Synthetic fiber granuloma. 'Teddy bear' granuloma of the conjunctiva.

Conjunctival synthetic fiber ("teddy bear") granulomas result from a granulomatous response to synthetic fabric fibers introduced into the conjunctival sac. There is a striking propensity for these lesions to develop in the pediatric age group, to be unilateral, and to be situated in the inferior conjunctival fornix. It is believed that proximity of the eye to comforting blankets, fabric toys, or forcibly applied pullover sweaters may provide the opportunity for ocular inoculation. Described below is the case of a 6-year-old girl who had a conjunctival synthetic fiber granuloma that exhibited the characteristic clinical and pathologic features of these lesions.

Carcinoma metastatic to the eye and orbit II. A clinicopathological study of 26 patients with carcinoma metastatic to the anterior segment of the eye.

In 26 of 227 patients with carcinoma metastatic to the eye or orbit, metastasis to the anterior uveal tract was the predominating feature. There was a definite propensity for the tumor to involve the horizontal meridian of the iris or ciliary body, rather than the upper or lower portions. The site of the primary tumor in the 26 patients was as follows: lung 14; breast, 9; kidney, 2; and rectum, 1. Ocular symptoms and signs produced by the metastatic tumors at onset or during the course of the disease included decreased vision (80%), a visible mass (72%), redness of the eye (56%), pain (56%), glaucoma (56%), iridocylitis (44%), and hyphema (24%). The median survival of the 26 patients with metastasis to the anterior segment of the eye was only 5.4 months from the time of ocular surgery. This is poorer than the median survival (7.2 months) of the patients with metastasis confined to the posterior segment, and much worse than the median survival (15.6 months) of the 28 patients with orbital involvement.

Bilateral amyloidosis of the vitreous body: report of a case without systemic of familial involvement.

Amyloidosis of the vitreous body is an uncommon and often misdiagnosed condition that causes progressive visual loss. It is usually associated with primary familial systemic amyloidosis. The patient described in this report has amyloidosis of both vitreous bodies; her case is unusual because no other family members are affected and because there has been no evidence of systemic involvement over a seven-year observation period. After part of the vitreous body of her right eye was removed surgically, visual acuity improved from light perception to 20/60. New concepts regarding the nature, classification, and histochemical identification of amyloid are discussed.

Lichen simplex chronicus of the eyelid.

Lichen simplex chronicus is a common dermatosis that rarely affects the eyelids. We report the clinical and pathologic features in the case of a middle-aged man who had lichen simplex chronicus of both lower eyelids. The clinical features suggested the presence of basal cell carcinoma.

The histopathology of filamentary keratitis.

Filamentary keratitis is characterized by the presence of fine filaments of epithelium and mucus that are attached to the cornea. The exact pathogenesis of this entity has remained unclear. One reason has been the lack of pathologic specimens of corneas from patients with filamentary keratitis. We examined the corneas of a patient who had died while suffering from filamentary keratitis. Scattered groups of inflammatory cells and fibroblasts were present just below the basal epithelium. It seemed that these cells had disrupted the epithelial basement membrane and Bowman's layer. To our knowledge, this is the first postmortem analysis of a cornea in a patient with acute filamentary keratitis. These findings support the theory that filamentary keratitis results from damage to the basal epithelial cells, epithelial basement membrane, or both.

Actinic granuloma of the conjunctiva.

The key histologic feature of pingueculas is the presence of actinic elastosis of the conjunctival connective-tissue fibers. We describe a patient who had a pinguecula that, on pathologic examination, exhibited a prominent foreign-body giant-cell reaction in association with the elastotic fibers. The term actinic granuloma has been coined recently to designate a similar reaction in sun-damaged skin. Because the nature of the inflammatory reaction may be misinterpreted on pathologic examination as indicating the presence of microorganisms or a foreign body, both the clinician and the pathologist should be aware of this peculiar inflammatory response, which has been described on two previous occasions in patients with pingueculas.

Primary nonkeratinized epithelial ('conjunctival') orbital cysts.

The types of orbital cysts that are most familiar to ophthalmologists are epidermoid and dermoid cysts, both of which are lined by keratinized stratified squamous epithelium. We studied six patients who had orbital cysts lined by nonkeratinized epithelium that resembled normal conjunctiva. Compared with epidermoid and dermoid cysts, these primary nonkeratinized cysts tend to cause symptoms later in life, occur preferentially in the superonasal aspect of the orbit, and are less likely to be associated with underlying bony changes.

Pathologic examination of ciliary body melanoma treated with proton beam irradiation.

Proton beam irradiation is one of the radiotherapeutic techniques currently used to manage uveal melanomas. Although this therapeutic modality has been in use for a decade and although nearly 500 patients have been so treated, there are only two published reports of the pathologic examination of these eyes. Key features found on pathologic examination of our patient's enucleated eye included vascular alterations in the tumor's blood supply, lymphocytic infiltrates, and lipoidal degeneration of tumor cells. The interval from therapy to enucleation in our patient was much longer than in the several previously reported cases, a factor allowing for the development of a more cumulative radiation effect on the tumor. The extent and degree of radiation-induced necrosis in our patient's tumor were more profound than in previous reports.

