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Optic Nerve Meningioma
Optic Nerve Meningioma JOHN R. SAMPLES, MD, DENNIS M. ROBERTSON, MD, JOEL Z. TAYLOR, MD, ROBERT R. WALLER, MD
Abstract: An obese white woman presented with a 10-month history of progressive loss of vision in the right eye associated with a moderate proptosis. Examination of the fundus revealed marked congestion of the retinal vasculature with dilation of both the arteries and the veins. Numerous blot hemorrhages were scattered throughout the retina. The retina was elevated and appeared to be displaced anteriorly by solid tissue, but B-scan ultrasonography demonstrated only a serous detachment of a thickened, markedly convoluted retina. A diagnosis of optic nerve meningioma was considered, but definitive treatment was deferred because of medical problems. One month later the patient returned with a blind, painful right eye associated with neovascular glaucoma. High resolution CT coronal sections of the orbit showed generalized thickening of the optic nerve. The blind eye was enucleated facilitating exposure of the optic nerve which was found to be diffusely thickened by meningioma. The orbital portion of the optic nerve was subsequently removed and meningioma was demonstrated along its entire length to the bony optic canal. A transfrontal craniotomy was later performed to rid the patient of residual tumor. [Key words: meningioma, optic nerve.] Ophthalmology 90: 1591-1594, 1983
Primary orbital meningiomas are difficult to diagnose and are relatively rare. In 1972, Spencer and Hoyt described the characteristic association of long-standing visual loss, optic disc edema, and optociliary veins that may be seen in patients with primary orbital meningiomas. 1 All of these manifestations are not always present. Patients suspected of having optic nerve meningiomas require radiologic evaluation including routine skull films and computerized tomography (CT) with and without contrast. Orbital tomograms have played an important role in the diagnosis of this entity in the past, but this has been less so recently. Older CT scanners may miss tumors that newer, higher resolution scanners have little difficulty in detecting. 2 Moreover, it is important for ophthalmologists to review CT scans with the radiologist to correlate relevant clinical information with the x-ray findings.
had previously been treated with systemic steroids for presumed optic neuritis. In December of 1978, she had seen an ophthalmologist who observed a markedly elevated optic disc associated with multiple hemorrhages throughout the posterior pole. Although a CT scan initially suggested thickening of the right optic nerve, it was interpreted as negative after the injection of contrast media. Polytomograms of the orbital canal were normal. The ophthalmologist suggested surgical exploration, favoring an intracranial approach to the optic nerve. Because of the patient's excessive obesity, however, immediate surgery was judged imprudent.
CASE REPORT A 365 pound, 5'10", 41-year-old white woman first presented to the Mayo Clinic Eye Department in May of 1979 with a 10month history of progressive loss of vision in the right eye. She From the Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota. Reprint requests to Dennis M. Robertson, MD, Department of Ophthalmology, Mayo Clinic, Rochester, MN 55905.
Fig 1. Appearance of the right fundus demonstrates dilation of both arteries and veins, numerous retinal folds radiating from the disc, and multiple blot hemorrhages scattered within the retinal convolutions.
1591
OPHTHALMOLOGY
•
DECEMBER 1983
•
VOLUME 91
•
NUMBER 12
Fig 2. B-scan ultrasound examination of the right eye demonstrating a thickened retina with a serous detachment. When seen at the Mayo Clinic, the right eye was blind. Visual acuity in the left eye was 20/20 and the visual field was full. Applanation tensions were normal. Mild periorbital edema was observed on the right side and there was a suggestion of increased resistance to retrodisplacement of the globe. A Krahn exophthalmometer measurement revealed 23 units on the right and 20 units on the left. After pupillary dilation, a moderate number of red blood cells were visible suspended in the vitreous. The fundus (Fig I) showed congestion and dilation of both arteries and veins with numerous blot hemorrhages scattered through the retinal tissue. The retina was elevated and appeared to be displaced anteriorly by solid tissue, but B-scan ultrasonography (Fig 2) demonstrated only a serous detachment of a markedly thickened and convoluted retina. Fluorescein angiography (Fig 3) made the vessel tortuosity especially apparent. There was slight delay in venous filling, and temporal to the macula the retinal circulation failed to fill, even in the later pictures. Leakage of dye into the posterior and peripapillary retina was visible in the late frames. An orbital CT scan, which included sections through the optic nerve was interpreted to be normal. The patient was asked to return at a later date for coronal views with a CT scanner capable of higher resolution. One month later, the patient returned with neovascular glaucoma and pain in the right eye. The intraocular tensions by applanation were 68 mmHg in the right eye and 18 mmHg in the left. Coronal sections obtained from the high resolution scanner demonstrated optic nerve enlargement (Fig 4).
