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Over the past few decades, there have been many advances in pediatric surgery, some using new devices (eg, VEPTR, MAGEC rods) and others using less invasive approaches (eg, Nuss procedure, endoscopic cranial suture release, minimally invasive tethered cord release). Although many of these procedures were initially met with caution or skepticism, continued experience over the past few decades has shown that these procedures are safe and effective. This article reviews the anesthetic considerations for these conditions and procedures.

Background We document the results of a protocol to reduce the incidence of cerebrospinal fluid (CSF) leak and other wound complications in patients undergoing tethered cord surgery (TCS). Methods Data from all patients undergoing TCS between January 2009 and April 2019 were reviewed retrospectively. Diagnosis (high risk or low risk; based on the presence of fascial and dural defects at surgery), type of graft used for dural or fascial repair, and CSF leak and other wound complications in the postoperative period were noted. All patients were nursed in the prone position with elevation of the foot end of the bed (Trendelenburg position) for at least 5 days after surgery with a subfascial drain in place. Results Of a total of 350 patients (191 high risk; 159 low risk), CSF leak from the wound was noted in 16 (4.5%). All but 4 of these patients were managed with wound suturing with or without insertion of a subcutaneous drain with continued nursing in the prone and Trendelenburg position. Two patients had meningitis and 3 patients had wound infection. Multivariate analysis revealed that the use of synthetic grafts (P Conclusion Postoperative prone nursing with Trendelenburg position minimizes the incidence of CSF leak and other wound complications.

Background Split cord malformations (SCMs) are among the rare congenital spinal anomalies. In 1992, Pang et al. proposed the unified theory of embryogenesis and explained the formation of SCM type 1 and 2. This theory has been widely accepted in the neurosurgical literature, backed by several studies. However, there have been reports in the literature that defy both the classification as well as the formation of SCMs, based on the unified theory of embryogenesis. We report a case of SCM that does not fit into this classification scheme and try to elucidate its embryologic basis, with review of the relevant literature. We also attempt to include this variety into the existing classification system of SCMs. Case Description An 11-year-old boy presented with low backache after trivial trauma. He was neurologically intact. Imaging showed low-lying tethered cord and a midline ventral bony spur (D12, L1) with a single dural sac encasing both the hemicords. Surgical exploration showed a ventral bony spur with 2 hemicords, enclosed in a single dural tube. Excision of the bony spur and detethering of the filum terminal were performed. The postoperative course was uneventful and the patient was discharged satisfactorily. Conclusions SCMs possibly represent a continuum of changes beginning at the gestational age of days 20–30. Terminology such as mixed or intermediate type is used to denote SCMs that show features of both type 1 and type II. We prefer using type 1.5 SCMs for all such cases, thereby avoiding confusion and maintaining uniformity in the nomenclature. However, further experimental studies are required to substantiate our understanding of these complex embryologic anomalies on the basis of current hypotheses.

Background Meningocele manque (MM) is characterized by tethering of the spinal cord, nerve roots, or filum terminale by fibrous attachments formed by atrophic or incomplete meningoceles. Patients with MM can present with symptoms of tethered cord syndrome (TCS). Case Description We present the case of an MM discovered incidentally during microsurgical resection of filum terminale for occult TCS. The MM was not visible on the preoperative magnetic resonance imaging (MRI) studies. After L2-L3 laminotomy, an extradural mass was appreciated adherent to the spinal dura, which was found to be an MM. The nerve roots of the cauda equina were found to protrude through the dura, consistent with tethered cauda equina. After microsurgical resection of the filum terminale, the tethered cauda nerve roots were stimulated, and redundant electromyographic signals were detected at both the left- and the right-sided muscles of the lower extremities. Microsurgical repair of the MM was performed, assuming that the patient's symptoms of TCS would also be explained by the MM and that her symptoms would likely have been incompletely addressed by filum terminale release alone. A review of the preoperative 3-dimensional constructive interference in steady state MRI sequence revealed pathological features at the L2-L3 level suspicious for our intraoperative findings of an MM. Conclusions Surgeons planning filum terminale release for occult TCS should always be aware of the possibility of associated pathological features of tethering that might be overlooked. In retrospect, the detection of MM was enabled by the high-resolution 3-dimensional constructive interference in steady state MRI sequence. This finding advocates for the use of high-resolution MRI for patients undergoing evaluation for occult TCS to detect pathological features that might otherwise be undetected.

