Tethered Cord Release Research Articles

Spinal Column Shortening for Secondary Tethered Cord Syndrome in Children: 2-Dimensional Operative Video.

Tethered cord syndrome is a constellation of signs and symptoms involving sensory and motor dysfunction of the lower extremities, bladder, and bowel. The pathophysiology may be from a mechanical stretch of the distal spinal cord causing deleterious changes in blood supply and metabolism. The standard of care for tethered cord syndrome has been tethered cord release. However, drawbacks of traditional tethered cord release include neurological injury, cerebrospinal fluid leak, and intradural scarring and retethering of the spinal cord (secondary tethering). As such, spinal column shortening was described to avoid these risks.1 We applied spinal column shortening to children and transitional adults with secondary tethered cord syndrome.1 As shown in cadaveric biomechanical studies, the objective of surgery is to shorten by the spinal column by 1.5 to 2.5 cm, to obtain the equivalent relief of traction on the spinal cord and nerve roots as direct circumferential untethering.2 In this video, we present the case of an 11-yr-old boy with a history of VATERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) and tethered cord syndromes manifest as bladder dysfunction. Our patient had segmentation and formation anomalies of the lumbosacral junction, imperforate anus, and leg length discrepancy. He had already undergone multiple tethered cord releases with no durable improvement in bladder function. After discussion of the advantages and disadvantages of spinal column shortening with the patient and his father, we decided to proceed with this treatment alternative. At 9 mo after surgery, the patient has satisfactory clinical and radiographic outcomes. Informed consent was obtained from the parents of the patient.

Decreased Movement Of Right Arm In A Non-verbal Child

HISTORY: A 4 y/o non-verbal male with Rubinstein-Taybi syndrome presented for poor motion of his right arm. The patient was noted to become fussy when his parents put his shirt on that morning and when they tried to move his right arm. There were no known injuries, trauma, fever, or recent illness. Birth history was unremarkable. He had a tethered cord release in his first year of life. At baseline he uses all extremities and can crawl but not walk. PHYSICAL EXAMINATION: T 97.9F BP 104/80 HR 115 Well appearing, nonverbal and wheelchair-dependent male. No ecchymosis or significant erythema to the right arm. Limited active and full passive ROM of right shoulder, elbow and wrist with fussiness noted on manipulation. No focal tenderness noted. 2/4 MSR in bilateral upper and lower extremities. DIFFERENTIAL DIAGNOSIS: 1. Radial Head Subluxation 2. Non-accidental Trauma - fracture or dislocation 3. Osteomyelitis 4. Septic Arthritis 5. Tumor/Malignancy TESTS AND RESULTS: R Shoulder and Elbow XRs: No fracture or soft tissue abnormality seen CRP 41.7 mg/L, ESR 44 mm/hr, Blood culture No growth MRI of Right Humerus: Effusion with surrounding enhancement in the right shoulder; subtle changes in the metaphysis of the right humerus with periosteal edema and enhancement FINAL WORKING DIAGNOSIS: Pre-B cell Acute Lymphoblastic Leukemia TREATMENT AND OUTCOMES 1. The patient underwent manual reduction of possible radial head subluxation, which was felt to be successful with improved ROM of the elbow. However, symptoms returned within a day. 2. Labs and imaging were concerning for infection and he was admitted for surgical wash-out of right shoulder and sent home on prolonged IV antibiotics. 3. He developed contralateral shoulder symptoms with prolonged fever and left shoulder MRI showed similar findings. 4. After multiple wash outs and rounds of IV antibiotics, Flow Cytometry was noted to be positive for Pre-B cell acute lymphoblastic leukemia, so patient was started on appropriate chemotherapy regimen and is currently in the maintenance phase. 5. This case demonstrates the difficulty in pediatrics of identifying the cause of medical issues in patients who are non-verbal, as well as the vast differential diagnoses possible in children with musculoskeletal complaints. It also highlights the need for close follow up to resolution of symptoms.

