Myelomeningocele Patients Research Articles

Bibliometric analysis of myelomeningocele management: National disease burden versus publication volume

Myelomeningocele (MMC) disproportionately affects low-resource areas and regions without mandatory folic acid fortification. No specific literature exists on the distribution of research output regarding neurosurgical management of myelomeningocele worldwide in relation to regional disease burden. We aimed to examine the country of origin and patient population of published papers on myelomeningocele and topics related to neurosurgical management of MMC, to determine whether these were proportionate to disease burden. A systematic literature search was conducted on neurosurgical aspects of myelomeningocele care. The geographic distribution of neurosurgical MMC research output was examined against the national burden of disease. Bibliometric analysis quantified author and patient country affiliations stratified by World Bank income group classification and folic acid fortification status, juxtaposed with disease burden. From 9,692 titles, 1,843 were included, representing 107,446 patients and 2,650 authorship instances. High-income countries (HICs) comprised 3% of 2019's global NTD births, 74% of authorships, and 83% of patients represented. Upper-middle-income countries (UMICs) represented 9% of NTD births, 16% of authorships, and 9% of published patients. Lower-middle-income countries (LMICs) represented 55% of NTD births but only 8.6% of authorships and 7% of patients. Low-income countries (LICs) shouldered 32% of NTD births and contributed 1.3% of authorships and 1.6% of patients. Countries with mandatory folic acid fortification represented 75% of patients and 54% of authorships. Postnatal repair, hydrocephalus, and postoperative complications were the most frequently studied topics. The global literature concerning neurosurgical management of myelomeningocele originates predominantly from HICs. Published experiences of myelomeningocele patients from LICs/LMICs are scarce, even though they comprise the majority of the affected population. Neurosurgeons and other health professionals must address this mismatch between disease burden and publication volume in order to inform practice, policy, and advocacy for myelomeningocele care worldwide.

Effect of early tethered cord release on urodynamic findings and lower urinary tract function in myelomeningocele patients.

Detecting neurological deterioration and diagnosing tethered cord syndrome (TCS) in patients with myelomeningocele (MMC) can be challenging due to the presence of symptoms at birth and the lack of objective indicators. This retrospective analysis focused on urological manifestations and evaluated whether tethered cord release (TCR) for TCS at an early stage could improve or stabilize video urodynamic study (VUDS) findings and lower urinary tract function. This study analyzed 55 of 64 children who underwent MMC repair at Miyagi Children's Hospital, Sendai, Japan, between 2003 and 2016. The follow-up duration for these children exceeded 6 years. Clinical records were reviewed, and surgical indicators and outcomes of TCR and preoperative and postoperative results of VUDS were evaluated. The duration of follow-up was mean ± SD (range) 12.6 ± 3.5 (6.0-19.0) years. TCR was performed on 27 (49.1%) of 55 patients with MMC, totaling 33 procedures. Five patients underwent 2 TCR procedures, whereas 1 received 3 TCR procedures. The mean ± SD (range) age was 7.5 ± 2.9 (2.2-12.7) years at the first TCR procedure (n = 27) and the mean (range) was 10.9 (10.1-11.7) years at the second TCR (n = 5); in addition, 1 patient underwent a third TCR procedure at age 15.9 years. There were no identified risk factors associated with the TCS. The first TCR procedure was performed due to worsened lower-extremity (LE) motor symptoms in 1 patient, worsened LE sensory symptoms in 3 patients, and worsened VUDS findings in 26 patients. After the procedure, LE motor symptoms improved in 1 patient (100%), LE sensory symptoms improved in 2 patients (66.7%), and VUDS findings improved in 18 patients (66.7%). Preoperative VUDS revealed urological deterioration characterized by a high-pressure bladder, reduced bladder capacity, increased detrusor overactivity (DO), and vesicoureteral reflux. Postoperative VUDS showed improvements in bladder function, including decreased bladder pressure and DO, increased bladder capacity, and compliance. None of the patients underwent augmentation cystoplasty or had renal dysfunction. Routine VUDS can detect urological deterioration, which can be a significant indicator for early diagnosis of TCS. Performing TCR at an early stage is beneficial, not only to protect renal function but also to improve VUDS findings and lower urinary tract function in patients with MMC. It is important to explore a standardized approach for the diagnosis and treatment of TCS.

