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1.
Artigo em Inglês | MEDLINE | ID: mdl-38560818

RESUMO

BACKGROUND AND OBJECTIVES: Growing skull fracture (GSF) is a rare complication of pediatric head trauma. Definitive treatment is surgical repair. We have attempted to assess whether use of autologous grafts for duraplasty and cranioplasty leads to better outcomes. We have also attempted to understand how timing of surgery might affect the degree of underlying damage to cortical tissue. METHODS: This is a single-center retrospective observational study based on review from the Great Ormond Street Hospital Neurosurgery prospective surgical database. All patients undergoing surgery for GSF repair between 1991 and 2015 were included. Surgical techniques included split calvarial grafts in 4 patients, whereas rest had full-thickness bone grafting. In all cases with full-thickness graft, the donor site was covered with morselized bone chips mixed with fibrin glue (Salami technique). RESULTS: Twenty-eight patients were identified (16 males, 12 females). The average age at the time of injury was 13 months. The mean duration of onset of symptoms from the time of injury was 4.4 months. The time interval from symptom onset to surgical repair was 5.92 months. Seven patients had Type I GSF (leptomeningeal cyst with minimal brain parenchyma), 13 had type II (hernia containing gliotic brain), and 8 had type III (porencephalic cyst extending through the skull defect into subgaleal space). Patients with delayed presentation had severe brain injury (Type III) and had more long-term complications (refractory epilepsy requiring temporo-occipito-parietal disconnection and development of hydrocephalus requiring ventriculoperitoneal shunt insertion). CONCLUSION: Autologous pericranium for duraplasty and split-thickness bone graft or the Salami technique are recommended for cranioplasty. Synthetic materials should be used if the index operation fails or there are complications. Patients with high-risk findings should be identified at the time of initial presentation and followed up in clinic early to prevent onset of neurological deficit. Early repair is associated with better neurological outcomes.

2.
Artigo em Inglês | MEDLINE | ID: mdl-38360788

RESUMO

Dysraphic malformations of the spine and spinal cord (DMSSC) represent a spectrum of common congenital anomalies typically (though not exclusively) affecting the lower spinal segments. These may be responsible for varying degrees of neurologic, orthopedic, and urologic morbidity. With advances in neuroimaging, it is now possible to better diagnose and evaluate these disorders both prenatally and postnatally. Neuroimaging, performed at the right time and with technique optimization, is integral in guiding clinical management. However, the terminology used to describe these lesions has become increasingly confusing, and there is a lack of consensus regarding the essential radiologic features and their clinical weighting. This variability in radiologic practice risks unstructured decision making and increases the likelihood of suboptimal, less informed clinical management. In this manuscript, the first of a series of consensus statements, we outline a standardized international consensus statement for the radiologic evaluation of children with suspected DMSSC derived from a critical review of the literature, and the collective clinical experience of a multinational group of experts. We provide recommendations for plain radiography, sonography, CT, and MR imaging in the evaluation of DMSSC with an emphasis on technique of imaging and imaging protocols.

3.
Childs Nerv Syst ; 40(1): 213-218, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37428253

RESUMO

OBJECTIVE: The fibroneural stalk of an LDM has variable thickness, complexity, and length, which can span 5 to 6 vertebral segments from its skin attachment to its "merge point" with the dorsal spinal cord. Therefore, complete resection may require extensive multi-level laminotomies. In this technical note, a modification of the procedure is presented that avoids long segment laminectomies while ensuring complete excision of long LDM stalks. RESULTS: An illustrative case of resection of LDM is presented using skip laminectomies. The technique ensures complete removal of the stalk, thus reducing the risk of future intradural dermoid development, while at the same time minimizes the risk for delayed kyphotic deformity. CONCLUSIONS: A technique of "skip-hop" proximal and distal short segment laminectomies in cases of LDM optimizes the objectives of complete stalk resection with preservation of spinal integrity.


