Mannose-binding lectin as part of the complement pathway: characterization in non-inflamed and inflamed human eyes.

BACKGROUND: Mannose-binding lectin plays a central effector role in the lectin pathway of complement activation. Frequently occurring MBL2 polymorphisms result in mannose-binding lectin deficiency, which increases susceptibility to infection. We characterized mannose-binding lectin levels and function in non-inflamed and inflamed human eyes, and evaluated its relationship to blood mannose-binding lectin levels and function. DESIGN: Prospective, observational clinical study with controls and cases. PARTICIPANTS: Twenty-seven patients with paired blood and ocular samples (aqueous and/or vitreous) including 15 controls (non-inflamed) and 12 cases (inflamed). METHODS: Blood and ocular samples were collected from controls (n = 15) with quiet eyes during elective cataract surgery and cases with inflamed eyes including proven/suspected endophthalmitis (n = 11) and herpetic retinal vasculitis (n = 1). Mannan-binding and C4 deposition enzyme-linked quantify mannose-binding lectin levels and function. MAIN OUTCOME MEASURES: Blood and ocular mannose-binding lectin levels and function. RESULTS: Of 27 patients, 10 (37%) were mannose-binding lectin-deficient (defined as blood mannose-binding lectin levels <500 ng/mL). Blood mannose-binding lectin levels (P= 0.16) or function (P= 0.43) were not significantly different between controls and cases. As expected, there was a high correlation between blood mannose-binding lectin levels and function (r(2) = 0.74). However, there was significantly more mannose-binding lectin in inflamed eyes than non-inflamed eyes measured as level (P < 0.01) or C4 deposition function (P < 0.01). CONCLUSIONS: Our study demonstrated that mannose-binding lectin is significantly elevated in inflamed human eyes but virtually undetectable in non-inflamed control eyes, suggesting a role in sight-threatening ocular inflammation.

Infratemporal inflammatory myofibroblastic tumour with orbital extension.

Inflammatory myofibroblastic tumour (IMT) is a rare distinctive neoplasm of intermediate biological potential with a predilection for the abdominopelvic region and lung of children and young adults. It is histologically composed of spindle cells (myofibroblasts) in a myxoid to collagenous stroma with a prominent inflammatory infiltrate composed primarily of plasma cells and lymphocytes. Its pathogenesis is controversial. Arising most commonly in the lungs, only two cases of histopathologically confirmed IMT originating from the infratemporal and pterygopalatine fossae have been documented in the literature. Neither had orbital involvement. We now report the first case of IMT originating from the infratemporal fossa with orbital extension in a previously well 31-year-old woman. The patient presented with a 5-month history of intermittent right-sided headaches, progressive trismus and right lower lid swelling. She then developed right proptosis, diplopia and paraesthesia in the ophthalmic and maxillary divisions of her trigeminal nerve. Magnetic resonance imaging showed a soft tissue mass occupying most of the right infratemporal fossa with extension into pterygopalatine fossa and orbit. Provisional diagnosis from an open biopsy was nodular fasciitis. She underwent surgical debulking of the infratemporal fossa and lateral orbit through a coronal and trans-oral approach with trans-zygomatic access via total zygomatic osteotomy. Review of intraoperative specimens revised the diagnosis to IMT. Further management included systemic corticosteroids and adjuvant radiotherapy.

Stability of visual outcome between 2 and 5 years following corneal transplantation in the UK.

BACKGROUND AND AIMS: Many studies of corneal transplantation focus on graft failure or rejection as endpoints, or report visual outcomes at one postoperative time point. We aimed to study the stability of visual outcomes between 2 and 5 years following corneal transplantation. METHODS: All patients with keratoconus (868) or Fuchs endothelial dystrophy (FED) (569) receiving their first corneal transplant for visual purposes in the UK between January 2003 and December 2009 were included. The probability of visual improvement or deterioration (gain or loss of ≥2 Snellen lines, respectively) between 2 and 5 years after keratoplasty was modelled by multivariable logistic regression. RESULTS: The majority of keratoconus patients with a penetrating keratoplasty (PK) or deep anterior lamellar keratoplasty maintained their visual acuity (651/868; 75%) while 15% (133/868) improved and 10% (84/868) deteriorated. Similarly, most patients with FED who received a PK maintained their vision (395/569; 70%) while 18% (105/569) improved and 12% (68/569) deteriorated.

Comparison of nonmydriatic digital retinal imaging versus dilated ophthalmic examination for nondiabetic eye disease in persons with diabetes.

