Introduction: Pediatric hydrocephalus is a common disease in sub-Saharan Africa. In Mali, 350-400 new cases are diagnosed in our center yearly. With a total land mass of 1,241,000 km2, patients in remote areas must travel up to 1,500 km to access neurosurgical care. Hence, treatment and follow-ups of "shunted" patients are difficult. In this context, endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) provides an opportunity for an affordable and less constraining treatment for hydrocephalus children under 12 months of age.
Methods: We performed a retrospective analysis of ETV/CPC performed on infants from July 2013 to January 2015. Patients were followed postoperatively on day 15, month 6, and month 12. Statistical analysis was conducted using Prism 9 GraphPad software. ETV successes were categorized according to the patient's age into 3 groups: ≤3 months, 3-6 months, and 6-12 months. Statistical significance was defined at p < 0.05.
Results: During the study period, 199 patients were included with 40% of patients aged between 0 and 6 months. The head circumference ranged from 35 cm to 79 cm. The etiology was congenital malformation in 55%. ETV/CPC was a success in 69% of 6- to 12-month-old patients, 54% in the 3- to 6-month-old patients, and 29% in ≤3-month-old patients. Overall, 94 (47%) patients were successfully treated without a shunt. The postoperative infection rate was 1% and mortality at 12 months was 8%.
Conclusion: In a low-income environment such as Mali, ETV/CPC stands as a viable and alternative treatment option for pediatric hydrocephalus patients; our findings suggest that age is an important factor in predicting ETV success.
{"title":"Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization in Infantile Hydrocephalus: An Experience from Mali.","authors":"Oumar Diallo, Mahamadou Dama, Landry Konan, Oumar Coulibaly, Daouda Sissoko, Abdoulaye Hima Maiga","doi":"10.1159/000529453","DOIUrl":"https://doi.org/10.1159/000529453","url":null,"abstract":"<p><strong>Introduction: </strong>Pediatric hydrocephalus is a common disease in sub-Saharan Africa. In Mali, 350-400 new cases are diagnosed in our center yearly. With a total land mass of 1,241,000 km2, patients in remote areas must travel up to 1,500 km to access neurosurgical care. Hence, treatment and follow-ups of \"shunted\" patients are difficult. In this context, endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) provides an opportunity for an affordable and less constraining treatment for hydrocephalus children under 12 months of age.</p><p><strong>Methods: </strong>We performed a retrospective analysis of ETV/CPC performed on infants from July 2013 to January 2015. Patients were followed postoperatively on day 15, month 6, and month 12. Statistical analysis was conducted using Prism 9 GraphPad software. ETV successes were categorized according to the patient's age into 3 groups: ≤3 months, 3-6 months, and 6-12 months. Statistical significance was defined at p < 0.05.</p><p><strong>Results: </strong>During the study period, 199 patients were included with 40% of patients aged between 0 and 6 months. The head circumference ranged from 35 cm to 79 cm. The etiology was congenital malformation in 55%. ETV/CPC was a success in 69% of 6- to 12-month-old patients, 54% in the 3- to 6-month-old patients, and 29% in ≤3-month-old patients. Overall, 94 (47%) patients were successfully treated without a shunt. The postoperative infection rate was 1% and mortality at 12 months was 8%.</p><p><strong>Conclusion: </strong>In a low-income environment such as Mali, ETV/CPC stands as a viable and alternative treatment option for pediatric hydrocephalus patients; our findings suggest that age is an important factor in predicting ETV success.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10064391/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9581307","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction: Most people who are infected with the novel severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) are asymptomatic or present with mild upper respiratory symptoms. This is especially true in the pediatric population; however, rarely, a massive cytokine storm can develop, causing multisystem inflammatory syndrome associated with COVID (MIS-C). Furthermore, children may also suffer from acute ischemic strokes secondary to SARS-CoV-2 infection. Case Presentation: Here, we present a 2-year-old male who was admitted to the hospital with MIS-C and evidence of a previous SARS-CoV-2 infection. On postadmission day 2, the patient was in cardiogenic shock, had acute kidney injury, liver dysfunction, and metabolic acidosis. He had concurrent altered mental status, and his computed tomography scan showed ischemic infarcts in the territory of the right middle cerebral artery and superior cerebellar artery bilaterally. Magnetic resonance angiography confirmed occlusion of the right middle cerebral artery and right superior cerebellar artery. He underwent an emergent decompressive craniectomy due to rapid deterioration and cerebral edema. After the procedure, he continued to improve and was discharged with moderate disability that improved during outpatient rehab. Conclusion: Though rare in children, SARS-CoV-2 can lead to AIS, especially in the presence of underlying risk factors such as MIS-C and hypercoagulopathy. AIS can be associated with severe mortality and morbidity; however, even in this severe case of AIS, the patient was successfully treated with a decompressive craniectomy.