Particularities of spasticity in myelomeningocele patients.

Myelomeningocele (MMC), a congenital neural tube defect, is the most common developmental anomaly of the central nervous system (CNS). Spasticity is among the main disabling factors in these patients, affecting up to 1/5 of children. The purpose of this article is to review the current knowledge regarding spasticity in MMC patients, mainly focusing on its manifestations and management. A literature search was conducted using the PubMed database for the terms "myelomeningocele" and "spasticity." Data were collected by 5 independent investigators and then synthesized in a scoping format. Causes of spasticity in these patients include hydrocephalus, tethered cord syndrome (TCS), syringomyelia, CNS infection, and associated congenital brain and spine anomalies. Clinical manifestations include limb spasticity and neurogenic bladder. Spasticity primarily affects muscles below the neurological level and contributes to gait impairment. Besides walking ability, spasticity also severely affects functional mobility in general, self-care, and quality of life. The majority of MMC patients experience neurogenic bladder. Treatment of spasticity can be symptomatic or target its cause. Medical and surgical options are available for both limb and bladder spasticity. Regular physiotherapy and orthotics are used to prevent contractures and the early introduction of orthoses is the cornerstone of a gait training program. Neurosurgical treatment options for spasticity include nonablative and ablative procedures such as selective dorsal rhizototmy. The urodynamic pattern guides treatment strategies, with intermittent catheterization being the best treatment option for patients with underactive detrusor and overactive sphincter muscles. Given the particularities of spasticity in MMC patients, a multidisciplinary team approach and early rehabilitation programs are keys for their optimal management.

Long-term mortality outcomes and mortality predictors in patients with myelomeningocele

ObjectiveDespite advances, myelomeningocele remains a major cause of mortality and disability. This study aims to analyze factors influencing mortality and suggest ways to reduce it. MethodsWe reviewed 173 patients who underwent surgery for myelomeningocele from January 2010 to December 2021. Variables affecting mortality were categorized into patient-related and indirectly related factors. Data were collected through patient file reviews and phone interviews with parents. ResultsMortality is influenced by multiple factors: female gender (3.2-fold increase), paraplegia (3.1-fold increase), absence of tethered cord release surgery (9.4-fold increase), scoliosis (4.2-fold increase), and renal failure (5.28-fold increase). Defect size and father's education level also significantly impact mortality. The overall mortality rate was 20.8 %, with hydrocephalus being the leading cause. ConclusionMortality and disability in myelomeningocele patients remain high, with over 50 % of deaths being preventable. Effective management could significantly improve mortality rates. Long-term studies are crucial for advancing research in this field.

Clinical predictors of the need for ventriculoperitoneal shunt placement in myelomeningocele patients

Clinical predictors of the need for ventriculoperitoneal shunt placement in myelomeningocele patients

263 Radiographic Structural Changes in Brain Anatomy of Pediatric Fetal Versus Postnatal Myelomeningocele Repair Patients

