Immunocytochemical localization of vascular endothelial growth factor in neurons and glial cells of human retina.

In order to establish the cellular and subcellular localization of the chemokine protein, vascular endothelial growth factor (VEGF) or vascular permeability factor, in adult human retina, we employed immunocytochemistry with double immunolabeling, using a primary antibody to amino acids 1-10 of VEGF, together with antibodies to vimentin (intermediate filaments, labeling Müller cells) or to neuron-specific enolase (labeling retinal neurons). In adult human retina, VEGF-like immunoreactivity (VEGF-IR) is found in Müller cell processes, where typically it is found in the cytoplasm in close association with Vimentin-labeled (VM-IR) intermediate filaments. VEGF-IR is sometimes found diffusely in Müller cell bodies and nuclei.

Congenital corneal opacification in De Barsy syndrome.

A newborn male was noted to have bilateral congenital corneal opacification. Findings from examination disclosed a variety of dysmorphic features, including cutis laxa, progeroid aspect, short stature, multiple hyperextensible subluxated joints, muscular hypotonia, and hyperreflexia. Bilateral penetrating keratoplasties were performed; histopathologic examination revealed diffuse epithelial thickening, loss of the Bowman layer, and stromal attenuation with anterior stromal scarring.

Immunolocalization of beta ig-h3 protein in 5q31-linked corneal dystrophies and normal corneas.

Objective: To characterize the relation of the beta ig-h3 protein to the diagnostic corneal deposits in the hereditary corneal dystrophies recently shown to have mutations in the beta ig-h3 gene on chromosome 5q31.

Methods: Corneas with lattice, granular, mixed granular-lattice ("Avellino"), and 2 types of Reis-Bücklers dystrophy were diagnosed by the histochemical and ultrastructural characteristics of their abnormal aggregates. Dystrophic and normal corneas were compared for immunolocalization of beta ig-h3 protein.

Potato gun ocular injury.

Purpose: This study aimed to identify a dangerous new weapon capable of causing damage to the ocular and periocular regions.

Methods: The authors report two patients who had penetrating ocular injury in the past year because of homemade recreational potato guns.

Results: In one 14-year-old boy, projectiles from the firing of a potato gun resulted in orbital and cranial injuries that were life threatening with widespread fractures, marked disruption of facial structures, a cerebrospinal fluid fistula requiring bifrontal surgical repair, and loss of one eye.

Ocular involvement in familial erythrophagocytic lymphohistiocytosis.

Background: Familial erythrophagocytic lymphohistiocytosis (FEL), a rare, rapidly fatal childhood disorder, is characterized by intermittent fevers, hepatosplenomegaly, cytopenia, hypercytokinemia and lymphohistiocytic infiltration with erythrophagocytosis involving multiple organs. We report the clinical and histological features of two infants with FEL and emphasize the ocular findings.

Methods: Microscopic examination of formalin-fixed, paraffin-embedded autopsy material was performed.

Exfoliation syndrome in a 17-year-old girl.

A 17-year-old girl with unilateral congenital glaucoma who had undergone trabeculectomy and peripheral iridectomy in infancy developed apparent exfoliation syndrome (XFS) in the eye that underwent the surgical procedures. A conjunctival biopsy was performed and the specimen was fixed in 2.5% glutaraldehyde, embedded in epoxy resin (Epon-Araldite, Electron Microscopy Sciences, Fort Washington, Pa), and processed for routine electron microscopy and immunostaining for elastin.

HNK-1 epitope in the lens-ciliary zonular region in normal and pseudoexfoliative eyes. Immunohistochemistry and ultrastructure.

Objective: To determine the specific binding sites for the HNK-1 adhesion molecule epitope in the lens-ciliary zonular region in normal and pseudoexfoliative (PSX) eyes.

Methods: Normal and PSX eyes and adjacent tissues from individuals aged 10 months to 89 years were investigated for the HNK-1 epitope by immunohistochemistry and immunogold electron microscopy.

