SPHENO-ORBITAL M E N I N G I O M A WITH OPTOCILIARY VEINS M E R L Y N M. R O D R I G U E S , M.D., P E T E R J. SAVINO, A N D N O R M A N J. S C H A T Z , M.D. Philadelphia,
M.D.,
Pennsylvania
An optociliary shunt rarely occurs be tween the central retinal vein and peripapillary choroid in the region of the lamina cribrosa. 1 It may indicate an asso ciated spheno-orbital meningioma, 2 - 4 chronic atrophie papilledema, 2 central retinal vein occlusion, 4 or glioma of the optic disk. 5 This paper describes the clinical syn drome of a cranio-orbital meningioma with optociliary veins demonstrated by fluorescein angiography and confirmed by histopathologic examination. CASE REPORT A 40-year-old white woman complained of gradu al decreasing vision and pain in the right eye associ ated with unilateral proptosis since 1966. In April 1974, she noted decreased vision in her left eye and was referred to the Neuro-Ophthalmology Service. General physical examination revealed hyperten sion. Visual acuity was R.E.: no light perception, and L.E.: 20/70 with a central scotoma. The right eye showed 4 to 5 mm of proptosis with resistance to retropulsion of the globe. No masses were palpable. Motility examination revealed right exotropia with decreased movement of the right eye in all direc tions of gaze. A full range of motion was present in the left eye. There was ectropion uveae and neovascularization of the angle extending to the anterior lens surface of the right eye. Vitreous clouding from an old hemorrhage was noted. Ophthalmoscopy revealed massive chronic disk edema of the right eye with prominent optociliary veins (Fig. 1, top). The left optic disk was mildly edematous with hemor rhages and exudates visible at the macula (Fig. 1, bottom). Fluorescein angiography of the right eye showed lack of filling of the optociliary veins in the arterial phase with fluorescence in the venous phase (Fig. 2). Routine laboratory investigations were within normal limits. An electrocardiogram showed left From the Neuro-Ophthalmology Service (Drs. Savino and Schatz), Department of Pathology (Dr. Rodrigues), Wills Eye Hospital, Thomas Jefferson University and the Naval Regional Medical Center (Dr. Savino), Philadelphia, Pennsylvania. Reprint requests to M. Rodrigues, M.D., or N. J. Schatz, M.D., Wills Eye Hospital, 1601 Spring Gar den St., Philadelphia, PA 19130.
Fig. 1 (Rodrigues, Savino, and Schatz). Top, Right eye. F u n d u s photograph showing two optociliary shunt vessels (arrows) and disk edema. Bottom, Left eye. Hypertensive retinopathy with macular exu dates. atrial enlargement. Roentgenograms of the chest and intravenous pyelography were normal. Polytomography demonstrated an enlarged right optic canal, expansion of the right orbit medially, a thinned lamina papyracea with a concavity consistent with elevated intraorbital pressure of long duration, and slight thickening of the planum sphenoidale. Carot id arteriography showed a dumbbell tumor " b l u s h " in the right juxtasellar region and irregularity of the supraclinoid portion of the internal carotid artery,
666
VOL. 81, NO. 5
SPHENO-ORBITAL MENINGIOMA
667
and filled the suprasellar space, measuring approxi mately 2.5 cm in diameter. The right orbit was unroofed and a firm, grayish tumor occupied the muscle cone and the posterior orbit. The optic nerve was four to five times thicker than normal from the posterior portion of the globe to the orbital apex. The nerve was sectioned posterior to the globe and residual tumor was noted in the muscle cone inferiorly. Microscopic examination of the tumor showed a meningothelial meningioma. On March 20, 1975, orbital exploration revealed an orbital tumor invad ing the optic nerve and sciera that we enucleated.
