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Show Journal of Nemo- Ophthalmology 16( 2): 99- 106, 1996. > 1996 Lippincott- Raven Publishers, Philadelphia Recurrent Chiasmal Apoplexy due to Cavernous Malformation Judith E. A. Warner, M. D., Joseph F. Rizzo III, M. D., Eugene W. Brown, M. D., and Christopher S. Ogilvy, M. D. We report a case of chiasmal apoplexy due to a cavernous malformation ( CM). Surgery was delayed because of the patient's advanced pregnancy, and nearly complete recovery of vision occurred spontaneously. Recurrent hemorrhage prompted surgical extirpation. The patient was left with a residual deficit. The literature pertaining to chiasmal apoplexy and CMs is reviewed. Nearly half of the reported cases of chiasmal CM had recurrent hemorrhages. The co- occurrence of hemorrhage from CM and pregnancy is too rare to merit any conclusions about cause and effect. Key Words: Chiasmal apoplexy- Cavernous malformation- Pregnancy- Recurrent hemorrhage- Surgery. Chiasmal apoplexy is a rare event, often caused by a vascular malformation. Patients typically present with sudden visual loss, often with headache. There are 29 cases in the literature of chiasmal apoplexy ( 1- 23), the majority attributable to cavernous malformations ( CM), which are low flow, angiographically occult, vascular malformations with no intervening neural tissue. We present a case of a pregnant patient who experienced recurrent visual loss and spontaneous recovery from a CM of the chiasm. The natural history of these lesions is reviewed, and the role of surgery is discussed. Manuscript received March 14, 1995; accepted October 20, 1995. From the Departments of Ophthalmology and Neurology, University of Utah, Salt Lake City, Utah ( J. E. A. W.); Departments of Ophthalmology ( J. F. R.) and Radiology ( E. W. B.), Boston, Massachusetts; and Harvard Medical School, Massachusetts Eye and Ear Infirmary, Department of Neurosurgery, Massachusetts General Hospital ( C. S. O.), Boston, Massachusetts, U. S. A. Address correspondence and reprint requests to Dr. Judith E. A. Warner, Assistant Professor of Ophthalmology and Neurology, Moran Eye Center, University of Utah, 50 North Medical Drive, Salt Lake City, UT 84132, U. S. A. CASE REPORT A 32- year- old right- handed legal secretary was 4.5 months pregnant when she developed " trouble seeing my computer." She was only able to see letters that were directly ahead of her, and she had to move her head around in order to read. There were no associated symptoms. Past medical history was not significant. Examination on the second day revealed visual acuity of 16/ 20 OD and 16/ 20 OS. She was able to read 12/ 12 Ishihara color plates OU, but reported " 10%" red desaturation OS. Amsler grids had a " fuzzy patch" inferotemperally OU. External examination, intraocular pressure, pupils, extraocular movements, dilated funduscopy, and neurologic testing were normal. Visual field testing showed an inferotemperal defect that respected the vertical meridian OD, and an inferotemperal defect that crossed the vertical meridian OS ( Fig- 1). Magnetic resonance imaging ( MRI) showed a 1.3 cm mass within the chiasm and enlargement of the adjacent optic nerves. The mass was of mixed signal on short Tl weighted images, with areas of hyperin- 99 100 J. E. A. WARNER ET AL. , t a8 a _ B ' 5 i . I H 11 B • • - » ' ' ' T" 1 7- f-ii n 12 „ IP. FIG. 1. Automated static perimetry, Humphrey 120 point program, performed on the day of presentation, demonstrating bitemporal defects. tensity and hypointensity. On T2 weighted images, the lesion was hypointense due to T2 shortening. This appearance was consistent with acute and subacute hemorrhage ( Fig. 2). Cerebrospinal fluid was clear and colorless without xanthochromia, with protein of 23 mg/ dl, glucose of 62 mg/ dl, 127 RBC/ mm3 and 2 WBC/ mm3. Cerebral angiography was normal. Surgery was planned, but was deferred because of concerns about the patient's advanced pregnancy. Over the next year, the patient's vision recovered completely with the exception of subtle bitemporal defects ( Fig. 3). She had an uneventful Cesarean section delivery. Serial MRIs showed that the chiasmal mass had become smaller, with resolution of the Tl bright, T2 dark areas ( Fig. 4). At 13 months after her initial presentation, a routine MRI showed evidence of subclinical rehemor-rhage. At 15 months after her initial presentation, she returned because of headache and a sudden change in her vision. The only change in her examination was increased density of the inferotem-peral defects, which crossed the midline OS. MRI showed evidence of a new hemorrhage, with