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Cyst of the Intraorbital Optic Nerve Sheaths
CYST O F T H E I N T R A O R B I T A L O P T I C N E R V E S H E A T H S G E R A L D J. H A R R I S , M . D . , P E T E R E. W E I N B E R G , M.D.,
J O E L G. S A C K S ,
M.D.,
A N D R I C H A R D B. O ' G R A D Y ,
Chicago,
M.D.
Illinois
Cystic lesions 1 - 5 and hemangiomas 4 - 7 of the intraorbital portion of the optic nerve or its sheaths are uncommon. We cared for a patient with a large cyst of the intraorbital optic nerve sheath that ap peared to be secondary to a hemangioma of leptomeningeal origin. To our knowl edge, only one similar case has been re ported. 5 CASE REPORT A 43-year-old white woman presented with com plaints of gradual loss of visual acuity and promi nence of the right eye over a three-year period. There had been no history of ocular or cranial trauma. Visual acuity was R.E.: no light perception, and L.E.: correctable to 20/20. Exophthalmometer read ings were 21 and 13 mm on the right and left eyes, respectively. There was increased resistance to retropulsion of the right eye; no bruit or pulsation was present. The right pupil reacted to light only on consensual stimulation. Motility was mildly com promised in upgaze, but was otherwise unrestricted. The right optic disk was pale; the vessels ,were of normal caliber. There were no opticociliary shunts or choroidal folds. The left fundus and visual field were normal. B-scan ultrasonography (Bronson-Turner) of the right orbit demonstrated a smooth-margined mass of uniform acoustic density behind the globe. Plain film radiographie examination, including optic foramen views and polytomography of the optic canals in the basal projection, revealed no abnormalities. Brain scan and cerebral angiography results were also normal. Orbital venography was performed by injecting contrast material into a frontal vein. In comparison to the normal left side, there was superior displace ment of the origin of the third segment of the superior ophthalmic vein on the right side (Fig. 1). This segment, which lies within the muscle cone, appeared attenuated on the axial projection. It was
From the Departments of Ophthalmology (Drs. Harris, Sacks, and O'Grady), Neurology (Dr. Sacks), and Radiology (Dr. Weinberg), Northwestern Uni versity Medical School, Chicago, Illinois. Reprint requests to Gerald J. Harris, M.D., De partment of Ophthalmology, Northwestern Univer sity Medical School, 303 E. Chicago Ave., Chicago, IL 60611.
Fig. 1 (Harris and associates). Orbital venogram (anteroposterior projection). The third portion of the right superior ophthalmic vein has a stretched ap pearance (arrow) and the junction between the sec ond and third segments is higher in position than the normal left side. not possible to differentiate an intraconical lesion from one lying outside the muscle cone. For this reason, we performed contrast orbitography, result ing in opacification of an ovoid space. This differed from the usual configuration of the muscle cone in that the anterior aspect had a convex outline rather than the normal concavity produced by the back of the globe (Fig. 2). There was a comma-shaped filling defect in the inferior lateral portion of the collec tion; this was believed to represent the optic nerve (Fig. 3). Because of the unusual size and contour of the opacified space, as well as the retention of the water-soluble contrast material as demonstrated in follow-up films at more than 90 minutes, we be lieved a cystic lesion had been injected. The patient underwent a transfrontal craniotomy with unroofing of the right orbit in a combined neurosurgical-ophthalmologic procedure. The mus cle cone was entered between the superior and lateral recti and a bluish cyst was seen continuous with the sheath of the optic nerve. In order to deliver the large cyst through the orbital roof, we aspirated a portion of its contents, a brownish fluid. Because it was not possible to dissect the lesion from the substance of the optic nerve sheath, we transected the nerve behind the globe and again at its entry into the optic canal (Fig. 4). The postoperative morbidity was minimal, al though the patient had blepharoptosis and paresis of upgaze that totally resolved within two months; exophthalmometric readings were 14 mm on the right eye and 13 mm on the left eye. The patient has continued to do well for more than one year after surgery.
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Fig. 4 (Harris and associates). Gross appearance of 2.5-cm cystic lesion continuous with sheath of optic nerve. Three milliliters of fluid contents had been aspirated before delivery.
