Cristina Barceló-López1, Antonio L López-Guerrero1, Antonio García-López1, José Hurtado-Marín2, Helena Alarcón-Martínez3, Juan F Martínez-Lage4. 1. Regional Service of Neurosurgery, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain. 2. Service of Anesthesiology, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain. 3. Section of Neuropediatrics, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain. 4. Regional Service of Neurosurgery, Virgen de la Arrixaca University Hospital, 30120, Murcia, Spain. juanf.martinezlage@gmail.com.
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.
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.
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