Aplastic Anemia Presenting as Spontaneous Orbital Hemorrhage

We report a case of a 51-year-old female presenting with a three-day history of a painful swollen right eye associated with loss of vision. She presented with no light perception in the affected eye, periocular ecchymosis, hemorrhagic chemosis, and an axial proptosis. CT scan revealed a unilateral proptosis with tension on the optic nerve and accompanying diffuse retrobulbar hemorrhage. Bone marrow studies confirmed aplastic anemia. A review of the English literature failed to reveal previous cases of aplastic anemia presenting with spontaneous orbital hemorrhage. We suggest that aplastic anemia be included in the differential diagnosis of a patient presenting with painful spontaneous orbital hemorrhage.     

Aplastic anemia presenting as spontaneous orbital hemorrhage

We report a case of a 51-year-old female presenting with a three-day history of a painful swollen right eye associated with loss of vision. She presented with no light perception in the affected eye, periocular ecchymosis, hemorrhagic chemosis, and an axial proptosis. CT scan revealed a unilateral proptosis with tension on the optic nerve and accompanying diffuse retrobulbar hemorrhage. Bone marrow studies confirmed aplastic anemia. A review of the English literature failed to reveal previous cases of aplastic anemia presenting with spontaneous orbital hemorrhage. We suggest that aplastic anemia be included in the differential diagnosis of a patient presenting with painful spontaneous orbital hemorrhage.     

Optic nerve cyst associated with optic disk pits

Purpose To report a case with two optic disk pits which were associated with an optic nerve cyst in the same eye.Methods Observational case report.Results A 47-year-old patient noted visual impairment in the right eye. On examination the best corrected visual acuity in the right eye was 20/80 and in the left eye was 20/20. Biomicroscopy revealed, in the right eye, a very pale optic disk with two optic disk pits without macular elevation. Magnetic resonance imaging (MRI) revealed a well circumscribed 6×6-mm² round cystic lesion within the right optic nerve sheath adjacent to the temporal aspect of the right optic nerve at its retrobulbar segment, which compressed and displaced the nerve.Conclusions In the case of an extremely pale optic disk with congenital pits and visual impairment without macular detachment, radiological examination is indicated in order to exclude the possibility of coexisting optic nerve anomalies.     

A case of optic nerve atrophy with severe disc cupping after methanol poisoning

We report a rare case of optic nerve atrophy with severe disc cupping resulting from methanol poisoning. A 30-year-old man presented to the hospital complaining of decreased visual acuity in both eyes a day after drinking alcohol containing methanol. His initial visual acuity allowed for only visualizing hand motion and not corrected in either eye. Initial intraocular pressure was within normal limits in both eyes. Initial fundus examination showed optic disc swelling in both eyes. Four years later, he visited our hospital for an eye evaluation. Visual acuity in both eyes still only allowed for visualizing hand motion. No nystagmus was observed in either eye during the optokinetic nystagmus test, and no waves were found in a visual evoked potential test. No specific change was noted on brain magnetic resonance imaging. On fundus examination, there was disc pallor in both eyes and disc cupping with a high cup/disc (C/D) ratio above 0.9 in the left eye. C/D ratio of the right eye was 0.5. Methanol poisoning may induce glaucomatous disc cupping in the late stage as well as optic atrophy. One possible mechanism of disc cupping is ganglion cell loss due to acute demyelination of the retrobulbar optic nerve. This report is the first photographic evidence of methanol induced optic disc cupping in Korea.     

A case of orbital myositis complicated with optic neuropathy--analysis of the pathological mechanism of optic neuropathy from magnetic resonance imaging findings

