Faculty Bibliography

OBJECTIVE:Fungal keratitis is a serious ocular infection that is considered to be rare among contact lens wearers. The recent Fusarium keratitis outbreak raised questions regarding the background rate of Fusarium-related keratitis and other fungal keratitis in this population. DESIGN/METHODS:Retrospective, multicenter case series. PARTICIPANTS/METHODS:Six hundred ninety-five cases of fungal keratitis cases who presented to 1 of 10 tertiary medical centers from 2001 to 2007. METHODS:Ten tertiary care centers in the United States performed a retrospective review of culture-positive fungal keratitis cases at their centers between January 2001 and December 2007. Cases were identified using microbiology, pathology, and/or confocal microscopy records. Information was collected on contact lens status, method of diagnosis, and organism(s) identified. The quarterly number of cases by contact lens status was calculated and Poisson regression was used to evaluate presence of trends. The Johns Hopkins Medicine Institutional Review Board (IRB) and the IRBs at each participating center approved the research. MAIN OUTCOME MEASURES/METHODS:Quarterly number of fungal keratitis cases and fungal species. RESULTS:We identified 695 fungal keratitis cases; 283 involved the use of contact lenses. The quarterly number of Fusarium cases increased among contact lens wearers (CLWs) during the period that ReNu with MoistureLoc (Bausch & Lomb, Rochester, NY) was on the market, but returned to prior levels after withdrawal of the product from the market. The quarterly frequency of other filamentous fungi cases showed a statistically significant increase among CLWs comparing October 2004 through June 2006 with July 2006 through December 2007 with January 2001 through September 2004 (P < 0.0001). CONCLUSIONS:The quarterly number of Fusarium fungal keratitis cases among CLWs returned to pre-Renu with Moistureloc levels after removal of the product from the market. However, the number of other filamentous fungal keratitis cases, although small, seems to have increased among refractive CLWs. Reasons for these apparent increases are unclear.

PURPOSE/OBJECTIVE:To describe the clinical and immunopathologic features of the first convincing apocrine hidrocystoma of the caruncle that happened to be pigmented. METHODS:Case report with clinical evaluation, ultrasound biomicroscopy, and paraffin-embedded tissue stained with hematoxylin and eosin, periodic acid-Schiff, and immunohistochemical markers. RESULTS:Ultrasound biomicroscopy identified a cyst occurring in a 56-year-old white woman. Histopathologically, it was lined by an inner eosinophilic columnar epithelium that was gross cystic disease fluid protein-15-positive and evinced apical decapitation secretion and periodic acid-Schiff-positive, diastase-resistant cytoplasmic granules. Fontana- and MART-1-positive melanin granules were demonstrated. There was an outer layer of smooth muscle actin-positive myoepithelial cells. An adjacent apocrine gland was discovered with identical staining characteristics but without melanin granules. CONCLUSIONS:Classical apocrine gland cysts can exceptionally develop in the caruncle. The light dispersion of cytoplasmic melanin found in the lining cells may have contributed to the cyst's clinical pigmentation, which is otherwise generally regarded as the result of the Tyndall effect, wherein blue wavelengths of light reflect from a colloidal solution of lipofuscin and cell debris.

PURPOSE/OBJECTIVE:In recent years, outbreaks of Acanthamoeba keratitis (AK) have been reported worldwide. The purpose of this study was to examine the clinical experience of AK at the Massachusetts Eye and Ear Infirmary. METHODS:A retrospective case review was completed on patients with infectious keratitis whose corneal cultures were positive for Acanthamoeba between January 2000 and December 2008. The clinical characteristics and visual outcomes were examined in those patients with a follow-up period greater than 6 months. RESULTS:Four cases were identified between January 2000 and December 2003, whereas 26 cases were identified between January 2004 and December 2008. Charts before 2004 were unavailable for review. A total of 15 cases between 2004 and 2008 had a follow-up period of greater than 6 months. In these cases, possible risk factors associated with AK included soft contact lens wear (12 of 15 cases), exposure to freshwater or saltwater sources (8 of 15 cases), chronic ocular surface disease (6 of 15 cases), ocular trauma (3 of 15 cases), and concomitant infectious keratitis (2 of 15 cases). Four cases were associated with the use of Advanced Medical Optics Complete MoisturePlus Multi-Purpose Solution. Many cases were recalcitrant to medical therapy alone, necessitating therapeutic penetrating keratoplasty in 8 of 15 cases. CONCLUSIONS:The number of AK cases at the Massachusetts Eye and Ear Infirmary has increased since 2004. Contact lens wear and exposure to contaminated water sources were potential risk factors for AK. Clinicians should maintain a high clinical suspicion for AK in cases of atypical keratitis with known risk factors for AK.

