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Biomedical subjects

R L Font

Publications and source records attributed to R L Font.

At least 181 records · Page 10Linked to original sources
PubMed1980

Jansky-Bielschowsky form of neuronal ceroid-lipofuscinosis. Ocular pathology of the Batten-Vogt syndrome.

The neuronal ceroid-lipofuscinoses (Batten-Vogt syndrome) are a group of at least four closely related entities that are characterized by the accumulation of autofluorescent lipopigments in neural, visceral, and somatic tissues. The ophthalmic pathology of this syndrome has been investigated less extensively than other tissues and organs. In this report, we present the postmortem ocular findings, including light, fluorescence, and electron microscopy, from a child with the Jansky-Bielschowsky form of this syndrome.

Child, Preschool↗
PubMed1980

Intracellular inclusions in meningothelial meningioma. A histochemical and ultrastructural study.

This is a study on the histochemical and electron microscope findings of the intracellular inclusions in a case of meningothelial meningioma of the frontoethmoidal region with orbital involvement. The inclusions are PAS-positive, diastase resistant, and stain strongly positive with the modified Morel-Sisley reaction for protein-bound tyrosine and weakly with dihydroxy-dinaphthyl-disulfide (DDD) for the demonstration of sulfhydryl (SH) and disulfide (S-S) groups of proteins. These staining reactions suggest the inclusions are composed of conjugated proteins (glycoproteins). Ultrastructurally, the inclusions are located within intracellular spaces lined by microvilli and are composed of granular material, usually forming a dense core that is intermixed with small vacuoles. Numerous desmosomes and whorls of tonofibrils are concentrated around them. Our findings support the view that the intracellular inclusions, which are rarely found in meningiomas, represent an active secretory product of the meningothelial cells rather than a degenerative or phagocytic process.

Female↗
PubMed1980

The iris naevus (Cogan-Reese) syndrome: light and electron microscopic observations.

The light and electron micrscopic findings in an eye enucleated from a 38-year-old woman with iris naevus (Cogan-Reese) syndrome are presented. The clinical manifestations of this disease, namely, iridic stromal matting, iridic nodule formation, and unilateral angle-closure glaucoma, appear to be secondary to an endothelial membrane on the anterior surface of the iris. The iris naevus syndrome is now thought to represent one end of the clinical spectrum of an iridocorneal endothelial syndrome which also includes those clinical entities classified previously as Chandler's syndrome and essential iris atrophy.

Adult↗
PubMed1979

The basement membrane exfoliation syndrome.

Previous observations suggest that "pseudoexfoliation of the lens capsule" may be a disease in which cells produce abnormal basement membrane material. Electron microscopic studies indicate that exfoliative material can be produced by cells throughout the anterior segment of the eye. This report describes abundant exfoliative material on the ciliary and iridic epithelia but not on the surface of the lens whose epithelial cells were completely necrotic. Exfoliative material also was observed in the anterior iridic stroma close to a newly formed abnormal endothelial basement membrane, suggesting that exfoliative material may be produced locally within the iris. Furthermore, typical exfoliative material, best considered a filamentous, banded basement membrane with 500-A periodicity, in the wall of a short posterior ciliary artery in the orbit indicates that the exfoliative process is not solely limited to ocular tissues. The term "basement membrane exfoliation syndrome" is proposed for this entity.

Aged↗
PubMed1979

Ocular inflammation and hemorrhage as initial manifestations of uveal malignant melanoma. Incidence and prognosis.

A consecutive series of 450 eyes enucleated because of a malignant melanoma of the choroid or ciliary body was reviewed in an effort to establish the incidence of ocular inflammation or intraocular hemorrhage as the initial clinical manifestation. Thirty-five patients had these clinical signs initially. Twenty-two (4.9%) initially had ocular inflammations. Eight of these had an episcleritis, and the remaining 14 had some form of uveitis, endophthalmitis, or panophthalmitis. Five of those with episcleritis had tumors in the ciliary body, and all six patients who developed panophthalmitis had necrotic choroidal melanomas. Thirteen patients (2.9%) were initially observed with some form of intraocular hemorrhage. Follow-up information was available for 26 of the 35 patients selected for detailed study. Fourteen patients died of metastatic disease. We believe the relatively poor prognosis of these tumors depends on three factors: cell type, maximal diameter of the tumor, and extraocular extension.

Adult↗
PubMed1979

Malignant lymphoma of the retina and brain. Initial diagnosis by cytologic examination of vitreous aspirate.

A case of malignant lymphoma reticulum cell sarcoma in a 42-year-old woman who had bilateral uveitis and vitreitis and neurologic manifestations is reported. The diagnosis was first established by cytologic examination of vitreous aspirate, which showed neoplastic cells consistent with malignant lymphoma and led to cancellation of a planned craniotomy for suspected intracranial tumor. The diagnosis was later confirmed postmortem by histopathologic examination of the eyes and brain. The role of cytologic examination of the vitreous in the diagnosis of malignant lymphoma is discussed briefly.

