Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis
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.
KeywordsBenign intracranial hypertension Intracranial lymphatic system Kaposiform lymphangiomatosis Pseudotumor cerebri Ventriculoperitoneal shunting
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest and that they have received no funding for the publication of this report. Consent was obtained from the child’s parents.