Filum disease fd is the congenital form of ncvs or when the cause of the cord traction can be due to a congenital anomaly of an apparently normal filum terminale, without that any anomalies of the ligament or of other conventional neurovertebral malformations can be seen on the neuroimaging. Original article scanning electron microscopy sem study. In a small number of cases such anatomic malformations are compatible with normal or near normal function until changes in growth produce locomotive or sphincteric disturbances or both. Spinal arteriovenous fistulas of the filum terminale. It gives longitudinal support to the spinal cord and consists of two parts. The filum terminale is the result of the empty cover of the spine cord at the lower back or lumbosacral region.
The first was a 33 yearold female who underwent surgical cord detethering. The histologic structure of the filum terminale is of neuroanatomic interest and clinical importance. Ultrasound of the spine was performed and showed the conus medullaris at the l23 level. In 21 patients 84%, the level of the tip of the conus was below the mid l2 vertebral body. Tethered cord syndrome tcs represents a spectrum of congenital anomalies characterized by an abnormal caudal position and traction of the conus medullaris. Preoperative predictors for improvement after surgical. Split cord malformations scms are rare congenital anomalies of the vertebrae and the spinal cord. Thick filum terminale, intraduralfilar lipoma, dorsal dermal sinus, lipomyelocele, lipomyelomeningocele, diastematomyelia. Aug 18, 2017 however, in the majority of human patients, tethered cord syndrome is associated with an abnormal short, thickened, and inelastic filum terminale without other spinal malformations. They occur in 16% of the population and are usually an incidental finding 95% of those detected by mri are asymptomatic cools et al. Termed caudal agenesis or dysgenesis, these congenital malformations in volve abnormal or incomplete formation of cau dal elements of the embryo. Filum terminale medical definition merriamwebster medical.
Frontiers coincidence of tethered cord, filum terminale. Filum terminale synonyms, filum terminale pronunciation, filum terminale translation, english dictionary definition of filum terminale. Tethered cord syndrome tcs is a condition where the spinal cord tissue attaches abnormally to the bones of the spine. This syndrome is closely associated with spina bifida. Watch this 2minute neuroscience video to learn more about the exterior of the spinal cord and this video to learn more about the interior of the spinal cord. Paragangliomas affecting the filum terminale are extremely rare, benign tumors. The filum terminale is an extension of the pia mater that is attached to the coccygeal segments. Tight filum terminale syndrome radiology reference. Tethered cord syndrome associated with a thickened filum. With the increasing use of magn we use cookies to enhance your experience on our website.
More recently, it has been proposed for the management of the occult tethered cord syndrome otcs, though it is still under debate. Fibrolipoma of the filum terminale the pain source. The filum terminale is a delicate strand of fibrous tissue, about 20 cm in length, proceeding downward from the apex of the conus medullaris. A lipoma or fibrolipoma of the filum terminale is a somewhat uncommon 46% and usually incidental, asymptomatic finding on mris of the lumbar spine. Annually, cauda equina syndrome ces is believed to newly affect between 1 person per 33,000 100,000. Spinal lipoma of the filum terminale lft is a congenital lumbosacral anomaly that can cause tethered cord syndrome. Filum terminale definition of filum terminale by medical. Filum terminale, tethered cord syndrome, ultrastructure introduction the filum terminale is a fibrous band, composed of two distinct segments intradural and extradural, that extends from the conus medul laris to the periosteum of the coccyx 1. However, the coincidence of tethered cord syndrome, lipoma, and sdavf on the sacral level is exceptionally rare. Mri of conus medullaris, cauda equina, and filum terminale. During subsequent growth, traction on the spinal cord causes stretching and deformity of the cord. Paraganglioma of the filum terminale is a rare tumor, often disclosed by lumbago and sciatica that are sometimes complicated by cauda equina syndrome. All forms involve the pulling of the spinal cord at the base of the spinal canal, literally a tethered cord.
