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1.
J Neurosurg Pediatr ; : 1-10, 2024 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-39094184

RESUMO

OBJECTIVE: Intracranial complications of acute bacterial sinusitis are rare pathologies that occur in children, and are associated with significant neurological morbidity and mortality. There is a subjective concern among neurosurgeons that the incidence of this rare disease has increased since the onset of the novel COVID-19 pandemic. The primary objective of this study was to review the presentation and management of patients admitted at the authors' institution with intracranial extension of sinusitis, to better understand the local disease burden relative to the COVID-19 pandemic. METHODS: This is a single-center retrospective observational cohort study. The patients underwent neurosurgical intervention for intracranial extension of sinusitis between January 1, 2007, and March 1, 2023. The historical cohort was defined as those patients who presented prior to March 2020. Clinical covariates such as surgical and microbiological data were collected and analyzed. RESULTS: A total of 78 patients (55 historical, 23 new) were included; they had a median age of 11.7 years and a male predominance of 69.2%. There was a significant increase in the annual rate of neurosurgical intervention for suppurative intracranial extension of acute bacterial sinusitis after the onset of the COVID-19 pandemic, with an average of 4.2 cases per year prior to March 2020 compared to 7.7 cases per year after that date (p = 0.013). This increase was largely driven by the unprecedented case volume of 13 cases in 2022. Patients in the new cohort were older (p = 0.009) and more likely to have Pott's puffy tumor/frontal bone osteomyelitis (p = 0.003) at the time of presentation than patients in the historical cohort. Patients in the new cohort had lower rates of readmission within 30 days of discharge than those in the historical cohort (p = 0.047). In both cohorts, patients with seizure on presentation were more likely to have neurological sequelae at last follow-up (p = 0.004), which occurred at a median of 2.9 months after discharge. CONCLUSIONS: Clinicians encountering pediatric patients presenting with persistent symptoms of acute bacterial sinusitis must have a high index of suspicion for suppurative intracranial extension. Prompt neuroimaging and subsequent neurosurgical intervention are critical to ensure timely diagnosis and treatment. The results in this study show a significant increase in the number of neurosurgical interventions for suppurative intracranial extension of sinusitis per year after the onset of the COVID-19 pandemic. Further research is needed to understand the underlying pathophysiology of this clinical phenomenon.

2.
J Neurosurg Case Lessons ; 7(20)2024 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-38739949

RESUMO

BACKGROUND: Skull lesions are a common finding in children, with dermoid cysts and eosinophilic granulomas observed most frequently. However, primary intraosseous xanthomas of the calvaria, which are lytic, expansile lesions that develop without underlying hyperlipidemic disease, are rare in children, with only one prior case reported. OBSERVATIONS: The authors describe the case of a healthy 6-year-old male who presented with a 2-month history of an enlarging midline skull mass that developed after a recent minor trauma. Imaging showed a full-thickness, lytic frontal bone lesion with an aggressive appearance and heterogeneous contrast enhancement. The patient underwent gross-total resection of the lesion with placement of a mesh cranioplasty. Histopathology revealed a primary intraosseous xanthoma. The patient was discharged on postoperative day 2 and required no further treatment at the 1-month follow-up. LESSONS: This is the first reported case of a primary intraosseous xanthoma in the frontal bone of a pediatric patient. It emphasizes the need to include primary xanthomas in the differential diagnosis for pediatric skull lesions, particularly when the lesion has an aggressive radiographic appearance or the patient has a history of focal trauma. Furthermore, our findings indicate that resection, together with subsequent monitoring for lesion reccurrence, is an adequate first-line treatment.