INTRODUCTION: Postnatally repaired myelomeningocele (MMC) patients have twice the risk of needing shunt treatment for hydrocephalus compared to fetally repaired patients, however it is unknown why exactly this occurs as well as what structural brain changes may be predictive of hydrocephalus. We radiographically characterized anatomic changes in fetally vs postnatally treated infants to understand early developmental anatomy. METHODS: MRIs were characterized by two board-certified neuroradiologists for the following variables: 1) The ratio of ventricular-to-subarachnoid space (VSAR) using bifrontal (BF), left frontal lobe thickness (LV), and extra-axial (EA) spaces as parameters. 2) The ratio of 2 planes within the lateral ventricle (LVR). These ratios, elongation of the choroid plexus glomus and body, and Chiari were measured. RESULTS: 54 MMC repair patients met criteria, yielding 109 MRIs. In the one-to-three month time frame, VSAR at BF and LV parameters in addition to LVR were all significantly different in the fetally repaired group (n = 14) compared to the postnatally repaired group (n = 13, p<0.05). VSAR was not significantly different in early life. Chiari was significantly different in the early life and one-to-three month time between fetal and postnatal repair cohorts (p < 0.05). CONCLUSIONS: Fetally and postnatally repaired MMC brain MRIs were examined by two neuroradiologists to characterize differences in early brain development. This study identified significant differences in the ventricular to subarachnoid space ratio during the one-to-three month time point between fetally and postnatally repaired patients. We suspect that subarachnoid anatomy may be predictive of the need for surgical treatment of hydrocephalus.

Survey of medications for myelomeningocele patients over their lifetime in Japan

BackgroundThis study aimed to investigate medication prescriptions for patients with myelomeningocele (MMC) across different age groups, particularly in adulthood and after middle age. MethodsThe Japan Medical Data Center (JMDC) database, based on medical claims data, was utilized for this analysis. Patients were divided into 10-year age groups, and prescriptions for analgesics, anticonvulsants, psychotropic drugs, lifestyle disease-related drugs, drugs for urinary incontinence, and laxatives were examined. To compare the differences in the utilization of medications unrelated to lifestyle-related diseases across different age groups, the data was categorized into three age groups: 19 or under, 20–39, and 40 or older. ResultsAmong the 556 MMC patients, the percentage of those regularly prescribed analgesics increased from 2.8% in patients ≤ 19 to 31.7% in patients 40 or older (p < 0.01). Psychotropic medication use also increased with age, rising significantly from 6.3% in patients ≤ 19 to 34.6% in patients 40 or older (p < 0.01). Patients with MMC showed an increasing trend in prescriptions for lifestyle-related disease medications compared to the normal control group. Notably, the percentage of patients in their 30 s taking hypertension medication was 4.9%, significantly higher than the 0.86% in the control group (p = 0.029). In their 40 s, 22.9% of MMC patients were prescribed hyperlipidemia medication, significantly higher than the 3.9% in the control group (p < 0.01). ConclusionComprehensive multidisciplinary support and follow-up are crucial to enhance the quality of life for MMC patients, with particular attention to pain management, psychological care, and treatment of lifestyle-related diseases.

Global neurosurgery: Reflections on myelomeningocele in the Zanzibar archipelago (Tanzania)

ObjectiveOur main goal was to describe the general characteristics and demographic data of myelomeningocele (MMC) patients at Mnazi Mmoja Surgical NED Institute (MMSNI) in Zanzibar and to assess the clinical characteristics and medium-term result-impact of the implemented health care measures. MethodsThis is a retrospective study on 41 MMC patients treated at the MMSNI in Zanzibar (Tanzania) from September 2016 to September 2018. Patient demographics, prenatal care, clinical and radiographic characteristics, surgical management and nursing care, and clinical outcomes were abstracted. ResultsThe mean age of the patients was 6.1 ± 4.6 days, and 53.7% were males. A total of 51.2% came from Zanzibar, 39% to Pemba, and 9.8% from mainland Tanzania. Maternal ultrasound checkups revealed hydrocephalus in 18.7% of the cases. 85.4% of the newborns were operated on. Surgical wound infection was the most frequent complication (28.6%). A significantly higher risk of complications was observed in children from Pemba Island (p = 0.046) and those born by vaginal delivery (p = 0.694), particularly infections. During follow-up, 48.57% of the patients presented with infantile hydrocephalus and in the majority of them, a ventriculoperitoneal shunt was inserted. ConclusionsProper prenatal care with early diagnosis, together with the neurosurgical and nursing standard of care in a specialized institution, are all essential to increase the chances of successful treatment of newborns harboring MMC and is one of the main goals pursued in the MMSNI, as the only referral public health center with locally trained health personnel in Zanzibar archipelago.

