion extending to T8, with perimedullary flow voids along the ventral and dorsal cord surfaces. Magnetic resonance angiography and spinal angiography confirmed the presence of a SEAVF with a large lumbosacral epidural venous lake supplied by dorsal somatic branches of bilateral L4 segmental arteries. Initially, the intradural venous drainage had been misinterpreted as a single route. The patient underwent surgical interruption of the dilated intradural draining vein after embolization of the feeding vessels. Another small intradural venous drainage route was found on the follow-up angiogram. The second operation with occlusion of the initial part of the epidural venous lake was sufficient to cure this lesion. Conclusions With the significant improvement of our patient after treatment of the SEAVF, we speculated that the intramedullary hemorrhage, which had occurred in the area of spinal cord edema, might have resulted from severe venous hypertension, rather than hemorrhagic infarction of the spinal cord. Careful evaluation of the angioarchitecture of SEAVFs is imperative to determine the most effective treatment modality.

Objective Conus medullaris teratomas are extremely rare, and the treatment experience has been limited. The purpose of the present study was to evaluate the clinical characteristics, radiological features, surgical outcomes, and prognosis of patients with conus medullaris teratoma. Methods We retrospectively reviewed the data from 39 patients who had undergone surgical resection for conus medullaris teratomas from January 2008 to December 2018. All the operations were performed by 1 senior doctor. The clinical features, pre- and postoperative magnetic resonance imaging findings, pathological features, treatment strategies, and outcomes were analyzed. The neurological status was evaluated using the modified Japanese Orthopaedic Association scale score. Results Of the 39 patients, the mean age was 30.9 years. Of the 39 patients, 20 were male and 19 were female. The symptom duration ranged from 0.3 to 252 months (mean, 61.6 months). Bladder and bowel dysfunction was the most common symptom (76.9%). Total resection was achieved in 25 patients (64.1%), subtotal resection in 11 (28.2%), and partial resection in 3 (7.7%). A mature teratoma was confirmed in all 39 patients. The neurological outcomes were improved 16 patients (45.7%), stable in 14 (40.0%), and aggravated in 5 (14.3%) at a mean follow-up of 62.7 months. Recurrence developed in 1 patient who had undergone subtotal resection. A second surgery with total resection was performed, and the patient's neurological symptoms were stable during follow-up. Conclusions Total surgical resection is the optimal treatment strategy for patients with conus medullaris teratoma. Safe maximum tumor removal and residual tumor inactivation using electrocoagulation are recommended when total resection cannot be achieved. Surgery can provide a low recurrence rate and an acceptable low complication rate.

Diastematomielia é um disrafismo espinhal oculto (HSD), descrito pela primeira vez em 1837 e representa 3,8% de todos os HSD. Poucos casos de diastematomielia cervical foram relatados, uma vez que a localização principal da doença é a coluna torácica baixa, no cone medular. Neste artigo, fizemos uma revisão da literatura e descrevemos um caso raro de diastematomielia cervical associada a lipoma e hipotrofia de membros superiores. Relato do caso: Uma mulher de 48 anos apresentou-se em nosso ambulatório com queixa de atrofia da porção distal do membro superior direito, que evoluiu para o lado contralateral após seis meses. No intraoperatório, a membrana epidural foi aberta, sendo encontrada uma lesão amarelada, reforçando o diagnóstico sugestivo de lipoma. Após a remoção, a medula espinhal dividida tornou-se clara. Discussão: Segundo nossa pesquisa, este é o primeiro caso de lipoma associado a diastematomielia na região cervical. Desde 1985, apenas 23 pacientes adultos foram diagnosticados com exames de imagem e tratados cirurgicamente. É possível dividir os pacientes com malformação da medula espinhal (SCM) em dois grupos. Um grupo de pacientes diagnosticados nos primeiros anos de vida com sintomas remanescentes e outro grupo formado por aqueles diagnosticados na idade adulta. Nosso paciente encontra-se no segundo grupo.

In conus medullaris and cauda equina surgery, identification of the sacral nerve roots may be uncertain in spite of their anatomical/radiological landmarks. Mapping the sacral roots by recording the muscular responses to their stimulation may benefit from EMG recording of the External Anal sphincter (EAS) in addition to the main muscular groups of the lower limbs. In a consecutive series of 27 lumbosacral dorsal rhizotomy (DRh), authors carried out a prospective study on the reliability of the EMG recording of the EAS for identification of the S1 and S2 sacral roots. An EAS-response was recorded in all the 27 (bilaterally) explored individuals, testifying good sensitivity and selectivity of the method. EAS-responses were obtained in 96.3% of the 54 stimulated sides of the S2 root versus in only 16.66% for the S1 root, so that an absence of response would indicate S1 rather than S2 level. Furthermore, comparison between myotomal distribution of the S1 and S2 roots showed a significant difference (p

The coexistence of vascular malformations in the conus medullaris and cauda equina has been rarely reported, and the complex angioarchitecture in multiple arteriovenous lesions remains poorly understood.A 17-year-old woman presented with a sudden-onset, stepwise worsening of weakness and pain in the bilateral legs. Angiography revealed conus medullaris arteriovenous malformation and cauda equina arteriovenous fistulas. One of the drainers was shared between the coexisting lesions and harboured a varix. Targeted embolisation of a fistulous point in the conus lesion was performed with precaution to prevent occluding the common drainage route, which led to symptom improvement with angiographical diminishment of the varix.Recognising that communications between drainers can be observed in multiple spinal arteriovenous lesions is important in facilitating a safe embolisation. Cautious assessment of angiogram with fusion images of cone-beam computed tomography and volumetric T2 magnetic resonance imaging can help in establishing the diagnosis and treatment strategy.

