Esen Karamursel Akpek

Ocular rosacea: Patient characteristics and follow-up

PURPOSEnThe purpose of this report is to review the presenting symptoms and signs, treatment regimens used, complications encountered, and outcome in a cohort of patients with ocular rosacea.nnnMETHODSnThe medical records of 131 patients with a diagnosis of ocular rosacea were reviewed retrospectively. Data were entered in a tabulated form, and a descriptive analysis was performed.nnnRESULTSnThe age range at presentation was between 23 and 85 years (mean, 56 years). Cutaneous manifestations of rosacea were present in 112 of the patients at their first visit. The most common presenting symptoms were foreign body sensation and burning, and the most common signs were telangiectasia and irregularity of lid margins, and meibomian gland dysfunction. Thirteen patients had decreased visual acuity at the time of presentation due to corneal complications. Six of these patients required penetrating keratoplasty during the course of their disease. Seven patients had severe cicatrizing conjunctivitis at the time of referral. One hundred thirteen patients were treated with oral tetracycline derivatives. Seven patients were left with visual acuity less than 20/400, and one patient underwent enucleation for corneal perforation and endophthalmitis.nnnCONCLUSIONSnOcular rosacea is a common disease involving the skin and the eyes. It is widely underdiagnosed by many ophthalmologists despite the blinding potential. Successful therapy requires a multidisciplinary approach.

Intraocular-central nervous system lymphoma: Clinical features, diagnosis, and outcomes

OBJECTIVEnTo analyze the clinical features, laboratory investigations, and diagnosis of intraocular-central nervous system (CNS) lymphoma in a cohort of patients who underwent diagnostic vitrectomy.nnnDESIGNnRetrospective case series. METHOD AND STUDY MATERIALS: Thirty-four vitreous biopsy specimens obtained from 26 patients with treatment-resistant or unusual uveitis were re-evaluated in a masked fashion. The specimens were classified into three groups: negative, suspicious of malignancy, and positive based on the cytologic features, immunomarkers, and flow cytometry. The medical records of the patients were reviewed retrospectively.nnnMAIN OUTCOME MEASURESnThe reliability of vitreous cytology in diagnosing intraocular-CNS lymphoma and the differences in clinical features of patients with intraocular-CNS lymphoma and uveitis.nnnRESULTSnThe two ocular pathologists concurred in their criteria for interpretation of all specimens. There was 100% concordance between the cytologic reports read independently by the two ocular pathologists over the 5-year period and the read-out done in a masked fashion at the time of the study. Ten patients were diagnosed with intraocular-CNS lymphoma based on the vitreous cytology and clinical features. The time interval between the initial presentation and vitreous biopsy was 1 week to 2 years, with 80% of the patients diagnosed within the first year. Retinal involvement in the form of lymphomatous subretinal pigment epithelial infiltrates, vasculitis, and apparent retinochoroiditis was present in six cases. Initial neuroimaging studies revealed concomitant CNS involvement in three patients, and an additional three developed CNS lymphoma following diagnosis by vitreous biopsy. Patients were treated with radiotherapy, chemotherapy, or both. Two of the four patients with a follow-up of greater than 12 months died due to CNS involvement.nnnCONCLUSIONSnVitreous cytology is a sensitive, reliable, and reproducible method of diagnosing intraocular-CNS lymphoma. A high index of suspicion based on the clinical findings and course of the uveitis is critically important in decision-making for diagnostic vitrectomy. Central nervous system involvement is frequent and associated with a high mortality rate. Ophthalmology 1999;106:1805-1810

TFOS DEWS II Definition and Classification Report

The goals of the TFOS DEWS II Definition and Classification Subcommittee were to create an evidence-based definition and a contemporary classification system for dry eye disease (DED). The new definition recognizes the multifactorial nature of dry eye as a disease where loss of homeostasis of the tear film is the central pathophysiological concept. Ocular symptoms, as a broader term that encompasses reports of discomfort or visual disturbance, feature in the definition and the key etiologies of tear film instability, hyperosmolarity, and ocular surface inflammation and damage were determined to be important for inclusion in the definition. In the light of new data, neurosensory abnormalities were also included in the definition for the first time. In the classification of DED, recent evidence supports a scheme based on the pathophysiology where aqueous deficient and evaporative dry eye exist as a continuum, such that elements of each are considered in diagnosis and management. Central to the scheme is a positive diagnosis of DED with signs and symptoms, and this is directed towards management to restore homeostasis. The scheme also allows consideration of various related manifestations, such as non-obvious disease involving ocular surface signs without related symptoms, including neurotrophic conditions where dysfunctional sensation exists, and cases where symptoms exist without demonstrable ocular surface signs, including neuropathic pain. This approach is not intended to override clinical assessment and judgment but should prove helpful in guiding clinical management and research.