Minimally Invasive Tubular Tethered Cord Release in the Pediatric Population

Tethered cord syndrome (TCS) refers to a clinical constellation of signs and symptoms associated with tension in the spinal cord and associated with pathologiesrelated to abnormally low-lying conus medullaris. As minimally invasive spinal operations have become more prevalent in the past decade, we applied expanding indications to selected intradural procedures, such as tethered cord release (TCR). We present the first series of minimally invasive muscle-sparing TCR in the pediatric population. A retrospective review of the Texas Children's Hospital, Houston, neurosurgical database was conducted for patients who underwent TCR between 2010 and 2017. Charts were reviewed to determine the source of TCS and whether surgery was conducted in a minimally invasive fashion. To establish a cohort of control population, age-matched cases of open TCR were selected in the same. Exclusion criterion was non-fatty filum sources of TCS. The length of stay, operative time, estimated blood loss, and postoperative complications were recorded. Eleven patients underwent minimally invasive TCR. Mean age of the patients was 10.1 years. All patients underwent L4-5 tubular laminotomy and cord detethering by 2 pediatric neurosurgeons at Texas Children's Hospital. The average length of stay in the hospital was 2.6 days. The average operative time was 167 minutes and average estimated blood loss was 8.2 mL. Ten patients reported clinical improvement and 1 patient was lost to follow-up. Minimally invasive TCR is a safe and represents a viable alternative to the open approach with advantages such as minimal estimated blood loss, shortened length of stay, and postoperative pain control.

Importance of Physical Examination and Imaging in the Detection of Tethered Cord Syndrome.

Tethered cord syndrome (TCS) is a type of occult spinal dysraphism that may lead to permanent neurologic and orthopedic deficits. Infants with TCS may have lumbosacral cutaneous malformations (LsCMs). We studied 67 infants referred to a single pediatric urology practice for a urological concern unrelated to occult spinal dysraphism with no prior diagnosis of LsCM between March 1, 2015 and September 30, 2018. Each infant underwent a spinal ultrasound. If an abnormality was detected, they were referred to a pediatric neurosurgeon. The most common cutaneous manifestations were duplicated or bifurcated (46%) gluteal folds and gluteal asymmetry (16%). Fourteen (21%) of the 67 patients had an abnormal spinal ultrasound; 5 of the 14 infants underwent a lumbar magnetic resonance imaging. One infant had urodynamics studies and a tethered cord release. Pediatricians should be familiar with TCS and perform lumbar physical examinations for LsCMs suggestive of TCS to ensure prompt diagnosis and management and avoid potentially devastating complications.

The conus medullaris ratio: A new way to identify tethered cord on MRI

PurposeApproximately 25% of patients with anorectal malformation have tethered cord. The traditional way of determining conus medullaris level on magnetic resonance imaging (MRI) relies on counting vertebrae, which may be challenging due to vertebral numeric variation, segmentation anomalies, as well as transitional vertebral body anatomy. The purpose of this study was to utilize more reliable anatomic landmarks (foramen magnum, conus termination, and the upper limit of the iliac crest) to establish a consistent ratio that may differentiate patients with normal and low-lying conus. MethodsA single institution database search identified two groups of patients: 255 with normal and 85 with abnormal spinal MRI, who underwent tethered cord release. The conus medullaris ratio was calculated in both groups. The ratio was obtained by dividing the distance between the conus level and the iliac crest by the distance between the foramen magnum and the conus level (IRB # 16–2330). ResultsThe mean ratio was significantly higher in the non-tethered group compared to the tethered patients (0.184 [sd 0.03] versus 0.118 [sd 0.09]; P < .0001). The ratio proved to be a good discriminator between normal and abnormal patients, with area under the curve (AUC) equal to 0.749, meaning that at random, there is a 75% chance that the tethered cord patient will have a lower ratio than the non-tethered cord patient. Conclusion“The Conus Medullaris Ratio” is a good predictor of low-lying conus level on MRI and offers an easy alternative to counting vertebral body levels, particularly in patients with variant or abnormal vertebral body anatomy. Level of EvidenceII, Study of Diagnostic Test.

Incidental durotomy in the pediatric spine population.