Introduction Multidisciplinary care for patients with tethered spinal cord syndrome (TCS) is valuable in ensuring comprehensive evaluation, timely follow-up, optimal functional outcome, and patient-centered care. The family-centered focus aims to minimize patient and parental burdens associated with care coordination. We present our first-year institutional experience in operationalizing a multidisciplinary, patient-centered, pediatric tethered cord clinic (TCC) to manage routine, long-term surgical follow-up for children with non-myelomeningocele-related tethered spinal cords. Methods TCC is composed of three surgical services: orthopedic surgery, neurosurgery, urology. A retrospective chart review of patients seen in the TCC from January 2019 to January 2020 was conducted. Patients enrolled in the clinic were intended for long-term follow-up. Demographic and outcome variables were collected. Results Fifty-nine patients were seen in TCC. Types of tethered spinal cords amongst these patients were the following: fatty filum (62.7%), dermal sinus tract (15.2%), meningocele manque (8.4%), lipomyelomeningocele (6.7%), low lying conus medullaris (5.1%), and sacral arachnoid cyst (1.7%). Age at diagnosis was 1.31 ± 2.21 (median: 0.25 years) and at follow-up was 9.0 ± 5.18 years (median: 8 years). A total of 50.9% of patients were female, and 93.2% had a prior untethering procedure. Of all patients, 6.8% have no surgical intervention and continue to be monitored conservatively for evidence of decline. All three services evaluated 84.8% of patients during the same clinic session, while 15.3% of patients were seen by two of the services, and 20.3% of patients were able to schedule related imaging or diagnostic testing during the same visit. Conclusion We describe successful implementation of a multidisciplinary pediatric TCC and document the first year of experience. The TCC streamlines care, decreases burden on families, and reduces those lost to follow-up. Complex disease pathologies, even when clinically stable, require long-term follow-up with multiple subspecialties and benefit from multidisciplinary clinics.

Background Spinal dural arteriovenous fistulas (DAVFs) and spinal arteriovenous malformations (AVMs) are relatively rare. Moreover, multiple spinal cord vascular lesions in an individual, such as double DAVFs and DAVF with AVM, are extremely rare. To date, there are only several reported cases of concomitant spinal DAVFs and AVMs. Case Description A 25-year-old woman presented with pain of the right lower limb persisting for 1 month. Spinal magnetic resonance imaging showed prominent signal flow voids in the lumbosacral canal. Spinal digital subtraction angiography (DSA) showed a sacral DAVF fed by the right lateral sacral artery and a spinal intradural AVM at the conus medullaris fed by the anterior spinal artery from the left T11 intercostal artery. The drainage veins in these 2 lesions were respective and without common anastomosis. The diagnosis of concomitant sacral DAVF and conus AVM was made. Microsurgery was performed to excise these lesions successfully in the same operation. The patient's symptoms gradually improved after surgery. Postoperative spinal DSA confirmed the complete disappearance of the 2 lesions. Conclusions The concomitant presence of sacral DAVF and conus AVM is extremely rare. Nonetheless, clinicians should be aware of the possibility of the coexistence of multiple types of spinal vascular lesions in a single patient.

Non-traumatic vertebral fractures occurring as a sole consequence of the violent muscle forces generated during the first episode of a GTCS(generalized tonic clonic seizure) in a previously healthy non-epileptic individual are very rare. Being clinically asymptomatic they are easily overseen at the time of initial presentation due to their rarity of occurrence and the presence of potentially distracting factors in the post-ictal phase. We present a 52-year-old healthy non-epileptic male who presented with unrelenting back pain and neurodeficit secondary to a four-month-old unstable burst fracture of the first lumbar vertebra sustained during an isolated single episode of a witnessed GTCS. A detailed inquiry revealed no history of a significant traumatic event either during the convulsive episode or thereafter. A meticulous history taking, a thorough clinical and neurological examination combined with a comprehensive radiological evaluation established the unusual etiology of the fracture and the presence of a thoracolumbar kyphotic deformity with compression of conus medullaris. A detailed neurological, and laboratory work-up, confirmed no attributable organic or metabolic cause for the seizure. His BMD(Bone Mineral Density) was normal. Patient was managed with posterior instrumented deformity correction by a posterior column shortening osteotomy, neural decompression and fusion of D12-L1 facets. Patient had complete neurological recovery with good clinical and functional outcomes at 28-months follow-up. A few cases of seizure-induced non-traumatic spinal fractures have been published in literature. A majority of these fractures occurred in individuals with either seizure-provoking risk factors (epileptics with recurrent seizures, brain tumors, drug overdose/withdrawal, metabolic disorders, or electrolyte imbalance) or in those with an increased susceptibility to fracture due to decreased BMD. This case demonstrates the rare occurrence of a non-traumatic vertebral fracture during the first episode of a GTCS in an otherwise healthy non-epileptic individual with normal BMD and no seizure-provoking risk factors. This is the first case report of a delayed unrelated presentation of a non-traumatic lumbar vertebral fracture with complications (spinal deformity and neurodeficit) consequent to a remote episode of a single convulsive seizure. It emphasizes the need for a high index of clinical suspicion,a meticulous history taking, thorough musculoskeletal and neurological examination in any individual presenting with a seemingly benign back pain following a remote isolated episode of seizure, even in the absence of overt trauma. A detailed radiological evaluation guided by a meticulous history taking and detailed clinical examination is essential to rule out a fracture unless proven otherwise. It also shows that a single convulsive seizure can result in a potentially unstable fracture that when neglected, can result in devastating complications like spinal deformity and neurodeficit. Level of evidence Level IV.