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Show Journal of Clinical Neuro-ophthalmology 13(4): 232-236, 1993. Apoplectic Optochiasmal Syndrome Due to Intrinsic Cavernous Hemangioma Case Report Jiunn-Feng Hwang, M.D., Chi-Wang Yau, M.D., Jon-Kway Huang, M.D., and Chung-Yau Tsai, M.D. © 1993 Raven Press, Ltd., New York A 42-year-old man suffered from acute evolving visual loss after drinking alcohol. Consecutive visual field changes suggested a mass within the left optic nerve extending its compression across the chiasm and encroaching on the left optic tract. Computed tomography scanning and magnetic resonance imaging confirmed the presence of an overt hemorrhage, a real apoplectic event of cavernous hemangioma. Cavernous hemangiomas located within the intracranial optic nerve are few. Occult hemorrhage within the confines of the intrinsic vascular tumor resulted in acute visual changes in most reported cases. Overt hemorrhage with blood extravasation beyond the original confines of the intrinsic optochiasmal tumor are rarely reported in the literature. Herein, we report one such case. The clinical course and the predisposing factors are also discussed. Key Words: Apoplectic optochiasmal syndrome-Overt hemorrhage-Cavernous hemangioma-Magnetic resonance image-Chiasmal apoplexy. From the Departments of Ophthalmology G-F.H., C-W.Y.), Radiology G-K.H.), and Neurosurgery (C-Y.T.), Mackay Memorial Hospital, Taipei, Taiwan. Address correspondence and reprint requests to Dr. JiunnFeng Hwang, Department of Ophthalmology, Mackay Memorial Hospital, 92, Sec. 2, Chung-San N. Rd. Taipei, Taiwan. 232 Intracranial cavernous hemangioma is usually located in the frontal lobe and temporal lobe. It may manifest seizure or focal neurologic deficit. However, most cases are clinically silent, being diagnosed incidentally on a computed tomography scan or during an autopsy examination. Cavernous hemangiomas confined within the intracranial anterior visual pathway are rarely found. Such cases will present very different clinical manifestations and require earlier surgical treatment. We report a case with dramatic visual changes and special radiologic findings. Clinical characteristics, predisposing factors, and mechanisms responsible for the specific clinical course are discussed. CASE REPORT A 42-year-old man was quite well until March 2, 1991. When he woke up in the morning, acutely evolving visual loss of the left eye was noted. He visited Mackay hospital without delay. His visual acuity was 20/40 in the right eye and no light perception in the left eye. Intraocular pressure was 17 mmHg in both eyes. The pupil of the left eye was mildly dilated with marked afferent pupillary conduction defect (Marcus Gunn pupil). Light projection test and finger confrontation test revealed total blindness of the left eye and normal visual field of the right eye. The optic fundi were both normal and no cherry-red spot was found. The optic discs were not pale. His systemic blood pressure was 140/80 mmHg. There was no headache complaint. The patient denied history of systemic disease or head injury. He had a habit of regular alcohol consumption every night. Several episodes of transient blurring of vision after drinking were mentioned, but all subsided spontaneously after sleep. APOPLECTIC OPTOCHIASMAL SYNDROME 233 Prior to this onset, he also drank some wine and felt the same blurring. He went to sleep as usual but woke up with loss of vision this time. Under the initial impression of retrobulbar neuritis of the left eye, he received treatment with corticosteroids. The next day, we found there was some light perception of the left eye. Afferent pupillary conduction defect also improved a little. Yet, no further improvement was found until the fifth day after onset. We suggested that the patient drink 200 ml of beer to test the influence of alcohol on his symptom. The next morning, 12 hours later, visual acuity improved to hand motion in the temporal field of the left eye but diminished to 20/70 in the right eye. Goldmann's perimetry demonstrated right homonymous hemianopsia with central visual field defect of the right eye. Hence a tumor of the anterior visual pathway was suspected and was proved by computed tomography scanning (Fig. 1). Bilateral carotid angiography revealed no vascular abnormality. All serum levels of pituitary hormones were within normal range. Magnetic resonance imaging (MRI), performed 12 days after the initial attack, showed enlargement of the optic nerve, the chiasm, and the optic tract, especially on the left side, with marked peripheral rim enhancement on Tl-weighted images (Fig. 