OBJECTIVESpine surgery is less common in children than adults. These surgeries, like all others, are subject to complications such as bleeding, infection, and CSF leak. The rate of incidental durotomy in the pediatric population, and its associated complications, has scarcely been reported in the literature.METHODSThis is a retrospective chart review of all pediatric patients operated on at Wake Forest Baptist Health from 2012 to 2017 who underwent spine surgeries. The authors excluded any procedures with intended durotomy, such as tethered cord release or spinal cord tumor resection.RESULTSFrom 2012 to 2017, 318 pediatric patients underwent surgery for a variety of indications, including adolescent idiopathic scoliosis (51.9%), neuromuscular scoliosis (27.4%), thoracolumbar fracture (2.83%), and other non-fusion-related indications (3.77%). Of these patients, the average age was 14.1 years, and 71.0% were female. There were 6 total incidental durotomies, resulting in an overall incidence of 1.9%. The incidence was 18.5% in revision operations, compared to 0.34% for index surgeries. Comparison of the revision cohort to the durotomy cohort revealed a trend toward increased length of stay, operative time, and blood loss; however, the trends were not statistically significant. The pedicle probe was implicated in 3 cases and the exact cause was not ascertained in the remaining 3 cases. The 3 durotomies caused by pedicle probe were treated with bone wax; 1 was treated with dry Gelfoam application and 2 were treated with primary repair. Only 1 patient had a persistent leak postoperatively that eventually required wound revision.CONCLUSIONSIncidental durotomy is an uncommon occurrence in the pediatric spinal surgery population. The majority occurred during placement of pedicle screws, and they were easily treated with bone wax at the time of surgery. Awareness of the incidence, predisposing factors, and treatment options is important in preventing complications and disability.

Tethered cord release: what are the orthopaedic indicators and when is orthopaedic surgery needed?

Tethered cord release: what are the orthopaedic indicators and when is orthopaedic surgery needed?

Twenty years' experience with myelomeningocele management at a single institution: lessons learned.

The authors reviewed 20 years' experience with the surgical management of open myelomeningocele in a well-defined retrospective cohort from a single large academic medical center. Their goal was to define the characteristics of a modern cohort of children with myelomeningocele to allow for evidence-based decision-making for the treatment of these patients. After IRB approval was obtained, the authors queried an operative database maintained by the Department of Neurological Surgery at Children's Hospital of Pittsburgh for patients who underwent closure of a myelomeningocele between 1995 and 2015. They identified 153 infants, and a retrospective chart review was performed. Eighty-eight percent of the patients required placement of a ventriculoperitoneal shunt, and 15% of these patients acquired shunt-related infections. Eighteen percent of patients underwent Chiari malformation type II (CM-II) decompression. Sixteen percent of patients underwent a tethered cord release. Three percent of patients died within the 1st year of life. Predictors of an early demise included poor Apgar scores, large head circumference, and need for early CM-II decompression. Functional motor outcome was slightly better than predicted by anatomical level of defect. Myelomeningoceles represent a severe birth defect with life-threatening complications. The authors provide long-term follow-up data and insight into factors that contribute to early death.

Management of iatrogenic spinal cerebrospinal fluid leaks: A cohort of 124 patients

ObjectivesCerebrospinal fluid leaks are a frequent complication of spinal surgery, with reported rates between 2 and 20%. Management is highly variable and dependent on comorbidities, complexity of the index procedure, and surgeons’ experience. Treatment options include primary or delayed repair, with or without spinal drainage. Using a retrospective cohort, the authors aim to identify the appropriate management of iatrogenic spinal cerebrospinal fluid (CSF) leaks. Patients and MethodsWe queried our institutional database for postoperative spinal CSF leaks between 1/1/2007 and 3/14/2017 using Current Procedural Terminology (CPT) and International Classification of Disease (ICD) codes. Excluded were patients who had primarily intradural procedures such as tethered cord release, tumor resection, and posterior fossa decompression. Information regarding patient demographics, surgical characteristics, and postoperative course was gathered, including whether primary closure (with nonabsorbable suture) was achieved, lumbar drain placement at initial surgery, use of fibrin sealant, number of subsequent explorations, rate of infection, length of stay, and number of hospital admissions. ResultsOur cohort consisted of 124 patients who suffered intraoperative iatrogenic CSF leak out of 3965 procedures, for a rate of 3.1%. Primary dural closure (±lumbar drain) was attempted in 64 patients, with successful repair in 47 (73.4%). Lumbar drain placement (±primary closure) was performed in 49, with success in 43 (87.8%). Delayed exploration of the surgical wound was required in 34 patients. Patients in whom primary closure could not be achieved and did not have a lumbar drain placed had a 39.5% reexploration rate. Patients who were treated with delayed exploration had statistically significant increase in length of stay (19.6 vs. 7.8 days), hospital admissions (2.1 vs. 1.0), and infections (15 vs. 0). ConclusionCSF leaks are fraught with complications requiring reexploration for repair in 27.4% of cases. Primary repair of the leak and use of fibrin sealant upon discovery, with consideration of lumbar drain, should be performed whenever possible, as they are associated with shorter hospital stays, fewer hospital admissions, and lower rates of reoperation and infection.

