Rapid keratitis and perforation after corneal collagen cross-linking.

PURPOSE: To describe a case of rapid keratitis and corneal perforation after epithelium off collagen cross-linking. OBSERVATIONS: We report a case of a 17-year-old male who underwent collagen cross-linking with the protocol and device approved by the United States Food and Drug Administration (FDA) that developed a corneal infiltrate 3 days after the procedure. He later developed corneal thinning and perforation on day 5 requiring the use of cyanoacrylate glue and a Kontur lens. Despite initial improvement in the infiltrate with fortified antibiotics he later had leakage of aqueous around the glue and a flat chamber requiring an emergent penetrating keratoplasty on postoperative day 16. CONCLUSION AND IMPORTANCE: While collagen cross-linking has been very effective for treating keratoconus and is being recommended more frequently since FDA approval in the United States, severe complications such as corneal perforation requiring early transplant can still occur.

Management of ocular manifestations of autosomal recessive congenital ichthyosis 4B, harlequin type, in the perinatal period.

Autosomal recessive congenital ichthyosis (ARCI4B [OMIM #242500]), also known as harlequin ichthyosis, presents at birth with extreme hyperkeratosis and thick-fissured plaques, leading to tightness of the skin around the eyes, mouth, ears, chest, abdomen, and extremities. Ocular manifestations include cicatricial ectropion and exposure keratitis. We present 2 infants with ARCI4B and cicatricial ectropion who were managed with aggressive nonsurgical therapy. Both infants avoided severe ocular sequelae and maintained corneal clarity, highlighting that management of exposure keratopathy with frequent ophthalmic ointment application can prevent severe ocular surface pathology in ARCI4B.

Treatment Limitations With PROSE (Prosthetic Replacement of the Ocular Surface Ecosystem): One Centers Experience.

OBJECTIVES: The prosthetic replacement of the ocular surface ecosystem (PROSE) device is used to treat difficult-to-manage ocular surface disease (OSD) and ectasia. Previous studies have demonstrated positive treatment outcomes. This study aims to document treatment failures to better tailor treatment and address limitations with its use. METHODS: Retrospective chart review of consecutive PROSE fits performed at Northwell Health from 2012 to 2016. Reasons for patient discontinuation of treatment were documented, and potential risk factors for treatment failure were assessed. RESULTS: The total number of eyes treated was 125. Fifty five eyes had ectasia, 67 had OSD, and 3 had both ectasia and OSD. A total of 8/125 (6.4%) of eyes failed treatment; 6/8 (75%) of failed treatments had worsening corneal edema, all of which had presumed risk factors for lower endothelial cell counts. Two eyes discontinued use secondary to intractable debris on the device, blurring vision. The most common diseases in patients failing therapy in descending order included: keratoconus and Fuch's dystrophy, ectasia after penetrating keratoplasty, and graft versus host disease. Those with presumed risks factors for lower endothelial cell counts (Fuch's dystrophy and patients with previous penetrating keratoplasty) were more likely to fail (22.2%) compared with those without a risk of a low endothelial cell count (1.6%). CONCLUSION: Although PROSE use has high success, corneal edema secondary to endothelial dysfunction is a potential limitation that may lead to treatment failure. Patients with lower endothelial cell counts after penetrating keratoplasty or with Fuch's dystrophy were more likely to fail treatment.

T-cell lymphoblastic lymphoma involving the ocular adnexa: report of two cases and review of the current literature.

The majority of ocular adnexal lymphomas are B-cell in origin. We report two cases of T-cell lymphoblastic lymphoma (T-LBL) involving the ocular adnexa. One patient presented with a painless pink conjunctival lesion and inferior orbital fullness. The second patient presented with a painless orbital mass. The diagnoses were confirmed by histopathology and immunohistochemistry. Both patients had extensive multifocal lesions during staging. Prompt intensified chemotherapy regimens were initiated. T-LBL is an aggressive disease with poor prognosis. This report emphasizes the importance of timely diagnosis by the ophthalmologist with co-management and treatment with an oncologist.

A Case of Everolimus-Induced Eyelid Edema.

Everolimus is a newer generation mammalian target of rapamycin inhibitor approved for immunosuppressive use in a number of advanced medical conditions. The authors report a unique case of persistent eyelid edema believed to be related to the immunosuppressive therapy. The therapy was being well tolerated despite the persistent eyelid edema, so the patient underwent a bilateral upper eyelid blepharoplasty. The patient responded well with resolution of her eyelid dermatochalasis and markedly decreased edema. Everolimus-induced eyelid edema should be recognized by clinicians as a potential side effect of the medication. Surgical excision of excess eyelid tissue by blepharoplasty can be a successful way to manage this side effect.Everolimus (zortress) was initially approved as an immunosuppressive agent for renal transplantation patients. Approval for the treatment of subependymal giant cell astrocytomas associated with tuberous sclerosis, progressive metastatic pancreatic neuroendocrine tumors, human epidermal growth factor receptor 2 negative breast cancer in postmenopausal woman, liver transplantation patients, and well-differentiated neuroendocrine tumors of gastrointestinal or pulmonary origin has followed., Everolimus is a derivative of sirolimus (rapamune), and similar to sirolimus acts as an inhibitor of mammalian target of rapamycin. Few prior studies have reported eyelid edema from sirolomus. Many prior medications have been implicated in eyelid edema formation. To date, periocular edema has not been reported as a side effect of everolimus. We report a patient with bilateral upper eyelid edema associated with everolimus therapy requiring surgical intervention to ameliorate the significant skin redundancy and the visual field defect. This report complies with the Declaration of Helsinki and Health Insurance Portability and Accountability Act regulations.