Tethered cord syndrome is a rare and intractable disease. The aim of this study was to investigate surgical outcome of homogeneous spinal-shortening axial decompression (HSAD) for tethered cord syndrome through a minimum 3-year follow-up.Patients who underwent HSAD for tethered cord syndrome in our institution between May 2011 and July 2015 were included in this study. After a minimum 3-year follow-up, radiologic and clinical data were collected. Surgical outcome was evaluated with Japanese Orthopaedic Association, visual analog scale, International Consultation on Incontinence Questionnaire-Short Form, and Rintala scores and urodynamics.The study included 64 patients (28 men and 36 women) with a mean age of 35.7 ± 18.1 years. Significant improvement of visual analog scale and Japanese Orthopaedic Association scores was noted postoperatively and at a minimum 3-year follow-up. At the final follow-up, Japanese Orthopaedic Association and visual analog scale scores in all patients were improved compared with preoperatively. The International Consultation on Incontinence Questionnaire-Short Form score was decreased from 14.94 ± 4.15 preoperatively to 7.63 ± 4.40 at final follow-up (P0.01). Rintala score was improved from 5.26 ± 3.45 preoperatively to 12.32 ± 3.43 at final follow-up (P0.01). All urodynamic parameters improved compared with preoperatively.HSAD was effective and safe. The results were satisfactory with a minimum 3-year follow-up. HSAD could be an alternative surgical treatment of TCS.

Background Spinal cord stimulation (SCS) is an evidence-based treatment for chronic neuropathic pain; however, there is a dearth of evidence investigating this modality in patients with tethered cord syndrome. Case Description We present a case of 55-year-old woman with history of lipo-myelomeningocele repair and multiple detethering surgeries who presented with chronic low back and leg pain accompanied by progressive gait dysfunction. After a successful trial, she underwent SCS paddle lead placement that resulted in decrease of her visual analog scale for pain from 9/10 to 0–2/10 as well as daily opioid intake from 90 to 199 mg morphine-equivalent doses to 40 to 60 mg morphine-equivalent doses. On last follow-up she reported 70%–85% relief of her low back and leg pain, better ambulation, and improved quality of life. Conclusions The literature review identified 2 other case reports of SCS in tethered cord syndrome with similar improvement in pain alleviation.

Approximately 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.A 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).The 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."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.II, Study of Diagnostic Test.

Heterozygous gain of function mutations in the ZIC1 gene have been described with syndromic craniosynostosis, variable cerebral or cerebellar abnormalities and mild to moderate developmental delay. Deletion of chromosome 3q25.1 including both adjacent ZIC1 and ZIC4 genes have been described as a cause of variable cerebellar abnormalities including Dandy-Walker malformation. We report two siblings presenting with neonatal microcephaly, agenesis of the corpus callosum, brachycephaly with reduced volume of the posterior fossa, cerebellar and pons hypoplasia, scoliosis and tethered cord (closed neural tube defect). One of the siblings had apparent partial rhombencephalosynapsis. Trio analysis of exome sequencing data revealed a novel heterozygous frameshift mutation in ZIC1 at the end of exon 3 in one sibling and was confirmed by Sanger sequencing in both children. The mutation was not detected in DNA of both parents, which suggests parental gonadal mosaicism. We show that expression of the mutant allele leads to synthesis of a stable abnormal transcript in patient cells, without evidence for nonsense-mediated decay. Craniosynostosis was not present at birth, which explains why ZIC1 mutations were not initially considered. This severe brain malformation indicates that premature closure of sutures can be independent of the abnormal brain development in subjects with pathogenic variants in ZIC1.