Wehby mc, ohollaren ps, abtin k, hume jl, richards bj. Prompt surgical treatment is often necessary to avoid permanent sequelae. We also performed research of the latest literature. Fatty filum terminale is sometimes demonstrated on mri incidentally. This is an enhanced pdf from the journal of bone and joint surgery j bone joint surg am. Histopathology of the filum terminale in children with and. Mr images of four patients with a thickened filum terminale showing a fat signal are presented. This is also referred to as tethered cord syndrome with a tight filum terminale 1, 4 and has been reported only once previously in the veterinary literature. In such cases, it is usually associated with a thickened filum and a lowlying conus. Sectioning the filum terminale does not usually involve maneuvers that contribute to retethering of the spinal cord.
Case report a case report of filum terminale paraganglioma. Structure of the filum terminale archives of neurology. Few congenital anomalies of the nervous system are amenable to surgical intervention. Filum terminale lipoma ftl is known to cause various spinal symptoms collectively referred to as tethered cord syndrome tcs. Pdf we report a case of 56yearold patient suffering from myxopapillary ependymoma of filum terminale at the level of the fifth lumbar vertebra. Fibrolipoma of the filum terminale the pain source makes. The pdf of the article you requested follows this cover page. The emphasis on syndrome highlights a clinical diagnosis made by the synthesis of symptoms, neurologic exam signs, and laboratory tests such as urodynamics. Ependymomas of the filum terminale eft form a specific and relatively uncommon subtype of spinal cord ependymomas. Pdf pathophysiology and clinical features of tethered cord.
The proximal structure of the filum is similar to spi. Mar 01, 2006 read millerdieker syndrome associated with tight filum terminale, pediatric neurology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Even more controversial appears to be the use of sft in patients with chiari type i malformation cim, which is. It is not intended to be and should not be interpreted as medical advice or a diagnosis of any health or fitness problem, condition or disease. Abnormal secondary neurulation can lead to a variety of other complex spinal dysraphisms in the caudal region. The filum terminale has often been overlooked in the literature due to its historical lack of research on its true morphology.
Most series in the literature are small, spanning a large time period. Introduction to the tethered cord syndrome thieme medical. In conclusion, avfs of the filum terminale, in general, are singlehole communications of 1 feeding artery that is a continuation of the anterior spinal artery and a single draining vein. Tight filum terminale syndrome is a complex of neurologic and orthopedic deformities associated with a short, thick filum terminale and low lying conus medullaris with variable clinical manifestations.
To improve the understanding in pathology and provide steady proof for diagnosis and treatment of tethered cord syndrome tcs, the ultrastructure of filum terminale ft with tcs will be. Feb 16, 2019 the filum terminale is the result of the empty cover of the spine cord at the lower back or lumbosacral region. Its protean manifestations, diagnosis and treatment. Filum terminale lipomas ftls are being identified with increasing frequency due to the increasing utilization of mri. Tethered spinal cord syndrome is a neurologic disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. The filum terminale is a structure usually less than 2 mm in diameter. Thirtytwo pediatric patients presenting with symptoms of urinary dysfunction, stool.
These attachments cause an abnormal stretching of the spinal cord. Filum terminale definition of filum terminale by the free. The tight filum terminale syndrome is characterized by a varying array of neurological, urological, andor orthopedic signs and symptoms. Differentiating tethered cord syndrome, neurocranio. Filum terminale definition neuroscientifically challenged. Ta a long connective tissue pia mater strand extending from the extremity of the medullary cone to the inner aspect of the spinal dural sac pial part of filum terminale ta, filum terminale internum. We report a 63yearold man with suddenonset severe right chest and upper back pain, followed by. The fatty filum involves fatty infiltration of the whole length or of part of the filum terminale. Duration of horizontal decubitus after section of a tight. Differential diagnoses included tethered cord syndrome and. Pdf cutting filum terminale is very important in split cord. It is only recently that the true anatomy and pathological involvement of the filum terminale in the tethered cord syndrome have been elucidated. Although an ftl may be associated with tethered cord syndrome tcs, in many cases ftls are diagnosed incidentally in patients without any symptoms of tcs. Frontiers dynamic lumbosacral magnetic resonance imaging in.
