Surgical Untethering Research Articles

Do Not Forget the Spine MRI in Children With Arthrogryposis Multiplex Congenita: High Prevalence of Tethered Spinal Cord and Preliminary Clinical Findings Following Detethering.

Arthrogryposis multiplex congenita (AMC) encompasses congenital conditions with joint contractures in 2 or more joints. Patients with AMC may have scoliosis and neural axis malformations. The study aimed to determine the prevalence of tethered spinal cord (TSC), characterized by a low-lying conus medullaris, and secondarily, present preliminary findings following surgical untethering in children with AMC. Patients 18 years of age and younger with a diagnosis of AMC and a spine MRI were identified. The presence of a TSC was defined as a low-lying conus with termination at or below the lower third of the L2 vertebral body. A pediatric neurosurgeon and a pediatric orthopaedic surgeon independently reviewed MRIs. The medical records of patients with AMC who underwent untethering were examined to evaluate preoperative and postoperative clinical findings. The prevalence of TSC in our AMC patients was compared with published normative data using χ2 analysis. Forty-two of 105 AMC patients (40%) had TSCs. There was a greater percentage of patients with AMC and a TSC compared with the unaffected population (P<0.0001). Sixteen patients underwent detethering through filum terminale sectioning. Nine patients had preoperative neurological deficits in addition to their AMC. There were no postoperative complications. All patients had improvement in their bowel and bladder symptoms within 3 months after their detethering procedure. TSC is more prevalent in the pediatric population with AMC compared with those without AMC. MRI is recommended for all patients with AMC due to its high prevalence. Although not a benign procedure, surgical detethering may have potential benefits for children with AMC. Level IV-therapeutic study.

Regression of Chiari malformation type 2 following early postnatal meningomyelocele repair-a retrospective observation from an institutional series of patients.

Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that issurgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair. From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion. Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51months. In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.

Lumbar Spinal Stenosis Due To Spodylolisthesis- Surgical Compared With Nonsurgical Treatment

Background: Lumbar spinal stenosis with spondylolisthesis stands for severe pain and dysfunction because of the narrowing of the spinal canal and vertebral slip. With patients being treated either surgically or by other methods, distinction between the two is necessary to resolve controversies. Objectives: To conduct a comparative analysis between surgery and non-surgery in the management of lumbar spinal stenosis with spondylolisthesis using observational and randomized control study groups. Study Design: A Observational and randomized cohort comparative study Place and duration of study : department of neurosurgery lady reading hospital Peshawar from jan-2020 to jan-2023 Methods: Based on the observational and randomized cohort study conducted in the period jan-2020 to jan-2023 in the Lady Reading Hospital, 604 patients were included in the study. Collectively, treatment embraced either surgical untethering or other mean. Specific patient outcomes such as pain relief and functional gain were measured during the follow-up which ranged up to 3 years. Results: In terms of body pain, surgical intervention provided statistically significant and more superior results in a mean of 18. 1 as compared to nonsurgical management over 3 years of follow up, similarly for physical functions the mean improvement was of 18. 3 and for Oswestry Disability Index it was 16. 7. Conclusion: The decision of preferring surgical intervention for the treatment of lumbar spinal stenosis with spondylolisthesis correspond to the maximum long term advantages of surgical over non surgical treatment principles focusing on the pain relief and functional recovery. Therefore, the present outcomes favour surgical management as the most preferred treatment approach with the adoption of individualised management plans following thorough clinical examinations of the patients. Keywords: Spinal Stenosis, Spondylolisthesis , Surgery Outcomes

Spinal cord tethering and syringomyelia after trauma: impact of age and surgical outcome

Posttraumatic spinal cord tethering and syringomyelia frequently lead to progressive neurological loss. Although several studies demonstrated favourable outcome following spinal cord detethering with/without shunting, additional research is required as no clear consensus exists over the ideal treatment strategy and knowledge about prognostic demographic determinants is currently limited. In this investigation, we retrospectively investigated 67 patients (56 men, 11 women) who were surgically treated and followed for symptomatic spinal cord tethering and syringomyelia from 2012 to 2022 at our center. Age (B-coefficient 0.396) and severity of trauma to the spinal cord (B-coefficient − 0.462) have been identified as independent predictors for the rate of development of symptomatic spinal cord tethering and syringomyelia (p < 0.001). Following untethering surgery including expansion duraplasty with/without shunting, 65.9% of patients demonstrated an improvement of neurological loss (p < 0.001) whereas 50.0% of patients displayed amelioration of spasticity and/or neuropathic pain (p < 0.001). Conclusively, active screening for symptomatic spinal cord tethering and syringomyelia, particularly in younger patients with severe spinal trauma, is crucial as surgical untethering with/without shunting is able to achieve favourable clinical outcomes. This knowledge may enable clinicians to tailor treatment strategies in spinal cord injury patients suffering from progressive neurological loss towards a more optimal and personalized patient care.