1592
Fig 3. Early, middle, and late phases of fluorescein angiogram. Initially, vessel toruosity is noted. There is a delay in venous filling and the retinal circulation fails to fill in even in late pictures. Leakage of dye into the posterior and peripapillary retina is visible in the late phase.
SAMPLES, et al
•
OPTIC NERVE MENINGIOMA
Fig 4, A, left, orbital CT scan showing diffusely thickened right optic nerve. B, right, coronal CT scan demonstrating cross section enlargement of right optic nerve.
It was elected to enucleate the right eye because of the painful glaucoma and in order to facilitate exposure to the optic nerve, rather than perform a transfrontal craniotomy. Frozen sections ofthe transected thickened nerve immediately behind the globe revealed the presence of meningioma. The optic nerve was then dissected 21 mm posteriorly towards the optic canal and removed. Frozen sections revealed meningioma remaining in the end cut nearest the orbital apex. In July 1979, in an effort to rid the patient of residual meningioma, the bony canal of the right optic nerve was unroofed through a transfrontal craniotomy and the remaining II mm of optic nerve was removed. At the distal portion of the resected optic nerve, a small island of residual tumor was seen. No tumor was recognized at the proximal end. When seen at follow-up, 2 months later, the patient was free of discomfort and had a satisfactory cosmetic result with an ocular prosthesis.
PATHOLOGIC FINDINGS The optic nerve was fixed in formalin and submitted for routine histopathologic diagnosis. Hematoxylin-eosin sections showed that the meningioma had replaced almost all of the normal optic nerve tissue (Fig 5). The typical pattern of sworling meningiothelial cells occurring in nests is seen. Extensive infiltration of the optic nerve along the line of resection was present. The retina was detached, disorganized, and gliotic. Subretinal exudate was present.
DISCUSSION The clinical finding of an elevated edematous retina, proptosis, and neovascular glaucoma are unusual man-
ifestations of optic nerve meningioma. Rarely, meningiomas can indent the posterior pole of the eye and produce choroidal folds. The retina of this patient appeared to be elevated by a mass but ultrasound examination showed only a serous detachment. Although there was marked congestion of the retinal vasculature with dilation of both arteries and veins, typical optic-ciliary shunt vessels, indicative of a retrobulbar mass lesion producing compression upon the optic nerve, were not visible. The presence of a progressive relentless visual loss and the later development of proptosis led to the clinical consideration of optic nerve meningioma. CT scanning remains the most helpful, nonsurgical modality for making the diagnosis of meningiomas of the optic nerve. CT scans may demonstrate a uniform or segmental enlargement of the nerve or a localized mass anywhere along the course of the nerve, up to and including the orbital apex. In this case, the first CT scan was negative. The second scan completed with higher resolution coronal views demonstrated generalized, rather uniform enlargement of the nerve extending from the back of the globe to the optic foramen. The need for surgery of primary orbital meningiomas is controversial. It has been difficult to determine the natural course of orbital meningiomas in the last several years since many cases diagnosed recently have had surgery. These tumors are relatively indolent and slow-growing, and patients may survive many years after a presumed diagnosis has been made. Alper4 has described a total of 55 patients with primary optic nerve meningiomas. He has found that the tumors are especially aggressive in young patients. Older patients tend to have a more benign course. The approach to surgery for meningiomas at this 1593
OPHTHALMOLOGY •
DECEMBER 1983 •
VOLUME 91
•
NUMBER 12
Fig 5. A, top left, microscopic hematoxylin-eosin section of the right optic nerve showing meningoepitheliomatous meningioma with extensive infiltration of the optic nerve. Extensive detachment of disorganized, g1iotic retina is seen. Intraretinal hemorrhages are present. B, top right, cross sectional view of the optic nerve demonstrating the extensive tumor infiltration which replaces all but a small island of the normal nerve tissue. The normal nerve tissue is visible as a small wedge at the bottom of the photograph (H&E X4). C, bottom left. and D, bottom right. whorls of syncitial polygonal cells of meningoepitheliomatous meningioma. A psammoma body present in lower right (H&E X400, X630).