Paraganglioma of the filum terminale mimicking neurinoma. The upper part, or filum terminale internum, is about 15 cm long and reaches as far as the lower border of the. Terminal filum definition of terminal filum by medical. Pain, dysesthesias, neurogenic bladder and spasticity are the common clinical presentations of tethered cord and tight filum terminale. Two boys, 14 and 18 years of age, presented with leg weakness and. Wehby mc1, ohollaren ps, abtin k, hume jl, richards bj.
The filum disease cis a form of the neurocraniovertebral syndrome caused by a congenital abnormality of an apparently normal filum terminale, causing a tractive force on the spinal cord and the rest of the nervous system. Minimally invasive surgical approach to filum sectioning. Regardless of the epidemiology, ces is a spinal emergency necessitating quick and effective care to mitigate permanent neurological sequelae. Gross anatomy the filum terminale is continuous with the pia mater and is described as having two sections. Incidental isolated fibrolipoma of filum terminale in adult. Purposes of this study are to clarify preoperative characteristics of. Historical aspects tulpuis in 1641 was the first to use the term spina bifida. Occult spinal dysraphism spinal lipoma fibrolipoma of the filum terminale intradural lipoma lipomyelocelelipomy elomeningocele. Retethering of sectioned fibrolipomatous filum terminales. The indications for sectioning the filum terminale in this situation are not well characterized and remain controversial. Morphometric parameters and histological study of the filum. Adult tethered cord syndrome atcs is a rare entity that usually presents with multiple neurological symptoms, including lower extremity pain, backache, lower extremity muscle weakness, and bowelbladder disturbances. Fatty filum terminale, also known as lipoma of the filum terminale or filar lipoma, is a relatively common finding on imaging of the lumbar spine, and in most cases is an incidental finding of no clinical concern.
Filum terminale syndrome w tethered cord syndrome w. There was no significant thickening of the filum terminale. The entity of an occult tight filum terminale syndrome, characterized by clinical findings consistent with a tethered cord syndrome, but with the conus ending in a normal position, has been recognized recently. Tight filum terminale syndrome radiology reference article. Management of tight filum terminale syndrome with special emphasis on normal level conus medullaris nlcm mehmet selc. This research was implemented to investigate the morphometric parameters and histological structure of normal ft in adult human cadavers and magnetic resonance imaging mri scans to correlate them with the pathophysiology of tcs. The upper part, or filum terminale internum, is about 15 cm long and reaches as far as the lower border of the second sacral vertebra. Tight filum terminale syndrome neurosurgery oxford. The most common causes of cauda equina and conus medullaris syndromes are the following. Fatty filum terminale on mri t iizuka citation t iizuka. We describe two patients, probably the fifth and sixth ever reported. A section of the filum terminale sft is used for the surgical treatment of isolated tethered cord or that resulting from neurulation disorders. Tethered spinal cord syndrome causes, diagnosis and.
Subtle imaging findings in a case of tight filum terminale syndrome. Subtle imaging findings in a case of tight filum terminale. Millerdieker syndrome is characterized by type i lissencephaly, severe mental deficiency. Pdf the proposed normal function of the terminal filum of the spinal cord is to fixate. It is the simplest form of conditions causing tethered cord syndrome tcs and the filum terminale can be thickened, normally with lipomatous tissue1,3. Yong rl, habrockbach t, vaughan m, kestle jr, steinbok p. The term filum terminale syndrome or cordtraction syndrome was first described by mckenzie kg in 1949, defined by garceau in 1953 3, and named tght. The treatment of these conditions is neurosurgical. The filum terminale terminal thread is a delicate strand of fibrous tissue, about 20 cm in length, proceeding downward from the apex of the conus medullaris.