Fig. 2 (Harris and associates). Right orbitography (lateral projection). The anterior margin (left) of the outlined collection of contrast material (arrows) has a convex contour. RESULTS
Cytologie analysis of the aspirated fluid revealed only red and white blood
cells. No microorganisms were recovered from cultures employing blood, MacConkey, Sabouraud, and chocolate agars and thioglycollate broth. Representative sections demonstrated a clear cyst lined by a thick fibrous wall containing embedded nests of meningothelial cells. Inferiorly it continued about an atrophie, gliotic optic nerve (Fig. 5). Numerous thick-walled vascular chan nels of varied diameter were grouped in an area adjacent to the fused meningeal sheaths (Figs. 6 and 7). Iron stain demon strated only a small amount of hemosiderin in the central cavity of the cyst. No iron was found in the cyst wall. We diagnosed a cyst of the optic nerve sheaths associated with vascular prolife ration (hamartoma?) and recent hemor rhage. DISCUSSION
Fig. 3 (Harris and associates). Right orbitography with anteroposterior polytomography obtained at 90 minutes after injection. Contrast material persists within the cystic lesion and reveals a comma-shaped filling defect (arrow) believed to represent the optic nerve.
We speculate that the original lesion was a hemangioma, probably of leptomeningeal origin, which may have oozed a proteinaceous fluid into the subarachnoid space. This led to a surrounding fibrosis and created a cystic deformity with compression atrophy of the optic nerve. The minimal hemosiderosis in the central cyst was compatible with a proba ble small hemorrhage during the orbitog raphy that occurred one week before sur gery.
658
AMERICAN JOURNAL OF OPHTHALMOLOGY
MAY, 1976
reported. 4 ' 7 However, as Spencer 4 pointed out, these may represent preexisting vas cular channels which have dilated sec ondary to venous obstruction, or, where no obstruction exists, have dilated in the manner of telangiectasia. Other reported cystic lesions of the intraorbital optic nerve have had a varied pathogenetic basis. Bane, 1 in 1918, de scribed an 8-year-old boy with a cyst behind the globe. The specimen showed a thickened, whorled endothelium lining the dura mater, containing a few rudimen tary blood vessels. Although no photomi crographs were published, the descrip tion suggests that this may have been a meningioma. Most other reported cysts appear to have originated from the arach noid. Wolter and McKenney 2 described an arachnoid cyst that developed in the
Fig. 5 (Harris and associates). Low-power appear ance of the cyst. The wall consists of a thick fibrous sheath enveloping the optic nerve inferiorly (wedge) and covering a fairly dense zone of blood vessels (arrows). Residual blood remains in the central cavity (Masson's trichrome, x4).
We are aware of only one similar case. Schneider 5 reported a cyst of the intraor bital optic nerve associated with a hemangioma in a 26-year-old woman and as cribed it to von Hippel-Lindau disease. To our knowledge, there was no associat ed intracranial or systemic vascular anomaly. However, from the written de scription of the fundus, it is possible that a retinal angioma coexisted. Hogan and Zimmerman 6 depicted a more cellular hemangioendothelioma of the intraorbital optic nerve in a patient with a similar tumor of the cerebellum. Although we did not rule out an oculocranial or oculosystemic vascular syndrome in the pres ent case, there was no clinical evidence of von Hippel-Lindau disease. Other hemangiomas in the substance of the intraorbital optic nerve have been
Fig. 6 (Harris and associates). High-power view of cyst wall. Dark-staining blood vessels, mixed with hemorrhage, are separated from the thickened meningeal sheaths by an artifactitious cleft (Masson's trichrome, XlOO).
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Fig. 7 (Harris and associates). High-power view of cyst lining. A mass of thick-walled, endothelially lined vessels lies internal to the thickened méninges (Masson's trichrome, x200).
