The Neuroradiology Journal 20: 179-181, 2007
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Bilateral Staphylomas Presenting as Bitemporal Hemianopia M. MATHEWS*, A. HASSO** * Department of Neurosurgery, ** Department of Radiology, University of California; Irvine, USA
Key words: Staphyloams, bilateral, scleral ectasia, bitemporal hemianopia, sellar mass
SUMMARY – Bitemporal hemianopia is usually secondary to pituitary and suprasellar masses. We present a case of bitemporal hemianopia secondary to bilateral staphylomas of the ocular globe. Staphylomas are degenerative outpouchings of the eyeball also known as scleral ectasia, secondary to congenital weakness of scleral tissue. Staphylomas can cause complications such as choroidal neovascularizations and hemorrhage. Early diagnosis and counselling are the mainstays of management. Bilateral staphylomas should be considered in the differential diagnosis of bitemporal hemianopia especially when imaging rules out sellar and suprasellar masses.
Introduction Peripapillary staphylomas (ectasia) are among the rarest congenital anomalies of the eye 1. Peripapillary ectasia is a deep excavation involving the posterior pole of the globe. Patients with staphylomas of the eye usually present with refractory errors in the affected eye. We report a unique case of bilateral peripapillary staphylomas that presented with bitemporal hemianopia. Case Report A 61-year-old woman with a medical history significant for osteoarthritis and ophthalmological history of keratoconjunctivitis sicca, high myopia and astigmatism complained of chronic visual disturbance and persistent blurriness of vision in her left eye. The patient had no known drug allergies and was taking daily aspirin as her only medication. On initial examination, the patient was found to have 20/60- vision in her right eye and 20/60- vision in her left eye, with corrective lenses. Her intraocular pressure was 13 in the right eye and ten in the left eye. At the time of presentation, no obvious abnormality was found on fundoscopic examination, or with optical coherence tomography. To further evaluate
her condition, visual field analysis was ordered and the patient was asked to return to clinic in one week for a re-examination. At follow up examination one week later, visual acuity and intraocular pressure were unchanged from previous measurements. Fundoscopic examination, however, revealed bilateral nuclear sclerosis, bilateral retinal pigment epithelial changes, and tilted optic discs. Also at this follow up visit, the patient’s visual field analysis was found to be significant for bitemporal visual field defects. The patient was at this time referred to neuro-ophthalmology for evaluation for possible optic neuropathy. Approximately two weeks after her initial presentation she complained of increasing vision loss in the central visual fields of her left eye. Examination at this point revealed pupils measuring seven millimeters that were round and equally reactive to light, intact extraocular muscles, and a normal slit lamp examination. Evaluation of the patient’s visual field with an Amsler Grid revealed bitemporal blurring of vision that was more pronounced on the left side. Fundoscopic examination confirmed the finding of tilted optic discs bilaterally. Cardiovascular and neurologic examinations revealed no significant findings. Magnetic resonance imaging of the brain and orbits was ordered at this time. T-2-weighted MR (TR 5225/ TE 119) images 179
Bilateral Staphylomas Presenting as Bitemporal Hemianopia
M. Mathews
Figure 1 Axial T2W MR image (5225/119) at the level of the ocular lenses. There is bilateral deformity of the posterior portion of both globes, more severe on the left side.
Figure 2 Visual fields obtained by automated perimetry testing. Dark areas represent areas of visual field loss.
done at 1.5 Tesla show bilateral flattening of the patients posterior globes, with the right side being affected more than the left (figure 1). Of note the left globe was more elongated posteriorly whereas the right globe was more elongated horizontally. Given these MRI findings and the patient’s changes in visual field, bilateral myopic staphyloma was diagnosed. Permietry testing showed bitemporal hemiano180
pia (figure 2). The patient was treated symptomatically. Discussion Bitemporal hemianopia is usually caused by pituitary tumors and suprasellar masses. This report highlights the need to look for causes
www. centauro. it
The Neuroradiology Journal 20: 179-181, 2007
Table 1 Classification of Staphylomas based on location 5.