Adult↗
PubMed1979

Proliferative endotheliopathy with iris abnormalities. The iridocorneal endothelial syndrome.

Sixteen cases of essential iris atrophy and two of iris nevus (Cogan-Reese) syndrome were reviewed retrospectively by light and electron microscopy. Varying degrees of corneal endothelial proliferation onto the trabecular meshwork and anterior iris were seen in all cases. The demonstration of an endothelial-abnormal basement membrane complex on the anterior iridic surface, deep to synechias in over half the cases, indicates that endothelialization may be primary, since it precedes the formation of anterior synechias. Pathologic and clinical evidence suggests that the iris nevus syndrome, Chandler's syndrome, and essential iris atrophy represent a continuum of clinical manifestations of a single disease process involving proliferation of the corneal endothelial and characteristic iris abnormalities; the latter may be secondary to variations in the pattern, rate, and extent of endothelial proliferation. We suggest the term "proliferative endotheliopathy" to emphasize the common pathogenetic role of corneal endothelial proliferation in all three entities (iris nevus syndrome, Chandler's syndrome, and essential iris atrophy). A more succinct alternate term is "the iridocorneal endothelial (ICE) syndrome."

Adult↗
PubMed1979

Mucinous sweat gland adenocarcinoma of eyelid: a clinicopathologic study of 21 cases with histochemical and electron microscopic observations.

This is a clinicopathologic study of 21 cases of mucinous sweat gland adenocarcinoma involving the eyelid. The tumor occurred in middle-aged adults (median age 60 years) and has a predilection for males. In this series eight patients (40%) had one or more local recurrences, one of whom died with extensive local invasion of the face after multiple recurrences in a 15-year interval. Only one patient had metastasis to the submandibular lymph nodes, which was treated by radical neck dissection. Involvement of the head, especially the face and scalp, was observed in 78% of the cases from the literature. Combining our cases with those previously reported, we found that the eyelid was involved in almost half of the cases (21 out of 45 lesions). We believe that the identification by light microscopy of a mixed population of light and dark secretory cells within the tumor lobules, the histochemical findings (presence of sialomucin and absence of iron as well as periodic acid-Schiff positive, diastase-resistant granules), and the results of ultrastructural studies support the histogenetic postulate that this mucinous adenocarcinoma is probably derived from the secretory cells of the eccrine coil. Our interpretation concurs with Headington's view of origin from the eccrine glands.

Adenocarcinoma, Mucinous↗
PubMed1979

Inverted papilloma of the conjunctiva.

We encountered inverted papilloma of the conjunctiva in three cases. To the best of our knowledge, this tumor, which typically involves the nose, paranasal sinuses, and lacrimal sac, has not been described previously in the conjunctiva. Two of the tumors occurred as purely inverted lesions of the inner canthal region )caruncle and plica) and the third as a mixed inverted-exophytic papilloma over the tarsus. Melanotic pigmentation in one of the lesions was clinically confused with malignant melanoma. Because of the high incidence of recurrence of these tumors in the sinuses and lacrimal sac, and their unknown biologic behavior in the conjunctiva, inverted papillomas should be removed completely and the patients carefully observed.

Aged↗
PubMed1979

Clinicopathologic characteristics of orbital lymphoid hyperplasia.

The clinical characteristics and microscopic features of the biopsy specimens of 66 patients with orbital lymphoid tumors were analyzed with respect to clinical outcome (disease remaining localized to the orbit or developing associated extraorbital lesions). The patients' average age was 63 years, and their tumors had a marked tendency to arise in the anterosuperior orbit. No clinical or radiographic findings helped to distinguish the cases of localized disease from disseminated disease. The lesions were divided microscopically into three categories: reactive lymphoid hyperplasia (16), atypical lymphoid hyperplasia (38), and malignant lymphoma (12). Extraorbital lesions developed in 25%, 50%, and 75% of cases, respectively. The five-year mortality rates were also significantly different for each type of lesion: 6%, 19%, and 58%, respectively.

Adult↗
PubMed1978

Cysticercosis of the eyelid.

A 14-year-old girl had a four-month history of a painless, enlarging mass in the right upper eyelid. Histopathologic examination revealed the cystic mass to be Cysticercus cellulosae, the larval form of Taenia solium. This patient had been in Vietnam and Laos several years previously and, while there, developed a flu-like syndrome. Despite the fact that she was asymptomatic for the two years preceding her clinical examination, the histopathologic diagnosis of C cellulosae prompted an extensive medical and neurologic work-up to rule out the possibility that the patient harbored the adult tapeworm or other larval forms. Fortunately, the results of this survey were negative.

Adolescent↗
PubMed1978

Ocular involvement in Whipple's disease: light and electron microscopic observations.