process of "collateral hyperplasia" asso ciated with an optic nerve glioma in a 4-year-old boy. Another lesion described as an arachnoidal cyst or, alternatively, a perioptic subdural hygroma, was reported by Smith, Hoyt, and Newton 3 in a woman with unilateral disk edema and an "empty" sella. The wall of the cyst was histologically "normal optic nerve sheath." Spencer 4 related the case of a 3-year-old boy with neurofibromatosis who presented with proptosis and en largement of the optic canal with visual acuity loss. A cystic dilation of the intra orbital optic nerve sheath was found and considered an arachnoid cyst. Apparently there was no associated glioma. We limited our discussion to the intra
orbital optic nerve because of the com mon manifestation of proptosis. How ever, of the few reported cysts of the intracanalicular or intracranial optic nerve, 8 - 1 0 only one appears to have had an angiomatous basis. 1 0 Orbitography was used with relative impunity in our case of a blind eye. However, in the presence of a sighted eye, penetration of a vascular lesion with po tential hemorrhage becomes a significant risk. We chose the trans cranial approach be cause of preoperative uncertainty of the extent of the lesion into, or proximal to, the optic canal. Unroofing of the canal may be required for total excision, if not only for complete examination, when the
660
AMERICAN JOURNAL OF OPHTHALMOLOGY
histologie nature and point of origin of a lesion are unknown. This proved to be unnecessary and the canal was not vio lated. SUMMARY
A 43-year-old woman had a history of gradual unilateral visual acuity loss and proptosis. A 2.5-cm cystic dilatation of the intraorbital dura mater was removed. Microscopic examination revealed a vas cular proliferation within the leptomeninges. We inadvertently injected the lesion during orbitography, providing us with an unusual radiographie appear ance. REFERENCES 1. Bane, W. C : Cyst of durai sheath of optic nerve. Am. J. Ophthalmol. 1:17, 1918. 2. Wolter, J. R., and McKenney, M. J.: Collateral
MAY, 1976
hyperplasia and cyst formation of orbital leptomeninx. Am. J. Ophthalmol. 57:1037, 1964. 3. Smith, J. L., Hoyt, W. F., and Newton, T. H.: Optic nerve sheath decompression for relief of chronic monocular choked disc. Am. J. Ophthalmol. 68:633, 1969. 4. Spencer, W. H.: Primary neoplasms of the optic nerve and its sheaths. Clinical features and current concepts of pathogenetic mechanisms. Trans. Am. Ophthalmol. Soc. 70:490, 1972. 5. Schneider, R.: Angioretikulöm des Sehnerven. Albrecht von Graefe's Arch. Klin. Ophthalmol. 145:163, 1942. 6. Högan, M. J., ahd Zimmerman, L. E.: Oph thalmic Pathology, 2nd ed. Philadelphia, W. B. Saunders, 1962, pp. 608-609. 7. Koyanagi, Y.: Ein fall von angiomatoser Ka vernenbildung im Sehnerven. Klin Monatsbl. Augenheilkd. 83:550, 1929. 8. Walsh, F. B., Chambers, J. W., and Lloyd, L. A.: The ocular signs of tumors involving the anterior visual pathways. Am. J. Ophthalmol. 42:347, 1956. 9. Holt, H.: Cysts of the intracraniàl portion of the optic nerve. Am. J. Ophthalmol. 61:1166, 1966. 10. Verga, P.: Angio-reticolo-glioma cistico del neuro ottico. Riv. Oto-Neuro-Oftalmol. 7:101, 1930.
J O E L G. S A C K S ,
M.D.,
A N D R I C H A R D B. O ' G R A D Y ,
Chicago,
M.D.
Illinois
Cystic lesions 1 - 5 and hemangiomas 4 - 7 of the intraorbital portion of the optic nerve or its sheaths are uncommon. We cared for a patient with a large cyst of the intraorbital optic nerve sheath that ap peared to be secondary to a hemangioma of leptomeningeal origin. To our knowl edge, only one similar case has been re ported. 5 CASE REPORT A 43-year-old white woman presented with com plaints of gradual loss of visual acuity and promi nence of the right eye over a three-year period. There had been no history of ocular or cranial trauma. Visual acuity was R.E.: no light perception, and L.E.: correctable to 20/20. Exophthalmometer read ings were 21 and 13 mm on the right and left eyes, respectively. There was increased resistance to retropulsion of the right eye; no bruit or pulsation was present. The right pupil reacted to light only on consensual stimulation. Motility was mildly com promised in upgaze, but was otherwise unrestricted. The right optic disk was pale; the vessels ,were of normal caliber. There were no opticociliary shunts or choroidal folds. The left fundus and visual field were normal. B-scan ultrasonography (Bronson-Turner) of the right orbit demonstrated a smooth-margined mass of uniform acoustic density behind the globe. Plain film radiographie examination, including optic foramen views and polytomography of the optic canals in the basal projection, revealed no abnormalities. Brain scan and cerebral angiography results were also normal. Orbital venography was performed by injecting contrast material into a frontal vein. In comparison to the normal left side, there was superior displace ment of the origin of the third segment of the superior ophthalmic vein on the right side (Fig. 1). This segment, which lies within the muscle cone, appeared attenuated on the axial projection. It was
From the Departments of Ophthalmology (Drs. Harris, Sacks, and O'Grady), Neurology (Dr. Sacks), and Radiology (Dr. Weinberg), Northwestern Uni versity Medical School, Chicago, Illinois. Reprint requests to Gerald J. Harris, M.D., De partment of Ophthalmology, Northwestern Univer sity Medical School, 303 E. Chicago Ave., Chicago, IL 60611.
