Canadian Rheumatology Association Recommendations for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis.

OBJECTIVE: To develop Canadian recommendations for the screening, monitoring, and treatment of uveitis associated with juvenile idiopathic arthritis (JIA). METHODS: Recommendations were developed using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE)-ADOLOPMENT approach. A working group of 14 pediatric rheumatologists, 6 ophthalmologists, 2 methodologists, and 3 caregiver/patient representatives reviewed recent American College of Rheumatology (ACR)/Arthritis Foundation (AF) recommendations and worked in pairs to develop evidence-to-decision (EtD) tables. A survey to assess agreement and recommendations requiring group discussion was completed. EtD tables were presented, discussed, and voted upon at a virtual meeting, to produce the final recommendations. A health equity framework was applied to all aspects of the adolopment process including the EtD tables, survey responses, and virtual meeting discussion. RESULTS: The survey identified that 7 of the 19 recommendations required rigorous discussion. Seventy-five percent of working group members attended the virtual meeting to discuss controversial topics as they pertained to the Canadian environment, including timing to first eye exam, frequency of screening, escalation criteria for systemic and biologic therapy, and the role of nonbiologic therapies. Equity issues related to access to care and advanced therapeutics across Canadian provinces and territories were highlighted. Following the virtual meeting, 5 recommendations were adapted, 2 recommendations were removed, and 1 was developed de novo. CONCLUSION: Recommendations for JIA-associated uveitis were adapted to the Canadian context by a working group of pediatric rheumatologists, ophthalmologists with expertise in the management of uveitis, and parent/patient input, taking into consideration cost, equity, and access.

Unsuspected and misdiagnosed posterior uveal melanoma following enucleation and evisceration in Ottawa-Gatineau.

OBJECTIVE: There is a gap in the recent literature on the topic of clinically misdiagnosed and unsuspected posterior uveal melanomas (PUM) with a calculation of the frequency of these events for a specific geographical area. As the only ophthalmic pathology laboratory in our region, we determined the rate of these outcomes over a 16-year period. METHODS: We retrospectively reviewed 2558 consecutive ophthalmic pathologic specimens in the Ottawa-Gatineau region, of which 334 were eviscerations and 227 were enucleations. We calculated the frequency of clinically misdiagnosed PUM and of clinically unsuspected PUM. We also determined the rate of uveal melanoma undergoing enucleation. RESULTS: From 100 diagnoses of PUM, 2 (2.0%) cases were clinically unsuspected and 2 (2.0%) cases were clinically misdiagnosed. The rate of uveal melanoma undergoing enucleation was 5.6 cases per 1 000 000 of population per annum. From 2009 to 2012, the incidence of this event was 3.8 cases per 1 000 000 per annum, which was lower than the previous three 4-year increments. CONCLUSIONS: We present the first and only single-centred, population-based data on the rates of unsuspected PUM and of clinical misdiagnosis of PUM in the era of modern diagnostic imaging. Our rate of clinical misdiagnosis is within the range of recent reports of this event. Unsuspected PUM occurred at a rate substantially lower than previously published. The incidence of uveal melanoma undergoing enucleation has decreased despite an increase in population, which reflects a shift in management from enucleation to radiation therapy.

Reactivation of Herpes Zoster Keratitis With Corneal Perforation After Zoster Vaccination.

PURPOSE: We present a case of reactivated herpes zoster keratouveitis of 6 years duration with corneal perforation requiring penetrating keratoplasty shortly after inoculation with herpes zoster vaccine (Zostavax, Merck, Quebec, Canada). METHODS: Retrospective case report. RESULTS: A 67-year-old woman with a 5-year history of recurrent unilateral herpes zoster keratouveitis in her right eye presented with another recurrence 2 weeks after Zostavax vaccination. Three months later, she developed descemetocele and 2 months afterward, corneal perforation, which was managed by penetrating keratoplasty. Immunohistopathological examination disclosed positive staining for varicella zoster virus in most of the keratocytes adjacent to the descemetocele and perforation, most vividly in the deeper two-thirds of the stroma where the keratocytes were most dense, but not in corneal epithelium or endothelium. Electron microscopic examination showed universally severely degenerated corneal keratocytes in the corneal stroma adjacent to the perforation with variable numbers of herpes virus capsids present in half of these cells. Only a rare normal-appearing keratocyte was identified in the more peripheral corneal stroma. CONCLUSIONS: We present a case of reactivation of herpes keratouveitis shortly after vaccination with Zostavax in a patient with previous herpes zoster ophthalmicus. We demonstrate, for the first time, ultrastructural evidence consistent with inactive virus capsids in diffusely degenerated keratocytes in the extracted corneal tissue.

Safety and comparative costs of preoperative assessments for cataract surgery: traditional mandatory assessment versus a novel graded assessment system.

