Bilateral Optic Nerve Head Angiomas and Retrobulbar Haemangioblastomas in von Hippel-Lindau Disease

Abstract

A 39-year-old man presented with progressive visual loss in both eyes. His past medical history was remarkable for biopsy-proven bilateral adrenal phaeochromocytomas. Ophthalmoscopy revealed an optic nerve head angioma and macular lipid exudates in the right eye and a regressed optic nerve head angioma in the left eye. A fluorescein angiogram revealed a lobulated pattern of leakage and venous staining from the optic nerve head angioma. Brain and orbital magnetic resonance imaging showed haemangioblastomas of the right intraorbital and left paraclinoid optic nerves and in the left cerebellopontine angle area. These findings were consistent with a clinical diagnosis of von Hippel-Lindau disease.     

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     

A rapidly fatal case of T-cell lymphoma presenting as idiopathic orbital inflammation

A 41-year-old Caucasian woman presented with a painful, red right eye with minimal systemic symptomatology, and was initially diagnosed with right idiopathic orbital inflammation. Ten days later, she developed abdominal and respiratory symptoms; this led to her demise within a further week. Post-mortem examination demonstrated widespread extranodal NK/T- cell lymphoma (nasal type), involving the right posterior orbit, lungs, uterus, left adrenal gland, pericardium and meninges. Thorough physical examination with early orbital biopsy should be considered to exclude underlying treatable pathology in managing patients with presumed idiopathic orbital inflammation.     

Sarcoma pleomorfo indiferenciado de alto grado orbitario: a propósito de un caso

Case reportA 27-year-old female presented to us with a complaint of proptosis of her right eye. Imaging tests showed an extraconal mass in the medial orbital wall, which affected the medial rectus. The tumor was removed and adjuvant radiotherapy was required. The histological examination showed an undifferentiated high-grade pleomorphic sarcoma of the orbit. There has not been recurrence after 6 years of follow-up.DiscussionUndifferentiated high-grade pleomorphic sarcoma is an uncommon cause of malignant orbital mass. It grows fast and can metastasize, so extension studies are required. Surgical excision is the treatment of choice.     

Electrophysiological evaluation of visual loss in Müller cell sheen dystrophy

AIMS—To describe the clinical picture and electrophysiological findings in Müller cell sheen dystrophy, a recently reported retinal dystrophy.
METHOD—A basic ophthalmological evaluation as well as recording of standard electro-oculography and electroretinography were performed in one patient at the onset of visual loss and after 1 year of follow up.
RESULTS—A 61 year old woman presented with visual loss in the right eye. Multiple folds at the level of the internal limiting membrane were seen at the posterior pole in both eyes. Macular oedema was present in the right eye. The visual acuity of the right eye was 6/30 and of the left 6/9. A paracentral scotoma was found in the right eye. Electro-oculographic examination of both eyes gave normal results. Electroretinography (ERG) revealed reduced b-wave and flicker amplitudes in the right eye; these potentials were normal for the left eye. The ON response in the right eye was reduced and delayed; it was normal in the left eye. A further loss of visual function was noted 1 year later in the right eye, but the ophthalmoscopic findings were unchanged. The ERG of the right eye had a negative waveform when dark adapted. Light adapted responses showed an unusual delayed b-wave, broad and delayed ON and OFF responses and a missing flicker response, suggesting a Müller cell dysfunction. Light adapted responses were slightly reduced in the left eye.
CONCLUSIONS—Electrophysiological data indicate Müller cell dysfunction as a background of functional loss in Müller cell sheen dystrophy. This is in agreement with previously reported histological findings in this disorder.

Keywords: internal limiting membrane; electroretinography; retinal degeneration; Müller cells     

Trombosis venosa retiniana en paciente joven con déficit de factor xii de la coagulación

Case reportA 35-year-old woman, with no relevant medical history, was referred for sudden vision loss in the left eye. Ophthalmological examination showed best corrected visual acuity of 1.0 in the right eye and 0.3 in left eye, with normal anterior pole and intraocular pressure. Fundus examination of the left eye revealed a venous thrombosis in the superior temporal branch, with dilated and tortuous retinal veins. The patient was referred to the hematology unit for thrombophilia study, and was diagnosed with a coagulation xii or Hageman factor deficiency.DiscussionThe development of retinal vessel occlusions, in patients under 50 years of age, is frequently associated with thrombophilia or hypercoagulability disorders. Factor xii deficiency is a rare condition, and its presence could contribute to a higher risk of thromboembolic events.     

