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World Neurosurgery Jun 2023Giant cavernous malformations (GCMs) are rare and poorly characterized neurovascular lesions in adults or children and often misclassified. In this study, we provide a... (Review)
Review
BACKGROUND
Giant cavernous malformations (GCMs) are rare and poorly characterized neurovascular lesions in adults or children and often misclassified. In this study, we provide a review of pediatric GCM cases to highlight this rare entity as an important differential diagnosis in preoperative assessment.
METHODS
We report a pediatric case of GCM that presented as an intracerebral, periventricular, and infiltrative mass lesion. We performed a systematic review of published literature describing cases of GCM in children using the PubMed, Embase, and Cochrane Library databases. Studies describing cerebral or spinal cavernous malformation >4 cm were included. Demographic, clinical, radiographic, and outcome data were extracted.
RESULTS
Thirty-eight studies accounting for 61 patients were reviewed. most patients were 1-10 years old and 55.73% were male. Average lesion sizes ranged between 4 and 6 cm (40.98% >6 cm; 8.19% >10 cm). Supratentorial localization was most common (75.40%), with frontal and parieto-occipital regions being frequent localizations. Infratentorial lesions (24.60%) were located within the cerebellum (16.39%) and brainstem (8.19%). One case of spinal cavernoma was found. The main clinical manifestations were seizures (44.26%), focal neurologic deficit (36.06%), and headache (22.95%). Imaging showed contrast enhancement (36.06%), cystic features (27.86%), and infiltrative growth pattern (4.91%).
CONCLUSIONS
GCMs show variable clinical and radiologic features, representing a diagnostic challenge for treating surgeons. Imaging may show various tumorlike features such as cystic or infiltrative patterns with contrast enhancement. The existence of GCM should be considered preoperatively. Gross total resection should be attempted whenever possible, because it correlates with a good recovery and long-term outcomes. Also, a clear definition criteria of when a cerebral cavernous malformation is termed giant should be established.
Topics: Adult; Humans; Child; Male; Infant; Child, Preschool; Female; Brain Neoplasms; Hemangioma, Cavernous, Central Nervous System; Hemangioma, Cavernous; Cerebellum; Diagnosis, Differential
PubMed: 36889633
DOI: 10.1016/j.wneu.2023.02.135 -
World Neurosurgery Feb 2024Intracranial dermoid cyst (DC) is a rare benign, slow-growing lesion, most commonly arising along the midline. They can occur in the supratentorial compartment, very... (Review)
Review
OBJECTIVE
Intracranial dermoid cyst (DC) is a rare benign, slow-growing lesion, most commonly arising along the midline. They can occur in the supratentorial compartment, very rarely involve the sellar region and only exceptionally are intrasellar. The aim of our study is to address the challenges in the diagnosis and management of sellar DCs.
METHODS
We performed a systematic review of sellar DCs, in keeping with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, and described an intrasellar DC in a 32-year-old female who presented with bilateral blurring vision.
RESULTS
The review identified 4 intrasellar, 29 suprasellar, and 28 parasellar cases. Intrasellar DCs more likely present with progressive visual impairment and pituitary hormone dysfunctions during the fifth decade of life. Suprasellar and parasellar DCs are typically diagnosed during the third decade of life because of diplopia, ptosis, trigeminal hypoaesthesia/para-esthesia or cyst's rupture. Sellar DCs are typically hypodense on computed tomography scans and contain calcifications. Magnetic resonance imaging features include T1 hyperintensity, T2 heterogeneous intensity, no restriction on diffusion-weighted images, and no contrast enhancement. Surgery is the treatment of choice. Gross total resection is achieved in 60% of intrasellar and 61.9% of suprasellar and parasellar DCs. Early postoperative complications are reported in 40.0%, 16.7%, and 23.8% of intrasellar, suprasellar, and parasellar DCs, respectively.
CONCLUSIONS
Intrasellar DCs are rare lesions typically diagnosed later than suprasellar and parasellar DCs due to their different clinical presentations. However, they should be considered in the differential diagnosis of cystic lesions of the sella, including epidermoid cysts, craniopharyngiomas, Rathke's cleft cysts, and teratomas.
