Topical 5-Fluorouracil 0.5% as primary treatment for Ocular Surface Squamous Neoplasia.

PURPOSE: To evaluate the efficacy of topical treatment with 5-Fluorouracil (5-FU) 0.5% in cases of Ocular Surface Squamous Neoplasia (OSSN), and to assess the tolerance of patients undergoing treatment. METHODS: Patients with clinical diagnosis of OSSN referred to the Ocular Oncology division from the Federal University of Sao Paulo, Brazil, were recruited for the current study. Patients were treated with topical 5-FU 0.5% using a regimen of 4 times daily for 10 days, followed by a 3-week drug holiday, continued up to 3 cycles before an alternative treatment. Lesions were evaluated at baseline and throughout treatment. Treatment adherence was assessed using the Morisky Medication Adherence scale. Any adverse events along the treatment were noted. RESULTS: A total of 30 eyes of 30 patients adherent to the treatment were included in the study. Among the total cases treated with 5-FU 0.5%, 24 patients achieved therapeutic success after a mean treatment duration of 21.71 ± 7.77 days, representing a success rate of 80.00% (95% CI: 60.75-91.18%). For each 1 mm2 increase in the lesion area, the odds of treatment success decrease by 6% (OR: 0.94; 95%CI: 0.88-0.99; p = 0.033). Only mild adverse events such as ocular discomfort, ocular burning and tearing were observed along the treatment in 8 patients. CONCLUSIONS: Topical 5-FU 0.5% is an effective therapeutic option in the treatment of OSSN, with an 80% therapeutic success rate, showing good tolerability. The size of the lesion was identified as a factor influencing treatment success, therefore it should be taken into consideration when defining treatment approaches.

Impression cytology of ocular surface in xeroderma pigmentosum.

PURPOSE: To describe cellular alterations detected by impression cytology of the ocular surface in patients with xeroderma pigmentosum. The secondary objective was to assess the reliability of impression cytology in diagnosing ocular surface squamous neoplasia. METHODS: Patients with xeroderma pigmentosum underwent a single-day complete ophthalmological examination and impression cytology for ocular surface evaluation using 13 mm diameter mixed cellulose esters membrane filters and combined staining with Periodic Acid Schiff, Hematoxylin and Eosin, and Papanicolaou stains followed by microscopic analysis. The cytological findings were correlated with the clinical diagnosis. The impression cytology findings at baseline and one-year follow-up were correlated with the clinical course (no tumor, treated tumor, residual tumor recurrent tumor, new tumor). RESULTS: Of the 42 patients examined, impression cytology was performed in 62 eyes of 34 participants (65% females). The mean age of patients was 29.6 ± 17 years (range 7-62). Fifteen eyes had a clinical diagnosis of ocular surface squamous neoplasia. Impression cytology showed goblet cells (47, 75%), inflammatory cells (12, 19%), keratinization (5, 8%), and squamous metaplasia (30, 48%). Impression cytology was positive for atypical cells in 18 patients (12 with and 6 without ocular surface squamous neoplasia). The sensitivity, specificity, positive predictive value, and negative predictive value of impression cytology (at baseline) for diagnosis of ocular surface squamous neoplasia were 80%, 87%, 67%, and 93%, respectively, using clinical diagnosis of ocular surface squamous neoplasia as the reference standard. CONCLUSION: Impression cytology has a moderate positive predictive value for the diagnosis of ocular surface squamous neoplasia in patients with xeroderma pigmentosum. However, the lack of detection of atypical cells on impression cytology has a high negative predictive value for ocular surface squamous neoplasia. Integration of impression cytology in the long-term management of high-risk patients, such as patients with xeroderma pigmentosum, can avoid unnecessary diagnostic biopsies.

Ocular metastases profile in a tertiary hospital in São Paulo, Brazil.