Assuntos
Laminectomia , Medula Espinal , Humanos , Medula Espinal/cirurgia , Pele , Coluna Vertebral/cirurgia
4.
Dev Med Child Neurol ; 66(2): 216-225, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37559345

RESUMO

AIM: To evaluate a lesion detection algorithm designed to detect focal cortical dysplasia (FCD) in children undergoing stereoelectroencephalography (SEEG) as part of their presurgical evaluation for drug-resistant epilepsy. METHOD: This was a prospective, single-arm, interventional study (Idea, Development, Exploration, Assessment, and Long-Term Follow-Up phase 1/2a). After routine SEEG planning, structural magnetic resonance imaging sequences were run through an FCD lesion detection algorithm to identify putative clusters. If the top three clusters were not already sampled, up to three additional SEEG electrodes were added. The primary outcome measure was the proportion of patients who had additional electrode contacts in the SEEG-defined seizure-onset zone (SOZ). RESULTS: Twenty patients (median age 12 years, range 4-18 years) were enrolled, one of whom did not undergo SEEG. Additional electrode contacts were part of the SOZ in 1 out of 19 patients while 3 out of 19 patients had clusters that were part of the SOZ but they were already implanted. A total of 16 additional electrodes were implanted in nine patients and there were no adverse events from the additional electrodes. INTERPRETATION: We demonstrate early-stage prospective clinical validation of a machine learning lesion detection algorithm used to aid the identification of the SOZ in children undergoing SEEG. We share key lessons learnt from this evaluation and emphasize the importance of robust prospective evaluation before routine clinical adoption of such algorithms. WHAT THIS PAPER ADDS: The focal cortical dysplasia detection algorithm collocated with the seizure-onset zone (SOZ) in 4 out of 19 patients. The algorithm changed the resection boundaries in 1 of 19 patients undergoing stereoelectroencephalography for drug-resistant epilepsy. The patient with an altered resection due to the algorithm was seizure-free 1 year after resective surgery. Overall, the algorithm did not increase the proportion of patients in whom SOZ was identified.


Assuntos
Epilepsia Resistente a Medicamentos , Epilepsia , Displasia Cortical Focal , Criança , Humanos , Pré-Escolar , Adolescente , Eletroencefalografia/métodos , Estudos Retrospectivos , Epilepsia/diagnóstico , Epilepsia/cirurgia , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgia , Convulsões
5.
J Neurosurg Pediatr ; 32(4): 478-487, 2023 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-37439477

RESUMO

OBJECTIVE: Invasive group A streptococcus (iGAS) infections are associated with a high rate of morbidity and mortality. CNS involvement is rare, with iGAS accounting for only 0.2%-1% of all childhood bacterial meningitis. In 2022, a significant increase in scarlet fever and iGAS was reported globally with a displacement of serotype, causing a predominance of the emm1.0 subtype. Here, the authors report on iGAS-related suppurative intracranial complications requiring neurosurgical intervention and prolonged antibiotic therapy. METHODS: The authors performed a retrospective chart review of consecutive cases of confirmed GAS in pediatric neurosurgical patients. RESULTS: Five children with a median age of 9 years were treated for intracranial complications of GAS infection over a 2-month period between November 2022 and December 2022. All patients had preceding illnesses, including chicken pox and upper respiratory tract infections. Infections included subdural empyema with associated encephalitis (n = 2), extradural empyema (n = 1), intracranial abscess (n = 1), and diffuse global meningoencephalitis (n = 1). Streptococcus pyogenes was cultured from 4 children, and 2 were of the emm1.0 subtype. Antimicrobial therapy in all patients included a third-generation cephalosporin but varied in adjunctive therapy, often including a toxin synthesis inhibitor antibiotic such as clindamycin. Neurological outcomes varied; 3 patients returned to near neurological baseline, 1 had significant residual neurological deficits, and 1 patient died. CONCLUSIONS: Despite the worldwide increased incidence, intracranial complications remain rarely reported resulting in a lack of awareness of iGAS-related intracranial disease. Awareness of intracranial complications of iGAS and prompt referral to a pediatric neurology/neurosurgical center is crucial to optimize neurological outcomes.