clinic has, in general, been a conservative one. Hollenhorst et al made the point that the overall prognosis remains poor with or without surgery. 3 Alper, however, advocates craniotomy with total removal of the roof of the orbit and the optic canal in young patients and more radical therapy if intracranial extension is present. When surgery is considered, CT scanning may be a valuable aid in deciding which patients require transcranial surgery to remove completely an orbital meningioma. Proptosis and secondary glaucoma due to rubeosis, as seen in this patient, have generally been considered unusual manifestations of optic nerve meningioma. In Alper's series, visual loss appeared before exophthalmos in 35 cases while 10 patients presented with exophthalmos simultaneously. The present case is of interest for several reasons. It calls attention to the necessity of entertaining the diagnosis of orbital meningioma even in the presence of a negative CT scan. It points out that proptosis and neovascular glaucoma can be seen with optic nerve me-
1594
ningioma, and it illustrates the problem inherent in an orbital approach to these tumors which, if they extend through the optic canal, may require a second transcranial procedure.
REFERENCES 1. Spencer WHo Hoyt WF. Chronic disc edema from neoplastic involvement of the perioptic meninges. Int Ophthalmol Clin 1971; 11 (3): 17187. 2. Trokel SL. Hilal SK. Submillimeter resolution CT scanning of orbital disease. Ophthalmology 1980; 87:412-18. 3. Hollenhorst RW Jr. Hollenhorst RW Sr, MacCarty CS. Visual prognosiS of optiC nerve sheath meningiomas producing shunt vessels on the optic disc. Mayo Clin Proc 1978; 53:84-92; Also: Trans Am Ophthalmol Soc 1977; 75:141-63. 4. Alper MG. Management of primary optic nerve meningiomas: current status-therapy in controversy. J Clin Neuro-Ophthalmol1981; 1:101-
17.
Abstract: An obese white woman presented with a 10-month history of progressive loss of vision in the right eye associated with a moderate proptosis. Examination of the fundus revealed marked congestion of the retinal vasculature with dilation of both the arteries and the veins. Numerous blot hemorrhages were scattered throughout the retina. The retina was elevated and appeared to be displaced anteriorly by solid tissue, but B-scan ultrasonography demonstrated only a serous detachment of a thickened, markedly convoluted retina. A diagnosis of optic nerve meningioma was considered, but definitive treatment was deferred because of medical problems. One month later the patient returned with a blind, painful right eye associated with neovascular glaucoma. High resolution CT coronal sections of the orbit showed generalized thickening of the optic nerve. The blind eye was enucleated facilitating exposure of the optic nerve which was found to be diffusely thickened by meningioma. The orbital portion of the optic nerve was subsequently removed and meningioma was demonstrated along its entire length to the bony optic canal. A transfrontal craniotomy was later performed to rid the patient of residual tumor. [Key words: meningioma, optic nerve.] Ophthalmology 90: 1591-1594, 1983
Primary orbital meningiomas are difficult to diagnose and are relatively rare. In 1972, Spencer and Hoyt described the characteristic association of long-standing visual loss, optic disc edema, and optociliary veins that may be seen in patients with primary orbital meningiomas. 1 All of these manifestations are not always present. Patients suspected of having optic nerve meningiomas require radiologic evaluation including routine skull films and computerized tomography (CT) with and without contrast. Orbital tomograms have played an important role in the diagnosis of this entity in the past, but this has been less so recently. Older CT scanners may miss tumors that newer, higher resolution scanners have little difficulty in detecting. 2 Moreover, it is important for ophthalmologists to review CT scans with the radiologist to correlate relevant clinical information with the x-ray findings.
had previously been treated with systemic steroids for presumed optic neuritis. In December of 1978, she had seen an ophthalmologist who observed a markedly elevated optic disc associated with multiple hemorrhages throughout the posterior pole. Although a CT scan initially suggested thickening of the right optic nerve, it was interpreted as negative after the injection of contrast media. Polytomograms of the orbital canal were normal. The ophthalmologist suggested surgical exploration, favoring an intracranial approach to the optic nerve. Because of the patient's excessive obesity, however, immediate surgery was judged imprudent.