The role of surgery in relieving calcified shunt site-related pain in patients with functioning VP shunt.

Shunt calcification is a known late sequela of ventriculoperitoneal (VP) shunt insertion and is associated with shunt malfunction. However, in some patients, while shunt functionality is preserved despite calcification of the catheters, they experience nociceptive symptoms. In this paper, the authors present their surgical experience in managing patients with a functional VP shunt and experiencing pain secondary to shunt calcification. We analysed outcomes of patients presenting with pain at the level of a calcified shunt who underwent surgical untethering of the calcified catheter from the soft tissues. This procedure was commenced by the senior author in 2015. Patients were collected prospectively from the databases of two institutions. Evidence of shunt calcification was confirmed on neuroimaging. Seven patients, two male and five female, were included. The mean age at untethering was 13.5years. The mean time interval between primary shunt surgery and symptom onset was 12years (range 6-16years). The commonest site of tethering was the neck (50%) followed by abdomen and chest (both 25%). Six patients underwent untethering of the catheter from soft tissues. One patient had removal of a redundant segment of calcified shunt left in situ during a previous revision. All patients experienced pain relief following shunt untethering. Untethering of calcified VP shunt catheters from soft tissue can be considered an effective treatment of shunt site pain and offered to patients presenting with a functional VP shunt.

TETHERED CORD SYNDROME: INSTITUTIONAL EXPERIENCE OF 40 CASES.

Tethered cord syndrome (TCS) encompasses a spectrum of congenital conditions that result in progressive neurological deterioration due to tension on the spinal cord and nerve root[1]. Patients with TCS present with a wide spectrum of symptoms hence detailed physical examination is vital in patients presenting with TCS. Although TCS is a well-known entity, it continues to pose challenges regarding diagnosis and management. Surgical untethering may either ameliorate these issues or halt their worsening if they have been long-standing for many years.

External validation of a new classification of spinal lipomas based on embryonic stage.

Contention exists regarding appropriate classification and management of spinal lipomas (SLs). Given the heterogeneity of SLs, omissions and overlap between surgically incomparable groups exist in conventional classification systems. The new classification of spinal lipoma (NCSL) recently proposed by Morota et al. delineates morphology by embryological pathogenesis and the resultant operative difficulty. Here, the authors aimed to validate the NCSL by applying it to patients who had been operated on at their institution. All children who had undergone resection for SL between 2014 and 2018 were included in this analysis. MRI studies were independently reviewed and classified by three adjudicators. Baseline characteristics, inter-adjudicator agreement, coexisting anomalies and/or malformations, and postoperative outcomes and complications were analyzed. Thirty-six patients underwent surgical untethering for SL: NCSL type 1 in 5 patients (14%), type 2 in 14 patients (39%), type 3 in 4 patients (11%), and type 4 in 13 patients (36%). All classification was agreed on first assignment by the adjudicators. Radical or near-radical resection, which was attempted in all patients, was always possible in those with type 1 and 4 SL, but never in those with type 2 and 3 SL. Neurological stabilization and/or improvement were observed in all patients at the last follow-up. The NCSL was found to be a logical and reproducible system to apply in this SL population. All cases were successfully classified with a high degree of inter-assessor agreement. Widespread establishment of a commonly adopted and clinically useful classification system will enable clinicians to improve patient selection as well as discussion with patient representatives during the decision-making process.

Predictors of surgical treatment in children with tethered fibrofatty filum terminale.