Assuntos
Abscesso Encefálico , Empiema Subdural , Criança , Humanos , Streptococcus pyogenes , Estudos Retrospectivos , Antibacterianos/uso terapêutico , Empiema Subdural/cirurgia
6.
Childs Nerv Syst ; 39(12): 3561-3570, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37368066

RESUMO

INTRODUCTION: Intracranial infection is often associated with contiguous sinus infection, with Streptococcus intermedius being the most common pathogen. Microbiological assessment is possible via sinus or intracranial sampling. While a sinus approach is minimally invasive, it is not clear whether this yields definitive microbiological diagnosis leading to optimized antimicrobial therapy and avoidance of intracranial surgery. METHODS: A retrospective review of a prospectively collected electronic departmental database identified patients between 2019 and 2022. Further demographic and microbiological information was obtained from electronic patient records and laboratory management systems. RESULTS: Thirty-one patients were identified with intracranial subdural and/or epidural empyema and concurrent sinus involvement during the 3-year study period. The median age of onset was 10 years with a slight male predominance (55%). All patients had intracranial sampling with 15 patients undergoing sinus sampling in addition. Only 1 patient (7%) demonstrated identical organism(s) grown from both samples. Streptococcus intermedius was the most common pathogen in intracranial samples. Thirteen patients (42%) had mixed organisms from their intracranial cultures and 57% of samples undergoing bacterial PCR identified additional organisms, predominantly anaerobes. Sinus samples had a significant addition of nasal flora and Staphylococcus aureus which was rarely grown from intracranial samples. Of concern, 7/14 (50%) of sinus samples did not identify the main intracranial pathogen diagnosed on intracranial culture and additional PCR. Literature review identified 21 studies where sinus drainage was used to treat intracranial empyemas, with only 6 authors reporting concurrent microbiology results. This confirmed our cohort to be the largest comparative study in the current literature. No center has observed a greater than 50% concordance in microbiological diagnoses. CONCLUSION: Endoscopic sinus surgery may have therapeutic benefit, but it is not an appropriate approach for microbiological diagnosis in pediatric subdural empyemas. High rates of contaminating nasal flora can lead to misdiagnosis and inappropriate treatment. Routine addition of 16S rRNA PCR to intracranial samples is recommended.


Assuntos
Empiema Subdural , Abscesso Epidural , Seios Paranasais , Sinusite , Criança , Feminino , Humanos , Masculino , Empiema Subdural/diagnóstico , Empiema Subdural/microbiologia , Abscesso Epidural/complicações , Estudos Retrospectivos , RNA Ribossômico 16S , Sinusite/complicações
7.
World Neurosurg X ; 19: 100200, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37181585

RESUMO

OBJECTIVES: Our objective was to review the outcomes of children with CIM and associated cerebrospinal fluid (CSF) disorders and ventriculomegaly undergoing endoscopic third ventriculostomy (ETV) as a primary intervention. MATERIALS AND METHODS: A retrospective, single-center, observational cohort study was conducted of consecutive children with CIM with associated CSF disorders and ventriculomegaly treated first by ETV between January 2014 and December 2020. RESULTS: Raised intracranial pressure symptoms were the most frequent in ten patients, followed by posterior fossa and syrinx symptoms in three cases. One patient had a later stoma closure and required a shunt insertion. The success rate of the ETV in the cohort was 92% (11/12). There was no surgical mortality in our series. No other complications were reported. The median herniation of the tonsils was not statistically different in the pre vs. post-operative MRI (1.14 vs. 0.94, p=0.1). However, the median Evan's index (0.4 vs. 0.36, p<0.01) and the median diameter of the third ventricle (1.35 vs. 0.76, p<0.01) were statistically different between the two measurements. The preoperative length of the syrinx did not change significantly compared with the postoperative (5 vs. 1; p=0.052); nevertheless, the median transverse diameter of the syrinx did improve significantly after the surgery (0.75 vs. 0.32, p=0.03). CONCLUSIONS: Our study supports ETV's safety and effectiveness for managing children with CSF disorders, ventriculomegaly, and associated CIM.