Fig. 2 (Rodrigues, Savino, and Schatz). Top, Fluorescein angiography. Arterial phase of fluorescein angiogram with non-filling of the optociliary shunt (arrows). Diffuse leakage is visible at the markedly elevated disk. Bottom, Venous phase demonstrating filling of the optociliary shunt vessels (arrows). with elevation of the main trunk of the anterior cerebral artery (Fig. 3, left). In the capillary phase, a diffuse tumor blush supplied primarily by the oph thalmic artery was noted in the region of the right orbit (Fig. 3, right). A right frontal craniotomy performed on April 24 revealed a small fungating tumor arising from the right optic nerve sheath. An additional meningeal blood supply was detected from the posterior part of the planum sphenoidale and the circular sinus. The right optic nerve was resected anterior to the chiasm. The tumor encircled the right internal carotid artery
Histopathology—Gross examination re vealed a firm right globe measuring 21 X 21 x 21 mm with 1 mm of attached optic nerve. The clear cornea measured 11.5 x 10.5 mm. Transillumination of the globe was poor. The eye was sectioned horizontally. The anterior chamber was of normal depth and the angle was open. The lens was in place. The iris and ciliary body were normal. The retina was de tached by hemorrhage and the optic disk was thickened and infiltrated by the me ningeal tumor. Microscopic examination revealed an orbital meningioma (Fig. 4) with invasion of the sciera and peripapillary choroid (Figs. 5 and 6). Prominent dilated shunt vessels connect ed the central retinal vein and peripapillary choroid, with adjacent nests of meningiomatous infiltration. Other oc ular findings included secondary optic atrophy, angle closure secondary to pe ripheral anterior synechiae, rubeosis iridis, hemorrhagic retinal detachment, and neovascularization of the vitreous humor. DISCUSSION
Invasion of the peripapillary choroid and sciera is rare in primary orbital me ningioma, 6 - 8 in meningioma of the tuberculum sellae, 9 and in spheno-orbital me ningioma, as in the present case. Optocili ary shunts have not been reported in primary orbital meningioma and these abnormal vascular channels suggest sphenoid ridge meningioma. 3 The presence of optociliary veins, re duced vision, and chronic atrophie disk
668
AMERICAN JOURNAL OF OPHTHALMOLOGY
MAY, 1976
Fig. 3 (Rodrigues, Savino, and Schatz). Left, Right carotid arteriogram (anteroposterior) demonstrates elevation of the Al portion of the anterior cerebral artery (arrows) indicative of intracranial extension of the tumor. Right, Carotid arteriogram showing a tumor blush (arrows) in the orbit supplied by the ophthalmic artery.
edema are characteristics of meningiomas involving the cranio-orbital junction. 3 Our case conforms to the syndrome de scribed by Frisen, Hoyt, and Tengroth. 3 Optociliary vessels are thought to rep resent enlargement of preformed capil lary vessels that shunt blood from the
central retinal vein to the peripapillary choroidal circulation when the normal drainage routes are obstructed. Chronicity of the obstruction causes dilatation of these channels, which becomes more evi dent when optic atrophy ensues. Optociliary veins may be congenital or
Fig. 4 (Rodrigues, Savino, and Schatz). Nests of meningothelial cells in the orbital tumor (hematoxylin-eosin, x200).
VOL. 81, NO. 5
SPHENO-ORBITAL MENINGIOMA
669
Fig. 5 (Rodrigues, Savino, and Schatz). Inset shows anomalous shunt vessels at the disk (arrows) adjacent to choroidal invasion by the peripapillary meningioma (M) (hematoxylin and eosin, x50). Nests of meningothelial meningioma (M) invading the choroid. Arrows indicate Bruch's membrane (hematoxylineosin, x200).
acquired. Braun 1 0 described two separate types of congenitally abnormal venous channels: Type 1 resembling retinal veins, and Type 2 appearing similar to choroidal veins (optociliary veins). He noted that they are broader than the papil lary veins, lighter in color, and lack the streak reflex. They can be differentiated from acquired optociliary veins by the tortuosity and ectasia of the latter shunts. Acquired optociliary veins are encoun tered in several conditions. They have been observed in chronic atrophie papilledema, 2 central retinal vein occlusion, 4 and gliomas of the optic disk. 5 Fluorescein angiography is helpful in documenting the true nature of the shunt. The optocili ary veins do not fluoresce during the arterial phase of fluorescein angiography but divert fluorescein to the choroidal circulation in the venous phase.
