AUTHOR(S): Ferreira, Nelson P., M.D.; Ferreira, Marcelo P., M.D. Institute of Neurosurgery, Porto Alegre, Brazil Neurosurgery 30; 262-264, 1992 ABSTRACT: A case of a cavernous angioma of the optic nerve is presented. The abrupt onset of monocular visual symptoms was accompanied by an intense bitemporal headache, indicating apoplexy of the optic nerve. The surgical and histological findings demonstrated a cystic cavernous angioma. The lesion was removed completely without any noticeable bleeding. The preoperative visual deficit persisted. KEY WORDS: Cavernous angioma; Computed tomography; Optic nerve apoplexy Acute dysfunction of the chiasma and/or optic nerve has been called chiasmatic apoplexy (6) and/or optic nerve apoplexy (12). Both are rare events (5). They may be a result of an intratumoral hemorrhage such as occurs in hypophyseal tumors and gliomas (6,14). More recently, cases have been reported in which apoplexy is caused by arteriovenous malformations (AVMs) and optochiasmatic cavernomas (1,4-8,12,13). This report concerns a boy who presented with optic nerve apoplexy caused by a cavernous angioma of the optic nerve. CASE REPORT This 8-year old boy had been normal until June 1983, when he suffered the acute onset of a headache that lasted a few days and improved with analgesics. On June 21, 1984, a second episode of an intense, bitemporal, pulsatile headache occurred, accompanied by vomiting, left visual disturbance, and photophobia. On July 14, 1984, he was hospitalized because of reduced visual acuity (right eye, 1,0; left eye, fingers at a 2 m), loss of nasal field, and atrophy of the left papilla (Fig. 1). A computed tomographic (CT) scan demonstrated a hyperdense suprasellar mass with greater projection to the left (Fig. 2). The angiogram showed an elevation of the A1 portion of the left anterior cerebral artery. The mass was not enhanced by contrast material. The patient underwent a right frontotemporal craniotomy. After elevation of the right frontal lobe and the removal of the arachnoid that covers the nerves and optic chiasm, a wine-colored, encapsulated mass was identified, located below the left optic nerve and the optic chiasm (Fig. 3A). When punctured, the mass produced dark blood. The incision in the capsule produced blood of the same color, and the capsule remained, with no stroma. The tumor extended under the chiasm and the left optic nerve, between the latter and the carotid artery on the
same side, with a small temporal expansion (Fig. 3B). The capsule tissue was removed carefully without bleeding, preserving the contiguous anatomical structures. The histological section showed fragments of loose connective tissue and nervous tissue among which were many vascular spaces with irregular shapes of different sizes, generally with thin walls, and little or no smooth muscle (Fig. 4A). Occasionally, vessel walls appeared thickened by degenerative changes or by the organization of laminar thrombi. Some broad lumina contained unorganized thrombi. Fragments of the cystic cavity showed structures similar to those of the other vessels (Fig. 4B). In the adjacent tissue, hemosiderin-loaded macrophages had accumulated. The postoperative course was uneventful; 6 months later a tomographic study showed nothing abnormal. The ophthalmic examination performed at that time showed the same abnormalities that were present before the operation. DISCUSSION Optochiasmatic apoplexy is characterized by the abrupt onset of symptoms with loss of vision and reduction of the visual field associated with a predominantly retroorbital headache (6). The name "chiasmatic apoplexy" was used initially by Maitland et al. (6). They reported 4 clinical cases with 3 AVMs and 1 glioma of the optic chiasm. Reilly and Oatey (12) introduced the term "optic nerve apoplexy" and described 2 cases of a hematoma in the optic nerve; an AVM was identified in 1 case. The occurrence of optochiasmatic apoplexy caused by a histologically demonstrated cavernous angioma has not been described often. Lejeune et al. (5), in a review of the literature, found only 7 reported cases. In all of them, the cavernoma affected the optic nerve and chiasm. In our patients, the clinical syndrome was monocular on the left side, coinciding with the location of the cavernoma in the left optic nerve. Most of the patients in the study of Lejeune et al. (5) were between 20 and 40 years of age. Our patient, who was 8 years old at the time of the diagnosis, is the youngest. Other cases of cavernous angioma in the optic nerve, in which no apoplexy occurred, have been reported (7, 16) . In diagnosing cavernous angiomas, the angiogram shows mass effect and rarely demonstrates abnormal circulation (9,17). The CT scan presents a hyperdense image, with or without calcification, and other possible causes are not excluded (11). At present, magnetic resonance imaging offers the best possibility of describing the pathological features (8, 15) . Surgical treatment is justified for 1) diagnostic purposes, 2) decompression of the optic nerve and/or chiasm, and 3) prophylaxis of lesion growth, new hemorrhages, and consequent deterioration (8,10,12,16). In our patient, there was a history suggesting two episodes of bleeding. The visual symptoms occurred during the second. A wine-colored mass, limited by a capsule that contained dark blood, without a stroma and forming a voluminous hematic cyst was found at surgery. The
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 February 1992, Volume 30, Number 2 262 Optic Nerve Apoplexy Caused by a Cavernous Angioma: Case Report Case Report
cyst adhered to the lower aspect of the left optic nerve and extended to the suprasellar region, pressing between the internal carotid artery and the optic nerve. The hematic cyst capsule was removed without further bleeding, indicating that there were no arterial feeders. This aspect of a cavernous angioma has been observed by others (2,3,18). No hemorrhage was seen in the optic nerve. Possibly, the acute visual disturbance was a result of bleeding inside the cyst. Optic atrophy is a sign of long-standing compression. The histological appearance was typical of a cavernous angioma. There are reports of improved visual function after surgical treatment, and some advise immediate surgery (8). In our patient, the visual deficits persisted. ACKNOWLEDGMENT We are grateful to Geraldo Geyer, M.D., for the pathological study.
