Faculty Bibliography

OBJECTIVES: To report a rare case of spontaneous Stenotrophomonas maltophilia keratitis in a diabetic patient. METHODS: A case report of this rare ulcerative keratitis case with an extensive review of the literature. RESULTS: The patient's corneal infiltrate was successfully cultured and a rare ocular organism identified. The patient's presentation and course and the response to empiric treatment are discussed. CONCLUSIONS: Consideration of the antibiotic combination chosen here can be considered in the treatment of S. maltophilia keratitis after appropriate cultures are taken and the organism isolated.

PURPOSE: Peters anomaly is a rare dramatic finding at birth and can be associated with other systemic malformations. We performed a literature review of multiple case reports and case series to better define the common characteristics and unusual findings associated with Peters anomaly. METHODS: A representative case is discussed followed by a literature review of multiple case reports and case series. The literature search was conducted for the years 1969 to 2009. Cases and case series were included in the review of published English ophthalmic literature. Cases were excluded if no information was reported on ocular and systemic malformations or if no information was reported on surgical interventions or outcomes. In addition, if cases did not report laterality of the lesion, they were excluded from the review. Fifty-eight cases were found that fit the above criteria, and the relevant cases were reviewed to better characterize the systemic malformations, interventions, and outcomes associated with Peters anomaly reported in the literature. RESULTS: Fifty-eight cases of Peters anomaly were reviewed. Of those cases reporting sex, 56% were men and 44% of cases were women. In terms of laterality, 67.2% of cases were bilateral versus 32.8% of cases that were unilateral. Moreover, bilateral cases of Peters anomaly were associated with a higher rate of systemic malformations (71.8%) versus unilateral Peters anomaly (36.8%). This difference was significant (P < 0.03 by Fischer exact test). In the 15 eyes where results of penetrating keratoplasty were reported, the overall success rate was 53%. However, the success rate was significantly higher in patients with Peters anomaly type I (87.5%), as opposed to those patients with Peters anomaly type II (14.2%) (P < 0.02 by Fischer exact test). DISCUSSION: The clinical features, epidemiology, genetics, complications, and treatments of Peters anomaly are presented. Cornea specialists who care for pediatric patients should be aware of the common and uncommon associations with Peters anomaly. Although bilateral Peters anomaly is much more commonly associated with systemic malformations, we believe that all patients with Peters anomaly should be screened for systemic malformations by both pediatricians and geneticists and undergo chromosomal analysis and molecular genetic testing.

Retinal vascular damages are the cardinal hallmarks of retinopathy of prematurity (ROP), a leading cause of vision impairment and blindness in childhood. Both angiogenesis and vasculogenesis are disrupted in the hyperoxia-induced vaso-obliteration phase, and recapitulated, although aberrantly, in the subsequent ischemia-induced neovessel formation phase of ROP. Yet, whereas the histopathological features of ROP are well characterized, many key modulators with a therapeutic potential remain unknown. The CCN1 protein also known as cysteine-rich protein 61 (Cyr61) is a dynamically expressed, matricellular protein required for proper angiogenesis and vasculogenesis during development. The expression of CCN1 becomes abnormally reduced during the hyperoxic and ischemic phases of ROP modeled in the mouse eye with oxygen-induced retinopathy (OIR). Lentivirus-mediated re-expression of CCN1 enhanced physiological adaptation of the retinal vasculature to hyperoxia and reduced pathological angiogenesis following ischemia. Remarkably, injection into the vitreous of OIR mice of hematopoietic stem cells (HSCs) engineered to express CCN1 harnessed ischemia-induced neovessel outgrowth without adversely affecting the physiological adaptation of retinal vessels to hyperoxia. In vitro exposure of HSCs to recombinant CCN1 induced integrin-dependent cell adhesion, migration, and expression of specific endothelial cell markers as well as many components of the Wnt signaling pathway including Wnt ligands, their receptors, inhibitors, and downstream targets. CCN1-induced Wnt signaling mediated, at least in part, adhesion and endothelial differentiation of cultured HSCs, and inhibition of Wnt signaling interfered with normalization of the retinal vasculature induced by CCN1-primed HSCs in OIR mice. These newly identified functions of CCN1 suggest its possible therapeutic utility in ischemic retinopathy.