Fig. 1 (Harris and associates). Orbital venogram (anteroposterior projection). The third portion of the right superior ophthalmic vein has a stretched ap pearance (arrow) and the junction between the sec ond and third segments is higher in position than the normal left side. not possible to differentiate an intraconical lesion from one lying outside the muscle cone. For this reason, we performed contrast orbitography, result ing in opacification of an ovoid space. This differed from the usual configuration of the muscle cone in that the anterior aspect had a convex outline rather than the normal concavity produced by the back of the globe (Fig. 2). There was a comma-shaped filling defect in the inferior lateral portion of the collec tion; this was believed to represent the optic nerve (Fig. 3). Because of the unusual size and contour of the opacified space, as well as the retention of the water-soluble contrast material as demonstrated in follow-up films at more than 90 minutes, we be lieved a cystic lesion had been injected. The patient underwent a transfrontal craniotomy with unroofing of the right orbit in a combined neurosurgical-ophthalmologic procedure. The mus cle cone was entered between the superior and lateral recti and a bluish cyst was seen continuous with the sheath of the optic nerve. In order to deliver the large cyst through the orbital roof, we aspirated a portion of its contents, a brownish fluid. Because it was not possible to dissect the lesion from the substance of the optic nerve sheath, we transected the nerve behind the globe and again at its entry into the optic canal (Fig. 4). The postoperative morbidity was minimal, al though the patient had blepharoptosis and paresis of upgaze that totally resolved within two months; exophthalmometric readings were 14 mm on the right eye and 13 mm on the left eye. The patient has continued to do well for more than one year after surgery.
656
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OPTIC NERVE CYST
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Fig. 4 (Harris and associates). Gross appearance of 2.5-cm cystic lesion continuous with sheath of optic nerve. Three milliliters of fluid contents had been aspirated before delivery.
Fig. 2 (Harris and associates). Right orbitography (lateral projection). The anterior margin (left) of the outlined collection of contrast material (arrows) has a convex contour. RESULTS
Cytologie analysis of the aspirated fluid revealed only red and white blood
cells. No microorganisms were recovered from cultures employing blood, MacConkey, Sabouraud, and chocolate agars and thioglycollate broth. Representative sections demonstrated a clear cyst lined by a thick fibrous wall containing embedded nests of meningothelial cells. Inferiorly it continued about an atrophie, gliotic optic nerve (Fig. 5). Numerous thick-walled vascular chan nels of varied diameter were grouped in an area adjacent to the fused meningeal sheaths (Figs. 6 and 7). Iron stain demon strated only a small amount of hemosiderin in the central cavity of the cyst. No iron was found in the cyst wall. We diagnosed a cyst of the optic nerve sheaths associated with vascular prolife ration (hamartoma?) and recent hemor rhage. DISCUSSION
Fig. 3 (Harris and associates). Right orbitography with anteroposterior polytomography obtained at 90 minutes after injection. Contrast material persists within the cystic lesion and reveals a comma-shaped filling defect (arrow) believed to represent the optic nerve.
We speculate that the original lesion was a hemangioma, probably of leptomeningeal origin, which may have oozed a proteinaceous fluid into the subarachnoid space. This led to a surrounding fibrosis and created a cystic deformity with compression atrophy of the optic nerve. The minimal hemosiderosis in the central cyst was compatible with a proba ble small hemorrhage during the orbitog raphy that occurred one week before sur gery.
658
AMERICAN JOURNAL OF OPHTHALMOLOGY
MAY, 1976
reported. 4 ' 7 However, as Spencer 4 pointed out, these may represent preexisting vas cular channels which have dilated sec ondary to venous obstruction, or, where no obstruction exists, have dilated in the manner of telangiectasia. Other reported cystic lesions of the intraorbital optic nerve have had a varied pathogenetic basis. Bane, 1 in 1918, de scribed an 8-year-old boy with a cyst behind the globe. The specimen showed a thickened, whorled endothelium lining the dura mater, containing a few rudimen tary blood vessels. Although no photomi crographs were published, the descrip tion suggests that this may have been a meningioma. Most other reported cysts appear to have originated from the arach noid. Wolter and McKenney 2 described an arachnoid cyst that developed in the
Fig. 5 (Harris and associates). Low-power appear ance of the cyst. The wall consists of a thick fibrous sheath enveloping the optic nerve inferiorly (wedge) and covering a fairly dense zone of blood vessels (arrows). Residual blood remains in the central cavity (Masson's trichrome, x4).