T2weighted mri hyperintense though not nearly as obvious clinical significance. Intramedullary tumors of spinal cord and gliomas of intradural portion of filum terminale. The literature yielded thirtytwo cases of paraganglioma in this site. A family is described in which all 3 siblings manifested the syndrome of tight filum terminale in association with spina bifida occulta of the first sacral segment. Spinal dysraphism dys bad, raphe seam nonspecific term for congenital abnormalities in the development of. The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. It is considered as one of the causes in tethered cord syndrome tcs. Modern metallic methods have been utilized in this study to demonstrate the cytologic structure of the filum and its transition to the conus medullaris. Lumbar stenosis multilevel spinal trauma including fractures15 herniated nucleus pulposus cause of 26% of cases of cauda equina syndrome 16, 17, 18 neoplasm, including metastases, astrocytoma, neurofibroma, and meningioma. Commonly, it is referred to as the tethered spinal cord, a clinical syndrome, recognized by specific imaging abnormalities low conus, thickened filum, spina bifida and coupled with symptoms produced through pathological traction of the lower cord. There were no related symptoms and no evidence of tethering.
In a similar way to scoliosis and chiari i syndromein the case of syringomyelia the sectioning of the filum terminale stops the disease. Symptomatic retethering of the spinal cord after section of a tight filum terminale. The thickened fatty filum terminale seemed to be a developmental anomaly and without clinical significance. In sacrum vertebra, but its continuation, the filum terminale, can be traced through the sacrum to the first coccygeal vertebra. Mri with gadolinium injection allows setting surgical strategies, but does not bring certainty in the diagnosis, which is obtained through an anatomopathology. The term tight filum terminale syndrome is synonymous with tethered cord syndrome secondary to a tight filum terminale. Sem study on filum terminale with tethered cord syndrome. Millerdieker syndrome associated with tight filum terminale. Various forms include tight filum terminale, lipomeningomyelocele, split cord malformations diastematomyelia, dermal sinus tracts, and dermoids. Tethered cord syndrome tcs refers to a group of neurological disorders that relate to malformations of the spinal cord. The causes of the tethering were spinal lipomas 72%, tight. Occult tight filum terminale syndrome is the clinical condition in which the symptoms of spinal cord traction occur in conjunction with nondiagnostic lumbosacral mri findings. The cordtraction this information is current as of october 5, 2007 reprints and permissions permissions link. However, in some patients it may be associated with signs and symptoms of tethered cord syndrome.
Kesler at el5 reported that the conus virtually never ends below the mid l2 level. Mri of conus medullaris, cauda equina, and filum terminale lesions rami eldaya, md, mba, omar eissa, md, gabriel calles, md, jorge leediaz, md, and tomas uribe, md after participating in this educational activity, the radiologist should be better able to diagnose conus medullaris, cauda equina, and. A diagnosis of an occult filum terminale syndrome was made based on. The filum terminale helps to anchor the spinal cord in place. Request pdf millerdieker syndrome associated with tight filum terminale an 8yearold female was diagnosed with millerdieker syndrome with typical facial presentation. Jul 29, 2011 a section of the filum terminale sft is used for the surgical treatment of isolated tethered cord or that resulting from neurulation disorders. Spinal dural arteriovenous fistula sdavf is the most common vascular malformation of the spine in adults. We treated one case of filum terminale paraganglioma invading the lumbar body and the spinal dura. The clinical, histological and radiological characteristics of this case, that brings the. Morphology and histology of filum terminale ft has a role in the pathophysiology of tethered cord syndrome tcs.
An unusual presentation of adult tethered cord syndrome. University of ankara, school of medicine, department of neurosurgery, division of pediatric neurosurgery, ankara, turkey selc. Differentiating tethered cord syndrome, neurocraniovertebral syndrome, and filum disease. Tight filum terminale syndrome, or tight filum syndrome, is a subtype of the tethered cord syndrome that is attributed to a thick, short, andor otherwise inelastic filum terminale rather than other tethering agents. Sep 27, 2018 spinal dural arteriovenous fistula sdavf is the most common vascular malformation of the spine in adults. A 49 yearoldman, whose presenting symptoms were low back pain and left leg weakness, was diagnosed as having a paraganglioma of the filum terminale. Up to date no consensus has been reached about the optimal treatment of these lesions. The indications for sectioning the filum terminale in this situation are not well characterized and are controversial.
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