Long-term outcomes of Boston type 1 keratoprosthesis implantation: A retrospective multicenter cohort

PURPOSEnTo study the long-term outcomes of Boston type 1 keratoprosthesis (KPro) surgery.nnnDESIGNnRetrospective, multicenter case series.nnnPARTICIPANTSnA total of 158 eyes of 150 patients underwent KPro implantation at 5 participating tertiary centers in the United States between January 2003 and December 2006. Of those, 139 eyes of 133 patients were included in the analyses.nnnMETHODSnThe medical records of consecutive adult patients who received KPro surgery were reviewed. All patients with at least 1 postoperative visit were retained in the outcomes analyses. In eyes in which a repeat KPro procedure was performed, only the outcomes of the initial surgery were analyzed.nnnMAIN OUTCOME MEASURESnVisual acuity (VA) outcomes, postoperative complications, and device retention.nnnRESULTSnThe mean follow-up was 46.7 ± 26 months with all but 4 eyes having at least 6 months of follow-up. Preoperatively, only 10.8% of the eyes had VA of ≥ 20/200. Postoperatively, the VA in 70% of eyes improved to ≥ 20/200. The probability of maintaining VA of ≥ 20/200 at 7 years was 50%. The device retention rate was estimated at 67% at 7 years. The 7-year cumulative incidence of complications was 49.7% for retroprosthetic membrane formation, 21.6% for glaucoma surgery, 18.6% for retinal detachment, and 15.5% for endophthalmitis.nnnCONCLUSIONSnAlthough the risk for complications with longer follow-up seemed to increase, this large multicenter cohort demonstrates favorable outcomes with KPro, with a large number of patients achieving and retaining useful vision over a 7-year period.

Successful treatment of serpiginous choroiditis with alkylating agents

OBJECTIVEnTo describe the management and long-term outcomes of patients with serpiginous choroiditis treated with alkylating agents.nnnDESIGNnRetrospective, noncomparative case series.nnnPARTICIPANTSnNine patients with active, vision-threatening serpiginous choroiditis who had progressive inflammation while on steroids and/or immunosuppressive agents other than alkylating agents treated at three tertiary care uveitis referral centers.nnnMETHODSnPatients received systemic immunosuppression with an alkylating agent, either chlorambucil or cyclophosphamide. Prednisone also was given initially and was tapered and discontinued.nnnMAIN OUTCOME MEASURESnVisual acuity, clinical disease activity, duration of treatment, duration of drug-free disease remission, and side effects of alkylating agent therapy.nnnRESULTSnNo patients had recurrences while on therapy. No further visual loss was encountered after starting the therapy. Six of the patients regained vision. All but two patients achieved prolonged drug-free remissions, ranging in duration between 15 and 96 months (median, 78 months). Side effects included transient bone marrow suppression, nausea, and fatigue. Secondary malignancy was encountered in one patient, whose carcinoma of the urinary bladder was treated successfully.nnnCONCLUSIONSnAdequate immunosuppression with alkylating agents may favorably alter the long-term prognosis of patients with serpiginous choroiditis.

Ocular graft-versus-host disease.

Purpose of reviewThis review was carried out to study the frequency, and severity of ocular surface involvement at the setting of allogeneic hematopoietic stem cell transplantation and subsequent graft-versus-host disease (GVHD) and evaluate the clinical outcomes of newer treatments. Recent findingsOcular involvement has been reported in 60–90% of patients with chronic GVHD. Although dry eye is the most frequent finding occurring in the great majority of patients (up to 90%), posterior segment complications are also not infrequent, seen in 12.8% of patients after bone marrow transplantation. Anti-inflammatory treatments particularly T-cell suppressants seem to have a beneficial effect in managing GVHD. Corticoteroids, calcineurin inhibitors, such as cyclosporine and tacrolimus, as well as antifibrotic agents such as tranilast are available options for topical application. Cyclosporine ophthalmic drop seems to be a well tolerated and effective treatment modality; favorable results have been demonstrated with increased dosage. SummaryGVHD is an increasingly frequent cause of ocular surface morbidity with the potential of visual loss from corneal involvement. Early diagnosis and aggressive local as well as systemic treatment can be vision saving.

Current concepts and techniques in keratoprosthesis.

Purpose of review Diseases affecting the cornea are a major cause of blindness worldwide, second only to cataract in overall importance, with an estimated 10 to 15 million affected people. Although keratoplasty is by far the most successful transplantation surgery, the outcomes in high-risk adult patients, including those with ocular surface diseases and multiple graft rejections, and in pediatric patients with congenital corneal opacities are disappointing. Recent developments Regrettably, no significant clinical developments have been achieved in the field of corneal transplantation since the introduction of steroids for graft rejection. Furthermore, obtaining donor corneal tissues and eye banking, particularly in the developing countries where corneal blindness is most prevalent, are problematic. Although the postoperative complications may be severe and limit the use of currently available devices, keratoprosthesis - artificial corneal implantation - has a role in the management of corneal blindness in carefully selected patients with complex ocular diseases who are at high risk for graft failure. Summary This article reviews the recent ophthalmic literature published on the current concepts and techniques of keratoprosthesis surgery.

Severity of episcleritis and systemic disease association.

