Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
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To analyze the varied presentation and management of atlas assimilation with associated radiographic abnormalities in children in the MRI era METHODS: Database analysis of 313 children (less than 10 years) RESULTS: Atlas assimilation (AA) was associated with atlantoaxial dislocation in 12, abnormal skull base and Chiari I abnormality in 42, C2-C3 segmentation failure and instability and Chiari I abnormality in 74, and condylar hypoplasia and basilar invagination in 74. Proatlas segmentation failures were 54, atlantoaxial rotary dislocation in 26 with Goldenhar's syndrome, abnormal C1 atlas posterior arch causing dynamic compression of cord in 31 children. Vascular compromise was documented in 26 children. The study encompassed ages 6 months to 10 years. Cranial nerves commonly affected were glossopharyngeal, vagal, and hypoglossal nerves. Children below 2 years presented with torticollis, failure to thrive, difficulty swallowing, and motor and sensory deficits. Craniovertebral junction instability associated with AA was treated with custom-built craniocervical orthosis below 5 years. Closed reduction of instability or basilar invagination was attempted with neuromuscular blockade under anesthesia and traction above age 5 years. Successful reduction was treated with dorsal foramen magnum and atlas decompression with occiput-C2 dorsal fusion using rib grafts below the age of 5 years and instrumentation after that. Follow-up was 2 to 32 years. Neurological recovery was seen in nearly all patients. ⋯ Children with atlas assimilation and associated abnormalities may be symptomatic in early childhood. The treatment depends on the age and tailored to the abnormalities present. The long-term results have been successful.
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Review Case Reports
The rolling cyst: migrating intraventricular neurocysticercosis-a case-based update.
Neurocysticercosis is the most frequent parasitic disease of the central nervous system, and its incidence in the developed countries is increasing due to immigration and travels from endemic areas. The intraventricular location has been found to involve up to 61.3% of the patients; moreover, only 22 cases of migrating intraventricular cyst have been reported so far. Despite the rarity of the condition in western countries, its occurrence generates some concerns and the aim of this paper is to update the information concerning pathogenesis, clinical presentation, diagnosis and management of this entity. ⋯ Migrating intraventricular neurocysticercosis is a pathognomonic entity usually presenting with hydrocephalus, and its treatment is mainly surgical, preferring an endoscopic approach. When the resection of the intraventricular cyst is not performed, an accurate follow-up is mandatory to detect clinical changes due to a recurrent hydrocephalus or to the effect of the dying cyst on the surrounding area. In case of permanent shunt placement, the cysticidal and steroid treatment is recommended to reduce the risk of shunt failure.
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Fibrocartilaginous embolism (FCE) is a rare cause of pediatric ischemic myelopathy. The pathology is thought to result from fragmentation with embolization into the microvasculature of the radicular artery often secondary to high axial force. While most cases arise in the setting of vigorous activity, our case reveals that FCE can also occur during relative physical inactivity. Additionally, while a majority of cases are associated with neck or back pain, our case also reveals that FCE can present without concurrent pain episodes. We describe a rare case of spinal cord infarction (SCI) likely due to FCE in a 14 year old male. Our patient was sitting with his feet elevated, playing a video game, when he developed sudden difficulty moving his arms. Initially presenting with a negative MRI scan and la belle indifference, our patient was suspected to exhibit functional quadraparesis secondary to psychosomatization/adjustment disorder. Repeat MR imaging 7 days later revealed typical findings for FCE with SCI (irregular, pencil-like T2 hyperintensity in the ventral cervical/upper thoracic cord and owl's eye pattern on axial images). Six months later, the diagnosis of FCE remains predominant. Our patient continues to improve with occupational and physical therapy. Ambulatory efforts and bladder function continue to progress. To improve functional gains, the patient is being considered for a chemodenervation procedure. ⋯ Our case reveals that FCE can occur during physical inactivity and present without concurrent pain. Outcome regarding pediatric fibrocartilaginous embolism is highly variant; however, the two largest outcomes reported were either patient death or discharge.
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The assessment of intracranial pressure (ICP) is essential in the management of neurocritical care paediatric patients. The gold standard for invasive ICP is an intraventricular catheter or intraparenchymal microsensor but is invasive and carries some risks. Therefore, a non-invasive method for measuring ICP (nICP) would be desirable especially in the paediatric population. The aim of this study is to assess the relationship between ICP and different ultrasound-based methods in neurocritical care paediatric patients. ⋯ Our preliminary results suggested that ONSD ultrasonography presents the best accuracy to assess ICP among the methods studied. Given its non-invasiveness, repeatability and safety, this technique has the potential of representing a valid option as non-invasive tool to assess the risk of intracranial hypertension in the paediatric population.
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Although the dismal clinical prognosis of diffuse intrinsic pontine glioma (DIPG) has not changed, there has been significant progress in the academic literature made in the biological understanding of this brainstem tumor. The aim of this analysis was to evaluate citation and other bibliometric characteristics of the 100 most-cited DIPG articles in the current literature in order to better understand the current state of our academic efforts in this area. ⋯ The 100 most-cited articles about DIPG were characterized in this analysis. Although smaller in overall proportion, BSc articles demonstrated significantly increased bibliometric parameters, supporting the recent dominance of BSc in this field, primarily involving histone biology of the H3K27M mutation. Moving forward, it will be of great interest to see how the findings of these high-impact BSc articles will translate into future high-impact CL articles.