The role of biopsy in lacrimal gland inflammation: A clinicopathologic study.

PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone. As was our routine practice, all these patients underwent lacrimal gland biopsy before starting any treatment. RESULTS: The histopathologic findings of the 60 patients showed that 37 (61.7%) had identifiable types of lacrimal inflammation including 10 with Sjogren's syndrome, seven with sarcoidal reaction, six with feature of granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis), five with lymphoma, two with sclerosing inflammation, two with IgG4-related dacryoadenitis, and one patient each with infectious dacryoadenitis, myoepithelial carcinoma, xanthogranuloma, eosinophilic angiocentric fibrosis, and eosinophilic allergic granulomatous nodule. The histopathologic findings of the remaining 23 (38.3%) patients showed nonspecific inflammation of the lacrimal gland. 23 patients (38.3%) had associated systemic diseases. 48 patients (80%) were treated successfully and 10 (16.7%) had recurrence of inflammation. CONCLUSIONS: We recommend that in patients presenting with lacrimal gland inflammation (i.e., erythema, edema, tenderness) in which the specific diagnosis cannot be made clinically and on imaging, biopsy is warranted for accurate diagnosis and appropriate treatment. We found that the majority of these patients (61.7%) had specific histopathology, and 38% had systemic diseases.

Long-term result of autologous cultivated oral mucosal epithelial transplantation for severe ocular surface disease.

The present study aimed to investigate the clinical outcomes of autologous cultivated oral mucosal epithelial transplantation (COMET) on human amniotic membrane (AM) for corneal limbal stem cell deficiency (LSCD). In this prospective, noncomparative case series, 20 eyes (18 patients) with bilateral severe ocular surface disease were chosen to undergo COMET on human AM. The primary outcome was clinical success, and the secondary outcomes were the best-corrected visual acuity difference, corneal opacification, symblepharon formation, and complications. The mean patient age was 48.2 ± 15.5 years. The mean follow-up time was 31.9 ± 12.1 months (range 8-50 months). All except one eye exhibited complete epithelialization within the first postoperative week. A successful clinical outcome, defined as a stable ocular surface without epithelial defects, a clear cornea without fibrovascular tissue invasion at the pupillary area, and no or mild ocular surface inflammation, was obtained in 15 of 20 eyes (75 %). The clinical success rate at 1 year was 79.3 %, and that at 4 years (end of follow-up) was 70.5 %. Fourteen of 20 (70 %) eyes exhibited improvement in visual acuity after COMET, and some required subsequent cataract surgery (2 eyes), penetrating keratoplasty (3 eyes), or keratoprosthesis implantation (1 eye). Preoperative symblepharon was eliminated in most eyes (8 of 13, 61.5 %) after COMET combined with eyelid reconstruction when needed. The only complication was corneal perforation (1 eye) induced by a severe eyelid abnormality; treatment with a tectonic corneal graft was successful. COMET can successfully restore ocular surface damage in most eyes with corneal LSCD.

Signet-ring cell/histiocytoid carcinoma of the eyelid: a case report and review of the literature.

Signet-ring cell/histiocytoid carcinomas of the eyelid are rare, slow-growing, and locally aggressive tumors. They predominantly affect elderly men and clinically resemble chronic inflammatory process such as blepharitis and chalazion. To date, the histogenesis of the tumors is still controversial. Only 27 cases of primary signet-ring cell/histiocytoid carcinomas of the eyelid are published in the literature. Local recurrence and distant metastasis occur in 8 and 7 cases, respectively. We report clinical, radiographic, and pathological features of a case of signet-ring cell/histiocytoid carcinoma of the eyelid with review of relevant literature.

NK/T-cell lymphoma of the nasal cavity causing contralateral dacryoadenitis.

PURPOSE: To report a case of NK/T-cell lymphoma of the nasal cavity with contralateral lacrimal gland involvement. METHODS: Observational case report. RESULTS: A 39-year-old woman with a 5-month history of right fungal rhinosinusitis was referred to our hospital. A nasal mucosal biopsy performed before referral was consistent with Aspergillus sp. Despite surgical and parenteral antifungal treatment, the symptoms continued to deteriorate. On admission, the ophthalmic evaluation showed inflammation over the left lacrimal gland area. The fundus examination revealed bilateral subretinal infiltration. Computed tomography scans of the orbits and sinuses showed mucosal thickening of the right nasal mucosa and sinuses. There was heterogeneous enhancement and infiltration of the left lacrimal gland. Lacrimal gland biopsy and repeated biopsies of the nasal cavity and sinus tissue were performed. Immunohistopathology of the lacrimal glands and nasal mucosa showed extranodal nasal-type NK/T-cell lymphoma. The patient was treated with cyclophosphamide, vincristine, adriamycin, prednisolone (3 cycles), and intrathecal methotrexate. The patient developed sepsis and died 2 months after initiation of chemotherapy. CONCLUSION: Dacryoadenitis can be a clinical manifestation of NK/T-cell lymphoma. To our knowledge, this is the first reported case of nasal NK/T-cell lymphoma with contralateral dacryoadenitis.

Entomophthoramycosis: a rare fungal orbital infection presenting with dacryocystitis.

