Paediatric orbital conjunctival epithelial cyst with positive asialotransferrin.

Conjunctival orbital cysts are rare; they are typically either conjunctival dermoid or conjunctival epithelial cysts - congenital or acquired (inclusion). We describe the case of a 15-month-old girl presenting with strabismus and proptosis who had a retrobulbar intraconal cystic lesion displacing the optic nerve, with an adjacent middle cranial fossa anomaly. Aspiration of the orbital cyst tested positive for asialotransferrin, raising the suspicion of a direct communication with cerebrospinal fluid (CSF). Subsequent fine cut CT scanning disproved any connection with the intracranial space, and the cyst was excised complete and intact. Histopathology showed a conjunctival epithelial cyst. To our knowledge, this is the first case report in the literature of an asialotransferrin positive pediatric orbital conjunctival epithelial cyst. It is of clinical relevance as it explores the possibility of either a false positive asialotransferrin or potentially a prior developmental communication with the subarachnoid space. These two diagnostic possibilities are discussed.

Repurposing blue laser autofluorescence to measure ocular sun exposure.

IMPORTANCE: Excessive ocular sun exposure is linked to various eye pathologies. Conjunctival ultraviolet autofluorescence (CUVAF) is a method of detecting sun-related conjunctival damage; however, the custom-built camera system required is not readily available. BACKGROUND: We investigated whether blue laser autofluorescence (BAF) on a commonly used confocal scanning laser ophthalmoscope (cSLO) can be utilized to measure CUVAF area. DESIGN: Cross-sectional evaluation of a diagnostic technology at a medical research institute. PARTICIPANTS: Sixty-four participants recruited from three on-going observational eye studies in Western Australia. METHODS: All participants had four images, two of each eye, captured using the CUVAF camera and BAF on the same day. Participants with pterygium or poor quality images were excluded from the analysis. Two graders measured CUVAF area in each image twice. CUVAF area measured by BAF was then compared to measurements determined with the conventional camera system. MAIN OUTCOME MEASURES: CUVAF area. RESULTS: After exclusions, 50 participants' images were analysed. Intra- and inter-observer repeatability were similar between the two systems. When comparing CUVAF area measured by BAF to the camera measurement, grader 1 had a mean difference of +1.00 mm2 , with 95% limits of agreement -5.75 to 7.77 mm2 . Grader 2 had a mean difference of +0.21mm2 , with 95% limits of agreement -7.22 to 7.64 mm2 . CONCLUSIONS AND RELEVANCE: BAF on a commercially available cSLO is a valid method for measuring CUVAF area. This finding provides broader opportunity for identifying, monitoring and educating patients with sun-exposure-related ocular conditions and for researching the ocular impacts of sun exposure.

Orbital and peri-orbital amyloidosis: a report of four cases.

Our objective was to describe our experience with orbital amyloidosis, and illustrate the different forms of presentation. This was a retrospective case series of four patients with biopsy-proven orbital amyloi- dosis, over the period from 2014 to 2016. We describe its diagnostic and clinical characteristics, management and systemic study. The series comprised three women and one man of mean age 52 ± 9.4 years. Affected sites were the lacrimal gland, tarsal conjunctiva, lacrimal sac and orbit. In three of the four patients, calcifications were observed. Three patients had associated ptosis. The patient with orbital involvement suffered an unusual vascular complication during surgery and systemic disease was detected. Management included debulking and complete resection of the lesion. In conclusion, orbital amyloidosis presents as a wide variety of forms. Its diagnosis is biopsy-based. Calcifications in biopsy specimens or images should raise suspicion of amyloidosis. It is important to always check for systemic amyloidosis.

Evaluation of lymphatic vessel dilatations by anterior segment swept-source optical coherence tomography: case report.

BACKGROUND: Conjunctival lymphangiectasia is a rare condition presumably caused by the obstruction of lymphatic channels or by an abnormal connection between conjunctival lymphatic and blood vessels. Diagnosis is based on clinical appearance and histology. We report a case of conjunctival lymphangiectasia in which anterior segment optical coherence tomography (OCT) was used to assist the diagnosis and the planning of the biopsy location. CASE PRESENTATION: A 31-year-old woman was referred with repeated episodes of conjunctival "hemorrhages" and chemosis with extended recovery periods over the last months. Other symptoms were dryness, redness, burning sensation and itching. Photo documentation, anterior segment OCT, ultrasound, computer tomography (CT) and magnetic resonance imaging (MRI) of the brain were performed. MRI revealed dilated atypical Virchow-Robin space (VRS). Conjunctival biopsy was taken and the location of the biopsy was selected based on OCT findings. Based on the clinical appearance we suspected the case to be conjunctival lymphangiectasia or lymphangioma. Histology and immunhistochemistry confirmed the diagnosis of conjunctival lymphangiectasia. CONCLUSIONS: Anterior segment OCT is a non-invasive tool, useful in the evaluation of conjunctival lesions and planning surgery.