BACKGROUND: We observed a rare case of orbital myositis involving the optic nerve. Case: A 52-year-old woman complained of visual disturbance, lid swelling, ocular pain, and conjunctival injection in her right eye. Her corrected vision was 0.15 in the right eye and 1.2 in the left eye. Relative afferent pupillary defect(RAPD) and central scotoma in visual field test were noted in the right eye. Ocular movement of her right eye was moderately disturbed in all directions. Although laboratory data showed elevation of erythrocyte sedimentation rate, other hematological data such as thyroid function, autoimmune antibodies, and viral infection antibodies were normal. Magnetic resonance imaging(MRI) findings showed compression of the optic nerve at the orbital apex by marked thickening of the right lateral rectus muscle and superior rectus muscle, and inflammation directly invading the optic nerve. Based on the above findings, we diagnosed the case as orbital myositis complicated with optic neuropathy, and started corticosteroid therapy. Her right corrected vision improved dramatically, and the RAPD, central scotoma, and lid swelling disappeared shortly after administration. Enlargement of the extraocular muscles was still present one month after corticosteroid therapy. CONCLUSION: MRI findings suggested that optic neuropathy in this case was induced not only by mechanical compression by the enlarged extraocular muscles at the orbital apex but also by direct inflammatory infiltration from the extraocular muscles.     

A case of anterior ischemic optic neuropathy associated with Vogt-Koyanagi-Harada disease

BACKGROUND: Although optic disc swelling is one of the common findings of Vogt-Koyanagi-Harada (VKH) disease, severe visual field loss from optic disc involvement is not common. We report a case of severe visual field contraction from optic disc involvement in VKH disease. CASE REPORT: A 51-year-old woman was diagnosed as having VKH disease and was treated with intravenous pulse methylprednisolone. Exudative retinal detachments disappeared and visual acuity improved, but optic disc swelling was persistent in both eyes. Ten weeks after VKH disease onset, she claimed acute visual field loss in the right eye. Marked optic disc swelling with peripapillary hemorrhages and severe visual field loss were observed in the right eye. Fluorescein angiography showed filling delay and late leakage of the optic disc in the right eye. One month later, right optic disc swelling disappeared, but visual field loss remained. In addition, 5.5 months later, the left optic disc swelled further, and the left visual field was markedly contracted. Hyperbaric oxygen therapy was added. The swelling in the left optic disc gradually decreased and disappeared in one month, but the visual field loss remained. CONCLUSIONS: The optic disc involvement with irreversible visual field loss in this case is thought to be due to anterior ischemic optic neuropathy, which may be a possible complication of VKH disease.     

Enlargement of the optic disk in childhood optic nerve tumors

Two children, a 9-year-old girl and an 8-month-old girl, were first examined because of unilateral proptosis. Both children were found to have unilateral optic nerve glioma, accompanied in the second case by neurofibromatosis. Two years after Patient 1 was first examined, the initial findings of thin vertical retinal striae and a gray opacification of the peripapillary retina had resolved. The enlarged left optic disk had an average diameter of 2 mm while that of the normal right optic disk was 1.6 mm. The disk-arteriolar ratio was R.E.:16:1 and L.E.: 20:1. Four years later, the patient's visual acuity was still 20/20 in both eyes and the only visual field abnormality was an enlarged blind spot corresponding to the enlarged left optic nerve head. Fundus photographs, orbital echography, and computed tomography showed no change in the size or location of the optic nerve glioma during the six-year follow-up period. In Case 2, the initial intraocular pressure was higher in the proptotic right eye than in the left eye (25 vs 19 mm Hg), but subsequent intraocular pressures were within normal limits and approximately equal in the two eyes. The disk-arteriolar ratio was R.E.:21:1 and L.E.: 18:1 and the cup-disk ratio was R.E.: 0.7 and L.E.: 0.3. Computed tomography disclosed an enlarged right retrobulbar optic nerve, a superior orbital fissure that was much larger on the right than on the left, and a larger cavernous sinus on the right. The right sphenoid bone was partially absent. Despite mild developmental delay, the patient's visual acuity apparently remained normal and the ophthalmologic findings did not change significantly during the 18-month follow-up period.     

Bilateral Optic Disc Swelling Following Anastrozole Therapy

A 56-year-old female with early-stage breast cancer, stage IA grade 1 endometrial cancer, and stage IC grade 1 ovarian cancer developed sudden-onset visual changes and right inferior visual field defect following anastrozole therapy. Examination revealed severe bilateral optic disc swelling and impaired visual acuity. Laboratory work-up was otherwise unremarkable. Anastrozole was discontinued and over the next month, patient had near-complete resolution of swelling in the right eye and improvement in the left eye. This is the only reported case of optic disc swelling following anastrozole therapy.     