Understanding factors influencing primary treatment with intradetrusor onabotulinumtoxinA versus augmentation cystoplasty in patients with spina bifida.

Surgical interventions in the urologic management of children with neurogenic bladder secondary to spina bifida aim to preserve upper tract function, prevent urinary tract infections, and optimize quality of life. However, since the introduction of intravesical onabotulinumtoxinA (Botox) in the management of these patients, the indications for choosing Botox over augmentation cystoplasty (AC) remain undefined. The objective of this study was to determine which factors lead patients to undergo Botox versus AC as a primary surgical treatment after failing medical management. We retrospectively reviewed the records of pediatric patients with myelomeningocele undergoing either primary Botox or primary AC at our institution between 2013 and 2018. We recorded demographic and clinical information. We identified 10 important clinical decision-making factors: bladder trabeculation, vesicoureteral reflux, or hydronephrosis on imaging; end-filling pressure (EFP) ≥40 cm H2O, detrusor overactivity, detrusor-sphincter dyssynergia, or reduced capacity on urodynamic studies; physician-perceived bladder hostility; and patient/family desire for continence and independence. The presence of these factors was compared between patients undergoing either primary Botox or primary AC. We identified 14 and 50 myelomeningocele patients who underwent primary AC and primary Botox, respectively. We found no significant differences in age, sex, race, or history of reconstructive surgery (antegrade continence enema or catheterizable channel). For the 10 decision-making factors, desire for independence/continence (p = <0.001) and reduced capacity (p = 0.002) were significantly associated with AC, while trabeculation (p = 0.006), EFP ≥40 cm H2O (p = 0.029), rising slope (p = 0.019), and physician-perceived hostility (p = 0.012) were significantly more common with Botox. At our institution, quality of life measures prompted AC over objective urodynamic or imaging findings before attempting Botox. These findings support a shared decision-making approach when considering surgical intervention for neurogenic bladder secondary to myelomeningocele.

Operative treatment of paralytic hip dislocation in myelomeningocele by Dega transiliac osteotomy combined with iliopsoas transfer: technique and historical case series.

The technique of one stage procedure - open reduction, Dega transiliac with or without femoral subtrochanteric osteotomy combined with iliopsoas transfer according to Mustard - has been described for the treatment of paralytic dislocation of the hip in myelomeningocele patients. Historical series of 16 children (26 hips) operated on between 1987 and 2003 were analyzed retrospectively. There were nine boys and seven girls with upper and lower lumbar level lesions (15 with Sharrard groups 3 and 4 and one with Sharrard 2) and 20 dislocated and six subluxated hips. The mean age at operation was 5.1 years (3-12.3). Ten children were operated bilaterally. Follow-up ranged from 1 to 17 years (mean 10). Concentric reduction was achieved in 23 hips, subluxation in 1 and redislocation in 2. In one hip, acute avascular necrosis of the femoral head was visible early after operation. Immediately after the operation, most of the patients improved ambulation. After operation, 15 patients out of 16 became community ambulators. At the final follow-up, none of the patients worsened their ambulation due to operation. Open reduction and Dega transiliac osteotomy with or without subtrochanteric derotation/varus shortening osteotomy combined with iliopsoas transfer seem to be safe and valuable procedure for operative treatment of dislocated hip in myelomeningocele patients. Levels of evidence: level IV - case series.