Results: Concentration of HNK-1 epitope was high in the anterior central lens capsule into old age, but it tapered earlier and progressively in the inner capsule and elsewhere.

Elastosis of the lamina cribrosa in pseudoexfoliation syndrome with glaucoma.

Background: Pseudoexfoliation syndrome is characterized by the presence of glycoprotein fibers in ocular and extraocular tissues, and often is associated with glaucoma. Pseudoexfoliation material may be associated closely with elastic microfibrillar-associated glycoprotein as well as elastin.

Methods: Four optic nerve heads of two patients with pseudoexfoliation syndrome and glaucoma were examined using electron microscopy and immunogold detection of elastin.

Burkitt lymphoma presenting as a conjunctival mass.

Purpose: A sporadic Burkitt lymphoma, which presented as a primary conjunctival mass, demonstrates the approach to clinical diagnosis, staging, and current management of this rapidly growing malignant tumor, as well as newer concepts of its pathogenesis.

Patient And Methods: A 16-year-old girl had rapid development of a conjunctival mass 6 weeks after an infectious mononucleosis-like illness, associated with a positive monospot test. A biopsy of the tumor was immunostained for lymphocyte markers.

Benign approach to a malignant orbital tumor: metastatic renal cell carcinoma.

A well-circumscribed, pseudoencapsulated benign-appearing lesion in the retrobulbar orbit of a 50-year-old man was found to be a metastatic renal cell carcinoma. The patient had a nephrectomy for renal cell carcinoma 15 years before, and had developed four other isolated metastases over the preceding 8 years. He had a further distant metastasis 1 year later, but is living and well 4 years after excision of the orbital tumor.

Pseudoexfoliative fibrillopathy in visceral organs of a patient with pseudoexfoliation syndrome.

Evidence is increasing that pseudoexfoliative material develops in widespread areas of skin and parabulbar tissues as well as intraocularly. To determine whether this process is even more diffusely distributed, ultrastructural examination was performed on visceral and ocular tissues of a patient with long-standing glaucoma found to have bilateral ocular pseudoexfoliation at autopsy. Aggregates consistent with pseudoexfoliative material were present in the lung, heart, liver, and gallbladder, in addition to the classic intraocular sites.

Molluscum contagiosum of the eyelids in patients with acquired immune deficiency syndrome.

Background: Infection with molluscum contagiosum has been reported in patients with acquired immune deficiency syndrome (AIDS). Involvement of the eyelids by molluscum in patients with AIDS has rarely been mentioned.

Methods: Two patients with AIDS presented with eyelid molluscum contagiosum.

Proboscis lateralis with associated orbital cyst: detailed MR and CT imaging and correlative embryopathy.

Proboscis lateralis is a rare craniofacial malformation. We present a case in a 1-week-old male infant, describe the clinical and imaging findings, and discuss the pertinent embryology. MR and CT proved to be complementary: CT provided anatomic detail in bone, defined the nasal cavity, and aided in determining the relationship of the proboscis and orbit; MR clarified the relationship of the proboscis to the orbit and skull base, and the relationships of normal brain to the dysplastic ethmoid centers.

Characterization of an associated microfibril protein through recombinant DNA techniques.

The complete primary structure of a new extracellular protein associated with elastic fiber microfibrils was determined by recombinant DNA techniques. Antiserum to insoluble bovine ocular zonule protein was used to screen a lambda gt11 cDNA expression library constructed from whole chick embryo poly(A)+ RNA. The cDNAs encoding immunoreactive fusion polypeptides were then used to rescreen the library by plaque hybridization.

Abnormal scleral collagen in nanophthalmos. An ultrastructural study.

Ten patients with bilateral nanophthalmos underwent sclerectomies for uveal effusion. Ultrastructural examination of the sclera revealed abnormal collagen in seven patients. Four showed dramatic fraying of the collagen fibrils into fine filaments 2 to 3 nm in diameter.

Elastic fiber components and protease inhibitors in pinguecula.

