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Biomedical subjects

S Brownstein

Publications and source records attributed to S Brownstein.

At least 91 records · Page 5Linked to original sources

Congenital hereditary (juvenile X-linked) retinoschisis. Histopathologic and ultrastructural findings in three eyes.

One surgically enucleated and two postmortem eyes were obtained from two related men with congenital hereditary retinoschisis. A periodic acid-Schiff-positive amorphous material was identified within the retina adjacent to the schisis cavities. Ultrastructural examination of this material showed numerous extracellular filaments, measuring approximately 11 nm in diameter. Similar filaments were found in the vitreous in all three globes and in a normal globe. We believe that the intraretinal filaments are produced by defective Müller cells and that their extracellular accumulation may lead to degeneration of cells and subsequent schisis formation.

Adolescent↗

Sebaceous carcinoma of the eyelid masquerading as superior limbic keratoconjunctivitis.

A 49-year-old woman had been treated for 18 months for superior limbic keratoconjunctivitis of the right eye. Pathologic examination of a biopsy specimen of the superior perilimbal conjunctiva disclosed intraepithelial carcinoma of sebaceous gland origin. Because of the extensive involvement of the conjunctival epithelium by tumor, a subtotal orbital exenteration was performed. To our knowledge, this is the first report of sebaceous carcinoma masquerading as superior limbic keratoconjunctivitis.

Adenocarcinoma↗

Lacrimal gland choristoma of the ciliary body.

A 22-month-old girl was found to have a pigmented mass of the ciliary body of the right eye. Five months later the globe was enucleated because of enlargement of the tumor and the appearance of brown pigment on the adjacent epibulbar surface. Histopathologic examination disclosed a lacrimal gland choristoma of the ciliary body with involvement of the limbal conjunctival surface and superficial sclera. Choristomas of the lacrimal gland are rare and only eight cases have been reported previously to occur intraocularly. Most of these cases showed extrascleral involvement which suggests that the anlage for these choristomas may be entrapped intraocularly during the development of the globe. We recommend conservative management, either by observation or local resection if possible.

Child, Preschool↗

Trilateral retinoblastoma. Report of two cases.

Two children had bilateral retinoblastoma and a morphologically similar intracranial neoplasm localized to the region of the pineal gland as shown on computed tomography and at postmortem examination. The first child's intracranial malignant neoplasm produced symptoms that indicated its probable presence when the ocular tumors were first diagnosed. With the second child, there was a latent period of more than three years between the diagnosis and initial management of the ocular neoplasms and the onset of symptoms from the pineal tumor. We diagnosed both cases as trilateral retinoblastoma, which in our experience is a relatively frequent cause of mortality in patients with heritable retinoblastoma.

Brain Neoplasms↗

Lacrimal drainage system inflammatory masses from retained silicone tubing.

In two cases studied we found inflammatory masses of the lacrimal system associated with retained silicone material. We saw the first patient two years after a failed dacryocystorhinostomy. A mass of the superior punctum mimicking a lacrimal sac or canalicular neoplasm consisted of chronic granulomatous inflammation centered around retained silicone tubing. In the second patient, a foreign body giant cell inflammatory reaction occurred in the lacrimal sac in which there were retained knots of silicone tubing. We believed the inflammation in these patients to be caused by the knots and the cut ends of the silicone tubing present in the lacrimal drainage system.

Aged↗

Orbital dacryops.

Two patients presented with an enlarging orbital mass that proved to be simple dacryops, a condition in which a cyst arises in the palpebral lobe of the lacrimal gland. The cyst in one patient was lined by one to two layers of relatively flat epithelial cells favoring a lacrimal gland ductal origin. In the second patient, the cyst was lined by a nonkeratinized stratified squamous epithelium with numerous goblet cells consistent with a conjunctival origin.

Adult↗

Nonteratoid medulloepithelioma of the ciliary body.

A 34-month-old girl was treated for anterior uveitis, a superiorly subluxated cataract, rubeosis iridis and secondary glaucoma of the right eye of 3 months duration. She was diagnosed as having medulloepithelioma only after a mass was subsequently identified on the anterior surface of the iris. A low grade malignant nonteratoid medulloepithelioma was found to involve the ciliary surface throughout the circumference of the enucleated right globe. These tumors are generally associated with a good prognosis for survival, but are potentially lethal when they extend extrasclerally.

Cataract↗

Keratoconus and Leber's congenital amaurosis: a clinicopathological correlation.

A 42-year-old man with Leber's congenital amaurosis, cataracts and keratoconus died following abdominal surgery. Postmortem pathological examination of the globes disclosed cone-shaped corneas with unusual central subepithelial scars and a retinopathy consistent with retinitis pigmentosa. A review of the family history revealed two siblings with congenital blindness.

Adult↗

Bilateral blindness in temporal arteritis with skip areas.

