J. Clin. Neuro-ophth'llmol. 2: 103-107, 1982.
Sudden Visual Loss and a Chiasmal Syndrome Due to an Intrachiasmatic Vascular Malformation
JOHN E. CARTER, M.D. JAMES WYMORE, M.D. LINDA ANSBACHER, M.D. WILLIAM S. REID, M.D.
with this impression, and cryosurgery was per Abstract formed. A 48-year-old woman presented with a chief com A routine exam in May 1980 was normal except plaint of visual loss and bitemporal hemianopia. A for the retinal break which was sealed with cryo similar episode of temporal visual field loss had oc therapy scars. Perimetry was not carried out at that curred 34 months earlier and spontaneously resolved. time. Computerized tomography demonstrated a suprasellar The patient experienced no further visual symp mass. Angiography was normal. At surgery a venous angioma of the optic chiasm with a fresh hematoma toms nor were there any headaches or other med was found and removed. Intrachiasmatic vascular mal ical complaints until November 1980, when she formation is an unusual finding which should be con awoke one morning and found that a shadow had sidered in the differential diagnosis in patients with a appeared in the temporal visual field of each eye. chiasmaI syndrome. When she was reexamined by her ophthalmologist the visual acuity in each eye was 20/200 and there was a bitemporal hemianopia on confrontation testing. Automated static perimetry verified the Intrachiasmatic vascular malformations repre bitemporal visual field defects (Fig. 1). There were sent an unusual cause of acute visual loss. We no other neurological complaints and no significant present a case of venous angioma of the optic past medical history. Neurological examination chiasm. was normal except for the visual deficit. The patient underwent CT scanning which was Case Report initially interpreted as being normal. When the scan was repeated with a different angle to the A 48-year-old woman first presented to her oph baseline and coronal views, a small lesion in the thalmologist in January 1978, complaining of a region of the optic chiasm was seen (Figs. 2-4). shadow which had appeared in the temporal visual Arteriography was performed and was normal. field of the right eye 6 weeks previously and then The patient underwent a frontotemporal crani resolved gradually. Visual acuity, pupillary reac otomy. At surgery the chiasmal region including tions, and confrontation visual fields were normal the right internal carotid artery, the A-I segments but perimetry was not carried out. Funduscopy of both anterior cerebral arteries, and the anterior was normal except for the presence of a horseshoe communicating artery were visualized and normal. tear in the temporal periphery of the left eye. This The chiasm was swollen and markedly discolored tear was thought to be the cause of the transient by what appeared to be a hematoma within the visual symptom. Confusion regarding which eye substance of the chiasm itself (Fig. 5). As the pia of was involved was attributed to the 6-week time the dorsal surface of the chiasm was opened, a lapse or to the right visual field symptoms expected hematoma began to extrude spontaneously. When from the temporal retinal location of the tear in the the hematoma had been completely cleared there left eye. A retinal surgeon was consulted, agreed were abnormal blood vessels seen within the chiasm which had the appearance of a small vas cular malformation. The vascular malformation From the Departments of Neurology (JEC). Ophthalmology was removed (Fig. 6). GEe, JW). and Pathology (LA), University of Missouri Health Sciences Center, and the Division of Neurosurgery, University The pathologic specimen (Fig. 7) showed dilated of Tennessee Hospital. Knoxville, Tennessee (W5R). thin-walled vascular channels with mild gliosis in
September 1982 163 Figure 1. Bitemporal visual field defect demonstrated preoperatively by perimetry performed with a Fieldmaster perimeter.
Figure 2. N(llll'Ilh.111ll'd IT ''''.In d,'m"",,tr.lli"!\ m.",,, 1"",,)11 III Figure 3. With contrast, the suprasellar mass is seen to enhance. thl' ,upr,,,dl.lf ,i,tl'rn.
Journal of Clinical Neuro-ophthalmology Clfter, Wymore, Ansbacher, Reid
Figure 4. Cor,'n.t) section .llso dem,'nstr.ttes .tn enh.tncing su prdselldr mdSS. The line.lr densities e>.lending I.lter.llly from eithE'r side of the m.tss .tre the middle cerebr.tl .trteries visible due 10 Ihe contr.tst injecti,'n.
Figure 6. Following surgicdl removdl of the v.tsculdr malfor m.ttion .t cdvity c.tn be seen within the chidsm which extends towJrd the right optic nerve.
adjacent tissue. No arteries were seen and there were no arteriolized veins found. A diagnosis of venous angioma was made. Immediately postoperatively, the patient noted subjective improvement in her visual fields. When she was seen by her ophthalmologist 7 weeks after surgery the visual acuity was finger-counting at 2 ft in the right eye and 20/20 in the left eye. Little change had occurred in the automated perimetry performed on the right eye but the lower quadrant of the temporal field of the left eye w.\s now int.let (Fig. 8).