Topics: Female; Humans; Adult; Dermoid Cyst; Pituitary Neoplasms; Central Nervous System Cysts; Epidermal Cyst; Magnetic Resonance Imaging; Sella Turcica
PubMed: 37995988
DOI: 10.1016/j.wneu.2023.11.057 -
Acta Neurochirurgica Nov 2016Aneurysms associated with brain arteriovenous malformations (bAVMs) influence the natural history of these lesions and pose important therapeutic challenges. However,... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Aneurysms associated with brain arteriovenous malformations (bAVMs) influence the natural history of these lesions and pose important therapeutic challenges. However, the epidemiology, natural history, and appropriate management of the aneurysms associated with bAVMs are not completely understood due to the paucity of large and uniform studies. We performed a systematic review of published series examining the association between aneurysms and bAVMs with the purpose of clarifying the prevalence, risk of hemorrhage, and appropriate management of these lesions.
METHOD
PRISMA/MOOSE guidelines were followed. We conducted a comprehensive literature search of three databases (PubMed, Ovid MEDLINE, and Ovid EMBASE) on aneurysms associated with bAVMs. Only studies examining consecutive case series of aneurysms associated with bAVMs were included. From the collected studies, we extracted data regarding prevalence of bAVM-associated aneurysms, risk of aneurysm rupture in relation to bAVM location and aneurysm characteristics, and treatment-related outcomes.
RESULTS
Our systematic review included 44 articles with a total of 10,093 bAVMs. The proportion of bAVMs with an associated aneurysm was 20.2 % (95 % CI = 19.4-20.9 %). Among ruptured bAVMs with associated aneurysms, the aneurysm was the source of hemorrhage in 49.2 % (95 % CI = 43.7-54.7 %) of cases. Flow-related aneurysms were the most common source of aneurysm rupture (78.5 %, 95 % CI = 70.6-84.9 %). Infratentorial bAVM-associated aneurysms presented a higher risk of rupture (60 %, 95 % CI = 47.4-71.9 %) when compared with supratentorial lesions (29 %, 95 % CI = 21.4-38.5 %). Endovascular treatment of aneurysms associated with bAVMs had a cure rate of 80.0 % (95 % CI = 73.3-85.3 %), complication rate of 8.7 % (95 % CI = 5.5-13.1 %), and a good neurological outcome rate of 78.8 % (95 % CI = 72.5-83.9 %).
CONCLUSIONS
Twenty percent of bAVMs harbored arterial aneurysms. The presence of aneurysm increases the risk of bleeding of the bAVM, especially when flow-related or infratentorially located. Aneurysms associated with bAVMs should be treated promptly. Selective endovascular treatment of bAVM-associated aneurysms appears safe and effective.
Topics: Aneurysm, Ruptured; Humans; Intracranial Aneurysm; Intracranial Arteriovenous Malformations
PubMed: 27644700
DOI: 10.1007/s00701-016-2957-3 -
Cerebellum (London, England) Apr 2024The term Pontocerebellar Hypoplasia (PCH) was initially used to designate a heterogeneous group of fetal-onset genetic neurodegenerative disorders. As a descriptive...
The term Pontocerebellar Hypoplasia (PCH) was initially used to designate a heterogeneous group of fetal-onset genetic neurodegenerative disorders. As a descriptive term, PCH refers to pons and cerebellum of reduced volume. In addition to the classic PCH types described in OMIM, many other disorders can result in a similar imaging appearance. This study aims to review imaging, clinical and genetic features and underlying etiologies of a cohort of children with PCH on imaging. We systematically reviewed brain images and clinical charts of 38 patients with radiologic evidence of PCH. Our cohort included 21 males and 17 females, with ages ranging between 8 days to 15 years. All individuals had pons and cerebellar vermis hypoplasia, and 63% had cerebellar hemisphere hypoplasia. Supratentorial anomalies were found in 71%. An underlying etiology was identified in 68% and included chromosomal (21%), monogenic (34%) and acquired (13%) causes. Only one patient had pathogenic variants in an OMIM listed PCH gene. Outcomes were poor regardless of etiology, though no one had regression. Approximately one third of patients deceased at a median age of 8 months. All individuals had global developmental delay, 50% were non-verbal, 64% were non-ambulatory and 45% required gastrostomy feeding. This cohort demonstrates that radiologic PCH has heterogenous etiologies and the "classic" OMIM-listed PCH genes underlie only a minority of cases. Broad genetic testing, including chromosomal microarray and exome or multigene panels, is recommended in individuals with PCH-like imaging appearance. Our results strongly suggest that the term PCH should be used to designate radiologic findings, and not to imply neurogenerative disorders.