2). OPERATION A left sided frontopterional craniotomy was performed on March 16, 1991. The optic nerve, the chiasm, and the optic tract were swollen over the left side. A fine-needle aspiration was introduced into the bulging region of the left optic nerve. About 1.5 ml brownish fluid was drained out. The pia mater was longitudinally incised on the junction. Multiple biopsies were taken from the wall of the cystic cavity. The pathologic examination documented it as a "cavernous hemangioma." FOLLOW-UP After 1 year, the patient's visual acuity recovered to 20/25 in the right eye and counting fingers in the left eye. Pupillary reflexes were normal, but the optic disc of the left eye was pale. There was no distinct field defect in the right eye, but only peripheral quadrant of temporal upper field remained in the left eye. Follow-up brain CT scanning and MRI showed disappearance of suprasellar mass. DISCUSSION Acute visual changes resulting from a mass lesion within the pia sheath of the intracranial portion of the optic nerve were rarely recognized in the past. More cases have been reported in the literature recently, since the application of CT and MRI, and have been described as "chiasmal apoplexy" because intrinsic hemorrhage within such a mass lesion was found. Up to now, only 28 similar cases, mostly occur- FIG. 1. Left: Noncontrast computed tomographic scan on th~ sixth day shows a slightly hyperde~se mass in the suprasellar cistern with density representing hemorrhage. Right: Contrast computed tomographiC scan reveals significant enhancement of the lesion. I Clin Neuro-ophthalmol, Vol. 13, No.4, 1993 234 J-F. HWANG ET AL. FIG. 2. Magnetic resonance images on the 12th day shows a slightly low-signal lesion with peripheral rim enhancement of the anterior visual pathway on T1-weighted images. Top left: Sagittal view shows involvement of the optic chiasm. Top right: Coronal view shows involvement of the optic nerve, especially over the left side. Bottom: Axial view shows involvement of the left optic tract. ring in the third or fourth decade of age, have been reported. (1-22) Regli reviewed these cases and described the characteristic clinical symptoms as the following major triad (17): 1. Abrupt headache 2. Abrupt change of visual acuity 3. Significant visual field change We also noticed that more than one-third of the reported cases had previous episodes of transient blurred vision. These cases were easily misdiag- J Gin Neuro-ophthalmol, Vol. 13, No.4, 1993 nosed as retrobulbar neuritis and were delayed to have early surgical decompression. (12,19). Lateral skull radiographs always demonstrate a normal sella turcica. Carotid angiography, though, fails to reveal any vascular abnormality, yet remains essential to exclude an aneurysm. Brain CT scanning often shows a high-density, slightlyenhanced suprasellar mass. MRI will reveal a mass with different signal characteristics according to the different stages of hemorrhage. (23,24). All the reported cases had received craniotomy, APOPLECTIC OPTOCHIASMAL SYNDROME 235 REFERENCE FIG. 3. Sketch shows a mass is located within the left optic nerve, extending its compression across the chiasm and encroaching on the left optic tract. q 0 1. Vihlein A, Rucker CWo The neurosurgeon's role in acute visual failure. Arch OphthalmoI1958;60:223-9. 2. Holt H. Cysts of the intracrania! portion of the optic nerve. Am J OphthalmoI1966;61:226-30. 3. Schneider RC, Kriss FC, Fall HF. Prechiasmal infarction associated with intrachiasmal and suprasellar tumors. J Neurosurg 1970;32:197-207. 4. Riishede J, Seedorff HH. Spontaneous hematoma of the optic chiasma. Acta OphthalmoI1974;52:317-22. 5. Fermaglich J, Kattah J, Manz H. Venous angioma of the optic chiasm. Ann NeuroI1978;4:47Q-1. 6. Manz HJ, Klein LH, Fermaglich J, et aI. Cavernous hemangioma of optic chiasm optic nerve and right optic tract. Virchows Arch 1979;383:225-31. 7. Roski RA, Gardner JH, Spetzler RF. Intrachiasmatic arteriovenous malformation. J Neurosurg 1981;54:54Q-1. 8. Carter JE, Wymore J, Ansbacher L, et aI. Sudden visua! loss and a chiasma! syndrome due to an intrachiasmatic vascular malformation. J Clin Neuro-ophthalmoI1982;2:163-7. 9. Maitland CG, Abiko S, Hoyt WF, et aI. Chiasma! apoplexy. J Neurosurg 1982;56:118-22. 10. Lavin PJM, McCrary JA, Roessmann V, et aI. Chiasmal apoplexy: hemorrhage from a cryptic vascular malformation in the optic chiasm. Neurology 1984;34:1007-11. 11. Mohr G, Hardy J, Gauvin P. Chiasma! apoplexy due to ruptured cavernous hemangioma of the optic chiasm. Surg Neurol 1985;24:636-40. 12. Reilly P, Oatey PE. Optic nerve apoplexy. JNeurosurg 1986; 64:313--6. 