Spontaneous Subperiosteal Orbital Hematoma as Initial Presentation of Metastatic Lung Adenocarcinoma to the Skull: Case Report

Abstract

Introduction: Subperiosteal orbital hematoma is a rare occurrence, typically developing as a result of orbital trauma. The spontaneous formation of a subperiosteal orbital hematoma (sSOH) may also occur but is less frequent. To date there has been no documented cases of sSOH as the initial presentation of an unknown metastatic neoplasm to the skull. We provide a case of a woman with unknown lung adenocarcinoma that metastasized to the skull which caused the formation of a sSOH resulting in orbital compression syndrome.

Case Report: A 57-year-old female presented with double vision, retro-orbital right eye pain, and vision loss in the right eye. A magnetic resonance imaging revealed a right orbital compressive lesion with an adjacent supraorbital skull lesion and separate left frontal skull lesion. Intra-operative findings along with post-operative immunohistochemistry staining revealed sSOH resulting from a metastatic lung adenocarcinoma to the skull. Further metastatic work up also revealed an occult lung mass and multiple spinal lesions.

Conclusion: Differential diagnosis of etiologies causing the formation of sSOH in an adult without history of trauma should include metastatic neoplasm to the skull and warrants metastatic workup. Treatment options of sSOH have included observation with spontaneous resolution; however, we opted for surgical decompression of the eye and biopsy of the skull mass.     

Bilateral Neuroretinitis in Cat Scratch Disease with Exudative,Obliterative Vasculitis in the Optic Disc

Abstract

A 29-year-old fisherman exhibited optic disc oedema and peripapillary retinal detachment in the right eye, whereas in the left eye, optic atrophy and intraretinal exudates were already observed on first examination. About 6 months earlier, he noticed blurred vision of the left eye but took no medication. Visual acuity was 0.4 OD and 0.01 OS. Perimetry showed a large lower-half field defect with sparing 10° central field in the right eye and a large central scotoma in the left eye. Fluorescein angiography showed existence of arteriole or capillary nonperfusion and hyperpermeability of surrounding capillaries. Since serological examinations showed positive Bartonella immunoglobulin G (IgG) and other causes of neuroretinitis (NR) were excluded, NR in the present case was caused by cat scratch disease (CSD). Optic atrophy appeared 2 weeks after onset. Optical coherence tomography 13 weeks after onset revealed severe loss of retinal nerve fibre layer (RNFL) superior and nasal to the optic disc in both eyes and temporal in the left eye. Visual acuity of the right eye improved to 1.2 by the treatment, whereas visual field defects were persistent. CSD-NR in the present case developed abrupt appearance of optic atrophy with severe RNFL loss in the right eye, which was elicited by exudative, obliterative vasculitis in the superficial layer of the optic disc.     

Conjunctival plasmacytoma

BackgroundPlasmacytomas are plasma cell tumors that may be a primary or secondary tumor focus, the latter of which are associated with multiple myeloma. We present a rare case of a solitary extramedullary plasmacytoma involving the conjunctiva.Case ReportA 33-year-old white man presented with the initial complaint of redness in both eyes, more in his right than left eye. A vascularized conjunctival lesion was noted in his right eye. The patient underwent excisional biopsy, which found a conjunctival plasmacytoma.ConclusionAlthough plasmacytomas of the eye and orbit are rare, it is important to be familiar with these tumors that may be associated with multiple myeloma. Definitive diagnosis is made by biopsy and histopathologic examination of the tissue. Plasmacytomas may be treated with external beam radiation, local excision, or radiotherapy after surgical excision. Long-term follow-up, including periodic systemic evaluation, is required to establish that orbital involvement is not an early manifestation of multiple myeloma.     

Orbital and Intraocular Myofibroblastoma

Abstract

A 66-year-old woman presented with a blind, painful, hypertensive, and proptotic left eye. Computed tomographic imaging revealed a well-circumscribed mass involving the left orbit and globe. Metastatic work-up failed to reveal extraorbital lesions and the tumor was removed in toto via an evisceration approach orbitotomy. Histopathology and immunohistochemistry were most consistent with mammary-type myofibroblastoma with fascicles of bland, uniform spindle cells that stained positive for desmin and CD34. We are not aware of previous reports of orbital or ocular myofibroblastoma. This neoplasm has not been shown to recur, undergo malignant transformation, or metastasize. Familiarity with its clinical, histopathologic, and immunohistochemical features may improve diagnostic accuracy and treatment decisions for patients presenting with similar findings.     