3.
J Neurosurg Pediatr ; 33(5): 405-410, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38428005

RESUMO

OBJECTIVE: Among patients with a history of prior lipomyelomeningocele repair, an association between increased lumbosacral angle (LSA) and cord retethering has been described. The authors sought to build a predictive algorithm to determine which complex tethered cord patients will develop the symptoms of spinal cord retethering after initial surgical repair with a focus on spinopelvic parameters. METHODS: An electronic medical record database was reviewed to identify patients with complex tethered cord (e.g., lipomyelomeningocele, lipomyeloschisis, myelocystocele) who underwent detethering before 12 months of age between January 1, 2008, and June 30, 2022. Descriptive statistics were used to characterize the patient population. The Caret package in R was used to develop a machine learning model that predicted symptom development by using spinopelvic parameters. RESULTS: A total of 72 patients were identified (28/72 [38.9%] were male). The most commonly observed dysraphism was lipomyelomeningocele (41/72 [56.9%]). The mean ± SD age at index MRI was 2.1 ± 2.2 months, at which time 87.5% of patients (63/72) were asymptomatic. The mean ± SD lumbar lordosis at the time of index MRI was 23.8° ± 11.1°, LSA was 36.5° ± 12.3°, sacral inclination was 30.4° ± 11.3°, and sacral slope was 23.0° ± 10.5°. Overall, 39.6% (25/63) of previously asymptomatic patients developed new symptoms during the mean ± SD follow-up period of 44.9 ± 47.2 months. In the recursive partitioning model, patients whose LSA increased at a rate ≥ 5.84°/year remained asymptomatic, whereas those with slower rates of LSA change experienced neurological decline (sensitivity 77.5%, specificity 84.9%, positive predictive value 88.9%, and negative predictive value 70.9%). CONCLUSIONS: This is the first study to build a machine learning algorithm to predict symptom development of spinal cord retethering after initial surgical repair. The authors found that, after initial surgery, patients who demonstrate a slower rate of LSA change per year may be at risk of developing neurological symptoms.


Assuntos
Algoritmos , Aprendizado de Máquina , Meningomielocele , Defeitos do Tubo Neural , Humanos , Defeitos do Tubo Neural/cirurgia , Defeitos do Tubo Neural/diagnóstico por imagem , Feminino , Masculino , Meningomielocele/cirurgia , Meningomielocele/diagnóstico por imagem , Lactente , Estudos Retrospectivos , Procedimentos Neurocirúrgicos/métodos , Imageamento por Ressonância Magnética , Valor Preditivo dos Testes
4.
Spinal Cord Ser Cases ; 10(1): 1, 2024 01 04.
Artigo em Inglês | MEDLINE | ID: mdl-38177120

RESUMO

INTRODUCTION: Traumatic injuries of the spine requiring surgery are rare in infancy. Fusion procedures in the very young are not well-described at the atlanto-occipital junction or subaxial spine. Here we describe novel segmental posterior instrumentation in a severe spinal column disruption in an infant. CASE PRESENTATION: A 13-month-old male with atlanto-occipital dislocation and severe C6-7 distraction (ASIA impairment scale A) presented after a motor vehicle accident. He underwent instrumented fusion (occiput-C2 and C6-7) and halo placement. Postoperative imaging demonstrated reduction of the C6-7 vertebral bodies. Physical examination showed lower limb paraplegia and preserved upper extremity strength except for mild weakness in hand grip (3/5 on the MRC grading scale). Occiput-C2 instrumentation was performed using occipital keel and C2 pedicle screws with sublaminar C1 polyester tape. C6-7 reduction and fixation was performed with laminar hooks. Arthrodesis was promoted with lineage-committed cellular bone matrix allograft and suboccipital autograft. Anterior column stabilization was deferred secondary to a CSF leak. Intraoperative monitoring was performed throughout the procedure. Within 1 month after surgery the patient was able to manipulate objects against gravity. CT imaging revealed bony fusion and spontaneous reduction of C6-7. DISCUSSION: Spinal instrumentation is technically challenging in infants, regardless of injury mechanism, particularly in cases with complete spinal column disruption, but an anterior fusion may be avoided in infants and small children with posterior stabilization and halo placement.


Assuntos
Luxações Articulares , Fusão Vertebral , Humanos , Lactente , Masculino , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Vértebras Cervicais/lesões , Força da Mão , Luxações Articulares/complicações , Luxações Articulares/diagnóstico por imagem , Luxações Articulares/cirurgia , Fusão Vertebral/métodos
5.
Childs Nerv Syst ; 40(5): 1361-1366, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38150037

