Child's Nervous System

, Volume 34, Issue 8, pp 1609–1611 | Cite as

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis

  • Cristina Barceló-López
  • Antonio L. López-Guerrero
  • Antonio García-López
  • José Hurtado-Marín
  • Helena Alarcón-Martínez
  • Juan F. Martínez-LageEmail author
Case Report


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.


Benign 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.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Regional Service of NeurosurgeryVirgen de la Arrixaca University HospitalMurciaSpain
  2. 2.Service of AnesthesiologyVirgen de la Arrixaca University HospitalMurciaSpain
  3. 3.Section of NeuropediatricsVirgen de la Arrixaca University HospitalMurciaSpain

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