extension above the chiasm ( Fig. 5). Over the next several days, she reported further decline in her vision OS, and neurosurgical intervention was undertaken. A left pteronial craniotomy was used to expose the left optic nerve and chiasm. The chiasm was enlarged and had a purple discoloration in the left superior aspect. A 3- mm incision was made over the abnormal area, clotted blood was aspirated, and a vascular malformation was revealed. Bipolar coagulation was used to shrink the lesion, allowing gross total resection. Pathologic evaluation revealed a CM, with many thin- walled sinusoids, evidence of new and old hemorrhage, and no intervening neural or glial tissue ( Fig. 6). Neuro- ophthalmic evaluations over the next 8 months revealed stable visual acuity of 16/ 15 OD and 16/ 70 OS, normal color vision, a left afferent pupillary defect, and dense bilateral inferotemp-eral field defects ( Fig. 7). The patient developed bilateral optic disc pallor. Follow- up MRI 2 months postoperatively showed almost complete resolution of the chiasmal lesion, with minimal residual T2 susceptibility and enhancement of the left optic nerve. The chiasm regained a near normal contour ( Fig. 8). DISCUSSION Vascular malformations of the anterior visual pathway include CM, ( cavernous angioma) venous angioma, and arteriovenous malformation ( AVM). There are 29 reported cases of chiasmal apoplexy, only 15 with pathology described as CM ( 2- 13,23,24). Some lesions are referred to as " venous angiomas" ( 1,14,16), but are probably more accurately classified as CMs because of the absence of intervening neural tissue. Several cases of chiasmal apoplexy are attributed to AVM ( 5,10,14,17, 22), a high flow vascular anomaly. Most of these had normal angiograms, more suggestive of CM. Some reports lacked details of pathology, but the / Neuro- Ophthalmol, Vol. 16, No. 2, 1996 CHIASMAL APOPLEXY AND CAVERNOUS MALFORMATION 101 descriptions are consistent with CM ( 18- 21). Table 1 gives an outline of the cases as well as some of the clinical and pathologic features. The course of the patient presented here is typical for a CM of the chiasm. Most patients present in their third or fourth decade with sudden visual loss. Men and women are affected equally. Typically, the events are apoplectic, but there have been several cases in which vascular malformations have acted as expansile mass lesions. For instance, Burnbaum ( 18) reported a man with progressive visual loss with apoplectic episodes. Pathology was not obtained, but the gross descrip- FIG. 2. MRI at initial presentation. A: T1- weighted sagittal image showing an enlarged hyperintense chiasm. B: T1- weighted coronal image showing the hyper- and hypointense mass within the chiasm. C: T2- weighted coronal showing marked hypointensity within the chiasm. tion of a " blue domed cyst" is typical of CMs seen at craniotomy. Malik and colleagues ( 24) described a 4- year- old boy with slowly progressive visual loss. The vessels in the vascular malformation were " thickened and hyalinized," somewhat atypical for CM, but other family members had more classic lesions. Steiger gives a convincing argument that cavernous angiomas can act as expansile mass lesions in the cerebral cortex, with slowly progressive neurologic deficit ( 25). The course of the patient presented here is instructive because of the well- documented relaps-ing- remitting course. Not surprisingly, misdiag- / Neuro- Ophthatmol, Vol. 16, No. 2, 1996 202 /. E. A. WARNER ET Ah. 30° : H h . 30" SO5 J 1- , r. v."" i r 30° gi-; FIG. 3. Automated static perimetry, Humphrey 30- 2 program, performed 1 year after initial presentation showing mild residual deficits. noses, such as optic neuritis ( 2,4,16,19), migraine ( 3), and retinal tear ( 1), have been made due to the pattern of abrupt visual loss with recovery. Of the 17 cases of CM or venous angioma, seven patients had two or more apoplectic events prior to surgery ( 1- 3,8,11,12,16). Of the 30 cases of chiasmal vascular malformations or hemorrhages, 12 had recurrent visual loss. In the recent literature, it is almost impossible to find a patient who is not taken im-t | at i\ i lWtttt 1 •- - !