Benign Intracranial Hypertension in Association with Arteriovenous malformation and Chiari I Malformation: First report of a rare pathological combination

Abstract

Benign Intracranial Hypertension (BIH) entity occurs in both, Chiari malformations and Arterio-Venous Malformations (AVMs) individually. Associations of Chiari and AVM occurring in the same individual have been reported in the literature. However, the synchronous association of BIH, Chiari malformation and AVM occurring in one individual, to the authors` knowledge, has not previously been reported.

We report the case of a 13-years-old girl who presented with a history of headaches and blurred vision. Ophthalmology examination confirmed papilloedema. Neuro-imaging identified a Chiari I malformation and occipital AVM with no hydrocephalus.

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Methods & Results
Our patient underwent
angiography and embolision of
a right occipital AVM. The
papilloedema failed to resolve
and therefore she underwent
insertion of a ventriculo-
peritoneal shunt. The Chiari
malformation was managed
conservatively.
The papilloedema and
headaches improved in the post-
operative period. Further
investigations with the insertion
of an intracranial pressure bolt
revealed normal intracranial
pressures. Her intellectual
development is in keeping at par
for her age group.
Conclusions
The association of BIH, AVM and
Chiari malformation is not well
recognised. Our report is the
first case where these entities
have occurred in the same
individual. We would like to
highlight this presentation
should be looked for through
careful screening and each
condition managed in relation to
the other.
Subject
We report the case of a 13-years-old
girl who presented with a history of
headaches and blurred vision.
Ophthalmology examination confirmed
papilloedema. Neuro-imaging
identified a Chiari I malformation and
occipital AVM with no hydrocephalus.
Benign Intracranial Hypertension in Association with
Arteriovenous malformation and Chiari I
Malformation: First report of a rare pathological combination
Introduction
Benign Intracranial Hypertension (BIH)
entity occurs in both, Chiari
malformations and Arterio-Venous
Malformations (AVMs) individually.
Associations of Chiari and AVM
occurring in the same individual have
been reported in the literature.
However, the synchronous association
of BIH, Chiari malformation and AVM
occurring in one individual, to the
authors` knowledge, has not
previously been reported.
B Dhamija, R Ramirez, S Lamin, A R Walsh
Birmingham Children`s Hospital, Birmingham, UK
.
MRA Right Occipital AVM draining to
deep and superficial venous systems
DSA, Occipital AVM draining into
Superior Sagittal Sinus
Sagittal T1 MRI. Chiari I malformation. Occipital
arterio venous malformation
DSA, Post embolisation of Occipital
Arterio-venous Malformation