In this setting I leave the filum alone. J Urol 101:545–549, 1969, Yamada S, , Won DJ, & Yamada SM: Pathophysiology of tethered cord syndrome: correlation with symptomatology. There is even less evidence for surgical treatment of split cord malformations than for surgery in cases of lipomyelomeningocele. Is surgery necessary for asymptomatic tethered cord in anorectal malformation patients? Surgery was complicated by a CSF collection requiring a subcutaneous peritoneal shunt. during surgery for adult tethered cord syndrome: Analysis of a series . HHS Surgery in adult patients with a tethered cord syndrome should be reserved for those with symptoms. However in symptomatic patients surgery is often recommended. Tethered spinal cord comprises a group of dysraphic conditions in which the conus medullaris is located in an abnormally low position. Figure 1 shows images obtained in such a patient. Post-traumatic tethered spinal cord is a condition which occurs following injury to the spinal cord where scar tissue forms and tethers or holds the spinal cord to the soft tissue covering which surrounds it called the dura. doi: 10.3171/FOC-07/08/E5. The good news is that tethered spinal cord is a very treatable condition, especially when diagnosed and treated early. This dermoid can often be completely excised, de novo, but following recurrent episodes of infection, and particularly an intraspinal abscess, an entangled mass of cyst, nerve root, and scar may develop—impossible to completely remove and almost certain to redevelop after incomplete removal. Childs Nerv Syst 13:298–335, 1997, Schneider SJ, , Rosenthal AD, , Greenberg BM, & Danto J: A preliminary report on the use of laser-Doppler flowmetry during tethered spinal cord release. NLM The exact steps will depend on individual needs. Tethered spinal cord syndrome in adults is an uncommon entity that can become sympto-matic. Treatment decision making is made difficult by the variety of lesions and clinical presentations comprised by this condition and the absence of high-quality clinical outcome data to provide guidance. Section of the terminal filum for occult tethered cord syndrome: toward a scientific answer. A tethered cord release is a procedure to separate the spinal cord from tissue that holds it in place. Minimal tethered cord syndrome: what's necessary to justify a new surgical indication? (Pp212, US$95). Childs Nerv Syst. Nevertheless, the indications for surgery remain controversial (1). Lipomyelomeningoceles manifest significant variability in their configuration and the technical difficulty of treatment. Br Med J 281:1243–1245, 1980, Tuuha SE, , Aziz D, , Drake J, , Wales P, & Kim PC: Is surgery necessary for asymptomatic tethered cord in anorectal malformation patients?. The defect in the thinned-out dura following lipoma excision can be significant and may require patching either with autologous fascia or dural substitute and sealing with any of various fibrin preparations. Childs Nerv Syst. 2), diastematomyelia, caudal regression syndromes (such as imperforate anus), and dermal sinus tract (not a normal sacral dimple). Subtle deterioration over the next 3 years—repeated bladder infections culminating in the recommendation for intermittent catheterizations, in-turning of the left foot, and loss of anal tone—prompted a repeated imaging study, which demonstrated an enlarged syrinx in the distal spinal cord (Fig. Tethered cord conditions may cause syrinxes, or fluid-filled cysts, elsewhere in the spinal cord. Epub 2020 Aug 15. The good news is that tethered spinal cord is a very treatable condition, especially when diagnosed and treated early. There has been controversy regarding the management of syringomyelia associated with tethered spinal cord. In some cases these findings are almost to be expected—for example, in cases of caudal regression syndrome or Currarino triad. Superficial in vivo measurements of changes in cytochrome gradients22 or laser Doppler blood flow9 have been demonstrated in patients, but again the significance of these changes is not clear. J Neurosurg. Tethering may also develop after spinal cord injury. In Chiari circles this has been a major controversy and some doctors have evidently been overusing the surgery. 2013 Sep;29(9):1625-34. doi: 10.1007/s00381-013-2180-y. For this reason, continued clinical monitoring, with careful documentation of motor, sensory, and urodymanic function, is important. 2006 May;104(5 Suppl):309-13. doi: 10.3171/ped.2006.104.5.309. Some have suggested documenting clear neurologic deficits before proceeding with surgery. There are apparently many patients with tethered cord who remain asymptomatic their entire life. 1. Tethered spinal cord is common in children with spina bifida, but it also occurs in children who don’t have this condition. Counting the vertebrae to assign conus position can be difficult, and the results may have little meaning. