Contralateral Autokeratoplasty, Pars Plana Vitrectomy for Luxated Cataract, and Flanged Intrascleral Intraocular Lens Fixation.

Purpose: To describe a case of a combined procedure including autokeratoplasty, pars plana vitrectomy (PPV), and scleral intraocular lens (IOL) fixation. Methods: Case report. Results: We describe a case of an 85-year-old patient presenting a right, blind eye with a clear cornea and a left eye with acceptable visual potential but affected by bullous keratopathy, aphakia, and a posteriorly dislocated nucleus. The patient underwent a contralateral autokeratoplasty, PPV, and flanged intrascleral IOL fixation with double needle technique. After 24 months of follow-up, the graft remained clear, and the IOL was stable. Conclusions: Complex cases comprising anterior and posterior segments pathology sometimes require combined procedures. A shortage of corneal tissue in developing countries is common. In strictly selected cases, autokeratoplasty may be an option and is associated with fewer complications than allograft corneal transplantation. Sutureless novel techniques for intrascleral fixation of IOL have shown good results and reliable lens stability.

Posterior Amorphous Corneal Dystrophy: New Chromosomal Breakpoints in the Small Leucine-Rich Proteoglycan-Coding Region.

PURPOSE: The purpose of this study was to report the clinical features and describe the results obtained by multimodal corneal imaging of a patient with novel chromosomal breakpoints of the 12q21.33 locus. METHODS: This study was a case report and literature review. RESULTS: A 12-year-old girl presented with visual loss whose examination revealed a best-corrected visual acuity of 20/50 in her right eye and 20/35 in her left eye and corneal flattening and gray sheet-like opacities deep in the stroma. Anterior segment optical coherence tomography and ultrabiomicroscopy showed an evenly distributed hyperreflective line in the posterior stroma. Confocal microscopy revealed enlarged keratocytes and the presence of small reflective deposits from the pre-Descemet line to the endothelium. In addition, a 447-kb deletion that included the small leucine-rich proteoglycan-coding region in locus 12q21.33 was found. She was, therefore, diagnosed with PACD. CONCLUSIONS: PACD is a rare genetic disorder of the cornea characterized by gray sheet-like opacification of the posterior stroma in combination with corneal flattening. Confocal microscopy provides histologic segmentation of each corneal layer and shows the degree to which they are affected. New chromosomal breakpoints of a deletion in the small leucine-rich proteoglycan-coding region are hereby reported. PACD may be a contiguous gene syndrome, and further tests are required to identify the exact position responsible for the phenotypic variation.

Association Between Skin Findings and Ocular Signs in Rosacea.

OBJECTIVES: To report the most frequent signs in ocular rosacea and evaluate their association with skin findings. MATERIALS AND METHODS: Fifty-one patients diagnosed with rosacea by a trained dermatologist were evaluated by an ocular surface specialist. A complete ophthalmological examination was performed. RESULTS: In our study, the prevalence of ocular signs in patients with rosacea was 74.5%. The average age at presentation was 50 years and women were more affected than men. The most common findings were lid margin erythema, meibomian gland dysfunction, and blepharitis. Fifteen patients had decreased visual acuity due to complications related to rosacea such as leukoma and corneal neovascularization. Interestingly, patients that had the lowest visual acuity presented with dermatological signs of papules and pustules (p=0.001) and rhinophyma (p=0.023). Two patients who showed subepithelial fibrosis and fornix foreshortening were diagnosed as having ocular cicatricial pemphigoid (OCP) by immunohistopathological analysis of conjunctival specimens. CONCLUSION: Ocular compromise is common in rosacea. Our study shows that there might be a relationship between the severity of ocular involvement and certain subtypes of cutaneous disease. Rosacea and OCP may coexist. In cases that present with conjunctival fibrotic changes, a diagnostic biopsy is mandatory.

Scleral Compromise in Hereditary Porphyria Cutanea Tarda.