88: Postnatal follow up of infants who underwent prenatal fetoscopic and open neural tube defect repair

To assess postnatal development and growth in infants who had prenatal neural tube defect (NTD) repair by either Fetoscopic or Open hysterotomy approach. Secondarily, we aimed to assess the need for hospital admission, surgeries and rehabilitative therapies in both groups. A total of 37 infants (19 Fetoscopic and 18 Open) were enrolled in this longitudinal prospective cohort study. Fetoscopically repaired infants were classified into Iterative (first 12 cases with changing technique) or Standardized (7 cases with standard technique) groups. Infant growth was assessed using anthropometric measurements from the medical record (weight, length and head circumference) transformed into percentiles using WHO nomograms. Information regarding rehabilitative (occupational, physical and speech) therapies, hospital admissions, surgeries and use of orthotics was obtained by parental interview. The tests were administered between 6 months and 5 years (Table). Gestational age at delivery was significantly higher in the Standardized group. No differences in weight, length or head circumference percentiles were detected. There was no difference in need for rehabilitative therapies between the Fetoscopic and Open groups, and nor was there any differences in the need for endoscopic third ventriculostomy (ETV), shunt placement or tethered cord release surgery detected between the groups. Rate of hospital admission and surgical intervention were also similar. The Open group had a higher need for orthotics (83.3%) than did the overall Fetoscopic group (43.8%) with lowest need being in the Iterative group (28.6%). Prenatal Fetoscopic NTD repair results in similar postnatal infant outcomes as those seen following NTD repair via open hysterotomy.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Lipomyelomeningocele for the urologist: Should we view it the same as myelomeningocele?

The primary urologic objectives for lipomyelomeningocele (LMM) and myelomeningocele (MM) are preserving renal integrity and achieving continence. Due to this common ground, LMM and MM are urologically treated the same. However, unlike MM, LMM may present with no evident functional concerns. Indications for and timing of tethered cord release (TCR) in LMM are therefore controversial. Long-term urologic outcomes are not well defined. Expectations for continence and potential for intermittent catheterization (CIC) following TCR in LMM are important for realistically counseling families regarding future needs. The present study aimed to identify prognostic factors for continence and need for CIC in LMM. The present study retrospectively identified 143 patients from the multidisciplinary clinic who underwent TCR for LMM between 1995 and 2010. Concomitant anorectal/genitourinary anomalies, filar lipoma, fatty filum, previous TCR, and follow-up <1 year were excluded. Analysis was limited to those toilet trained or aged ≥6years at latest follow-up. Lipomyelomeningocele was classified as dorsal, distal, transitional or chaotic. Pre- and post-TCR urologic status was assessed. Ability to achieve urinary continence, with or without CIC, was the primary outcome, and need for CIC was the secondary outcome of interest. A total of 56 patients met inclusion criteria. Median age at TCR was 4.4 months (range 1.0-224.0) with a median follow-up of 10.7 years (range 1.3-19.1); 68% were asymptomatic at presentation. Clinical symptoms were urologic in 7%. At the latest follow-up, 86% of patients were continent spontaneously or with CIC (Summary Fig.). Of the four patients who presented with urologic symptoms, all were continent, but three required CIC. Overall, 23% of patients required CIC. Median age at CIC initiation was 7.6 years (range 1.6-17.4). Long-term continence was not associated with any demographic, anatomic, surgical or functional variable. Need for CIC at latest follow-up was associated with symptomatic presentation, partial TCR, transitional lipoma, and high-risk pre-operative urodynamics. In this series of primary TCR for LMM, where 93% of patients were urologically asymptomatic before TCR, prospects for continence were excellent. No studied parameter clearly impacted continence; however, need for CIC was associated with multiple variables. Clear predictors for continence after TCR will require additional long-term patient outcomes. Families can anticipate 23% likelihood of CIC, which is considerably less than in MM, but long-term urologic follow-up is still strongly recommended.