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS. Methods: Follow-up imaging showed reduction in the size of the syrinx and lipoma component, but the lipoma was apposed to the overlying dura (Fig. The child underwent surgery and the cord was completely untethered. Other common scenarios include cases of scoliosis with what appears to be an idiopathic curve, in which the conus is reported as being low or “borderline low” at the inferior margin of L-2 with or without fat in the filum. Conclusions: The syndrome, treatments, outcomes, and current controversies are re-viewed. 2001 Jan 15;10(1):e1. Recently, surgery has been offered to children who have a normally positioned conus medullaris and no terminal filum abnormality visible on 1.5-tesla magnetic resonance images (referred to as minimal or occult tethered cord syndrome [TCS]). The 10 patients in group 4 were treated conservatively. The exact steps will depend on individual needs. J Neurosurg. The spinal cord is stretched. Instead, it pulls against the restriction. Tethered cord means that your child's spinal cord cannot move freely inside their spinal column. PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. COVID-19 is an emerging, rapidly evolving situation. Recently, surgery has been offered to children who have a normally positioned conus medullaris and no terminal filum abnormality visible on 1.5-tesla magnetic resonance images (referred to as minimal or occult tethered cord syndrome [TCS]). Occult tethered cord syndrome: not an indication for surgery. The common factor in all tethered cord conditions is that the spinal cord’s movement is restricted at its base. of 44 patients with long‑term follow‑up. Childs Nerv Syst. Spinal cord tethering secondary to lipomyelomeningocele or retethering following a spina bifida repair is surgically more complex and is best managed by those experienced with the disorder. A tethered cord refers to a condition in which the lower spinal cord is restricted (“tethered”) and is not free to develop normally with in the spinal column. Previous reports on the topic have included only a small number of patients, considered open/closed spinal dysraphism together, or had a short follow-up. Tethered cord syndrome (TCS) is a complex and progressive clinical and radiological condition, secondary to an embryologic failure of spinal cord development, which induces various types of spinal dysraphisms. The neurological surgeon makes an incision in the lower back to expose the site where the spinal cord is pinned, then frees it by releasing the stuck portion of the cord. Background: Tethered cord syndrome (TCS) is a progressive clinical condition including a series of neurological dysfunctions and deformities attributable to abnormally increased tension on the spinal cord, which is lacking effective treatment to relieve the symptoms and prevent progression. Tethered cord release is performed in the prone position with neural monitoring of lower extremity motor and sensory responses and monitoring of the rectal sphincter innervation. Axial (left) and sagittal (right) MR images obtained in a 3-month-old patient with a lumbar dermal sinus tract. Subtle changes in the results of urodynamic testing alone, however, do not appear to warrant intervention as there is considerable variability in the tests and their interpretation.18 One should be prepared to regard changes that could reflect maturation of the nervous system or could be due to extremity growth (progressive Achilles tendon shortening) as not being mandatory indications for surgery. The true dermal sinus tract, usually in the lower lumbar area and sometimes discharging detritus or pus, is clearly of some risk to the patient, and should be investigated and treated expeditiously (Fig. 1A,B). Epub 2015 Feb 25. Tethered spinal cord is an abnormal attachment of the spinal cord to tissue around it. The tethered spinal cord has to be untethered, i.e., adhesions between nervous tissue and surrounding connective tissue have to be broken. More than 40 percent of children who have spina bifida will need surgery to untether the spinal cord during their lifetimes. For this procedure, the patient is placed under general anesthesia. Although several reports of successful surgical treatment exist, controversy remains with respect to patient selection and efficacy. Tethered cord syndrome, manifested by motor and sensory dysfunction and incontinence, is caused by excessive tension in the lumbosacral cord. Ann Neurol 54:403–414, 2003, Pierre-Kahn