Edward L. Howes

Demonstration of Herpes Group Virus in Acute Retinal Necrosis Syndrome

Tissue for pathologic examination was obtained from three cases of acute retinal necrosis syndrome. Virus particles belonging to the herpesvirus family were demonstrated in retinal biopsies from two patients, one of whom was immunosuppressed. Despite removal of large biopsy specimens, the retina has remained attached for 20 months postoperatively in one case and for three months in the other. In a third patient with acquired immune deficiency syndrome, the clinical course and postmortem immunopathology were suggestive of a herpes simplex virus infection, initially affecting the retina and subsequently the optic nerves, chiasm, tracts, and central nervous system. These cases illustrate that the virus associated with the acute retinal necrosis syndrome is easily demonstrable using vitrectomy and endoretinal biopsy in the acute phase of the disease, but may be difficult to demonstrate in chronically detached atrophic retinas.

Endotoxin-induced uveitis in the rat: observations on altered vascular permeability, clinical findings, and histology.

A single intraperitoneal dose of endotoxin (lipopolysaccharide or LPS) induces an acute inflammatory response in the uveal tract of rats. This inflammation is characterized by a breakdown of the blood/aqueous barrier within 3 hr after the LPS and the subsequent development of clinical disease and a cellular infiltrate. Early change in vascular permeability, clinical, and pathological changes were dose dependent with the two highest doses (100 micrograms or 500 micrograms) producing more severe pathology. Clinical and histopathologic abnormalities peaked at 24 hr and were resolving by 48 hr. Although clinical and histologic changes correlated well, the degree of breakdown of the blood/aqueous barrier at 3 hr failed to predict the extent of the cellular exudate measured by either clinical or histologic criteria. In addition, pharmacologic suppression of the early vascular permeability changes with indomethacin, cyproheptadine, or both agents failed to protect the animals consistently from subsequently developing significant clinical disease or cellular infiltrates on histopathology. LPS-induced uveitis in the rat provides a simple, reproducible model for ocular inflammation without requiring direct eye manipulation. The mediators responsible for the early vascular permeability in this model appear to be distinct from the mediators primarily responsible for the subsequent cellular exudate.

Ocular manifestations of leukemia: leukemic infiltration versus infectious process

OBJECTIVE To determine whether specific guidelines can be developed to distinguish whether retinal infiltration in leukemia patients represents infection or neoplasia. DESIGN Retrospective noncomparative interventional case series. PARTICIPANTS Six patients recently seen at University of California San Francisco with retinal infiltrates in a setting of leukemia, for which adequate written and photographic information of disease course was available. INTERVENTION Observation consisted of retrospective review of clinic charts, hospital medical records, and fundus photographs. MAIN OUTCOME MEASURES Determination of whether retinal infiltrates represented neoplasia or infection was made by review of medical records. RESULTS In this series, neoplastic retinal infiltrates were found in patients who had newly diagnosed leukemia and those who were in blast crisis. In contrast, the two patients who were in complete remission, but had undergone bone marrow transplantation, had retinal infiltrates attributable to infection. CONCLUSIONS Every patient with retinal infiltrates in the setting of newly or previously diagnosed leukemia requires a systemic and central nervous system workup before the initiation of ophthalmologic treatment. The systemic status of the patient is highly informative in determining whether infection or neoplasia is responsible for the infiltration.

Complex limbal choristomas in linear nevus sebaceous syndrome

OBJECTIVE This study aimed to describe the clinical and histopathologic findings in four patients with complex limbal choristomas associated with linear nevus sebaceous syndrome (LNSS), a rare disorder including nevus sebaceous, seizures, and mental retardation, and often accompanied by ocular anomalies. DESIGN Small observational case series. METHODS A retrospective review of the clinical and histopathologic records of four patients. RESULTS Each of four patients had complex limbal choristomas in the setting of clinical and histopathologic LNSS. The limbal choristomas were multiple in three patients and bilateral in two patients. Most choristomas involved the superotemporal limbus (6 of 10), although nasal (3 of 10) and inferior (1 of 10) limbal tumors also were present. Three patients had significant corneal astigmatism or involvement of the central cornea requiring surgical removal of their choristomas, one accompanied by a lamellar keratoplasty and another accompanied by two consecutive penetrating keratoplasties. Each graft eventually vascularized, reducing vision. One patients vision was limited by amblyopia and another by occipital cortical dysgenesis with visual impairment. Histopathologic examination of the excised choristomas showed foci of lacrimal gland (3 of 4 patients), adipose tissue (3 of 4), neural tissue (1 of 4), cartilage (1 of 4), lymphoid follicles (1 of 4), skin adnexal tissue (1 of 4), and smooth muscle (1 of 4). Other associated ocular findings included an eyelid mass (1 of 4), colobomas of the eyelid (3 of 4), colobomas of the choroid and retina (2 of 4), nonparalytic strabismus (2 of 4), scleral ectasia (1 of 4), partial oculomotor palsy with ptosis and anisocoria (1 of 4), microphthalmia (1 of 4), hypertelorism (1 of 4), and cortical visual impairment (1 of 4). CONCLUSIONS Complex limbal choristomas, although rare, can occur in the setting of LNSS and can be associated with multiple ocular and systemic abnormalities. Visual prognosis appears poor in most cases despite aggressive management.

