Panophthalmitis masquerading as retinoblastoma—an eye opener

We report a clinicopathological correlation of a 12-year-old female child who manifested as retinoblastoma clinically and radiologically but turned out to have acute panopthalmitis on histopathological examination. The authors feel that there is significant clinical implication in sharing the knowledge of this clinicopathological correlation. A 12-year-old female child born out of a consanguineous marriage reported to us with bilateral blindness. She was evaluated clinically. Ultrasound B mode and computerized tomogram (CT) examination were done. Based on all the inputs, right eye enucleation was performed, and the same was subjected to a histopathology examination. The patient had proptosis of the right eye with (LE) calcification of the lens. CT scan revealed irregular thickening of the right globe with heterogeneous uveoscleral enhancement. The vitreous showed irregular hyperdense thickening with multiple calcifications. There was mild thickening of the retrobulbar optic nerve and tendinous insertions of extraocular muscles. The right eye was enucleated, and its histopathology examination revealed panophthalmitis with intraocular ossification. The right globe revealed no evidence of malignancy. Painful blind eye is a major therapeutic dilemma for an ophthalmologist. Radiological findings of irregular thickening of sclera, vitreous with calcifications, and optic nerve thickening are highly suggestive of retinoblastoma. In our patient, we wanted to share the differential diagnosis of acute panopthalmitis which masqueraded as retinoblastoma.     

Review of the publications of the Nigeria national blindness survey: methodology, prevalence, causes of blindness and visual impairment and outcome of cataract surgery

This is a review of the major publications from the Nigeria national blindness survey in order to highlight major findings and challenges of eye care in Nigeria. The review summarizes methodology and key findings. Survey publications on methodology, prevalence and causes of visual impairment and outcome of cataract surgery were retrieved, reviewed and relevant data extracted, reported and discussed. The study was the largest and more detailed eye survey in Nigeria (15,375 people 40 years and older recruited). Participants had detailed eye examination including visual acuity, autorefractokeratometry, A- scan biometry, visual field and basic eye examination. Cause(s) of visual impairment in each eye using WHO algorithm was determined among participants with vision < 6/12. Some of the participants also had qualitative questions on barriers to uptake of services, quality of life and visual function. Major highlights of the results as contained in the publications include a high prevalence of blindness with 4.2% (95% CI: 3.8-4.6%;),of the study population having blindness (using presenting vision (PVA)) even with best correction the prevalence was 3.4% (95% CI: 3.0-3.8%. The prevalence of SVI using PVA was 1.5% (95% CI: 1.3-1.7%).and with best correction 0.8% (95% CI: 0.7-1.0%). Blindness varied by age groups, sex, literacy level and geopolitical zone. Furthermore, 84% of blindness was due to avoidable causes with cataract responsible for 43% of blindness, glaucoma 16.7%, uncorrected aphakia 8.4% and corneal opacity 7.9%. Of the total 538 eyes that had cataract surgery procedures, 42.7% had couching and the remaining had cataract surgery, but only 41.4% of cataract operated eyes had IOL surgery. Outcome of cataract surgery was good at presentation for only 30.8% of eyes (84 eyes) which improved to 56.8% with correction. The possible remedy for the high burden of needless blindness and harmful eye health practices in Nigeria are discussed.     

Familial spinocerebellar degeneration with corneal dystrophy

We report on two sisters born to normal but consanguineous parents, with the unusual combination of spinocerebellar degeneration and corneal dystrophy. Their manifestations include mental subnormality, bilateral corneal opacification starting in the second year of life and leading to severe visual impairment, and slowly progressive cerebellar abnormalities with variable dorsal column and upper motor neuron involvement. A third sister had only minor spinocerebellar signs but no eye findings, and three other sibs were completely normal. Both affected sisters underwent penetrating keratoplasty and their vision improved. Histologic examination showed findings of corneal dystrophy including corneal edema, thickening of Descemet membrane, and degenerative pannus. High-resolution light and electron microscopy of muscle and sural nerve performed on both patients was abnormal. It is suggested that, in this family, the corneal dystrophy and spinocerebellar degeneration are pleiotropic manifestations of an autosomal-recessive disorder.     