••>: I- £ w- • • - s i n r n ' i M T j^ f A fljP r ft T *** . X i • i i 1 t , B ^ ^ • ^ • i , ' < * t \ * r ^ j / mWl i % 1 r FIG. 4. T1- weighted MRI 1 year after presentation showing considerable resolution of the chiasmal enlargement, with some residual T1 hypointensity on coronal images. mediately to surgery. Steinberg ( 11) reported on one woman in whom the diagnosis of vascular malformation was made. She was observed for 18 months before visual deterioration prompted surgical extirpation. Our patient not only had a second episode of visual loss, but also, while visually stable, had evidence of fresh hemorrhage on routine MRI. There have been several analyses of large series' of patients with cerebral CM. One series of 57 patients with CM ( 26), with a mean follow- up of 26 months, reported clinically significant hemorrhage at presentation in seven patients. One patient had recurrent hemorrhage, but four had been treated surgically. Thus, one of three untreated patients presenting with hemorrhage had clinically significant rehemorrhage. Kattapong ( 27) reported a 2 month to 6- year follow- up interval. Of 14 patients with 67 lesions, four had growth in the malformation size and seven developed new lesions. Extrapolation from the literature to predict chiasmal CM behavior is problematic because of the small numbers of cases and limited clinical information. In these series, however, the tendency for recurrent hemorrhage or expansion of the mass is evident. The chiasm is unusual in that, frequently, the patient is acutely aware of minor fluctuations in vision. Serial visual field testing allows for precise documentation of progression and regression. If the natural history of CM is to rebleed at a low rate, perhaps a CM in the chiasm will cause a more clinically apparent deficit with a lower threshold. As our patient experienced, visual deficit can accrue with successive hemorrhages. Evidence of acute and chronic hemorrhage is characteristic in pathologic and radiologic studies / Neuro- Ophthalmol, Vol. 16, No. 2, 1996 CHIASMAL APOPLEXY AND CAVERNOUS MALFORMATION 103 FIG. 5. T1 weighted ( A) coronal and ( B) axial ( with gadolinium) MRI images 15 months after presentation, showing recurrent hemorrhage. of CMs ( 12,25,28,29). Earlier neuro- imaging methods were insensitive to this diagnosis because of the presence of a hemorrhagic mass on imaging studies, with angiographic silence. Routine angiography does not reveal the vascular nature of CMs. Modern neuro- imaging permits an accurate diagnosis at first presentation. MRI is the preferred imaging technique due to its capability to show old and new blood products ( 11,28). Demonstration of high flow within the lesion raises the possibility of AVM. In 1976, Voight and Yasargil ( 30) suggested removal of surgically accessible cortical CMs. Many cortical lesions can be removed with low morbidity, given current stereotactic methods. Some centers use lasers to coagulate and shrink lesions, attempting to minimize damage to surrounding structures ( 11). As surgical techniques develop, the definition of " surgically accessible" becomes broader, and most CMs in the chiasm reported in recent years have been treated with surgical extirpation. There are no reports of long- term follow- up of patients operated on for chiasmal CMs, but resection is thought to be curative. Most patients who have undergone surgery for CM either improved or remained unchanged postoperatively ( see Table 1). Decompression of the hematoma is thought to be the mechanism of early improvement. In reviewing surgical results, the outcome is somewhat dependent upon the nature of the lesion. High- flow malformations seem to have a greater potential for recurrent hemorrhages. Lavin FIG. 6. Photomicrograph ( x10) showing typical CM with dilated thin- walled sinuses. No intervening neural or glial tissue is seen. J Neuro- Ophthalmol, Vol. 16, No. 2, 1996 104 }. E. A. WARNER ET AL. 3U'- • • l l * ' f t M p ; <%% S • •-•': *' i :•'•: • fl^^ ilif^ S!;^ , : : ; ; : : ; : : : : : . : ; : ; t ^ < ^ ? ^ a FIG. 7. Automated static perimetry, Humphrey 30- 2 program, 8 months postoperatively, showing residual defects OU. et al. ( 5) reported on one patient in whom a high-flow chiasmal AVM was operated on after two episodes of visual loss. The patient had several subsequent events that prompted a second attempt at resection. Tien and colleagues ( 12) reported on a patient who had progressive visual loss following biopsy of a CM, suggesting that gross total resection should be the goal. The influence of pregnancy on the behavior of vascular malformations is controversial. Robinson and colleagues ( 31,32) reviewed the relationship FIG. 8. MRI 2 months after surgery showing resolution of the chiasmal enlargement on T1 coronal images. Minimal gadolinium enhancement is seen at the operative site. between subarachnoid hemorrhage, aneurysm, AVM, and pregnancy, and found that the risk of hemorrhage was increased during pregnancy. Pregnancy was thought to increase the rate of hemorrhage from AVMs, particularly during " high risk" times of