Foreign-body giant cell reaction to liquid silicone

Although intravitreal injections of liquid silicone have long been used in complicated retinal detachments and long-term complications such as keratopathy, glaucoma, and cataract are well-known, the effect of liquid silicone on the retina, iris, and angle structures is not as well defined. Histopathologic study of one human eye that was enucleated 20 months after liquid silicone injection showed an intraocular foreign-body giant cell reaction.

Resection of intraocular squamous cell carcinoma.

A patient with recurrent squamous cell carcinoma of the conjunctiva was referred with 20/20 vision in an eye with obvious intraocular extension. A modified iridocyclochoroidectomy was performed and the tumour was removed. Three and a half years later the patients vision is 20/30 and there is no recurrence. This is the first case in which an eye has been successfully salvaged with documented intraocular squamous cell carcinoma of the conjunctiva.

Analysis of radiation failure after uveal melanoma brachytherapy

We analyzed the number of cycling cells in the DNA synthesis phase and in mitosis in two uveal melanomas unsuccessfully treated with iodine 125 brachytherapy. In one patient, the tumor appeared to have been radioresistant, whereas the other patient had a clinically inapparent area of tumor that was not included in the tumoricidal radiation field. In the first patient, there was uniform tumor cell cycling, whereas the second patient had cycling cells predominantly in the area that did not receive optimal radiation. This gradient of cycling tumor cells in the second patient was observed with both standard histologic examination (mitoses per high-powered field) and bromodeoxyuridine staining (DNA synthesis). The bromodeoxyuridine technique was significantly more sensitive than standard histologic examination in the detection of cycling cells.

Systemic non-Hodgkin's lymphoma with optic nerve infiltration in a patient with AIDS.

Purpose To report the clinicopathologic features of a patient with AIDS and clinically regressed systemic non-Hodgkin’s lymphoma who subsequently developed lymphomatous infiltration of the optic nerve and occlusion of the central retinal vein in both eyes. Methods The eyes of this patient were examined ophthalmologically and by fluorescein angiography. The eyes, brain, and body were obtained after death and studied by light microscopy. Results Ophthalmic examination and fluorescein angiography revealed optic nerve swelling and central retinal vein occlusion first in the left eye and shortly thereafter in the right eye. Postmortem histopathologic examination showed dense infiltration of both optic nerves by lymphoma as well as necrotizing vasculitis involving the retinal vessels near the left optic nerve head. Examination of the brain revealed lymphomatous involvement in the paraventricular region with associated necrosis and inflammation. No bone marrow recurrence or other residual systemic lymphoma was present. Conclusion This case demonstrates that infiltrative optic neuropathy may occur as the sole ocular manifestation of disease recurrence in a patient with systemic non-Hodgkin’s lymphoma otherwise thought to be in clinical remission.

Viridans group Streptococcus subretinal abscess.

Editor,—Subretinal abscess is an unusual entity, occurring primarily in immunocompromised individuals. Reported causative organisms include fungi1 2 and Gram negative rods.3 4 Here we report a subretinal infection resulting from the Gram positive organism viridans group Streptococcus in a pancytopenic patient. ### CASE REPORT A 56 year old man undergoing chemotherapy for IgG multiple myeloma presented with 3 days of fever. He denied injecting drug abuse. He had a temperature of 101.5°F, poor dentition, a quiet indwelling catheter site on the chest, and no cardiac murmur. He was pancytopenic with a haematocrit of 14.2% (normal 39–49%), white blood cell count of 0.2 × 109/l (normal 4.5–11.0), absolute neutrophil count of 0.1 × 109/l (normal 1.8–6.8), and platelet count of 12 × 109/l (normal 150–450). Chest x ray and urinalysis …

Correlation between Degree of Malignancy and the Radioactive Phosphorus Uptake Test in Ocular Melanomas

Twenty-nine patients who had undergone enucleation for ocular malignant melanoma were studied. The results of the radioactive phosphorus uptake (32P) tests were correlated with histopathologic observations. There was a positive correlation between the 32P test results and the histologic degree of malignancy. Mixed cell melanomas were more frequent in the patients ranked with higher 32P test results. Spindle cell melanomas occurred more frequently in patients with lower 32P test results. Mitoses per field and degree of malignancy correlated positively with 32P test results when ranked in a masked manner by two ocular pathologists. Tumor volume and clinical factors did not correlate with the 32P test results. While patients with mixed cell melanomas had higher mean 32P test results than those with spindle cell melanomas, a large variance was noted. Because of this the 32P test is unreliable in predicting the histopathology of a tumor in individual cases.