Methods : All 83 patients with a lipomyelomeningocele … The patient had experienced concomitant clinical progression. 2018 Mar;31(2):61-70. doi: 10.1055/s-0037-1609020. Could you get more than one opinion? In cases of tethered spinal cord secondary to tight or lipomatous filum terminale, the surgical risk-to-benefit ratio is clearly in favor of surgery in both symptomatic and asymptomatic cases. 2007 May;106(5 Suppl):411-3; author reply 413-4. doi: 10.3171/ped.2007.106.5.411. | One must be aware of “glacial” deterioration as discussed above. Pathophysiology of the tethered spinal cord in cats. Though the adolescent presentation of TCS is well-recognized, it continues to pose significant diagnostic and management controversies. Interobserver and intra-observer agreement in interpreting urodynamic measurements in children, Tethered cord syndrome and the conus in a normal position, Occult tight filum terminale syndrome: results of surgical untethering, Stress incontinence in young, healthy nulliparous female subjects, Pathophysiology of tethered cord syndrome: correlation with symptomatology, Pathophysiology of “tethered cord syndrome”, Significant Dysraphic Abnormality, Clear Clinical Deterioration, Significant Dysraphic Abnormality, Clinically Normal or Stable Deficit, Incidentally Discovered Abnormality, Other Problem, Tethered Spinal Cord Symptomatology, Normal Imaging Findings, Top 25 Cited Gamma Knife® Surgery Articles - Trigeminal Neuralgia, Top 25 Cited Gamma Knife® Surgery Articles - Volume 111, Volume 23: Issue 2 (Aug 2007): Tethered Cord, Division of Neurosurgery, Hospital for Sick Children, University of Toronto, Toronto, Canada. Nevertheless, the indications for surgery remain controversial (1). Magnetic resonance images obtained in a patient with lipomyelomeningocele. Utility of CISS imaging in the management of tethered cord syndrome. Tethered cord syndrome, manifested by motor and sensory dysfunction and incontinence, is caused by excessive tension in the lumbosacral cord. Neurosurg Focus. However in symptomatic patients surgery is often recommended. Clin Colon Rectal Surg. Nevertheless, even in the presence of dysraphic components, including a low spinal cord and a thickened and fat-filled filum, progressive deterioration is not inevitable.17. These conditions are usually obvious at birth and are a good reason to evaluate children for spinal cord tethering. Clinical practice varies; therefore, performance of a prospective randomized clinical trial of surgical terminal filum lysis for the treatment of occult TCS is advocated. The good news is that tethered spinal cord is a very treatable condition, especially when diagnosed and treated early. 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. 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. NLM Therefore, during everyday activities, the spinal cord cannot move up and down inside the spinal canal. Tethered cord may be treated with surgery (an operation) to … This can occur during fetal development or may be associated with build up of scar tissue after previous surgery in or around the spinal cord. PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. 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. Although clinical deterioration can be fairly rapid, and occasionally acute, in cases in this category, it may more often be described as “glacial,” especially in cases involving older children. Surgery was complicated by a CSF collection requiring a subcutaneous peritoneal shunt. NIH In some cases these findings are almost to be expected—for example, in cases of caudal regression syndrome or Currarino triad. 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. An early imaging study, performed when the patient was 3 months old, demonstrated the typical features of a lipomyelomeningocele, with extension of the lipoma into the left buttock suggesting that untethering might be technically difficult (Fig. Pathophysiology of the tethered spinal cord in cats. The author provides case examples to illustrate potential treatment approaches and suggests balancing the risks and benefits for each general category. Surgery —The goal of surgery is to release the tethered spinal cord, ease symptoms, and prevent further damage. doi: 10.3171/FOC-07/08/E5. Some cases of tethered spinal cord are associated with other congenital malformations in the spine, kidneys, digestive system, and less commonly, the heart. Tethered cord syndrome, usually discovered in childhood, is a developmental abnormality impairing the longitudinal movement of the spinal cord that can be combined with various forms of spinal dysraphism. 2006 Sep;105(3 Suppl):214-8. doi: 10.3171/ped.2006.105.3.214. In contrast, the spinal dural arteriovenous fistula (SDAVF) is an acquired condition. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS. J Neurosurg. of 44 patients with long‑term follow‑up. 2015 May;31(5):759-63. doi: 10.1007/s00381-015-2627-4. Although there are still questions raised as to whether prophylactic surgery changes the natural long-term history of patients with tethered cords [ 1 ], I am convinced that prophylactic surgery is safe and does in most cases prevent the ongoing damage from flexion/extension injury to the distal spinal cord. J Neurosurg 54:494–503, 1981. PURPOSE: Occult tethered cord syndrome applies to patients with signs and symptoms consistent with a caudal spinal cord malformation despite normal neuroimaging. 