Journal of Neurology, Neurosurgery, and Psychiatry 1989;52:1095-1099 Short report Chiasmal apoplexy: haemorrhage from a cavernous malformation in the optic chiasm LUCA REGLI,* NICOLAS DE TRIBOLET,* FRANCO REGLI,** JULIEN BOGOUSSLAVSKY** From the Departments of Neurosurgery* and Neurology**, University Hospital, Lausanne, Switzerland SUMMARY We present a patient who experienced sudden onset of orbital headache, visual loss and bitemporal visual field defect. MRI of the optic chiasm suggested a diagnosis of haemorrhage and hence a vascular malformation. Pterional craniotomy revealed an intrachiasmatic haematoma with a cavernous angioma. The malformation was totally excised and vision improved after surgery. The syndrome of chiasmal apoplexy is discussed. Visual field defects due to chiasmal compression from a tumour usually progress slowly. An acute syndrome may be due to ischaemic, haemorrhagic or demyeliniating disease. "Chiasmal apoplexy" is essentially associated with haemorrhage in the chiasm itself and is typically of sudden onset. We report a case of spontaneous haematoma in the optic chiasm from a cavernous malformation which could be suspected on magnetic resonance imaging (MRI). The clinical and radiological features of 16 cases reported previously are discussed, only our patient had MRI. no pupillary defect. Computerised tomography showed a high-density, poorly enhanced mass in the anterior part of the right optochiasmal cistern (fig 2). The carotid angiography was normal. MRI, performed three days after onset, showed enlargement of the chiasm on the right side by an intrachiasmal lesion with a mixed hypo- and a slight hyperintense signal, when compared with grey matter, on mildly Tland proton-weighted images (figs 3 and 4). Operation A 28 year old healthy woman suddenly complained of right sided supra- and retro-orbital headache, blurred vision on the right eye and a visual defect on both temporal fields. This was the first episode and she had no other complaints. A diagnosis of chiasmal apoplexy was made and a right sided pterional craniotomy was performed to explore the chiasm. The anterior and right paramedian parts of the chiasm were distended by haemorrhage and protruded into the optochiasmal cistern. The pia-mater was incised on the chiasm and a characteristic "mulberry-like" vascular malformation was apparent. A dissection plane separating the haematoma and the surrounding gliosis enabled a complete excision of the malformation. It measured six mm in diameter. A histological examination showed a typical cavernous angioma Examination Physical examination was entirely normal four hours after onset, except for her vision. Visual acuity was 1/60 in the right eye and 20/20 in the left eye. Her visual field revealed a dense right temporal hemianopia and a relative left temporal hemianopia (fig 1). The optic fundi were normal. There was Post-operative course One month after the operation the visual acuity was 20/20 on the right eye and 20/10 on the left eye and the visual field defect also improved (figure 1). Three months after the operation she returned to work. Case report Correspondence to: Dr N de Tribolet, Department of Neurosurgery, University Hospital, Lausanne, Switzerland Received 23 December 1988. Accepted 11 April 1989 (figure 5). Discussion Maitland introduced' the term "chiasmal apoplexy" for sudden retro-orbital or frontal headache associated with abrupt visual loss by acute bleeding in 1095 Regli, de Tribolet, Regli, Bogousslavsky 1096 225_ 225 240 255 202 285 300 220 255 270 285 3002 240 255 2i0 285 Fig 1 Visualfield defect 4 hours after the haemorrhage (upper): bitemporal hemianopia. Visualfield defect 3 months after surgery (lower): relative right temporal quadrantanopia. the optic chiasm. Our case reflects the syndrome's typical triad: (1) abrupt retro-orbital and frontal headache; (2) abrupt loss of visual acuity; (3) visual field defect of chiasmal type. Sixteen similar cases'-" have been reported in the literature. Females and males were equally affected, mostly during the second and third decade of life. Headache was mostly of sudden onset, retro-orbital, frontal or sometimes temporo-occipital9 and was usually the first symptom. Patients typically complained of visual blurring and some temporal visual field defect. They all had diminished visual acuity and visual field defects were either characteristic of a chiasmal lesion (temporal hemianopia or quadrantanopia some associated with scotomas) or of a junctional defect. Palor of the optic disc and relative afferent pupillary defect were often found. Four patients had xanthochromic CSF indicating bleeding into the subarachnoid space'5912 and two became stuporose.9 12 Recurrent haemorrhage was clearly established in three patients.3 689 One experienced five acute episodes over a period of four years. Only the present case was investigated with MRI, the most sensitive and specific imaging method for cavernous malformations of the CNS.'s The coronal and sagittal slices on MRI clearly showed enlargement of the optic chiasm, predominantly on the right side (figs 3 and 4). Three days after the haemorrhage, the mixed hypo- and slightly hyperintense signals (compared with the grey matter), on Tl- and protonweighted images suggested an acute haemorrhage. The lack of evidence of either tumour parenchyma or peritumoural oedema was suggestive of vascular malformation.'2 There are few reports of MRI with lesions of the chiasm.'3 14 In one report a haemorrhage into the chiasm was secondary to chiasmal glioma.'3 Chiasmal apoplexy: haemorrhagefrom a cavernous malformation in the optic chiasm 1097 6. 