Bilateral choroidal metastases as the initial presentation of breast carcinoma

A 64 year old female presented with a right eye visual impairment. On examination, the visual acuity was decreased on the right side. Slit lamp examination showed bilateral non-pigmented choroidal lesions. Physical examination was unremarkable; bilateral mammogram, however, showed a mass in the left breast, the biopsy from which confirmed the lesion as infiltrative carcinoma. Other tests were normal apart from the isotope bone scan which showed evidence of metastatic disease. She received a short course of radiotherapy to both eyes as well as a 6-month course of chemotherapy. At 6 months follow-up, the choroidal lesions were no longer present and the visual acuity had stabilized. Choroidal metastasis as the initial presentation of breast carcinoma is unusual. Any patient with an ocular tumour should undergo a systemic check-up to rule out an underlying malignancy.     

Blindness from bilateral bullous retinal detachment: tragedy of a Nigerian family

Objective

To present a scourge of blindness possibly due to an inherited condition causing retinal detachment in 3 siblings

Methods

In February 2004, three siblings from a monogamous family from Ipetu-Ijesha in Osun state, Nigerian presented to the author consecutively with history of visual impairment/ blindness. A detailed history and comprehensive ocular examination was conducted on each of them. Information obtained included patients demographic, family history of blindness, eye diseases and other medical conditionsVisual acuity assessment, slit lamp examination, direct and indirect ophthalmoscopy, intra-ocular pressure measurement, visual field test and systemic evaluation were conducted on each of the patients.

Results

Two males and a female patient from the same parents who were systemically healthy are reported. Their ages were 67years, 58years and 52years respectively. Each presented with bilaterally poor vision ranging from 6/36 to no light perception, intra-ocular pressure ranging between 6 and 18mmHg and bilateral grayish white bullous retinal detachment.

Conclusion

The mode of inheritance in these patients was most probably autosomal dominant or X-linked recessive. Ignorance and poverty causing failure to seek prompt specialize eye care were responsible for blindness in these siblings.     

No Colavita effect: equal auditory and visual processing in people with one eye

Previous research has shown that people with one eye have enhanced spatial vision implying intra-modal compensation for their loss of binocularity. The current experiments investigate whether monocular blindness from unilateral eye enucleation may lead to cross-modal sensory compensation for the loss of one eye. We measured speeded detection and discrimination of audiovisual targets presented as a stream of paired objects and familiar sounds in a group of individuals with monocular enucleation compared to controls viewing binocularly or monocularly. In Experiment 1, participants detected the presence of auditory, visual or audiovisual targets. All participant groups were equally able to detect the targets. In Experiment 2, participants discriminated between the visual, auditory or bimodal (audiovisual) targets. Both control groups showed the Colavita effect, that is, preferential processing of visual over auditory information for the bimodal stimuli. The monocular enucleation group, however, showed no Colavita effect, and further, they demonstrated equal processing of visual and auditory stimuli. This finding suggests a lack of visual dominance and equivalent auditory and visual processing in people with one eye. This may be an adaptive form of sensory compensation for the loss of one eye and could result from recruitment of deafferented visual cortical areas by inputs from other senses.     

Cataract surgery in an African lion (<Emphasis Type="Italic">Panthera leo</Emphasis>) with anterior lens luxation

Mature cataract had caused change in appearance of the eye and complete blindness in an aged African lion. Early surgical removal of the lens is the only effective treatment for mature cataract, and if surgery is not performed soon after maturation of the cataract, total blindness may remain after surgery. The present report describes a mature cataract of unknown cause with anterior lens luxation in a 20-year-old African lion treated by intracapsular extraction of the lens. On clinical examination, immature cataract of the right eye and mature cataract with anterior lens luxation of left eye were diagnosed. The entire lens, including anterior and posterior capsules, was removed by routine surgical techniques described for such a procedure. The lion suffered self-trauma of the eye 48 h after surgery, and three sutures were disrupted. Antibiotic drops and Cefazolin were administrated with extreme difficulty for 1 week. The lion was saved, but keratitis developed in the left eye.     

A 47-year-old man with sudden onset of blindness, pleocytosis, and temporary hearing loss. Vogt-Koyanagi-Harada syndrome (Uveomeningoencephalitic syndrome)

The clinicopathologic findings of this patient were typical for Vogt-Koyanagi-Harada syndrome. Clinically, the patient had sudden onset of blindness, pleocytosis, and temporary hearing loss, with no history of eye trauma or surgery. Histopathologically, the choroid and iris showed chronic inflammation, loss of choroidal melanocytes, and dense chorioretinal adhesions. Presence of HLA-DRB1*0405 was consistent with the chronic phase of this syndrome.     