Frequency Of Hydrocephalus In Preoperative Patients Of Myelomeningocele

Objective: To find out the frequency of hydrocephalus in preoperative patients of lumbosacral myelomeningocele.Place of study: Department of Pediatric Neurosurgery, The Children's Hospital, and the Institute of child health, Multan.Study design: Descriptive cross-sectional studyDuration of study: March 2020 to April 2021.Methodology: A total of 139 patients with lumbosacral myelomeningocele were included in the study. Myelomeningocele patients were diagnosed using MRI images which revealed herniation of a meningeal lined sac having a spinal cord and nerve roots through the defect and the herniated sac was filled with cerebrospinal fluid. (hyperintense on T2 weighed images) The pressure of neural tissue in myelomeningocele patients was assessed by ultrasonography and MRI of the Spine. Associated hydrocephalus was diagnosed with an MRI brain plain and CT scan. Data were analyzed using SPSS.21. Quantitative variables including age and gender were analyzed by mean and standard deviation. The presence or absence of hydrocephalus was analyzed by frequency and percentages.Results: The mean age of the patients was 6.392 + 3.517 months, the minimum age was 0.5 months and the maximum age limit was 12 months. Out of 139 patients, 76 (54.7%) were males while 63 (45.3%) were females. Out of 139 patients, 121 (87.5%) had hydrocephalus, while 18 (12.5%) did not have hydrocephalus.Conclusion: There is a high frequency of hydrocephalus in patients with myelomeningocele.

Recurrent heel ulcers with calcaneal osteomyelitis in myelomeningocele: Treatment by partial calcanectomy and posterior transfer of tibialis anterior tendon

OverviewCalcaneus deformity of the foot is common in patients with myelodysplasia, mainly due to muscle imbalance. This deformity, especially in ambulatory patients, can result in gait problems and development of pressure sores, which can be complicated by calcaneal osteomyelitis. Material and methodsThis retrospective cross-sectional study included 12 patients (18 feet), with calcaneus deformity due to myelomeningocele, and presented with penetrating heel ulcers complicated by calcaneal osteomyelitis. The mean age of the included cases was 11 years. The ulcers were unilateral in six patients and bilateral in six. Sensation was absent on the plantar aspect of the foot in all cases. The treatment was done in two stages; The first stage was eradication of infection and obtaining good soft tissue coverage, and the second stage was obtaining motor balance to achieve a more plantigrade and braceable foot. ResultsThe average follow-up period was 19.2 months. In the final follow-up, twelve feet were graded as good, five as fair and one as poor according to Legaspi grading system. ConclusionThe combination of partial calcanectomy and subsequent transfer of tibialis anterior tendon, to improve the foot position and gait, can efficiently prevent ulcer recurrence in myelomeningocele patients with calcaneal deformity. Level of clinical evidence: 4Retrospective case series study.

In utero myelomeningocele repair and high-risk bladder pattern. a prospective study.

ABSTRACTObjectivesHigh-risk bladder pattern can be defined by Urodynamic Evaluation (UE) as overactive bladder with detrusor leak point pressure higher than 40 cmH2O and/or higher filling pressures also above 40 cmH2O. We wanted to evaluate response to treatment in myelomeningocele patients operated in utero in this subgroup.Patients and MethodsFrom our prospective cohort of in utero MMC we have identified patients in the high-risk group. Treatment consisted of anticholinergics (Oxybutynin 0.2 mg/Kg) 2 or 3 times daily in association with CIC. At every UE, patients were reclassified in high-risk or low-risk patterns. Patients not responding were proposed bladder reconstruction or diversion according to age.ResultsBetween 2011 to 2020, we have been following 121 patients and 60 (49.6%) of them were initially categorized as high-risk. The initial UE was performed at a mean age of 7.9 months and detrusor overactivity was found in 83.3% (mean maximum pressure of 76.5cmH20). When evaluating patients with 2 or more UE, we identified 44 patients (follow-up: 36.8months). It was observed in the group of patients who underwent 2 to 5 UE, that response to treatment was validated by the finding of 40% of low-risk bladder patterns in the second UE and between 62% to 64% in the third to the fifth UE. The incidence of surgery was 13.3%.ConclusionsEarly urological treatment of high-risk bladder pattern was effective in approximately 60%. We reinforce the need to correctly treat every patient with myelomeningocele, in accordance with UE, whether undergoing in utero or postnatal treatment.

Current status and challenges of neurosurgical procedures for patients with myelomeningocele in real-world Japan.