REFERENCES: (1-18) 1. 2.
3. 4.
5.
6. 7. 8.
9.
Castel JP, Delorge-Kerdiles C, Rivel J: Angiome caverneux du chiasma optique. Neurochirurgie 35:252-256, 1989. Iplikçioglu AC, Benli K, Bertan V, Ruacan S: Cystic cavernous hemangioma of the cerebellopontine angle: Case report. Neurosurgery 19:641-642, 1986. Khosla VK, Banerjee AK, Mathuriya SN, Mehta S: Giant cyst cavernoma in a child. Case report. J Neurosurg 60:1297-1299, 1984. Lavin PJM, McCrary JA III, Roessmann U, Ellenberger C Jr: Chiasmal apoplexy: Hemorrhage from a cryptic vascular malformation in the optic chiasm. Neurology 34:1007-1011, 1984. Lejeune JP, Hladky JP, Dupard T, Parent M, Hache JC, Christiaens JL: Apoplexie optochiasmatique. Neurochirurgie 36:303-307, 1990. Maitland CG, Abiko S, Hoyt WF, Wilson CB, Okamura T: Chiasmal apoplexy. Report of four cases. J Neurosurg 56:118-122, 1982. Maruoka N, Yamakawa Y, Shimauchi M: Cavernous hemangioma of the optic nerve. Case report. J Neurosurg 69:292-294, 1988. Mohr G, Hardy J, Gauvin P: Chiasmal apoplexy due to a ruptured cavernous hemangioma of the optic chiasm. Surg Neurol 24:636-640, 1985. Orenstein D, Kiesman M, Dietemann JL, Krause D, Maitrot D, Buchheit F, Marescaux C, Wackenheim A, Tongio J, Froidevaux JL: Les malformations vasculaires angiographiquement muettes évocatrices d'un cavernome: Etude clinique de 18 cas. Neurochirurgie 35:95-97, 1989.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Received for publication, June 12, 1991; accepted, July 30, 1991. Reprint requests: Nelson Pires Ferreira, M.D., Av. Independência, 1211 conj. 301, Porto Alegre, RS, Brazil 90210.
Figure 2. Contrast-enhanced CT scan shows a homogeneously enhanced suprasellar mass with greater projection toward the left side.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 1. Visual field demonstrates a nasal defect on the left side.
Figure 4. A, photomicrograph of the surgical specimen shows irregular blood vessels with rudimentary walls, some of them containing unorganized thrombi (hematoxylin and eosin, ×40). B, photomicrograph of the wall of the larger cavity, thin and internally lined with endothelium. Part of this wall shows hyalinized fibrous thickening, (hematoxylin and eosin, ×40).
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 3. Photograph taken during surgery. A, cavernous angioma adheres to the lower aspect of the left optic nerve, filling the left suprasellar region. B, operative aspect after the cyst was emptied. ON, left optic nerve; ICA, left internal carotid artery.