The nature of the abnormal elastotic materials seen in pingueculae and their insensitivity to elastase are poorly understood. The authors investigated their composition by immunoelectron microscopy using antibodies to elastic fiber components, serum and tissue components known to be associated with elastosis in other sites. The abnormal elastic fibers showed labeling for elastin, microfibrillar protein, and amyloid P where these components never co-localize normally, indicating the fibers are not simply immature but aberrant in organization.

Rotary shadowing of elastic system microfibrils in the ocular zonule, vitreous, and ligamentum nuchae.

Rotary shadowing of zonular fibrils in human and bovine eyes revealed a "string of beads" configuration with multiple interconnecting filaments, identical to that recently reported in fibrils of unknown type within the vitreous. These 29 nm beaded fibrils were the only macrostructures present in zonular samples, showing ultrastructural features correlating with both the macro and microperiodicity of zonular fibrils in tissues. Interbead periodicity varied from 30-57 nm and interbead filaments appeared capable of stretching even further, possibly explaining the inherent elasticity of zonular fibrils.

Precapsular film on the aging human lens: precursor of pseudoexfoliation?

In many older patients we observed a layer of subtle opacification on the anterior lens capsule, appearing as a ground glass film biomicroscopically. This precapsular film (PCF) could be uniform but often had radial grey lines in the mid zone, holes in the paracentral region, and was occasionally rolled up in strings. Lens capsular material obtained at cataract extraction was studied in patients with and without the film.

Necrobiotic xanthogranuloma with IgA multiple myeloma: a case report and literature review.

The association between necrobiotic xanthogranuloma of the dermis and paraproteinemia and/or B-cell malignancy is best described in the ophthalmologic literature. We report a case which occurred in the eyelid and orbit of a 64 year old man that led to the diagnosis of an IgA multiple myeloma. To our knowledge, this is the first report of an IgA type paraproteinemia and IgA type multiple myeloma associated with necrobiotic xanthogranuloma.

Pseudoexfoliative fibrillopathy in the skin of patients with ocular pseudoexfoliation.

In addition to its occurrence within the eye, pseudoexfoliative fibrillopathy has been reported in the conjunctiva and around a posterior ciliary artery. To determine whether it has a more diffuse extraocular distribution, we studied skin biopsy specimens ultrastructurally from one to three areas in 13 patients with classic pseudoexfoliation. A fibrillopathy closely resembling that in the eye was found in 11 of the 13 patients.

Amyloid P protein in pseudoexfoliative fibrillopathy.

Amyloid P protein was demonstrated by immunostaining in all 14 samples of ocular and conjunctival pseudoexfoliative (PSX) material studied, although amyloid was not found by Congo red staining or ultrastructurally. Immunostaining of PSX aggregates for other common amyloid proteins, including amyloid A, prealbumin, and immunoglobulin light chains, was negative in most cases. In three eyes with advanced neovascular glaucoma there was irregular immunostaining of the PSX aggregates for 2-4 of these other amyloid proteins, besides diffuse staining of the iris and vitreous.

Association of elastin with pseudoexfoliative material: an immunoelectron microscopic study.

Using immunoelectron microscopy, the presence of elastin and tropoelastin was demonstrated in pseudoexfoliative (PSX) material in all its classical sites on the lens capsule, ciliary non-pigment epithelium, iris epithelium and stroma, and conjunctiva. Some variability in binding affinity was seen in different sites, and labelling was more often on the periphery than the center of the PSX fibers. The elastin epitope on PSX material was more sensitive to processing than the remarkably stable epitope on mature elastic fibers.

Identification of extractable proteins from the bovine ocular zonule: major zonular antigens of 32kD and 250kD.

Using Western immunoblotting, the extractable proteins of the bovine zonular fibers were examined for reactivity with two zonular antisera known to have strong affinity for zonular fibers in tissues, in order to identify the antigenic components. The extracts were also tested with antisera to several matrix proteins that have been reported to be associated with zonular fibers. Proteins reactive with antisera to bovine serum albumin, serum immunoglobulins and fibronectin were present.