Combined central retinal artery and vein occlusion from orbital inflammatory pseudotumour

Progressive, painful blurred vision and proptosis developed in the left eye of a 32-year-old man over an 8-day period. On initial neuro-ophthalmic evaluation the visual acuity in the left eye was no light perception. Erythema of the periorbital skin, 5 mm of proptosis, and ophthalmoplegia were noted on the left side. Funduscopy revealed occlusion of the central retinal artery and central retinal vein. Computed tomography and magnetic resonance imaging revealed marked distension of the left optic nerve and enhancement of the contents within the left orbit, with clear paranasal sinuses. The diagnosis of orbital inflammatory pseudotumour was made and the orbital signs improved rapidly with 80 mg of prednisone per day. Combined occlusion of the central retinal artery and vein is a rare complication of orbital inflammatory pseudotumour. It is postulated that marked distension of the optic nerve caused mechanical compression of the central retinal vessels leading to the vascular occlusions.     

Optic nerve toxoplasmosis and orbital inflammation as initial presentation of AIDS

Purpose To report a case of toxoplasmosis with optic nerve and orbital involvement as the initial presentation of HIV infection. Method Case report. Results A 46-year-old zookeeper, who had had right central retinal vein occlusion (CRVO) 2 weeks previously, presented with painless lid and conjunctival swelling and profound visual loss in his right eye (RE). Examination revealed no light perception (NLP) RE with axial proptosis and ocular motility restriction; fundal examination revealed a clinical picture of an ischaemic CRVO. MRI of the brain and orbit showed ring-enhancing targetoid lesions in the brain and inflammatory changes in the right optic nerve, extraocular muscles and orbital fat. He was subsequently found to be HIV positive and had positive toxoplasma IgG serology. Conclusions Immunocompromised individuals have an increased likelihood for more severe and atypical presentations; this highlights the need for increased index of suspicion for HIV infection as ocular or orbital disease may be the first manifestation of life-threatening systemic toxoplasmosis. No financial support was received for this paper. The authors have no proprietary interests in the drugs and equipment used in this paper.     

Frontoethmoidal mucocele with ocular involvement

CASE REPORT: We report a case of a large mucocele occurring in a 64-year-old male. The patient presented with severe proptosis and long standing complete visual loss of the left eye. Computer tomography showed a large cystic mass arising from the left fronto-ethmoidal sinus that invaded the orbit and the anterior cranial fossa. Additional ophthalmologic findings were retinal hemorrhages, optic disc swelling, chorio-retinal folds and a complete afferent pupillary defect of the left eye. Surgical management of the mucocele resolved the proptosis and retinal hemorrhages but the visual loss and the chorio-retinal folds persisted. DISCUSSION: Mucoceles of the periorbital sinus may cause chorio-retinal alterations, optic neuropathy and permanent vision loss.     

Breast Cancer Metastasis Presenting as Conjunctival Chemosis

A 72-year-old woman presented with a painful right eye. A few weeks before, she had noticed a red, swollen area in the conjunctiva of the same eye. On slit lamp examination, it appeared as chemosis and vascular injection; artificial tears were prescribed. A month later, a firm mass developed on the superotemporal orbital rim, in the area of the lacrimal gland. A CT scan revealed infiltrative structures in both the left and right orbit, with contrast staining in the right lacrimal gland and near the left optic nerve. A biopsy was taken of the conjunctival swelling as well as of the lacrimal gland. Both tissues showed infiltration with lobular breast carcinoma metastases.Key Words: Breast cancer, Orbital metastasis, Conjunctival chemosis     

Opticociliary veins in a primary optic nerve sheath meningioma.

A 59-year-old man developed painless visual loss in his left eye over a one-year period, with rapid progression to blindness over three months. Ocular examination of the left eye revealed proptosis, marked optic disk pallor, dilated retinal veins, and congeries of vessels at the disk margin (opticociliary veins). His right eye was unremarkable. Fluorescein angiography and histopathologic examination confirmed the presence of opticociliary veins communicating between branches of the central retinal vein and the adjacent choroidal vasculature. Primary orbital optic nerve meningioma associated with opticociliary veins was diagnosed.     

