Management of Recurrent Adult Tethered Cord Syndrome (printer-friendly)

and Introduction Abstract Recurrent tethered cord syndrome (TCS) can lead to significant progressive disability in adults. The diagnosis of TCS is made with a high degree of clinical suspicion. In the adult population, many patients receive inadequate care unless they are seen at a multidisciplinary clinic. Successful detethering procedures require careful intradural dissection and meticulous wound and dural closure. With multiple revision procedures, vertebral column shortening has become an appropriate alternative to surgical detethering.Recurrent tethered cord syndrome (TCS) can lead to significant progressive disability in adults. The diagnosis of TCS is made with a high degree of clinical suspicion. In the adult population, many patients receive inadequate care unless they are seen at a multidisciplinary clinic. Successful detethering procedures require careful intradural dissection and meticulous wound and dural closure. With multiple revision procedures, vertebral column shortening has become an appropriate alternative to surgical detethering. Introduction Tethered cord syndrome is a condition that appears most commonly in the pediatric population. It results from stretching of the spinal cord between 2 fixation points, and treatment entails untethering the spinal cord. Results can vary depending on the time period between presentation and the initiation of treatment. If treated early, patients can recover from their neurological deficits. Chronic or excessive stretching of the spinal cord can lead to permanent disability. Currently the literature suggests that metabolic derangements and alterations in oxidative metabolism contribute to the reversible symptoms associated with TCS. Once neuronal damage occurs, however, partial neurological deficits can persist despite untethering. For some patients, TCS can present de novo in adulthood or can impart recurrent clinical manifestations that extend into adulthood. Adult TCS may be neglected without the multidisciplinary care provided by a pediatric spina bifida clinic familiar with the natural history, diagnosis, and management of TCS. Given the fact that a majority of patients with spina bifida now live into adulthood, it is increasingly important to diagnose and appropriately treat TCS in adults. A lack of understanding or enthusiasm in treating adult TCS can lead to significant deterioration and Authors and Disclosures Patrick Shih, M.D., Ryan J. Halpin, M.D., Aruna Ganju, M.D., John C. Liu, M.D., and Tyler R. Koski, M.D. Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois Address correspondence to Tyler R. Koski, M.D., Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, 676 North St. Clair Street, Suite 2210, Chicago, Illinois 60611. email: [email protected]. Disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. Author contributions to the study and manuscript preparation include the following. Conception and design: Shih. Drafting the article: Shih, Halpin, Liu. Critically revising the article: Koski, Ganju. Reviewed final version of the manuscript and approved it for submission: Koski. Management of Recurrent Adult Tethered Cord Syndrome Patrick Shih, M.D.; Ryan J. Halpin, M.D.; Aruna Ganju, M.D.; John C. Liu, M.D.; Tyler R. Koski, M.D. Posted: 08/02/2010; Neurosurg Focus. 2010;29(1):E5 © 2010 American Association of Neurological Surgeons Management of Recurrent Adult Tethered Cord Syndrome (print... http://www.medscape.com/viewarticle/725081_print 1 of 10 8/11/10 10:25 AM permanent disability. Epidemiology The incidence of neural tube defects is 0.17–6.39 per 1000 live births worldwide. There has been a decline in incidence over the past 20 years largely due to folic acid supplementation, termination of pregnancy after prenatal diagnosis, and other unknown factors. At centers experienced with myelomeningocele, the 20to 25-year mortality rate is 24%. Significant improvements over the past couple of decades in the care of patients with myelomeningocele at centers for excellence have led to a growing population of these patients reaching adulthood. However, spinal cord tethering is a problem in this population. It occurs in 23% of patients between 1 month and 23.3 years after surgery. Twenty-nine percent of these cases occur in patients who have undergone > 1 tethered cord release procedure. Clinical Presentation In dealing with patients with TCS, a multidisciplinary team consisting of neurosurgeons, urologists, physiatrists, neurologists, orthopedic surgeons, and physical therapists is preferred. Patients with symptomatic TCS present with back pain, worsening motor weakness, or new bowel or bladder symptoms. Of these presenting symptoms, pain is the most common. Back pain occurs in 73% of patients presenting with TCS, whereas leg pain or sciatica is the presenting symptom in 56% of patients. Muscular weakness and bladder dysfunction occur as the presenting symptom in 78% and 71% of patients, respectively. Adults with a tethered cord tend to present in a delayed fashion compared with their pediatric counterparts. Adults may be in denial of any significant symptoms and unwilling to see their practitioner, and thus delaying their diagnosis and increasing their chances of disability. Progressive scoliosis, foot deformity, leg length discrepancy, or muscular atrophy diagnosed by a multidisciplinary team can also suggest TCS. Untreated, TCS can progress in 27.5%, 40%, and 60% of cases at 1, 2, and 5 years after diagnosis, respectively.