Primary Tethered Cord Syndrome Research Articles

Disparities in Indications and Outcomes Reporting for Spinal Column Shortening for Tethered Cord Syndrome: The Need for a Standardized Approach.

Systematic review. To identify commonly reported indications and outcomes in spinal column shortening (SCS) procedures. SCS is a surgical procedure used in patients with tethered cord syndrome (TCS)-characterized by abnormal attachment of neural components to surrounding tissues-to shorten the vertebral column, release tension on the spinal cord/neural elements, and alleviate associated symptoms. PubMed and EMBASE searches captured SCS literature published between 1950 and 2023. Prospective/retrospective cohort studies and case series were included without age limit or required follow-up period. Review articles without new patient presentations, meta-analyses, systematic reviews, conference abstracts, and letters were excluded. Studies included adult and pediatric patients. The 29 identified studies represented 278 patients (age 5-76y). In 24.1% of studies, patients underwent primary TCS intervention via SCS. In 41.4% of studies, patients underwent SCS after failed previous primary detethering (24.1% of studies were mixed and 10.3% were unspecified). The most commonly reported non-genitourinary/bowel surgical indications were back pain (55.2%), lower-extremity pain (48.3%), lower-extremity weakness (48.3%), lower-extremity numbness (34.5%), and lower-extremity motor dysfunction (34.5%). Genitourinary/bowel symptoms were most often described as nonspecific bladder dysfunction (58.6%), bladder incontinence (34.5%), and bowel dysfunction (31.0%). After SCS, non-genitourinary/bowel outcomes included lower-extremity pain (44.8%), back pain (31.0%), and lower-extremity sensory and motor function (both 31.0%). Bladder dysfunction (79.3%), bowel dysfunction (34.5%), and bladder incontinence (13.8%) were commonly reported genitourinary/bowel outcomes. In total, 40 presenting surgical indication categories and 33 unique outcome measures were reported across studies. Seventeen of the 278 patients (6.1%) experienced a complication. The SCS surgical literature displays variability in operative indications and postoperative outcomes. The lack of common reporting mechanisms impedes higher-level analysis. A standardized outcomes measurement tool, encompassing both patient-reported outcome measures and objective metrics, is necessary. Level 4.

Urological presentations of adult primary tethered cord syndrome.

To investigate the urological presentations of patients initially diagnosed with closed spinal dysraphism in adulthood: adult primary tethered cord syndrome (ApTCS). Patients with ApTCS who underwent subsequent untethering were studied. Urological presentations were evaluated grouped by symptom onset period of either after (group 1) or before (group 2) the completion of physical growth. Of the 43 patients, median age at diagnosis and initial untethering was 35.5 (18.4-67.2) years. At diagnosis, 90.7% (39/43) had urological symptoms and 18.6% (8/43) initially consulted a urologist without noticeable neurological symptoms. Enuresis was observed in 16.3% (7/43) and common among patients without significant neurological symptoms (62.5%, 5/8). Urgency urinary incontinence and frequency prevailed in group 1. Stress urinary incontinence and straining to void prevailed in group 2. Among 26 patients with pre- and post-operative urodynamics, detrusor overactivity predominated in group 1 and acontractile detrusor predominated in group 2. Glomerular filtration rate ≥90 mL/min/1.73m2 was noted in 86.0% (37/43) and 84.9% (73/86) of renal units were morphometrically intact. More patients in group 2 (93.3%) relied on assisted emptying than in group 1 (53.6%). In ApTCS, enuresis was prevalent among patients without noticeable neurological symptoms. Over 80% in both groups had favorable renal function. Bladder conditions were different based on symptom onset period. Significantly more patients whose symptoms developed before completion of growth relied on assisted emptying. Proper discerning might be crucial in ApTCS which has a potential of misdiagnosis even in the modern developed era.