PURPOSE/OBJECTIVE:To report a case of pigmented deposits on a type I Boston keratoprosthesis (KPro) associated with the use of topical ibopamine as a treatment for hypotony. METHODS:Case report and literature review. RESULTS:The dopamine-like agent ibopamine caused black deposits on the bandage lens and on the front plate of the Boston KPro that resulted in reduced visual acuity. Change to a daily disposable contact lens and regular cleaning of the KPro front plate with diluted baby shampoo eliminated this problem. CONCLUSION/CONCLUSIONS:This is the first report of this complication with topical ibopamine use and should be considered when ibopamine is used chronically for hypotony.

PURPOSE/OBJECTIVE:The purpose of this study is to review the current literature on keratoprosthesis and its use in the pediatric population. METHODS:Case report and review of recent literature on outcomes of pediatric keratoplasty and keratoprosthesis. RESULTS:Penetrating keratoplasty in children is fraught with shortcomings, including difficulty maintaining a clear graft (as low as 44% at 50 months), poor visual acuity outcomes (only 30-34% achieving 20/400 or better), and a high rate of allograft rejection (40-50%). Placement of the Boston type I keratoprosthesis has the advantage of achieving a clear visual axis and stable refractive error within days of surgery without risk of rejection. However, life-long care is required, including topical steroids and antibiotic prophylaxis, as well as bandage contact lens wear and a frequent a need for further interventions for retroprosthetic membranes and glaucoma. CONCLUSIONS:With motivated parents, a successful anatomic and functional outcome can be achieved with the Boston keratoprosthesis in children of amblyogenic age.

Riboflavin-induced ultraviolet light (UV) cross linking has received a significant amount of attention in recent years. It is currently approved in Europe as a treatment for keratoconus and is also being used for other corneal disorders. The goal of this paper is to review in detail seminal papers and studies that have been done to support cross linking as a safe and effective treatment for patients with early stages of keratoconus.

A healthy 10-year-old girl developed synchronous, bilateral, temporal redness of the eyes regarded as sectoral conjunctivitis for 5 years that was unresponsive to topical steroids and antihistamines. Finely vascularized, minimally elevated, amelanotic or faintly focally pigmented, epibulbar conjunctival lesions were present bilaterally. The lesions were completely excised. Histopathologic and immunohistochemical evaluations confirmed that they were both predominantly junctional nevi with conspicuous chronic inflammation. Juvenile conjunctival nevi frequently have atypical histopathologic traits that in an adult could be suggestive of melanoma. The differential diagnosis of much less likely disorders includes leukemia, lymphoid tumor (salmon patch), and conjunctival sarcoidosis, among other conditions. The patient has had no recurrence 3 years after surgery.

PURPOSE/OBJECTIVE:To present a case of a corneoscleral juvenile xanthogranuloma (JXG) with diagnostically challenging features. METHODS:A 15-year-old boy's small corneoscleral mass of recent onset was examined histologically and immunohistochemically. RESULTS:The biopsy had some superficially misleading histological and immunohistochemical features: S-100 positivity (however, observable in 30% of JXG lesions) and lack of Touton giant cells (often absent in early lesions). Most importantly, the histiocytes stained negatively for CD1a and strongly positively for both lysozyme and CD68 antigen. CONCLUSIONS:JXG is a benign histiocytic disorder that usually appears early in childhood but is also encountered in 13%-18% of cases in the second decade. The histiocytes usually stain positively for CD68 and negatively for S-100 and CD1a. Correctly distinguishing JXG from the more aggressive spectrum of Langerhans cell diseases (100% have CD1a positivity) is essential for patient treatment.

PURPOSE/OBJECTIVE:To describe an acquired, smooth white lesion of the caruncle and to underscore the role of subsurface keratinizing squamous epithelium in its formation. METHODS:Clinical photographic documentation, histopathologic evaluation, and immunohistochemical staining of an excised specimen from a 25-year-old woman. RESULTS:A cyst was found that was lined by keratinizing squamous epithelium without a keratohyaline layer (trichilemmal keratinization), typical of lesions of the pilosebaceous unit. A portion of the cyst's lining was replaced by granulomatous inflammation resulting from an earlier spontaneous partial rupture. Ki-67 immunolabeling demonstrated relatively few nuclei in S-phase (DNA synthesis) in comparison with the overlying epithelium, thereby suggesting an obstructive, nonproliferative cause for the cyst. CONCLUSION/CONCLUSIONS:A white caruncular lesion is a very rare finding according to the literature. It is most likely caused by a cyst lined by squamous epithelium elaborating trichilemmal-type keratin. A sebaceous gland duct was established as the source for the current lesion.

PURPOSE/OBJECTIVE:To report two cases of persistent epitheliopathy after treatment of conjunctival melanoma and review management options for this condition. METHODS:Case report and literature review. RESULTS:Persistent epitheliopathy, presumably resulting from limbal stem cell deficiency, is an uncommon complication of treatment of conjunctival malignant melanoma with surgical excision, cryotherapy, and mitomycin C. The Boston Ocular Surface Prosthesis was successful in improving vision, comfort, and epithelial status in our patients. CONCLUSION/CONCLUSIONS:Persistent epitheliopathy is a rare complication of treatment of conjunctival melanoma. The Boston Ocular Surface Prosthesis is a viable treatment option in this situation.