PURPOSE: To report a case of bilateral scleral compromise in a male patient with hereditary porphyria cutanea tarda (PCT). METHODS: Case report. RESULTS: A 57-year-old male was referred to the Cornea Service at Hospital de Clinicas in Buenos Aires for bilateral scleral thinning. He claimed ocular discomfort and photophobia. Slit-lamp biomicroscopy revealed an oval area of deep scleral thinning without uveal prolapse, adjacent to a conjunctival hyperemic zone in the interpalpebral area, 2 mm temporal to the limbus in the right eye. The left eye presented with a conjunctivalized scleral thinning in the interpalpebral area, 2 mm temporal to the limbus. Physical examination revealed facial hyperpigmentation and hypertrichosis and multiple hypopigmented scars in hands and nails. His family history was positive for PCT. The diagnosis was made by urine porphyrin test and genetic molecular testing. In an attempt to reduce ocular and systemic levels of porphyrins, the patient was treated with oral hydroxychloroquine and repeated phlebotomies, altogether with specially designed glasses to avoid local exposure to sunlight. CONCLUSIONS: Scleral involvement is a rare manifestation of PCT. An adequate treatment, including interdisciplinary management may ameliorate ocular signs and symptoms.

Ectodermal Dysplasia: Association with Anti-Basement Membrane Autoantibodies.

Ectodermal dysplasia (ED) is a group of several genetic conditions with absence or dysgenesis of at least two ectodermal derivatives: teeth, skin and its appendages including hair, nails, eccrine and sebaceous glands. The most important clinical findings in patients with ED are hypodontia, hypotrichosis, and hypohidrosis, which can lead to episodes of hyperthermia. Few reports have focused on the progressive keratopathy in ED. Cicatrizing conjunctivitis associated with anti-basement membrane autoantibodies has been described. We report a series of three ectodermal dysplasia patients with an ocular phenotype typically seen in ocular mucous membrane pemphigoid; conjunctival immunohistopathology revealed anti-basement membrane autoantibodies in all of them, and systemic immunosuppression proved to be effective in improving symptoms and helping to stabilize ocular surface disease.

The First Argentinian Family with Familial Amyloidosis of the Finnish Type.

Familial amyloidosis of the Finnish type or Meretoja syndrome is a rare autosomic dominant inherited systemic condition. It was first described by Meretoja in Finland in 1969. It is a disease produced by a single mutation in the gene coding for gelsolin, which generates an abnormal protein that cumulates in tissues and leads to various signs. Obtaining an early diagnosis can be challenging, as the first manifestations of the disease are ophthalmological and may only be seen with slit-lamp biomicroscopy. We present the first 3 cases diagnosed in Argentina, confirmed by genetic molecular testing.

In vitro susceptibility patterns of methicillin-resistant Staphylococcus aureus and coagulase-negative Staphylococcus corneal isolates to antibiotics.

PURPOSE: To determine the in vitro susceptibility of methicillin-resistant Staphylococcus aureus (MRSA) and methicillin-resistant coagulase-negative Staphylococcus (MRCNS) isolates to various antibiotics. METHODS: All cases of bacterial keratitis caused by Staphylococcus species during 2006 and 2007 were identified. The isolates were divided according to species and susceptibility to methicillin into 4 groups: methicillin-susceptible S. aureus, methicillin-susceptible coagulase-negative Staphylococcus, MRSA, and MRCNS. Routine susceptibility testing for Staphylococcus species to methicillin and 19 other antibiotics was performed using the MicroScan POS Breakpoint Combo Panel Type 20. RESULTS: One hundred fifty-seven isolates were identified. Forty isolates were S. aureus, including 21 MRSA, and 117 isolates were coagulase-negative Staphylococcus, including 29 MRCNS. All MRSA isolates were susceptible to gentamicin, linezolid, rifampin, tetracycline, and vancomycin and were resistant to penicillin, cefazolin, cefepime, azithromycin, erythromycin, and ofloxacin. Ninety percent of MRSA isolates were resistant to fourth-generation fluoroquinolones. All MRCNS isolates were susceptible to vancomycin, chloramphenicol, linezolid, and rifampin and were resistant to penicillin, cefazolin, cefepime, and azithromycin. Sixty-five percent of the MRCNS isolates were susceptible to fourth-generation fluoroquinolones and gentamicin. CONCLUSIONS: All MRSA and MRCNS isolates were sensitive to vancomycin, linezolid, and rifampin. MRSA isolates were generally sensitive to gentamicin and tetracycline and resistant to fourth-generation fluoroquinolones. MRCNS isolates were not consistently sensitive to gentamicin, tetracycline, or fourth-generation fluoroquinolones.

Keratoconus associated with other corneal dystrophies.