Hemorrhage into the lens as a complication of glaucoma surgery.

Intralenticular hemorrhage is a rare complication of glaucoma surgery. In a 45-year-old man, the intralenticular blood caused loss of the red reflex from the fundus postoperatively. Clinically, hemorrhage into the lens has usually been misdiagnosed initially as a hyphema.

Subepidermal calcified nodules of the eyelid.

Two children had subepidermal calcified nodules of the upper eyelid. The first patient was a 13-year-old girl who noted painless increase in size of a lesion involving her right upper eyelid over four months. The clinical diagnosis was "warty papilloma." The second patient was a 13-year-old boy who had a slowly growing, keratinized lesion involving his left upper eyelid. The clinical diagnosis was "cutaneous horn." On histopathologic examination, the nodules demonstrated the characteristic changes associated with subepidermal calcified nodules, including the presence of calcified material in the uppermost dermis, occasional foreign body giant cells around the calcific masses, acanthosis of the overlying epithelium, and calcium granules in the epidermis.

Cutaneous leishmaniasis (oriental sore) of the eyelid.

A 45-year-old American residing along the basin of the Mediterranean Sea developed a cutaneous lesion involving his right lower eyelid. He returned to the United States several months after it first appeared and sought treatment. On clinical examination the lesion was regarded as either a tumor or a cyst. It was excised, and on pathological examination a chronic granulomatous inflammatory lesion, containing innumerable Leishman-Donovan bodies, was found.

Granular cell tumor (myoblastoma) of the palpebral conjunctiva causing pseudoepitheliomatous hyperplasia of the conjunctival epithelium.

Granular cell tumors rarely occur in the ocular adnexal structures and only three cases have been described in which the eyelid alone was involved. I examined a patient who was, I believe, the first in whom a granular cell tumor appeared clinically as a conjunctival lesion. She was also the first patient with a granular cell tumor of the ocular adnexa to exhibit pseudoepitheliomatous hyperplasia of the overlaying epithelium, a pathologic feature associated with granular cell tumors originating elsewhere in the body.

Recurrence of lattice corneal dystrophy type 1 in the corneal grafts of two siblings.

Amyloid was identified by light and electron microscopy within corneal grafts of two male siblings from a family with lattice corneal dystrophy type 1. These deposits indicate a recurrent disease within the donor tissue, and we believe this reflects an infiltration of the grafts by genetically abnormal host corneal fibroblasts. One of the patients developed bilateral recurrent disease in the grafts eight and 13 years after penetrating keratoplasty. His brother required a regraft 16 years after a penetrating keratoplasty. Although both of these patients required regrafts because of impaired visual acuity, the decrease in visual acuity in one case was not solely the result of reaccumulation of amyloid, but was at least in part caused by a plaque of fibrous tissue behind the cornea.

Orbital invasion by an intracranial chordoma.

A 26-year-old woman experienced bilateral hearing loss, progressive nasal obstruction, and rhinorrhea. Examination disclosed a retropharyngeal mass. A needle biopsy specimen of the mass showed that it was a chordoma. The patient underwent surgery to remove the mass and received a postoperative course of radioactive cobalt. She did well for 18 months, at which time proptosis gradually developed in her right eye. Although the optic nerve heads and visual fields appeared normal, roentgenograms showed a large lesion involving the anterior and middle cranial fossae and destruction of the right posterior ethmoid sinus and right superior orbital fissure. Shortly after completing a course of methotrexate therapy (total dose, 89 mg), the patient experienced sudden pain and visual loss in her right eye. Surgical decompression of the orbit failed to restore light perception. The following year, her left eye became involved. A transfrontal craniotomy and extradural orbital decompression provided only temporary improvement. Four months later, left lateral rectus muscle palsy developed and her visual acuity decreased to 6/60 (20/200). Radiation therapy (400 rads per week; total dose, 3,200 rads) and treatment with methotrexate, vincristine sulfate, and prednisone did not improve her condition. At the time of her death, six years after the first symptom appeared, the patient was blind in both eyes, almost completely deaf, and suffered from severe dysphagia.

Fusarium solani endophthalmitis without primary corneal involvement.

A 45-year-old woman developed a fungal endophthalmitis caused by Fusarium solani. No primary corneal lesion existed. Therefore, the focus of infection was considered endogenous. Although disseminated systemic infections with Fusarium species have been described in compromised hosts, including a patient with metastatic panophthalmitis, we believe this is the first report of an endogenous endophthalmitis caused by F. solani in a healthy individual.

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.

Combined granular-lattice ('Avellino') corneal dystrophy.