8.
J Neurosurg Pediatr ; 32(2): 214-222, 2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-37209074

RESUMO

OBJECTIVE: Robot-assisted (RA) stereotactic MRI-guided laser ablation has been reported to be a safe and effective technique for the treatment of epileptogenic foci in children and adults. In this study the authors aimed to assess the accuracy of RA stereotactic MRI-guided laser fiber placement in children and to identify factors that might increase the risk of misplacement. METHODS: A retrospective single-institution review of all children from 2019 to 2022 who underwent RA stereotactic MRI-guided laser ablation for epilepsy was undertaken. Placement error was calculated at the target by measuring the Euclidean distance between the implanted laser fiber position and the preoperatively planned position. Collected data included age at surgery, sex, pathology, date of robot calibration, number of catheters, entry position, entry angle, extracranial soft-tissue thickness, bone thickness, and intracranial catheter length. A systematic review of the literature was also performed using Ovid Medline, Ovid Embase, and the Cochrane Central Register of Controlled Trials. RESULTS: In 28 children with epilepsy, the authors assessed 35 RA stereotactic MRI-guided laser ablation fiber placements. Twenty (71.4%) children had undergone ablation for hypothalamic hamartoma, 7 children (25.0%) for presumed insular focal cortical dysplasia, and 1 patient (3.6%) for periventricular nodular heterotopia. Nineteen children were male (67.9.%) and 9 were female (32.1%). The median age at the time of the procedure was 7.67 years (IQR 4.58-12.26 years). The median target point localization error (TPLE) was 1.27 mm (IQR 0.76-1.71 mm). The median offset error between the planned and actual trajectories was 1.04° (IQR 0.73°-1.46°). Patient age, sex, pathology and the time interval between date of surgery and robot calibration, entry position, entry angle, soft-tissue thickness, bone thickness, and intracranial length were not associated with the placement accuracy of the implanted laser fibers. However, the number of catheters placed did correlate with the offset angle error on univariate analysis (ρ = 0.387, p = 0.022). There were no immediate surgical complications. Meta-analysis indicated that the overall pooled mean TPLE was 1.46 mm (95% CI -0.58 to 3.49 mm). CONCLUSIONS: RA stereotactic MRI-guided laser ablation for epilepsy in children is highly accurate. These data will aid surgical planning.


Assuntos
Epilepsia Resistente a Medicamentos , Epilepsia , Terapia a Laser , Robótica , Adulto , Humanos , Masculino , Criança , Feminino , Pré-Escolar , Técnicas Estereotáxicas , Estudos Retrospectivos , Epilepsia/diagnóstico por imagem , Epilepsia/cirurgia , Lasers , Terapia a Laser/métodos , Imageamento por Ressonância Magnética/métodos , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgia
9.
Epilepsia ; 63(12): 3168-3179, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36177545

RESUMO

OBJECTIVE: To determine whether an ictal electroencephalographic (EEG) recording as part of presurgical evaluation of children with a demarcated single unilateral magnetic resonance imaging (MRI) lesion is indispensable for surgical decision-making, we investigated the relationship of interictal/ictal EEG and seizure semiology with seizure-free outcome. METHODS: Data were obtained retrospectively from consecutive patients (≤18 years old) undergoing epilepsy surgery with a single unilateral MRI lesion at our institution over a 6-year period. Video-telemetry EEG (VT-EEG) was classified as concordant or nonconcordant/noninformative in relation to the MRI lesion location. The odds of seizure-free outcome associated with nonconcordant versus concordant for semiology, interictal EEG, and ictal EEG were compared separately. Multivariate logistic regression was conducted to correct for confounding variables. RESULTS: After a median follow-up of 26 months (interquartile range = 17-37.5), 73 (69%) of 117 children enrolled were seizure-free. Histopathological diagnoses included low-grade epilepsy-associated tumors, n = 46 (39%); focal cortical dysplasia (FCD), n = 33 (28%); mesial temporal sclerosis (MTS), n = 23 (20%); polymicrogyria, n = 3 (3%); and nondiagnostic findings/gliosis, n = 12 (10%). The odds of seizure freedom were lower with a nonconcordant interictal EEG (odds ratio [OR] = .227, 95% confidence interval [CI] = .079-.646, p = .006) and nonconcordant ictal EEG (OR = .359, 95% CI = .15-.878, p = .035). In the multivariate logistic regression model, factors predicting lower odds for seizure-free outcome were developmental delay/intellectual disability and higher number of antiseizure medications tried, with a nonsignificant trend for "nonconcordant interictal EEG." In the combined subgroup of patients with FCD and tumors (n = 79), there was no significant relationship of VT-EEG factors and seizure outcomes, whereas in children with MTS and acquired lesions (n = 25), a nonconcordant EEG was associated with poorer seizure outcomes (p = .003). SIGNIFICANCE: An ictal EEG may not be mandatory for presurgical evaluation, particularly when a well-defined single unilateral MRI lesion has been identified and the interictal EEG is concordant.