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. Ann Neurol 54:403–414, 2003, Pierre-Kahn A, , Zerah M, , Renier D, , Cinalli G, , Sainte-Rose C, & Lellouch-Tubiana A, : Congenital lumbosacral lipomas. did so in patients beyond 10 years of age [8]; non-progressive patients should certainly not be operated on in adult age [35]. Cotntail. Tethered cord can cause problems with bladder control, bowel control or walking. 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]). Other risk factors associated with tethered cord include: Dermal sinus tract (a rare congenital deformity) Diastematomyelia (a split spinal cord) Tumor; A history of spine trauma; A history of spine surgery; Symptoms. Section of the terminal filum for occult tethered cord syndrome: toward a scientific answer. Scar tissue can block the flow of fluids around the spinal cord. Although surgery in adults involves greater risk Methods: The exact steps will depend on individual needs. The tethered spinal cord has to be untethered, i.e., adhesions between nervous tissue and surrounding connective tissue have to be broken. Patients with these conditions frequently have dysfunction related to their abnormality and experience bowel and/or bladder incontinence because the innervation of these organs overlaps with the lower lumbar and sacral roots. There is a normal distribution of position of the conus in healthy patients, and a position at one end of this range does not mean surgery is indicated. Epub 2015 Feb 25. Most of these congenital disorders are amenable to surgical treatment. Syringomyelia and tethered cord in children. 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. It is worth remembering that recent randomized trials with a sham surgery arm demonstrated significant clinical improvement in the sham surgery group.6,7 Incontinence is so common in children as to be regarded as almost normal, and it is difficult to quantify, even with urodynamic testing.1,10,14,16,21. Patients with alleged tethered spinal cords are fairly common in pediatric neurosurgical practice. The significance of a conus that is low by one vertebral level, with “lumbarized” or “sacralized” vertebrae at the lumbosacral junction is unknown, and such a finding should probably be ignored, especially if the patients are normal neurologically (as are those under consideration in the current discussion. Tethered Cord Syndrome . The child underwent surgery and the cord was completely untethered. Surgery is the most common treatment to “untether” the spinal cord from the spinal tissue. 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. 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?. Officially, once the patient has stopped growing, asymptomatic Tethered Cord Syndrome is usually not recommended for surgery, the recommended treatment is observation. Progress in resolving issues related to the myriad conditions that can masquerade as a tethered spinal cord will only come with better information from basic research and prospective clinical trials. Neurosurg Focus. Patient Resources. Childs Nerv Syst 19:3–10, 2003, Selden NR: Occult tethered cord syndrome: the case for surgery. 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. N Engl J Med 347:81–88, 2002, Olanow CW, , Goetz CG, , Kordower JH, , Stoessl AJ, , Sossi V, & Brin MF, : A double-blind controlled trial of bilateral fetal nigral transplantation in Parkinson's disease. Epub 2018 Feb 25. The pathogenesis of scoliosis is as poorly understood as that of the tethered spinal cord, so invoking one to explain the other really muddies the waters. ✓The tethered spinal cord is a fascinating yet controversial condition seen frequently in neurosurgical practice. The exact steps will depend on individual needs. More than 40 percent of children who have spina bifida will need surgery to untether the spinal cord during their lifetimes. Surgery in adult patients with a tethered cord syndrome should be reserved for those with symptoms. By making a cut in the skin, removing some bone from the spine so the spinal cord is exposed. These studies document encouraging clinical outcomes following surgery. 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. There are several caveats about surgery for these lesions. J Urol 169:2344–2346, 2003, Warder DE, & Oakes WJ: Tethered cord syndrome and the conus in a normal position. The syndrome, treatments, outcomes, and current controversies are re-viewed. Counting the vertebrae to assign conus position can be difficult, and the results may have little meaning. The underlying mechanism is related to impairment of oxidative metabolism in this region. Childs Nerv Syst. This theory is derived from redox changes of cytochrome a, a3, and supported by spinal cord blood flow, evoked potential, and histological studies. Tethered Cord Release Surgery. 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. With a fatty filum and a normal-level conus controversy regarding the management of syringomyelia associated with either spins abnormality! 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