Salzmann 1 1 reported a case of myxosarcoma (glioma) of the optic nerve with associated Fuchs' coloboma and a probable optociliary shunt. These shunt vessels were not noted clinically and his tologie documentation was inadequate. Elschnig 1 2 described clinically a congen ital optociliary shunt in a 16-year-old boy with no evidence of an orbital or intracranial neoplasm. He also observed anoma lous cilioretinal vessels in a 43-year-old woman with an orbital endothelioma (meningioma). In both cases the shunts were not demonstrated histologically. In our case, we demonstrated clinically documented optociliary shunt vessels with histopathologic verification of a true retinal choroidal vascular anastomosis, and correlated the fluorescein angio graphie and histopathologic appearance of these shunt vessels.
Fig. 6 (Rodrigues, Savino, and Schatz). Numerous prominent shunt vessels (arrows) between the central retinal vein and peripapillary choroid, adjacent to meningiomatous invasion (M) (hematoxylin-eosin, x200). SUMMARY
A 40-year-old white woman had slowly progressive unilateral loss of visual acuity and increasing proptosis during an eightyear period. Ophthalmoscopy and fluorescein angiography revealed chronic disk edema and optociliary shunt vessels in the right eye. Polytomography showed an enlarged right optic canal. Cerebral arteriography demonstrated a dumbbellshaped tumor blush in the right juxtasellar region and a diffuse tumor blush in the right orbit. Right frontal craniotomy and orbital exploration . showed a cranioorbital junction (spheno-orbital) meningi oma that invaded the sciera and peripa pillary choroid. Anomalous optociliary veins were demonstrated histologically at the optic disk. ACKNOWLEDGMENT
Carol Starrels, Ph.D., assisted with translation.
REFERENCES 1. Hayreh, S. C : Blood supply of the optic nerve head and its role in optic atrophy, glaucoma and
edema of the optic disc. Br. J. Ophthalmol. 53:721, 1969. 2. Hedges, T. P.: Papilledema. Its recognition and relation to increased intracranial pressure. Survey Ophthalmol. 19:201, 1975. 3. Frisen, L., Hoyt, W. F., and Tengroth, B. M.: Optociliary veins, disc pallor and visual loss. A triad of signs indicating spheno-orbital meningioma. Acta Ophthalmol. 51:241, 1973. 4. Sanders, M. D.: A classification of papilledema based on a fluorescein angiographie study of 69 cases. Trans. Ophthalmol. Soc. U. K. 89:117, 1969. 5. Hoyt, W. F.,and Beeston, D.: The Ocular Fun dus in Neurologic Disease. St. Louis, C. V. Mosby, 1966, p . 42. 6. Karp, L. A., Zimmerman, L. E., Borit, A., and Spencer, W.: Primary intraorbital meningiomas. Arch. Ophthalmol. 91:24, 1974. 7. Martin, V. A. F., and Schofield, P. B.: Meningi oma invading the optic nerve. Br. J. Ophthalmol. 41:161, 1957. 8. Hudson, A. C : Primary tumors of the optic nerve. R. Ophthalmol. Hosp. Rep. 18:317, 1912. 9. Newell, F. W., and Beaman, T. C : Ocular signs of meningioma. Am. J. Ophthalmol. 45:30, 1958. 10. Braun: Ein Beitrag zur Kenntnis optikociliarer Gefässe. Klin. Monatsbl. Augenheilkd. 43:579, 1905. 11. Salzmann, M.: Zur Anatomie der angeborenen. Sichel nach innen-unten. Albrecht von Graefe's Arch Klin. Ophthalmol. 39:131, 1893. 12. Elschnig, A.: Ueber opticociliare Gefässe. Klin. Monatsbl. Augenheilkd. 26:93, 1898.