{"title":"Post-COVID-19 Multisystem Inflammatory Syndrome-Related Cerebral Infarction in a Pediatric Patient Managed with Decompressive Craniectomy.","authors":"Ryan D Morgan, Reagan A Collins, Laszlo Nagy","doi":"10.1159/000529682","DOIUrl":"https://doi.org/10.1159/000529682","url":null,"abstract":"Introduction: Most people who are infected with the novel severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) are asymptomatic or present with mild upper respiratory symptoms. This is especially true in the pediatric population; however, rarely, a massive cytokine storm can develop, causing multisystem inflammatory syndrome associated with COVID (MIS-C). Furthermore, children may also suffer from acute ischemic strokes secondary to SARS-CoV-2 infection. Case Presentation: Here, we present a 2-year-old male who was admitted to the hospital with MIS-C and evidence of a previous SARS-CoV-2 infection. On postadmission day 2, the patient was in cardiogenic shock, had acute kidney injury, liver dysfunction, and metabolic acidosis. He had concurrent altered mental status, and his computed tomography scan showed ischemic infarcts in the territory of the right middle cerebral artery and superior cerebellar artery bilaterally. Magnetic resonance angiography confirmed occlusion of the right middle cerebral artery and right superior cerebellar artery. He underwent an emergent decompressive craniectomy due to rapid deterioration and cerebral edema. After the procedure, he continued to improve and was discharged with moderate disability that improved during outpatient rehab. Conclusion: Though rare in children, SARS-CoV-2 can lead to AIS, especially in the presence of underlying risk factors such as MIS-C and hypercoagulopathy. AIS can be associated with severe mortality and morbidity; however, even in this severe case of AIS, the patient was successfully treated with a decompressive craniectomy.","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9529085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nadjib Belfatmi, Ahmed S Motawei, Ayoub Rezkallah, Nour Djihane Benarous, Adnan Hassan Ahmed, Sherief Ghozy, Mohamed A Zaazoue, Mohamed Si Saber
Introduction: Myelomeningocele (MMC) is a malformation resulting from the neural tube's failure to close during embryonic development, and the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine; however, multiple NTDs (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature.
Case presentation: We report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication.
Conclusion: Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode, and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.
{"title":"Double Lumbar Localization of Myelomeningocele: Case Report.","authors":"Nadjib Belfatmi, Ahmed S Motawei, Ayoub Rezkallah, Nour Djihane Benarous, Adnan Hassan Ahmed, Sherief Ghozy, Mohamed A Zaazoue, Mohamed Si Saber","doi":"10.1159/000530726","DOIUrl":"https://doi.org/10.1159/000530726","url":null,"abstract":"<p><strong>Introduction: </strong>Myelomeningocele (MMC) is a malformation resulting from the neural tube's failure to close during embryonic development, and the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine; however, multiple NTDs (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature.</p><p><strong>Case presentation: </strong>We report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication.</p><p><strong>Conclusion: </strong>Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode, and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9741025","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01Epub Date: 2023-09-27DOI: 10.1159/000534283
Nir Shimony, Lissa Baird, Moise Danielpour, George I Jallo
{"title":"Contemporary Knowledge Update of Pediatric Neuro-Oncology Management: An Overview for Neurosurgeons.","authors":"Nir Shimony, Lissa Baird, Moise Danielpour, George I Jallo","doi":"10.1159/000534283","DOIUrl":"10.1159/000534283","url":null,"abstract":"","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41161062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01Epub Date: 2023-05-26DOI: 10.1159/000531256
Tom Rosenberg, Tabitha Cooney
Background: Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children. Even with intensive multimodal treatment, the prognosis of many types is guarded, and treatment-related toxicity is significant. Recent advances in molecular diagnostics allowed the discovery of novel entities and inter-tumor subgroups, with opportunities for improved risk-stratification and treatment approaches.