OBJECTIVE: To report the concomitant presentation of keratoconus and corneal dystrophies at Wills Eye Hospital for the 10-year period from January 1, 1997, to December 31, 2006. METHODS: Patients with concomitant keratoconus and corneal dystrophies were identified using a computer database. Complete ophthalmologic examination, keratometry, pachymetry, and computerized videokeratography were performed in all patients. When present, cornea guttata were confirmed by clinical examination and specular microscopy. Histopathologic examination with special stains of excised corneal buttons was performed. RESULTS: Fifty-one patients manifested typical signs and topographic evidence of keratoconus associated with another corneal dystrophy. Fuchs dystrophy was the most common association accounting for 27 cases (52.9%), followed by anterior basement membrane dystrophy with 13 cases (25.5%) and posterior polymorphous dystrophy with 7 cases (13.8%). A bilateral combination of Fuchs dystrophy and anterior basement membrane dystrophy with keratoconus was seen in 3 cases (5.8%). Finally, there was 1 bilateral case (2%) of granular dystrophy. Histopathologic studies in cases that underwent penetrating keratoplasty confirmed the clinical diagnoses. CONCLUSION: To our knowledge, this is the largest report of such a concurrence in the English literature and could lead to further studies on the possible pathophysiologic or genetic links between these entities, although a chance association cannot be excluded.

Indications for penetrating keratoplasty and associated procedures, 2001-2005.

PURPOSE: To identify current indications and trends in indications for penetrating keratoplasty (PKP) and associated procedures. METHODS: Retrospective chart review of all patients who underwent PKP at Wills Eye Institute from January 1, 2001, to December 31, 2005. RESULTS: A total of 1,162 cases were performed in this 5-year period. Leading indications for PKP were pseudophakic corneal edema (PCE) in 330 (28.4%) cases, followed by regraft in 250 (22.0%), keratoconus in 186 (16%), and Fuchs' endothelial dystrophy in 126 (10.8%) cases. Of the 330 cases of PCE, 232 (70.3%) were associated with posterior chamber intraocular lenses (PCIOLs) and 96 (29.1%) with anterior chamber lenses. In 330 eyes with PCE, the lens was not exchanged in 246 (74.5%) cases and was exchanged in 76 (23%) cases. Seventy of the exchanged lenses were anterior chamber intraocular lenses (ACIOLs) and six lenses were PCIOLs. In cases of ACIOL exchanges, 10 were for scleral sutured IOLs, 18 for PCIOLS, and 42 for another ACIOL. CONCLUSIONS: Pseudophakic corneal edema remains the leading indication for PKP at our institution followed by regraft, continuing a trend noted in our previous studies. Although the percentage of cases of PCE associated with PCIOLS increased, fewer lenses were exchanged, perhaps reflecting increased confidence in biocompatibility of newer IOLs. The decrease in overall number of corneal transplants in these 5 years continues a trend noted in our previous study and mirrors the national decline in PKP.

Meesmann corneal dystrophy associated with epithelial basement membrane and posterior polymorphous corneal dystrophies.

PURPOSE: To report a rare case of bilateral and symmetric Meesmann corneal dystrophy concurrent with bilateral epithelial basement membrane dystrophy and bilateral but asymmetric posterior polymorphous corneal dystrophy in a patient of Armenian origin. METHODS: Complete ophthalmologic examination was performed on a 6-year-old boy from Armenia who was diagnosed with bilateral symmetric Meesmann corneal dystrophy combined with bilateral epithelial basement membrane dystrophy and bilateral but asymmetric posterior polymorphous corneal dystrophy. This case was observed and treated for 24 years. RESULTS: On slit-lamp biomicroscopy, the patient showed bilateral multiple intraepithelial cystic lesions, bilateral irregularly shaped grayish-white opacities in the superficial corneal epithelium, and bilateral but asymmetric transparent vesicles surrounded by gray halos at the level of the Descemet membrane and the endothelium. CONCLUSIONS: This case is reported because of the unusual occurrence of Meesmann corneal dystrophy with other corneal dystrophies.

Trends in penetrating keratoplasty in the United States 1980-2005.

In this review, we analyze the trends in corneal transplantation over the past 25 years in the United States. The most dramatic change was the progressive sharp increase in the number of corneal transplants performed during the 1980s, corresponding with the rise of pseudophakic bullous keratopathy (PBK) as the leading indication for keratoplasty. More recently, there has been a steady annual decline in total keratoplasties for more than a decade, corresponding with a decline in the cases performed for PBK, which still accounts for the highest percentage of cases done. Regrafts have been an increasingly important indication for keratoplasty, as older grafts fail. Keratoconus, Fuchs dystrophy, and other inherited diseases have remained consistent indications for keratoplasty during the last quarter century.