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Biomedical subjects

R L Font

Publications and source records attributed to R L Font.

At least 109 records · Page 6Linked to original sources

Combined hamartoma of sensory retina and retinal pigment epithelium.

We report two cases of combined hamartoma of the sensory retina and retinal pigment epithelium (CHR-RPE) in which apparent growth of the lesion was observed. In case 1, the eye was enucleated with a presumed diagnosis of juxtapapillary malignant melanoma. Histopathologically, the enucleated globe showed an elevated peripapillary mass containing disorganized retinal tissue intermixed with vascular and glial elements as well as tubules of proliferating retinal pigment epithelium. We have summarized the clinical features of 53 patients with CHR-RPE reported between 1952 and 1988 excluding the cases compiled by the Macular Society Collaborative Study. While the latter study found an equal sex predilection among their cases, we found a 70% male preponderance among the 53 patients. Of the 54 lesions observed in 53 patients, 76% were juxtapapillary, 17% were macular, and 7% were peripheral. Furthermore, periodic follow-up examination disclosed apparent enlargement of the mass in six patients, five of whom underwent enucleation of the globe for suspected melanoma.

Child↗

Demonstration of HIV-1 and HHV-6 in AIDS-associated retinitis.

Using an immunohistochemical technique and monoclonal antisera, HIV-1 and CMV antigens were demonstrated in lesioned areas of retinal tissues from selected AIDS patients. Polymerase chain reaction (PCR) was utilized to detect HIV-1 and HHV-6 DNA sequences in total retinal tissues from these patients. In this study of six eyes from four patients, two of the retinas contained three different viruses, HIV-1, HHV-6 and CMV. To determine whether HIV-1 and HHV-6 DNA sequences were restricted to the intraretinal lesions, normal and lesioned areas were dissected from the retina, DNA was extracted and subjected to amplification using PCR. The results showed that HIV-1 and HHV-6 DNA were restricted to the lesioned areas. All four lesions (from two different patients) utilized in this study showed the presence of CMV antigens immunohistochemically. The combination of viruses present in each lesion was either HIV-1 and CMV or HHV-6 and CMV. Two of four lesions contained HIV-1 and CMV; a third lesion showed the presence of HHV-6 and CMV. The fourth lesion contained only CMV antigens.

Acquired Immunodeficiency Syndrome↗

Nonmelanized macromelanosomes in a cellular blue nevus. Light and electron microscopic observations.

We noted nonmelanized and partially melanized macromelanosomes in a cellular blue nevus and studied their light microscopic and ultrastructural features. Numerous intracytoplasmic eosinophilic inclusions were found in the lesion; individual cells contained up to nine, although most cells demonstrated two or three. The "macromelanosomelike" inclusions ranged in size from 1 to 15 microns. Most of them were partially melanized, and some were nonmelanized. These globules were periodic acid-Schiff positive and diastase resistant, with a centrally melanized core, best seen with the Fontana-Masson technique. S100 protein stained positively the cytoplasm of some nevus cells but not the inclusions. Electron microscopy confirmed the centrifugal melanization of these structures and their targetlike morphologic characteristics. Nine other cellular blue nevi from our files failed to show similar intracytoplasmic inclusions.

Female↗

Peripunctal melanocytic nevi. Distinctive clinical findings and differential diagnosis.

A peripunctal nevus is a rare lesion of the eyelid margin, six examples of which are included in this report. The lesions all involved the lower punctum and had been present for many years without producing epiphora. In addition to their variable clinical pigmentation and translucent appearance on biomicroscopy, all shared several other diagnostically useful clinical features: an overall dome-shape with a fine micronodularity; a prominent ramifying vascularity; and, most importantly, circumferentially swollen punctal lips that created a slit-like punctal orifice. Histopathologically, the lesions were predominantly or exclusively subepithelial melanocytic nevi and were demonstrated not to compress the punctum or canaliculus. Infiltration of the nevus cells within the orbicularis striated muscle fibers was a common finding owing to the latter's superficial location in the eyelids. If patients request excision for cosmetic reasons, these lesions are best managed by a horizontal circular shave excision with the temporary placement of a silicon punctual plug. The differential diagnosis includes cyst, papilloma, melanoma, and basal cell carcinoma.