BACKGROUND/PURPOSE: In 1988, a report was published describing the histopathologic examination of corneal buttons of 4 patients who had undergone unilateral keratoplasty because of decreased vision caused by what had been diagnosed clinically as granular dystrophy. But on pathologic examination, lesions characteristic of both granular dystrophy and lattice dystrophy were found in each of the 4 corneal buttons. The patients came from 3 different families, each of which traced its origin to the Italian province of Avellino. We studied the clinical and histopathological features of 4 corneas affected by combined granular-lattice dystrophy, adding thereby to the total of 12 other corneas that have been so-described in the literature. METHODS: Two women underwent bilateral penetrating keratoplasty for what was diagnosed clinically as Reis-Bücklers dystrophy in the first patient, and as granular dystrophy in the second patient. We studied all 4 corneas pathologically, using both conventional hematoxylin and eosin stains as well as special histochemical techniques. RESULTS: All 4 corneas contained lesions characteristic of both granular dystrophy and lattice dystrophy, a circumstance that has given rise to the name "combined granular-lattice dystrophy." The patients are not known to be of Italian ancestry. CONCLUSIONS: Three clinical signs characterize combined granular-lattice dystrophy: (1) anterior stromal discrete, grayish-white deposits; (2) lattice lesions located in mid-stroma to posterior stroma; and (3) anterior stromal haze. Both clinically and histopathologically, the lattice lesions are of greater diameter than are those that occur in lattice dystrophy type I. In the past few years, striking advances have been made in understanding the genetics of combined granular-lattice dystrophy. The most recent of these was published just 2 months before the 1997 meeting of the American Ophthalmological Society, and establishes a common molecular origin for granular dystrophy, lattice dystrophy type I, Avellino dystrophy, and Reis-Bücklers dystrophy.

Histopathologic observations on human eyes following neodymium: YAG laser cyclophotocoagulation for glaucoma.

PURPOSE: Although Nd:YAG laser cyclophotocoagulation has been extensively used for nearly a decade in treatment of severe glaucoma, there have been remarkably few reports (each of them extremely brief) of histopathological examination of glancomatous human eyes that had been so treated. We undertook this study to provide further details regarding the nature of the lesions produced in this type of ciliary ablation. METHODS: We chose three representative cases in which a glaucomatous human eye had been subjected to Nd: YAG cyclophotocoagulation, and was subsequently enucleated. To better understand the temporal evolution of the lesions, we selected eyes that were enucleated 1 day, 20 days, and 3 months, respectively, after they had been treated with noncontact Nd: YAG laser cyclophotocoagulation. RESULTS AND CONCLUSIONS: (1) energy levels ranging from 4.4 Joules to 5.6 Joules were effective in producing appropriate lesions; (2) direction of the laser beam 1 to 1.5 mm behind the limbus caused severe destructive lesions of the pars plicata: (3) toward the periphery of the individual treatment sites, the stroma and ciliary muscle continued to exhibit severe degeneration, as did the epithelium lining the valleys between the crests of the ciliary processes; but in those peripheral zones of individual treatment sites, the epithelium lining the crests of the ciliary processes survived and appears normal; (4) bleb-like separations of the ciliary epithelium from the adjacent stroma, particularly along the posterior aspect of the ciliary body lesions, are a prominent early feature of Nd:YAG cyclophotocoagulation; (5) the pigmented epithelium is more vulnerable to laser energy than is the nonpigmented epithelium of the ciliary body; (6) the destruction of the ciliary epithelium is permanent; (7) deeply pigmented persons have more melanocytes in the ciliary body muscle and stroma than do more lightly pigmented individuals, a circumstance that renders the tissues more vulnerable to laser energy; (8) the ciliary muscle was always severely damaged; (9) no scleral injury was observed other than evanescent, focal areas of edema of the deep sclera; and (10) except in the episclera, inflammatory cells were strikingly few in number, a circumstance providing support for the clinical observation that eyes treated with laser cyclophotocoagulation exhibit less of an inflammatory response than do those treated with cyclocryotherapy.

Immunotherapy with dinitrochlorobenzene (DNCB) for recurrent squamous cell tumor of conjunctiva.

Over the course of nine years a young man sustained repeated recurrences of a squamous papillary epithelial tumor of the conjunctiva and canaliculus. Treatment had included repeated surgical excisions, fulguration, cryotherapy, and localized applications of Thiotepa. When we examined him in 1972 he had a recurrent lesion measuring about 1 cm in diameter. Because of past failures with the above-noted therapeutic modalities, we decided to treat him with immunotherapy using the immunological adjuvant DNCB (dinitrochlorobenzene). We sensitized him systemically to DNCB by applying 2,000 microng of this agent to the skin of his forearm. A spontaneous "flare" reaction, indicative of delayed hypersensitivity, developed. Delayed hypersensitivity to DNCB was confirmed by in vitro testing of his lymphocytes for their ability to produce migration inhibitory factor (MIF) in response to DNCB. Subsequent localized application of DNCB to the conjunctival tumor resulted in rapid regression of the lesion and there has been no recurrence during a follow-up period of three years. We believe this is the first time a conjunctival tumor has been successfully treated with immunotherapy.