Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).

PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. METHODS: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. Ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.

Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings.

PURPOSE AND METHODS: To describe the clinical and histopathologic features of a patient with viral retinitis secondary to subacute sclerosing panencephalitis. RESULTS: The patient was a human immunodeficiency virus-negative intravenous drug abuser with an acute retinitis that later progressed to encephalitis despite aggressive treatment for possible viral, protozoal, bacterial, and rickettsial infections. The patient had many of the characteristic findings of subacute sclerosing panencephalitis, including a history of measles in early childhood, myoclonus, periodic complexes on electroencephalographic testing, persistently elevated serum and cerebrospinal fluid antimeasles immunoglobulin G (IgG) titers, and a cerebrospinal fluid oligoclonal IgG gammopathy. Ultrastructural examination demonstrated numerous filamentous microtubular intranuclear viral inclusions in the nuclear layers of the retina consistent with the measles virus. This case is unusual in that our patient developed subacute sclerosing panencephalitis later in life and because there was an 8-year period between presumed viral infections in the two eyes. CONCLUSIONS: An acute retinitis in an intravenous drug abuser is not always caused by human immunodeficiency virus-related infections; not all viral retinitis responds to therapy; and mortality as well as the usual morbidity may be associated with viral retinitis. One might consider the diagnosis of subacute sclerosing panencephalitis in a young person with an acute retinitis with little or no vitreal inflammation and lack of response to anticytomegalovirus and antitoxoplasmosis therapy.

Epiretinal macular membranes in eyes with attached posterior cortical vitreous.

BACKGROUND: The authors define the clinical and anatomic characteristics of patients who had epiretinal membrane formation and traction over the macula in the absence of vitreous detachment from the retina. METHODS: Patients were treated and followed by one surgeon (JCF). All patients had epiretinal membrane formation over the macula without vitreous detachment. The clinical and surgical findings of these patients were compared along with histopathologic examination of the removed epiretinal membranes and posterior cortical vitreous. RESULTS: Fourteen patients were identified over a 9.75-year period. All patients had a thin and transparent-or at most minimally translucent-membrane overlying the posterior pole causing traction. The membrane appeared continuous with the posterior hyaloid face. At vitrectomy, no vitreous detachment was found in any eye. Transmission electron microscopy of all three specimens tested found collagen of less than 16 nanometers in diameter, consistent with native vitreous collagen. After surgery, visual acuity improved in 13 of 14 eyes, was 20/50 or better in 12 eyes, and was 20/25 or better in 7 eyes. CONCLUSION: Epiretinal membrane formation can occur in eyes without vitreous detachment probably because of cellular growth along the posterior hyaloid face. Such eyes respond particularly well to surgery.

Argon laser treatment of trichiasis: a clinical and histopathologic evaluation.

Trichiasis is a common condition of multiple etiologies. Cryosurgery has been advocated as an effective means of treatment but may be associated with serious complications, including scar, skin depigmentation, and lid notching. We treated eight consecutive eyelids with the argon laser. At the end of a 3-month follow-up period, no recurrence was noted. The only observed complication was mild eyelid notching in one eyelid. Histopathologic evaluation of two specimens obtained 10 days and 5 weeks after laser treatment confirmed the highly localized coagulative effect of the laser with minimum destruction of adjacent tissues. Orientation of the eyelid so that the laser beam and the lash follicle are coaxial and careful measurement of the depth of vaporization of the lash follicle are important in confirming complete treatment and minimizing recurrence.

Non-caseating conjunctival granulomas in patients with multifocal choroiditis and panuveitis.

PURPOSE: This study describes a group of patients with clinical findings of peripheral punched-out lesions and panuveitis who had non-caseating granulomas found by non-directed conjunctival biopsy. METHODS: A series of 10 patients seen at The University of Iowa Hospitals and Clinics between August 1989 and August 1990 with ocular findings similar to those of multifocal choroiditis with panuveitis, including peripheral punched-out chorioretinal lesions, vitritis, and frequently, cystoid macular edema, was examined. All patients underwent ophthalmic examination, ancillary testing, and non-directed conjunctival biopsy. RESULTS: Of the ten patients, nine were women. Results of FTA-ABS were negative for all patients. A non-directed conjunctival biopsy disclosed non-caseating granulomata in seven of the patients. Acid-fast bacilli were not detected in any specimens. Four of the seven patients with positive biopsy results had either elevated serum angiotensin-converting enzyme levels or chest x-rays consistent with sarcoid. Six of these seven patients were 58 years of age or older. CONCLUSION: It is recommended that non-directed conjunctival biopsy specimens be examined to exclude the presence of non-caseating granulomata in uveitis patients with small, inferior, peripheral, punched-out chorioretinal scars, especially in women older than 55 years of age.

Preretinal and optic nerve head neovascularization induced by photodynamic venous thrombosis in domestic pigs.

OBJECTIVE: To develop a reliable model of ischemic preretinal and optic nerve head neovascularization in young male domestic pigs. METHODS: Using laser-induced branch retinal venous obstruction assisted by photodynamic thrombosis with rose bengal, 15 of 15 treated eyes developed neovascularization of the optic nerve head and retina. All cases of neovascularization were followed up clinically for up to 12 weeks. RESULTS: Clinical, fluorescein angiographic, and histopathologic features were consistent with neovascular proliferation into the vitreous. CONCLUSIONS: Further evaluation of the long-term natural history, role of the degree of ischemia, and the effect of interventions for various types of ischemic retinopathy (eg, panretinal photocoagulation) is warranted. This model is simple and relatively inexpensive and allows easy clinical assessment.