Adolescent↗

Endogenous endophthalmitis with lenticular abscess caused by Enterobacter agglomerans (Erwinia species).

We report for the first time a case of metastatic endophthalmitis caused by the gram-negative organism Enterobacter agglomerans (Erwinia species). One day following internal hemorrhoidal ligation, the patient developed anterior uveitis that progressed to panophthalmitis with a lenticular abscess and a necrotic retina. Despite aggressive surgery and appropriate antibiotic therapy, the affected eye lost useful vision.

Abscess↗

Ligneous conjunctivitis after pingueculae removal in an adult.

A 45-year-old man who had uneventful excision of bilateral pingueculae developed bilateral membranous lesions involving the bulbar conjunctivae and corneas. Histologically, the membranes were composed mainly of large fibrinous deposits intermixed with acute and chronic inflammatory cells with areas of fibroblastic and capillary proliferation resembling granulation tissue. By electronmicroscopy the amorphous acidophilic masses were composed of electron-dense, fibrillar material with a periodicity of 10-12 mm, which was consistent with fibrin. Despite mechanical removal of the membranes, they continued to recur rapidly over a period of several months. The lesions apparently responded slowly to topical enzymatic therapy that consisted of hyaluronidase (175 U/ml) and alpha-chymotrypsin (1:5000) drops. Follow-up examination, approximately 1 year after surgery, revealed that the patient was asymptomatic. Ocular examination disclosed slight persistence of gelatinous membranes on the bulbar conjunctivae, most prominent in the left eye.

Adipose Tissue↗

Diffuse uveal melanoma in a 5-year-old child.

A 5-year-old white boy developed a gray-tan discoloration with nodular thickening of the iris stroma in his left eye six weeks earlier. Ophthalmologic examination demonstrated keratic precipitates, peripheral anterior synechiae, and several pale-tan choroidal infiltrates in the involved eye. Cytologic examination of the aqueous humor and results of iris biopsy were interpreted as malignant melanoma of the iris, predominantly of the epithelioid cell type. The globe was enucleated one week later. Histopathologic examination disclosed a diffuse malignant melanoma of the uvea, mixed cell type, with multiple cysts of the ciliary epithelium. Study by electron microscopy indicated bizarre neoplastic melanocytes containing numerous immature melanosomes in various stages of melanogenesis, as well as fully melanized mature melanosomes. Eight months after enucleation the patient developed metastasis to the nasopharynx and base of the skull.

Child, Preschool↗

Malakoplakia of the eyelid. Clinical, histopathologic, and ultrastructural characteristics.

A 56-year-old man had a slowly growing, elevated mass excised over the right medial canthus of the right eye. Results of histopathologic examination disclosed a dermal infiltrate of histiocytes ("von Hansemann histiocytes") containing the characteristic laminated, basophilic inclusions (Michaelis-Gutmann [M-G] bodies). Results of histochemical studies showed that the M-G bodies stained positively for calcium, iron, and mucopolysaccharides. Results of electron microscopic studies showed that the histiocytes contained numerous membrane-bound lysosomal bodies of variable size and density. Some histiocytes demonstrated coccobacilli, some of which were degenerated and incorporated into large phagolysosomes. The latter provided the nidus for the deposition of inorganic elements (calcium and iron) and mucopolysaccharides to form the M-G bodies which displayed concentric calcific rings with radiating spicules of hydroxyapatite crystals. The pathogenesis of malakoplakia is discussed. The case reported herein represents the third one involving the ocular adnexa. Only ten cases of cutaneous malakoplakia have been reported previously.

Cytoplasmic Granules↗

Pulmonary blastoma with rhabdomyosarcomatous differentiation: an electron microscopic and immunohistochemical study.

