Alan H. Friedman

Ultrastructure and Clinicopathologic Correlation of Idiopathic Preretinal Macular Fibrosis

Detailed light and electron microscopic examination of early idiopathic preretinal fibrosis lesions showed that the fibrosis consisted of a layer of glial cells on the retinal surface. Two cases supported the clinical observation that a glinting retinal reflex preceded the development of retinal folds and traction lines. In both cases there was a break in the inner limiting lamina through which glial cells migrated and, presumably, proliferated on the retinal surface. There was no evidence for vitreoretinal adhesions. The ultrastructural similarity of the lesion to preretinal glial membranes seen in other disease entities suggested the possibility of a common biochemical stimulus for glial cell migration and proliferation on the retinal surface.

Drusen of the optic disc

Although optic disc drusen have been of interest to ophthalmologists for more than 100 years, their etiology and many aspects of their relationship to other clinical conditions remain obscure. They have been clinically observed in 0.3% of the population, although autopsy studies suggest an incidence of 2%. Drusen are seldom accompanied by visual symptoms; however, decreased vision and visual field changes may occur. The historical and epidemiological aspects of drusen are reviewed; clinical and histological features, as well as special diagnostic techniques (ultrasonography and fluorescein angiography) are discussed in detail.

Ocular manifestations of multiple myeloma, Waldenström's macroglobulinemia and benign monoclonal gammopathy

Multiple myeloma, Waldenströms macroglobulinemia and benign monoclonal gammopathy are examples of diseases in which there is an uncontrolled proliferation of cells involved in antibody synthesis. Myeloma and macroglobulinemia are considered malignant diseases, whereas monoclonal gammopathy is benign or possibly a pre-myelomatous condition. Following a brief review of the immunoglobulins the various clinical manifestations of these disorders are described. The ophthalmic signs of these disorders can be seen in every ocular structure.

Proton-Beam Irradiated Epithelioid Cell Melanoma of the Ciliary Body

A malignant ciliary body melanoma received proton-beam irradiation. After an apparent failure of the tumor to respond, the eye was enucleated. A predominantly epithelioid cell tumor appeared viable by light microscopy, and a low degree of mitotic activity persisted, despite therapy. The tumor cells, however, displayed degenerative changes ultrastructurally, presumably results of the radiotherapy. These consisted of numerous cytoskeletal filaments, lipid vacuoles, prominent phagolysosomes, and nuclear convolutions and fragmentations. The mitochondria were fewer in number in the present tumor than typically encountered in epithelioid cells. A rare leptomeric structure was discovered, probably an organizational modification of the cytoplasmic filaments. The tumors capillaries showed radiation-induced changes in terms of thickened basement membranes and perivascular fibrin deposition. The foregoing features are indicative of cellular and metabolic injury from the radiotherapy, but these were evidently not sufficiently injurious to sterilize the tumor.

Sugiura’s Sign: Perilimbal Vitiligo in the Vogt-Koyanagi-Harada Syndrome

Abstract We present four clinical cases and one histopathological specimen from patients who had the Vogt-Koyanagi-Harada (VKH) syndrome. Ophthalmological examination of these patients demonstrated perilimbal vitiligo, as initially described by Sugiura, which developed during the course of their disease. The presence of this sign should alert the ophthalmologist and suggest the diagnosis of VKH syndrome.

Squamous cell carcinoma of the conjunctiva. Value of exfoliative cytology in diagnosis

Cytologic examination is of potential value to the practicing ophthalmologist in confirming the clinical impression of cancer of the eye. Scrapings of conjunctival lesions can be prepared in the office, stained with Giemsa stain and rapidly examined. Carcinoma in situ and squamous cell carcinoma of the bulbar conjunctiva were presented as illustrations of the usefulness of exfoliative cytology in clinical ophthalmology.

Choroidal (Subretinal) Neovascularization Secondary to Choroidal Nevus and Successful Treatment with Argon Laser Photocoagulation

Retinal detachment secondary to choroidal nevus may be caused by subretinal fluid accumulation or neovascularization. Foveal subretinal fluid or leakage of choroidal neovascularization may impair visual acuity. 10 cases of nevus with serous sensory retinal detachment successfully treated with laser photocoagulation are reviewed. 2 additional cases of successful laser therapy for retinal detachment secondary to choroidal neovascularization with nevus of the choroid are presented.

Lymph node sampling in patients with epithelial ovarian carcinoma

Lymph node sampling is part of the FIGO staging of patients with ovarian carcinoma and is usually part of a meticulous second look operation. We analyzed the primary lymph node status of patients and compared this to the lymph node status at second look operation. From 3/86-3/91, 97 patients with epithelial ovarian tumors were treated at this institution. Seventy-one of the 97 patients (73.2%) had lymph node sampling at primary surgery. Thirty of the 71 patients had positive lymph nodes (42.2%) and 41 patients were lymph node negative (57.8%). Of the initial 97 patients, 58 were eligible for second look operation (59.8%), and 48 of these patients had lymph nodes sampled at second look operation. Nine of the 48 patients had positive lymph nodes (18.7%) and 39 had negative lymph nodes at second look operation (81.3%). Of the patients with negative lymph nodes at primary surgery, 25 patients had second look operation and 24 of these patients had lymph node sampling at second look operation. All patients with negative lymph nodes at primary surgery had negative lymph nodes at second look operation. Of the 30 patients with positive lymph nodes at primary surgery, 12 underwent second look operation. Four patients had persistent positive lymph nodes and 8 patients had negative lymph nodes. Our data suggest that patients with negative lymph nodes at primary surgery are unlikely to have positive lymph nodes at second look operation. Therefore, we believe that lymph node sampling under these circumstances is unnecessary.