Summary: Medulloblastomas separate into four distinct subgroups with distinct clinicopathologic characteristics, and data from recent clinical trials for newly diagnosed medulloblastoma support subgroup-specific treatment approaches. Atypical teratoid rhabdoid tumor (ATRT), embryonal tumor with multilayered rosettes (ETMR), and pineoblastoma, as well as other rare embryonal tumors, can be distinguished from histologically similar tumors by virtue of characteristic molecular findings, with DNA methylation analysis providing a strong adjunct in indeterminate cases. Methylation analysis can also allow further subgrouping of ATRT and pineoblastoma. Despite the dire need to improve outcomes for patients with these tumors, their rarity and lack of actionable targets lead to a paucity of clinical trials and novel therapeutics.
Key messages: (1) Embryonal tumors can be accurately diagnosed with pediatric-specific sequencing techniques. (2) Medulloblastoma risk stratification and treatment decisions should take into account molecular subgroups. (3) There is a dire need for a novel collaborative clinical trial design to improve outcomes is rare pediatric embryonal tumors.
{"title":"Current Open Trials and Molecular Update for Pediatric Embryonal Tumors.","authors":"Tom Rosenberg, Tabitha Cooney","doi":"10.1159/000531256","DOIUrl":"10.1159/000531256","url":null,"abstract":"<p><strong>Background: </strong>Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children. Even with intensive multimodal treatment, the prognosis of many types is guarded, and treatment-related toxicity is significant. Recent advances in molecular diagnostics allowed the discovery of novel entities and inter-tumor subgroups, with opportunities for improved risk-stratification and treatment approaches.</p><p><strong>Summary: </strong>Medulloblastomas separate into four distinct subgroups with distinct clinicopathologic characteristics, and data from recent clinical trials for newly diagnosed medulloblastoma support subgroup-specific treatment approaches. Atypical teratoid rhabdoid tumor (ATRT), embryonal tumor with multilayered rosettes (ETMR), and pineoblastoma, as well as other rare embryonal tumors, can be distinguished from histologically similar tumors by virtue of characteristic molecular findings, with DNA methylation analysis providing a strong adjunct in indeterminate cases. Methylation analysis can also allow further subgrouping of ATRT and pineoblastoma. Despite the dire need to improve outcomes for patients with these tumors, their rarity and lack of actionable targets lead to a paucity of clinical trials and novel therapeutics.</p><p><strong>Key messages: </strong>(1) Embryonal tumors can be accurately diagnosed with pediatric-specific sequencing techniques. (2) Medulloblastoma risk stratification and treatment decisions should take into account molecular subgroups. (3) There is a dire need for a novel collaborative clinical trial design to improve outcomes is rare pediatric embryonal tumors.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9532785","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01Epub Date: 2023-08-21DOI: 10.1159/000531998
Thierry A G M Huisman, Rajan Patel, Stephen Kralik, Nilesh K Desai, Avner Meoded, Karen Chen, Howard L Weiner, Daniel J Curry, Maarten Lequin, Mariette Kranendonk, Gunes Orman, George Jallo
Background: Neuroimaging has evolved from anatomical imaging toward a multi-modality comprehensive anatomical and functional imaging in the past decades, important functional data like perfusion-weighted imaging, permeability imaging, diffusion-weighted imaging (DWI), and diffusion tensor imaging (DTI), tractography, metabolic imaging, connectomics, event-related functional imaging, resting state functional imaging, and much more is now being offered.
Summary: Precision diagnostics has proven to be essential for precision treatment. Many minimal invasive techniques have been developed, taking advantage of digital subtraction angiography and interventional neuroradiology. Furthermore, intraoperative CT and/or MRI and more recently MR-guided focused ultrasound have complemented the diagnostic and therapeutic armamentarium.