Assuntos
Epilepsia , Imageamento por Ressonância Magnética , Criança , Humanos , Adolescente , Estudos Retrospectivos , Espectroscopia de Ressonância Magnética , Epilepsia/diagnóstico por imagem , Epilepsia/cirurgia
10.
Childs Nerv Syst ; 38(11): 2235-2238, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-35578116

RESUMO

INTRODUCTION: Atlantoaxial rotatory fixation (AARF) is a rare condition that occurs most commonly in children. The torticollis caused by AARF usually presents as abnormal posturing of the head and neck, with rotation of the chin to the opposite side. AARF in children could occur due to congenital bony malformation, minor trauma, upper respiratory tract infections (Grisel's syndrome), postoperatively after head and neck (ENT) surgery, and unknown reasons. AARF in the postoperative patient is a rare and poorly understood entity. METHODS: The authors present a case report of a 2-year-old boy with Crouzon Syndrome undergoing posterior calvarial vault expansion (PVE) surgery that developed AARF as a complication. RESULTS: The authors believe that cranial vault surgery should be considered a potential risk procedure for AARF, especially if it is done in susceptible populations (syndromic craniosynostosis patients) with other underlying sequelae (tonsillar ectopia or syringomyelia). During surgery, careful attention should be paid to maintaining a neutral alignment of the patient's cervical spine as rotatory movements under anaesthesia and muscle relaxation may be contributory factors. CONCLUSION: AARF should be suspected and investigated in children with painful torticollis after craniofacial surgery.


Assuntos
Articulação Atlantoaxial , Luxações Articulares , Torcicolo , Criança , Masculino , Humanos , Pré-Escolar , Articulação Atlantoaxial/diagnóstico por imagem , Articulação Atlantoaxial/cirurgia , Articulação Atlantoaxial/lesões , Torcicolo/diagnóstico por imagem , Torcicolo/etiologia , Torcicolo/cirurgia , Rotação , Luxações Articulares/etiologia , Vértebras Cervicais
11.
Childs Nerv Syst ; 38(6): 1113-1123, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35262755

RESUMO

PURPOSE: In 2009, Pang described a radical resection technique for congenital lumbosacral lipomas, with lower long-term symptomatic re-tethering rates compared with partial resections and conservative management, and low surgical morbidity. We adopted this technique in 2011, and aim to describe our first results. METHODS: In this monocentric retrospective audit, we included dorsal, transitional, chaotic, and caudal-type lumbosacral lipomas. Exclusion criteria were previously operated lipomas, pure filar lipomas, and concomitant major congenital anatomical urogenital/gastrointestinal abnormalities. Neuro-uro-orthopaedic status at presentation and at three months, one year and last postoperative follow-up, intraoperative electrophysiology, and extent of resection were collected. RESULTS: From January 2011 to September 2019, 91 patients were operated (median age 2y2m; 63 transitional; 14 caudal; 8 dorsal; 6 chaotic). Preoperatively, 67% were symptomatic. Preoperative and one-year postoperative rates of impaired ambulation (44% to 43%), hypoesthesia (8% to 5%), urodynamic/uroradiological abnormalities (49% to 37%), and foot/ankle deformities (8% to 5%) were comparable, whilst pain improved (25% to 5%) but catheterisation rates increased (21% to 36%). 23/92 (25%) suffered wound-related complications. 2/91 (2%) developed symptomatic re-tethering requiring second surgery. Mean cord/sac ratio was 0.47. 43% had > 20 mm3 residual fat, which improved with increasing surgical experience. CONCLUSION: Radical lipoma resection, guided by intraoperative neuromonitoring, with reconstruction of the neural placode and expansion duraplasty is technically feasible and results in low rates of late deterioration and re-tethering. Lipoma-type and pre-operative status are important outcome predictors. Operative risks are not insignificant. Future studies need to determine appropriate selection criteria for surgery.