Progressive visual loss. What type of mass lesion?

A 43-year-old woman presents with painless loss of vision in her left eye. She has decreased visual acuity, an afferent pupillary defect, slight proptosis and disc swelling, implying an optic nerve lesion. Computerized tomographic scanning shows generalized enlargement of the posterior orbital optic nerve. A presumed diagnosis of an optic nerve sheath meningioma is made. The discussion deals with the problems of rational treatment.     

The transconjunctival approach to a large retrobulbar cavernous hemangioma of the orbit

Cavernous hemangiomas are one of the most common benign tumors of the orbit in adults. We report a case of a longstanding retrobulbar hemangioma that was removed successfully through a temporal transconjunctival approach combined with lateral canthotomy. A 45-year-old female patient, with a 15-year history of slowly progressive proptosis and decreased visual acuity of the left eye, had a corrected visual acuity of finger count at 50 cm OS, compared with 1.0 OD. Exophthalmometry by the Nagle's method measured 15 mm OD by 26 mm OS. Magnetic resonance imaging (MRI) revealed a well-encapsulated retrobulbar main mass, 2.3 x 3.0 x 3.7 cm in size along with multiple small satellite nodules that were displacing the optic nerve and globe superiorly. The tumors were removed through a superotemporal transconjunctival approach combined with lateral canthotomy. Pathological examination revealed an intraorbital cavernous hemangioma. The patient was free of visible scars, proptosis and any other noticeable complications at her last follow-up, 6 months after surgery.     

Optic Nerve Meningeal Hemangiopericytoma: A Clinicopathologic Case Report

A 36-year-old woman presented with progressive loss of vision in the left eye for 3 years, and rapid progression and painful protrusion of the eye for one month. Clinical evaluation revealed no light perception, severe proptosis and hypoglobus, optic atrophy, and optociliary shunt vessels. Orbital imaging showed a well-defined heterogeneous intraconal mass partially encasing the optic nerve. A clinical diagnosis of optic nerve sheath meningioma was made, and the tumor was completely excised along with enucleation, followed by postoperative adjuvant external beam radiotherapy. There was no local recurrence at 15 month follow-up. Histopathologically, the tumor was found to be arising from the optic nerve meninges with classical “stag-horn” pattern and abundant cellularity. Immunohistochemistry supported the histopathological diagnosis of hemangiopericytoma. Optic nerve meningeal hemangiopericytoma is extremely rare—only two such cases have been reported in the literature.     

Subperiosteal orbital hemorrhage as initial manifestation of Christmas disease (factor IX deficiency)

PURPOSE: To report a case of subperiosteal orbital and subgaleal hemorrhage with optic nerve compromise in a patient with a factor IX deficiency. DESIGN: Interventional case report. METHODS: A 5-year-old male presented 10 days after mild trauma with progressive left-sided scalp swelling, proptosis, and visual loss. RESULTS: The patient had marked proptosis of the left eye, 20/200 visual acuity, and an afferent pupillary defect. Magnetic resonance imaging demonstrated a large subgaleal and left subperiosteal orbital hematoma. Quantitative assays of coagulation proteins identified a factor IX deficiency (Christmas disease). CONCLUSION: Delayed-onset subgaleal and subperiosteal orbital hematoma can rarely be an initial manifestation of Christmas disease.     

