1/63. Keratomalacia. xerophthalmia and keratomalacia are public health problems of great magnitude which are usually associated with multiple vitamin and protein deficiencies. The authors report the case of a 27-year-old commune member who subjected herself to a bizarre protein and vitamin deficient diet for many months. This ultimately produced nyctalopia, xerophthalmia and keratomalacia with bilateral corneal perforation. Despite therapy, she remained comatose and expired shortly after admission. Ocular pathological changes included bilateral corneal melting with prolapse of intraocular contents, conjunctival epidermidalization, goblet cell atrophy and thinning of the outer nuclear layer of the retina. It is noted that ocular findings in pure avitaminosis A produced experimentalyy include epithelial atrophy followed by keratinization. ( info) |
2/63. GVHD dry eyes treated with autologous serum tears. Two cases of GVHD with severe dry eyes are reported where conventional therapy failed to control ocular signs and symptoms. Autologous serum tears, however, resulted in a beneficial clinical effect with marked attenuation of the symptoms. This therapy proved to be safe during 10 months of treatment. bone marrow transplantation (2000). ( info) |
3/63. Transient hemifacial sensory loss with xerophthalmia following temporal lobectomy. OBJECTIVE AND IMPORTANCE: The occurrence of a unilateral sensory loss in the second trigeminal distribution and the inability to tear following an ipsilateral temporal lobectomy has not been noted despite a number of reports of cranial nerve compromise under similar situations. CLINICAL PRESENTATION: A 48-year-old woman experienced complex partial seizures over three years attributable to the presence of cavernous malformations of the right temporal lobe. INTERVENTION: An anterior temporal extrahippocampal resection was performed. The surgery was marked by the need for electrocoagulation of the dural base of the temporal lobe where numerous bleeding points were encountered. Postoperatively, the patient experienced an ipsilateral maxillary division sensory loss, absence of tearing, and diminished nasal congestion for an eight-month period until resolution. CONCLUSION: Injury of the fibers of the maxillary division of the trigeminal nerve and the adjacent greater superficial petrosal nerve appears to be the cause. No prior account of such an occurrence has been published. ( info) |
4/63. Late onset diffuse lamellar keratitis associated with an epithelial defect in six eyes. PURPOSE: To report six cases of late onset diffuse lamellar keratitis associated with epithelial defects 2 to 12 months following uncomplicated laser in situ keratomileusis (LASIK). methods: Retrospective case series. RESULTS: The interface inflammation and epithelial defects were treated aggressively with topical corticosteroids and topical antibiotics with complete resolution over 1 to 2 weeks. There were no complications or loss of best spectacle-corrected visual acuity. These cases illustrate new understanding in the etiology of diffuse lamellar keratitis following lamellar surgery. CONCLUSION: Late onset diffuse lamellar keratitis in association with epithelial defects may occur following LASIK. Treatment with topical antibiotics and topical corticosteroids may result in uncomplicated, complete resolution. ( info) |
5/63. Corneal melt as the initial presentation of primary sjogren's syndrome. Corneal melting is a rare complication of S ogren's syndrome (SS). Previously reported cases of corneal ulceration occurred in patients with established SS, usually secondary to RA. We describe the first case of corneal ulceration with stromal melting as the initial presentation of primary SS. A 79-year-old man without prior sicca symptoms developed a large sterile corneal ulcer that required extensive treatment over several months with ocular lubricants, systemic immunosuppressives, and surgical repair. Evaluation for an underlying connective tissue disease revealed positive antinuclear antibodies (1:640 speckled) and anti-SSA antibody. A lip biopsy established the diagnosis of SS. Ulceration later occurred in the contralateral eye. Two years after the last corneal ulcer and no longer taking prednisone, the patient's ocular disease remained quiescent taking azathioprine 175 mg and hydroxychloroquine 400 mg daily. This case highlights the potential for primary SS to present with serious ocular complications despite lack of a priori sicca symptoms, as well as the importance of immunosuppressive therapy in the treatment of this complication. ( info) |
6/63. Hypovitaminosis A in metropolitan Adelaide. Hypovitaminosis A is a well-recognized condition in many developing countries. However, in the developed world the diagnosis is frequently missed or delayed because of its rarity. A 67-year-old man from metropolitan Adelaide presented to us with gradual but severe bilateral visual loss. He had marked punctate epithelial keratopathy in both eyes. Hypovitaminosis was suspected because of his bizarre dietary habit, and this was confirmed by a combination of impression cytology of the ocular surface and biochemical testing of his venous blood. His vision responded dramatically to vitamin a supplementation. Hypovitaminosis A should be suspected in severe cases of 'dry-eye', especially in those patients with unusual dietary habit or malabsorption. ( info) |
7/63. Treatment of chronic graft-versus-host disease with anti-CD20 chimeric monoclonal antibody. We reviewed the clinical outcome of 8 patients with steroid-refractory chronic graft-versus-host disease (GVHD) who received an anti-CD20 chimeric monoclonal antibody (rituximab). Rituximab was given by intravenous infusion at a weekly dose of 375 mg/m(2) for 4 weeks. All patients had received extensive treatment with various immunosuppressive agents; 6 patients had also received extracorporeal photopheresis. All patients had extensive chronic GVHD with diffuse or localized sclerodermoid GVHD and xerophthalmia. Other extracutaneous involvements included cold agglutinin disease with the Raynaud phenomenon, membranous glomerulonephritis, and restrictive or obstructive lung disease. Four patients responded to treatment with ongoing resolution or improvement ranging from 265 to 846 days after therapy, despite recovery of B cells in 3 patients. Rituximab seems to have significant activity in the treatment of refractory chronic GVHD and should be considered for further study in patients with early disease. This study suggests a participating role of B cells in the pathogenesis of chronic GVHD. ( info) |
8/63. Ocular reaction to oxprenolol. A case report. A case is presented of a patient who developed ocular symptoms and a skin rash after treatment with oxprenolol (Trasicor) for 2 months. The lesions improved on withdrawal of the drug and her eye symptoms became worse when she was again treated with oxprenolol. patients who receive beta-adrenergic blocking drugs must be watched carefully for side-effects. ( info) |
| blindness affects approximately 42 to 52 million people worldwide. This article examines the major causes of blindness, including cataract, trachoma, glaucoma, onchocerciasis, nutritional deficiencies, and ocular trauma. Numerous case studies supplement the text. ( info) |
10/63. association of chronic symptomatic neutropenia with the triple A syndrome. Chronic neutropenia syndromes include distinct hereditary disorders with varying degrees of neutropenia. Among the more common inherited disorders associated with symptomatic neutropenia are cyclic neutropenia, severe congenital neutropenia (Kostmann disease), and Schwachman-diamond syndrome. The authors describe a 17-year-old girl with triple A syndrome who developed a progressive decrease in the granulocyte count, finally resulting in long-standing neutropenia. Its probable pathogenesis may be related to dysfunction of ALADIN (the protein known to be mutated in triple A syndrome), resulting in abnormal nucleocytoplasmic transport of essential proteins, in myeloid precursor cells. Chronic neutropenia should therefore be considered among the clinical manifestations of triple A syndrome. ( info) |