Clinico pathological study of tethered cord syndrome

Background: The disorder remains a challenge for both diagnostic and therapeutic measures. Today there is no question, however, that neurosurgery plays an important role in its treatment and indications for surgery have now widened rather than narrowed. Early diagnosis to prevent neurological deterioration is an urgent requirement. The objective of the study was, the current study focuses on tethered cord syndrome by age group, of the more common causes of the condition, as well as the adult presentation of occult TCS, its presentation and management. Methods: This is a prospective study conducted on 44 patients who presented to outpatient department of Neurosurgery, Osmania Medical College/Hospital, Hyderabad between August 2011 to February 2014. All the patients were diagnosed based on clinical features investigations like X-ray spine and brain screening apart from routine investigations to assess fitness for surgery. All patients were counseled regarding the diagnosis, its treatment options, possible complications including intra operative bleeding, infection, cerebrospinal fluid leak/fistula formation, wound dehiscence, possibility of improvement, worsening, persistence of symptoms, retethering of cord and after obtained informed consent were included in the study and subjected to surgery. Results: Infants (<1 year) are the most common age group of presentation as was observed in other series. There is no significant difference in incidence of tethered cord syndrome among males or females with a slight inclination towards males as was observed in other studies. Swelling over lower back with associated cutaneous lesion since birth are the most common presentations among children. Neurological deficits, bladder incontinence, bone deformities associated with tethering are commonly seen in elderly children or adults presenting late and do not subside / improve easily once they set in. Primary Tethered cord syndrome is more common than acquired. Back pain is generally a feature of presentation in elderly children and adults and probably the only symptom which subsides after detethering. Conclusions: MRI is the best diagnostic tool for identification of tethering and associated anomalies. Myelomeningocele and Lipomyelomeningocele are the most common associated anomalies with tethered cord syndrome. Proper identification of filum terminale, arachnoid bands and rootlets by electrophysiological monitoring intra operatively and good anatomical knowledge and proper establishment of CSF flow are essential for preventing retethering.

Tethered cord syndrome in children: a single-center experience with 162 patients.

Tethered cord syndrome (TCS) is not an uncommon clinical problem in children. The aim of this retrospective study is to document our experience on the surgical treatment of TCS in childhood. The data of 162 children who underwent surgical treatment for TCS in a 15-year period were reviewed retrospectively. Their demographic, clinical, radiological, and surgical features were documented. They were divided into two groups as primary and secondary TCS, and the surgical technique for each group was demonstrated. Untethering the spinal cord and correction of the associated malformation were the standard surgical technique for each patient. The results of the treatment were summarized. Among the 162 children, 101 (62.3%) of them were female and 61 were male with a mean age of 62 months. Primary TCS was detected in 43 patients while secondary TCS was found in 119 (73.4%) patients. Hypertrichosis was the most common physical finding while back pain was the common complaint. Lipoma, split cord malformation, dermal sinus tract, and myelomeningocele were the associated malformations for secondary TCS. Children should be individualized for the treatment of TCS. Each patient must be evaluated neurologically and radiologically for the accurate diagnosis. Surgical untethering is the safe and effective method of treatment for children with TCS.

The Effect of Untethering on Urologic Symptoms and Urodynamic Parameters in Children With Primary Tethered Cord Syndrome

