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Society for Pediatric Radiology – Poster Archive
Final ID: Poster #: EDU-020
Pontine stroke in a child with Neurofibromatosis 2 (NF-2)
Purpose or Case Report: To present a very rare case of a pontine stroke in a 13-year-old boy with NF-2.
Methods & Materials: 13-year-old boy with clinically proven neurofibromatosis type 2 (bilateral acusticus schwannoma, meningeoma of the cavernous sinus, schwannoma of the cauda equina), who developed a left-sided brachium pontis stroke. Clinical, laboratory and imaging findings (MRI, MRA, angiography) are presented and discussed.
Results: The patient was referred for sudden onset of dizziness and vertigo. He presented an ataxic gait pattern and complained in addition about a reduced hearing ability on the left side.
MRI revealed the known bilateral acustic schwannoma and a lesion in the right cavernous sinus presumed to be a meningioma.
In addition a lesion of the left brachium pontis with intense restriction of diffusion in the DWI was seen initially interpreted as a glioma.
Biopsy was performed that showed thickening of the vessel wall of the small vessels indicative of vasculopathy. No tumor cells or signs of inflammation could be observed.
Intense coagulation work-up including protein C, protein S, homocystein, anticardiolipid antibodies were unremarkable. MR-angiography and conventional angiography could not detect any vascular pathology.
Conclusions: Childhood stroke -although rare- is a well known complications of NF-1 but not of NF-2.
Until now there are only 3 case reports about childhood stroke in NF-2. Two of these describe an infratentorial location as in our case.
Our pathologic specimen revealed changes of the vessel wall that could be an expression of a vasculopathy although the imaging findings of the larger vessels do not support this assumption.
Methods & Materials: 13-year-old boy with clinically proven neurofibromatosis type 2 (bilateral acusticus schwannoma, meningeoma of the cavernous sinus, schwannoma of the cauda equina), who developed a left-sided brachium pontis stroke. Clinical, laboratory and imaging findings (MRI, MRA, angiography) are presented and discussed.
Results: The patient was referred for sudden onset of dizziness and vertigo. He presented an ataxic gait pattern and complained in addition about a reduced hearing ability on the left side.
MRI revealed the known bilateral acustic schwannoma and a lesion in the right cavernous sinus presumed to be a meningioma.
In addition a lesion of the left brachium pontis with intense restriction of diffusion in the DWI was seen initially interpreted as a glioma.
Biopsy was performed that showed thickening of the vessel wall of the small vessels indicative of vasculopathy. No tumor cells or signs of inflammation could be observed.
Intense coagulation work-up including protein C, protein S, homocystein, anticardiolipid antibodies were unremarkable. MR-angiography and conventional angiography could not detect any vascular pathology.
Conclusions: Childhood stroke -although rare- is a well known complications of NF-1 but not of NF-2.
Until now there are only 3 case reports about childhood stroke in NF-2. Two of these describe an infratentorial location as in our case.
Our pathologic specimen revealed changes of the vessel wall that could be an expression of a vasculopathy although the imaging findings of the larger vessels do not support this assumption.
More abstracts on this topic:
A Rare Case of Malignant Atrophic Papulosis (Degos disease) Complicated by Small Bowel Perforation
Elhefnawi Yara, Atasoy Duygu, Herliczek Thaddeus, Lee Edward, Larosa Michelle, Tivnan Patrick
Chest radiographic findings in congenital heart disease –comparison with CT images-Nakagawa Motoo, Ozawa Yoshiyuki, Tanaka Yoshihiro, Shibamoto Yuta
Poster____EDU-020.pdf
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