Topics: Male; Child; Female; Humans; Infant; Cerebellar Diseases; Cerebellum; Pons; Magnetic Resonance Imaging; Developmental Disabilities; Nervous System Malformations
PubMed: 36971923
DOI: 10.1007/s12311-023-01544-2 -
Acta Neurochirurgica Apr 2020Posterior fossa arteriovenous malformations (pfAVMs) can be challenging lesions to manage. AVMs in this location may have distinct features compared with supratentorial... (Comparative Study)
Comparative Study Meta-Analysis
BACKGROUND
Posterior fossa arteriovenous malformations (pfAVMs) can be challenging lesions to manage. AVMs in this location may have distinct features compared with supratentorial AVMs. Our aim was to systematically review the literature on the presenting characteristics of pfAVMs and compare clinical and angiographic outcomes after the various types of treatment employed.
METHODS
The review was conducted according to the Cochrane Collaboration guidelines. Electronic databases from 1900 to March 2018 were searched and complemented by hand-searching and cross-referencing. Articles were categorized into (i) AVM studies that included those in the posterior fossa, (ii) those that focused exclusively on pfAVM, and (iii) those that further specified a cerebellar or brainstem location of the AVM.
RESULTS
Seventy-seven articles with 4512 pfAVM patients were retained for analyses. Compared with historical supratentorial controls, pfAVMs were reported to more frequently present with rupture, to more commonly have associated arterial aneurysms, and to more frequently lead to poor clinical and angiographic outcomes. The quality of the literature and lack of standardization of outcome reporting precluded performing a meta-analysis on the results of the various different treatment modalities.
CONCLUSIONS
Posterior fossa AVMs may have some distinct features compared with supratentorial AVMs. The available reports on pfAVMs are not sufficiently standardized to provide reliable guidance for patient management decisions. This goal will require future studies to be multicentric and to focus on standardized, repeatable clinical and angiographic outcomes.
Topics: Adult; Cranial Fossa, Posterior; Female; Humans; Intracranial Arteriovenous Malformations; Male; Middle Aged; Neurosurgical Procedures; Postoperative Complications
PubMed: 32067118
DOI: 10.1007/s00701-020-04260-6 -
Prenatal Diagnosis Jan 2021Posterior fossa abnormalities (PFAs) are commonly identified within routine screening and are a frequent indication for fetal magnetic resonance imaging (MRI). Although...
BACKGROUND
Posterior fossa abnormalities (PFAs) are commonly identified within routine screening and are a frequent indication for fetal magnetic resonance imaging (MRI). Although biometric measurements of the posterior fossa (PF) are established on fetal ultrasound and MRI, qualitative visual assessments are predominantly used to differentiate PFAs.
OBJECTIVES
This systematic review aimed to assess 2-dimensional (2D) biometric measurements currently in use for assessing the PF on fetal MRI to delineate different PFAs.
METHODS
The protocol was registered (PROSPERO ID CRD42019142162). Eligible studies included T2-weighted MRI PF measurements in fetuses with and without PFAs, including measurements of the PF, or other brain areas relevant to PFAs.
RESULTS
59 studies were included - 6859 fetuses had 62 2D PF and related measurements. These included linear, area and angular measurements, representing measures of PF size, cerebellum/vermis, brainstem, and supratentorial measurements. 11 measurements were used in 10 or more studies and at least 1200 fetuses. These dimensions were used to characterise normal for gestational age, diagnose a range of pathologies, and predict outcome.
CONCLUSION
A selection of validated 2D biometric measurements of the PF on fetal MRI may be useful for identification of PFA in different clinical settings. Consistent use of these measures, both clinically and for research, is recommended.