A, , Zerah M, , Renier D, , Cinalli G, , Sainte-Rose C, & Lellouch-Tubiana A, : Congenital lumbosacral lipomas. J Neurosurg. Introduction: Although MRI has improved the diagnosis of tethered cord, many controversies still exist in the treatment of tethered cord syndrome (TCS). This entity was first described by Garceau (1953) and subsequently named by Hoffman et al. Among these studies, it has been shown that symptomatic tethered cord will produce clinical deterioration with regard to bladder spasticity and orthopedic foot deformity if only treated symptomatically. In contrast, the spinal dural arteriovenous fistula (SDAVF) is an acquired condition. Although this case represents only a single anecdote, it nevertheless provides evidence that progressive deterioration occurs in some patients and illustrates the kind of early improvement that can occur following surgery. Surgical steps may include: Removal or release of the tether Clinical presentations may be divided into four general categories or typical scenarios: 1) significant dysraphic abnormality, clear clinical deterioration; 2) significant dysraphic abnormality, clinically normal or stable deficit; 3) incidentally discovered abnormality, other problem; and 4) tethered spinal cord symptomatology, normal imaging. Clinicians suspect tethered cord readily in toe walking children; however, the incidence of tethered cord in this population has not been established. The patient had experienced concomitant clinical progression. Clearer diagnostic and treatment strategies for the tethered spinal cord will only result from high-quality clinical and basic research. Spine (Phila Pa 1976) 2006;31:2095‑102. tethered cord creates traction, or a pulling effect, and can lead to leg weakness and urinary and bowel problems. Born with an obvious lipomyelomeningocele, the patient was apparently neurologically normal. Split spinal cord malformations, affecting women more often than men [2, 5, 6], represent inborn anomalies which are often associated with a low conus, tethered cord and eventual lipoma, spinal teratoma, vertebral deformities, Klippel–Feil syndrome, syringomyelia, Arnold–Chiari malformation, lumbar cutaneous malformations (50–75% of patients ) and dysplasia of the legs [5-10, 14-17]. Few topics in pediatric neurosurgery generate as much controversy, debate, or angst as the diagnosis of tethered spinal cord. J Neurosurg 104:5 Suppl 302–304, 2006, Steinbok P, , Garton HJ, & Gupta N: Occult tethered cord syndrome: a survey of practice patterns. Walking the fine line between over- and undertreatment seems a rational approach until better information becomes available. Syringomyelia and tethered cord in children. 14. If surgery is not advisable, spinal cord nerve roots may be cut to relieve pain. Tethered cord syndrome is a stretch-induced functional disorder associated with the fixation (tethering) effect of inelastic tissue on the caudal spinal cord, limiting its movement. The most problematic area of controversy is pathogenesis. The author evaluates existing clinical and scientific evidence relevant to this controversy. ✓The tethered spinal cord is a fascinating yet controversial condition seen frequently in neurosurgical practice. USA.gov. | Controversy exists regarding the indications to operate on patients with a tethered spinal cord as well as the appropriate timing for surgical tethered cord release (TCR) , , . This scar tissue prevents the normal flow of spinal fluid around the spinal cord and impedes the normal motion of the spinal cord. For this reason it is a good idea to scan the entire spinal cord, and potentially the brain, prior to treatment. The author evaluates existing clinical and scientific evidence relevant to this controversy. J Neurosurg 54:494–503, 1981. In cases involving neurologically normal patients without a true dermal sinus tract, I think a reasonable approach is careful observation, with a plan to intervene should deterioration occur. There are several caveats about surgery for these lesions. Recovery From Surgery for Tethered Cord Syndrome With a Normally Positioned Conus in Children. [4 14] However, controversy still surrounds the repair of asymptomatic tethered cord. Get the latest public health information from CDC: https://www.coronavirus.gov, Get the latest research information from NIH: https://www.nih.gov/coronavirus, Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. The controversy surrounding surgery for occult tethered cord was highlighted recently in the May, 2006 issue of the Journal of Neurosurgery: Pediatrics, which published several papers on the subject. 2015 Apr;31(4):597-601. doi: 10.1007/s00381-015-2615-8. Complications in neurologically normal patients are particularly distressing to surgeons as well as patients. 