Introduction: The prevalence of Chiari malformation type I (CM-I) has been estimated as up to 1% of the general population. The majority of patients are asymptomatic and usually do not need treatment. Symptomatic patients, and some asymptomatic patients with associated conditions, may benefit from further assessment and treatment. Research question: The aim of this review was to describe the clinical and radiological assessment of patients presenting with a CM-I. Material and methods: A literature search was performed using the PubMed and Embase databases focused on clinical assessment and imaging techniques used to diagnose CM-I. Results: Following a complete clinical evaluation in patients with symptomatic CM-I and/or radiologically significant CM-I (tonsillar impaction, resulting tonsillar asymmetry and loss of CSF spaces), MRI of the brain and whole spine enables an assessment of the CM-I and potential associated or causative conditions. These include hydrocephalus, syringomyelia, spinal dysraphism, and tethered cord. Flow and Cine MRI can provide information on CSF dynamics at the craniocervical junction, and help in surgical decision-making. Hypermobility or instability at the upper cervical and craniocervical junction is less common and can be measured with CT imaging and flexion/extension or upright MRI. Discussion and conclusion: The majority of CM-I detected are incidental findings on MRI imaging of brain or spine, and do not require intervention. Once a radiological diagnosis and concern has been raised, clinical assessment by an appropriate specialist is required. A MRI brain and cervical spine is indicated in all radiologically labelled CM-I. In symptomatic patients or cases of radiologically significant CM-I, MRI of the brain and entire spine is indicated. Further investigations should be tailored to individuals’ needs.

Background Tethered cord syndrome (TCS) is an increasingly recognized clinical condition, with untethering surgery considered the classically effective treatment. Yet, as evidence has aggregated, the surgical outcomes of untethering remain controversial. This study aimed to systematically evaluate the clinical outcomes in patients who underwent primary or revision untethering. Methods This retrospective study was conducted at the Department of Spine Surgery of Changzheng Hospital between January and December 2016. Patients with TCS who underwent untethering surgery were recruited for the study. Information collected included demographic data, main clinical manifestations, and outcomes after untethering surgery. Results A total of 112 patients (60 males and 52 females) were included in this study, with mean follow-up of 13.7 years. The surgical outcomes of the primary untethering varied among symptoms, with remission rates of 30.0% for pain, 24.4% for paresthesia, 18.6% for motor deficit, 12.6% for bladder dysfunction, and 21.2% for bowel dysfunction. Moreover, 23.3%–40.2% of patients suffered deterioration after surgery during the follow-up period. Twenty-eight patients underwent revision untethering surgery. In these patients, the remission rate decreased by 5.6%–16.7% compared with the primary operation. Moreover, most patients (58.8%–70.6%) experienced no benefits after revision surgery. Conclusions With a possible high risk of recurrence, further exploration of the indications and timing of the untethering surgery is needed. Conservative treatment and omnidirectional care might be a better choice for patients with retethering with recurrent symptoms.

This is a nationwide query into surgical management techniques for tethered cord syndrome, focusing on patient demographic, hospital characteristics, and treatment outcomes. Our hypothesis is that detethering vs. fusion for TCS results in different in-hospital complications.Retrospective review of the Nationwide Inpatient Sample 2001-2010. Inclusion: TCS discharges undergoing detethering or fusion. Sub-analysis compared TCS cases by age (pediatric [≤9years] vs. adolescent [10-18year]). Independent t-tests identified differences between fusion and detethering for hospital-related and surgical factors; multivariate analysis investigated procedure as a risk factor for complications/mortality.6457 TCS discharges: 5844 detetherings, 613 fusions. Fusion TCS had higher baseline Deyo Index (0.16 vs. 0.06), procedure-related complications (21.3% vs. 7.63%), and mortality (0.33% vs. 0.09%) than detethering, all p0.001. Detethering for TCS was a significant factor for reducing mortality (OR 0.195, p0.001), cardiac (OR 0.27, p0.001), respiratory (OR 0.26, p0.001), digestive system (OR 0.32, p0.001), puncture nerve/vessel (OR 0.56, p=0.009), wound (OR 0.25, p0.001), infection (OR 0.29, p0.001), posthemorrhagic anemia (OR 0.04, p=0.002), ARDS (OR 0.13, p0.001), and venous thrombotic (OR 0.53, p=0.043) complications. Detethering increased nervous system (OR 1.34, p=0.049) and urinary (OR 2.60, p0.001) complications. Adolescent TCS had higher Deyo score (0.08 vs. 0.03, p0.001), LOS (5.77 vs. 4.13days, p0.001), and charges ($54,592.28 vs. $33,043.83, p0.001), but similar mortality. Adolescent TCS discharges had increased prevalence of all procedure-related complications, and higher overall complication rate (11.10% vs. 5.08%, p0.001) than pediatric.With fusion identified as a significant risk factor for mortality and multiple procedure-related complications in TCS surgical patients, this study could aid surgeons in counseling TCS patients to optimize outcomes.