Thickened or fatty filum terminale is an occult lesion that can cause tethered cord syndrome requiring surgical untethering. This study's objectives were to estimate the incidence of tethered fibrofatty filum terminale (TFFT) in a large insured pediatric population, identify predictors of surgery among those TFFT patients, and assess a diagnostic algorithm. TFFT was defined according to the ICD-9-CM code for cord tethering (742.59), after excluding codes for diastematomyelia, lipomyelomeningocele, terminal myelocystocele, meningocele, and myelomeningocele. Utilizing the Optum Insight database for 2001-2014, the authors identified pediatric patients (< 21 years) in the US who were diagnosed with a tethered cord and estimated the TFFT incidence rates in that source population and the surgical untethering probability among TFFT patients over the 14-year period. Logistic regression was used to estimate the effects (adjusted OR and 95% CI) of age at diagnosis, sex, Charlson Comorbidity Index (CCI) score, diagnosis of Chiari malformation type I, diagnosis of syrinx, and the probability of surgery by US census region. Lastly, to evaluate their algorithm for identifying TFFT from ICD-9 codes, the authors estimated its positive predictive value (PPV) among 50 children who were diagnosed at their institution and met the ICD-9-CM criteria. There were 3218 diagnoses of TFFT, with 482 of these pediatric patients undergoing tethered cord release during the study period. The estimated incidence rate was 12.0 per 100,000/year (95% CI 11.6-12.4 per 100,000/year). The incidence rate was slightly higher in females than in males (12.7 vs 11.4 per 100,000/year). The probability of surgery in the total pediatric TFFT population was 15.0% (95% CI 13.8%-16.2%) and was greater in children with a syrinx (OR 2.2, 95% CI 1.6-3.0), children 7-11 years of age at diagnosis versus < 1 year (OR 1.5, 95% CI 1.1-2.0), CCI score ≥ 3 versus 0 (OR 2.3, 95% CI 1.4-3.8), and residents of the Western vs Northeastern US (OR 2.3, 95% CI 1.6-3.5). In the authors' own institution's database, the PPV of TFFT was 35/50 (70.0%, 95% CI 57.3%-82.7%) for identifying tethered cord due to fibrofatty filum terminale among childhood positives. Patients with comorbidities or an associated syrinx showed a higher risk of untethering procedures for TFFT. Also, surgery was appreciably more frequent in the Western US. These findings signify the need for a collaborative prospective cohort study of long-term outcomes for TFFT patients with and without surgery to determine which patients should have surgery.

Outcome of surgical untethering of tethered cord syndrome in adults due to spina bifida occulta

Background:. Children with spina bifida occulta require early surgery to prevent neurological deficits. The treatment of patients with a congenitally tethered cord who present in adulthood remains controversial.&#x0D; Objective: The aim of this study is to describe the outcome obtained in 61 adult patients with congenital TCS and no prior surgical treatment who underwent surgical untethering.&#x0D; Methods: This prospective study was conducted on 61 adult patients who underwent surgical untethering for spina bifida occulta at four neurosurgical centers in Baghdad / Iraq between March 2000 and January 2018. Patients who had undergone prior myelomeningocele repair or tethered cord release surgery were excluded.&#x0D; The most common intraoperative findings were lipomyelomeningocele (41%) and a tight terminal filum (36%). The follow-up duration ranged from 10.8 to 149.5 months (mean 20.9 months). Of the 34 patients with back pain, status improved in 65%, worsened in 3%, remained unchanged in 18%, and improved and later recurred in 15%. Lower-extremity pain improved in 16 patients (53%), remained unchanged in 23%, improved and then recurred in 17%, and worsened in 7%. Lower-extremity weakness improved in 47%, remained unchanged in 47%, and improved and then recurred in 5%. Finally, of the 17 patients with lower-extremity sensory changes,&#x0D; status improved in 35%, remained unchanged in 35%, and the information on five patients was unavailable. Surgical complications included three wound infections, one cerebrospinal fluid leak, and two pseudomeningoceles requiring surgical revision. One patient developed acute respiratory distress syndrome and sepsis postoperatively and died several days later.&#x0D; Conclusions: Adult-age presentation of a congenital tethered cord is unusual. Despite a slight increase in postoperative neurological injury in adults, surgery has relatively low risk and offers good potential for neurological improvement or stabilization. As in children, we recommend early surgery in adults with this disorder. The decision to undertake surgery, however, should be modulated by other factors such as a patient’s general medical condition and risk posed by anesthesia.

Contemporary management and outcome of myelomeningocele: the Rotterdam experience.

Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases. This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence. A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence. This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.