First case of Arthrographis kalrae ethmoid sinusitis and ophthalmitis in the People's Republic of China

We present here the first case in the People's Republic of China of human disease caused by the fungus Arthrographis kalrae. The male patient had fungal panophthalmitis and invasive sinusitis involving the maxillary and ethmoid sinuses. He was an apparently healthy man before receiving trauma to his left eye. He complained of pain and loss of visual acuity in the injured eye, which displayed redness and edema and eventually discharged pus. His symptoms became more severe after he was treated with steroids and several antibacterial agents. A computed tomography scan of the left eye revealed that the maxillary and ethmoid sinuses were involved. A smear of purulent material from the left eye orbit revealed fungal elements, and cultures of the material grew a fungus. The isolate was identified as A. kalrae based on gross and microscopic morphologies, biochemical assays, and DNA sequence analysis. The patient received amphotericin B intravenously, itraconazole orally, and atomized allitridum by nebulizing allitridum therapy. The patient's wound healed following surgical intervention, but the patient lost the use of his left eye. This case should remind ophthalmologists and other clinicians to consider the possibility of infections being fungal when antibacterial agents have no effect and the patient's condition worsens.     

Discriminating emotional faces without primary visual cortices involves the right amygdala

Destruction of the brain's primary visual areas leads to blindness of cortical origin. Here we report on a subject who, after bilateral destruction of his visual cortices and ensuing cortical blindness, could nevertheless correctly guess the type of emotional facial expression being displayed, but could not guess other types of emotional or non-emotional stimuli. Functional magnetic resonance imaging showed activation of the right amygdala during the unconscious processing of emotionally expressive faces.     

The primary care physician's role in preventing vision loss and blindness in patients with diabetes

While not trained to specifically diagnose or treat ophthalmic conditions, primary care physicians can play a significant role in the visual health of their patients, specifically those with diabetes. Diabetic retinopathy is a leading cause of vision loss and blindness in the United States and because of its asymptomatic nature, patients often do not seek eye care until they notice changes in their vision. Patients with diabetes look to their physicians for guidance in managing all aspects of the disease, and research has shown people are more likely to have their eyes examined if their physician suggests it. Physicians should discuss visual health with patients and make referrals for annual comprehensive dilated eye examinations. Vision loss and blindness from diabetes can often be prevented through early detection and timely treatment; however, many patients seek eye care at a stage too late for treatment to be effective.     

A case of allergic fungal sinusitis caused by Bipolaris spicifera]

We describe a case of allergic fungal sinusitis (AFS) caused by Bipolaris spicifera, the first case reported in Japan. A 70-year-old man was admitted to our hospital because of diplopia following bilateral nasal obstruction and discharge. Radiological studies using computed tomographic scan showed a large soft tissue mass occupying the right frontal, bilateral ethmoid and sphenoid sinuses. He underwent drainage surgery and histopathological examination of the contents of the paranasal sinuses revealed scattered fungal hyphae within "allergic mucin". By cytological examination, these hyphae showed septation at irregular intervals, and were swollen to various sizes. Microbiological studies identified the fungus as Bipolaris spicifera. The presence of allergic mucin and scattered fungal hyphae were very important findings in making a diagnosis of AFS histopathologically, so squash cytology of the contents of the paranasal sinuses was quite useful to observe fungal elements and identify the strain in this case.     

