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- Title
Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.
- Authors
Barceló-López, Cristina; López-Guerrero, Antonio L.; García-López, Antonio; Martínez-Lage, Juan F.; Hurtado-Marín, José; Alarcón-Martínez, Helena
- Abstract
Case Report: A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child’s condition has markedly improved.Discussion and conclusions: A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child’s pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.
- Subjects
INTRACRANIAL hypertension; CHEST (Anatomy); COMPUTED tomography; MAGNETIC resonance imaging
- Publication
Child's Nervous System, 2018, Vol 34, Issue 8, p1609
- ISSN
0256-7040
- Publication type
Case Study
- DOI
10.1007/s00381-018-3794-x