Intraosseous CSF Fistula in a Patient with Gorham Disease Resulting in Intracranial Hypotension

F Adler,W.P Dillon,N Gupta,C.P Hess,C.F Dowd

doi:10.3174/ajnr.a2413

Abstract

A 7-year-old girl with a history of headaches and Gorham disease was surgically treated in infancy for Chiari I malformation. Subsequent investigation revealed that her cerebellar tonsillar ectopia was due to a long-standing spinal CSF-lymphatic fistula causing intracranial hypotension. Percutaneous fistula closure was performed several times, resulting in transient symptomatic improvement.

Highlights

Massive osteolysis, is a rare disorder characterized by proliferation of lymphatic channels with resulting bone destruction
We describe a child with Gorham disease who was initially treated for Chiari I malformation, which was, in retrospect, secondary to intracranial hypotension from a CSF fistula between a lumbar nerve root sleeve and a lymphatic malformation
Cerebellar tonsillar ectopia in our patient was a manifestation of intracranial hypotension caused by a high-volume lumbar CSF fistula, with syrinx formation secondary to foramen magnum obstruction

Summary

Introduction

The patient underwent posterior fossa decompression and duraplasty, with improved but persistent symptoms (Fig 1A). Serial MR imaging revealed progressive replacement of bone with fluid, consistent with Gorham disease (Fig 1B, -C).

Results

Conclusion