Lipomes du filum terminal : à propos de 17 cas et revue de littérature

Introduction Filum terminal lipomas are being identified with increasing frequency due to the use of modern imaging (MRI). It is a benign pathology when asymptomatic. Surgical untethering is the treatment. Its incidence is unknown, but it had been estimated to be approximately 0.1%. Surgical intervention in pediatric neurosurgery has increased during the last two decades. Tethered cord syndrome may be due to thickened and fatty structural elements within the filum terminal that render it appropriately tense preventing normal stretching and movement. Material and methods We study the clinical and imaging for 17 patients; this filum was associated to other pathologies in 41.17% of cases: diastematomyelia, lipomyelomeningocele, dermal sinus, myelomeningocele. Clinical findings were mainly the cutaneous stigmatas and the orthopedic deformities and less frequently urinary disorders 29.41% of patients were asymptomatic. All patients were explored with MRI. A medullary CT scan was performed in 58.82% of cases. Results All patients were operated between 2010 to 2016. Evolution was favorable in 8 cases, stabilisation in 7 and aggravation in 2 patients. Conclusion Filum terminal lipomas are a common incidental finding on lumber spinal. MRI surgery is the best option. Evolution after surgery is generally good.

Minimally Invasive Surgical Approach to Filum Sectioning: Technical Note.

Filum terminale lipoma (FTL) is known to cause various spinal symptoms collectively referred to as tethered cord syndrome (TCS). With the increasing use of magnetic resonance imaging, there has been an increase in the identification of FTLs. The treatment for FTL is surgical untethering by sectioning the filum terminale, which can prevent symptom progression and often results in improvement of symptoms. We recently introduced a new surgical strategy for filum terminale sectioning named interlaminar approach (ILA). In this surgical video, we present a minimally invasive technique of ILA. The surgical approach and postoperative result are outlined. A minimal 2 cm length of midline skin incision was performed at the L5 vertebral level. After skin incision, dissection was extended to expose the ligamentum flavum. The ligamentum flavum was then incised and retracted medially to expose the dural sac. After dural incision, the filum terminale was identified and pulled out from the dural sac and then sectioned. The maximum diameter of the filum terminale was 3 mm in our series. The surgical time for children was about 1 h. Adult patients need a deeper surgical field, so the operation takes more time. Until now, 46 patients (11 mo to 27 yr) have undergone ILA and been followed for more than 5 yr. Surgical complication was seen in only one patient who developed a subcutaneous fluid collection. All patients remained stable or showed improvement in preoperative symptoms. This technique provides the advantage of reduced soft tissue injury and minimal intradural procedures. Thin FTL showing TCS are candidates for ILA. This article complies with the guideline for patient information confidentiality and privacy protection. All the patients in this study provided written, informed consent for the procedure. Watch now at https://academic.oup.com/ons/article-lookup/doi/10.1093/ons/opx127 {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Minimally Invasive Surgical Approach to Filum Sectioning: Technical Note","caption":"","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_kcc30hx5"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} Disclosure The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.

Minimally Invasive Surgical Approach to Filum Lipoma

Filum terminale lipoma (FTL) causes various spinal symptoms known as tethered cord syndrome. The treatment for FTL is surgical untethering by sectioning the FTL, which can prevent symptom progression and often results in improvement of symptoms. This report describes a minimally invasive surgical strategy that we have introduced for FTL sectioning. The pediatric patients with FTL since 2007 were treated using this minimally invasive surgical strategy, which we refer to as an interlaminar approach (ILA). In summary, the surgical technique involves: minimal skin incision to expose the unilateral ligamentum flavum in the lower lumbar region; ligamentum flavum incision to expose the dural sac, and dural incision followed by identification and sectioning of the filum. Postoperatively, no bed rest was required. Prior to introducing ILA, we had used standard one level laminectomy/laminotomy (LL) with more than 1 week of postsurgical bed rest until 2007, providing an adequate control group for the benefit of the ILA. A total of 49 consecutive patients were treated using ILA. While 37 patients were treated using LL. Surgical complications that need surgery were seen only in one patient, who developed cerebrospinal fluid (CSF) leak in LL patients. No retethering or additional neurological symptoms were seen during follow-up. All patients complained of minimal postsurgical back pain, but no patients required postoperative bed rest in ILA patients, while LL patients need postsurgical bed rest because of back pain. The ILA strategy provides the advantage of a minimal tissue injury, associated with minimal postoperative pain, blood loss, and bed rest.