Staphyloma
Location
Type 1
Extends from the nasal border of the optic nerve into the macular region. (Most common staphyloma seen in myopes)
Type 2
Centered on the macular
Type 3
Centered on the optic disc without macular extension
Type 4
Located nasal to the optic disc
Type 5
Located inferior to the optic disc
intrinsic to the eyeball in the differential diagnosis of bitemporal hemianopia, especially when sellar and suprasellar masses are absent in imaging studies of the brain 2. Peripapillary staphyloma needs to be distinguished from
coloboma of the optic disc and myopic conus 1. While in the defect in peripapillary staphyloma is within the abnormal nerve head itself, in coloboma the defect is usually temporal to the disk and progressive. Histologic examination of scleral actasia by Zimmermann showed it to be lined with remnants of retina and choroid 3. Rarely bilateral peripapillary staphylomas can present with normal vision 4. Staphylomas can be divided into five types based on their location (table 1). Our patient had Type 4 staphylomas bilaterally. Staphylomas can be associated with multiple complications including pigment epithelial atrophy, lacquer cracks, retinal and subretinal hemorrhages, and choroidal neovascularizations which can progress to complete vision loss 5,6. Follow-up for early detection of complications and patient education constitute the mainstay of management.
References 1 Singh D, Verma A: Bilateral Peripapillary staphyloma (ectasia). Ind. J. Ophthal 25: 50, 1978. 2 Manfre L, Vero S, Focarelli-Barone C et Al: Bitemporal pseudohemianopia related to the “Tilted Disk Syndrome”; CT, MR, and Fundoscopic findings. Am J Neuroradiol 20: 1750-1751, 1999. 3 Zimmermann RA: Quoted by Singh D and Verma A 1: 1897. 4 Caldwell JBH, Sears ML, Gilman M: Bilater peripapillary staphyloma with normal vision. Am J Ophthal. Jan 71: 423-425, 1971. 5 Apte RS, Sunnes JS, Goldstein BG et Al: Bilateral macular staphylomas in a patient with cone dystrophy. Br J Ophthalmol 87: 1049-51, 2003
6 Steidl SM, Pruett RC: Macular complications associated with posterior staphyloma. Am J Ophthal 123: 181-7, 1997.
Marlon S. Mathews, MD 101 The City Drive South, Bldg 56, Suite 400, Orange, CA 92868-3298 U.S.A. Tel.: (714) 456-6966 Fax: (714) 456-8284. E-mail: [email protected]
181
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Bilateral Staphylomas Presenting as Bitemporal Hemianopia M. MATHEWS*, A. HASSO** * Department of Neurosurgery, ** Department of Radiology, University of California; Irvine, USA
Key words: Staphyloams, bilateral, scleral ectasia, bitemporal hemianopia, sellar mass
SUMMARY – Bitemporal hemianopia is usually secondary to pituitary and suprasellar masses. We present a case of bitemporal hemianopia secondary to bilateral staphylomas of the ocular globe. Staphylomas are degenerative outpouchings of the eyeball also known as scleral ectasia, secondary to congenital weakness of scleral tissue. Staphylomas can cause complications such as choroidal neovascularizations and hemorrhage. Early diagnosis and counselling are the mainstays of management. Bilateral staphylomas should be considered in the differential diagnosis of bitemporal hemianopia especially when imaging rules out sellar and suprasellar masses.
Introduction Peripapillary staphylomas (ectasia) are among the rarest congenital anomalies of the eye 1. Peripapillary ectasia is a deep excavation involving the posterior pole of the globe. Patients with staphylomas of the eye usually present with refractory errors in the affected eye. We report a unique case of bilateral peripapillary staphylomas that presented with bitemporal hemianopia. Case Report A 61-year-old woman with a medical history significant for osteoarthritis and ophthalmological history of keratoconjunctivitis sicca, high myopia and astigmatism complained of chronic visual disturbance and persistent blurriness of vision in her left eye. The patient had no known drug allergies and was taking daily aspirin as her only medication. On initial examination, the patient was found to have 20/60- vision in her right eye and 20/60- vision in her left eye, with corrective lenses. Her intraocular pressure was 13 in the right eye and ten in the left eye. At the time of presentation, no obvious abnormality was found on fundoscopic examination, or with optical coherence tomography. To further evaluate
her condition, visual field analysis was ordered and the patient was asked to return to clinic in one week for a re-examination. At follow up examination one week later, visual acuity and intraocular pressure were unchanged from previous measurements. Fundoscopic examination, however, revealed bilateral nuclear sclerosis, bilateral retinal pigment epithelial changes, and tilted optic discs. Also at this follow up visit, the patient’s visual field analysis was found to be significant for bitemporal visual field defects. The patient was at this time referred to neuro-ophthalmology for evaluation for possible optic neuropathy. Approximately two weeks after her initial presentation she complained of increasing vision loss in the central visual fields of her left eye. Examination at this point revealed pupils measuring seven millimeters that were round and equally reactive to light, intact extraocular muscles, and a normal slit lamp examination. Evaluation of the patient’s visual field with an Amsler Grid revealed bitemporal blurring of vision that was more pronounced on the left side. Fundoscopic examination confirmed the finding of tilted optic discs bilaterally. Cardiovascular and neurologic examinations revealed no significant findings. Magnetic resonance imaging of the brain and orbits was ordered at this time. T-2-weighted MR (TR 5225/ TE 119) images 179
Bilateral Staphylomas Presenting as Bitemporal Hemianopia
M. Mathews
Figure 1 Axial T2W MR image (5225/119) at the level of the ocular lenses. There is bilateral deformity of the posterior portion of both globes, more severe on the left side.