El ángulo papila-fóvea y la torsión ocular compensatoria,clave en su interpretación

PurposeDescribe a time-sparing technique to measure disc-foveal angle (DFA), determine normal values and its role when analyzing paired fundus photographs.MethodsDFA was analysed using the software program Keynote v.6.2.2 on 440 fundus photographs (3 D OCT 2000, Topcon Corporation, Tokio, Japan) of 20 individuals. The 11 different head positions were determined with the cervical range of motion device (CROM, Performance Attainment Associates). A reproducibility and correlation study between two fundus cameras (OCT 3D-2000 and TRC-50EX, Topcon Corporation, Tokio, Japan) was performed.ResultsMean DFA of the right and left eye was 5.5±3.4° and 8.6±2.9°, with a difference of 3.1° (P=0.001 Wilcoxon signed-rank test) in the upright head position. Mean absolute difference in DFA between eyes was 3.5±2.6°; an increase was seen with increasing head tilt (P=0.000 Wilcoxon signed-rank test). Mean sum of DFA in both eyes was 14.1±5.4°. On head-tilt of 20° and 40° to the right, mean ocular counterrolling (OCR) was 7.1° and 12.2° in the right eye and 7.7° and 12.1° in the left eye. On head-tilt of 20° and 40° to the left, OCR was 4.4° and 8° in the right eye and 4.2° and 8.7° in the left eye (P=0.000 Wilcoxon signed-rank test). The two cameras showed strong correlation and high reproducibility.ConclusionsOur DFA measurement technique is time-sparing and reproducible. Left eye shows higher DFA than right eye. OCR occurs only in the roll plane. This information is of value when analyzing paired fundus photographs.     

Painful orbital inflammatory lesions and mastocytosis

A 15-year-old female patient developed painful nodules in an anophthalmic socket that were exacerbated by treatment with steroids and narcotics. Although orbit biopsy showed chronic inflammation, the diagnosis of mastocytosis was confirmed with skin biopsy and urinary levels of histamine. The patient responded to excision of nodules and treatment for mastocytosis. Painful orbital lesions associated with mastocytosis have not been reported previously.     

Macular oedema due to letrozole: A first case report

A 72‐year‐old woman presented with unexplained, progressive, painless visual loss in the right eye during the past six months. At presentation visual acuity (VA) was 3/60 in the right eye and 6/6 in the left eye. Anterior segment examination and intraocular pressures were normal. Dilated fundoscopy revealed significant macular oedema in the right eye and a normal fundus appearance in the left eye. Her medical history was noteworthy for breast ductal carcinoma in situ, for which she had undergone right mastectomy three years earlier. She had not received chemotherapy or radiotherapy but she had been under treatment with letrozole 2.5 mg/day over the past three years. She did not receive any other medication. Optical coherence tomography showed intraretinal fluid and a significant increase in retinal thickness in the foveal and parafoveal areas, while fluorescein angiography detected foveal hyperfluorescence and leakage of the dye in the late phase. Multifocal electroretinogram showed a decreased response in both eyes. In suspicion of letrozole‐related retinopathy, the patient was advised to stop the medication. The patient agreed to receive an intravitreal injection of 0.05 ml/0.5 mg ranibizumab. One month later, VA in the right eye was 6/9 and macular oedema had apparently improved. This is the first reported case of letrozole‐associated macular oedema treated with intravitreal ranibizumab.     

Bevacizumab intravítreo en neovascularización coroidea asociada a enfermedad de Best

Case reportA 27-year old woman presented with loss of vision in the right eye (20/200). Ophthalmoscopic examination showed intrarretinal hemorrhage in the macular region with neurosensory detachment in the right eye, and viteliform deposit on the left eye. Fluorescein angiography and the electrooculogram confirmed the diagnosis of choroidal neovascularization associated with Best's disease. Four weeks after a single bevacizumab intravitreal injection, visual acuity was restored (20/25) and remained stable after a 12 month follow-up.DiscussionIntravitreal bevacizumab appears to be an effective treatment for choroidal neovascularization associated to Best's disease.     

Maculopatía por drogas de inhalación

Case reportA case of maculopathy due to «poppers» is described.DiscussionPoppers is a drug composed of various forms of alkyl nitrite. A 39 year-old man, who had been using poppers for years, was seen in the clinic with phosphenes, reduced visual acuity and central scotoma. The SD-OCT in the right eye showed disruption at the level of the IS/OS junction line. The SD-OCT scan in the left eye showed an outer rectangular retinal hole and an outer retinal cyst.     

Bilateral Simultaneous Nonarteritic Anterior Ischaemic Optic Neuropathy: Case Report

Abstract

Bilateral simultaneous nonarteritic anterior ischaemic optic neuropathy (NAION) is extremely rare. A 57-year-old woman presented with bilateral optic disc oedema and peripapillary splinter haemorrhages. Initial visual acuities were hand movements in the right eye and light perception in the left eye. The patient had a mildly elevated diastolic blood pressure and glucose intolerance. Erythrocyte sedimentation rate and C-reactive protein levels were within normal limits. Temporal artery biopsy was negative for temporal arteritis. Marked visual improvement occurred in both eyes (0.8 in the right eye, 0.6 in the left eye) after systemic steroid therapy in the 16th month of follow-up.     