The Association of Demodex with Chalazia: A Histopathologic Study of the Eyelid.

PURPOSE: To investigate the association of Demodex with chalazia and to describe the associated histopathologic changes in the eyelid tissue. METHODS: In a retrospective histopathologic case-control study, 78 ectropion/entropion repair eyelid wedge resections were examined. Serial sections were obtained on formalin-fixed, paraffin-embedded tissues, stained with hematoxylin-eosin and studied by light microscopy. Thirty-eight patient specimens had evidence of chalazia and 40 did not. The main outcome measures included quantification of total eyelid hair follicles, meibomian glands, Demodex folliculorum and Demodex brevis; determining the presence of lipogranulomatous and non-lipogranulomatous inflammation, hair follicle and meibomian gland duct dilatation and hyperkeratinization, and meibomian gland acinar dilation, keratinization, and atrophy. RESULTS: There was a significantly greater mean number of D. folliculorum in biopsies with chalazia, when compared with biopsies without chalazia (5.55 vs. 2.68, p = 0.044). The presence of D. folliculorum was strongly associated with hair follicle duct dilatation and hyperkeratinization and with perifollicular nongranulomatous inflammation (p = 0.00). Hair follicle duct dilatation, hyperkeratinization, and perifollicular inflammation were also independently associated with chalazia (p = 0.040 and 0.031, respectively). D. brevis was observed only in meibomian glands with chalazia (mean = 0.342), but this finding did not reach statistical significance; p= 0.068. CONCLUSION: While the authors cannot establish causality between Demodex and chalazia, these findings suggest that D. folliculorum indirectly may be involved in the pathogenesis of chalazia via its effect on the anterior eyelid margin.

A histological investigation into the correlation of central retinal artery atherosclerosis with the systemic circulation.

Current research suggests that retinal arterial changes such as arteriovenous nicking and arterial narrowing are pathologically distinct from atherosclerosis. Other studies have found a positive correlation between retinal changes and systemic atherosclerosis. However, limited recent histopathologic evidence assessing atherosclerosis in the central retinal artery exists. We investigated atherosclerosis in the central retinal artery and how it correlates to atherosclerosis in the carotid and coronary arteries. Twenty-two cadavers (12 males, 10 females) were dissected, obtaining one orbit, one carotid artery, and one coronary artery from each. The specimens were sectioned and stained for histologic analysis by light microscopy using hematoxylin and eosin, Verhoeff's elastic, and Gomori's trichrome stains. The degree of atherosclerosis was graded from absent, or I (least severe) to VIII (most severe) based on the current American Heart Association guidelines. Atherosclerotic changes were present in the central retinal, coronary, and carotid arteries. A positive correlation was found between the central retinal artery and the carotid artery (r = 0.23, P = 0.15), the central retinal artery and the coronary artery (r = 0.31, P = 0.08), and the carotid artery and the coronary artery (r = 0.45, P = 0.02). The presence of low-grade atherosclerosis in the central retinal artery is prevalent in a population of advanced vascular disease. However, central retinal artery atherosclerotic lesion severity is poorly correlated with disease severity in the carotid and coronary arteries.

Diagnostic utility of adipophilin immunostain in periocular carcinomas.

PURPOSE: To determine the efficacy of adipophilin immunohistochemistry in the diagnosis of sebaceous carcinoma of the ocular adnexal region and to provide the guidelines for interpretation of this immunostain. DESIGN: Retrospective, histopathologic case series. PARTICIPANTS: A total of 25 patients with sebaceous carcinoma, 21 patients with basal cell carcinoma, 22 patients with conjunctival squamous cell carcinoma, 9 patients with cutaneous squamous cell carcinoma, and 5 patients with conjunctival mucoepidermoid carcinoma. METHODS: Immunohistochemical staining for adipophilin was performed on paraffin-embedded tissues and correlated with hematoxylin-eosin, periodic acid-Schiff (PAS), and mucicarmine-stained preparations. Immunostaining was quantified by light microscopy and with a computerized image analysis system of scanned images. Statistical analysis was performed to compare immunostaining patterns within the tumor categories by stage and grade, between the different neoplasms, and for correlation between light microscopy observations and computerized image analysis. MAIN OUTCOME MEASURES: Localization of adipophilin immunostain, intensity of immunostaining, percent of immunoreactive cells, percentages of vacuolar staining and granular staining, and vacuole size. RESULTS: Adipophilin expression was observed in 100% of sebaceous carcinomas, 100% of cutaneous squamous cell carcinomas, 95% of basal cell carcinomas, 73% of conjunctival squamous cell carcinomas, and 60% of mucoepidermoid carcinomas. Sebaceous carcinomas demonstrated significantly stronger adipophilin expression, a greater number of intracytoplasmic vacuoles, and larger vacuoles. The specificity and sensitivity of adipophilin immunostaining in the diagnosis of sebaceous carcinoma were both 100% when more than 5% of the staining occurred in vacuoles (<95% granular staining). The diagnostic sensitivity and specificity were 100% and 96%, respectively, when the staining was graded as moderately or strongly intense and were 92% and 85% when the vacuoles were greater than 1.5 µm in diameter. CONCLUSIONS: Although upregulation of neoplastic steatogenesis is observed in both sebaceous and nonsebaceous carcinomas, the pattern and intensity of adipophilin immunostaining are helpful in distinguishing sebaceous carcinoma from other neoplasms with overlapping histology.