Scoliosis in myelomeningocele: epidemiology, management, and functional outcome.

OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis. METHODS A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study. RESULTS Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42-0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature. CONCLUSIONS Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.

Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults.

OBJECTIVE Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique. METHODS The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome. RESULTS Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8-30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26-37 months). Computed tomography-based radiographic outcomes showed solid fusion and no instrumentation failure in all cases by the most recent follow-up. Five of 7 patients (71%) reported improvement in preoperative symptoms during the follow-up period. The mean differences in initial and most recent Scoliosis Research Society Outcomes Questionnaire and Oswestry Disability Index scores were 0.26 and -13%, respectively; minimum clinically important difference in SRS-22 and ODI were assumed to be 0.4% and -12.8%, respectively. CONCLUSIONS Spinal column shortening seems to represent a safe and efficacious alternative to traditional untethering of the spinal cord for tethered cord syndrome.

Clubfoot and Tethered Cord Syndrome: Results of Treatment With the Ponseti Method.

The Ponseti technique has demonstrated high success rates worldwide for the treatment of idiopathic clubfoot. The purpose of this study was to determine whether clubfoot associated with tethered cord syndrome (TCS) was more resistant to Ponseti treatment than isolated clubfoot. An IRB-approved retrospective cohort study of subjects undergoing Ponseti treatment of clubfoot between 2002 and 2013 was conducted. Subjects with TCS were matched to subjects with isolated clubfoot (1:2) on the basis of laterality, date of birth, sex, and age at presentation. Subject demographics, number of casts placed (pretenotomy and posttenotomy), and recurrence data were collected. Generalized logistic regression and linear mixed model regression analyses were used to compare recurrence within 2 years of the initiation of casting and the log number of casts needed to achieve an acceptable correction, respectively. Data from 24 subjects (16 isolated clubfeet, 8 with TCS) with clubfoot (12 bilateral, 12 unilateral) were analyzed. The isolated clubfoot group was the same age at presentation on average (21.9±4.7 d) as the TCS group (28.3±9.6 d) (P=0.55). The number of casts required to achieve an acceptable correction was 54% higher (95% CI, 7.8%-120.3%; P=0.0217) in the TCS group compared with the isolated clubfoot group. The cumulative crude incidence of deformity recurrence within the first 2 years after casting initiation was 8% in the isolated clubfoot group compared with 42% in the TCS group. The odds of deformity recurrence in the TCS group were 5.6 (95% CI, 0.7-45.2; P=0.1054) times the odds of deformity recurrence in the isolated clubfoot group. Furthermore, the incidence of deformity recurrence was higher among subjects who had a tethered cord release posttenotomy (56%, 5/9) as compared with pretenotomy (0%, 0/3). Clubfoot associated with TCS required more casts to achieve an acceptable correction. Subjects with tethered cord were also at an increased risk of deformity recurrence compared with subjects with isolated clubfoot. Level II-retrospective prognostic study.

Response of Scoliosis in Children with Myelomeningocele to Surgical Release of Tethered Spinal Cord.

Objective: To examine the effect of surgical tethered cord release (TCR) on scoliosis in children with myelomeningocele. Methods: A retrospective review of 65 pediatric patients with myelomeningocele and TCR. The final sample consisted of 20 patients with scoliosis who were managed conservatively after TCR. Results: Average age at TCR was 6.2 years with average follow-up of 3.8 years. Scoliosis of 1 (5%) patient improved, 7 (35%) were stable, and 12 (60%) worsened (≥10°). Fifty percent of patients ultimately required definitive spinal surgery. TCR release delayed definitive spine surgery for an average of 3.2 years. Sixty-four percent of patients with curves less than or equal to 45° had progression of their curves compared to 50% with curves greater than 45°. For patients with curves less than or equal to 45°, curves progressed in 80% of those younger than 10 years as compared to 25% of those older than 10 years. For patients with curves less than or equal to 45°, 43% required definitive spine surgery as opposed to 83% with curves greater than 45°. Level of neurological involvement (ie, lumbar versus thoracic) and age at untethering emerged as factors influencing the effects of TCR for patients with curves less than or equal to 45°. Lumbar curves had more favorable results. Conclusion: Pediatric patients with myelomeningocele and scoliosis should be closely assessed and monitored. A selective approach for youth with lumbosacral level myelomeningocele and progressive curves less than or equal to 45° may result in scoliosis stabilization and avoidance of definitive surgery.

