Primary Periocular Orofacial Granulomatosis: Case Series and Systematic Review.

PURPOSE: There is no known optimal treatment for primary periocular orofacial granulomatosis (PPOFG), a disorder that results in periocular edema. This case series and systematic review identifies management strategies and their reported improvement. METHODS: Systematic review and case series. PubMed and MEDLINE databases were searched following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines to identify published cases of PPOFG. Cases were included when edema involved the periocular tissues and when a biopsy was interpreted to be PPOFG. Cases were excluded when edema did not involve the periocular tissues or when the edema was secondary to another process. The electronic medical records of the pathology department were studied to identify cases that were in keeping with PPOFG. The clinical charts were examined to confirm the diagnosis and provide a local case series. RESULTS: There are 38 published cases of PPOFG. An additional 9 cases were identified locally. These cases were combined and analyzed. Most PPOFG has eyelid swelling in isolation, without other facial swelling (36/47; 76.6%). It is most commonly a bilateral disease (30/47; 63.8%). Fissured tongue and facial nerve palsy occur, just as they do in other cases of orofacial granulomatosis. Treatment with surgical debulking or intralesional steroids resulted in high rates of symptomatic improvement of eyelid swelling, but recurrences were common. CONCLUSIONS: In light of no curative or highly successful treatment currently available, intralesional steroids and/or surgical debulking are therapies in the treatment of eyelid swelling associated with PPOFG that demonstrate reasonable short- and medium-term results. There is no established therapy that can offer disease remission or long-term symptom improvement.

Bacterial pyomyositis of the extraocular muscles: case report and systematic review.

OBJECTIVE: To describe the manifestations and treatment of extraocular muscle (EOM) bacterial pyomyositis. DESIGN: A systematic review following PRISMA guidelines and a case report. METHODS: PubMed and MEDLINE databases were searched for case reports and case series of EOM pyomyositis using the term "extraocular muscle" combined "pyomyositis" and "abscess". Patients were included as bacterial pyomyositis of the EOMs when there was a response to antibiotics alone or if a biopsy was consistent with the diagnosis. Patients were excluded when pyomyositis did not involve the EOMs or when diagnostic tests or treatment were not in keeping with the diagnosis of bacterial pyomyositis. An additional patient with bacterial myositis of the EOMs, treated locally, was added to the cases identified in the systematic review. Cases were grouped for analysis. RESULTS: There are 15 published cases of EOM bacterial pyomyositis including the one reported in this paper. Bacterial pyomyositis of the EOMs typically affects young males and is caused by Staphylococcus species. Most patients present with ophthalmoplegia (12/15; 80%), periocular edema (11/15; 73.3%), decreased vision (9/15; 60%) and proptosis (7/15; 46.7%). Treatment involves antibiotics alone or in combination with surgical drainage. CONCLUSIONS: Bacterial pyomyositis of the EOM presents with the same signs as orbital cellulitis. Radiographic imaging identifies a hypodense lesion with peripheral ring enhancement within the EOM. An approach to cystoid lesions of the EOMs is helpful in reaching the diagnosis. Cases can be resolved with antibiotics aimed at treating Staphylococcus, and surgical drainage may be required.

Decrease in Anti-VEGF Injections After Post-injection Endophthalmitis in Patients With Neovascular Age-Related Macular Degeneration.

Introduction: To evaluate the effect of antivascular endothelial growth factor (anti-VEGF)-related endophthalmitis on intravitreal injection (IVI) frequency in patients with neovascular age-related macular degeneration (nAMD). Methods: A retrospective chart review was performed of all cases of post IVI endophthalmitis that occurred in Edmonton, Alberta, Canada, between 2012 and 2019. Contralateral eyes affected by nAMD but without endophthalmitis served as a control group. The main outcome measures were the frequency of anti-VEGF injections, visual acuity, and activity of choroidal neovascularization before and after endophthalmitis. Results: Seventeen eyes met the inclusion criteria, 2 (12%) of which never resumed IVI after endophthalmitis because of the quiescence of disease. Post-endophthalmitis eyes received IVI less frequently in the 1 year after endophthalmitis (mean 0.52 ± 0.42 IVI/month) than those that received IVI 1 year before endophthalmitis (1.09 ± 0.36 IVI/month) (P = .001). The 17 contralateral eyes also received anti-VEGF injections less frequently after endophthalmitis than before (P = .001). There was no significant change in optical coherence tomography markers of disease activity in cases or controls. Conclusions: In patients with nAMD, endophthalmitis resolution is associated with a decrease in anti-VEGF injection frequency. The same decrease in anti-VEGF injection frequency is also seen in contralateral eyes unaffected by endophthalmitis. Markers of disease activity remain unchanged in both eyes, suggesting disease quiescence despite reduced IVI frequency.

