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Concept: Conus medullaris

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OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed. Bowel function and LUTS were assessed by questionnaire. Patients with extensive sacral anomalies or meningomyelocele were excluded. RESULTS Of 89 patients (median age 15 years, range 5-29 years), MRI was available in 90% (n = 80; 40 male patients with RUF), and 80% of patients returned the questionnaire (n = 64; 31 male patients with RUF). Spinal cord anomalies were found in 34%, comprising a filum terminale lipoma in 30%, low conus medullaris in 10%, and thoracolumbar syrinx in 6%. Bowel functional outcomes between patients with SCAs (n = 23) and those with a normal spinal cord (n = 41) were not significantly different for soiling (70% vs 63%), fecal accidents (43% vs 34%), and constipation (57% vs 39%; p = not significant for all). The LUTS, including urge (65% vs 54%), urge incontinence (39% vs 24%), stress incontinence (17% vs 22%), and straining (32% vs 29%) were also comparable between groups (p = not significant for all). No patients developed lower-limb neurological abnormalities. CONCLUSIONS The results suggest that the long-term functional outcomes for patients with SCAs who had VF/PF and RUF may not differ significantly from patients with the same type of ARMs and a normal spinal cord. The results favor a conservative approach to their management in the absence of abnormal neurological findings in the lower limbs.

Concepts: Central nervous system, Spinal cord, Urinary incontinence, Urinary bladder, Lower urinary tract symptoms, Cauda equina, Filum terminale, Conus medullaris

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This is the first reported case of an adult presenting with tethering symptoms, limb discrepancy on physical examination, a low-lying spinal cord, and duplicate filum terminale discovered intraoperatively. Intraoperative imaging and pathological analysis of a specimen confirmed the diagnosis of duplicate filum. This is the first reported adult case with duplication of the filum terminale. Release of both fila was necessary in this case to relieve the tethering symptoms.

Concepts: Spinal cord, Biology, Pathology, Medical diagnosis, Physical examination, Cauda equina, Filum terminale, Conus medullaris

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Tethered cord syndrome (TCS) occurs as a constellation of neurologic signs and symptoms resulting from longitudinal traction on the spinal cord between fixed points. This condition involves a tug-of-war between ascent and inhibition of ascent of intrathecal nervous tissue within the vertebral canal during growth. Causes include thickened filum terminale, myelomeningocele, split cord malformation, and previous intradural surgery. Patients report low back, lower extremity, and perineal pain; lower extremity sensory and motor deficits; urinary and bowel incontinence; and sexual dysfunction. When not treated early or adequately, TCS can lead to neurologic devastation.

Concepts: Central nervous system, Spinal cord, Vertebral column, Scoliosis, Vertebra, Paralysis, Conus medullaris

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The cauda equina is composed of the lumbosacral and the coccygeal nerve roots and the filum terminale. In the embryonic period, discrepancy in development between the termination of the spinal cord and the spinal column results in elongation of the nerve roots as well as the filum terminale in this region. Although the vascular anatomy of the caudal spinal structure shares many common features with the other metameric levels, this elongation forms the basis of the characteristic vascular anatomy in this region. With the evolution of the high quality imaging techniques, vascular lesions in the cauda equina are being diagnosed more frequently than ever before. Albeit the demand for accurate knowledge of the vascular anatomy in this region, descriptions are often fragmented and not easily accessible. In this review, the author attempted to organize the existing knowledge of the vascular anatomy in the cauda equina and its implication on the vascular lesions in this region. Also reviewed is the clinically relevant embryological development of the cauda equina.

Concepts: Spinal cord, Prenatal development, Vertebra, Spinal nerve, Cauda equina, Corticospinal tract, Filum terminale, Conus medullaris

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Symptoms of cauda equina syndrome due to ependymoma in the conus medullaris or filum terminale develop slowly. However, hemorrhagic change inside spinal tumors can induce acute neurologic decline. Here, we report a case of posttraumatic hemorrhage inside a filum terminale myxopapillary ependymoma presenting as acute neurologic decline, which had a positive prognosis after surgical resection.