M.D.,
Pennsylvania
An optociliary shunt rarely occurs be tween the central retinal vein and peripapillary choroid in the region of the lamina cribrosa. 1 It may indicate an asso ciated spheno-orbital meningioma, 2 - 4 chronic atrophie papilledema, 2 central retinal vein occlusion, 4 or glioma of the optic disk. 5 This paper describes the clinical syn drome of a cranio-orbital meningioma with optociliary veins demonstrated by fluorescein angiography and confirmed by histopathologic examination. CASE REPORT A 40-year-old white woman complained of gradu al decreasing vision and pain in the right eye associ ated with unilateral proptosis since 1966. In April 1974, she noted decreased vision in her left eye and was referred to the Neuro-Ophthalmology Service. General physical examination revealed hyperten sion. Visual acuity was R.E.: no light perception, and L.E.: 20/70 with a central scotoma. The right eye showed 4 to 5 mm of proptosis with resistance to retropulsion of the globe. No masses were palpable. Motility examination revealed right exotropia with decreased movement of the right eye in all direc tions of gaze. A full range of motion was present in the left eye. There was ectropion uveae and neovascularization of the angle extending to the anterior lens surface of the right eye. Vitreous clouding from an old hemorrhage was noted. Ophthalmoscopy revealed massive chronic disk edema of the right eye with prominent optociliary veins (Fig. 1, top). The left optic disk was mildly edematous with hemor rhages and exudates visible at the macula (Fig. 1, bottom). Fluorescein angiography of the right eye showed lack of filling of the optociliary veins in the arterial phase with fluorescence in the venous phase (Fig. 2). Routine laboratory investigations were within normal limits. An electrocardiogram showed left From the Neuro-Ophthalmology Service (Drs. Savino and Schatz), Department of Pathology (Dr. Rodrigues), Wills Eye Hospital, Thomas Jefferson University and the Naval Regional Medical Center (Dr. Savino), Philadelphia, Pennsylvania. Reprint requests to M. Rodrigues, M.D., or N. J. Schatz, M.D., Wills Eye Hospital, 1601 Spring Gar den St., Philadelphia, PA 19130.
Fig. 1 (Rodrigues, Savino, and Schatz). Top, Right eye. F u n d u s photograph showing two optociliary shunt vessels (arrows) and disk edema. Bottom, Left eye. Hypertensive retinopathy with macular exu dates. atrial enlargement. Roentgenograms of the chest and intravenous pyelography were normal. Polytomography demonstrated an enlarged right optic canal, expansion of the right orbit medially, a thinned lamina papyracea with a concavity consistent with elevated intraorbital pressure of long duration, and slight thickening of the planum sphenoidale. Carot id arteriography showed a dumbbell tumor " b l u s h " in the right juxtasellar region and irregularity of the supraclinoid portion of the internal carotid artery,
666
VOL. 81, NO. 5
SPHENO-ORBITAL MENINGIOMA
667
and filled the suprasellar space, measuring approxi mately 2.5 cm in diameter. The right orbit was unroofed and a firm, grayish tumor occupied the muscle cone and the posterior orbit. The optic nerve was four to five times thicker than normal from the posterior portion of the globe to the orbital apex. The nerve was sectioned posterior to the globe and residual tumor was noted in the muscle cone inferiorly. Microscopic examination of the tumor showed a meningothelial meningioma. On March 20, 1975, orbital exploration revealed an orbital tumor invad ing the optic nerve and sciera that we enucleated.