Clinicopathologic spectrum of primary uveal melanocytic lesions in an animal model.

BACKGROUND: Currently, there are no animal models of primary uveal melanoma in an eye large enough to allow documentation of the clinical evolution of the lesion by either funduscopy or fundus photography. METHODS: The authors induced primary uveal melanocytic lesions in the eyes of Dutch (pigmented) rabbits using a two-stage carcinogenesis protocol involving initiation with 4 weekly topical applications of 10 microliters of a 1% solution of 7,12-dimethyl-benz[a]anthracene (DMBA) in acetone (21 eyes) followed by 12 weekly topical applications of a 10 microliters solution of either 0.25% or 0.5% croton oil in acetone. They also investigated the effect of initiation with DMBA without promotion and the effects of chronic topical exposure to acetone and proparacaine. RESULTS: Exposure to DMBA followed by promotion with croton oil in either concentration was the most effective means of inducing clinically detectable fundus lesions. Histologically, a spectrum of melanocytic proliferations developed including benign nevi, nevi with varying grades of cytologic atypia, and clusters of confluent atypical melanocytes that may represent early melanomas. Although clinical regression of fundus lesions was noted in eight eyes after promotion had been stopped, five of these eyes showed unequivocal histologic evidence of a residual uveal melanocytic lesion. Chronic ocular irritation is capable of inducing cytologically benign subclinical uveal melanocytic proliferations. CONCLUSIONS: The conventional classification of human uveal melanocytic lesions includes only nevi and melanomas, but a comparison of the results of this study with descriptions of human uveal melanocytic nevi suggests the existence of a spectrum of intermediate atypical precursor lesions in humans.

Paraneoplastic pemphigus, cicatricial conjunctivitis, and acanthosis nigricans with pachydermatoglyphy in a patient with bronchogenic squamous cell carcinoma.

A 77-year-old man with chronic conjunctivitis, acanthosis nigricans with pachydermatoglyphy, and pemphigus-like mucocutaneous lesions was found to have a well-differentiated bronchogenic squamous cell carcinoma. Histopathologic and immunofluorescence studies confirmed the diagnosis of paraneoplastic pemphigus. Skin lesions resolved with oral prednisone and azathioprine therapy, but the conjunctivitis and mucous membrane erosions persisted. The conjunctiva later became scarred with foreshortening of the fornices and development of symblepharon. External beam irradiation arrested the growth of the tumor but did not have any effect on the diseased conjunctiva and other mucous membranes. This case demonstrates that cicatrizing conjunctivitis with bullous mucocutaneous lesions may be a clinical sign associated with an occult neoplasm.

Vitreoretinal traction and perimacular retinal folds in the eyes of deliberately traumatized children.

The pathophysiology of perimacular folds in eyes of deliberately traumatized children is disputed. The authors reviewed the clinical and forensic records and systemic and ocular findings at autopsy of three children with perimacular retinal folds who died after being violently shaken. Two of the children suffered direct head trauma in addition to being shaken; one patient was violently shaken without any physical or forensic evidence of direct head trauma. No direct ocular trauma was detected. In each case, the vitreous had partially separated from the retina but remained attached to the internal limiting membrane at the apices of the folds and the vitreous base, implicating traction in the pathogenesis of these folds. Although some intraocular findings in deliberately traumatized children may be explained by direct head injury, the possibility of both direct head trauma and shaking must be considered. Perimacular folds may develop without direct ocular or head trauma and may constitute evidence supporting violent shaking.

Olfactory neuroblastoma metastatic to the eye.

A case of olfactory neuroblastoma metastatic to the choroid is reported and described. Histologically, the tumor consisted of islands of small round cells, many of them surrounding blood vessels in a 'pseudorosette' pattern. Numerous mitotic figures were present. Transmission electron microscopy reveals neuritic processes containing neurosecretory granules and microtubules, features characteristic of olfactory neuroblastoma. This is the first published case of an olfactory neuroblastoma demonstrating intraocular metastasis.

Retinal vascular endothelial cells and pericytes. Differential growth characteristics in vitro.

Long-term cultures of retinal capillary endothelial cells (RCEC) and of pericytes were grown from collagenase-treated calf retinal vessels. By the use of mechanical separation and differential growth response in various media, pure cell lines were derived from cloned capillary cells as well as from multiple vessel fragments. RCEC and pericytes appeared different under phase contrast microscopy. In addition, RCEC produced factor VIII antigen and angiotensin-converting enzyme, and pericytes did not. RCEC preferred to grow in tumor-conditioned medium, whereas pericytes preferred a standard tissue culture medium supplemented with calf serum. Extracellular matrix, together with tubular structures, developed in postconfluent RCEC but not in pericytes. This study demonstrates that it is possible to grow long-term, pure cultures of RCEC and of pericytes that possess distinctive morphology, growth, and synthetic capabilities in vitro.

Retinal vessels: proliferation of endothelium in vitro.

Tissue culture of retinal vessels from fetal calf eyes produced colonies of endothelium, pericytes, and smooth muscle. Identification of endothelial cells was based upon culture morphology, [3H]thymidine labeling of isolated vessels, and factor VIII immunofluorescence. Thimerosal added to the culture medium destroyed pericytes and muscle cells, leaving only endothelial cell colonies in the primary cultures. This tissue culture system may be useful in the study of retinal vascular cell biochemistry and pathophysiology.