We report a case of a rare fungal orbital infection in an infant presenting with dacryocystitis. The causative organism was Conidiobolus sp. of the order Entomophthorales. There is no standard treatment for entomophthoramycosis. Our patient responded well to combined antifungal therapy without aggressive surgical débridement.

Orbital cellulitis and endophthalmitis in pseudomonas septicemia.

We report a case of concurrent orbital cellulitis and endophthalmitis that resulted from endogenous complications of community-acquired Pseudomonas aeruginosa bacteremia in an apparently healthy individual. Pseudomonas pneumonia and extensive focal skin lesions of ecthyma gangrenosum also complicated the condition. The presence of drug-induced neutropenia was a risk factor in this patient. Simultaneous orbital cellulitis and endophthalmitis developed and rapidly progressed. Intravenous, intravitreal, and topical antibiotics were administered along with frequent eye wash with normal saline to dilute copious purulent discharge from a deep subcutaneous abscess of lower eyelid. Because of the exocellular products of Pseudomonas aeruginosa, the sclera and corneal stroma were degraded, resulting in nearly perforated cornea. Tarsoconjunctival flap from the upper eyelid was performed to reconstruct the thinning areas. After the infection was controlled, the patient's ultimate visual acuity was light perception.

Microbiology of primary acquired nasolacrimal duct obstruction: simple epiphora, acute dacryocystitis, and chronic dacryocystitis.

PURPOSE: The aim of this study was to determine the microbiology of primary acquired nasolacrimal duct obstruction (PANDO) and its antimicrobial susceptibilities. METHODS: Ninety-three patients (100 eyes) diagnosed with PANDO, categorized as acute, chronic dacryocystitis, or simple epiphora, were prospectively enrolled. Lacrimal sac contents were cultured for aerobic and anaerobic bacteria and fungi. Cultured organisms were identified, and antimicrobial susceptibility testing was performed for aerobic bacteria. RESULTS: Seventy-nine of the 100 samples were culture positive. One hundred twenty-seven organisms were isolated, and 29 different species were identified. Most microorganisms were Gram-positive bacteria (45 samples or 57.0% of all positive culture samples), whereas Gram-negative bacteria, anaerobic bacteria, and fungi were found in 39 (49.4%), 24 (30.4%), and four samples (5.1%), respectively. The most frequently isolated group was coagulase-negative staphylococci (27.8%), followed by nonspore-forming Gram-positive rods (anaerobe) (17.7%) and Pseudomonas aeruginosa (15.2%). Of the 100 samples, five, 45, and 50 samples were obtained from patients with acute dacryocystitis, chronic dacryocystitis, and simple epiphora, respectively. Subgroup analysis showed that Gram-negative organisms were isolated more frequently from the chronic dacryocystitis subgroup than from the simple epiphora subgroup (P=0.012). Antimicrobial susceptibility testing demonstrated that ciprofloxacin was the most effective drug against all Gram-positive and Gram-negative organisms. CONCLUSION: Patients with PANDO, with or without clinical signs of lacrimal infection, were culture positive. Gram-negative organisms were frequently isolated, which were different from previous studies. Ciprofloxacin was the most effective agent against all Gram-positive and Gram-negative organisms.

Cavernous venous malformations of the orbit (so-called cavernous haemangioma): a comprehensive evaluation of their clinical, imaging and histologic nature.

PURPOSE: The purpose of this investigation is to describe the clinical, imaging, histologic and flow dynamic characteristics of orbital cavernous haemangioma. METHODS: In this clinicopathologic series, clinical features were obtained from patient records. All imaging studies were reviewed. All specimens were reviewed with haematoxylin and eosin, and 10 were subject to a staining protocol including: Movat Pantachrome, periodic acid Schiff, D2-40, CD31, GLUT-1, Ki-67, vascular endothelial growth factor receptor 1 (VEGF-r1) (flt-1), VEGF-r2 (Flk-1), VEGF, anti-smooth muscle actin (SMA), CD20, CD4, CD8 and CD68. Imaging and pathology were reviewed in a systematic fashion. RESULTS: Clinically, lesions were more common in middle-aged females presenting with axial proptosis and pain. One-third demonstrated signs of optic nerve dysfunction. Dynamic imaging revealed focal early and diffuse late enhancement. Lesions demonstrated slow growth at 0.2 cm3/year. Histologically, all lesions demonstrated large vascular channels with mature-appearing endothelium and abundant stroma. Three salient features were noted and characterised: thrombosis, nests of perivascular hypercellularity and expanded stromal elements. Acute thrombosis was a feature of each specimen (<10% of channels). Fibrin clots were lined by a layer of CD31+ endothelium. Perivascular hypercellular areas stained uniformly with CD31 and less so with VEGFr2. Additionally, focal areas of Ki67+ and CD68+ cells were found in these regions. Expanded stroma contained CD31+ microcapillary networks and stained diffusely with anti-SMA. CONCLUSIONS: Cavernous haemangioma demonstrate clinical features and growth characteristics of a benign mass. Dynamic imaging highlights their slow flow vascular nature. Histologically, the hypercellularity and stromal changes identified can be understood within the pathogenic context of thrombosis and recanalisation in an organised lesion.