RESUMO

PURPOSE: Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) represent a rare pediatric-type tumor that most commonly presents as medically refractory epilepsy. PLNTY has only recently been recognized as a distinct clinical entity, having been first described in 2016 and added to the World Health Organization classification of CNS tumors in 2021. Molecular studies have determined that PLNTY is uniformly driven by aberrant MAPK pathway activation, with most tumors carrying either a BRAF V600E mutation or activating FGFR2 or FGFR3 fusion protein. Although it is known that these driver mutations are mutually exclusive, little is known about differences in clinical presentation or treatment outcomes between PLNTY cases driven by these distinct mutations. METHODS: We performed a systematic review and cumulative analysis of PLNTY cases to assess whether or not PLNTY tumors carrying the BRAF V600E mutation exhibit different clinical behaviors. By searching the literature for all cases of PLNTY wherein BRAF V600E status was characterized, we compiled a dataset of 62 unique patient instances. Using a logistic regression-based approach, we assessed a primary outcome of what factors of a clinical presentation were associated with BRAF V600E mutations and a secondary outcome of what factors predicted total seizure freedom post-surgical resection. RESULTS: PLNTY cases carrying BRAF V600E mutations in the literature were strongly positively associated with adult patients (p = 0.0055, OR = 6.556; 95% Conf. Int. = 1.737-24.742). BRAF V600E status was also positively associated with tumor involvement of the temporal lobe (p = 0.0046, OR = 11.036; 95% Conf. Int. = 2.100-58.006). Male sex was also positively associated with BRAF V600E status, but the result did not quite achieve statistical significance (p = 0.0731). BRAF V600E status was not found to be associated with post-operative seizure freedom. CONCLUSIONS: These findings indicate that BRAF V600E-positive PLNTY exhibit characteristic clinical presentations but are not necessarily different in treatment responsiveness. Non-BRAF V600E tumors are more commonly associated with young patients.


Assuntos
Neoplasias Encefálicas , Neoplasias Neuroepiteliomatosas , Proteínas Proto-Oncogênicas B-raf , Criança , Humanos , Masculino , Neoplasias Encefálicas/patologia , Mutação , Neoplasias Neuroepiteliomatosas/genética , Proteínas Proto-Oncogênicas B-raf/genética , Convulsões/complicações
6.
Artigo em Inglês | MEDLINE | ID: mdl-37582615

RESUMO

OBJECTIVES: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an immune-mediated neuroinflammatory disorder leading to demyelination of the CNS. Interleukin (IL)-6 receptor blockade is under study in relapsing MOGAD as a preventative strategy, but little is known about the role of such treatment for acute MOGAD attacks. METHODS: We discuss the cases of a 7-year-old boy and a 15-year-old adolescent boy with severe acute CNS demyelination and malignant cerebral edema with early brain herniation associated with clearly positive serum titers of MOG-IgG, whose symptoms were incompletely responsive to standard acute therapies (high-dose steroids, IV immunoglobulins (IVIGs), and therapeutic plasma exchange). RESULTS: Both boys improved quickly with IL-6 receptor inhibition, administered as tocilizumab. Both patients have experienced remarkable neurologic recovery. DISCUSSION: We propose that IL-6 receptor therapies might also be considered in acute severe life-threatening presentations of MOGAD.


Assuntos
Doenças Desmielinizantes , Humanos , Doenças Desmielinizantes/terapia , Imunoglobulinas Intravenosas , Glicoproteína Mielina-Oligodendrócito , Troca Plasmática , Plasmaferese , Masculino , Criança , Adolescente
7.
Neurooncol Adv ; 5(1): vdad049, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37197736

RESUMO

Diffuse leptomeningeal glioneuronal tumor (DLGNT) occurs predominantly in children and is typically characterized by diffuse leptomeningeal lesions throughout the neuroaxis with focal segments of parenchymal involvement. Recent reports have identified cases without diffuse leptomeningeal involvement that retain classic glioneuronal features on histology. In this report, we present a case of a 4-year-old boy with a large cystic-solid intramedullary spinal cord lesion that on surgical biopsy revealed a biphasic astrocytic tumor with sparsely distributed eosinophilic granular bodies and Rosenthal fibers. Next-generation sequencing revealed a KIAA1549-BRAF fusion, 1p/19q codeletion, and lack of an IDH1 mutation. Methylation profiling demonstrated a calibrated class score of 0.98 for DLGNT and copy number loss of 1p. Despite the morphologic similarities to pilocytic astrocytoma and the lack of oligodendroglial/neuronal components or leptomeningeal dissemination, the molecular profile was definitive in classifying the tumor as DLGNT. This case highlights the importance of molecular and genetic testing in the characterization of pediatric central nervous system tumors.