The clinical spectrum of amiodarone-associated optic neuropathy

PURPOSE: To describe the clinical spectrum of amiodarone-associated optic neuropathy. METHODS: Observational cases series and review. RESULTS: Of 55 cases, the median interval for onset of optic neuropathy was four months after initiating amiodarone; 88% occurred within 12 months. Seven (13%) patients were asymptomatic. Twenty-two (40%) patients presented with sudden visual loss, while 26 (47%) had insidious loss of vision. Visual acuity ranged from 20/15 to light perception; 10 (18%) patients had legal blindness with visual acuity of 20/200 or worse. Visual field loss was present in 91% of cases. Color vision loss was present in eight (40%) of 20 cases. Optic disc edema was present in 85% of cases, while eight (15%) patients had retrobulbar optic neuropathy, without evidence of disc edema. Optic disc edema resolved over a median time of three months. Five patients had raised intracranial pressure on lumbar puncture. CONCLUSION: We were able to classify amiodarone-associated optic neuropathy into five clinical categories with respect to temporal characteristics and optic nerve appearance: insidious-onset (43%), acute-onset (28%), retrobulbar (13%), increased intracranial pressure (8%), and delayed-progressive onset (8%). Most cases of optic neuropathy commenced within 12 months of initiating amiodarone, with the median onset being four months. Over 10% of patients will have no visual symptoms at the onset. Ophthalmologic examinations within the first 12 months--and particularly within four months of initiating amiodarone--should improve early detection of amiodarone-associated optic neuropathy.     

Blindness and scalp haematoma in a child following a snakebite

BackgroundSnake envenomation is a major public health problem of the Savannah regions of West Africa. Ocular manifestations of snakebites are rare with few reports documenting blindness as a complication.ObjectiveTo highlight an unusual manifestation of snake bites and its attendant problemsMethodsA report of scalp haematoma and blindness in a 10 year old child presenting 2 weeks after a snake bite (presumably carpet viper) is a rare manifestation. Local swelling, epistaxis, bilateral proptosis, exposure keratopathy and use of traditional eye medications were associated findings. Anti-venom though administered late saved the child''s life but blindness could not be reversed.ResultsOcular ultrasonography revealed layered retrobulbar collection in the left eye, presumably due to hemorrhage. The skull x-ray showed a soft tissue swelling and aspirate from scalp swelling was bloody. Cranial Computed Tomography (CT) scan done late detected no abnormalities.ConclusionSnakebite is associated with lifelong morbidity. Ocular manifestations must be treated as emergency. This case highlights the effect of ignorance and poverty in a setting of a common medical emergency leading to blindness and reduced quality of life.     

The effect of eye enucleation on the number of fibres in the supraoptic decussation of posthatch chicks (Gallus gallus)

In this study of the young chick we examine the effects of unilateral or bilateral eye enucleation on the number of axons in the supraoptic decussation, a major interhemispheric tract subserving visual function. On posthatch day 25, chicks underwent removal of either one or both eyes. After a survival period of 32 days, electron microscopic examination of the supraoptic decussation revealed a substantial loss of fibres. In the bilaterally enucleated group, a significant decrease in the number of axons occurred in both the dorsal and ventral aspects of the supraoptic decussation. The effect of unilateral enucleation was not as severe. The results indicate that enucleation of the precocial and functioning visual system of the chick leads to a transneuronal loss of axons. The degree of loss may reflect the amount of fibres in the crossed visual projection within the supraoptic decussation.     

A heterozygous mutation in RPGR associated with X‐linked retinitis pigmentosa in a patient with Turner syndrome mosaicism (45,X/46,XX)

Turner syndrome with retinitis pigmentosa (RP) is rare, with only three cases reported based on clinical examination alone. We summarized the 4‐year follow‐up and molecular findings in a 28‐year‐old patient with Turner syndrome and the typical features of short stature and neck webbing, who also had X‐linked RP. Her main complaints were night blindness and progressive loss of vision since the age of 9 years. Ophthalmologic examination, optical coherent tomographic imaging, and visual electrophysiology tests showed classic manifestations of RP. The karyotype of peripheral blood showed mosaicism (45,X [72%]/46,XX[28%]). A novel heterozygous frameshift mutation (c.2403_2406delAGAG, p.T801fsX812) in the RP GTPase regulator (RPGR) gene was detected using next generation sequencing and validated by Sanger sequencing. We believe that this is the first report of X‐linked RP in a patient with Turner syndrome associated with mosaicism, and an RPGR heterozygous mutation. We hypothesize that X‐linked RP in this woman is not related to Turner syndrome, but may be a manifestation of the lack of a normal paternal X chromosome with intact but mutated RPGR.     

