Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis

Karl A. Soderlund, Mark D. Mamlouk, Vinil N. Shah, Jarod L. Roland, William P. Dillon

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Spontaneous intracranial hypotension is an uncommon etiology of secondary headaches in children. We report a unique case of a girl with kaposiform lymphangiomatosis who developed postural headaches and imaging features of spontaneous intracranial hypotension without a spinal extradural collection. The girl underwent dynamic computed tomography myelography which revealed a cerebrospinal fluid (CSF)-lymphatic fistula related to a lymphatic malformation associated with the right T10 nerve. She underwent surgical ligation of the CSF-lymphatic fistula, resulting in resolution of the headaches. Spinal CSF-lymphatic fistulas are rare and have previously been reported in two patients with Gorham-Stout disease. The current report suggests that patients with systemic lymphatic anomalies who develop postural headaches should undergo evaluation for spontaneous intracranial hypotension and a CSF-lymphatic fistula. If discovered, surgical ligation is a potential treatment.

Original languageEnglish
Pages (from-to)2093-2097
Number of pages5
JournalPediatric radiology
Volume51
Issue number11
DOIs
StatePublished - Oct 2021

Keywords

  • Cerebrospinal fluid
  • Cerebrospinal fluid leak
  • Cerebrospinal fluid-lymphatic fistula
  • Child
  • Kaposiform lymphangiomatosis
  • Lymphatic anomaly
  • Spontaneous intracranial hypotension

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