Orbital Conjunctival Cyst Associated With the Superior Rectus-Levator Muscles: A Clinicopathologic Study.

A 55-year-old woman had a right orbital cyst detected incidentally on radiographic imaging. The patient's symptoms were mild and included intermittent pain and vertical diplopia; the patient was not aware of any visual decline. There was a palpable mass beneath the superior orbital rim. Radiographic imaging revealed a well-demarcated cystic lesion in the right superior orbit between the levator palpebrae superioris and superior rectus muscles. The mass was completely excised via a transconjunctival approach. Histopathologic evaluation disclosed a conjunctival cyst lined by nonkeratinized squamous epithelium with scattered, rare goblet cells. This case combined with 5 other similar reported cases suggests that an intermuscular cyst located in the superior rectus-levator complex is most likely of congenital embryonic conjunctival origin.

Medical Decision Making: Hyposphagma Prior to Intravenous Tissue Plasminogen Activator in Acute Ischemic Stroke.

The decision to administer intravenous tissue plasminogen activator (IV tPA) is based on standard exclusion and inclusion criteria, which include laboratories, imaging, and time of last known well. When patients present with a clinical scenario that is not addressed in these standards, the decision to administer IV tPA is more complex. We present a case of a patient with an acute stroke syndrome that also included acute subconjunctival hemorrhage (i.e., hyposphagma). We provide the medical decision making that occurred prior to the administration. Ultimately, the finding of hyposphagma should not disqualify eligible patients from receiving IV tPA.

[The significance of imaging in a case of intraorbital cystic tumor]. / Influenta investigatiilor imagistice într-un caz de tumora chistica intraorbitara.

Conjunctival inclusion cyst represents a congenital or, in most cases, an acquired disorder. The most frequent cause of an acquired conjunctival cyst is the implantation of conjunctival epithelium after surgical interventions or ocular trauma. Usually, these cysts are located supero-medially, with a stationary evolution, without a progression in dimension, but in some cases can evolve into enormous translucent cysts. Histologically, they are lined by stratified, nonkeratinized, squamous epithelium and contain desquamated cellular debris, chronic inflammatory cells and mucus, when goblet cells are present. Most cysts can be treated adequately by complete excision with marsupialization of the entire epithelial lining to prevent fluid reaccumulation.

Arthropathy, osteolysis, keloids, relapsing conjunctival pannus and gingival overgrowth: a variant of polyfibromatosis?

Polyfibromatosis is a rare fibrosing condition characterized by fibromatosis in different body areas and by keloid formation, and which can be associated with arthropathy and osteolysis. Familial occurrence has been described, but the cause remains unknown. Here, we describe a patient with characteristics of polyfibromatosis with arthropathy who had in addition severe conjunctival fibrosis, distinctive face, gingival overgrowth, and pigmented keloids. We discuss the resemblances and differences with polyfibromatosis and descriptions of other, similar patients. We conclude that at present it remains uncertain whether the patient has a variant of polyfibromatosis or a separate entity.

Suppression of avascular bleb formation by a thin biodegradable film in a rabbit filtration surgery with mitomycin C.