Abstract: Myofascial Flap Closures Decrease Cerebrospinal Fluid Leaks in Tethered Cord Patients

INTRODUCTION: The benefits of spinal surgery for patients with symptomatic tethered cord syndrome are well-described, however complications such as cerebrospinal fluid leak, seroma, and infection frequently occur. Recent studies have shown many advantages of myofascial flap closure for various spinal procedures. This study investigated whether the collaboration between neurosurgery and plastic surgery results in better outcomes and lower complications for patients undergoing tethered cord release. METHODS: A retrospective review of patients presenting to Weill Cornell Medical Center from 2010 to 2014 for tethered cord surgery was performed. All patients who underwent tethered cord release by three neurosurgeons with or without involvement of a plastic surgeon were included in the study. Data including patient demographics, medical co-morbidities, surgical history, complications, and follow-up information were collected and analyzed from clinical charts. RESULTS: Sixty-six patients underwent tethered cord release at Weill Cornell Medical Center from 2010 to 2014. Fifty-one (77%) patients had tethered cord release without myofascial flap closure, while fifteen patients (23%) had tethered cord release with myofascial flap closure. In the non-flap group, there were 46 primary dural closures (92%) with 6 cases of cerebrospinal fluid leak (12.7%), 10 reoperations (19.6%), 5 infections (9.8%), and 3 seromas (6.4%). In the myofascial flap group, there were 11 primary dural closures (78%) with no cerebrospinal fluid leaks, no reoperations, 1 infection (6.7%), and 1 seroma (6.7%). CONCLUSION: Our results suggest that patients undergoing tethered cord release may benefit from myofascial flap spine closure. Bringing in well-vascularized tissue to obliterate potential space and create a water-tight seal around the dural repair can decrease rates of cerebrospinal fluid leak. This study supports the idea that neurosurgical patients should consider plastic surgical consultation when considering tethered cord surgery. DISCLOSURE/FINANCIAL SUPPORT:None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this manuscript.

Foreword.

Spina bifida is one of the most common congenital birth defects in the United States, with prevalence estimated at 3 per 10,000 live births.1 Prevalence varies considerably worldwide, with some regions in China reporting rates more than 20 times as high.2 Within the United States, the highest incidence rate is among the Hispanics (4.2 per 10,000 live births), followed by non-Hispanic whites (3.2 per 10,000) and non-Hispanic African Americans (2.6 per 10,000).3 Spina bifida is caused by the failure of the neural tube to close during embryonic development; however, causal factors remain largely unknown. Genetic factors are estimated at 60% to 70%, although specific genes have not been identified.4 The primary nongenetic factor is inadequate maternal folic consumption. Other factors include anticonvulsant therapy, diabetes mellitus, and obesity. Primary prevention efforts focus on folic acid supplementation, including countrywide food fortification programs (eg, United States, Canada, Chile, Costa Rica, and South Africa). Woman of childbearing age are recommended to take 0.4 mg per day and up to 4 mg for those at high risk (eg, first-degree relative or previous pregnancy with neural tube defect).There are 4 types of spina bifida, ranging in severity. Severity is dependent on the location and size of the malformation and its effect on the spinal cord and nerves. The most common and severe subtype of spina bifida compatible with life is myelomeningocele, in which the spinal cord and nerves protrude through open vertebrae. In meningocele, a more moderate form, spinal fluid and meninges protrude through the vertebral opening, but the spinal cord remains intact. Closed neural tube defects include lipomeningocele in which a lipoma involves the spinal cord and tethered cord syndrome. Occulta, the mildest form often resulting in no impairment, occurs when there is a small gap in the spinal column but no open lesion or sac.Prenatal diagnosis of a neural tube defect can be accomplished by ultrasonic evaluation of the shape of the fetal skull and spine and measurement of α-fetoprotein in the maternal blood. Following prenatal diagnosis, woman may elect termination or surgical repair of the lesion, either in utero or postnatally. In addition to neurological deficits related to the level of the spinal cord lesion, affected children may also experience a Chiari II malformation, hydrocephalus, orthopedic, urological, and neuropsychological sequelae. Moreover, youth with spina bifida are at risk for psychosocial challenges, including depressive symptoms, lower levels of self-concept, social difficulties, and lower perceived health-related quality of life.56 Spina bifida also poses challenges to caregivers and their families and has significant economic consequences.7This issue of Topics in Spinal Cord Injury Rehabilitation includes several articles on spina bifida. The first focuses on the treatment options of hydrocephalus associated with myelomeningocele. Ventriculoperitoneal shunts as well as endoscopic third ventriculostomy with choroid plexus cauterization and conservative management of relatively stable ventriculomegaly are presented by Norkett et al. Next, Altiok et al discuss the development of scoliosis in youth with spina bifida and the effect of tethered cord release on the progression of scoliosis. Then Dr. Wasserman and colleagues investigate the common concern of attention problems and executive function deficits in children with spina bifida, examining differences in identification, diagnosis, and treatment of attention-deficit/hyperactivity disorder in children with and without spina bifida.We hope you will find this issue informative, enjoyable to read, and thought provoking. As always, we thank you for your readership and contributions to TSCIR. Please continue to contact us with your questions, reflections, and ideas for future issues.