Little is known about the real-world status of neurosurgical treatment of myelomeningocele patients. To investigate the real-world status of neurosurgical treatment of myelomeningocele patients, medical claims data provided by the Japan Medical Data Center (JMDC) were analyzed. The health claims data of 556 patients with myelomeningoceles from January 2005 to March 2020 were examined. The number of neurosurgical procedures, including myelomeningocele repair, tethered cord release, cerebrospinal fluid (CSF) shunt, CSF drainage, and endoscopic third ventriculostomy (ETV), was determined. A total of 313 neurosurgical procedures were performed for 135 patients in 74 institutions during the study period. The shunt survival rate was most affected by shunts that were revised when the patient was less than 1year old, which had a significantly lower survival rate than all of the initial shunts performed when the patient was less than on1 year old; the 1-year shunt survival rate was 35 vs 64% (P = 0.0102). The survival rate was significantly lower in patients younger than 1year who had CSF drainage before shunting compared to those younger than 1year who did not have CSF drainage before shunting; the 1-year shunt survival rate was 27 vs 59% (P = 0.0196), and 81% of patients remained free of tethered cord release 10years later. In this study, a revised shunt of less than 1year of age and CSF drainage before shunting were the factors that lowered the shunt survival rate in the real world for CSF shunts for hydrocephalus associated with myelomeningocele.

Evaluating the Efficacy of Rib-to-pelvis Growth-friendly Surgery for the Treatment of Non-ambulatory Early-Onset Scoliosis Myelomeningocele Patients.

Introduction:Early-onset scoliosis (EOS) is a well-known orthopaedic manifestation in patients with myelomeningocele. The rib-based growing system (RBGS) has been proposed as an alternative for these individuals because of the poor outcomes with traditional surgical techniques. We aimed to describe the effect of RBGS in patients with nonambulatory EOS myelomeningocele.Methods:We retrospectively reviewed the Pediatric Spine Study Group Multicenter Database for all patients with nonambulatory EOS myelomeningocele treated with RBGS from 2004 to 2019. Demographics, surgical data, radiographic findings, and postoperative complications were obtained. The quality-of-life parameters were assessed postoperatively using the Early-onset Scoliosis Questionnaire-24.Results:Thirty patients (18 women; 60%) were patients with nonambulatory EOS myelomeningocele treated with RBGS. The mean age at the initial surgery was 5.3 years. The thoracic (T1-T12) spine height showed a significant increase from initial surgery to the most recent follow-up (P < 0.001). Spine (T1-S1) height was also significantly increased (P < 0.001). The postoperative complication rate was 87%. The Early-onset Scoliosis Questionnaire-24 demonstrates significant improvements in the quality-of-life scores (P = 0.037).Conclusion:This study demonstrated that RBGS could improve the reported quality-of-life scores in patients with nonambulatory EOS myelomeningocele when assessed with an EOS-oriented tool. Moreover, we confirmed the ability of RBGS to hold or even correct spinal deformity.

A pilot study assessing average detrusor pressure garnered from area under a urodynamic curve: Evaluation of clinical outcomes