Figure 2 Visual fields obtained by automated perimetry testing. Dark areas represent areas of visual field loss.
done at 1.5 Tesla show bilateral flattening of the patients posterior globes, with the right side being affected more than the left (figure 1). Of note the left globe was more elongated posteriorly whereas the right globe was more elongated horizontally. Given these MRI findings and the patient’s changes in visual field, bilateral myopic staphyloma was diagnosed. Permietry testing showed bitemporal hemiano180
pia (figure 2). The patient was treated symptomatically. Discussion Bitemporal hemianopia is usually caused by pituitary tumors and suprasellar masses. This report highlights the need to look for causes
www. centauro. it
The Neuroradiology Journal 20: 179-181, 2007
Table 1 Classification of Staphylomas based on location 5.
Staphyloma
Location
Type 1
Extends from the nasal border of the optic nerve into the macular region. (Most common staphyloma seen in myopes)
Type 2
Centered on the macular
Type 3
Centered on the optic disc without macular extension
Type 4
Located nasal to the optic disc
Type 5
Located inferior to the optic disc
intrinsic to the eyeball in the differential diagnosis of bitemporal hemianopia, especially when sellar and suprasellar masses are absent in imaging studies of the brain 2. Peripapillary staphyloma needs to be distinguished from
coloboma of the optic disc and myopic conus 1. While in the defect in peripapillary staphyloma is within the abnormal nerve head itself, in coloboma the defect is usually temporal to the disk and progressive. Histologic examination of scleral actasia by Zimmermann showed it to be lined with remnants of retina and choroid 3. Rarely bilateral peripapillary staphylomas can present with normal vision 4. Staphylomas can be divided into five types based on their location (table 1). Our patient had Type 4 staphylomas bilaterally. Staphylomas can be associated with multiple complications including pigment epithelial atrophy, lacquer cracks, retinal and subretinal hemorrhages, and choroidal neovascularizations which can progress to complete vision loss 5,6. Follow-up for early detection of complications and patient education constitute the mainstay of management.
References 1 Singh D, Verma A: Bilateral Peripapillary staphyloma (ectasia). Ind. J. Ophthal 25: 50, 1978. 2 Manfre L, Vero S, Focarelli-Barone C et Al: Bitemporal pseudohemianopia related to the “Tilted Disk Syndrome”; CT, MR, and Fundoscopic findings. Am J Neuroradiol 20: 1750-1751, 1999. 3 Zimmermann RA: Quoted by Singh D and Verma A 1: 1897. 4 Caldwell JBH, Sears ML, Gilman M: Bilater peripapillary staphyloma with normal vision. Am J Ophthal. Jan 71: 423-425, 1971. 5 Apte RS, Sunnes JS, Goldstein BG et Al: Bilateral macular staphylomas in a patient with cone dystrophy. Br J Ophthalmol 87: 1049-51, 2003
6 Steidl SM, Pruett RC: Macular complications associated with posterior staphyloma. Am J Ophthal 123: 181-7, 1997.
Marlon S. Mathews, MD 101 The City Drive South, Bldg 56, Suite 400, Orange, CA 92868-3298 U.S.A. Tel.: (714) 456-6966 Fax: (714) 456-8284. E-mail: [email protected]
181
