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Case 1

Cavernous Hemangioma of the Optic Chiasm

Cavernous hemangiomas (Fig. 1A,1B,1C,1D), also known as cavernous angiomas or cavernomas, represent 5-13% of all intracranial vascular malformations. They are usually supratentorial involving the cerebral hemispheres but can also involve the cerebellum, spinal cord, and orbits. Very rarely they can occur at the optic chiasm, with only 14 cases previously reported. Although occult bleeds are common in cavernous hemangiomas, the reported symptomatic hemorrhage rate per year is less than 1% for all lesions. However, for chiasmatic cavernous hemangiomas, symptomatic hemorrhage occurs in essentially every case, with most patients presenting with chiasmal apoplexy, as was the case with our patient. The increased signal intensity on T1-weighted MR images represents blood products likely related to hemorrhage within the lesion [1,2,3,4,5,6,7,8].

View larger version (115K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A. —38-year-old man with 1-week history of headache and left visual disturbance. Axial unenhanced CT scans of head show hyperdense mass in suprasellar cistern (arrows).

View larger version (100K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B. —38-year-old man with 1-week history of headache and left visual disturbance. Axial unenhanced CT scans of head show hyperdense mass in suprasellar cistern (arrows).

View larger version (175K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C. —38-year-old man with 1-week history of headache and left visual disturbance. Unenhanced sagittal T1-weighted MR image of brain shows heterogeneous mass of optic chiasm containing focus of increased signal intensity (arrow).

View larger version (175K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1D. —38-year-old man with 1-week history of headache and left visual disturbance. Enhanced coronal T1-weighted MR image through optic chiasm shows heterogeneous mass with only minimal contrast enhancement (arrows).

The other diagnoses are not correct for various reasons. The signal characteristics and pattern of enhancement are not suggestive of a meningioma. Meningiomas are typically isointense to gray matter on all pulse sequences, show rather intense and uniform contrast enhancement, and more classically involve the prechiasmatic optic nerve. Although the CT scan raises a question of a possible aneurysm, the MR image clearly shows the lesion to be a mass of the optic chiasm with no flow void noted. The angiogram also had negative findings. Gliomas of the chiasm typically occur in the first decade. They generally have marked contrast enhancement. Calcification and hemorrhage are typical features of chiasmatic hemangiomas but not of chiasmatic gliomas.

References

1. Corboy JR, Galetta SL. Familial cavernous angiomas manifesting as an acute chiasmal syndrome. Am J Opthalmol 1989;108:245 -250[Medline]
2. Klein LH, Fermaglich J, Kattah J, Lussenhop AJ. Cavernous hemangioma of the optic chiasm, optic nerves and the right optic tract: case report and review of the literature. Virchows Arch A Pathol Anat Histol 1979;383:225 -231[Medline]
3. Malik S, Cohen BH, Robinsos J, Fried A, Sila CA. Progressive vision loss: a rare manifestation of familial cavernous angiomas. Arch Neurol 1992;49:170 -173[Abstract]
4. Mohr G, Hardy J, Gauvin P. Chiasmal apoplexy due to ruptured cavernous hemangioma of optic chiasm. Surg Neurol 1985;24:636 -640[Medline]
5. Shibuya M, Baskaya MK, Saito K, Suzuki Y, Ooka K, Hara M. Cavernous malformations of the optic chiasm. Acta Neurochir (Wien) 1995;136:29 -36[Medline]
6. Steinberg GK, Marks MP, Shur LM, et al. Occult vascular malformations of the optic chiasm: magnetic resonance imaging diagnosis and surgical laser resection. Neurosurgery 1990;27:466 -470[Medline]
7. Regli L, de Tribolet N, Regli F, Bogousslavsky J. Chiasmal apoplexy: haemorrhage from a cavernous malformation in the optic chiasm. J Neurol Neurosurg Psychiatry 1989;52:1095 -1099[Abstract/Free Full Text]
8. Zentner J, Gradd W, Hassler W. Cavernous angioma of the optic tract. J Neurol 1989;236:117 -119[Medline]

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This Article Figures Only Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bourekas, E. C. Articles by Christoforidis, G. A. Search for Related Content PubMed Articles by Bourekas, E. C. Articles by Christoforidis, G. A. Social Bookmarking                      What's this?