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Ocular features and management in the mucopolysaccaridosis
No full textPurpose
To evaluate the ocular findings and the visual outcome in a group of patients with mucopolysaccaridoses(MPS) followed and treated with new therapeutic options.
Methods
18 pazients with MPS (9 males and 9 females) were examinated: 3 cases with MPS type I, 4 with MPS type II,7 with MPS type III, 1 with MPS type IV and 3 with MPS type VI. The patients underwent an ophthalmological assessment with : visual acuity (with Teller Acuity Cards, Lea Symbols o letter chart, depending on patient age or degree of cohoperation), slit-lamp examination, fundus ophthalmoscopy, intraocular pressure, refractometry, electroretinography and eye echography.
Results
The mean follow-up was 48 months (ranged from 3 to 190 months).The mean age at first ophtalmologic evaluation was 6,9 years ( ranged from 1 to 20 years). 8 case presented progressive corneal clouding, 5 case retinal pigmentary degeneration, and 1 papilledema. Visual acuity worsened severely in 3 cases with MPS type I: 1 case from 10/10 to 5/10, 1 case from 5/10 to 1/10; in another case visual acuity decreased to 1/20 and underwent to corneal transplantation with good visual outcome (RE 5/10 and LE 7/10). The other cases showed slightly reduction of visual function.
3 patients had ocular hypertension and were treated with hypotensive eye drops.
8 patients underwent enzyme replacement therapy.
Conclusions
The mucopolysaccaridoses(MPS) are rare systemic disorders caused by accumulation of glycosaminoglycans. Ophthalmological manifestations are frequent in MPS, particularly in MPS I, II and VI, characterized by corneal clouding, retinal distrophy and blindness.Regular ophthalmic monitoring to determine disease progression and management of complications are necessary as a part of multidisciplinary approach
Aspetti oftalmologici funzionali nel cerebral visual impairment: studio longitudinale di 50 casi
No full textfollow-up a lungo termine ed outcome visivo in due fratelli affetti da sindrome di Alstrom con supplementazione di DHA
No full text
VISUAL OUTCOME IN BILATERAL CONGENITAL CATARACT ASSOCIATED WITH OCULAR AND SYSTEMIC DISEASES
No full textTreatment and visual outcome in orbital and palpebral hemangiomas of infancy
No full textPurpose
To study the relationship between the location of orbital and palpebral(eyelid)hemangiomas and ocular problems in children and the interventions needed to prevent or reduce the severity of visual impairment.
Methods
This study included 12 cases (10 female and 1 male;age range:1 day to 5 months old at first evaluation;mean age 2,5 months)of eyelid and/or orbit hemangiomas gathered over a 6 year period (2005-2011).Ultrasonography and magnetic resonance imaging(MRI)identified these lesions to be:palpebral in 2 cases, palpebral and orbital involvement in 9 cases.Pediatric and ophthalmological evaluation(orthoptic examination,visual acuity determination with Teller Acuity Cards,refractometry and fundus opthalmoscopy)were performed in all cases.
Results
The mean follow-up was 36 months (range 11-63 months)
Treatments for infants and children with hemangiomas obstructing the visual axis included oral steroid administration(9 cases);in 1 case a surgical treatment was associated.4 cases developed amblyopia and 1 case strabismus.Treatment of early amblyopia through selective patching part-time patch occlusion of the uninvolved eye and early prescription of astigmatism correction was performed.10 children achieved normal vision at last evaluation.
Conclusions
In our study 10 patients treated for visual impairment and available for long-term follow-up showed an excellent functional outcome with no residual amblyopia.In most cases,early intervention to address hemangioma-related visual disturbances can lead to a good functional outcome. Intervention typically involves medical debulking of the hemangioma(using oral steroids)combined with the treatment of early amblyopia through selective patching and/or optical correction with refractive lenses
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