The clinical case is presented on a patient with an extensive sacral dysraphism, a history of myelomeningocele surgical repair in her childhood, as well as tethered cord syndrome. The patient was also diagnosed with pseudomeningocele and a cerebrospinal fluid cutaneous fistula. A surgical approach was used, with encouraging results being obtained in the clinical outcome of the patient. A review of the literature was performed to support the surgical decision in this case.

Objective To evaluate urinary system symptoms (USSs) and urodynamic parameters (UPs) before and after untethering in children with primary tethered cord syndrome (pTCS). Methods 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. Results 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. Conclusion 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.

Lipomyelomeningocele (LMM) as a cause of tethered cord syndrome (TCS) commonly presents in childhood in the lumbosacral spine. Patients frequently present with cutaneous manifestations, progressive neurological deterioration, bladder dysfunction, and intractable pain. Early surgical intervention with untethering is recommended for symptomatic patients. We report an unusual case of a woman who presented with a subcutaneous lump, pain, and neurological decline found to have a cervicothoracic LMM. The patient underwent laminectomy and subtotal resection of the mass; seventeen years later she was confined to a wheelchair with severe neurological decline ultimately requiring three additional attempts at surgical excision and repair. This case emphasizes the need for early recognition of and intervention in adult patients with LMM.

Background Spinal dermal sinuses consist of an epithelium-lined tract extending from the skin towards the spinal cord, often resulting in infections or tethered cord syndrome. Recently, a variant called dermal sinus-like stalk was described as an analogous tract but not containing an epithelium-lined lumen. Aims We aimed to describe the findings in our patients, subdivide our specimens into both conditions, compare the characteristics of both groups and search for possible embryologic mechanisms of dermal sinus-like stalks. Methods We performed a retrospective analysis of all patients operated in our hospital for both conditions between 1996 and 2012. Results 14 patients were operated upon for spinal dermal sinuses ( n = 5) and spinal dermal sinus like-stalks ( n = 9). Patients were mainly referred from other hospitals due to skin abnormalities and were evaluated at mean age of 7 weeks and operated upon at mean age of 1 year and 2 months. Primary reason for referral was skin abnormalities in both groups, though there were two cases of meningitis in dermal sinus patients and 2 of recurrent urinary tract infections in dermal sinus-like stalk patients. Consistent with previous findings, dermal sinus-like stalk patients do not have a history of meningitis, lack dermoid or epidermoid tumours along their tract, and are histologically of pure mesodermal origin. Dermal sinus-like stalks might result from interposition of mesenchyme during primary or secondary neurulation. Conclusions We consider dermal sinus-like stalks as a rare but currently under diagnosed condition with different clinical, pathological and probably also embryologic characteristics compared to spinal dermal sinuses.

Resumen Introduccion El sindrome de medula anclada es una malformacion de la region caudal de la columna vertebral de inicio inhabitual en la vida adulta. Provoca ocupacion de espacio que compromete raices y medula caudal, resultando en un trastorno variable de la marcha de progresion lenta y asimetrica. Casos clinicos Presentamos 4 pacientes adultos diagnosticados en el area de Albacete en 2009 con caracteristicas clinicas tipicas, neuroimagen demostrativa, estigmas cutaneos especificos y electrodiagnostico confirmatorio de sindrome de medula anclada, asi como evolucion clinica y tratamiento requerido en todos los casos. En los 4 pacientes realizamos estudio de la via motora piramidal mediante tecnica de estimulacion magnetica transcraneal, aportando en todos ellos una buena tipificacion del proceso, incluso en alguno en momentos incipientes de su desarrollo. Conclusiones Presentamos 4 individuos con sindrome de medula anclada de inicio sintomatico en la vida adulta. En todos ellos los estigmas de la piel orientaron en el diagnostico. La evaluacion de la via motora mediante electromiografia, y especialmente estimulacion magnetica, ayudaron a la caracterizacion, asi como a la diferenciacion de otras enfermedades que pudieran provocar confusion, en especial enfermedad de motoneurona. La electrofisiologia es un instrumento eficiente que puede facilitar el manejo del sindrome de medula anclada.