Technical Note: Inlay-Onlay Sandwich Graft Technique for Repairing Durotomy in Spine Surgery for Fragile Dura

Incidental durotomy and cerebrospinal fluid leak in spine surgery is a common, well described complication encountered in spine surgery that can have good outcomes with prompt recognition and appropriate repair or management. Additional levels of technical challenge arise when the dura is very thin and fails to hold suture lines in primary repair. We present three lumbar-spine cases with dural opening, intended or unintended, in the setting of thin native dura providing poor suture substrate. In each case, inlay-onlay sandwich graft technique was used to achieve a successful lasting dural closure. Two of these cases initially involved delayed failure of primary suture repair or standard basic dural draft. Re-exploration in these two cases revealed widened suture defects due to fragile native dura, resulting in delayed post-operative CSF leak. We also report a case of inlay-onlay sandwich patch graft for initial dural closure in adult tethered cord release in the setting of thin native dura, in which simple primary closure was not feasible. We illustrate this inlayonlay graft operative technique, which can potentially be used for repairing dural openings involving thin native dura without intrinsic tissue strength to support primary suture repair. We envision that this technique can also be further extrapolated or adapted to other regions of dural repair as needed.

Surgical treatment outcomes in children with tethered spinal cord syndrome. A prognosis on the basis of spinal 3T MRI tractography

The study objective was to identify factors affecting surgical treatment outcomes in children with tethered cord syndrome (TCS). The study included 21 TCS patients aged 1 to 14 years who underwent tethered cord release. The preoperative and postoperative data of clinical and neurophysiological examination and high field (3T) MRI tractography of the caudal spinal cord were compared. Regression of the TCS clinical and electrophysiological signs and the lack of pathological changes in the spinal cord tracts were observed in patients with filum terminale abnormalities and caudal lipomas after surgery. In patients with secondary spinal cord tethering caused by scar formation after lumbosacral myelomeningocele repair, a motor deficit was related to the interruption level of the spinal tracts, and surgical treatment did not lead to significant regression of the TCS clinical and electrophysiological signs. We consider the absence of pathological changes in the caudal spinal cord, based on spinal MRI tractography, as a favorable prognostic factor in TCS surgical treatment.

Iatrogenic intradural arachnoid cyst following tethered cord release in a child

Iatrogenic arachnoid cysts represent uncommon complications of intradural spinal procedures. Here we present the case of a 7-year-old girl who was found to have a symptomatic, pathologically proven, intradural arachnoid cyst 3years following tethered cord release. The patient originally presented with abnormal urodynamics testing and was found to have fatty infiltration in her filum terminale. She underwent sectioning of the filum terminale without complications. The patient presented 3years later with pain and neurogenic claudication. The patient was successfully treated with subarachnoid cyst fenestration with resolution of her bilateral lower extremity pain. Spinal intradural arachnoid cysts represent an important, though rare, postoperative complication of dural opening.