OBJECTIVES: To report the treatment of a limbal dermoid tumor with excision of the lesion combined with the placement of a processed pericardial graft. METHODS: A case report of a new approach to treat large limbal dermoids after excision of the lesion from the cornea and limbus. RESULTS: The surgical result was very reasonable from a cosmetic result, and the patient recovered quite well. CONCLUSIONS: The use of processed pericardium on the scleral side of the lesion has not been reported previously with limbal dermoid excision. Intraoperatively, the abnormal tissue blended with the sclera and excision of perilimbal tissue was necessary to obtain a clear margin. One might want to consider the method described here for the treatment of large limbal dermoid lesions when the pathology seems indistinct at the conjunctival-corneal border during surgery.

OBJECTIVES: To demonstrate an unusual case of necrotizing scleritis in ulcerative colitis successfully managed by a combination of a Prokera implant and processed pericardium. METHODS: A case report of a novel approach to treat scleral melts that may be seen in several clinical situations. RESULTS: The dual use of amniotic membrane material (Prokera) with processed tuttoplast for necrotizing scleritis salvaged the eye without the need for enucleation while also preserving most of the vision. CONCLUSIONS: Consideration of the combined method discussed in this article should be considered in the treatment of severe scleromalacia seen in inflammatory cases of the eye.

OBJECTIVE: To investigate the in vitro effects of benzalkonium chloride (BAK) at various concentrations and dosing schedules on adenoviral infection. METHODS: Adenovirus was placed in Refeed media and at 30 min after infection, various concentrations and doses of BAK were placed into the 1 mL solution. All assays were incubated at 37 degrees C. Dosing of BAK was performed for 3 days and qualitative viral immunofluorescence was compared with that of controls. RESULTS: BAK at multiple concentrations lysed the epithelial cell lines to varying degrees as did viral infection. Further, adenoviral infection was halted with many concentrations as well. CONCLUSIONS: Adenoviral infection was inhibited in vitro by different concentrations of BAK. CLINICAL RELEVANCE: Zymar solution which contains BAK could potentially have a role in the treatment of adenoviral ocular infection in humans.

OBJECTIVE: To quantify retinal thickness in patients with Parkinson disease (PD). METHODS: Forty-five eyes of 24 PD patients and 31 eyes of 17 control subjects underwent a comprehensive ophthalmologic examination. We used optical coherence tomography to examine retinal thickness, separately quantifying the inner and outer retinal layers. Intraocular pressure was measured by Goldmann applanation tonometry. RESULTS: The mean (SD) ages of the patients with PD and healthy subjects were 64.0 (6.5) years vs 63.5 (10.7) years (P = .77). The mean (SD) intraocular pressure was 13.6 (+/-2.7) mm Hg in the PD patients. No difference was found in either the superior or inferior outer retinal layer thickness of PD vs control eyes. The mean (SD) superior inner retinal layer thickness of PD vs control eyes was 88.79 (11.3) microm vs 103.5 (24.3) microm (P = .01), and the mean inferior inner retinal layer thickness was 89.83 (11.1) microm vs 104.0 (23.5) microm (P = .01). CONCLUSIONS: The inner retinal layer is significantly thinner in PD patients than in healthy subjects. Idiopathic PD, distinct from glaucoma, needs to be considered in the differential diagnosis of retinal nerve fiber layer thinning.

PURPOSE: To demonstrate an unusual case of bilateral superior keratectasia complicated by a corneal perforation. METHODS: Case report of idiopathic superior keratectasia with a spontaneous perforation managed by amniotic membrane transplantation. RESULTS: Superior keratectasia was confirmed by a pattern of superior corneal steepening seen on corneal topography and keratometry in the fellow eye. CONCLUSIONS: Although rare, keratectasia with hydrops and a corneal perforation may present superiorly, and an amniotic membrane should be considered for management.