BACKGROUND CONTEXT A few studies in surgical treatment of congenital scoliosis have been associated with asymptomatic tethered cord. PURPOSE To evaluate the safety and effectiveness of correction of congenital scoliosis associated with asymptomatic tethered cord without untethering it. STUDY DESIGN/SETTING A case series. PATIENT SAMPLE Twenty-six patients. OUTCOME MEASURES Cobb angle of major coronal and kyphotic curve. METHODS Twenty-six patients with asymptomatic tethered cord who underwent congenital scoliosis correction were presented. There were 17 male and 9 female with an average 15.2years (range 13–22). RESULTS The preoperative Cobb angle of major coronal curve averaged 55° (range 38°–74°), and it measured 21° (range 13°–35°) at the last follow-up, for a 62% correction. Maximal kyphosis averaged 43° (range 32°–54°) preoperatively, and improved to 23° (range 16°–38°) at ultimate follow-up, for a 46% correction. The average follow-up was 6.5years (4–8 years). None of the patients experienced deterioration in their neurologic status. CONCLUSIONS The study results suggested that prophylactic neurosurgery may be unnecessary in patients with asymptomatic tethered cord who underwent congenital scoliosis correction. One-stage posterior correction of scoliosis in this patient population does not involve significant complications and seems to be an alternative and safe treatment option.

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.

Myelodysplasia with attendant spinal dysraphic disorders can be and often is progressive. In addition to the neurologic deficit present since the prenatal period, hydrocephalus, Chiari malformations with hydromyelia, tethered cord syndrome, or combinations of one or all lead to changing functional ability and progressive deformities. With recent advances in neurologic monitoring and treatment, many of these deformities can be prevented from appearing or becoming destabilizing if the subtle signs are picked up and early intervention is provided. Advances in urologic surgery have also provided better control of continence in addition to reduced infections and renal destruction. This article reviews the monitoring and treatment considerations that have led to such a significant improvement in outcomes in patients who have myelodysplasia.

Tethered cord syndrome is a clinical phenomenon resulting from anatomic restriction of the normal movement of the spinal cord or vascular compromise leading to hypoxia of its distal structures. Tethering can be acquired (secondary) or congenital (primary). This article presents the relevant embryology, primary and secondary causes of tethering, clinical presentations, the treatment of specific entities, indications and options for Tethered cord syndrome is a clinical phenomenon resulting from anatomic restriction of the normal movement of the spinal cord or vascular compromise leading to hypoxia of its distal structures. Causes of tethering can be acquired (secondary) or congenital (primary). This article presents the relevant embryology, primary and secondary causes of tethering, clinical presentations, the treatment of specific entities, indications and options for surgical treatment, and surgical complications.

Cervical and thoracic myelomeningocoeles differ from common lumbosacral myelomeningocoeles in many respects. We review the surgical technique and outcome achieved for a series of six infants who underwent surgery for cervical or thoracic myelomeningocoele. Five patients, who had intradural exploration and microsurgical untethering of the spinal cord, were neurologically stable on follow-up. The other patient, who had a simple subcutaneous resection of the sac without release of the intradural tethering bands, was re-operated on 16 months later, with progressive neurological symptoms due to cord tethering. Following re-exploration and microsurgical untethering of the spinal cord, the neurological deficits significantly improved. We suggest that the surgical technique in these lesions should include careful intradural exploration and microsurgical release of the spinal cord by meticulous resection of all tethering bands. This enables postoperative neurological improvement and possible prevention of future neurological deficits due to cord tethering.

The term “tethered cord” refers to any condition in which the spinal cord is rendered partly or completely immobile within the spinal canal. As a clinicopathologic entity, tethered cord generally involves an abnormally low and relatively fixed conus medullaris, often arising in a distal spinal cord and/or vertebral malformation. This article reviews the essentials of etiology, pathogenesis, and neuropathology of tethered cord.

The authors review clinical and surgical nuances for the management of tethered cord syndrome in the setting of the less commonly encountered entities of split cord malformation and lipomyelomeningocele.

Spinal dysraphism is used to describe a group of developmental abnormalities in which there is defective closure of the neural tube. The authors review the clinical manifestations and surgical treatment of occult and open spinal dysraphism.