9.
Childs Nerv Syst ; 39(3): 701-709, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36394609

RESUMO

BACKGROUND: Variables interacting to predict outcomes following spring-mediated cranioplasty (SMC) for non-syndromic craniosynostosis, including spring parameters and calvarial thickness, are poorly understood. This study assessed interactions between spring parameters and calvarial thickness to predict changes in cephalic index (CI) following SMC. METHODS: Patients undergoing SMC for non-syndromic sagittal craniosynostosis at our institution between 2014 and 2021 were included. Calvarial thickness was determined from patient preoperative CTs using Materalise Mimics at 27 points in relation to the sagittal suture. Linear mixed effects models were used to determine interactions between anterior, middle, and posterior calvarial thickness with spring force and length. RESULTS: Sixty-nine patients undergoing surgery at mean age 3.7 months were included in this study. Stronger posterior spring force interacted with thinner posterior calvarial thickness to predict greater changes in CI at 3 months postoperatively (p = 0.022). When evaluating spring force and calvarial thickness set distances from the sagittal suture, stronger posterior spring force interacted with thinner posterior calvarial thickness 5 mm (p = 0.043) and 10 mm (p = 0.036) from the sagittal suture to predict changes in CI. Interactions between spring parameters and calvarial thickness in the anterior and middle positions did not significantly predict changes in CI. CONCLUSIONS: Stronger posterior spring force interacted with thinner posterior calvaria to predict greater changes in CI 3 months following SMC for non-syndromic sagittal craniosynostosis. These results suggest dynamic interactions between several variables may impact outcomes following SMC.


Assuntos
Craniossinostoses , Procedimentos de Cirurgia Plástica , Humanos , Lactente , Craniotomia/métodos , Estudos Retrospectivos , Crânio/diagnóstico por imagem , Crânio/cirurgia , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia
10.
Childs Nerv Syst ; 39(1): 295-299, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-35849142

RESUMO

BACKGROUND: Venous malformations (VMs) are slow-flow vascular anomalies present at birth that enlarge during adolescence, subsequently causing thrombosis, hemorrhage, and pain. CASE PRESENTATION: We describe a case of an adolescent male presenting with a large scalp venous malformation. Given the size and location of the lesion, a hybrid approach employing both sclerotherapy and surgical resection was utilized. The VM was successfully removed without complication. CONCLUSION: A hybrid approach is a safe and effective treatment consideration for immediate management of large venous malformation in higher-risk locations.


Assuntos
Escleroterapia , Malformações Vasculares , Adolescente , Recém-Nascido , Humanos , Criança , Masculino , Couro Cabeludo , Malformações Vasculares/cirurgia , Resultado do Tratamento
11.
Neurocrit Care ; 38(2): 242-253, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36207491

RESUMO

BACKGROUND: Ketamine has traditionally been avoided as an induction agent for tracheal intubation in patients with neurologic conditions at risk for intracranial hypertension due to conflicting data in the literature. The objective of this study was to evaluate and compare the effects of ketamine versus other medications as the primary induction agent on peri-intubation neurologic, hemodynamic and respiratory associated events in pediatric patients with neurologic conditions at risk for intracranial hypertension. METHODS: This retrospective observational study enrolled patients < 18 years of age at risk for intracranial hypertension who were admitted to a quaternary children's hospital between 2015 and 2020. Associated events included neurologic, hemodynamic and respiratory outcomes comparing primary induction agents of ketamine versus non-ketamine for tracheal intubation. RESULTS: Of 143 children, 70 received ketamine as the primary induction agent prior to tracheal intubation. Subsequently after tracheal intubation, all the patients received adjunct analgesic and sedative medications (fentanyl, midazolam, and/or propofol) at doses that were inadequate to induce general anesthesia but would keep them comfortable for further diagnostic workup. There were no significant differences between associated neurologic events in the ketamine versus non-ketamine groups (p = 0.42). This included obtaining an emergent computed tomography scan (p = 0.28), an emergent trip to the operating room within 5 h of tracheal intubation (p = 0.6), and the need for hypertonic saline administration within 15 min of induction drug administration for tracheal intubation (p = 0.51). There were two patients who had clinical and imaging evidence of herniation, which was not more adversely affected by ketamine compared with other medications (p = 0.49). Of the 143 patients, 23 had pre-intubation and post-intubation intracranial pressure values recorded; 11 received ketamine, and 3 of these patients had intracranial hypertension that resolved or improved, whereas the remaining 8 children had intracranial pressure within the normal range that was not exacerbated by ketamine. There were no significant differences in overall associated hemodynamic or respiratory events during tracheal intubation and no 24-h mortality in either group. CONCLUSIONS: The administration of ketamine as the primary induction agent prior to tracheal intubation in combination with other agents after tracheal intubation in children at risk for intracranial hypertension was not associated with an increased risk of peri-intubation associated neurologic, hemodynamic or respiratory events compared with those who received other induction agents.