Assuntos
Lipoma , Neoplasias da Medula Espinal , Neoplasias da Coluna Vertebral , Criança , Pré-Escolar , Hospitais , Humanos , Lactente , Lipoma/diagnóstico por imagem , Lipoma/cirurgia , Região Lombossacral/cirurgia , Estudos Retrospectivos , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/cirurgia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Resultado do Tratamento
12.
Epileptic Disord ; 24(1): 111-122, 2022 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-34750096

RESUMO

OBJECTIVE: Focal cortical dysplasia (FCD) is a malformation of cortical development and is associated with drug-resistant epilepsy. Standard indication for epilepsy surgery is drug resistance (as defined by the ILAE). Given the high incidence of drug resistance in these children, this delay may not be warranted. The aim of the study was to determine the proportion of patients with a presumed FCD who develop drug resistance, and evaluate post-operative outcomes. METHODS: This study incorporated a survey within a regional paediatric epilepsy network and a retrospective database review of a paediatric epilepsy centre serving the network to identify children with epilepsy and a presumed FCD on MRI. RESULTS: The survey revealed that 86% of the patients with epilepsy and presumed FCD on MRI within the network were referred to our centre. Of 139 paediatric patients included in the study, 131 (94.2%) had drug-resistant epilepsy. One hundred and ten (83.9%) patients were referred to epilepsy surgery, of whom 97 underwent surgery. Of 92 with one-year postoperative follow-up, 59.8% had an Engel Class 1 (seizure-free) outcome. Concordance of location between MRI and ictal EEG was strongly associated with Engel Class 1 outcome (p<0.001), as was older age at seizure onset (p=0.03). Time from diagnosis to surgery, number of medications, type of surgery and histology were not associated with improved outcome. SIGNIFICANCE: Our data suggest that most children presenting with seizures and a radiological diagnosis of FCD will develop drug-resistant epilepsy and are candidates for epilepsy surgery. The main outcome predictors are the correlation between MRI and ictal EEG localization and age at onset. This suggests that patients with FCD and epilepsy may be considered for surgery before traditional criteria of drug resistance are met. This change in practice has the potential to improve quality of life and cognitive function, and reduce burden on epilepsy services.


Assuntos
Epilepsia Resistente a Medicamentos , Malformações do Desenvolvimento Cortical , Convulsões , Criança , Epilepsia Resistente a Medicamentos/diagnóstico , Epilepsia Resistente a Medicamentos/epidemiologia , Epilepsia Resistente a Medicamentos/cirurgia , Diagnóstico Precoce , Eletroencefalografia , Humanos , Imageamento por Ressonância Magnética , Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Estudos Retrospectivos , Convulsões/fisiopatologia , Resultado do Tratamento
13.
Epilepsy Res ; 177: 106769, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34560348