Key messages: In the current manuscript, we discuss standard imaging sequences including advanced techniques like DWI, DTI, susceptibility-weighted imaging, and 1H magnetic resonance spectroscopy, various perfusion weighted imaging approaches including arterial spin labeling, dynamic contrast enhanced imaging, and dynamic susceptibility contrast imaging. Pre-, intra, and postoperative surgical imaging including visualize imaging will be discussed. The value of connectomics will be presented for its value in neuro-oncology. Minimal invasive therapeutic possibilities of interventional neuroradiology and image-guided laser ablation and MR-guided high-intensity-focused ultrasound will be presented for treatment of pediatric brain and spinal cord tumors. Finally, a comprehensive review of spinal cord tumors and matching neuropathology has been included.
{"title":"Advances in Imaging Modalities for Pediatric Brain and Spinal Cord Tumors.","authors":"Thierry A G M Huisman, Rajan Patel, Stephen Kralik, Nilesh K Desai, Avner Meoded, Karen Chen, Howard L Weiner, Daniel J Curry, Maarten Lequin, Mariette Kranendonk, Gunes Orman, George Jallo","doi":"10.1159/000531998","DOIUrl":"10.1159/000531998","url":null,"abstract":"<p><strong>Background: </strong>Neuroimaging has evolved from anatomical imaging toward a multi-modality comprehensive anatomical and functional imaging in the past decades, important functional data like perfusion-weighted imaging, permeability imaging, diffusion-weighted imaging (DWI), and diffusion tensor imaging (DTI), tractography, metabolic imaging, connectomics, event-related functional imaging, resting state functional imaging, and much more is now being offered.</p><p><strong>Summary: </strong>Precision diagnostics has proven to be essential for precision treatment. Many minimal invasive techniques have been developed, taking advantage of digital subtraction angiography and interventional neuroradiology. Furthermore, intraoperative CT and/or MRI and more recently MR-guided focused ultrasound have complemented the diagnostic and therapeutic armamentarium.</p><p><strong>Key messages: </strong>In the current manuscript, we discuss standard imaging sequences including advanced techniques like DWI, DTI, susceptibility-weighted imaging, and 1H magnetic resonance spectroscopy, various perfusion weighted imaging approaches including arterial spin labeling, dynamic contrast enhanced imaging, and dynamic susceptibility contrast imaging. Pre-, intra, and postoperative surgical imaging including visualize imaging will be discussed. The value of connectomics will be presented for its value in neuro-oncology. Minimal invasive therapeutic possibilities of interventional neuroradiology and image-guided laser ablation and MR-guided high-intensity-focused ultrasound will be presented for treatment of pediatric brain and spinal cord tumors. Finally, a comprehensive review of spinal cord tumors and matching neuropathology has been included.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10413946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01Epub Date: 2023-06-28DOI: 10.1159/000531719
Elena Filimonova, Azniv Martirosyan, Konstantin Ovsiannikov, Anton Pashkov, Jamil Rzaev
Introduction: Surgical revascularization is very effective in patients with moyamoya angiopathy (MMA) and leads to improvements in cortical perfusion parameters. However, changes in white matter hemodynamics are still underestimated. To date, only a few studies have examined brain perfusion changes within deep white matter after bypass surgery in patients with MMA.
Methods: Ten children with MMA were evaluated using the CT perfusion technique before and after revascularization surgery. Brain perfusion parameters within gray and white matter were compared before and after surgery. The correlations between the perfusion parameters before surgery and the Suzuki stage, as well as between the perfusion parameters and the cognitive scores, were also evaluated.
Results: Brain perfusion parameters improved significantly in both gray matter (predominantly due to cerebral blood flow within the anterior circulation, p < 0.01) and white matter (predominantly due to cerebral blood volume within the semiovale centrum, p < 0.001). We revealed that the pattern of improvement in perfusion in white matter differed from the pattern of improvement in perfusion in gray matter. Significant correlations were revealed between the Suzuki stage before surgery and the perfusion parameters within the posterior cerebral artery circulation (adjusted p < 0.05). There were also significant correlations between cognitive scores and brain perfusion parameters in gray matter and white matter (adjusted p < 0.05).
Conclusions: The perfusion parameters of gray matter and white matter in the brain improve differently after bypass surgery in patients with MMA. Different hemodynamics within these compartments could explain this.