A 52-year-old man had a prolonged history of nondeforming migratory polyarthritis and a short episode of pericarditis preceding the onset of bilateral vitreitis and retinitis. The clinical course was characterized by progressive deterioration of vision, increasing lethargy, and dementia, leading to coma and death from pneumonia (21 months later). No intestinal manifestations were recorded. Both eyes, which were removed postmortem, disclosed numerous PAS-positive macrophages throughout the inner retina and vitreous. Electron microscopic studies of the macrophages displayed intracytoplasmic, degenerating, rod-shaped bacteria and membranous structures identical to those seen in the intestine, brain, heart, and other tissues of patients with Whipple's disease. Clinicians should include Whipple's disease, and reticulum cell sarcoma, in the differential diagnosis of patients with bilateral retinitis and vitreitis, especially if these disorders are associated with CNS manifestations.

Eye Diseases↗
PubMed1978

Osseous choristoma of the choroid simulating a choroidal melanoma. Association with a positive 32P test.

We report the clinical and histopathologic features of an osseous choristoma of the choroid that appeared in a 26-year-old woman. Her diagnostic evaluation included a radioactive phosphorus (32P) uptake test, which was 270% positive and led us to believe that the lesion was an atypical malignant melanoma. The extent of true ossification within this tumor, seen microscopically, explains the false-positive uptake of radioactive phosphorus by this benign tumor; it is well known that bone avidly accumulates radioactive phosphorus. We recommend that all "atypical choroidal melanomas" be studied for the presence of bone, with bone-free roentgenograms, ultrasonography, and possibly, computerized tomographic scans before 32P testing or enucleation. This, to our knowledge, is the second histopathologically proved case of an osseous choristoma of the choroid to be reported in the literature.

Adult↗
PubMed1978

Mucogenic glaucoma and goblet cell cyst of the anterior chamber.

A 25-year-old woman had a unilateral open-angle glaucoma that was initially thought to be caused by an anterior uveitis. A deformity of the chamber angle was noted and interpreted as a peripheral anterior synechia or an old scar. A peculiar haze was noted in the anterior chamber. The intraocular pressure was controlled by trabeculectomy. A slowly enlarging cyst was observed at the site of a previous anterior synechia. Excision of the cyst resulted in clearing of the anterior chamber haze. The second trabeculectomy specimen contained mucous strands. Problems exist, both clinically and histopathologically, in diagnosing and managing this rare form of secondary glaucoma (mucogenic glaucoma).

Adult↗
PubMed1978

Malignant medulloepithelioma of the optic nerve in a horse.

An 18-month-old Standardbred filly had a large intraocular tumor involving the optic nerve. The tumor was a malignant medulloepithelioma, a rare intraocular neoplasm derived from the primitive medullary epithelium. By light microscopy the tumor had cords and lobules of primitive neuroepithelial cells that formed clefts and true rosettes. Electron microscopy of the rosettes showed a girdle of zonulae adherentes joining the apices of the cells as well as several basal bodies. This is the sixth report of equine intraocular medulloepithelioma, and, to the best of our knowledge, the first intraocular medulloepithelioma arising from the optic nerve head in a horse. Massive involvement of the optic nerve should be suspected when an eye containing an intraocular mass in the posterior segment lacks light perception. In such cases a long segment of opitc nerve should be resected.

Animals↗
PubMed1978

Sporotrichosis in a Pacific white-sided dolphin (Lagenorhynchus obliquidens).

A severe necrotizing granulomatous lymphadenitis caused by Sporothrix schenckii was diagnosed in a Pacific white-sided dolphin (Lagenorhynchus obliquidens) that had been in captivity for about 3 years. Histopathologic and electron microscopic studies, as well as fluorescent antibody techniques, were used to identify S schenckii as the etiologic agent.

Animals↗
PubMed1977

Toxoplasmic retinochoroiditis: electron-microscopic and immunofluorescence studies of formalin-fixed tissue.

An 82-year-old white man had bilateral, chronic recurrent uveitis caused by Toxoplasma gondii. In spite of extensive therapy for toxoplasmosis, the left eye became painful and blind and was enucleated. Histopathologically, the retina disclosed unusually large numbers of necrotic cysts and a few presumably viable cysts containing crescent-shaped organisms and scanty free forms of T gondii. Electron-microscopic studies confirmed the presence of a true wall of the cysts as well as additional ultrastructural features highly characteristic of the parasite. Immunofluorescent studies of the formalin-fixed tissue demonstrated quite vividly the cysts, which stained variably according to the stage of viability of the organisms. In cases in which toxoplasmic retinochoroiditis has been suspected clinically but routine histologic preparations fail to reveal the organisms, the remaining formalin-fixed tissue should be examined by specific immunofluorescent methods to demonstrate antigenic material of the causative parasite.

Aged↗