Orbital mass as the presenting symptom of diffuse large B-cell lymphoma causing spontaneous tumor lysis syndrome and rapid multisystem organ failure.

A 68-year-old male presented with a one-month history of progressive proptosis and vision loss in the left eye. Examination of the left eye showed visual acuity of NLP, marked relative proptosis of 10 mm, and complete external ophthalmoplegia. CT orbits showed an extensive left orbital lesion with proptosis. Urgent orbital biopsy was undertaken. Intraoperatively, the patient developed new atrial flutter and fever. Bloodwork revealed metabolic derangements suggestive of tumor lysis syndrome. Systemic evaluation revealed a large tumor burden involving the retroperitoneal space. Histopathology of the orbital specimen showed non-germinal center diffuse large B-cell lymphoma. The patient passed away 3 days postoperatively due to rapidly progressive multisystem organ failure. Our case demonstrates an unusually aggressive presentation of DLBCL in which orbital mass was the first presentation of spontaneous tumor lysis syndrome owing to large systemic tumor burden.

Cilioretinal Artery Occlusion in Posterior Scleritis Secondary to Ankylosing Spondylitis.

The authors describe the case of a 25-year-old male who presented with a cilioretinal artery occlusion secondary to posterior scleritis. The patient had a history of juvenile spondyloarthritis that evolved into ankylosing spondylitis. Cilioretinal artery occlusion is a rare complication of posterior scleritis, having only been described once previously in the literature. This is the first reported case of a cilioretinal artery occlusion in posterior scleritis that was associated with an underlying systemic disease. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:102-106.].

Nonosseous Periocular Manifestations of Langerhans Cell Histiocytosis: A Case Report and Systematic Review.

PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation that typically involves bone. It can be diagnostically challenging when LCH presents without bony involvement, leading to delays in diagnosis and treatment. In this study, the periocular manifestations of LCH in cases where the underlying orbital bones are not involved are described through a systematic review. METHODS: A systematic review of the literature was performed to capture all cases of LCH that involved the periocular region but not the underlying orbital bones. These included LCH cases that involved the periocular skin, the ocular surface, and the orbital tissue. The authors also highlight an additional case where LCH presented with periocular edema and multifocal, nodular conjunctival lesions. RESULT: This review illustrates that LCH rarely presents with periocular infiltration without orbital bone involvement. In these atypical cases, LCH can present as an eyelid mass, a chalazion-like lesion, generalized periocular swelling, ocular surface lesions, or infiltration of any orbital structure. Ocular surface LCH has a higher rate of recurrence than other periocular LCH. Orbital LCH can involve any tissue including extraocular muscles, the lacrimal gland, or indistinct areas within the orbit. CONCLUSIONS: LCH is a clinicopathologic diagnosis. Although most cases involve the bone, any soft tissue can be involved. Biopsy is required to confirm the diagnosis of this heterogeneous disease.

Severe retinal degeneration in a patient with Canavan disease.

Background: Canavan disease is an autosomal recessive, neurodegenerative disorder caused by mutations in ASPA, a gene encoding the enzyme aspartoacylase. Patients present with macrocephaly, developmental delay, hypotonia, vision impairment and accumulation of N-acetylaspartic acid. Progressive white matter changes occur in the central nervous system. The disorder is often fatal in early childhood, but milder forms exist. Materials and methods: Case report. Results: We present the case of a 31-year-old male with mild/juvenile Canavan disease who had severe vision loss due to a retinal degeneration resembling retinitis pigmentosa. Prior to this case, vision loss in Canavan disease had been attributed to optic atrophy based on fundoscopic evidence of optic nerve pallor. Investigations for an alternative cause for our patient's retinal degeneration were non-revealing. Conclusion: We wonder if retinal degeneration may not have been previously recognized as a feature of Canavan disease. We highlight findings from animal models of Canavan disease to further support the association between Canavan disease and retinal degeneration.

Combination Treatment with Rituximab and Bortezomib in a Patient with Non-Paraneoplastic Autoimmune Retinopathy.