Topics: Biometry; Brain Stem; Cerebellum; Cranial Fossa, Posterior; Female; Fetus; Humans; Magnetic Resonance Imaging; Organ Size; Pregnancy; Ultrasonography, Prenatal
PubMed: 33251640
DOI: 10.1002/pd.5874 -
The British Journal of Radiology Sep 2021Blood flow is the rate of blood movement and relevant to numerous processes, though understudied in gliomas. The aim of this review was to pool blood flow metrics...
OBJECTIVE
Blood flow is the rate of blood movement and relevant to numerous processes, though understudied in gliomas. The aim of this review was to pool blood flow metrics obtained from MRI modalities in adult supratentorial gliomas.
METHODS
MEDLINE, EMBASE and the Cochrane database were queried 01/01/2000-31/12/2019. Studies measuring blood flow in adult Grade II-IV supratentorial gliomas using dynamic susceptibility contrast (DSC) MRI, dynamic contrast enhanced MRI (DCE-MRI) or arterial spin labelling (ASL) were included. Absolute and relative cerebral blood flow (CBF), peritumoral blood flow and tumoral blood flow (TBF) were reported.
RESULTS
34 studies were included with 1415 patients and 1460 scans. The mean age was 52.4 ± 7.3 years. Most patients had glioblastoma ( = 880, 64.6%). The most common imaging modality was ASL ( = 765, 52.4%) followed by DSC ( = 538, 36.8%). Most studies were performed pre-operatively ( = 1268, 86.8%). With increasing glioma grade (II IV), TBF increased (70.8 145.5 ml/100 g/min, < 0.001) and CBF decreased (85.3 49.6 ml/100 g/min, < 0.001). In Grade IV gliomas, following treatment, CBF increased in ipsilateral (24.9 ± 1.2 26.1 ± 0.0 ml/100 g/min, < 0.001) and contralateral white matter (25.6 ± 0.2 26.0± 0.0 ml/100 g/min, < 0.001).
CONCLUSION
Our findings demonstrate that increased mass effect from high-grade gliomas impairs blood flow within the surrounding brain that can improve with surgery.
ADVANCES IN KNOWLEDGE
This systematic review demonstrates how mass effect from brain tumours impairs blood flow in the surrounding brain parenchyma that can improve with treatment.
Topics: Adult; Brain; Brain Neoplasms; Cerebrovascular Circulation; Glioma; Humans; Magnetic Resonance Imaging; Neoplasm Grading
PubMed: 34106749
DOI: 10.1259/bjr.20201450 -
Topics in Stroke Rehabilitation Apr 2023Lateropulsion with active Pushing (LwP) is characterized by impairments in postural control. Previous research suggests an association between LwP, lesion location and...
BACKGROUND
Lateropulsion with active Pushing (LwP) is characterized by impairments in postural control. Previous research suggests an association between LwP, lesion location and verticality misperception. This first-ever systematic review evaluates the association between LwP, lesion location and the perception of verticality (PROSPERO: CRD42020159248).
METHODS
PubMed, Web of Science, REHABDATA, Embase, Cochrane Library and PEDro were systematically searched on December 16, 2021. Studies were included when examining lesion location or perception of verticality (Subjective Haptic, Visual or Postural Vertical) in supratentorial stroke patients showing LwP. Two reviewers independently screened and assessed risk of bias using the Newcastle Ottawa Scale. Data were qualitatively analyzed and extracted.
RESULTS
Nineteen studies were included, examining a total of 340 LwP patients. Lesions in: the thalamus, internal capsule, inferior parietal lobule at the junction of the postcentral gyrus, the posterior insula and the superior temporal gyrus, were associated with LwP. Whereas all studies examining the Subjective Postural and Haptic Vertical (haptic only examined once) reported a significant increased deviation in LwP patients, inconsistent results were found for the Subjective Visual Vertical. Furthermore, the Subjective Visual and Postural Vertical showed inconsistent results for magnitude, direction and variability of this deviation.
DISCUSSION
A complex brain network, rather than only one brain region, seems responsible for body control with respect to gravity. A disruption within this network might lead to a bias in the construction of a correct internal reference frame, crucial for perceiving verticality. There was an association of LwP with verticality misperception in all three modalities.