early pregnancy, labor, and delivery. The increased risk was believed to be due to increased cardiac output. Recently, more conservative estimates ( 33) have not shown an increased risk of hemorrhage from AVM during pregnancy. This information may or may not be relevant for CMs. Only three instances of hemorrhage from chiasmal CMs associated with pregnancy have been reported. Maruoka et al. ( 7) reported on a woman with chiasmal apoplexy occurring 10 days postpartum. Reilly and colleagues ( 10), however, found a CM of the optic nerve and chiasm causing sudden visual disturbance in a 36 year- old woman who had been " either pregnant or breast- feeding for the preceding 8 years." Thus, she was " at risk" and asymptomatic for many years before the apoplectic event. One of the series of cortical CM ( 26) found that two of six women presenting with overt hemorrhage were in the first trimester of pregnancy. Given the age group of patients typically affected by CM, this may not be significant. Our patient had her first hemorrhage while in her fourth month of pregnancy, not generally considered a high- risk period. Her second symptomatic hemorrhage occurred 10 months after she delivered. These cases do not confirm or refute an association between pregnancy and the risk of hemorrhage from a chiasmal CM. In summary, this case illustrates several interesting points about chiasmal CMs. First, substantial / Neuro- Ophthalmol, Vol. 16, No. 2, 1996 CHIASMAL APOPLEXY AND CAVERNOUS MALFORMATION 105 TABLE 1. Summary of reported cases of chiasmal apoplexy and vascular malformations No. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 Author ( ref) Carter ( 1) Corboy ( 2) Hassler ( 3) Hwang ( 4) Lavin ( 5) Lavin ( 5) Lavin ( 5) Manz ( 6, 15) Maruoka ( 7) Mohr ( 8) Regli ( 9) Reilly ( 10) Reilly ( 10) Steinberg ( 11) Steinberg ( 11) Tien ( 12) Zentner ( 13) Hankey ( 14) Hankey( 14) Hufnagel( 16) Roski( 17) Burnbaum ( 18) Holt ( 19) Maitland ( 20) Maitland ( 20) Maitland ( 20) Riishede ( 21) Malik ( 24) Lejeune ( 22) Castel ( 23) Initial Dx Retinal tear Optic neuritis Migraine Optic neuritis Chiasmal Syndrome vs hysteria Optic neuritis Age 48 44 24 42 43 37 27 30 24 30 28 31 36 58 33 32 35 26 36 39 37 43 33 26 23 63 20 4 26 23 Sex F F F M M M F M F M F F F M F F M M F F M M M M F F F F F F Malformation type VA CA CA CH CA AVM, angio(-) AVM, angio(-) CH/ VA CH CH CA AVM, angio (-) CA CA CA CH CA ( tract) VA AVM, angio (-) VA AVM, angio(-) Blue domed cyst, blue fluid Bulging chiasm, filled cyst Blue domed cyst Enlarged blue ch blood Mass in chiasm, i blood Enlarged brown i clotted blood , viscous amber fluid , blood iasm, clotted chiasm, CA " hyalinized thickened vessels" AVM CA No. of events ( interictal period) 2 ( 3 yr) 3 ( 7 yr, 2 yr) 2 ( 3 yr) 1 1 5 ( 6 mo, 30 mo, 2 wk, 12 mo) 1 1 1 > 3 ( 18 mo, 2 mo, 2 mo) 1 1 1 1 2 ( 18 mo) Fluctuation for 9 yr 1 1 1 3 ( 7 yr, 2 yr) 2 ( 6 mo) 2 ( 23 mo) 3 ( 1 mo, 24 mo) 1 1 Unknown Unknown Progressive 1 1 Outcome Improved after surgery No change after surgery Worsened visual field defect after surgery Improved after fine needle aspiration No changes OS, worse OD after surgery 2 improved after surgery, 3 improved spontaneously Improved after surgery Improved after surgery No change after surgery Improved after survery Improved after surgery Improved after surgery Improved after surgery No change after surgery Improved after surgery Radiation decreased fluctuation Improved after surgery Surgery, no follow- up Improved after surgery Improved after surgery Improved after surgery Improved after aspiration Aspiration, radiation, deterioration 2 yr later ( optic neuritis?) Improved after surgery Improved after surgery No information Improved after surgery No change after surgery Improved after surgery No change after surgery AVM, arteriovenous malformation; CM, cavernous malformation; CA, cavernous angioma; CH, cavernous hemangioma; VA, venous angioma. improvement in vision may occur spontaneously, probably due to resorption of a small hematoma. Second, these lesions have a tendency to recurrent hemorrhages, often within 1- 2 years. Third, surgery can improve or maintain vision, although there is not enough follow- up data to know whether surgery prevents recurrence. Surgical improvement is predicated on pathology consistent with CM, since high- flow AVMs may be more likely to rebleed. Finally, there is insufficient evidence to confirm an association between pregnancy and hemorrhage from chiasmal CMs. Acknowledgment: Supported, in part, by a grant from Research to Prevent Blindness, Inc., New York, NY, U. S. 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