PURPOSE: We conducted this study to evaluate the safety and costs of traditional mandatory preoperative assessment for cataract surgery patients compared with a novel graded preoperative assessment system. METHODS: Patients were recruited at a high-volume surgical centre from May to November 2013. Patients completed a health-related questionnaire which allowed for a graded preoperative assessment of all participants. Based on responses to the questionnaire, patients were classified preoperatively into a) low-risk patients not requiring a preoperative assessment and b) high-risk patients requiring this assessment. Anesthesiologists still assessed all patients immediately before surgery but with staff blinded to preoperative assessment information for low-risk patients. Observed complication rates and costs were compared with those expected in the mandatory assessment system. RESULTS: We examined 3,347 cataract surgeries on 2,766 patients and categorized 59.9% of patients as low risk. In the graded system cohort, there were no major complications and a low rate of minor complications occurred. Wherever a complication occurred in a low-risk patient, the anesthesiologist doubted that the preoperative assessment information would have prevented the complication. If implemented, the graded system would save approximately 4,414 preoperative assessments per year in our region, with an associated cost of approximately $40.00 per surgery, or $359,000 in total. The cost to prevent a single minor complication with the mandatory system was approximately $8,976, with a number needed to treat of 223. CONCLUSION: The graded system resulted in no major complications and a low rate of minor complications. The information obtained from the mandatory assessment is unlikely to prevent complications. Additionally, the cost effectiveness of the mandatory system was poor. This novel graded preoperative assessment system for cataract surgery patients can save time and resources by eliminating unnecessary patient visits.

Secondary corneal amyloidosis after perforating corneal trauma: A series of 5 cases and review of the literature.

We retrospectively reviewed the clinical and surgical histories of 5 patients with traumatic secondary corneal amyloidosis, a relatively rare sequela of nonsurgical and surgical perforating corneal trauma. Four had history of nonsurgical trauma, and 1 had surgical trauma to the cornea. Three specimens were obtained by penetrating keratoplasties and 2 by excision of the cornea during evisceration of the ocular contents. All the corneal specimens showed full-thickness scars of a prior perforating wound with congophilic amyloid deposits that exhibited apple-green birefringence under polarized light and dichroism. All cases had variable degrees of predominantly chronic nongranulomatous inflammation. Ultrastructural examination in 1 patient disclosed 8-nm diameter fibrils in disarray, consistent with amyloid. Amyloid P immunostaining was positive in all 3 patients tested for this protein.

Dacryops: a series of 5 cases and a proposed pathogenesis.

IMPORTANCE: Lacrimal gland ductal cysts (dacryops) are uncommon, occurring anywhere that lacrimal gland tissue is present. While dacryops has long been recognized, its pathogenesis has not been well established. OBSERVATIONS: Five cases of dacryops were identified at the Ottawa Hospital over a 3-year period (2009-2012). Histopathological examination showed immunoreactivity to IgA on the luminal surface of the epithelial ductal cyst and mild chronic inflammation in the underlying stroma or lacrimal gland. CONCLUSIONS AND RELEVANCE: Our results suggest that a multifactorial mechanism leads to dacryops including chronic inflammation, an immune response, and IgA hypersecretion with an osmotic effect, all contributing to the cyst formation. Further research on the pathogenesis is recommended.

Bilateral necrobiotic xanthogranuloma of the eyelids followed by a diagnosis of multiple myeloma 20 years later.

A uniquely indolent case of necrobiotic xanthogranuloma with bilateral, periorbital involvement was presented. This patient presented with cutaneous eyelid lesions of 20 years' duration. Although symptomless, the patient underwent testing for hematologic malignancy, which led to a diagnosis of multiple myeloma. A review of the literature revealed that this is the longest interval between the onset of the skin manifestations and the diagnosis of the systemic malignancy in this rare disease.

Histochemical analysis and immunohistochemical profile of mucoepidermoid carcinoma of the conjunctiva.

PURPOSE: To elucidate the distinct histochemical and immunohistochemical profile of mucoepidermoid carcinoma of the conjunctiva (MECC) and to determine which combination of stains is most useful in diagnosing MECC and differentiating it from squamous cell carcinoma of the conjunctiva (SCC) in cases where the clinical or cytological findings are not definitive. METHODS: Eight specimen of MECC from 4 patients and 4 specimens of SCC from 4 patients were examined using a variety of special stains and immunohistochemical markers. The results were then analyzed for usefulness in diagnosing MECC. RESULTS: The most useful markers in diagnosing MECC and differentiating it from SCC are mucicarmine, colloidal iron, and alcian blue all with sensitivities of 88%, and a specificity of 100%; CEA with a sensitivity of 83% and a specificity of 75%; and, mucin-1 with a sensitivity of 100% and a specificity of 25%, but which showed a distinct pattern of staining of MECC when compared to SCC. In our series, the sensitivity of the CK7+/CK20- combination for MECC was only 38%. CONCLUSIONS: The most useful stains in ruling out SCC in a suspected case of MECC were shown to be mucicarmine and the glycosaminoglycan (GAG) stains. However, in cases where mucicarmine and the GAG stains are negative or difficult to interpret and there is suspicion of a diagnosis of MECC, CEA and mucin-1 may be helpful for this diagnosis. The findings of CK7+/CK20- also may be of assistance, but are not as sensitive when compared to analogous salivary gland lesions, when differentiating MECC from SCC.

Bilateral benign reactive lymphoid hyperplasia of the conjunctiva in a pediatric patient.

A 13-year-old boy presented with asymptomatic, bilateral, nasal bulbar conjunctival lesions. Excisional biopsy of the larger lesion showed a mature, mixed lymphocytic infiltrate with germinal centers, consistent with a diagnosis of benign reactive lymphoid hyperplasia, a rare condition in children.