BACKGROUND: Avascularity of the bleb is regarded as a risk of bleb infection, which is the most serious complication after filtration surgery with mitomycin C (MMC). There is no perfect way to prevent avascular bleb formation. We hypothesized that keeping the conjunctiva away from direct exposure to aqueous filtration may suppress avascular bleb formation after filtration surgery with MMC. In order to prove our hypothesis, we investigated whether a thin biodegradable, honeycomb-patterned film (HPF) can reduce bleb avascularity in a rabbit model of filtration surgery with mitomycin C (MMC). METHODS: A fornix-based full-thickness filtration surgery was performed in one eye of each of five rabbits (control group). The same surgery with intraoperative MMC treatment was done in both eyes of six rabbits, with one eye receiving a 14-µm-thick HPF made from poly(L-lactide-co-ε-caprolactone), which was placed subconjunctivally over the filtration site with the honeycomb surface facing up. Intraocular pressure (IOP) measurements, bleb evaluations using ultrasound biomicroscopy (UBM), and in vivo confocal microscopy (IVCM) were performed periodically for 4 weeks postoperatively followed by histological examination. RESULTS: The postoperative IOP decrease and bleb survival were significantly greater in MMC-treated eyes than in control eyes, and were similar between MMC-only and MMC + HPF eyes. The avascular area in the bleb persisted for 4 weeks in MMC-only eyes. Postoperative IVCM showed morphological changes of the conjunctival epithelial cells (i.e., enlarged and variable in size and shape) and epithelial defects in MMC-only eyes, and significantly lower density of connective tissue and vascularity in the subepithelial space in MMC-only eyes compared to the control eyes. These IVCM findings agreed with those by UBM and histology. Bleb avascularity shown by clinical bleb appearance, IVCM and histology, conjunctival epithelial damage shown by IVCM and histology, and loose subepithelial connective tissue shown by UBM, IVCM, and histology were all reduced in MMC + HPF eyes compared to MMC-only eyes. CONCLUSIONS: IVCM successfully showed the characteristic conjunctival damage in MMC-treated blebs. The concomitant use of a thin biodegradable HPF reduced avascularity and conjunctival damage in blebs, without compromising filtration in rabbits following filtration surgery with MMC.

New Freeware for Image Analysis of Lissamine Green Conjunctival Staining.

PURPOSE: Lissamine green (LG) is often used in addition to fluorescein to assess the severity of conjunctival damage in dry eye syndrome, which is graded manually. Our purpose was to describe an algorithm designed for image analysis of LG conjunctival staining. METHODS: Twenty pictures of patients suffering from dry eye with visible LG conjunctival staining were selected. The images were taken by 2 different digital slit lamps with a white light source and a red filter transmitting over the wavelengths absorbed by LG. Conjunctival staining appeared in black on a red background. The red channel was extracted from the original image. Stained areas were then detected using a Laplacian of Gaussian filter and applying a threshold whose value was determined manually on a subset of images. The same algorithm parameters remained constant thereafter. LG-stained areas were also drawn manually by 2 experts as a reference. RESULTS: The delineation obtained by the algorithm closely matched the actual contours of the punctate dots. In 19 cases of 20 (95%), the algorithm found the same Oxford grade as the experts, even for confluent staining that was detected as a multitude of dots by the algorithm but not by the experts, resulting in a high overestimation of the total number of dots (without mismatching the Oxford grade estimated by the experts). The results were similar for the 2 slit-lamp imaging systems. CONCLUSIONS: This efficient new image-analysis algorithm yields results consistent with subjective grading and may offer advantages of automation and scalability in clinical trials.

Paraneoplastic conjunctival cicatrization: two different pathogenic types.

PURPOSE: To describe the clinical and immunopathologic features of patients with 2 different types of paraneoplastic conjunctival cicatrization. DESIGN: Retrospective observational case analyses with a review of the literature. PARTICIPANTS: One patient with paraneoplastic ocular cicatricial pemphigoid (POCP) and 1 patient with paraneoplastic pemphigus (PNP) with ocular involvement. METHODS: Critical review of clinical history, diagnostic studies, and immunopathologic results of biopsies in the 2 cases, together with a review of the literature. MAIN OUTCOME MEASURES: Ability to recognize paraneoplastic conjunctival cicatrization and to diagnose the conditions accurately. RESULTS: The first patient, 46 years of age, presented with conjunctival scarring and symblephara, cough, oral lesions, and chest rash. Concurrently, a diagnosis of pulmonary squamous cell carcinoma was made. Conjunctival biopsy revealed a subepithelial bulla, an inflammatory infiltrate of T and B lymphocytes, and basement membrane zone deposition of immunoglobulin (Ig)-G and C3 consistent with POCP. The second patient, 54 years of age, had a recently diagnosed B-cell chronic lymphocytic leukemia, followed 1 month later with ocular irritation and bilateral extensive symblephara. Extensive oral lesions and skin involvement of the lower half of the body were seen. Skin biopsy disclosed subepidermal bullae and mostly T cells with virtually no B cells in the dermal infiltrate (the patient was being treated with rituximab). Linear subepithelial deposition of IgG and C3 and deposition within the epidermis were consistent with PNP. Further indirect immunofluorescence and immunoprecipitation studies with the patient's serum-derived antibodies established PNP as the definitive diagnosis. CONCLUSIONS: Underlying malignancy is an important consideration in younger patients with puzzling bilateral cicatrizing conjunctivitis, and a paraneoplastic condition can be established from either a conjunctival or a skin biopsy. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Conjunctival lymphangiectasia as a biomarker of severe systemic disease in Ser77Tyr hereditary transthyretin amyloidosis.