Tamsulosin and the Intraoperative Floppy Iris Syndrome

THE INTRAOPERATIVE FLOPPY IRIS SYNDROME (IFIS) was first described by Chang and Campbell in 2005. These authors and others observed that there was a tendency for poor pupillary dilation and the intraoperative triad of the billowing of a flaccid iris, the propensity for iris prolapse, and progressive intraoperative pupillary constriction. In the current technique ophthalmologists perform cataract surgery through a 2.5-mm incision. A widely dilated pupil is essential for complication-free surgery. Billowing of the iris into the surgical field and poor pupillary constriction are potentially catastrophic barriers to successful surgery. Intraoperative floppy iris syndrome is encountered mainly in cataract surgery and in prospective studies has been found to occur in 2% to 3% of all cataract operations and to a lesser extent in glaucoma surgery. The strong association of IFIS with systemic (oral) administration of the 1aselective adrenergic antagonist tamsulosin, the commonly prescribed medication for treatment of benign prostatic hyperplasia, has been noted. In a prospective study involving 167 cataract operations, nearly 90% of the eyes of patients taking tamsulosin were diagnosed with IFIS. Indeed, all drugs of this class of 1antagonists are associated with the development of IFIS, although drugs with an affinity for 1a-receptors (tamsulosin and silodosin) are more commonly associated with IFIS. 1-Receptors are present in the dilator muscle and in the smooth muscle of the arteriolar wall in the iris. Intraoperative floppy iris syndrome has also been associated with the administration of finasteride and duasteride and saw palmetto. Women are not immune to the syndrome because tamsulosin is used in the treatment of obstruction due to renal calculi. The IFIS is not associated with iris color, diabetes mellitus, or pseudoexfoliation. Successful full dilation of the pupil is proportional to the amount of pigment present (eg, blue irides will dilate more rapidly and more fully than heavily pigmented brown irides). However, IFIS is associated with increased intraoperative risk and complications, such as iris prolapse, pupillary miosis, iris trauma, iris aspiration, iridodialysis, hyphema, posterior lens capsular rupture, and vitreous loss. Posterior capsular rupture may lead to dislocation of lens fragments into the vitreous body and vitreous loss, setting the stage for secondary complications such as retinal detachment and severe postoperative inflammation (phacoanaphylactic endophthalmitis). These untoward events are associated with the necessity of performing additional surgical procedures in the early postoperative period. Until now, however, those in the ophthalmologic community have not had good evidence about how great the risk of tamsulosinassociated IFIS might be and whether the risk is modifiable through cessation of the drugs. In this issue of JAMA, Bell and colleagues report the results of a nested case-control analysis of a populationbased retrospective cohort study using linked health care databases from Ontario, Canada. Among men aged 66 years or older who had cataract surgery between 2002 and 2007, 3550 patients (3.7%) had recent exposure to tamsulosin and 7426 (7.7%) had recent exposure to other -blockers. Serious ophthalmologic adverse events occurred in 284 patients (0.3%) and were significantly more common among patients with recent tamsulosin exposure (adjusted odds ratio, 2.33; 95% confidence interval, 1.22-4.43). These findings regarding the serious consequences of tamsulosin-related IFIS in the 14-day period following cataract surgery are most certainly the consequence of posterior capsular rupture, loss of lens fragments into the vitreous body, and vitreous loss. Retinal detachment is a potential consequence of these events and one of the more serious complications of IFIS. The presence of retained lens material in the vitreous body is a strong irritant and may lead to phacoanaphylactic endophthalmitis. It is not surprising that cytological study of vitreous material removed at the time of surgery for retinal detachment or retained lens material or both is invariably associated with the preoperative administration of tamsulosin and an attendant IFIS at the time of cataract surgery. The presence of 1-adrenoreceptor subtypes in the iris dilator muscle has been previously demonstrated. Through careful studies of these receptors, our group has proposed

Localized Conjunctival Amyloidosis: Case Reports and Review of Literature

Two men in their 20s had flat, stable, pink, well-vascularized conjunctival lesions found unexpectedly which on biopsy proved to be primary localized amyloidosis. The diagnosis was made possible by the absence of systemic manifestations or antecedent ocular disease, the presence of diffuse homogeneous connective tissue deposits and characteristic histochemical staining properties. The cases were remarkable for their color, texture, location, and presentation and suggest that a high index of suspicion, if not an alacrity to biopsy, is needed for the diagnosis of primary localized amyloid to be made.