13. Hankey GJ, Khangure MS. Chiasmal apoplexy due to intrachiasmatic vascular malformation rupture. Aust NZ J Med 1987;17:444-6. 14. Maruoka N, Yamakawa Y, Shimauchi M. Cavernous hemangioma of the optic nerve. J Neurosurg 1988;64:292-4. 15. Hufnagal TJ, Cobbs WHo Microangioma and optochiasmatic apoplexy. J Fr OphthalmoI1988;11:81-4. 16. Zentner J, Grodd W, Hassler W. Cavernous angioma of the optic tract. J NeuroI1989;236:117-9. 17. Regli L, Tribolet NO, Regli F, Bogousslavsky J. Chiasmal apoplexy: hemorrhage from a cavernous malformation in the optic chiasm. J Neuro Neurosurg Psychiatry 1989;52: 1095-9. 18. Hassler W, Zentner J, Petersen O. Cavernous angioma of the optic nerve. Surg NeuroI1989;31:444-7. which revealed asymmetric enlargement of the opos 00 tic nerve or chiasm to several times its normal diameter by a cystic lesion. Decompression by aspiration or longitudinal incision of the swollen area may disclose the cystic lesion containing mixed hematoma with fresh and/or old blood. Only 17 of the 28 reported cases had histopathologic confirmation. They were all documented to be intrinsic cryptic vascular malformations: 5 were small arteriovenous malformations (10,12,13,22), 10 were cavernous hemangiomas (6, 10,11,14,16--21), and 2 were venous angiomas (8,13). However, it is quite difficult and easily confused in histologic confirmation because many of the obtained specimens were destroyed during the hemorrhage. Our case showed a close relationship between alcohol drinking and visual changes. This finding suggests that alcohol may play an important role in provoking an occult cavernous hemangioma to be symptomatic. Other predisposing factors discussed in the literature include hormone effects during pregnancy, increased venous pressure during labor, and Valsalva's maneuver (10,14,23). Two mechanisms of compression by the intrinsic cavernous hemangioma can be presumed. The first mechanism is "occult hemorrhage" found in most lesions. There is MRI signal of fresh or old blood within the confines of the tumor. The symptoms may improve spontaneously as the blood or transudate within the tumor resolves. This explain why there were episodes of transient blurring in many of the reported cases. The second mechanism is "overt hemorrhage," a real apoplectic event of the cavernous hemangioma on the basis of the criteria that the MRI signal of acute or subacute blood outside the hemosiderin rim of the lesion, or evidence of blood outside the confines of the lesion during surgery (23). Only a few cases of cavernous hemangioma will lead to overt bleeding (23,25). But once it happens, there will be marked and more persistent symptoms, which are hard to remit. It, is obvious that primary focus of the vascular mass, causing visual failure of the left eye of our presenting case, was localized within the left optic nerve initially. Dramatic visual field change and CT scanning suggested that an overt hemorrhage had occurred and extravasated beyond the original confines of the mass (Fig. 3). MRI on day 12 revealed the hemorrhage had become a subacute state (Fig. 2) (24,26,27). No matter what the nature of the compression is, apoplectic or nonapoplectic, the intrinsic vascular tumor of the anterior visual pathway should have early surgical decompression and removal to preserve the vision as much as possible. J elin Neuro-ophthalmol, Vol. 13, No.4, 1993 236 J-F. HWANG ET AL. 19. Corboy JR, Galetta SL. Familial cavernous angiomas manifesting with an acute chiasmal syndrome. Am J Ophthalmol 1989;108:245--50. 20. Tien R, Dillon WP. MR imaging of cavernous hemangioma of the optic chiasm. J Comput Assist Tomogr 1989;13:1087~. 21. Castel JP, Delorge-Kerdiles C, Rivel J. Cavernous hemangioma of the optic chiasm. Neurochirurgie 1989;35:252~. 22. Lejeune JP, Hladky JP, Dupard T, et al. Opticochiasmatic apoplexy. Neurochirurgie 1990;36:303-7. 23. Robinson JR, Awad lA, Little JR. Natural History of the cavernous angioma. J Neurosurg 1991;75:709-14. 24. Requena I, Arias M, Lopez-Ibor L, et al. Cavernomas of the JClin Neuro-ophthalmol, Vol. 13, No.4, 1993 central nervous system: clinical and neuroimaging manifestations in 47 patients. J Neuro Neurosurg Psychiatry 1991;54: 590--4. 25. Del Curling 0 Jr, Kelly DL Jr, Elster AD, et al. An analysis of the natural history of cavernous angiomas. J Neurosurg 1991;75:702~. 26. Rigamonti D, Drayer BP, Johnson PC, et al. The MRI appearance of cavernous malformations (angiomas). JNeurosurg 1987;67:51S-24. 27. Thulbom KR, Atlas SW. Intracranial hemorrhage. In Atlas SW, ed. Magnetic resonance imaging of the brain and spine. New York: Raven Press; 1991:175--220. |