Optic nerve breast metastasis mimicking meningioma

Purpose: We report on an optic nerve breast metastasis masquerading initially as a central retinal vein occlusion and later as an optic nerve meningioma. Methods: A 60-year-old female presented with a left central retinal vein occlusion (CRVO). She represented 7 months later with left upper ptosis, proptosis and painful rubeotic glaucoma. Computed tomography (CT) and magnetic resonance imaging suggested an optic nerve meningioma. On referral to the regional orbital unit, a mild left external ophthalmoplegia was noted and, in view of previous right mastectomy and chemotherapy 3 years earlier, the left optic nerve was biopsied simultaneously with left enucleation of her painful eye. Results: Histopathology showed infiltration of the optic nerve and meningeal sheath spreading into the subretinal space and vitreous by malignant epithelial cells, consistent with breast origin. Further CT imaging and bone scans revealed no other metastases. Single field left orbit radiotherapy of 20 Gy was given in five fractions and Arimidex (Zeneca Pharmaceuticals, Cheshire, England) was commenced with the cessation of tamoxifen. The patient was also given an ocular prosthesis. Sadly, she lost vision in her other eye due to retrograde malignant invasion of her optic chiasm and died 6 weeks later. Conclusions: Orbital and choroidal metastases are relatively common but isolated optic nerve metastases are extremely rare. Progressive infiltration of the nerve is likely to enhance CRVO ischaemia and resultant rubeotic glaucoma. In the diagnosis of CRVO, proptosis or external ophthalmoplegia, the presence of pre-existing malignant disease should raise concerns, as delay in diagnosis may affect outcome, particularly if the metastases are sensitive to pharmacological therapy.     

The value of VEP in the diagnosis and post-operative monitoring of meningioma

Meningiomas are the most frequently reported primary intracranial neoplasms. At first they usually cause unilateral visual loss but eventually result in a bilateral loss of vision. Depending upon the size and location of the tumour, the ocular signs and symptoms of meningiomas may include visual field abnormalities, optic atrophy, papilledema, diplopia and proptosis. This case report highlights the value of visual evoked potentials (VEP) in a patient with unexplained bilateral optic atrophy and a progressive loss of vision over 2 years. As a result of a delayed response in the VEP recorded from the right eye, a compressive lesion of the optic nerve was suspected. That prompted the referring ophthalmologist to request a MRI scan which led to the diagnosis of meningioma. Following the subtotal removal of the suprasellar meningioma, the remaining vision in the right eye improved and the latency of the VEP returned to the normal range. However, the VEP from the blind eye (left) did not show any measurable response either pre- or postoperatively. Experience with this patient suggests that early recognition of optic nerve compression is vital to an optimal outcome and the VEP technique, which is much more cost-effective than MRI, is clinically useful for detecting such compressive lesions.     

Optic disc structure and shock-induced anterior ischemic optic neuropathy

PURPOSE: To describe a patient who developed unilateral shock-induced anterior ischemic optic neuropathy (SIAION) after gastrointestinal hemorrhage followed by presumed idiopathic nonarteritic anterior ischemic optic neuropathy (NAION) in the fellow eye. DESIGN: Retrospective, observational case report and literature review. METHODS: The case history of an 80-year-old man who developed SIAION, followed by NAION in the fellow eye, was reviewed. All previously reported cases of SIAION were reviewed. MAIN OUTCOME MEASURES: Neuro-ophthalmic examination, including visual acuity, funduscopy, and automated perimetry. RESULTS: An 80-year-old man, with a history of gastrointestinal bleeding from a duodenal ulcer, was hospitalized and received four units of packed red blood cells after he was found to be severely anemic (hemoglobin 6.7 g/dl). Three days later he complained of loss of vision of the right eye. Neuro-ophthalmic examination 2 weeks later disclosed a visual acuity of counting fingers at 6 inches in the right eye and 20/40 in the left eye, with a right relative afferent pupillary defect and a superior altitudinal visual field defect. Funduscopy revealed optic disc edema with a temporal parapapillary hemorrhage in the right eye and a small optic disc, with no cup, in the left eye. A diagnosis of SIAION secondary to anemia was made. Six weeks later he developed a new inferior altitudinal visual field defect in the left eye and diffuse optic disc swelling. He had no signs or symptoms of giant cell arteritis or polymyalgia rheumatica, his hemoglobin at this time was 11.9 g/dl, and the Westergren erythrocyte sedimentation rate was 6 mm/hour. CONCLUSIONS: Our patient developed optic disc swelling of the right eye after an episode of gastrointestinal hemorrhage (SIAION). The disc swelling in the left eye occurred 8 weeks later, when his hemoglobin had increased to 11.9 g/dl. The timing of the ischemic optic neuropathies suggests that the acute anemia led to involvement of the first but not the second eye. The configuration of the optic disc may have predisposed not only to the second event (NAION) but also to the first episode (SIAION).