Faculty Bibliography

Conjunctival nevi in children and adolescents often have histologic features that can be difficult to differentiate from malignancy. We have identified a subset of childhood nevi displaying a confluent growth pattern and a lack of maturation that we have defined as juvenile conjunctival nevi (JCN), with the aim of further describing the clinicopathologic features of these lesions. Lesions identified as conjunctival nevus in a tertiary referral hospital were reviewed and the subset of lesions identified as JCN were further evaluated. Clinical details including follow-up data were also gathered. Of the 40 conjunctival nevi identified, 33 fit the criteria for JCN. The mean age at time of excision was 10.9 years (range: 4 to 19 y). Thirty-two lesions were of the compound type; one was a junctional nevus. All showed a nested junctional growth pattern. In 17 lesions (61%), the junctional component extended beyond the subepithelial component (shoulder phenomenon). Maturation was absent in 21 of the compound nevi (66%, average age 10.3 y), and incomplete in the remaining 11 lesions (34%, average age 12.1 y). The nuclei of the subepithelial nevus cells were larger than the epithelial nevus cells in 19 nevi (59%) and the same size in 13 (41%). A lymphocytic host response was present in 17 lesions (52%). Mitotic figures were rarely seen. None of the lesions had recurred over an average follow-up period of 34 months. Recognition of JCN as a distinct morphologic variant of a conjunctival nevus with characteristic histologic features may help to distinguish this benign lesion from melanoma.

PURPOSE/OBJECTIVE:To describe cataract extraction and intraocular lens placement in a patient with an existing Type I Boston keratoprosthesis. METHODS:Case report to describe the surgical technique used to remove a cataract that developed after Boston keratoprosthesis placement. RESULTS:Extracapsular cataract extraction by using an open-sky technique can be used to remove a cataractous lens in the setting of an existing Boston keratoprosthesis. CONCLUSIONS:Although this case shows that it is possible to perform cataract surgery after Boston keratoprosthesis placement, it is prudent to remove the crystalline lens during the initial surgery, even in the absence of a visually significant cataract, because several factors work together to almost guarantee eventual cataract development if the patient is left phakic.

Corneal dystrophies are a group of heterogenous conditions that are characterized by the progressive loss of corneal transparency that results from the accumulation of deposits within the different corneal layers. Up until recently, corneal dystrophies were classified according to their slit lamp appearance, the morphology of the deposits, the depth of the corneal involvement and their histopathological features. This classification has been challenged because of the significant inter-and intra- familial variability in phenotypic expression of the corneal dystrophies and the overlapping characteristics between the different types. In addition, there are dystrophies with atypical characteristics that involve multiple corneal layers or are associated with extraocular involvement that can not be classified into a single type based on morphologic criteria. Recent developments in corneal genetics have shed light on the pathogenesis of corneal dystrophies and have led to the implementation of a new classification scheme that takes into account the responsible gene defect. The discovery that mutations in Tissue Growth factor beta inducible (TGFBI) responsive gene, BIGH3, are the cause of a group of corneal dystrophies (granular, Avellino, lattice and Reis Bucklers) has revolutionized our understanding of these conditions (Stone et al., 1994; Munier et al., 1997). Since this discovery, the genetic basis of many corneal dystrophies and a chromosomal locus for several others has been described. It has also been shown that mutations in the same gene can cause different phenotypes. The association of specific gene mutations with specific phenotypes has given the clinician the opportunity to use molecular genetic analysis to diagnose dystrophic corneal disorders. In parallel, characterization of the mutations responsible for different types of corneal dystrophies has expanded our knowledge regarding their genetic and inheritance patterns. The finding of a highly penetrant, dominantly inherited corneal dystrophy in an affected offspring of unaffected parents has confirmed the existence of spontaneous pathogenic mutations. Additionally, molecular analysis for pathogenetic mutations can confirm the diagnosis in cases with atypical presentations of corneal dystophies. On the other hand, we still know little about how interactions between the environment and the genetic composition affect the phenotype of these conditions. It is highly likely that even when our understanding of the molecular basis of the corneal dystrophies is complete, this knowledge will be used as an adjunct to clinical findings to make the diagnosis of a corneal dystrophy.

Congenital corneal opacities present in approximately 3/100,000 newborns. Many different disorders may result in corneal opacifications of infancy, including Peters' anomaly (PA), congenital hereditary endothelial dystrophy (CHED), congenital hereditary stromal dystrophy (CHSD) and posterior polymorphous dystrophy (PPMD). Current studies have localized defects using genetic testing in PA, CHED, CHSD and PPMD. Identifying mutations for specific disorders may lead to better understanding of the underlying pathogeneses and may help with diagnosis and prognosis. This article will review the clinical presentations, treatments and genetics of Peters' anomaly, congenital hereditary endothelial dystrophy, congenital hereditary stromal dystrophy and posterior polymorphous dystrophy.

OBJECTIVE:To describe the surgical procedure for placement of an implantable telescope prosthesis for end-stage age-related macular degeneration. METHODS:As part of a phase 2/3 clinical trial for patients with bilateral, irreversible age-related macular degeneration, the optimal procedure for monocular placement of the telescope prosthesis was determined. RESULTS:Because of the unique configuration of the telescope prosthesis, proper wound construction, anterior chamber management, and device insertion after phacoemulsification are critical for successful surgery. CONCLUSION/CONCLUSIONS:A unique surgical technique ensures appropriate placement of the telescope prosthesis, while reducing surgical trauma to the corneal endothelium.

The current World Health Organization classification of conjunctival melanocytic proliferations divides them into conjunctival nevi and invasive melanoma but, in contrast to other anatomic sites, does not recognize melanoma in situ. All atypical intraepithelial conjunctival proliferations are included in a heterogeneous category designated as primary acquired melanosis (PAM) with atypia. We performed clinicopathologic analysis of 29 cases of PAM with atypia. On the basis of histologic features and frequency of association with invasive melanoma and metastases, we were able to divide our cases into 2 histologic groups. The low-risk group (13 cases) included lesions composed of small to medium size melanocytes with high nuclear to cytoplasmic ratio and small to medium size hyperchromatic nuclei devoid of nucleoli showing predominantly single cell lentiginous growth pattern. Invasive melanoma occurred in only 2 cases from this group. None of these lesions metastasized. The second, high-risk group (16 cases), showed increased frequency of association with invasive melanoma (15/16 cases, 94%) and metastases (4/16 cases, 25%). These lesions were more heterogeneous architecturally but were all composed of melanocytes showing various degrees of epithelioid features such as abundant cytoplasm, vesicular nuclei, or prominent nucleoli. In 4 cases discrete areas showing high-risk and low-risk features were identified. All 4 lesions were associated with invasion. Our findings offer a practical approach for prognostically useful subclassification of PAM with atypia, which emphasizes cytologic features of intraepithelial conjunctival melanocytic proliferation.

We review the use of three topical medications for the therapy of ocular surface tumors: mitomycin C, 5-fluorouracil, and interferon alpha-2B. One hundred sixty patients with histologically or cytologically proven epithelial and melanocytic tumors were identified in the literature. Side effects occurred most often with mitomycin C, followed by 5-fluorouracil, and interferon alpha-2B. Patients most frequently experienced transient keratitis, redness, and irritation. Topical agents were used as both primary and adjuvant therapy. Rates of tumor regression for CIN and squamous cell carcinoma ranged from 80 to 96%, and 70% of pigmented tumors regressed after an average follow-up of 27 months.