Assuntos
Hipertensão Intracraniana , Ketamina , Humanos , Criança , Ketamina/uso terapêutico , Hipertensão Intracraniana/tratamento farmacológico , Analgésicos/uso terapêutico , Fentanila/efeitos adversos , Midazolam/uso terapêutico
12.
Cleft Palate Craniofac J ; : 10556656221135284, 2022 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-36357356

RESUMO

Head and neck positioning is a key element of craniofacial reconstructive surgery and can become challenging when intervention necessitates broad exposure of the calvarium. We present a case of craniosynostosis secondary to Apert's syndrome requiring anterior and posterior cranial vault access during surgical correction. A modified sphinx position was used that required significant neck extension. The patient had concurrent Chiari I malformation with brain stem compression so intraoperative neuromonitoring (IONM) was used to ensure that there were no negative effects on the neural elements with positioning. This highlights benefits of IONM in a setting not typically associated with its use.

13.
Neurosurg Focus ; 53(4): E3, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36183186

RESUMO

OBJECTIVE: Stereoelectroencephalography (SEEG) is a widely used technique for localizing seizure onset zones prior to resection. However, its use has traditionally been avoided in children under 2 years of age because of concerns regarding pin fixation in the immature skull, intraoperative and postoperative electrode bolt security, and stereotactic registration accuracy. In this retrospective study, the authors describe their experience using SEEG in patients younger than 2 years of age, with a focus on the procedure's safety, feasibility, and accuracy as well as surgical outcomes. METHODS: A retrospective review of children under 2 years of age who had undergone SEEG while at Children's Hospital of Philadelphia between November 2017 and July 2021 was performed. Data on clinical characteristics, surgical procedure, imaging results, electrode accuracy measurements, and postoperative outcomes were examined. RESULTS: Five patients younger than 2 years of age underwent SEEG during the study period (median age 20 months, range 17-23 months). The mean age at seizure onset was 9 months. Developmental delay was present in all patients, and epilepsy-associated genetic diagnoses included tuberous sclerosis (n = 1), KAT6B (n = 1), and NPRL3 (n = 1). Cortical lesions included tubers from tuberous sclerosis (n = 1), mesial temporal sclerosis (n = 1), and cortical dysplasia (n = 3). The mean number of placed electrodes was 11 (range 6-20 electrodes). Bilateral electrodes were placed in 1 patient. Seizure onset zones were identified in all cases. There were no SEEG-related complications, including skull fracture, electrode misplacement, hemorrhage, infection, cerebrospinal fluid leakage, electrode pullout, neurological deficit, or death. The mean target point error for all electrodes was 1.0 mm. All patients proceeded to resective surgery, with a mean follow-up of 21 months (range 8-53 months). All patients attained a favorable epilepsy outcome, including Engel class IA (n = 2), IC (n = 1), ID (n = 1), and IIA (n = 1). CONCLUSIONS: SEEG can be safely, accurately, and effectively utilized in children under age 2 with good postoperative outcomes using standard SEEG equipment. With minimal modification, this procedure is feasible in those with immature skulls and guides the epilepsy team's decision-making for early and optimal treatment of refractory epilepsy through effective localization of seizure onset zones.