{"title":"White and Gray Matter Perfusion in Children with Moyamoya Angiopathy after Revascularization Surgery.","authors":"Elena Filimonova, Azniv Martirosyan, Konstantin Ovsiannikov, Anton Pashkov, Jamil Rzaev","doi":"10.1159/000531719","DOIUrl":"10.1159/000531719","url":null,"abstract":"<p><strong>Introduction: </strong>Surgical revascularization is very effective in patients with moyamoya angiopathy (MMA) and leads to improvements in cortical perfusion parameters. However, changes in white matter hemodynamics are still underestimated. To date, only a few studies have examined brain perfusion changes within deep white matter after bypass surgery in patients with MMA.</p><p><strong>Methods: </strong>Ten children with MMA were evaluated using the CT perfusion technique before and after revascularization surgery. Brain perfusion parameters within gray and white matter were compared before and after surgery. The correlations between the perfusion parameters before surgery and the Suzuki stage, as well as between the perfusion parameters and the cognitive scores, were also evaluated.</p><p><strong>Results: </strong>Brain perfusion parameters improved significantly in both gray matter (predominantly due to cerebral blood flow within the anterior circulation, p < 0.01) and white matter (predominantly due to cerebral blood volume within the semiovale centrum, p < 0.001). We revealed that the pattern of improvement in perfusion in white matter differed from the pattern of improvement in perfusion in gray matter. Significant correlations were revealed between the Suzuki stage before surgery and the perfusion parameters within the posterior cerebral artery circulation (adjusted p < 0.05). There were also significant correlations between cognitive scores and brain perfusion parameters in gray matter and white matter (adjusted p < 0.05).</p><p><strong>Conclusions: </strong>The perfusion parameters of gray matter and white matter in the brain improve differently after bypass surgery in patients with MMA. Different hemodynamics within these compartments could explain this.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10070134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gelsomina Aruta, Pietro Fiaschi, Marco Ceraudo, Gianluca Piatelli, Valeria Capra, Andrea Bianconi, Andrea Rossi, Francesca Secci, Marco Pavanello
Introduction: The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature, especially in children with paediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association, and several theories have been proposed. In particular, it is now accepted that the hypothesis of the overcrowding of the posterior fossa due to precocious suture fusion is the cause of the cerebellar herniation in syndromic and non-syndromic patients, against the theory of intrinsic cerebellar anomalies, ventriculomegaly, and venous hypertension. However, whatever the pathophysiological mechanism, it is still unclear what the best management and treatment of CM and hydrocephalus are in multisutural craniosynostosis patients. The aim of this study was to report our 25 years' experience in treating paediatric patients affected by these rare pathologies in order to propose a simple and effective therapeutic flow chart for their management.
Materials and methods: We retrospectively collected data of each patient who underwent a cranial vault remodelling (CVR) for complex multisutural craniosynostosis in our institution in the last 25 years, while monosutural craniosynostosis was excluded. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly, and CM at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow chart that could help physicians choose the best surgical treatment when different pathological conditions, as Chiari malformation I (CMI) or hydrocephalus, affect complex craniosynostosis children.
Results: Thirty-nine patients (39 out of 55; 70.9%), with an isolated multisutural craniosynostosis at presentation, underwent a two-step CVR as first surgery; 36 patients (92.3%) had an improved outcome, 2 patients (5.1%) had a stable outcome, and 1 patient (2.56%) had a deteriorated outcome. Other eight children (8 out of 55; 14.5%) had a radiological evidence of asymptomatic CMI at presentation. In this group, we performed CVR as first surgery. As for the final outcome, 7 patients had an improved outcome (87.5%) with good aesthetic result and stability or resolution of CMI. Finally, 7 patients (7 out of 55; 12.7%) presented a various combination of CMI and ventriculomegaly or hydrocephalus at presentation. Among them, 3 patients had an improved outcome (42.8%), and 4 patients had a deteriorated outcome (57.1%).