PURPOSE: To describe outcomes of combined rituximab and bortezomib treatment for non-paraneoplastic autoimmune retinopathy. CASE: A 37-year-old female developed photopsias and reduced vision. Electroretinography, optical coherence tomography, and positive serum anti-retinal antibodies were consistent with autoimmune retinopathy. A negative malignancy work-up specified her non-paraneoplastic presentation. Given absence of response to periocular steroids, azathioprine, and methotrexate, a combination of rituximab and bortezomib was initiated as fifth-line therapy. RESULTS: There was no significant improvement in the patient's symptoms or visual function following treatment. The full field electroretinogram amplitudes were reduced with progressive outer retinal degeneration evident on optical coherence tomography. Post-treatment anti-retinal antibody testing demonstrated the persistence of antibodies and revealed additional antibodies not previously detected. CONCLUSION: Combined rituximab and bortezomib treatment did not result in significant clinical improvement and there was evidence of disease progression. Further prospective studies are required to assess the efficacy of immunotherapy in patients with autoimmune retinopathy.

Tube shunt surgery in pig eyes: a wet lab teaching model.

OBJECTIVE: To describe a teaching model for aqueous tube shunt surgery using freshly enucleated pig eyes. DESIGN: A descriptive methods report, containing a small prospective noncomparative interventional case series. PARTICIPANTS: Six ophthalmology residents from the University of Alberta program. METHODS: A descriptive report outlines the materials and methods required for creating a surgical wet lab that teaches aqueous draining device implantation. Residents practiced each step associated with the insertion of plated aqueous tube shunt devices in porcine eyes. RESULTS: The porcine model worked well to demonstrate and perform steps associated with tube implant surgery. Trainee comfort improved in all surgical steps practiced during the session when reassessed at 3-month follow-up: priming and anchoring the drainage device (p = 0.042), inserting the tube into the eye (p = 0.025), creating and securing a scleral patch graft (p = 0.034), and closure of the conjunctiva (p = 0.034). Overall confidence in performing tube shunt surgery also remained above baseline at follow-up (p = 0.042). CONCLUSIONS: Implantation of tube shunt devices in the porcine model closely resembles surgery in human eyes. Practicing each step associated with tube shunt surgery on porcine eyes in a supervised wet-lab environment improves trainee confidence in the procedure.

VITRECTOMY WITH SUBRETINAL TISSUE PLASMINOGEN ACTIVATOR AND GAS TAMPONADE FOR SUBFOVEAL HEMORRHAGE: Prognostic Factors and Clinical Outcomes.

PURPOSE: To study the prognostic factors and clinical outcomes of patients who underwent pars plana vitrectomy, subretinal injection of tissue plasminogen activator, and gas tamponade for the treatment of subfoveal hemorrhage (SFH). METHODS: A retrospective noncomparative interventional case series. RESULTS: Seventy-eight eyes from 77 patients were included. A total of 84.6% of eyes developed SFH from age-related macular degeneration. Partial or complete displacement of the SFH was achieved in 91.5% of eyes within 2 months of surgery. Visual acuity improved from 20/1,449 preoperatively to 20/390 after a mean follow-up time of 6.3 months, corresponding to approximately 5 lines of Snellen acuity improvement (P < 0.001). Better visual acuity was associated with the absence of age-related macular degeneration (P = 0.02) and less hemorrhage superior to the fovea (P < 0.001). Final visual acuity was not associated with the area of SFH (P = 0.17), use of anticoagulants (P = 0.14), or visibility of the ellipsoid layer by optical coherence tomography (P = 0.64). Nine patients (11.5%) developed a recurrence of SFH within the follow-up period. Recurrence of SFH was not associated with concurrent anticoagulant therapy (P = 0.52). CONCLUSION: An etiology other than age-related macular degeneration with less hemorrhage superior to the fovea predicts a better outcome in patients with SFH treated with pars plana vitrectomy, subretinal tissue plasminogen activator, and gas tamponade.

A diagnosis of Stevens-Johnson Syndrome (SJS) in a patient presenting with superficial keratitis.

PURPOSE: To describe a case of Stevens-Johnson syndrome (SJS) diagnosed in a patient presenting with primarily ocular findings where SJS had not been initially suspected. OBSERVATIONS: A 23-year-old female presented with a 2 day history of bilateral eye pain, conjunctival injection, decreased visual acuity, and photophobia in the context of a 4 day history of fever, headache, and sore throat. She was found to have bilateral superficial keratitis and treated for suspected early infectious keratitis secondary to extended contact lens wear. She returned the next day with worsening visual symptoms, a new macular rash over her upper torso, and new ulcerating lesions over her buccal and perioral tissue. The patient was diagnosed with SJS. She was successfully treated using systemic cyclosporine with antibiotics and steroid eye drops. CONCLUSIONS AND IMPORTANCE: Ophthalmologists may be the first physicians to diagnose SJS, a life-threatening condition that can initially present with non-specific viral prodromal symptoms and ocular signs alone. This case emphasizes the importance of considering a patient's entire clinical history, especially when the presentation is atypical and the diagnosis is not obviously apparent.