AIMS: To investigate the relationship between the ophthalmic and systemic phenotypes in patients with hereditary transthyretin amyloidosis with the S77Y mutation (ATTRS77Y). METHODS: In this cross-sectional study, patients with genetically confirmed ATTRS77Y amyloidosis were enrolled. All patients underwent complete neurological examination, including staging with the Neuropathy Impairment Score (NIS), Polyneuropathy Disability (PND) score; complete cardiological evaluation, including echocardiography, cardiac MRI and/or cardiac scintigraphy and complete ophthalmic evaluation, including slit lamp examination and fundus examination. Ocular ancillary tests (fluorescein and indocyanine green angiography, and anterior segment optical coherence tomography) were performed in cases with abnormal findings. The Kruskal-Wallis test was used for quantitative outcomes and Fisher's exact test for qualitative outcomes. Statistical significance was indicated by p<0.05 (two tailed). RESULTS: The study sample was composed of 24 ATTRS77Y patients. The mean patient age was 58.4±12.4 years. None of the patients presented with amyloid deposits in the anterior chamber, secondary glaucoma or vitreous amyloidosis. Retinal angiopathy was observed in four patients, complicated with retinal ischaemia in one patient. Conjunctival lymphangiectasia (CL) was detected in 13 patients (54%), associated with perilymphatic amyloid deposits. The presence of CL was statistically associated with more severe neurological disease (NIS=43.3±31.9 vs 18.9±20.4; PND=2.6±1.0 vs 1.4±0.7 in patients with and without CL, respectively; both p<0.05) and amyloid cardiomyopathy (p=0.002). CONCLUSION: In ATTRS77Y patients, CL is common and could serve as a potential biomarker for severe systemic disease. There were neither anterior chamber deposits, secondary glaucoma nor vitreous deposits in ATTRS77Y patients.

Subconjunctival prolapse of the palpebral lobe of the lacrimal gland occurring in association with occult orbital fat herniation.

INTRODUCTION: Sub-conjunctival prolapse of the palpebral lobe of the lacrimal gland can be a disfiguring and sometimes uncomfortable problem, usually caused by lacrimal gland enlargement or associated orbital lobe displacement. However, we present two novel cases associated with occult orbital fat herniation. METHOD: Retrospective interventional case series of two middle-aged/elderly male patients presenting with unilateral prolapse of the palpebral lobe of the lacrimal gland and no clinically apparent orbital fat prolapse. Neither patient had proptosis, globe displacement or diplopia, but both were aware of a subconjunctival mass in the lateral fornix causing a cosmetic concern. RESULTS: Imaging in both cases revealed a normal sized lacrimal gland with a prominent orbital fat pocket behind this. This fat was excised through an upper-eyelid skin crease approach with temporary detachment of the levator muscle in one case, and through a trans-conjunctival incision in the other. In both cases fat excision resulted in retro-placement of the palpebral lobe of the lacrimal gland such that it was no longer visible. Neither patient experienced any additional dry eye post-operatively. CONCLUSION: Sub-conjunctival prolapse of the palpebral lobe of the lacrimal gland can be associated with occult orbital fat herniation, revealed on imaging. Localised orbital fat excision is an effective means of treating this condition.

The Eyes Do not Lie: A Case of Bilateral Subconjunctival Hemorrhages and Orbital Hematoma Postpartum.

Subconjunctival hemorrhages are common in trauma and with increased intra-abdominal pressure. Despite the sustained pressure endured while pushing, they are rarely observed postpartum and can be an indicator of underlying ophthalmic disease. We present a case of a postpartum patient who developed bilateral subconjunctival hemorrhages with a retro-orbital hematoma. The patient is a 29-year-old gravida-1-para-0 at 41 weeks gestation who underwent an uncomplicated induction and delivery. She developed diplopia and orbital pain postpartum. Imaging revealed an orbital hematoma with an ophthalmologic workup revealing suspected glaucoma. She was followed outpatient with symptomatic treatment and had resolution of symptoms after 4 weeks. This case demonstrates a rare postpartum scenario and the subsequent workup. Subconjunctival hemorrhages are rare in the postpartum period despite prolonged pushing efforts. Incorporating a multidisciplinary approach for diagnosis of a pregnant patient with ophthalmologic disease is required to optimize management in the antepartum, intrapartum, and postpartum periods.