Assuntos
Epilepsia Resistente a Medicamentos , Epilepsia , Esclerose Tuberosa , Criança , Pré-Escolar , Epilepsia Resistente a Medicamentos/cirurgia , Eletrodos Implantados , Eletroencefalografia/métodos , Epilepsia/diagnóstico por imagem , Epilepsia/cirurgia , Proteínas Ativadoras de GTPase , Histona Acetiltransferases , Humanos , Lactente , Estudos Retrospectivos , Convulsões/cirurgia , Técnicas Estereotáxicas , Esclerose Tuberosa/cirurgia
14.
J Craniofac Surg ; 33(8): 2333-2338, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35905391

RESUMO

BACKGROUND: Spring-mediated cranioplasty (SMC) is an increasingly utilized technique to treat patients with nonsyndromic sagittal craniosynostosis, but variables impacting outcomes are incompletely understood. The purpose of this study was to determine variables most predictive of outcomes following SMC, primarily changes in cephalic index (CI). METHODS: Patients with nonsyndromic sagittal craniosynostosis undergoing SMC at our institution between 2014 and 2021 were included. Cephalic index was measured from patient computed tomography scans, x-rays, or by caliper-based methods. Parietal bone thickness was determined from patient preoperative computed tomography. Stepwise multiple regression analysis, least absolute shrinkage and selection operator, and random forest machine learning methods were used to determine variables most predictive of changes in CI. RESULTS: One hundred twenty-four patients were included. Stepwise multiple regression analysis identified duration of spring placement ( P =0.007), anterior spring force ( P =0.034), and anterior spring length ( P =0.043) as statistically significant predictors for changes in CI. Least absolute shrinkage and selection operator analysis identified maximum spring force (ß=0.035), anterior spring length (ß=0.005), posterior spring length (ß=0.004), and duration of spring placement (ß=0.0008) as the most predictive variables for changes in CI. Random forest machine learning identified variables with greatest increase in mean squared error as maximum spring force (0.0101), anterior spring length (0.0090), and posterior spring length (0.0056). CONCLUSIONS: Maximum and total spring forces, anterior and posterior spring lengths, and duration of spring placement were the most predictive variables for changes in CI following SMC. Age at surgery and other demographic variables were inferior predictors in these models.


Assuntos
Craniossinostoses , Anormalidades Maxilomandibulares , Procedimentos de Cirurgia Plástica , Humanos , Lactente , Craniotomia/métodos , Procedimentos de Cirurgia Plástica/métodos , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Anormalidades Maxilomandibulares/cirurgia , Algoritmos , Aprendizado de Máquina , Estudos Retrospectivos
15.
Urol Case Rep ; 44: 102129, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-35800148

RESUMO

Spinal arachnoid cysts (SAC) are uncommon benign spinal cord lesions, particularly in children, that can result in a variety of neurologic symptoms, including neurogenic bladder. Here we present the case of a 7-year-old female with new onset, isolated urinary and stool incontinence who was found to have a T4-T7 SAC. Though this was initially believed to be an incidental imaging finding, after thorough work-up and persistence of her symptoms despite conservative measures she underwent neurosurgical intervention with complete resolution of incontinence. SAC represents a very rare but potentially reversible cause of neurogenic bladder that the urologist should be aware of.

16.
Childs Nerv Syst ; 38(6): 1105-1111, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35377009

RESUMO

PURPOSE: Approximately 1 to 2% of patients with Wilms' tumor (WT), or nephroblastoma, will have metastasis to the brain. Due to the rarity of intracranial metastasis, the clinical characteristics, prognosis, and a standardized treatment approach to this occurrence remain poorly understood. Here we review the surgical management and treatment outcome of WT patients with intracranial metastasis at our institution. METHODS: A retrospective chart review of patients with WT at the Children's Hospital of Philadelphia was performed from 2007 to 2021. Clinical characteristics, operative details, radiographic studies, pathology, and patient outcomes were collected and analyzed. RESULTS: A total of 3 patients with histologically confirmed intracranial metastatic disease from WT were identified with a mean age of 5.7 years (range 3-10 years). 2 of the 3 patients were male. The mean time from diagnosis of primary WT to development of central nervous system metastasis was 15.3 months. Both supratentorial (n = 3) and infratentorial (n = 1) sites of metastasis were observed. Surgical resection was performed, and gross total resection was achieved in all 3 patients. All cases had favorable histology with no anaplasia and received whole-brain irradiation and chemotherapy. Two of 3 patients had a good neurologic function at postoperative follow-up. One patient died from their disease 4 months after resection of the brain metastasis. CONCLUSION: In WT patients with limited systemic disease burden, the combination of surgery, chemotherapy, and radiotherapy may play a role in enhancing survival when intracranial metastasis is present, despite the perioperative risk associated with surgery.