2007;23(2):E1. There are apparently many patients with tethered cord who remain asymptomatic their entire life. However, some researchers believe that trauma alone is not enough to cause the disorder. Chin-Peuckert L, , Komlos M, , Rennick JE, , Jednak R, , Capolicchio JP, & Salle JL: What is the variability between 2 consecutive cystometries in the same child?. J Urol 169:2344–2346, 2003, Warder DE, & Oakes WJ: Tethered cord syndrome and the conus in a normal position. Acute tension on the spinal cord has been shown to produce spinal cord dysfunction and deranged metabolism in animal experiments,15,23 but it is not known how these findings relate to the chronic situation typical of the patient with a tethered spinal cord. Intraoperative monitoring can be used to identify partially obscured roots or detect an early neurological injury, manifest by evidence of persistent spontaneous muscle activation, but it has not been convincingly proven to improve the outcome of surgery. Surgery for tethered cord is another area of controversy that was not well addressed in this paper. Tethered cord can cause problems with bladder control, bowel control or walking. While the lesions are classically divided into dorsal, transitional, and terminal, typically lipomyelomeningoceles are transitional, and vary most in terms of their relationship to the spinal cord and surrounding nerve roots. Previous reports on the topic have included only a small number of patients, considered open/closed spinal dysraphism together, or had a short follow-up. In such scenarios, it is probably better to perform only a partial untethering rather than risk an immediate atonic bladder, the most common serious complication. While it has been held that clinical deterioration in cases of lipomyelomeningocele, in particular, is inevitable, and therefore that treatment should be undertaken prophylactically,5 as discussed previously, this tenet is not universally held. The management of tethered cord syndrome with onset emains controversial, although the necessity of early surgery in the pediatric tethered cord syndrome population is well established. Treatment. Although a preponderance of Class III clinical evidence supports the use of surgical filum lysis to treat occult TCS, no Class I or II evidence exists. Recently, surgery has been offered to children who have a normally positioned conus medullaris and no terminal filum abnormality visible on 1.5-tesla magnetic resonance images (referred to as minimal or occult tethered cord syndrome [TCS]). Usually tethered cord is associated with either spins bifida-type abnormality in early childhood, or with scarring from spine surgery later in life. Once the problem is identified, the treatment of the tethered cord is surgical. Tethered Cord Syndrome . The author evaluates existing clinical and scientific evidence relevant to this controversy. doi: 10.3171/FOC-07/08/E1. The patterns of late deterioration in patients with transitional lipomyelomeningocele, Occult tethered cord syndrome: not an indication for surgery, Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale, The tethered spinal cord: diagnosis, significance, and management, A controlled trial of arthroscopic surgery for osteoarthritis of the knee, A double-blind controlled trial of bilateral fetal nigral transplantation in Parkinson's disease, A preliminary report on the use of laser-Doppler flowmetry during tethered spinal cord release. NIH Object: Controversy exists regarding proper indications for surgical lysis of the terminal filum in children with voiding dysfunction and tethered spinal cord. In patients who do become symptomatic, the constellation of clinical symptoms that develop are referred to as tethered cord syndrome (TCS). 1B). Selden NR, Nixon RR, Skoog SR, Lashley DB. Although controversy existed in the past (over the concept of prophylactic surgery of asymptomatic patients), most surgeons now believe that the risk of waiting for deterioration to begin is not justified, because the deficit often is … Controversy exists regarding proper indications for surgical lysis of the terminal filum in children with voiding dysfunction and tethered spinal cord. J Pediatr 137:814–818, 2000, Tani S, , Yamada S, & Knighton RS: Extensibility of the lumbar and sacral cord. Clinicopathological evidence suggests that occult TCS may result from radiographically occult structural abnormalities of the terminal filum. J Neurosurg 104:5 Suppl 305–308, 2006, Khoury AE, , Hendrick EB, , McLorie GA, , Kulkarni A, & Churchill BM: Occult spinal dysraphism: clinical and urodynamic outcome after division of the filum terminale. In this report, we