To evaluate urinary system symptoms (USSs) and urodynamic parameters (UPs) before and after untethering in children with primary tethered cord syndrome (pTCS). USSs and UPs of patients undergoing untethering for pTCS during the period January 2008-July 2012 were evaluated preoperatively and at the postoperative third and 12th months. For analysis, patients were separated into 4 groups according to the presence of USSs: group 1, USSs preoperative positive and postoperative negative; group 2, USSs preoperative positive and postoperative positive; group 3, USSs preoperative negative and postoperative positive; group 4, USSs preoperative negative and postoperative negative. Preoperative and postoperative USSs and UPs were compared. Forty patients (average age, 7.2 years, follow-up of 2.8 years) were included. There were 13 patients in group 1, 11 in group 2, 3 in group 3, and 13 in group 4. All patients showed improvement when preoperative and postoperative USSs and UPs were compared. There was no correlation between USSs and UPs, both preoperatively and postoperatively. USSs and UPs at the postoperative third and 12th months were similar. Patients with no USS showed the most significant improvement in UP after untethering. Our study has demonstrated that untethering in patients with pTCS improves urologic symptoms and UPs. However, there is no correlation between improvement in symptoms and urodynamic findings. Urodynamic changes are similar at the postoperative third and 12th months. As the most significant improvement was seen in patients without USSs, it is important that these patients undergo urodynamic studies preoperatively and postoperatively.

Primary Tethered Cord Syndrome - Clinical and Urological Manifestations, Diagnosis and Management: A Prospective Study

: Primary tethered cord syndrome refers to a group of neural tube defects that are not externally obvious, and, if detected at an early age, surgical intervention may prevent the significant irreversible neurological deficits. This study was performed to evaluate the presenting clinical features of patients with primary tethered cord syndrome and the indications of surgery in such patients as well as the clinical and urological outcome. In all cases, the indication for surgery was the presence of a tethered cord on magnetic resonance imaging, the criteria for tethering being a low-lying conus (below L₁-L₂) and a thickened filum (>2 mm). Urodynamic studies were performed before detethering. Microsurgical detethering of low-lying cord was then performed, and the patients were then followed clinically and urologically for 6 months. Pain responded the most to detethering while limb weakness and urological symptoms responded the least. Clinical improvement in urological symptoms correlated with improvement in urodynamic parameters. A urodynamic study identified improvement in a larger number of patients and also deterioration in a few patients which was not visible clinically; this may point to its high sensitivity and usefulness in preceding clinical manifestations in a future follow-up.

Safety and Efficacy of Concurrent Pediatric Spinal Cord Untethering and Deformity Correction

A retrospective clinical records analysis of concurrent pediatric spinal cord deformity correction and tethered cord release compared with a 2-staged approach. To compare the safety and efficacy of a single-staged approach for pediatric spinal deformity correction and tethered cord release to a conventional 2-staged approach. Tethered cord syndrome (TCS) is frequently associated with scoliosis in the pediatric population. Conventional practice suggests waiting several months after untethering for scoliosis correction; however, some patients will experience progression of their spinal deformity. We report the efficacy and safety of concurrent tethered cord release and scoliosis and/or kyphosis deformity correction in a series of pediatric patients. We retrospectively reviewed 15 consecutive pediatric cases of concurrent spinal cord untethering and deformity correction with fusion for scoliosis and/or kyphosis. The clinical and radiologic presentation, operative details, morbidity, and postoperative outcomes were evaluated. Outcomes of this cohort were then compared with 21 patients who underwent a 2-staged untethering surgery followed by scoliosis correction. We provide a review of the literature of the treatment of tethered cord associated with spine deformities. The mean age of patients undergoing concurrent untethering and curve correction was 9.6 years (5 male, 10 female). Tethered cord was because of myelomeningocele (5 patients), thickened filum terminale (5 patients), lipomyelomeningocele (4 patients), and retethering from an unknown primary TCS etiology (1 patient). The mean scoliosis Cobb angle (±SD) at presentation was 55.4±21.0 degrees (range, 32.3 degrees to 95.0 degrees) whereas average kyphosis was 112.7±43.6 degrees (range, 68.0 degrees to 155.0 degrees). Average postoperative scoliosis curve was 40.0 degrees, resulting in an average correction of 27%; kyphosis curve was 55.7 degrees resulting in an average correction of 50%. The average operation time was 8.6 hours (range, 3.9 to 13.7 h) and the average blood loss was 1266 mL (range, 400 to 5000 mL). Average length of hospitalization was 10.1 days (range, 4 to 34 d). New onset or worsening of neurologic deficits, bowel or bladder dysfunction, or TCS associated pain did not occur in any patients. At a mean follow-up of 5.7 years (range, 1.3 to 11.8 y), only 1 (7%) patient required subsequent surgery for pseudoarthrosis. The 2-staged cohort experienced a longer cumulative operative time (11.2 vs 8.6 h, P<0.05), more total blood loss (1534 vs 1266 mL, P<0.05), longer total days of hospitalization (14.8 vs 10.1 d, P<0.05), and a greater incidence of dural tear (9.5% vs 0%), wound infection (26% vs 0%), and retethering (9.5% vs 0%). Concurrent tethered cord release and spinal fusion for correction of scoliosis and/or kyphosis may be a safe and effective approach in patients likely to experience deformity progression.