Pulmonary blastoma is a rare lung tumor composed of epithelial and mesenchymal elements; the latter element may show various patterns of differentiation toward mature tissue, such as cartilage, smooth muscle, and bone. Rhabdomyoblastic differentiation in pulmonary blastoma is quite rare; only five such cases have been reported. We report two cases of pulmonary blastoma with rhabdomyoblastic differentiation documented for the first time by electron microscopy and immunohistochemistry including documentation for myoglobin, actin, vimentin and desmin. The diffuse and prominent rhabdomyoblastic differentiation in one case is most unusual.

Aged↗

Nocardial endophthalmitis: report of two cases studied histopathologically.

We report two cases of nocardial endophthalmitis. Case 1 is a 66-year-old man whose left eye was enucleated about one month after onset of decreased vision. Ophthalmoscopic examination disclosed multiple choroidal masses. Six weeks before the onset of ocular manifestations he had undergone a prolonged carotid endarterectomy with intraoperative complications. The source of the ocular infection was probably exogenous. Case 2 is a 49-year-old woman who had systemic sclerosis (scleroderma) with severe pulmonary insufficiency. She had received moderate doses of corticosteroids. Seventeen months after initiation of therapy she developed ocular manifestations leading to enucleation of the eye one month later. Histopathologically, the enucleated eyes in both cases showed numerous branching, Gram-positive, filamentous organisms involving mainly the plane of Bruch's membrane and the subretinal space. The nosological and microbiological aspects of nocardiosis are reviewed.

Aged↗

Intraosseous capillary hemangioma of the frontal bone.

A 31-year-old woman with a history of slowly progressive proptosis associated with blepharoptosis was found radiographically to have a lytic lesion of the frontal bone. Complete surgical excision of the bony mass was performed. Histopathologically the tumor was a capillary hemangioma. Follow-up information two years later showed that the patient was alive and well, without clinical evidence of recurrence. Plain x-ray films appear to be more reliable for diagnosis than computed tomography exhibiting the characteristic sunburst pattern of intraosseous hemangiomas.

Adult↗

Corneal immunoglobulin deposition in the posterior stroma. A case report including immunohistochemical and ultrastructural observations.

Corneal buttons from a patient with bilateral, central, deeply located white corneal deposits that resembled the clinical description of filiform dystrophy were examined by means of light microscopy, immunohistochemistry, and electron microscopy. With light microscopy, the acidophilic fusiform deposits were seen to be oriented parallel to the stromal lamellae and were mostly in the pre-Descemet region. Immunohistochemical studies disclosed that the stromal lesions stained positively for IgG and lambda light chain. With electron microscopy, the immunoglobulin deposits within the deep corneal stroma appeared to be locally synthesized by the keratocytes. Recent examination of the patient's serum failed to disclose dysproteinemia.

Adult↗

Heterotopic brain tissue in the orbit.

A 3-month-old female infant with Turner's syndrome was noted to have progressive swelling of the left upper lid at the age of 1 month. A computed tomogram demonstrated a well-circumscribed, low-density, homogeneous orbital mass. Initially, the main clinical differential diagnosis included dermoid cyst, hemangioma, and encephalocele. Medial orbitotomy followed by histopathologic examination disclosed a cystic choristomatous malformation containing brain tissue. No choroid plexuses within the mass were demonstrated on microscopic examination. Aspiration of fluid from the cystic mass and subsequent biochemical studies unequivocally demonstrated that the mass was filled with cerebrospinal fluid. Despite five surgical procedures, including two frontal craniotomies, and specialized roentgenographic studies, no communication could be demonstrated between the orbital mass and the central nervous system. We postulate that the cerebrospinal fluid was produced within the orbital mass, which represented ectopic brain tissue. Problems in diagnosis and management of this unusual orbital malformation are discussed.

Brain↗

Endogenous Clostridium panophthalmitis.