Discussion: The prevalence of one pathological condition with associated symptoms over the others was the key factor leading our therapeutic strategy. When craniosynostosis is associated with a radiological CM, the assessm
{"title":"Practical Algorithm for the Management of Multisutural Craniosynostosis with Associated Chiari Malformation and/or Hydrocephalus.","authors":"Gelsomina Aruta, Pietro Fiaschi, Marco Ceraudo, Gianluca Piatelli, Valeria Capra, Andrea Bianconi, Andrea Rossi, Francesca Secci, Marco Pavanello","doi":"10.1159/000529129","DOIUrl":"https://doi.org/10.1159/000529129","url":null,"abstract":"<p><strong>Introduction: </strong>The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature, especially in children with paediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association, and several theories have been proposed. In particular, it is now accepted that the hypothesis of the overcrowding of the posterior fossa due to precocious suture fusion is the cause of the cerebellar herniation in syndromic and non-syndromic patients, against the theory of intrinsic cerebellar anomalies, ventriculomegaly, and venous hypertension. However, whatever the pathophysiological mechanism, it is still unclear what the best management and treatment of CM and hydrocephalus are in multisutural craniosynostosis patients. The aim of this study was to report our 25 years' experience in treating paediatric patients affected by these rare pathologies in order to propose a simple and effective therapeutic flow chart for their management.</p><p><strong>Materials and methods: </strong>We retrospectively collected data of each patient who underwent a cranial vault remodelling (CVR) for complex multisutural craniosynostosis in our institution in the last 25 years, while monosutural craniosynostosis was excluded. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly, and CM at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow chart that could help physicians choose the best surgical treatment when different pathological conditions, as Chiari malformation I (CMI) or hydrocephalus, affect complex craniosynostosis children.</p><p><strong>Results: </strong>Thirty-nine patients (39 out of 55; 70.9%), with an isolated multisutural craniosynostosis at presentation, underwent a two-step CVR as first surgery; 36 patients (92.3%) had an improved outcome, 2 patients (5.1%) had a stable outcome, and 1 patient (2.56%) had a deteriorated outcome. Other eight children (8 out of 55; 14.5%) had a radiological evidence of asymptomatic CMI at presentation. In this group, we performed CVR as first surgery. As for the final outcome, 7 patients had an improved outcome (87.5%) with good aesthetic result and stability or resolution of CMI. Finally, 7 patients (7 out of 55; 12.7%) presented a various combination of CMI and ventriculomegaly or hydrocephalus at presentation. Among them, 3 patients had an improved outcome (42.8%), and 4 patients had a deteriorated outcome (57.1%).</p><p><strong>Discussion: </strong>The prevalence of one pathological condition with associated symptoms over the others was the key factor leading our therapeutic strategy. When craniosynostosis is associated with a radiological CM, the assessm","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10061157","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-01-01Epub Date: 2023-02-06DOI: 10.1159/000529507
Chelsea Kotch, Stephanie Nicole Brosius, Thomas De Raedt, Michael Jay Fisher
Background: Neurofibromatosis type 1 and neurofibromatosis type 2 are unrelated, distinct genetic disorders characterized by the development of central and peripheral nervous system tumors.
Summary: Neurofibromatosis type 1 is the most common inherited tumor predisposition syndrome with a lifelong increased risk of benign and malignant tumor development, such as glioma and nerve sheath tumors. Neurofibromatosis type 2 classically presents with bilateral vestibular schwannoma, yet it is also associated with non-vestibular schwannoma, meningioma, and ependymoma. Historically, the number of effective therapies for neurofibromatosis-related neoplasms has been limited.
Key message: In the past decade, there have been significant advances in the development of precision-based therapies for NF-associated tumors with an increased emphasis on functional outcomes in addition to tumor response. Continued scientific discovery and advancement of targeted therapies for NF-associated neoplasms are necessary to continue to improve outcomes for patients with NF.