Improved symptoms and lifestyle more than 20 years after untethering surgery for primary tethered cord syndrome

Evaluation of the long-term effectiveness of untethering surgery on social adaptations to urinary symptoms at the time of the survey. Postoperative analysis of the current activities of daily life implemented as a postal questionnaire survey. Completed questionnaires were returned by 33 of 52 patients with primary tethered cord syndrome (p-TCS) who had undergone untethering surgery more than 20 years ago, including 10 patients with tight filum terminale (TFT) and 23 with cauda equina adhesion syndrome (CEAS). Long-term follow-up surveys in patients with p-TCS after untethering surgery indicated excellent effects on lower urinary tract symptoms in 9 of 10 patients with TFT, and the progression of symptoms tended to improve or halted in over half of the 23 patients with CEAS. However, one patient with TFT in whom new symptoms of voiding difficulty appeared immediately after surgery and 7 with CEAS showed deterioration of urinary and/or bowel symptoms. Eighteen patients complained of some symptoms caused by lower urinary tract dysfunction after untethering surgery in terms of difficulty in activities of daily life. With the exception of one female p-TCS patient with short stature and low body weight, all data of physical status, as well as educational level, employment, occupational category and marriage statistics were within the normal respective ranges taken from the Japanese census. Postoperative long-term follow-up survey in patients with p-TCS indicated significant improvement of urological symptoms. However, 55% of patients had some lower urinary tract dysfunction that affected their activities in daily life.

Wirbelsäulenerkrankungen und assoziierte ZNS-Fehlbildungen – Tethered-Cord und Arnold-Chiari-Fehlbildung

Tethered cord syndrome (TCS) and the Chiari malformation (CM) are generally related to congenital malformations, but both entities can be acquired.TCS often presents with progressing sensory and motor symptoms that can be attributed to increased tension of the spinal cord. Fixation of the spinal cord can occur congenitally (primary TCS) or in association with other intraspinal pathologies or postoperative scarring (secondary TCS). Exact diagnosis in the adult can be difficult if symptoms are interpreted as being related to degenerative disorders of the spine. In the presence of neurological symptoms, surgery is indicated. A preventive surgical procedure in asymptomatic patients is discussed controversially in the literature and is established in TCS patients only with correction of a scoliosis. However, most patients suffering from TCS benefit from surgical treatment.CM can be divided into four abnormal alterations of the posterior fossa, with most cases being of types I and II. In adults, Chiari type I is most common. Chiari I may present congenitally; however, it is also secondarily observed following lumboperitoneal shunting or in association with hydrocephalus. Surgical intervention is recommended depending on the extent of herniated tissue and the neurological symptoms. The optimal surgical procedure is still controversial. Most centers recommend bony decompression with dural graft extension for Chiari I.The appropriate treatment of both complex disorders requires a close and critical interdisciplinary approach in order to offer patients an individually adapted therapy.