A 73-year-old woman received systemic corticosteroids for suspected temporal arteritis after blindness developed in the right eye during the previous day. Because a right temporal artery biopsy specimen was reported as normal, a diagnosis of arteriosclerotic ischemic optic neuropathy was made and the corticosteroid therapy was discontinued. Two weeks later the patient rapidly lost vision in her left eye. A left temporal artery biopsy specimen showed granulomatous arteritis adjacent to normal artery (skip area). Deeper sections of the original right temporal artery biopsy specimen also demonstrated a small focus of granulomatous arteritis adjacent to normal artery. In patients with suspected temporal arteritis, numerous step sections of a long segment of temporal artery should be examined; if the results are normal, the contralateral temporal artery should be studied. A nonspecific inflammatory reaction within or adjacent to the artery should alert the pathologist to the possible presence of a nearby focus of granulomatous arteritis.

Aged↗

Ocular Candida with pale-centered hemorrhages.

A 54-year-old man with severe thermal burns had Candida corneal ulcers and Candida septicemia develop, and he died 39 days after admission to the hospital. At autopsy examination, fungi were noted in the cornea to extend across the intact Descemet's membrane and were also found within foci of retinitis and choroiditis. Also, hemorrhages (some of which had pale centers) were present in both retinas. Histopathologic examination of these hemorrhages disclosed that the pale centers were comprised of a disrupted small blood vessel surrounded by an acellular material that most likely represented fibrin-platelet aggregates.

Candida↗

Ocular neurofibromatosis.

A 4 1/2-month-old girl with unilateral congenital glaucoma of the left eye subsequently underwent five surgical procedures that proved to be unsuccessful in controlling the intraocular pressure. Neurofibromatosis was diagnosed at the age of 5 1/2 years when she was found to have a plexiform neuroma of the left eyelids and multiple café au lait spots including in the left temple. Two years later the buphthalmic left globe was enucleated. Histopathologic findings included a diffuse neurofibroma of the ciliary body and choroid, peripheral anterior synechiae, total angle closure, and endothelialization of the iris, all of which probably contributed to the glaucoma. Other interesting features were melanocytic hamartomas of the iris, an anterior subcapsular cataract and ovoid bodies in the diffuse neurofibroma of the choroid.

Child, Preschool↗

Extraocular extension of malignant melanoma of the uvea.

Two patients, each with a large malignant melanoma of the choroid, underwent enucleation of the affected eye. Gross examination of each globe in the pathology laboratory disclosed a substantial area of extrascleral extension of the intraocular neoplasm, although this had not been noted at the time of enucleation. Since subsequent management and the prognosis for survival may be altered by recognition of extrascleral extension, the external surface of all globes that may harbour a malignant neoplasm should be carefully inspected by the surgeon during and immediately after enucleation.

Choroid Neoplasms↗

Cryotherapy for precancerous melanosis (atypical melanocytic hyperplasia) of the conjunctiva.

Two patients with progressive, diffuse precancerous melanosis (intraepithelial atypical melanocytic hyperplasia or melanocytic dysplasia) of the conjunctiva were treated with cryotherapy. In one patient, the contiguous cutaneous portions of both eyelids were involved and received the same therapy; the extensive disease of the patient was managed by two cryotherapy sessions during a period of 21/2 months. The melanocytic process in both patients showed a good response to this therapy, which resulted in a slough of the superficial conjunctiva with reepithelialization from adjacent areas. Neither patients has shown recurrent disease in more than seven months of follow-up, although one patient had incomplete regression of the melanocytic condition in the perilimbal region, which was subsequently treated by an excisional biopsy. Serious complications occurred in one patient who had corneal and lenticular opacities; this patient had severe extensive disease for which exenteration was considered the only feasible alternative therapy to control the melanocytic proliferation disorder adequately. Less severe complications also developed, including trichiasis, ptosis, transient hypotony, and macular edema. In this article, we discuss the clinical and histologic features of acquired pigmentations of the conjunctiva, the criteria for their management, and the approaches to minimize the complications that we have encountered with cryotherapy.

Aged↗

Pseudomonas aeruginosa scleritis.

In two patients Pseudomonas aeruginosa scleral infection led to ocular perforation. In one patient, a scleral abscess was identified anteriorly. A scleral perforation occurred at a more posterior focus, but the eye was salvaged with minimal residual visual function. In the other patient, perforation at the corneoscleral limbus occurred after initial corneal improvement with antibiotic therapy; histopathologic examination of the enucleated globe disclosed an abscess extending from the corneoscleral limbus to the equator superiorly.

Aged↗

Cryptophthalmos syndrome with bilateral renal agenesis.

We conducted a postmortem study of an infant with the abortive form of the cryptophthalmos syndrome. He had syndactyly and anal atresia and also showed features of Potter's (renal nonfunction) syndrome, including bilateral renal agenesis, pulmonary hypoplasia, and the typical facies. Histopathologic examination of both globes disclosed that the superior eyelid folds were partially replaced by skin that fused with the superior third of each cornea; there were also anomalies of the superior canaliculi and scarring of the corneas. We found no specific abnormality of the intraocular contents.

Abnormalities, Multiple↗