Åland eye disease: no albino misrouting

Electrophysiological studies showed that a patient with Åland eye disease had no misrouting of the optic pathways which is always found in all forms of albinism as a consequence of the retinogeniculate anomaly. Also the spontaneous and optokinetic nystagmus did not resemble that of the large majority of human albinos. The marked asymmetry found in this patient seems to be typical for humans with a defective development of foveal binocular vision. These findings are in agreement with clinical, nystagmographic and EM findings that Åland eye disease is distinct from the Nettleship-Falls type of X–linked ocular albinism. Furthermore, Åland eye disease is different from X-chromosomal congenital stationary night blindness with myopia by the fact that the scotopic functions are only moderately affected and there is no restriction of the peripheral photopic visual fields. In addition, there is latent nystagmus of extraocular type that appears also in female carriers. There is no ophthalmoplegia, there is a progression of the myopia and the dyschromatopsia is of secondary type.     

猫视乳头三维模型重建及有限元分析

BACKGROUND: Glaucoma is a kind of eye disease that can cause irreversible blindness which is characterized by visual field loss. Clinical research shows that the optic nerve head has changed before the visual field loss. The morphological changes of the optic nerve head have become the key to determine the early diagnosis of glaucoma and disease development. So it has important significance for us to study the morphological changes of the tissues of optic nerve head under the high intraocular pressure.     

Respiratory hyperinfection caused by Strongyloides stercoralis in a patient with pemphigus vulgaris and minireview on diagnosis and treatment of strongyloidiasis

We explain a case of strongyloidiasis in a woman with pemphigus vulgaris from the north of Iran. The patient, aged 59 years old, had been receiving immunosuppressive therapy (oral prednisone) for the last 1 year. She presented with a recent history of diarrhea, epigastric pain, vomiting, fever, loss of consciousness, and respiratory failure. Biochemical and hematological findings were abnormal, and eosinophilia was remarkable. Bronchoscopic alveolar lavage and stool examination revealed larval forms of Strongyloides stercoralis as well as severe acute inflammation. A chest X-ray revealed alveolar opacity in the bilateral mid- and lower zones and bilateral blunting of the costopherenic angle. The high-resolution computed tomography findings showed diffuse ground glass and alveolar opacity, bilateral pleural effusion, and multiple cavitary lesions in mid- and lower zones of the lung. The patient responded well to the treatment with ivermectin plus albendazole.     

Response properties of striate cortex neurons in cats raised with divergent or convergent strabismus

Recordings were made from striate cortex in five groups of cats that had been raised with strabismus produced by sectioning the extraocular muscles. These groups included animals reared with exotropia, unilateral or bilateral esotropia, and esotropia combined with lid suture of the unoperated eye. In addition, a group of esotropes was studied in which the unoperated eye was removed a few hours prior to recording. For comparison, five normal adult cats were also studied. In each of the above groups, cells were sampled in the representations of the central and peripheral visual fields in area 17 ipsilateral and contralateral to the deviated eye. We mapped the receptive field of each responsive cell, determined its ocularity, and tested it for selectivity. Confirming previous work, we found a marked loss of cortical binocularity in cats raised with strabismus. On average only 7% of the neurons that we recorded could be driven by both eyes. This percentage was relatively constant at all cortical locations that were studied and was not influenced by whether cats had been reared with exotropia, unilateral esotropia, or bilateral esotropia. The percentage of selective cells driven by the deviated eye in exotropes or esotropes did not appear to be different from normal at most cortical locations (but see 5, below). In addition, we did not observe any bias in the axial preference of selective cells in strabismic cats when compared with normal adult cats. In both exotropes and esotropes the deviated eye drove fewer cells when compared with the proportion that are driven by one eye in normal cats. In exotropes this deficit did not vary at different cortical representations of the visual field. In esotropes, however, this deficit was graded, being least in the representation of the peripheral visual field in area 17 contralateral to the deviated eye, intermediate in the representations of the central visual field in the contralateral and ipsilateral hemispheres, and greatest in the representation of the peripheral visual field in ipsilateral area 17. Furthermore, only when recording from the peripheral field representation in the ipsilateral hemisphere did we encounter significant numbers of cells driven by the deviated eye that lacked normal selectivity. Since it is possible that deprivation of the converged eye during development might account for the deficits noted above, we attempted to evaluate this factor using several independent lines of evidence. First, we could find no correlation between the angle of esotropia and the ability of the deviated eye to drive ipsilateral cortical cells representing the peripheral visual field.(ABSTRACT TRUNCATED AT 400 WORDS)