Assuntos
Neoplasias Renais , Tumor de Wilms , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Masculino , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento , Tumor de Wilms/patologia , Tumor de Wilms/cirurgia
17.
Childs Nerv Syst ; 38(5): 893-901, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35192026

RESUMO

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis.


Assuntos
Craniossinostoses , Criança , Craniossinostoses/complicações , Craniossinostoses/psicologia , Craniossinostoses/cirurgia , Ossos Faciais , Humanos , Procedimentos Neurocirúrgicos , Suturas
18.
J Neurosurg Pediatr ; 29(4): 419-426, 2022 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-35090136

RESUMO

OBJECTIVE: Variables that can predict outcomes in patients with craniosynostosis, including bone thickness, are important for surgical decision-making, yet are incompletely understood. Recent studies have demonstrated relative risks and benefits of surgical techniques for correcting head shape in patients with nonsyndromic sagittal craniosynostosis. The purpose of this study was to characterize the relationships between parietal bone thickness and perioperative outcomes in patients who underwent spring-mediated cranioplasty (SMC) for nonsyndromic sagittal craniosynostosis. METHODS: Patients who underwent craniectomy and SMC for nonsyndromic sagittal craniosynostosis at a quaternary pediatric hospital between 2011 and 2021 were included. Parietal bone thickness was determined on patient preoperative CT at 27 suture-related points: at the suture line and at 0.5 cm, 1.0 cm, 1.5 cm, and 2.0 cm from the suture at the anterior parietal, midparietal, and posterior parietal bones. Preoperative skull thickness was compared with intraoperative blood loss, need for intraoperative transfusion, and hospital length of stay (LOS). RESULTS: Overall, 124 patients with a mean age at surgery ± SD of 3.59 ± 0.87 months and mean parietal bone thickness of 1.83 ± 0.38 mm were included in this study. Estimated blood loss (EBL) and EBL per kilogram were associated with parietal bone thickness 0.5 cm (ρ = 0.376, p < 0.001 and ρ = 0.331, p = 0.004; respectively) and 1.0 cm (ρ = 0.324, p = 0.007 and ρ = 0.245, p = 0.033; respectively) from the suture line. Patients with a thicker parietal bone 0.5 cm (OR 18.08, p = 0.007), 1.0 cm (OR 7.16, p = 0.031), and 1.5 cm (OR 7.24, p = 0.046) from the suture line were significantly more likely to have undergone transfusion when controlling for age, sex, and race. Additionally, parietal bone thickness was associated with hospital LOS (ß 0.575, p = 0.019) when controlling for age, sex, and race. Patient age at the time of surgery was not independently associated with these perioperative outcomes. CONCLUSIONS: Parietal bone thickness, but not age at the time of surgery, may predict perioperative outcomes including transfusion, EBL, and LOS. The need for transfusion and EBL were most significant for parietal bone thickness 0.5 cm to 1.5 cm from the suture line, within the anticipated area of suturectomy. For patients undergoing craniofacial surgery, parietal bone thickness may have important implications for anticipating the need for intraoperative transfusion and hospital LOS.


Assuntos
Craniossinostoses , Osso Parietal , Perda Sanguínea Cirúrgica , Criança , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Craniotomia/métodos , Humanos , Lactente , Osso Parietal/diagnóstico por imagem , Osso Parietal/cirurgia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Crânio/diagnóstico por imagem , Crânio/cirurgia , Resultado do Tratamento
19.
J Neurosurg Pediatr ; 29(1): 40-47, 2022 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-34598159