Journal of Neuroradiology - In Press.Proof corrected by the author Available online since jeudi 21 fevrier 2013

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 3 years 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 3 years 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.

Tethered cord syndrome is a complication of spinal dysraphism. The tethering of the cord does not permit the normal cranial migration of the conus within the vertebral canal. The result is a neural dysfunction due to a traction neuropathy. Although this condition commonly presents in childhood, less severe degrees of tethering may remain asymptomatic until adult life. The authors report a new case of tethered cord syndrome in a 25-years-old young girl, cause of bladder dysfunction. Clear improvement of urinary repercussions is obtained after surgery.

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-up1 year were excluded. Analysis was limited to those toilet trained or aged ≥6 years 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.

Latex allergy has become a health problem of growing interest due to the severe clinical manifestations it may produce. Previous reports indicate a prevalence of latex allergy in patients with spina bifida (SB) ranging between 10% and 73%. From the initial surgical repair of the myelomeningocele, children with SB are submitted to multiple surgeries due to the neurologic, orthopedic and urologic problems they may present. Consequently, these children are exposed to several latex-containing products during their multiple interventions. Several reports indicate that latex allergy in SB children is related to age, and especially to the number of operations that they are given, and perhaps to an unknown genetic predisposition. Accordingly, latex allergy manifestations can be prevented. In this connection, we have elaborated at our hospital protocols for the management of the three clinical situations in which these patients can present. These measures include: (1) To avoid using latex-containing products since the birth of children with SB. (2) In children already operated of myelomeningocele but without evidence of latex allergy, to prevent the use of latex materials during their successive admissions to hospital and during further surgeries. And (3) In children known to have developed latex allergy, to dispose of especial nursing measures and of a latex-free operating room. The preventive measures that have been started at our Hospital are easy to carry out, do not suppose a high economic cost, and are not uncomfortable for the hospital's staff.

B large congenital melanocytic nevus (LCMN), defined herein as a nevus present at birth that is predicted to be at least 20 cm in greatest diameter in adulthood, occurs infrequently. Approximately one in 20,000 infants, amounting to 200 newborns per year in the United States, is born with a LCMN. In addition, one in 500,000 newborns, translating to 10 infants, is born each year with very large LCMN of the garment or bathing-trunks type. 1 The implications for the affected infant are significant. Beside cosmetic disfigurement, a LCMN increases an individual's risk for developing malignant melanoma (MM), and may be associated with neurocutaneous melanocytosis (NCM). 2"5 There are many definitions for what constitutes a LCMN (Table 1). 6-13 As mentioned above, we elect to define LCMN based on the predicted maximum diameter of the nevus in adulthood: at least 20 cm. Congenital melanocytic nevi (CMN) generally enlarge in proportion to the growth of the skin coveting the involved area of the body. 14 Figure 1 depicts the graphs we use to predict the adult diameter of an infant's or child's CMN. 15 Clinically, LCMN can be light brown, dark brown, black, blue, or exhibit a combination of colors. The surface may be macular, papular, mamillated, hyperkeratotic, rugose, and/or nodular. The nevus may be hypertrichotic. 16 With time, LCMN may get darker or lighter, and rarely, completely disappear.5,! 6,17 LCMN can sometimes be associated with anomalies such as NCM, neurofibromatosis type I, occult spinal dysraphism, and/or the tethered cord syndromeJ 8 NCM may affect over 10% of patients with LCMN. 15 It is a s2cndrome with a poor prognosis for survival. According to one definition, NCM is characterized by the presence of an LCMN, or multiple (three or more) smallto medium-size CMN, accompanied by a benign and/or malignant melanocytic proliferation in the leptomeningesJ 9 Of the patients with central ner

Myelomeningocele is a common birth defect that is associated with significant lifelong morbidity. Despite improvements in technology and overall patient care, little progress has been made in the postnatal surgical management of the child with spina bifida. Postnatal surgery is aimed at covering the exposed spinal cord and preventing infection. Numerous interventions for ventricular shunts, tethered cord, scoliosis, incontinence, urologic complications, and extremity anomalies are frequently required. Although myelomeningocele is a nonlethal fetal anomaly, the limitations with current postnatal treatment strategies has led to extensive investigation of prenatal treatment options. This article outlines the rationale for fetal intervention and discusses the preliminary experience with human fetal myelomeningocele surgery.

yringomyelia is a challenging neurosurgical condition to manage. The classic causes of syringomyelia include S altered cerebrospinal fluid (CSF) flow in patients with Chiari malformations, trauma, tumors, tethered cord syndrome, or infections. The management of syringomyelia involves treating the underlying condition, observation, arachnolysis, and/or syrinx shunting to the subarachnoid, pleural, or peritoneal space. Even with treatment, recurrence rates are high. As such, it has been long debated how to best manage this condition.