Discussion In 1951, Margolis et .11.' called attention to sm.1ll angiomatous malformations .1S a source of intra cerebral hematomas. In 1050, Crawford and Rus sell~ first applied the trrm "cryptic v
September 1982 165 Intr,1Chi,l~m,ltic V,lsculJr MJlformJtion
Figure 7. Four dilated, thin-walled vessels are pictured. surrounded by hemorrhagic stroma. The dark material in the left comer represents fibrin. (H & E. reduced from x250.)
Figure 8. Poslopcr,lliv(' visual fidds p('rform('d wilh ,I Fi('ldmasl('r perimeter show minimal change in the field of the right eye but ('nIMg('m('nt of Ih(' I('mporal fi('ld and int,let (('nlral vision in th(' left eye.
Journal of Clinical Neuro-ophthalmology Carter, Wymore, Ansbacher, Reid
Cryptic vascular malformations may be catego after her first episode of visual loss are strongly rized as capillary telangiectasias, venous angiomas, suggestive of a vascular disease process, although or arteriovenous malformations. Unlike large ar this presentation might be confused with demye teriovenous malformations which are found pre linating optic neuritis which involved the optic dominantly supratentorially, cryptic vascular mal chiasm. The differential diagnosis in any patient formations have an equal incidence above and presenting with a chiasmal syndrome, with or with below the tentorium.4 out headache, should include intrachiasmatic vas Intrinsic vascular malformations of the optic cular malformation. chiasm are rare and have been reported only twice 5 6 before. . Both patients were in their 30s and both presented with a chief complaint of visual loss and References headache. In one case" the patient described 3 I. Margolis, G., Odom, G., Woodhall, B., and Bloor, weeks of progressive visual loss in one eye and B.: The role of small angiomatous malformations in was found to have a central scotoma with a tem the production of intracerebral hematomas. 1. Neu poral visual field defect in the asymptomatic eye. rosurg. 8: 564-575, 1951. The other case6 had two episodes of visual loss and 2. Crawford, J.V., and Russel, D.5.: Cryptic arteriove headache over a 6-month period of time. The first nous and venous hamartomas of the brain. 1. Neurol. episode had cleared. At the time of the second Neurosurg. Psychiatry 19: 1-11, 1956. 3. Terao, H., Hori, T., Matsutani, M., and Okeda, R.: episode he had a mild loss of acuity in one eye and Detection of cryptic vascular malformations by com a bitemporal visual field defect. In both of these puterized tomography. 1. Neurosurg. 51: 546-551, cases angiography was normal but computerized 1979. tomography demonstrated the lesion. 4. McCormick, W., and Nofzinger, J.: "Cryptic" vas Hemorrhage into the chiasm has been reported cular malformations of the central nervous system. in an additional seven7-9 cases and termed 1. Neurosurg. 24: 865-875, 1966. "chiasmal apoplexy" by Maitland et al.9 In two of 5. Fermaglich, J.. Kaffah. J., and Manz. H.: Venous the cases/·9 the source of the hemorrhage was a angioma of the optic chiasm. Ann. Neurol. 4: chiasmatic glioma. The source of the hemorrhage 470-471, 1978. in those cases not associated with tumor has been 6. Roski, R., Gardner, J., and Spetzler, R.: Intrachias al.,~ matic arteriovenous malformation. 1. Neurosurg. 54: identified only in the cases of Fermaglich et 540-541, 1981. Roski et .11.,6 and the present case. In the remainder 7. Schneider, R.C, Kriss, F.C, and Falls, H.F.: Prechias of the cases the source of bleeding has been as mal infarction associated with intrachiasmal and sumed to be venous or cryptic vascular malfor suprasellar tumors. 1. Neurosurg. 32: 197-208,1970. mations although no abnormal vessels were seen 8. Riishede, L and Seedorff, H.H.: Spontaneous he at the time of surgery. matoma of the optic chiasma. Report of a case. Acta Although chiasmal hemorrhage may present Ophthalmol. 52: 317-322, 1974. with a sudden onset or a more subacute course, 9. Maitland, CG., Abiko, 5.. Hoyt, W.F., et .11.: Chias headache is an almost invariable symptom in pre mal apoplexy. Report of four cases. 1. Neurosurg. 56: Viously published cases. The subject of the present 118-122, 1982. report did not have headache associated with either of her episodes of visual loss. She shared other features with the earlier cases including normal angiography with diagnosis being possible only Write for reprints to: John E. Carter, M.D., Depart through the use of computed tomography. The ment of Neurology, University of Missouri Health Sci sudden onset and the recovery which occurred ences Center, Columbia, Missouri 05212.
September 1982 167