The management of neurogenic bladder mostly relies on urodynamic studies; however, several studies have shown low interobserver reproducibility. The aim of this study was to evaluate if a new objective cystometric parameter was superior to other cystometric measures. A new parameter (pressure adjusted area under curve ratio, PAUC) was formulated from the ratio of area under the cystometry curve to a DLPP-adjusted total area (AT). AT was figured from a rectangle designed on the cystometrogram curve with a fixed height of 200 cmH2O and a width defined by the filling phase (figure). Two different estimated pressure measures derived from this (upper mean static pressure, UMSP and theoretical end filling pressure TEFP) were then calculated. Medical records of myelomeningocele patients with ultrasonography and renal scintigraphy performed at the time of urodynamics (with an interval of >5 years) were reviewed. Hydronephrosis and new scars in scintigraphy were used as the outcome measures. The study group consisted of 115 subjects with a median age of 4 (0-23) years at the time of the first urodynamic study. The median follow-up was 6 (5-14) years. PAUC and its derivatives (UMSP and TEFP) had the best discriminative power in predicting high grade hydronephrosis (0.830, 95% CI:0.732-0.927, p<0.001), worsening in hydronephrosis (0.827, 95% CI:0.723-0.931, p<0.001), and new scar formation (0.704, 95% CI: 0.576-0.832, p=0.002). PAUC>0.1 significantly correlated with urinary tract dilatation (p<0.001) and new scar formation (p=0.002). In the multivariate analysis, our three parameters and having scars at admission were the only independent risk factors for new scars (p=0.001 and p=0.002, respectively) and worsening in hydronephrosis (p<0.001 and p=0.001, respectively). Our results show that our three parameters derived from area under the urodynamic curve are more reliable than other urodynamic measures. Their major theoretical advantage is to incorporate all the pressure during filling phase giving a more accurate picture of what the intravesical pressure milleu is. Using these measures, we demonstrated their superiority in predicting clinical outcomes. Major limitations of this study are the retrospectively collected data and lack of longitudinal follow-up starting from infancy in each patient. Our new parameters (PAUC, UMSP and TEFP) which incorporate the impact of entire filling phase pressure changes in the analysis, may be useful tools to identify those patients who are under the risk of kidney damage with neurogenic lower urinary tract dysfunction.

Postoperative seizures following endoscopic third ventriculostomy and choroid plexus cauterization: a case series.

In this study, the authors sought to investigate variables associated with postoperative seizures following endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) for treatment of pediatric hydrocephalus. A retrospective analysis of 37 patients who underwent ETV/CPC for treatment of hydrocephalus at an academic medical center from September 2016 to March 2021 was conducted. Demographics, etiology of hydrocephalus, operative details, electroencephalography (EEG) data, MRI findings, need for subsequent procedures, perioperative laboratory tests, medical history, and presence of clinical postoperative seizures were collected. Postoperative seizures were defined as clinical seizures within 24 hours of surgery. Eighteen patients received levetiracetam intraoperatively as well as over the next 7 days postoperatively for seizure prophylaxis. Of 37 included patients, 9 (24%) developed clinical seizures within 24 hours after surgery, 5 of whom subsequently had electroclinical seizures captured on video-EEG. The clinical seizures in 4 of those 5 patients (80%) may have been associated with the hemisphere of the brain through which the endoscope was introduced. The median corrected age of the cohort was 3.4 months. The median corrected age of patients who did not develop postoperative seizures was 2.3 months compared with 0.7 months for patients who did develop postoperative seizures (p > 0.99). Postoperative seizures occurred in 43% (3/7) of prenatally repaired myelomeningocele patients versus 29% (2/7) of postnatally repaired myelomeningocele patients. Of the 18 patients who received prophylactic levetiracetam, none (0%) developed postoperative seizures compared with 9 of the 19 patients (47%) who did not receive prophylactic levetiracetam (p = 0.014). Postoperative seizures were recorded in 24% of the pediatric patients who underwent ETV/CPC for hydrocephalus, which is higher than previously reported rates in the literature of 5%. Since 80% of the postoperative electrographic seizures may have been associated with the hemisphere through which the endoscope was introduced, the surgical entry site may contribute to postoperative seizure development. In patients who received prophylactic perioperative levetiracetam, the postoperative seizure incidence dropped to 0% compared with 47% in those who did not receive prophylactic perioperative levetiracetam. This finding indicates that the use of prophylactic perioperative levetiracetam may be efficacious in the prevention of clinical seizures in this patient population.

Increased medial pedicle angulation of the lumbar spine as a function of the posterior osseous defect in patients with myelomeningocele.