We are aware of only one similar case. Schneider 5 reported a cyst of the intraor bital optic nerve associated with a hemangioma in a 26-year-old woman and as cribed it to von Hippel-Lindau disease. To our knowledge, there was no associat ed intracranial or systemic vascular anomaly. However, from the written de scription of the fundus, it is possible that a retinal angioma coexisted. Hogan and Zimmerman 6 depicted a more cellular hemangioendothelioma of the intraorbital optic nerve in a patient with a similar tumor of the cerebellum. Although we did not rule out an oculocranial or oculosystemic vascular syndrome in the pres ent case, there was no clinical evidence of von Hippel-Lindau disease. Other hemangiomas in the substance of the intraorbital optic nerve have been
Fig. 6 (Harris and associates). High-power view of cyst wall. Dark-staining blood vessels, mixed with hemorrhage, are separated from the thickened meningeal sheaths by an artifactitious cleft (Masson's trichrome, XlOO).
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659
Fig. 7 (Harris and associates). High-power view of cyst lining. A mass of thick-walled, endothelially lined vessels lies internal to the thickened méninges (Masson's trichrome, x200).
process of "collateral hyperplasia" asso ciated with an optic nerve glioma in a 4-year-old boy. Another lesion described as an arachnoidal cyst or, alternatively, a perioptic subdural hygroma, was reported by Smith, Hoyt, and Newton 3 in a woman with unilateral disk edema and an "empty" sella. The wall of the cyst was histologically "normal optic nerve sheath." Spencer 4 related the case of a 3-year-old boy with neurofibromatosis who presented with proptosis and en largement of the optic canal with visual acuity loss. A cystic dilation of the intra orbital optic nerve sheath was found and considered an arachnoid cyst. Apparently there was no associated glioma. We limited our discussion to the intra
orbital optic nerve because of the com mon manifestation of proptosis. How ever, of the few reported cysts of the intracanalicular or intracranial optic nerve, 8 - 1 0 only one appears to have had an angiomatous basis. 1 0 Orbitography was used with relative impunity in our case of a blind eye. However, in the presence of a sighted eye, penetration of a vascular lesion with po tential hemorrhage becomes a significant risk. We chose the trans cranial approach be cause of preoperative uncertainty of the extent of the lesion into, or proximal to, the optic canal. Unroofing of the canal may be required for total excision, if not only for complete examination, when the
660
AMERICAN JOURNAL OF OPHTHALMOLOGY
histologie nature and point of origin of a lesion are unknown. This proved to be unnecessary and the canal was not vio lated. SUMMARY
A 43-year-old woman had a history of gradual unilateral visual acuity loss and proptosis. A 2.5-cm cystic dilatation of the intraorbital dura mater was removed. Microscopic examination revealed a vas cular proliferation within the leptomeninges. We inadvertently injected the lesion during orbitography, providing us with an unusual radiographie appear ance. REFERENCES 1. Bane, W. C : Cyst of durai sheath of optic nerve. Am. J. Ophthalmol. 1:17, 1918. 2. Wolter, J. R., and McKenney, M. J.: Collateral
MAY, 1976
hyperplasia and cyst formation of orbital leptomeninx. Am. J. Ophthalmol. 57:1037, 1964. 3. Smith, J. L., Hoyt, W. F., and Newton, T. H.: Optic nerve sheath decompression for relief of chronic monocular choked disc. Am. J. Ophthalmol. 68:633, 1969. 4. Spencer, W. H.: Primary neoplasms of the optic nerve and its sheaths. Clinical features and current concepts of pathogenetic mechanisms. Trans. Am. Ophthalmol. Soc. 70:490, 1972. 5. Schneider, R.: Angioretikulöm des Sehnerven. Albrecht von Graefe's Arch. Klin. Ophthalmol. 145:163, 1942. 6. Högan, M. J., ahd Zimmerman, L. E.: Oph thalmic Pathology, 2nd ed. Philadelphia, W. B. Saunders, 1962, pp. 608-609. 7. Koyanagi, Y.: Ein fall von angiomatoser Ka vernenbildung im Sehnerven. Klin Monatsbl. Augenheilkd. 83:550, 1929. 8. Walsh, F. B., Chambers, J. W., and Lloyd, L. A.: The ocular signs of tumors involving the anterior visual pathways. Am. J. Ophthalmol. 42:347, 1956. 9. Holt, H.: Cysts of the intracraniàl portion of the optic nerve. Am. J. Ophthalmol. 61:1166, 1966. 10. Verga, P.: Angio-reticolo-glioma cistico del neuro ottico. Riv. Oto-Neuro-Oftalmol. 7:101, 1930.