RESUMO

OBJECTIVE: In this study, we investigate the seizure outcomes of temporo-parieto-occipital (TPO) and frontal disconnections or resections in children with drug-resistant epilepsy (DRE) in order to determine factors which may predict surgical results. METHODS: Children with DRE, who underwent either TPO or frontal disconnection or resection at Great Ormond Street Hospital for Children between 2000 and 2017, were identified from a prospectively collated operative database. Demographic data, age at surgery, type of surgery, scalp EEGs and operative histopathology were collected. Magnetic resonance imaging (MRI) was assessed to determine completeness of disconnection and presence of radiological lesion beyond the disconnection margins. Seizure outcome at 6, 12, and 24 months post-surgery was assessed using the Engel Scale (ES). Logistic regression was used to identify relationships between data variables and seizure outcome. RESULTS: 46 children (males = 28, females = 18; age range 0.5-16.6 years) who underwent TPO (n = 32, including a re-do disconnection) or frontal disconnection or resection (n = 15) were identified. Patients in the TPO treatment group had more favourable seizure outcomes than those in the frontal treatment group (ES I-II in 56 %vs 47 % at 6 months, 52 % vs 46 % at 12 months). Presence of the lesion beyond disconnection boundaries and older age at the time of surgery were associated with poorer seizure outcome. Gender, surgery type, completeness of disconnection, scalp EEG findings and underlying pathology were not related to seizure outcome, but subgroup numbers were small. CONCLUSIONS: Both TPO and frontal disconnection are effective treatments for selected children with posterior multi-lobar or diffuse frontal lobe epilepsy. Confinement of the MRI lesion within the disconnection margins and a younger age at surgery are associated with favourable seizure outcomes. Further studies are required to elucidate these findings.


Assuntos
Epilepsia Resistente a Medicamentos , Epilepsia do Lobo Frontal , Adolescente , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia , Epilepsia do Lobo Frontal/cirurgia , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Convulsões/diagnóstico por imagem , Convulsões/cirurgia , Resultado do Tratamento
14.
Childs Nerv Syst ; 35(10): 1763-1768, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31372737

RESUMO

PURPOSE: Chiari malformation incorporate numerous forms of congenital or acquired cerebellar herniation through the foramen magnum. This may lead to brain stem, high spinal cord and cranial nerve compression resulting in obstructive and central apneas. This review highlights he high prevalence of sleep-disordered breathing in this population and the importance of refering these patients for sleep studies as part of their workup. METHODS: A review of the literature was performed through a PubMed and EMBASE search of original articles and reviews using the key words "chiari" "chiari malformation" "hindbrain herniation" "sleep disordered breathing" "obstructive sleep apnea" "central sleep apnea" "sleep study" and "foramen magnum decompression". DISCUSSION: We highlight the pathophysiology of sleep disordered breathing in patients with Chiari malformation, how it can be diagnosed and what the treatment options are. CONCLUSIONS: Sleep-disordered breathing is highly prevalent in patients with CM1. Clinicians caring for these patients should be aware of this and prioritise sleep diagnostic testing to allow for early diagnosis and management particularly in the presence of neurological symptoms and specific brain MRI pointers.


Assuntos
Malformação de Arnold-Chiari/diagnóstico , Malformação de Arnold-Chiari/fisiopatologia , Síndromes da Apneia do Sono/diagnóstico , Síndromes da Apneia do Sono/fisiopatologia , Malformação de Arnold-Chiari/complicações , Criança , Humanos , Polissonografia/métodos , Síndromes da Apneia do Sono/complicações
15.
Epilepsia ; 59(1): 170-178, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29178251

RESUMO

OBJECTIVE: Glioneuronal tumors (GNTs) are well-recognized causes of chronic drug-resistant focal epilepsy in children. Our practice involves an initial period of radiological surveillance and antiepileptic medications, with surgery being reserved for those with radiological progression or refractory seizures. We planned to analyze the group of patients with low-grade GNTs, aiming to identify factors affecting seizure and cognitive outcomes. METHODS: We retrospectively reviewed the medical records of 150 children presenting to Great Ormond Street Hospital with seizures secondary to GNTs. Analysis of clinical, neuroimaging, neuropsychological, and surgical factors was performed to determine predictors of outcome. Seizure outcome at final follow-up was classified as either seizure-free (group A) or not seizure-free (group B) for patients with at least 12-months follow-up postsurgery. Full-scale intelligence quotient (FSIQ) was used as a measure of cognitive outcome. RESULTS: Eighty-six males and 64 females were identified. Median presurgical FSIQ was 81. One hundred twenty-one patients (80.5%) underwent surgery. Median follow-up after surgery was 2 years, with 92 patients (76%) having at least 12 months of follow-up after surgery. Seventy-four patients (80%) were seizure-free, and 18 (20%) continued to have seizures. Radiologically demonstrated complete tumor resection was associated with higher rates of seizure freedom (P = .026). Higher presurgical FSIQ was related to shorter epilepsy duration until surgery (P = .012) and to older age at seizure onset (P = .043). SIGNIFICANCE: A high proportion of children who present with epilepsy and GNTs go on to have surgical tumor resection with excellent postoperative seizure control. Complete resection is associated with a higher chance of seizure freedom. Higher presurgical cognitive functioning is associated with shorter duration of epilepsy prior to surgery and with older age at seizure onset. Given the high rate of eventual surgery, early surgical intervention should be considered in children with continuing seizures associated with GNTs.


