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Brain gliomas, hydrocephalus and idiopathic aqueduct stenosis in children with neurofibromatosis type 1
M. Glombova, B. Petrak, J. Lisy, J. Zamecnik, D. Sumerauer, P. Liby,
Jazyk angličtina Země Nizozemsko
Typ dokumentu časopisecké články
- MeSH
- dítě MeSH
- gliom zrakového nervu epidemiologie MeSH
- gliom epidemiologie patofyziologie MeSH
- hydrocefalus epidemiologie patofyziologie MeSH
- incidence MeSH
- kojenec MeSH
- lidé MeSH
- longitudinální studie MeSH
- mladiství MeSH
- mozek patologie MeSH
- nádory mozku epidemiologie patologie MeSH
- neurofibromatóza 1 komplikace metabolismus patofyziologie MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- progrese nemoci MeSH
- rizikové faktory MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Geografické názvy
- Česká republika MeSH
PURPOSE: To evaluate the incidence and clinical importance of brain gliomas - optic pathway gliomas (OPGs) and especially gliomas outside the optic pathway (GOOP) for children with neurofibromatosis type 1 (NF1), additionally, to assess the causes of obstructive hydrocephalus in NF1 children with an emphasis on cases caused by idiopathic aqueduct stenosis. SUBJECTS AND METHODS: We analysed data from 285 NF1 children followed up on our department from 1990 to 2010 by the same examination battery. RESULTS: We have found OPGs in 77/285 (27%) children and GOOPs in 29/285 (10,2%) of NF1 children, of who 19 had OPG and GOOP together, so the total number of brain glioma was 87/285 (30,5%). GOOPs were significantly more often treated than OPGs (p > 0.01). OPGs contain clinically important subgroup of 14/285 (4.9%) spreading to hypothalamus. Spontaneous regression was documented in 4/285 (1.4%) gliomas and the same number of NF1 children died due to gliomas. Obstructive hydrocephalus was found in 22/285 (7.7%) patients and 14/22 cases were due to glioma. Idiopathic aqueduct stenosis caused hydrocephalus in 6/22 cases and was found in 2.1% of NF1 children. Two had other cause. CONCLUSIONS: The total brain glioma number (OPGs and only GOOPs together) better reflected the overall brain tumour risk for NF1 children. However, GOOPs occur less frequently than OPGs, they are more clinically relevant. The obstructive hydrocephalus was severe and featuring frequent complication, especially those with GOOP. Idiopathic aqueduct stenosis shows an unpredictable cause of hydrocephalus in comparison with glioma and is another reason for careful neurologic follow up.
Citace poskytuje Crossref.org
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- $a Glombova, Marie $u Department of Paediatric Neurology, Second Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic; Paediatric Department, District Hospital Kolin, Czech Republic. Electronic address: m.glombova@gmail.com.
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- $a PURPOSE: To evaluate the incidence and clinical importance of brain gliomas - optic pathway gliomas (OPGs) and especially gliomas outside the optic pathway (GOOP) for children with neurofibromatosis type 1 (NF1), additionally, to assess the causes of obstructive hydrocephalus in NF1 children with an emphasis on cases caused by idiopathic aqueduct stenosis. SUBJECTS AND METHODS: We analysed data from 285 NF1 children followed up on our department from 1990 to 2010 by the same examination battery. RESULTS: We have found OPGs in 77/285 (27%) children and GOOPs in 29/285 (10,2%) of NF1 children, of who 19 had OPG and GOOP together, so the total number of brain glioma was 87/285 (30,5%). GOOPs were significantly more often treated than OPGs (p > 0.01). OPGs contain clinically important subgroup of 14/285 (4.9%) spreading to hypothalamus. Spontaneous regression was documented in 4/285 (1.4%) gliomas and the same number of NF1 children died due to gliomas. Obstructive hydrocephalus was found in 22/285 (7.7%) patients and 14/22 cases were due to glioma. Idiopathic aqueduct stenosis caused hydrocephalus in 6/22 cases and was found in 2.1% of NF1 children. Two had other cause. CONCLUSIONS: The total brain glioma number (OPGs and only GOOPs together) better reflected the overall brain tumour risk for NF1 children. However, GOOPs occur less frequently than OPGs, they are more clinically relevant. The obstructive hydrocephalus was severe and featuring frequent complication, especially those with GOOP. Idiopathic aqueduct stenosis shows an unpredictable cause of hydrocephalus in comparison with glioma and is another reason for careful neurologic follow up.
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- $a Petrak, Borivoj $u Department of Paediatric Neurology, Second Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic.
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