A lot of clot

A 37-year-old man presented with fever and a red, painful right eye. He had proptosis, conjunctival chemosis, and ophthalmoplegia OD. The patient had extremely poor dentition and had self decompressed a dental abscess prior to admission. Magnetic resonance imaging of the brain and orbital revealed extraocular muscle engorgement and a dilated superior ophthalmic vein OD. Orbital echography revealed a lack of flow in the right superior ophthalmic vein. An extensive hematologic evaluation for infection and inflammation was negative. A chest radiograph showed a lung abscess for which he received intravenous antibiotics. Over time, the periorbital erythema, ophthalmoplegia, proptosis, and pain resolved. Repeat MRI showed resolution of the orbital findings and repeat chest x-ray showed resolution of the left upper lobe abscess.     

Orbital Metastases

BackgroundOrbital metastasis, although uncommon, is a condition optometrists should consider in a patient presenting with proptosis, ptosis, diplopia, or a lid mass with a history of cancer. However, in as many as 19% of cases, patients have no prior or concurrent history of systemic cancer when presenting with ophthalmic symptoms. If suspecting an orbital metastasis, neuroimaging is important, as well as a referral to the patient's primary care provider, oncologist, and ophthalmologist.Case ReportsThree patients with orbital metastasis are discussed. The first was a 55-year-old white man who initially presented with a left ptosis of unclear etiology. Magnetic resonance imaging of his orbits and an orbital biopsy found metastatic esophageal adenocarcinoma. Radiotherapy and chemotherapy were initiated, but the patient died shortly afterward. The second patient was a 49-year-old black man who also presented with a ptosis of the right upper eyelid. An area of the retina appeared elevated; ophthalmic B-scan and computed tomography of the orbits confirmed the presence of a mass, determined to be metastatic lung carcinoma to the right orbit. A course of radiotherapy was initiated, but the patient died 3 days after completing therapy. The last case was a 77-year-old white man with a history of metastasis to the left orbit from non-Hodgkin's lymphoma. On examination, he had proptosis of the left eye, initially thought to be caused by a recurrence of the metastasis. However, a computed tomography scan showed a new meningioma in the same orbit, and treatment was started. The proptosis improved, and the patient continues to be followed up regularly.ConclusionsAny patient with proptosis and/or ptosis with a history of cancer should be evaluated for orbital metastasis. Optometrists should keep in mind that an orbital metastasis may represent the initial manifestation of undiagnosed systemic cancer. Prognosis can be poor, and thus treatment is sometimes palliative in nature, intending to slow the progression of the disease instead of providing a cure.     

A rare case of peripartum endogenous bacterial endophthalmitis

We describe the case of a healthy, pregnant female who developed endogenous endophthalmitis at the time of delivery, and discuss the possible mechanism of infection and the management of this case. A 26-year-old Asian woman presented with a 3-week history of visual deterioration and pain in the right eye. There was no history of ocular trauma or surgery. The ocular symptoms developed one day after vaginal delivery of a healthy baby. The pregnancy had been uncomplicated until premature rupture of membranes one week prior to delivery. Right visual acuity was light perception. There was marked right anterior chamber activity with a hypopyon and fibrin. A B-scan ultrasound showed dense vitritis. Examination of the left eye was normal. Blood tests and a chest X-ray were normal. A vitreous tap was performed and bacterial culture grew Sphingomonas paucimobilis. Intravitreal antibiotics were injected (amikacin 0.4 mg/0.1 ml and vancomycin 2.0 mg/0.1 ml) and the patient was treated with oral moxifloxacin and corticosteroids. Right visual acuity improved to 6/9. This case highlights the need for clinicians to have a high level of awareness of endogenous bacterial endophthalmitis (a rare, potentially sight-threatening condition) in any patient with a painful eye or visual deterioration in the peripartum period, particularly if associated with complications such as premature rupture of membranes or perineal laceration.     

Steroid-responsive optic neuropathy in a patient with Tolosa-Hunt syndrome: Electrophysiologic findings

We examined visual evoked potentials and pattern electroretinograms in a patient with Tolosa-Hunt syndrome associated with optic nerve involvement. The 82-year-old woman developed unilateral painful ophthalmoplegia and visual loss in the right eye. Magnetic resonance imaging showed an abnormal soft-tissue area in the right cavernous sinus and the right orbital apex. Symptoms responded rapidly to treatment with corticosteroid. Visual evoked potentials to flash and pattern stimuli were both remarkably reduced and delayed in the right eye in the acute stage; however they improved to almost normal after steroid therapy. The pattern electroretinogram recorded in the acute stage was normal bilaterally. These results indicate that optic nerve involvement in Tolosa-Hunt syndrome can be mild and reversible.