Bony anatomy in patients with immature spines has been previously characterized. Alterations in lumbosacral morphology in patients with myelodysplasia, demonstrating increasing medial pedicle angulation (MPA) from L1-S1, have been examined; however, MPA related to size of the defect in myelomeningocele patients has not been defined. We aimed to establish that magnitude of posterior vertebral arch defects determines the extent of MPA in the lumbar spine, with larger posterior defects associated with increased MPA. This retrospective case-control study compared lumbar morphometric measurements of patients with and without myelomeningocele. Eighteen patients with myelomeningocele underwent computed tomography with three-dimensional reconstruction; there was no concurrent pathology. Advanced imaging software was utilized to manipulate the axial, sagittal, and coronal axes at each lumbar level, and obtain accurate measurements of pedicle width, height, length, MPA, and posterior laminar defect. In lumbar vertebrae with a posterior arch defect (PAD), increased magnitude was associated with a concurrent increase in MPA between 16.2° (43.4%) and 28.1° (299.4%) depending on lumbar level. At levels without a PAD, increases in MPA were between 0.8° (4.3%) and 5.7° (60.6%) depending on lumbar level. Although the actual degree of medial angulation increases from cephalad to caudal levels, the percentage of deviation from normal is higher at the more cephalad levels compared with controls. In patients with myelomeningocele, larger posterior vertebral arch defects were associated with increased MPA in the lumbar spine. Lumbar levels without a PAD also demonstrated increases in MPA compared with normal values.

Kyphectomy with anterior column reconstruction using titanium mesh cage in meningomyelocele patients.

Study design: Prospective case series. Purpose: To describe a new technique for anterior column reconstruction after kyphectomy in myelomeningocele patients using titanium mesh cage and to evaluate outcomes and complications. Methods: Sixteen patients with severe dorsolumbar kyphosis 2ry to myelomeningocele were enrolled with a mean age of 10.1 years. Kyphectomy procedure and long spinopelvic fixation were done, titanium mesh cage was used to reconstruct the anterior column. Operative time and intraoperative blood loss were calculated. Using the Cobb method, pre and postoperative measurements of local/regional kyphosis were done. Degree and mean percentage of correction were calculated. Anterior intervertebral height of the kyphotic area was also measured. The mean follow-up period was 27 months. Results: Operative time was 271.3 min ± 25, and estimated intraoperative blood loss was 781.3 mL ± 92.3. On average, 2.5 vertebrae were resected. All 16 patients were able to lie supine immediately postoperatively. The mean preoperative local/regional kyphosis was 107.5°, and 106.9° respectively, corrected to 22.5° and 28.8° postoperatively, with a mean degree of correction of 85° and 78.1° respectively. Mean preoperative anterior intervertebral height was 3.54 cm, improved to 4.64 cm postoperatively. Only 2 cases had a superficial wound infection managed conservatively. At the latest follow-up, no loss of correction pseudoarthrosis occurred, and all patients showed solid fusion. Conclusion: Titanium mesh cage is an efficient, easy method for anterior reconstruction following kyphectomy in myelomeningocele patients, to maintain postoperative correction.Level of evidence: Therapeutic studies, Level IV study

Frequency, Predictors, and Outcome of Seizures in Patients With Myelomeningocele: Single-Center Retrospective Cohort Study.

To determine the frequency, predictors, and outcomes of seizures in patients with myelomeningocele, we retrospectively analyzed the data from patients with myelomeningocele followed longitudinally at a single center from 1975 to 2013. We identified a total of 122 patients (61% female). The mean follow-up duration was 11.1 years (minimum-maximum = 0-34.5 years, SD = 8.8, median = 9.1 years). A total of 108 (88.5%) patients had hydrocephalus, and 98 (90.7%) of those patients required a ventriculoperitoneal shunt procedure. Twenty-four (19.7%) patients manifested with seizures, 23 of whom had hydrocephalus. The average age of seizure onset was 4.8 years (median 2 years of age). Falx dysgenesis (P = .004), lumbar myelomeningocele (P = .007), and cortical atrophy (P = .028) were significantly associated with epileptic seizure development. The average seizure-free period at the last follow-up in patients with a history of myelomeningocele and seizures was 8.1 years. We conclude that myelomeningocele patients with seizures have an overall good prognosis with considerable long-term seizure freedom.