RESUMO

OBJECTIVE: Digital subtraction angiography (DSA) is commonly performed after pial synangiosis surgery for pediatric moyamoya disease to assess the degree of neovascularization. However, angiography is invasive, and the risk of ionizing radiation is a concern in children. In this study, the authors aimed to identify whether arterial spin labeling (ASL) can predict postoperative angiogram grading. In addition, they sought to determine whether patients who underwent ASL imaging without DSA had similar postoperative outcomes when compared with patients who received ASL imaging and postoperative DSA. METHODS: The medical records of pediatric patients who underwent pial synangiosis for moyamoya disease at a quaternary children's hospital were reviewed during a 10-year period. ASL-only and ASL+DSA cohorts were analyzed. The frequency of preoperative and postoperative symptoms was analyzed within each cohort. Three neuroradiologists assigned a visual ASL grade for each patient indicating the change from the preoperative to postoperative ASL perfusion sequences. A postoperative neovascularization grade was also assigned for patients who underwent DSA. RESULTS: Overall, 21 hemispheres of 14 patients with ASL only and 14 hemispheres of 8 patients with ASL+DSA were analyzed. The groups had similar rates of MRI evidence of acute or chronic stroke preoperatively (61.9% in the ASL-only group and 64.3% in the ASL+DSA group). In the entire cohort, transient ischemic attack (TIA) (p = 0.027), TIA composite (TIA or unexplained neurological symptoms; p = 0.0006), chronic headaches (p = 0.035), aphasia (p = 0.019), and weakness (p = 0.001) all had decreased frequency after intervention. The authors found a positive association between revascularization observed on DSA and the visual ASL grading (p = 0.048). The visual ASL grades in patients with an angiogram indicating robust neovascularization demonstrated improved perfusion when compared with the ASL grades of patients with a poor neovascularization. CONCLUSIONS: Noninvasive ASL perfusion imaging had an association with postoperative DSA neoangiogenesis following pial synangiosis surgery in children. There were no significant postoperative stroke differences between the ASL-only and ASL+DSA cohorts. Both cohorts demonstrated significant improvement in preoperative symptoms after surgery. Further study in larger cohorts is necessary to determine whether the results of this study are validated in order to circumvent the invasive catheter angiogram.


Assuntos
Angiografia Cerebral/métodos , Doença de Moyamoya/diagnóstico por imagem , Doença de Moyamoya/cirurgia , Neuroimagem/métodos , Imagem de Perfusão/métodos , Angiografia Digital , Revascularização Cerebral/métodos , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Masculino , Marcadores de Spin
20.
World Neurosurg ; 158: e196-e205, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34718196

RESUMO

BACKGROUND: Urgent neurosurgical interventions for pediatric patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are rare. These cases pose additional stress on a potentially vulnerable dysregulated inflammatory response that can place the child at risk of further clinical deterioration. Our aim was to describe the perioperative course of SARS-CoV-2-positive pediatric patients who had required an urgent neurosurgical intervention. METHODS: We retrospectively analyzed pediatric patients aged ≤18 years who had been admitted to a quaternary children's hospital with a positive polymerase chain reaction test result for SARS-CoV-2 virus from March 2020 to October 2021. The clinical characteristics, anesthetic and neurosurgical operative details, surgical outcomes, and non-neurological symptoms were collected and analyzed. RESULTS: We identified 8 SARS-CoV-2-positive patients with a mean age of 8.83 years (median, 8.5 years; range, 0.58-18 years). Of the 8 patients, 6 were male. All children had had mild or asymptomatic coronavirus disease 2109. The anesthetic and surgical courses for these patients were, overall, uncomplicated. All the patients had been admitted to a specialized isolation unit in the pediatric intensive care unit for cardiopulmonary and neurological monitoring. The use of increased protective personal equipment during anesthesia and surgery did not impede a successful neurosurgical operation. CONCLUSIONS: SARS-CoV-2-positive pediatric patients with minimal coronavirus disease 2019-related symptoms who require urgent neurosurgical interventions face unique challenges regarding their anesthetic status, operative delays due to SARS-CoV-2 polymerase chain reaction testing, and requirements for additional protective personal equipment. Despite these clinical challenges, the patients in our study had not experienced adverse postoperative consequences, and no healthcare professional involved in their care had contracted the virus.


Assuntos
COVID-19 , Procedimentos Neurocirúrgicos , Doenças Assintomáticas , Criança , Feminino , Pessoal de Saúde , Humanos , Masculino , Estudos Retrospectivos , SARS-CoV-2
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