Surgical Outcome of Children and Adolescents with Tethered Cord Syndrome

Study DesignRetrospective cohort study.PurposeTo compare long-term results of surgery with the outcomes of symptomatic and asymptomatic tethered cord syndrome (TCS) in children and adolescents and to assess the surgery duration for those with TCS.Overview of LiteraturePediatric patients with TCS continue to pose significant diagnostic and management challenges.MethodsWe retrospectively analyzed the outcomes of 31 patients (16 males, 15 females) with TCS, including 21 with lumbosacral lipoma. All were surgically treated between 1989 and 2015. They were divided into symptomatic and asymptomatic TCS groups. The results of the treatment were summarized and analyzed using a non-parametric Mann–Whitney U test.ResultsNineteen patients had symptomatic TCS and 12 had asymptomatic TCS. Patients had a median age of 34 months (range, 0–201 months). The median follow-up period was 116 months (range, 7–223 months). Of the 19 symptomatic TCS patients, preoperative deficits improved after surgery in two (10.5%) and remained stable in 17 (89.5%) patients. One of the 12 asymptomatic TCS (8.3%) patients showed an exacerbated illness after surgery, and one in 11 patients remained stable (11.7%). There were significant differences in monthly age at surgery, preoperative bowel and bladder dysfunction, neurological function, presence/absence of clean intermittent catheterization introduction, and presence/absence of motor disorder at final follow-up (all p<0.05). In the univariate analysis, the presence/absence of preoperative bowel and bladder dysfunction, and symptoms were strongly associated with the risk of children and adolescents with TCS (p<0.05).ConclusionsEarly accurate diagnosis and adequate surgical release might lead to successful outcomes in children and adolescents with TCS. Surgical untethering is a safe and effective method for treatment of children with TCS.

Symptomatic retethering of the spinal cord in postoperative lipomyelomeningocele patients: a meta-analysis.

Timing of surgical treatment for tethered cord syndrome due to a lipomyelomeningocele (LMM) has been controversial. The purpose of this study was to evaluate populations of patients treated surgically for LMM in a meta-analysis in order to better understand how outcomes differ based on follow-up time, symptomatology, and LMM classification. An extensive search on PubMed and Google Scholar was performed for LMM and surgical outcomes to identify case series of patients for inclusion in this analysis. Patients were sorted based upon symptomatology prior to surgery and Chapman's LMM classification, where possible. Deterioration rates were determined by symptomatic retethering of the spinal cord that led to repeat surgery. Of 608 (19%) patients, 115 were included in the study experienced deterioration leading to repeat surgery. Symptomatic and asymptomatic patients did not experience significantly different rates of deterioration after surgical untethering. There was a significant positive linear correlation between follow-up time of studies and percentage of patients deteriorating with an increase of 3.3% per year of follow-up. Transitional LMM had a significantly higher rate of deterioration compared to the caudal type along with the entire patient pool. Outcomes of primary surgical treatment in regard to late deterioration are not significantly affected by patient symptomatology. Patient deterioration increases linearly over time. Additional studies should be performed to adequately determine the natural history of asymptomatic patients that are treated conservatively for LMM.

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.

Cervicothoracic nonterminal myelocystocele with mature teratoma

Nonterminal myelocystocele is a rare type of spinal dysraphism characterized by a closed defect with an underlying CSF-filled cyst, either contiguous with the central spinal canal or attached to the spinal cord by a fibrovascular stalk. The authors report the unusual case of a neonate with a prenatal diagnosis of cervicothoracic nonterminal myelocystocele who underwent postnatal surgical untethering of the lesion. Pathological analysis of the excised lesion revealed neuroglial tissue with an ependymal lining associated with a mature teratoma. Three months after surgery, the patient has normal lower-extremity sensorimotor function and no evidence of bowel or bladder dysfunction. To the best of the authors' knowledge, this is the first report of a patient with a nonterminal myelocystocele found to have an associated mature teratoma.