CASE REPORT A 365 pound, 5'10", 41-year-old white woman first presented to the Mayo Clinic Eye Department in May of 1979 with a 10month history of progressive loss of vision in the right eye. She From the Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota. Reprint requests to Dennis M. Robertson, MD, Department of Ophthalmology, Mayo Clinic, Rochester, MN 55905.
Fig 1. Appearance of the right fundus demonstrates dilation of both arteries and veins, numerous retinal folds radiating from the disc, and multiple blot hemorrhages scattered within the retinal convolutions.
1591
OPHTHALMOLOGY
•
DECEMBER 1983
•
VOLUME 91
•
NUMBER 12
Fig 2. B-scan ultrasound examination of the right eye demonstrating a thickened retina with a serous detachment. When seen at the Mayo Clinic, the right eye was blind. Visual acuity in the left eye was 20/20 and the visual field was full. Applanation tensions were normal. Mild periorbital edema was observed on the right side and there was a suggestion of increased resistance to retrodisplacement of the globe. A Krahn exophthalmometer measurement revealed 23 units on the right and 20 units on the left. After pupillary dilation, a moderate number of red blood cells were visible suspended in the vitreous. The fundus (Fig I) showed congestion and dilation of both arteries and veins with numerous blot hemorrhages scattered through the retinal tissue. The retina was elevated and appeared to be displaced anteriorly by solid tissue, but B-scan ultrasonography (Fig 2) demonstrated only a serous detachment of a markedly thickened and convoluted retina. Fluorescein angiography (Fig 3) made the vessel tortuosity especially apparent. There was slight delay in venous filling, and temporal to the macula the retinal circulation failed to fill, even in the later pictures. Leakage of dye into the posterior and peripapillary retina was visible in the late frames. An orbital CT scan, which included sections through the optic nerve was interpreted to be normal. The patient was asked to return at a later date for coronal views with a CT scanner capable of higher resolution. One month later, the patient returned with neovascular glaucoma and pain in the right eye. The intraocular tensions by applanation were 68 mmHg in the right eye and 18 mmHg in the left. Coronal sections obtained from the high resolution scanner demonstrated optic nerve enlargement (Fig 4).
1592
Fig 3. Early, middle, and late phases of fluorescein angiogram. Initially, vessel toruosity is noted. There is a delay in venous filling and the retinal circulation fails to fill in even in late pictures. Leakage of dye into the posterior and peripapillary retina is visible in the late phase.
SAMPLES, et al
•
OPTIC NERVE MENINGIOMA
Fig 4, A, left, orbital CT scan showing diffusely thickened right optic nerve. B, right, coronal CT scan demonstrating cross section enlargement of right optic nerve.
It was elected to enucleate the right eye because of the painful glaucoma and in order to facilitate exposure to the optic nerve, rather than perform a transfrontal craniotomy. Frozen sections ofthe transected thickened nerve immediately behind the globe revealed the presence of meningioma. The optic nerve was then dissected 21 mm posteriorly towards the optic canal and removed. Frozen sections revealed meningioma remaining in the end cut nearest the orbital apex. In July 1979, in an effort to rid the patient of residual meningioma, the bony canal of the right optic nerve was unroofed through a transfrontal craniotomy and the remaining II mm of optic nerve was removed. At the distal portion of the resected optic nerve, a small island of residual tumor was seen. No tumor was recognized at the proximal end. When seen at follow-up, 2 months later, the patient was free of discomfort and had a satisfactory cosmetic result with an ocular prosthesis.
PATHOLOGIC FINDINGS The optic nerve was fixed in formalin and submitted for routine histopathologic diagnosis. Hematoxylin-eosin sections showed that the meningioma had replaced almost all of the normal optic nerve tissue (Fig 5). The typical pattern of sworling meningiothelial cells occurring in nests is seen. Extensive infiltration of the optic nerve along the line of resection was present. The retina was detached, disorganized, and gliotic. Subretinal exudate was present.