{"title":"Updates in the Management of Central and Peripheral Nervous System Tumors among Patients with Neurofibromatosis Type 1 and Neurofibromatosis Type 2.","authors":"Chelsea Kotch, Stephanie Nicole Brosius, Thomas De Raedt, Michael Jay Fisher","doi":"10.1159/000529507","DOIUrl":"10.1159/000529507","url":null,"abstract":"<p><strong>Background: </strong>Neurofibromatosis type 1 and neurofibromatosis type 2 are unrelated, distinct genetic disorders characterized by the development of central and peripheral nervous system tumors.</p><p><strong>Summary: </strong>Neurofibromatosis type 1 is the most common inherited tumor predisposition syndrome with a lifelong increased risk of benign and malignant tumor development, such as glioma and nerve sheath tumors. Neurofibromatosis type 2 classically presents with bilateral vestibular schwannoma, yet it is also associated with non-vestibular schwannoma, meningioma, and ependymoma. Historically, the number of effective therapies for neurofibromatosis-related neoplasms has been limited.</p><p><strong>Key message: </strong>In the past decade, there have been significant advances in the development of precision-based therapies for NF-associated tumors with an increased emphasis on functional outcomes in addition to tumor response. Continued scientific discovery and advancement of targeted therapies for NF-associated neoplasms are necessary to continue to improve outcomes for patients with NF.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10668127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amparo Saenz, Yamila Basilotta Marquez, Emma A Dalton, Romina Argañaraz, Beatriz Mantese
Introduction: This study aimed to identify factors affecting progression-free survival (PFS) in pediatric patients with giant supratentorial brain tumors (GSBTs) treated with surgical excision. The secondary aim was to analyze how these same factors affected the functional outcome in the long term.
Methods: We performed a retrospective, analytical, single-center cohort study. We included all pediatric patients with GSBT between January 2014 and June 2018. Patients were followed for a minimum of 24 months for the PFS and overall survival (OS) analysis. Functional status score (FSS) was used to assess the functional outcome.
Results: We included 27 patients with GSBT, the median age was six (range 2-12), and eleven patients had a grade IV tumor. The 24-month PFS and OS were 51.85% and 74.04%, respectively. A PFS-ending event or treatment failure occurred in 13 patients. We found that patients with postoperative FFS >16 have a worse PFS than patients with a postoperative FSS <15 (HR 4.51; p = 0.03). Patients with more than three surgeries had worse PFS than patients with one or two procedures (HR 11.39; p = 0.004). High-grade tumors were associated with worse PFS than low-grade tumors (HR 1.55; p = 0.04). Finally, patients with CNS infections had worse PFS than patients without that complication (HR 2.70; p = 0.04).
Conclusions: GSBTs in pediatric patients are complex lesions that require multidisciplinary management. Surgical management and quality of life should be considered when choosing the best treatment. Factors influencing long-term PFS were high-grade histopathology, the need for three or more surgeries, postoperative FSS >16, and CNS infections.
{"title":"Giant Supratentorial Brain Tumors in Children: Functional Outcome and Progression-Free Survival Analysis.","authors":"Amparo Saenz, Yamila Basilotta Marquez, Emma A Dalton, Romina Argañaraz, Beatriz Mantese","doi":"10.1159/000530592","DOIUrl":"https://doi.org/10.1159/000530592","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to identify factors affecting progression-free survival (PFS) in pediatric patients with giant supratentorial brain tumors (GSBTs) treated with surgical excision. The secondary aim was to analyze how these same factors affected the functional outcome in the long term.</p><p><strong>Methods: </strong>We performed a retrospective, analytical, single-center cohort study. We included all pediatric patients with GSBT between January 2014 and June 2018. Patients were followed for a minimum of 24 months for the PFS and overall survival (OS) analysis. Functional status score (FSS) was used to assess the functional outcome.</p><p><strong>Results: </strong>We included 27 patients with GSBT, the median age was six (range 2-12), and eleven patients had a grade IV tumor. The 24-month PFS and OS were 51.85% and 74.04%, respectively. A PFS-ending event or treatment failure occurred in 13 patients. We found that patients with postoperative FFS >16 have a worse PFS than patients with a postoperative FSS <15 (HR 4.51; p = 0.03). Patients with more than three surgeries had worse PFS than patients with one or two procedures (HR 11.39; p = 0.004). High-grade tumors were associated with worse PFS than low-grade tumors (HR 1.55; p = 0.04). Finally, patients with CNS infections had worse PFS than patients without that complication (HR 2.70; p = 0.04).</p><p><strong>Conclusions: </strong>GSBTs in pediatric patients are complex lesions that require multidisciplinary management. Surgical management and quality of life should be considered when choosing the best treatment. Factors influencing long-term PFS were high-grade histopathology, the need for three or more surgeries, postoperative FSS >16, and CNS infections.</p>","PeriodicalId":54631,"journal":{"name":"Pediatric Neurosurgery","volume":null,"pages":null},"PeriodicalIF":0.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10106822","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}