Acute visual loss, pain, and redness in the left eye developed in a 61-year-old diabetic man. Results of ophthalmologic examination showed 4+ conjunctival hyperemia and chemosis, no light perception, and restricted ocular motility. Through a hazy cornea, a gas bubble was visualized filling about 50% of the anterior chamber. A vitreous tap disclosed myriad gram-positive rods with terminal and subterminal spores. Cultures of the vitreous grew Clostridium septicum, a gas-forming organism. Exploratory abdominal laparotomy showed a ruptured diverticulum of the sigmoid colon with acute peritonitis. In spite of intensive antibiotic therapy, the patient died approximately 24 hours after admission. Results of histopathologic examination of the postmortem left eye demonstrated massive necrosis of the intraocular structures with numerous gram-positive rods confined mainly to the retinal pigment epithelium (RPE), detached retina, and vitreous. To the best of the authors' knowledge, this is the second reported case of C. septicum panophthalmitis. Only 69 cases of clostridial infections with ocular involvement have been recorded previously in the literature.

Clostridium Infections↗

The value of calcofluor white in the diagnosis of mycotic and Acanthamoeba infections of the eye and ocular adnexa.

In search of an easy and quick method for the diagnosis of fungal and Acanthamoeba infections of the eye, the authors have used calcofluor white (CFW), a fluorescent brightener with marked affinity for chitin and cellulose which are present in the cell walls of fungi and cysts of Acanthamoeba. Paraffin-embedded tissues containing mycotic organisms were stained with CFW and examined under a fluorescent microscope. Several fungi such as Candida sp, Aspergillus sp, Phycomycetes, Fusarium solani, Histoplasma capsulatum, Cryptococcus neoformans, Rhinosporidium seeberi, and others were studied. Trephine corneal biopsy specimens (2 mm) and methanol-fixed corneal scrapings containing Acanthamoeba sp were also examined. The walls of fungi and Acanthamoeba cysts stained bright greenish white in contrast to the reddish-orange background of the supporting tissues. The results demonstrate that the CFW method is a highly reliable and rapid technique for identification of mycotic organisms as well as cysts of Acanthamoeba. This method may also be applied in surgical pathology when performing frozen sections, as well as cytologic examination of corneal smears, vitrectomy specimens, and others.

Amebiasis↗

Nevus sebaceus associated with major ophthalmologic abnormalities.

Nevus sebaceus rarely occurs as part of a syndrome consisting of central nervous system and ophthalmologic abnormalities. We describe a case of nevus sebaceus associated with an epibulbar complex choristoma and colobomas of the optic disc and peripapillary choroid, and review the dermatologic, ophthalmologic, and neurologic literature on the nevus sebaceus syndrome. When associated with other developmental abnormalities, nevus sebaceus and epidermal nevus have erroneously been considered to be a single entity. In this article, we delineate and emphasize the ophthalmologic abnormalities associated with the nevus sebaceus syndrome.

Adolescent↗

Progressive blindness caused by metastatic occult signet-ring cell gastric carcinoma.

We described a metastatic signet-ring cell gastric adenocarcinoma in a 60-year-old woman who complained of progressive visual loss. The discovery of signet-ring cells in the cerebrospinal fluid established the diagnosis of mucus-secreting adenocarcinoma. Histologic study of an ulcerated lesion in the lesser curvature of the stomach established the diagnosis of primary gastric adenocarcinoma. When confronted by a patient with impaired function of the cerebral cortex, cranial nerves, and spinal nerve roots that results in progressive neurologic deterioration, leptomeningeal carcinomatosis should be considered and repeated cytologic examinations of spinal fluid should be obtained.

Adenocarcinoma, Mucinous↗

Diffuse toxoplasmic retinochoroiditis in a patient with AIDS.

Ocular toxoplasmosis typically presents as a focal necrotizing retinochoroiditis. We report a case of diffuse necrotizing retinochoroiditis due to toxoplasmosis in a patient with the acquired immunodeficiency syndrome (AIDS). The appearance at initial presentation was similar to the acute retinal necrosis syndrome. We recommend that toxoplasmosis be considered in the differential diagnosis of diffuse retinal necrosis in patients with AIDS. Although systemic disseminated toxoplasmosis is not rare in AIDS, this is, to our knowledge, the first reported case of diffuse ocular involvement.

Acquired Immunodeficiency Syndrome↗