Concepts: Spinal cord, Medical terms, Bleeding, Cauda equina, Cauda equina syndrome, Filum terminale, Conus medullaris

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An understanding of the underlying pathophysiology of tethered cord syndrome (TCS) and modern management strategies have only developed within the past few decades. Current understanding of this entity first began with the understanding and management of spina bifida; this later led to the gradual recognition of spina bifida occulta and the symptoms associated with tethering of the filum terminale. In the 17th century, Dutch anatomists provided the first descriptions and initiated surgical management efforts for spina bifida. In the 19th century, the term “spina bifida occulta” was coined and various presentations of spinal dysraphism were appreciated. The association of urinary, cutaneous, and skeletal abnormalities with spinal dysraphism was recognized in the 20th century. Early in the 20th century, some physicians began to suspect that traction on the conus medullaris caused myelodysplasia-related symptoms and that prophylactic surgical management could prevent the occurrence of clinical manifestations. It was not, however, until later in the 20th century that the term “tethered spinal cord” and the modern management of TCS were introduced. This gradual advancement in understanding at a time before the development of modern imaging modalities illustrates how, over the centuries, anatomists, pathologists, neurologists, and surgeons used clinical examination, a high level of suspicion, and interest in the subtle and overt clinical appearances of spinal dysraphism and TCS to advance understanding of pathophysiology, clinical appearance, and treatment of this entity. With the availability of modern imaging, spinal dysraphism can now be diagnosed and treated as early as the intrauterine stage.

Concepts: Spinal cord, Scoliosis, 20th century, Centuries, Spina bifida, Filum terminale, Conus medullaris, Tethered spinal cord syndrome

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There are very few reported cases of a meningioma circumferentially surrounding the spinal cord. To date this entity has only been described at the conus medullaris and in the cervical cord. Herewith, the authors describe a case of an intradural extramedullary meningioma that completely encircled the thoracic spinal cord.

Concepts: Spinal cord, Case, Conus medullaris

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Sonographic evaluation of the fetal conus medullaris (CM) level is not reproducible. The objectives of this study were to determine the normal position of the fetal CM during pregnancy as well as the normal intradural filum terminale (FT) length and to evaluate their use in detecting tethered cord.

Concepts: Spinal cord, The Normal, Normal distribution, Filum terminale, Conus medullaris

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The development of a suitable animal model is important for clarifying the pathogenesis of tethered cord syndrome (TCS). This study was undertaken to develop a new animal model for investigating the pathogenesis and therapeutic strategies for TCS. A traction device, a filum terminale tractor, was designed exclusively for this experiment. A TCS model was produced in cats using the tractor to fixate the filum terminale to the dorsal aspect of the second sacrum. The responses to tethering were evaluated by electron microscopy and electromyography for detection of somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) at designated time points. Progressive swaying gait and lameness in clinical performance were observed with cord traction. Histopathological examination revealed an association between the increasing traction in the spinal cord and the increase in impaired nerve cells. No changes of SEPs and MEPs were detected in the untethered cats, while the latencies of SEPs and MEPs significantly increased in the tethered cats. The TCS model established in this study is simple and reproducible, in which varying degrees of tension could be applied to the neural elements.

Concepts: Nervous system, Neuron, Spinal cord, Electroencephalography, Evoked potential, Cauda equina, Filum terminale, Conus medullaris

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We discuss the association between tethered cord syndrome (TCS) and Chiari type 1 malformation (CM1), and report on the surgical outcomes of children with CM1 and TCS who underwent sectioning of the filum terminale (SFT). The relationship between TCS and CM1 is unclear. A retrospective review of 170 consecutive spinal cord detetherings between 2008 and 2012 was performed. We identified 17 children with CM1 who underwent SFT. Information regarding clinical presentation, radiographic findings, surgical procedures, and clinical outcomes was analyzed. A mean tonsillar herniation of 10.0mm (range: 5-21) was noted. Children with a fatty or thickened filum terminale demonstrated a greater amount of tonsillar displacement (p<0.005). A low conus medullaris was found in 12 children and a syrinx was present in three. The preoperative symptoms improved in all children. The postoperative MRI (mean 21.8months) revealed an unchanged tonsillar position in all but one child. No worsening of neurologic function was noted. Pediatric patients who have both CM1 and TCS, but do not demonstrate classic Chiari-related symptoms, may experience symptomatic improvement after filum terminale sectioning.

Concepts: Spinal cord, Surgery, Arnold-Chiari malformation, Filum terminale, Conus medullaris, Tethered spinal cord syndrome