DISCUSSION The clinical finding of an elevated edematous retina, proptosis, and neovascular glaucoma are unusual man-
ifestations of optic nerve meningioma. Rarely, meningiomas can indent the posterior pole of the eye and produce choroidal folds. The retina of this patient appeared to be elevated by a mass but ultrasound examination showed only a serous detachment. Although there was marked congestion of the retinal vasculature with dilation of both arteries and veins, typical optic-ciliary shunt vessels, indicative of a retrobulbar mass lesion producing compression upon the optic nerve, were not visible. The presence of a progressive relentless visual loss and the later development of proptosis led to the clinical consideration of optic nerve meningioma. CT scanning remains the most helpful, nonsurgical modality for making the diagnosis of meningiomas of the optic nerve. CT scans may demonstrate a uniform or segmental enlargement of the nerve or a localized mass anywhere along the course of the nerve, up to and including the orbital apex. In this case, the first CT scan was negative. The second scan completed with higher resolution coronal views demonstrated generalized, rather uniform enlargement of the nerve extending from the back of the globe to the optic foramen. The need for surgery of primary orbital meningiomas is controversial. It has been difficult to determine the natural course of orbital meningiomas in the last several years since many cases diagnosed recently have had surgery. These tumors are relatively indolent and slow-growing, and patients may survive many years after a presumed diagnosis has been made. Alper4 has described a total of 55 patients with primary optic nerve meningiomas. He has found that the tumors are especially aggressive in young patients. Older patients tend to have a more benign course. The approach to surgery for meningiomas at this 1593
OPHTHALMOLOGY •
DECEMBER 1983 •
VOLUME 91
•
NUMBER 12
Fig 5. A, top left, microscopic hematoxylin-eosin section of the right optic nerve showing meningoepitheliomatous meningioma with extensive infiltration of the optic nerve. Extensive detachment of disorganized, g1iotic retina is seen. Intraretinal hemorrhages are present. B, top right, cross sectional view of the optic nerve demonstrating the extensive tumor infiltration which replaces all but a small island of the normal nerve tissue. The normal nerve tissue is visible as a small wedge at the bottom of the photograph (H&E X4). C, bottom left. and D, bottom right. whorls of syncitial polygonal cells of meningoepitheliomatous meningioma. A psammoma body present in lower right (H&E X400, X630).
clinic has, in general, been a conservative one. Hollenhorst et al made the point that the overall prognosis remains poor with or without surgery. 3 Alper, however, advocates craniotomy with total removal of the roof of the orbit and the optic canal in young patients and more radical therapy if intracranial extension is present. When surgery is considered, CT scanning may be a valuable aid in deciding which patients require transcranial surgery to remove completely an orbital meningioma. Proptosis and secondary glaucoma due to rubeosis, as seen in this patient, have generally been considered unusual manifestations of optic nerve meningioma. In Alper's series, visual loss appeared before exophthalmos in 35 cases while 10 patients presented with exophthalmos simultaneously. The present case is of interest for several reasons. It calls attention to the necessity of entertaining the diagnosis of orbital meningioma even in the presence of a negative CT scan. It points out that proptosis and neovascular glaucoma can be seen with optic nerve me-
1594
ningioma, and it illustrates the problem inherent in an orbital approach to these tumors which, if they extend through the optic canal, may require a second transcranial procedure.
REFERENCES 1. Spencer WHo Hoyt WF. Chronic disc edema from neoplastic involvement of the perioptic meninges. Int Ophthalmol Clin 1971; 11 (3): 17187. 2. Trokel SL. Hilal SK. Submillimeter resolution CT scanning of orbital disease. Ophthalmology 1980; 87:412-18. 3. Hollenhorst RW Jr. Hollenhorst RW Sr, MacCarty CS. Visual prognosiS of optiC nerve sheath meningiomas producing shunt vessels on the optic disc. Mayo Clin Proc 1978; 53:84-92; Also: Trans Am Ophthalmol Soc 1977; 75:141-63. 4. Alper MG. Management of primary optic nerve meningiomas: current status-therapy in controversy. J Clin Neuro-Ophthalmol1981; 1:101-
17.