Fig. 2 (Rodrigues, Savino, and Schatz). Top, Fluorescein angiography. Arterial phase of fluorescein angiogram with non-filling of the optociliary shunt (arrows). Diffuse leakage is visible at the markedly elevated disk. Bottom, Venous phase demonstrating filling of the optociliary shunt vessels (arrows). with elevation of the main trunk of the anterior cerebral artery (Fig. 3, left). In the capillary phase, a diffuse tumor blush supplied primarily by the oph thalmic artery was noted in the region of the right orbit (Fig. 3, right). A right frontal craniotomy performed on April 24 revealed a small fungating tumor arising from the right optic nerve sheath. An additional meningeal blood supply was detected from the posterior part of the planum sphenoidale and the circular sinus. The right optic nerve was resected anterior to the chiasm. The tumor encircled the right internal carotid artery
Histopathology—Gross examination re vealed a firm right globe measuring 21 X 21 x 21 mm with 1 mm of attached optic nerve. The clear cornea measured 11.5 x 10.5 mm. Transillumination of the globe was poor. The eye was sectioned horizontally. The anterior chamber was of normal depth and the angle was open. The lens was in place. The iris and ciliary body were normal. The retina was de tached by hemorrhage and the optic disk was thickened and infiltrated by the me ningeal tumor. Microscopic examination revealed an orbital meningioma (Fig. 4) with invasion of the sciera and peripapillary choroid (Figs. 5 and 6). Prominent dilated shunt vessels connect ed the central retinal vein and peripapillary choroid, with adjacent nests of meningiomatous infiltration. Other oc ular findings included secondary optic atrophy, angle closure secondary to pe ripheral anterior synechiae, rubeosis iridis, hemorrhagic retinal detachment, and neovascularization of the vitreous humor. DISCUSSION
Invasion of the peripapillary choroid and sciera is rare in primary orbital me ningioma, 6 - 8 in meningioma of the tuberculum sellae, 9 and in spheno-orbital me ningioma, as in the present case. Optocili ary shunts have not been reported in primary orbital meningioma and these abnormal vascular channels suggest sphenoid ridge meningioma. 3 The presence of optociliary veins, re duced vision, and chronic atrophie disk
668
AMERICAN JOURNAL OF OPHTHALMOLOGY
MAY, 1976
Fig. 3 (Rodrigues, Savino, and Schatz). Left, Right carotid arteriogram (anteroposterior) demonstrates elevation of the Al portion of the anterior cerebral artery (arrows) indicative of intracranial extension of the tumor. Right, Carotid arteriogram showing a tumor blush (arrows) in the orbit supplied by the ophthalmic artery.
edema are characteristics of meningiomas involving the cranio-orbital junction. 3 Our case conforms to the syndrome de scribed by Frisen, Hoyt, and Tengroth. 3 Optociliary vessels are thought to rep resent enlargement of preformed capil lary vessels that shunt blood from the
central retinal vein to the peripapillary choroidal circulation when the normal drainage routes are obstructed. Chronicity of the obstruction causes dilatation of these channels, which becomes more evi dent when optic atrophy ensues. Optociliary veins may be congenital or
Fig. 4 (Rodrigues, Savino, and Schatz). Nests of meningothelial cells in the orbital tumor (hematoxylin-eosin, x200).
VOL. 81, NO. 5
SPHENO-ORBITAL MENINGIOMA
669
Fig. 5 (Rodrigues, Savino, and Schatz). Inset shows anomalous shunt vessels at the disk (arrows) adjacent to choroidal invasion by the peripapillary meningioma (M) (hematoxylin and eosin, x50). Nests of meningothelial meningioma (M) invading the choroid. Arrows indicate Bruch's membrane (hematoxylineosin, x200).
acquired. Braun 1 0 described two separate types of congenitally abnormal venous channels: Type 1 resembling retinal veins, and Type 2 appearing similar to choroidal veins (optociliary veins). He noted that they are broader than the papil lary veins, lighter in color, and lack the streak reflex. They can be differentiated from acquired optociliary veins by the tortuosity and ectasia of the latter shunts. Acquired optociliary veins are encoun tered in several conditions. They have been observed in chronic atrophie papilledema, 2 central retinal vein occlusion, 4 and gliomas of the optic disk. 5 Fluorescein angiography is helpful in documenting the true nature of the shunt. The optocili ary veins do not fluoresce during the arterial phase of fluorescein angiography but divert fluorescein to the choroidal circulation in the venous phase.