Surgical indication and outcome in patients with postoperative retethered cord syndrome

After untethering of spinal dysraphism, some patients present with neurological deterioration, defined as retethered cord syndrome. It is known that surgical untethering is an option for improving the symptoms of retethered cord syndrome. Previous reports have shown that postoperative improvement in retethered cord syndrome was noted in the majority of patients presenting with pain, and in more patients with motor weakness than in those with urological symptoms. The authors speculate that subjective symptoms may be detected while symptoms are still reversible. In contrast, changes in urological function are less easy to diagnose, and delays in treatment may be complicated by advanced symptoms. In this study, patients with retethered cord syndrome were evaluated to investigate the benefits of performing routine urodynamic study to detect detrusor overactivity, which is considered to be a subclinical change of urological function, and to investigate the efficacy of early untethering surgery on the symptoms of retethered cord syndrome. Surgical indications and outcomes of 78 untethering operations (20 for myelomeningocele, 58 for spinal lipoma) for retethered cord syndrome were examined. Diagnosis of retethered cord syndrome was defined by a multidisciplinary spina bifida team, and included routine urodynamic study. Preoperative symptoms included urological symptoms (70%), lower-extremity symptoms (45%), and others. The most frequent urological symptom was detrusor overactivity detected by urodynamic study (88.7%). Urinary incontinence was only found in 9.4% of patients. Postoperatively, progressive motor weakness improved in all patients, and sensory symptoms improved in 94%. Urological symptoms improved in 80% of the patients with urinary incontinence and in 75% of the patients with detrusor overactivity. Postoperative urodynamic study showed a significant increase in bladder volume (p < 0.05). The most common complication was temporary lower leg paresthesia that recovered at follow-up. Aggravated dysuria was noted in 3 patients. Early untethering operations offer symptomatic relief to patients with retethered cord syndrome. Urodynamic study findings, especially detrusor overactivity, are considered to be the most significant indicators for early diagnosis of retethered cord syndrome.

Asymptomatic lumbosacral lipomas—a natural history study

Inevitable deterioration due to mechanical tethering is perceived as the natural history for complex congenital spinal lipomas of the conus medullaris region, even if asymptomatic at presentation. The conventional wisdom that prophylactic surgical untethering improves outcome has been challenged recently [1, 2]. This study examines the natural history of asymptomatic un-operated children with lumbosacral lipomas (LSL) and investigates whether predictive factors herald deterioration. Over the past decade, children presenting with complex LSL to a single clinician at Great Ormond Street Hospital (GOSH), London, UK have undergone a thorough assessment focusing on neurological and urological evaluation and MRI of the lumbosacral spine. For children deemed to be asymptomatic, conservative management has been adopted with close periodic surveillance of neurological and urological function, thus avoiding untethering surgery unless symptomatic deterioration occurs. A retrospective review identified this cohort of children asymptomatic of their LSL and their progress closely recorded. This study suggests that the natural history of this subgroup of dysraphic patients may be more benign than hitherto considered. Conservative management with adoption of a novel surveillance policy and timely intervention only in the presence of symptomatic deterioration resulted in 71% of this series remaining clinically asymptomatic at mean follow up period of 5.9 years (range, 1.0-19.3 years). At 10 years, the cumulative risk of deterioration determined by the Kaplan-Meier method was 40%. Children aged<2 years, female, with presence of a transitional type of LSL and associated syrinx were independently associated with a higher risk of deterioration.

Inappropriate surgical interventions for midline fusion defects cause secondary tethered cord symptoms: implications for natural history report of four cases

The causes of tethered spinal cord are various. In order to release the tethering effect of these malformations, the surgical interventions must include removal of all tethering components, reconstruction of the neural tube and sectioning of tight filum terminale as well. The cases reported in this paper have had an operation many years before for various developmental defects. After a certain period of time (5-10years) of the first operation, the patients reapplied to the hospital with various symptoms of spinal cord tethering, either vertical or horizontal. At surgical intervention, it was noted that inappropriate surgical procedures caused retethering of the spinal cord in all patients. Postoperative period of all patients were uneventful. All patients declared relief in their symptoms. We would like to draw attention that untreated (or inappropriately treated) midline developmental defects will invariably cause syndrome of tethered cord. Consequently, prophylactic surgical untethering must be applied to all patients with developmental midline defects as soon as possible. It looks that tethered cord symptoms invariably appear as enough negative influence accumulates as the time passes. Elapsed time may vary but unpleasant end result invariably arrives. While these cases with tethered spinal cord develop progressive neurological symptoms, prophylactic and appropriate surgical intervention should be considered as early as possible. There is no acceptable rationale to wait for the appearance of tethered cord syndrome symptoms to perform surgical untethering of the spinal cord because of the probability of irreversibility of the symptoms (incontinence of urine in particular) of tethered spinal cord syndrome.