An 8-year-old female was diagnosed with Miller-Dieker syndrome with typical facial presentation. Brain magnetic resonance imaging disclosed lissencephaly, and chromosome study revealed 17p13.3 deletion. She developed infantile spasms at an early age, and her seizures were poorly controlled by multiple antiepileptics. Recurrent urinary tract infections were diagnosed during routine out-patient department follow-up. Urodynamic study disclosed a neurogenic bladder. Spinal magnetic resonance imaging revealed a tethered cord resulting from tight filum terminale, and untethering surgery was performed. Four months after the surgery, repeated urine cultures indicated that she was free from the urinary tract infection. Urodynamic study after untethering surgery demonstrated improved compliance of the urinary bladder.

Background Differentiation between nerve roots and the tethered filum terminale in magnetic resonance images remains challenging. It is, however, crucial to select patients for surgical intervention. Method In 1 patient with progressive and 2 patients with stable clinical signs of tethered cord syndrome (TCS), images reconstructed from a 3D constructive interference in steady-state (CISS) sequence were compared with those obtained from a regular T2-weighted fast spin echo sequence. Results Nerve roots of the cauda equina were suppressed, whereas visualization of the filum terminale was much improved in high-resolution CISS sequences. In addition, the level of adhesion of the filum could be localized in relation to elements of the spinal cord. The imaging results were confirmed during surgery and the patient improved considerably after the intervention. The advantages of CISS were also apparent in 2 other patients who are still being observed. Conclusion Constructive interference in steady-state imaging should be considered in the presurgical routine for patients with TCS.

Objective Clinical and neurophysiological evaluation in patients with lumbo-sacral lesions, and intraoperative (IOM) and postsurgical assessment. Material and methods We studied 44 patients clinically and electrophysiologically, complaining spine lumbosacral lesions (tumors, lumbar canal stenosis, tethered cord). IOM was performed using SEP, MEP, continuous and triggered EMG, using Neuronic 5 (SA) equipment, and IMO specially designed software for intraoperative monitoring (EMG WORKPLACE AND MNIO 1.0). Results Electrophysiological signals changes after surgery were correlated with clinical scale scores showing dysfunction or functional improvement. IOM showed in 9.4% of patients, signs of alarm indicating a neural damage. In 3.7% of cases after surgical decompression, P40 cortical response reappeared (P40), with increments of amplitude and latency decrement. In 87% of cases, no changes were demonstrated (signs of good prognosis). Conclusions Multimodal IOM allows assessing somatic and motor pathways, showing signs of alarms during the neurosurgical procedures. Significance The development of a specially designed IOM software is an effective tool for an early detection of functional abnormalities during spine surgery.

Tethered cord syndrome, manifested by motor and sensory dysfunction and incontinence, is caused by excessive tension in the lumbosacral cord. The underlying mechanism is related to impairment of oxidative metabolism in this region. This theory is derived from redox changes of cytochrome a, a3, and supported by spinal cord blood flow, evoked potential, and histological studies. The well-known finding of elongated spinal cord attached to the tight filum or a tumor is not always demonstrated in imaging studies, and understanding of pathophysiology allows physicians to correctly diagnose and treat patients with tethered cord syndrome.

Congenital spinal malformations discussed include myelomeningocele, tethered cord, diastematomyelia, diplomyelia, neuroenteric cysts, anterior meningocele, and Chiari malformations. The authors discuss the clinical presentation, radiologic evaluation, and treatment of these congenital anomalies.

The records of 181 patients with myelodysplasia were reviewed. The prevalence of associated genitourinary anomalies as well as cardiac, facial, anal, and tethered cord were determined. The prevalence of congenital malformations associated with myelodysplasia were less in this series than in previous reports.