Assuntos
Neoplasias Encefálicas/cirurgia , Transtornos Cognitivos/etiologia , Ganglioglioma/cirurgia , Neoplasias Neuroepiteliomatosas/cirurgia , Neurocirurgia/métodos , Convulsões/etiologia , Adolescente , Anticonvulsivantes/uso terapêutico , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico por imagem , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Ganglioglioma/complicações , Ganglioglioma/diagnóstico por imagem , Humanos , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Neoplasias Neuroepiteliomatosas/complicações , Neoplasias Neuroepiteliomatosas/diagnóstico por imagem , Testes Neuropsicológicos , Estudos Retrospectivos , Convulsões/tratamento farmacológico , Resultado do Tratamento
16.
Epilepsy Res Treat ; 2012: 547382, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22957232

RESUMO

Background. Developing countries, home to 80% of epilepsy patients, do not have comprehensive epilepsy surgery programs. Considering these needs we set up first epilepsy surgery center in Pakistan. Methods. Seventeen teleconferences focused on setting up an epilepsy center at the Aga Khan University (AKU), Karachi, Pakistan were arranged with experts from the University of Alberta Hospital, Alberta, Canada and the University of West Virginia, USA over a two-year period. Subsequently, the experts visited the proposed center to provide hands on training. During this period several interactive teaching sessions, a nationwide workshop, and various public awareness events were organized. Results. Sixteen patients underwent surgery, functional hemispherectomy (HS) was done in six, anterior temporal lobectomy (ATL) in six, and neuronavigation-guided selective amygdalohippocampectomy (SAH) using keyhole technique in four patients. Minimal morbidity was observed in ATL and, SAH groups. All patients in SAH group (100%) had Grade 1 control, while only 5 patients (83%) in ATL group, and 4 patients (66%) in HS group had Grade 1 control according to Engel's classification, in average followups of 12 months, 24 months and 48 months for SAH, ATL, and HS, respectively. Conclusion. As we share our experience we hope to set a practical example for economically constrained countries that successful epilepsy surgery centers can be managed with limited resources.

17.
Surg Neurol Int ; 2: 183, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-22276237

RESUMO

OBJECTIVE: To observe interdepartmental variation in the availability of resources and academic activities within the various neurosurgery programs of Pakistan. METHODS: This was a proforma-based survey of neurosurgery trainees and young neurosurgeons of Pakistan, looking at the academic infrastructure and output of their programs. The proforma was filled by 36 respondents from 11 neurosurgery centers of the country. All these centers were accredited for neurosurgery training in Pakistan. RESULTS: Out of the 36 respondents, 30 were completing a Fellowship training (FCPS) and six were enrolled for a Master in Surgery (MS) program. About 80% of the participants used the Youman's Textbook of Neurosurgery as a reference book. Only 40% of the candidates had access to more than one indexed neurosurgery journal. Structured academic sessions (e.g., journal clubs and neuropathology meetings) were lacking in a majority of the training institutes, 95% of the trainees had no microsurgical laboratory experience, and modern neurosurgical tools (frameless neuronavigation system, neuroendoscopy) were in use at a few centers only. CONCLUSION: Neurosurgery training in Pakistan is not uniform and wide variations exist between the programs at the centers evaluated. We recommend exchange programs between centers at national and international levels, to allow trainees to gain first-hand exposure to training components not available in their own center.

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