describe two cases of adolescent onset TCS associated with two different etiologies. Occult tethered cord syndrome: a survey of practice patterns. These congenital curves have a clearer mechanical mechanism: asymmetrical growth. Axial (left) and sagittal (right) MR images obtained in a patient with a fatty filum and a normal-level conus. A tethered cord release is a procedure to separate the spinal cord from tissue that holds it in place. Tethered Cord Release Surgery. Childs Nerv Syst 19:3–10, 2003, Selden NR: Occult tethered cord syndrome: the case for surgery. Epub 2015 Jan 13. In most of those cases, the spinal cord is tethered to the tough membrane called the dura, which covers the spinal cord. Neurosurg Focus 16:2 E6, 2004, Yamada S, , Zinke DE, & Sanders D: Pathophysiology of “tethered cord syndrome”. In the presence of such symptoms, treatment is usually surgical and involves freeing the cord by either cutting the filum or repairing the lipomyelomeningocele defect. PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. Magnetic resonance images obtained in a patient with lipomyelomeningocele. Tethered = fastened or tied down Cord = the spinal cord, a bundle of nerves that connects brain and bodyTethered cord is an umbrella term for several different conditions. Pathological evaluation of the filum terminale tissue after surgical excision. J Neurosurg 66:116–123, 1987, Thomas TM, , Plymat KR, , Blannin J, & Meade TW: Prevalence of urinary incontinence. Different techniques are available to reduce traction on the spinal cord, e.g., removal of scar tissue in an open-surgery approach, endoscopic untethering, section of filum terminale and vertebral osteotomy to shorten the spine. Neurosurg Focus. In this scenario, the surgeon and the parents or patient usually agree that the potential benefits outweigh the risks. Note extension of the lipoma into the left buttock. Object: Edited by shokei yamada. Surgery for tethered cord is another area of controversy that was not well addressed in this paper. This is clearly a difficult task, and the need to be careful in interpreting test results when monitoring a patient's condition cannot be overemphasized. It is useful to obtain a baseline measurement of bladder function with a voiding cystourethrogram, and in the presence of incontinence or recurrent bladder infections urodynamic testing is an important method of monitoring. The worst of the most common complications is a neurogenic bladder; patients who experience this complication can expect a lifetime of incontinence, drug therapy, and intermittent catheterizations. One must be aware of “glacial” deterioration as discussed above. The tethered spinal cord syndrome remains a fascinating surgical enigma, surrounded by much debate, controversy, and uncertainty. Tethered spinal cord is often linked to spina bifida. 2006 May;104(5 Suppl):305-8. doi: 10.3171/ped.2006.104.5.305. The exact type and extent of surgery will depend on what is causing the TCS and how much the nearby tissue is affected. Five retrospective, observational, noncontrolled studies of surgical terminal filum lysis for occult TCS in children were identified. Citation: Przepiórka Ł, Kunert P, Juszyńska P, Zawadzki M, Ciszek B, Glowacki M and Marchel A (2018) Coincidence of Tethered Cord, Filum Terminale Lipoma, and Sacral Dural Arteriovenous Fistula: Report of Two Cases and a Literature Review. Therefore, the anesthetic induction may take place with the patient supine on a stretcher. Chiari Malformation is a serious neurological disorder where the bottom part of the brain, the cerebellum, descends out of the skull and crowds the spinal cord, putting pressure on both the brain and spine and causing many symptoms. Surgery involves gently releasing the spinal cord from the tissue that it is stuck to. Parents and patients themselves may find that the pace of deterioration is so slow that surgery should be delayed until a certain point is reached, although that point is usually ill defined. These conditions are usually obvious at birth and are a good reason to evaluate children for spinal cord tethering. Utility of CISS imaging in the management of tethered cord syndrome. J Pediatr Surg 39:773–777, 2004, Venhola M, , Reunanen M, , Taskinen S, , Lahdes-Vasama T, & Uhari M: Interobserver and intra-observer agreement in interpreting urodynamic measurements in children. Definition . 1C.). Most of these congenital disorders are amenable to surgical treatment. Other treatment is symptomatic and supportive. Scar tissue can block the flow of fluids around the spinal cord. Surgery is the most common treatment to “untether” the spinal cord from the spinal tissue. 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