same side, with a small temporal expansion (Fig. 3B). The capsule tissue was removed carefully without bleeding, preserving the contiguous anatomical structures. The histological section showed fragments of loose connective tissue and nervous tissue among which were many vascular spaces with irregular shapes of different sizes, generally with thin walls, and little or no smooth muscle (Fig. 4A). Occasionally, vessel walls appeared thickened by degenerative changes or by the organization of laminar thrombi. Some broad lumina contained unorganized thrombi. Fragments of the cystic cavity showed structures similar to those of the other vessels (Fig. 4B). In the adjacent tissue, hemosiderin-loaded macrophages had accumulated. The postoperative course was uneventful; 6 months later a tomographic study showed nothing abnormal. The ophthalmic examination performed at that time showed the same abnormalities that were present before the operation. DISCUSSION Optochiasmatic apoplexy is characterized by the abrupt onset of symptoms with loss of vision and reduction of the visual field associated with a predominantly retroorbital headache (6). The name "chiasmatic apoplexy" was used initially by Maitland et al. (6). They reported 4 clinical cases with 3 AVMs and 1 glioma of the optic chiasm. Reilly and Oatey (12) introduced the term "optic nerve apoplexy" and described 2 cases of a hematoma in the optic nerve; an AVM was identified in 1 case. The occurrence of optochiasmatic apoplexy caused by a histologically demonstrated cavernous angioma has not been described often. Lejeune et al. (5), in a review of the literature, found only 7 reported cases. In all of them, the cavernoma affected the optic nerve and chiasm. In our patients, the clinical syndrome was monocular on the left side, coinciding with the location of the cavernoma in the left optic nerve. Most of the patients in the study of Lejeune et al. (5) were between 20 and 40 years of age. Our patient, who was 8 years old at the time of the diagnosis, is the youngest. Other cases of cavernous angioma in the optic nerve, in which no apoplexy occurred, have been reported (7, 16) . In diagnosing cavernous angiomas, the angiogram shows mass effect and rarely demonstrates abnormal circulation (9,17). The CT scan presents a hyperdense image, with or without calcification, and other possible causes are not excluded (11). At present, magnetic resonance imaging offers the best possibility of describing the pathological features (8, 15) . Surgical treatment is justified for 1) diagnostic purposes, 2) decompression of the optic nerve and/or chiasm, and 3) prophylaxis of lesion growth, new hemorrhages, and consequent deterioration (8,10,12,16). In our patient, there was a history suggesting two episodes of bleeding. The visual symptoms occurred during the second. A wine-colored mass, limited by a capsule that contained dark blood, without a stroma and forming a voluminous hematic cyst was found at surgery. The
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 February 1992, Volume 30, Number 2 262 Optic Nerve Apoplexy Caused by a Cavernous Angioma: Case Report Case Report
cyst adhered to the lower aspect of the left optic nerve and extended to the suprasellar region, pressing between the internal carotid artery and the optic nerve. The hematic cyst capsule was removed without further bleeding, indicating that there were no arterial feeders. This aspect of a cavernous angioma has been observed by others (2,3,18). No hemorrhage was seen in the optic nerve. Possibly, the acute visual disturbance was a result of bleeding inside the cyst. Optic atrophy is a sign of long-standing compression. The histological appearance was typical of a cavernous angioma. There are reports of improved visual function after surgical treatment, and some advise immediate surgery (8). In our patient, the visual deficits persisted. ACKNOWLEDGMENT We are grateful to Geraldo Geyer, M.D., for the pathological study.
REFERENCES: (1-18) 1. 2.
3. 4.
5.
6. 7. 8.
9.
Castel JP, Delorge-Kerdiles C, Rivel J: Angiome caverneux du chiasma optique. Neurochirurgie 35:252-256, 1989. Iplikçioglu AC, Benli K, Bertan V, Ruacan S: Cystic cavernous hemangioma of the cerebellopontine angle: Case report. Neurosurgery 19:641-642, 1986. Khosla VK, Banerjee AK, Mathuriya SN, Mehta S: Giant cyst cavernoma in a child. Case report. J Neurosurg 60:1297-1299, 1984. Lavin PJM, McCrary JA III, Roessmann U, Ellenberger C Jr: Chiasmal apoplexy: Hemorrhage from a cryptic vascular malformation in the optic chiasm. Neurology 34:1007-1011, 1984. Lejeune JP, Hladky JP, Dupard T, Parent M, Hache JC, Christiaens JL: Apoplexie optochiasmatique. Neurochirurgie 36:303-307, 1990. Maitland CG, Abiko S, Hoyt WF, Wilson CB, Okamura T: Chiasmal apoplexy. Report of four cases. J Neurosurg 56:118-122, 1982. Maruoka N, Yamakawa Y, Shimauchi M: Cavernous hemangioma of the optic nerve. Case report. J Neurosurg 69:292-294, 1988. Mohr G, Hardy J, Gauvin P: Chiasmal apoplexy due to a ruptured cavernous hemangioma of the optic chiasm. Surg Neurol 24:636-640, 1985. Orenstein D, Kiesman M, Dietemann JL, Krause D, Maitrot D, Buchheit F, Marescaux C, Wackenheim A, Tongio J, Froidevaux JL: Les malformations vasculaires angiographiquement muettes évocatrices d'un cavernome: Etude clinique de 18 cas. Neurochirurgie 35:95-97, 1989.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Received for publication, June 12, 1991; accepted, July 30, 1991. Reprint requests: Nelson Pires Ferreira, M.D., Av. Independência, 1211 conj. 301, Porto Alegre, RS, Brazil 90210.
Figure 2. Contrast-enhanced CT scan shows a homogeneously enhanced suprasellar mass with greater projection toward the left side.
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 1. Visual field demonstrates a nasal defect on the left side.
Figure 4. A, photomicrograph of the surgical specimen shows irregular blood vessels with rudimentary walls, some of them containing unorganized thrombi (hematoxylin and eosin, ×40). B, photomicrograph of the wall of the larger cavity, thin and internally lined with endothelium. Part of this wall shows hyalinized fibrous thickening, (hematoxylin and eosin, ×40).
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 3. Photograph taken during surgery. A, cavernous angioma adheres to the lower aspect of the left optic nerve, filling the left suprasellar region. B, operative aspect after the cyst was emptied. ON, left optic nerve; ICA, left internal carotid artery.