OBJECTIVE: To evaluate the ability of stereoscopic nonmydriatic digital retinal imaging to detect ocular pathologic features other than diabetic retinopathy (DR) in patients with diabetes mellitus (DM) compared with dilated retinal examination by retinal specialist ophthalmologists. DESIGN: Clinic-based comparative instrument study and retrospective chart review. PARTICIPANTS: Two hundred eighty Joslin Diabetes Center outpatients (560 eyes) with type 1 or type 2 DM. METHODS: Nonsimultaneous stereoscopic nonmydriatic digital retinal images (640 x 480 pixels) of three 45 degrees retinal fields were acquired and graded for clinical level of DR and other ocular pathologic features by certified readers according to Joslin Vision Network (JVN) protocol. Retrospective chart review compared findings from JVN digital images with findings from dilated retinal examination by retinal specialists performed within an average of 39.6 days of digital imaging. An independent senior retinal specialist adjudicated disagreements by review of 7 standard field 35-mm Early Treatment Diabetic Retinopathy Study protocol fundus photographs and JVN images. MAIN OUTCOME MEASURES: Detection of non-DR ocular pathologic features by digital imaging as compared with clinical examination. RESULTS: Nonmydriatic digital evaluation identified at least 1 non-DR ocular finding in 40.7% of patients (114/280). Non-diabetes mellitus ocular pathologic features identified by digital images, clinical examination, or both included cataract (n = 100); age-related maculopathy (n = 52); suspicion of glaucoma (n = 18); choroidal lesions (n = 18); evidence of systemic disorder (e.g., hypertension or renal disease; n = 15); epiretinal membrane (n = 11); chorioretinal atrophy, scar, or both (n = 6); retinal emboli (n = 3); retinitis pigmentosa (n = 1); and asteroid hyalosis (n = 1). Agreement of nonmydriatic imaging with clinical examination for presence and absence of these findings was 95.4%, 91.3%, 98.2%, 98.6%, 98.2%, 99.6%, 100%, 100%, 100%, and 100%, respectively. Kappa values for all non-DR lesions demonstrated near perfect agreement (kappa> or =0.80) except for age-related maculopathy (kappa = 0.71) and choroidal lesions (kappa = 0.73), where agreement was substantial. Overall, only 55 eyes (9.8%) were ungradable for level of DR and 85 eyes (15.2%) were ungradable for macular edema. CONCLUSIONS: Joslin Vision Network nonmydriatic digital imaging demonstrated excellent agreement with dilated ophthalmic examination by retinal specialists in the detection of ocular disease other than DR, suggesting a potential role for this technology in evaluating non-DR disorders and highlighting the extent of findings other than retinopathy in patients with diabetes.

Long-Term Outcomes of High-Risk Keratoplasty in Patients Receiving Systemic Immunosuppression.

PURPOSE: Immunological graft rejection after corneal transplantation remains the leading cause of graft failure. Systemic immunosuppression is used for keratoplasty at a high risk of rejection to improve graft survival. We examined the long-term outcomes of high-risk corneal grafts in patients receiving systemic immunosuppression. METHODS: Thirty-five corneal transplants with a high risk of rejection were identified from 29 patients within a regional immunosuppression service in the United Kingdom. Definition of keratoplasty at "high risk" of rejection included one or more of the following: a history of ipsilateral graft rejection and/or failure, 2 or more quadrants of stromal vascularization, perforation or ocular inflammation at the time of surgery, presence of atopy, and a large-diameter (≥9 mm) graft. Median follow-up duration was 5 years after transplantation. RESULTS: Graft survival at 5 years in patients receiving systemic immunosuppression was 73.5%. Rejection episodes occurred in 14 grafts (40%); these episodes were reversible in 10 grafts (71%). Indications for transplantation were mostly visual (n = 19; 54%) and tectonic (n = 14; 40%). Eighteen grafts (51%) had 2 or more high-risk characteristics. Most patients (n = 20; 69%) received monotherapy, commonly with tacrolimus (n = 15; 52%) or mycophenolate mofetil (n = 8; 28%). Three patients (10%) experienced severe systemic side effects. Median "day-to-day" logMAR visual acuity was 0.5 in grafts for all indications and 0.2 for visual indications. CONCLUSIONS: Systemic immunosuppression in patients with high-risk keratoplasty seems to improve graft survival with a median follow-up duration of 5 years and is tolerated by most patients. Despite rejection episodes occurring in 40% of grafts, these were mostly reversible.

Long-term outcomes of deep anterior lamellar keratoplasty versus penetrating keratoplasty in Australian keratoconus patients.

PURPOSE: The aim of this study is to compare long-term visual and surgical outcomes after performing deep anterior lamellar keratoplasty (DALK) and penetrating keratoplasty (PK) for keratoconus. METHODS: This is a retrospective review of 73 consecutive patients with keratoconus, managed with DALK or PK, between 2000 and 2010, by a single surgeon. Data were collected on baseline parameters, best-corrected visual acuity (BCVA) in logarithm of the mean angle of resolution (logMAR), subjective refraction, graft survival, and complications. RESULTS: Thirty-one (42.5%) eyes underwent a DALK, and 42 (57.5%) eyes underwent a PK. All PK-operated and 29 out of 31 (93.5%) DALK-operated eyes remained clear at the last review. Postoperative complications were significantly more frequent after PK (57.1%) than after DALK (26.5%; P = 0.0197). The mean BCVA was not significantly different for DALK (0.14 logMAR, SD 0.2) versus PK (0.05 logMAR, SD 0.11); however, eyes that underwent PK were more likely to achieve a BCVA of 0.0 logMAR (P = 0.0029). Subjective refraction and method of visual correction (spectacles or contact lenses) were similar for each group. CONCLUSIONS: DALK-operated patients in this study showed similar graft survival, fewer postoperative complications, and equivalent refractive outcomes. No significant difference in the mean BCVA was noted between DALK and PK cases.