'K., Iz. . . _ F _ _ jq Fig 3 MRI three days after haemorrhage (sagittal section), mildly Tl-WI (TR:500/TE:40): enlargment ofthe optic chiasm (arrows) by an acute intrachiasmal haematoma with mild peripheral hyperintensity and central hypointensity (compared with grey matter). formation.'617 Cavernous malformations are characterised by large sinusoidal vascular spaces which are not separated by parenchyma. The walls of these dilated channels are thin with no smooth muscle or elastic tissue and are prone to rupture and lead to severe haemorrhage. Visual defects caused by intracranial vascular malformations are usually * I Fig 2 One day after haemorrhage (coronal section): highdensity, poorly enhancing, mass in the right optochiasmal cistern (upper: unenhanced; lower: enhanced). CT scans were performed in 14156"` of the 17 reviewed cases of chiasmal apoplexy. All showed a high-density, variably enhancing, mass in the suprasellar region. Subarachnoid haemorrhage was not seen. Carotid angiography (17 patients) demonstrated no vascular abnormalities except for an ectatic vein near the chiasm in one,' a vascular density in the suprasellar region in another9 and a suprasellar mass in 3 cases." ' Angiography remains essential to exclude an aneurysm. Vascular malformations are commonly divided into five types: telangiectasias, varix, arteriovenous malformation (AVM), venous malformation, cavernous mal- V Fig 4 MRI three days after haemorrhage (coronal section), proton-WI (TR:2000/TE:40): enlargment of the optic chiasm (arrows). Mixed hypo- and slight hyperintense signal (compared with grey matter). 1098 Regli, de Tribolet, Regli, Bogousslavsky Demyelinating chiasmal lesions are described in ,4 _ patients with multiple sclerosis.23 D l:: .All patients reported underwent a frontal, frontotemporal or fronto-parietal craniotomy. One patient had a transphenoidal pituitary exploration for suspected pituitary apoplexy and then had a frontal X 4 iUraM X craniotomy.'° allThe chiasm, the adjacent optic tract or nerve were all clearly swollen by a haematoma or a cystic lesion %1k A,containing old blood. After incision, distention of the ~~~ f ~ ~ Lamnerve was less and visual deficit improved in 11I of the 16 patients. We think that surgical decompression is most effective if done early. We conclude that "chiasmal apoplexy" due to acute intrachiasmatic haemorrhage produces a characteristic clinical picture (headache, diminished visual acuity and visual field defect). CT-scan can identify a haemorrhage in the optic chiasm, but the superior specificity and sensitivity of MRI makes it a more Fig 5 Photomicrograph of the cavernous malformation (6 powerful examination. Rapid surgical decompression, mm in diameter). Note the sinusoidal, thin-walled vascular with excision of the vascular malformation to avoid spaces and the partial thromboses. No neural parenchyma is recurrence, may improve or even restore vision. present between the vessels. Hand E staining, x 25. We thank Dr Antoine Uske for the radiological associated with occipital and and Dr Judith Miklossy for the pathological images or with extrinsic compression of the chiasm, Optic material. nerve or tract by such abnormalities. Of the 17 reported cases, 10 had histological -confir- Referenc mation of an intrachiasmatic vascular malformation, I Maitland CG, Abiko S, Hoyt WF, Wilson CB, Okamura T. six presented with coagulated or liquefied blood in a malformation Chiasmal apoplexy. Report of four cases. J Neurosurg 1982; a vascular to have were but thought cyst 56:118-22. that was destroyed by the haemorrhage and one was a 2 Uihlein A, Rucker W. The neurosurgeon's role in acute visual failure. AMA Arch Ophtal 1958;60:223-9. haemangioendothelioma. Of the 10 malformations, four were AVMs, two were venous angiomas and four 3 Holt H. Cysts of the intracranial portion of the optic nerve. Am J Ophthalmology 1966;61:1166-70. were cavernous angiomas. haematoma of the optic The diferenial dagnoss of an a abrupt abrut "chasmal The differential diagnosis "chiasmal 4 Riishede chiasma.J, Seedorff case. Acta Ophthalmologica Report ofHH.a Spontaneous 1974;52: 317-22. syndrome" includes: 1) ischaemic, 2) haemorrhagic, 3) ischaemic and haemorrhagic and 4) demyelinating 5 Fermaglich J, Kattah J, Manz H. Venous angioma of the optic chiasm. NeurolJH, 1978;4:470-1. diseases. Ischaemia may originate from extrinsic com- 6 Roski RA,Ann Gardner Spezler RF. 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