Salzmann 1 1 reported a case of myxosarcoma (glioma) of the optic nerve with associated Fuchs' coloboma and a probable optociliary shunt. These shunt vessels were not noted clinically and his tologie documentation was inadequate. Elschnig 1 2 described clinically a congen ital optociliary shunt in a 16-year-old boy with no evidence of an orbital or intracranial neoplasm. He also observed anoma lous cilioretinal vessels in a 43-year-old woman with an orbital endothelioma (meningioma). In both cases the shunts were not demonstrated histologically. In our case, we demonstrated clinically documented optociliary shunt vessels with histopathologic verification of a true retinal choroidal vascular anastomosis, and correlated the fluorescein angio graphie and histopathologic appearance of these shunt vessels.
Fig. 6 (Rodrigues, Savino, and Schatz). Numerous prominent shunt vessels (arrows) between the central retinal vein and peripapillary choroid, adjacent to meningiomatous invasion (M) (hematoxylin-eosin, x200). SUMMARY
A 40-year-old white woman had slowly progressive unilateral loss of visual acuity and increasing proptosis during an eightyear period. Ophthalmoscopy and fluorescein angiography revealed chronic disk edema and optociliary shunt vessels in the right eye. Polytomography showed an enlarged right optic canal. Cerebral arteriography demonstrated a dumbbellshaped tumor blush in the right juxtasellar region and a diffuse tumor blush in the right orbit. Right frontal craniotomy and orbital exploration . showed a cranioorbital junction (spheno-orbital) meningi oma that invaded the sciera and peripa pillary choroid. Anomalous optociliary veins were demonstrated histologically at the optic disk. ACKNOWLEDGMENT
Carol Starrels, Ph.D., assisted with translation.
REFERENCES 1. Hayreh, S. C : Blood supply of the optic nerve head and its role in optic atrophy, glaucoma and
edema of the optic disc. Br. J. Ophthalmol. 53:721, 1969. 2. Hedges, T. P.: Papilledema. Its recognition and relation to increased intracranial pressure. Survey Ophthalmol. 19:201, 1975. 3. Frisen, L., Hoyt, W. F., and Tengroth, B. M.: Optociliary veins, disc pallor and visual loss. A triad of signs indicating spheno-orbital meningioma. Acta Ophthalmol. 51:241, 1973. 4. Sanders, M. D.: A classification of papilledema based on a fluorescein angiographie study of 69 cases. Trans. Ophthalmol. Soc. U. K. 89:117, 1969. 5. Hoyt, W. F.,and Beeston, D.: The Ocular Fun dus in Neurologic Disease. St. Louis, C. V. Mosby, 1966, p . 42. 6. Karp, L. A., Zimmerman, L. E., Borit, A., and Spencer, W.: Primary intraorbital meningiomas. Arch. Ophthalmol. 91:24, 1974. 7. Martin, V. A. F., and Schofield, P. B.: Meningi oma invading the optic nerve. Br. J. Ophthalmol. 41:161, 1957. 8. Hudson, A. C : Primary tumors of the optic nerve. R. Ophthalmol. Hosp. Rep. 18:317, 1912. 9. Newell, F. W., and Beaman, T. C : Ocular signs of meningioma. Am. J. Ophthalmol. 45:30, 1958. 10. Braun: Ein Beitrag zur Kenntnis optikociliarer Gefässe. Klin. Monatsbl. Augenheilkd. 43:579, 1905. 11. Salzmann, M.: Zur Anatomie der angeborenen. Sichel nach innen-unten. Albrecht von Graefe's Arch Klin. Ophthalmol. 39:131, 1893. 12. Elschnig, A.: Ueber opticociliare Gefässe. Klin. Monatsbl. Augenheilkd. 26:93, 1898.