OBJECTIVEnTo analyze patient characteristics and correlate between the site and severity of the inflammation and ocular and/or systemic disease association in a cohort of patients with episcleritis.nnnDESIGNnRetrospective case series.nnnMETHODSnMedical records of 100 patients with episcleritis were reviewed. Data were analyzed using a customized database software.nnnRESULTSnThe age range at presentation was 18 to 76 years (mean, 43; median, 44). Sixty-nine percent of the patients were female. Thirty-two (32%) patients had bilateral involvement. The episcleritis was nodular in 23 eyes (16%). Half of the patients had a concurrent eye disease. Associated systemic disease was found in 36 patients (36%). In two patients, episcleritis preceded a systemic vasculitic disease (Wegener granulomatosis and Cogan syndrome). Ocular complications included uveitis (11.4%), corneal involvement (15%), and glaucoma (7.8%). No significant correlation of the site and severity of inflammation to the presence of associated systemic or ocular diseases was found. The mean follow-up was 16.5 months. Twenty-eight patients experienced recurrence of episcleritis during the follow-up. Half of the patients required treatment with oral nonsteroidal anti-inflammatory drugs.nnnCONCLUSIONSnEpiscleritis is usually a benign, self-limited disease, but it should not be trivialized since it may be associated with systemic disease and ocular complications. A careful review of systems should be performed in all patients presenting with episcleritis, and this should be repeated at least annually during the follow-up. A thorough eye examination is obviously essential to detect and treat ocular complications.

Elevated vitreous interleukin-10 level is not diagnostic of intraocular-central nervous system lymphoma

OBJECTIVEnDiagnosis of intraocular-central nervous system (CNS) lymphoma is commonly made by identifying malignant lymphocytes in the vitreous. However, such cells are in the minority in the vitreous cellular infiltrate (most are reactive lymphocytes), and therefore lack of cytologic support from biopsied vitreous samples in patients suspected of having intraocular-CNS lymphoma may occur. Recent data suggest that interleukin-10 (IL-10) levels are elevated in the serum and vitreous of patients with non-Hodgkins lymphoma, whereas IL-12 and IL-6 levels are elevated in patients with uveitis of non-neoplastic etiology. The authors evaluated the usefulness of measuring vitreous levels of IL-6, -10, and -12 in the diagnosis of intraocular-CNS lymphoma.nnnDESIGNnProspective case series.nnnPARTICIPANTSnSeventeen patients with intraocular inflammation who underwent a diagnostic or therapeutic vitrectomy: 4 patients with intraocular-CNS lymphoma and 13 patients with uveitis unrelated to a neoplasm.nnnINTERVENTIONnEighteen vitreous specimens were obtained prospectively. Concentrations of IL-6, -10, and -12 were measured by enzyme immunosorbent assay, and relative ratios of the interleukins were calculated. Cytopathologic examination and flow cytometry of vitreous cells were also performed.nnnMAIN OUTCOME MEASURESnThe ratio of IL-10/IL-12 and IL-10/IL-6 was calculated to assess any association of intraocular-CNS lymphoma and high vitreous IL-10 relative to IL-6 and IL-12 levels.nnnRESULTSnThe IL-10/IL-6 and IL-10/IL-12 ratio was greater than 1 in 8 of 14 vitreous specimens obtained from 13 patients with non-neoplastic uveitis. One of the four specimens from patients with cytologically proven intraocular-CNS lymphoma had vitreous IL-10/IL-6 and IL-10/IL-12 ratios of less than 1.nnnCONCLUSIONnAlthough a helpful diagnostic tool, an elevated vitreous IL-10 to IL-6 or IL-12 ratio is not always associated with intraocular-CNS lymphoma.

Treatment of Sjögren's syndrome-associated dry eye an evidence-based review.

BACKGROUNDnOutcomes-based review of reported treatment options for patients with dry eye secondary to Sjögrens syndrome (SS).nnnCLINICAL RELEVANCEnDry eye affects many individuals worldwide. Significant proportion of patients with dry eye has underlying SS, a progressive autoimmune condition. The few suggested guidelines for the treatment of dry eye are mostly based on severity of symptoms and/or clinical findings rather than on outcomes analysis, and do not differentiate SS from other causes of dry eye. METHODS AND LITERATURE REVIEW: A search strategy was developed to identify prospective, interventional studies of treatments for SS-associated dry eye from electronic databases. Eligible references were restricted to English-language articles published after 1975. These sources were augmented by hand searches of reference lists from accessed articles. Study selection, data extraction, and grading of evidence were completed independently by ≥4 review authors.nnnRESULTSnThe searches identified 3559 references as of August 10, 2010. After duplicate review of the titles and abstracts, 245 full-text papers were assessed, 62 of which were relevant for inclusion in the review.nnnCONCLUSIONSnIn the current literature on SS-associated dry eye, there is a paucity of rigorous clinical trials to support therapy recommendations. Nonetheless, the recommended treatments include topical lubricants, topical anti-inflammatory therapy, and tear-conserving strategies. The efficacy of oral secretagogues seems greater in the treatment of oral dryness than ocular dryness. Although oral hydroxychloroquine is commonly prescribed to patients with SS to alleviate fatigue and arthralgias, the literature lacks strong evidence for the efficacy of this treatment for dry eye.