Outcome in Patients Who Underwent Tethered Cord Release for Occult Spinal Dysraphism

Tethered cord syndrome encompasses a group of clinical symptoms caused by abnormal spinal cord fixation. We evaluated a select cohort of patients with primary tethered cord syndrome in regard to urodynamic and clinical outcome after cord release. We retrospectively reviewed the records of patients with the diagnosis of tethered cord from May 2001 to October 2004. Patients were assessed preoperatively by standard urodynamic studies, which was repeated a mean of 6.4 months after tethered cord release. Clinical and urodynamic outcomes were analyzed. Ten male and 14 female patients 1 month to 12 years old (median age 6 years) were evaluable. Preoperatively 14 of the 24 patients with a median age of 8.1 years were toilet trained and 7 (50%) had diurnal incontinence. Constipation was noted in 10 of 24 patients (42%) and urinary tract infections developed in 6 (25%). Postoperatively only 1 patient (7%) experienced diurnal incontinence (p = 0.04). Constipation was observed in 6 patients (25%) and urinary tract infections developed in 1 (4.2%) (p = 0.29 and 0.07, respectively). Ten of the 21 children (48%) with abnormal urodynamics had normalized studies postoperatively. Ten of the 17 patients with neurogenic detrusor overactivity achieved normalization and 7 remained unchanged. Two of 3 patients with normal preoperative urodynamics had neurogenic detrusor overactivity and 1 had poor bladder compliance. Four patients with low bladder capacity and/or low compliance preoperatively did not improve. Our results suggest that tethered cord release is beneficial in terms of clinical and urodynamic outcomes. Patients with abnormal urodynamics had 48% improvement after tethered cord release. Neurogenic detrusor overactivity seems to respond better with 59% improvement in urodynamics. The level of the conus on magnetic resonance imaging did not seem to be predictive of urodynamic outcome. Patients with a normal bladder may show urodynamic deterioration postoperatively, which raises cause for concern.

Prevalence of Primary Tethered Cord Syndrome Associated with Occult Spinal Dysraphism in Primary School Children in Turkey

The prevalence and associated factors of primary tethered cord syndrome (PTCS) in primary school children were investigated. A cross-sectional study was performed in four demographically different primary schools in Turkey. Demographic, familial and physical data were collected from 5,499 children based on enuresis as a predominant symptom and dermatologic and orthopedic signs as clues of occult spinal dysraphism. Statistical analysis and input of the data were carried out with the SPSS package program 10.00, and logistic regression analysis was used to identify discriminating factors between enuretic children with or without neurologic signs. Of 5,499 analyzed children, 422 (7.7%) had enuresis nocturna, and 19.9% of 422 children had also daytime incontinence. Sixteen of these 422 enuretic children (3.8%) had several dermatologic signs. Five of them had spina bifida on plain radiographies, and 4 of them had cord tethering on lumbar MRI. Fifteen of 422 enuretic children (3.7%) had gait disturbances and orthopedic anomalies without cutaneous manifestations. Six of 15 children had spina bifida on plain graphies and 2 of them had tethered cord syndrome on MRI. The general prevalence of PTCS was found to be 0.1% of 5,499 analyzed children and 1.4% of enuretic children. A good outcome after untethering was found in 83.0% in this series. Practitioners should be aware of these clues of occult spinal dysraphism and resort to further radiologic and neurosurgical assessment. Early surgical intervention may halt the progression of the neurologic deficits and stabilize or reverse symptoms.

Review of tethered cord syndrome with a radiological and anatomical study: Case report

The primary tethered cord syndrome has been documented mainly in children and adolescents but also in adults, and patients may present with backache, neuromuscular skeletal changes such as club-foot, scoliosis, muscular atrophy, disturbances of gait, or dysfunction of bladder and rectum, or a combination of these conditions. The cadaveric case presented describes plain film radiographic and anatomical findings of spina bifida occulta at the first and second sacral levels, and an enlarged spinous process of the fifth lumbar vertebra, in a 78 year old male cadaver with a tethered spinal cord terminating at the first sacral level. During life, this man had not undergone surgery for tethered spinal cord.