Topics: Humans; Visual Perception; Stroke; Space Perception; Postural Balance; Internal Capsule
PubMed: 35102816
DOI: 10.1080/10749357.2022.2026563 -
Neurological Sciences : Official... Jul 2022Intradural disc herniation (IDH) can manifest with radicular or medullary syndrome. In about 15% of cases, IDH may be responsible, through a dural laceration, for a CSF... (Review)
Review
Diagnostic approach, therapeutic strategies, and surgical indications in intradural thoracic disc herniation associated with CSF leak, intracranial hypotension, and CNS superficial siderosis.
BACKGROUND AND PURPOSE
Intradural disc herniation (IDH) can manifest with radicular or medullary syndrome. In about 15% of cases, IDH may be responsible, through a dural laceration, for a CSF leak, determining spontaneous intracranial hypotension (SIH) and CNS superficial siderosis (CNSss). This paper attempts to present an overview on IDH as the cause for both CSF leak, and subsequent SIH, and CNSss, and to describe a peculiar clinical and neuroradiological scenario related to this condition.
METHODS
A search on the PUBMED database was performed. Although the investigation did not rigorously follow the criteria for a systematic review (we consider only articles about thoracic IDH), nonetheless, the best quality evidence was pursued. Furthermore, an illustrative case was presented.
RESULTS
A 69-year-old woman was referred to our hospital for slowly progressive gait disturbances and hearing impairment. Brain imaging revealed diffuse bilateral supratentorial and infratentorial superficial siderosis, mostly of the cerebellum, the eighth cranial nerves, and the brainstem. Spinal imaging disclosed a posterior disc herniation determining a dural tear at D6-D7. Lumbar puncture revealed low opening pressure and hemorrhagic CSF with siderophages. A posterior transdural herniectomy and dural sealing determined a stabilization of hearing and a significant improvement in both gait and balance.
CONCLUSIONS
The diagnostic workup of CNSss with suspected CNS leak demands whole neuraxis imaging, especially in cases presenting SIH or myelopathic symptoms. This may avoid delays in detection of IDH and spinal dural leaks. The different forms of treatment available depend on the type and severity of the clinical picture.
Topics: Aged; Cerebrospinal Fluid Leak; Female; Humans; Intervertebral Disc Displacement; Intracranial Hypotension; Magnetic Resonance Imaging; Siderosis; Thoracic Vertebrae
PubMed: 35396636
DOI: 10.1007/s10072-022-06059-y -
Neurosurgical Review Jun 2021There is a lack of class I evidence concerning the impact of surgery in the treatment of diffuse low-grade glioma; the early maximal resection with preservation of... (Meta-Analysis)
Meta-Analysis
There is a lack of class I evidence concerning the impact of surgery in the treatment of diffuse low-grade glioma; the early maximal resection with preservation of eloquent brain areas has been accepted as the first therapeutic option. We performed a systematic review of the literature using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and protocol. Inclusion criteria: only case series with at least 100 patients containing supratentorial hemispheric diffuse low-grade glioma (according to any of the WHO classification used in papers published between 2000 to 2019), with pre- and postoperative MRI study were included in the qualitative and quantitative analyses. The extent of resection should be defined based on MRI at least in two categories and correlated with patients' outcomes (with univariate or multivariate analyses) using overall survival (OS) or malignant progression-free survival (MPFS). A total of 18 series with 4386 patients, published in 20 papers, were included in this systematic review. All the series that evaluates the relation between the extent of resection (EOR) and OS showed a statistically significant improvement of OS at univariate and/or multivariate analyzes with a greater EOR. Six studies showed a statistically significant improvement of MPFS with a greater EOR. We demonstrate that when a more rigorous analysis of EOR is performed, a benefit of a more aggressive resection on OS and MPFS is observed. Our review about EOR in different molecular groups of DLGG also suggests a benefit of maximum safe resection for all different subtypes, even though "radical surgery" may be associated with better OS and MPFS in tumors with a more aggressive signature.
Topics: Biomarkers, Tumor; Brain Neoplasms; Glioma; Humans; Magnetic Resonance Imaging; Neoplasm Grading; Neurosurgical Procedures; Prospective Studies; Retrospective Studies; Treatment Outcome
PubMed: 32770298
DOI: 10.1007/s10143-020-01362-8