OBJECTIVE: Ocular metastases are the most common intraocular tumours in adults. Data regarding the occurrence of these tumours in the Brazilian population is scarce. We aimed to investigate the profile of ocular metastases of patients referred to tertiary hospital service in São Paulo, Brazil. DESIGN: Retrospective study. PARTICIPANTS: Patients referred to the Ocular Oncology service of the Federal University of São Paulo with initial diagnostic hypothesis of ocular metastasis. METHODS: Data was retrospectively collected from medical records from June 2017 to June 2023. Age, sex, primary tumour site, previous knowledge of the systemic diagnosis, laterality, initial visual acuity (VA), local or systemic treatment and mean follow-up period were obtained. RESULTS: A total of 37 cases were referred to the ocular oncology division due to a suspected ocular metastasis, 15 (40.5%) were confirmed. Mean age at diagnosis was 53.47 ± 16.01 years old, the majority (86.7%) of patients already knew the systemic diagnosis. Breast cancer (66.7%) was the most common primary site, followed by Lung cancer (26.7%). Both eyes were affected in 66.67% of the cases, all patients had metastases at the choroid (100.0%), and the mean initial VA was 1.37 ± 1.04 logMAR. Chemotherapy was the main systemic treatment modality (73.3%), and most patients had no ocular treatment (53.3%). The mortality rate along the follow-up period was 30.0%. CONCLUSIONS: Considering the number of new patients absorbed by the Ocular Oncology service over the study period, the frequency of ocular metastases was relatively low. The patients' characteristics was comparable to data published in the international literature.

Evaluation of Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum.

PURPOSE: To assess Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum and correlate with ocular surface changes. METHODS: This cross-sectional study evaluated patients with xeroderma pigmentosum. All patients underwent a comprehensive and standardized interview. The best-corrected visual acuity of each eye was determined. Detailed ophthalmic examination was conducted, including biomicroscopy examination of the ocular surface, Schirmer test type I, and meibography, and fundus examination was also performed when possible. Meibomian gland dysfunction was assessed by non-contact meibography using Oculus Keratograph® 5M (OCULUS Inc., Arlington, WA, USA). Saliva samples were collected using the Oragene DNA Self-collection kit (DNA Genotek Inc., Ottawa, Canada), and DNA was extracted as recommended by the manufacturer. Factors associated with abnormal meiboscores were assessed using generalized estimating equation models. RESULTS: A total of 42 participants were enrolled, and 27 patients underwent meibography. The meiboscore was abnormal in the upper eyelid in 8 (29.6%) patients and in the lower eyelid in 17 (62.9%). The likelihood of having abnormal meiboscores in the lower eyelid was 16.3 times greater than that in the upper eyelid. In the final multivariate model, age (p=0.001), mutation profile (p=0.006), and presence of ocular surface malignant tumor (OSMT) (p=0.014) remained significant for abnormal meiboscores. For a 1-year increase in age, the likelihood of abnormal meiboscores increased by 12%. Eyes with OSMT were 58.8 times more likely to have abnormal meiboscores than eyes without ocular surface malignant tumor. CONCLUSION: In the final model, age, xeroderma pigmentosum profile, previous cancer, and clinical alterations on the eyelid correlated with a meiboscore of ≥2. Meibomian gland dysfunction was common in patients with xeroderma pigmentosum, mainly in the lower eyelid. The severity of Meibomian gland dysfunction increases with age and is associated with severe eyelid changes.

Evaluation of Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum

ABSTRACT Purpose: To assess Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum and correlate with ocular surface changes. Methods: This cross-sectional study evaluated patients with xeroderma pigmentosum. All patients underwent a comprehensive and standardized interview. The best-corrected visual acuity of each eye was determined. Detailed ophthalmic examination was conducted, including biomicroscopy examination of the ocular surface, Schirmer test type I, and meibography, and fundus examination was also performed when possible. Meibomian gland dysfunction was assessed by non-contact meibography using Oculus Keratograph® 5M (OCULUS Inc., Arlington, WA, USA). Saliva samples were collected using the Oragene DNA Self-collection kit (DNA Genotek Inc., Ottawa, Canada), and DNA was extracted as recommended by the manufacturer. Factors associated with abnormal meiboscores were assessed using generalized estimating equation models. Results: A total of 42 participants were enrolled, and 27 patients underwent meibography. The meiboscore was abnormal in the upper eyelid in 8 (29.6%) patients and in the lower eyelid in 17 (62.9%). The likelihood of having abnormal meiboscores in the lower eyelid was 16.3 times greater than that in the upper eyelid. In the final multivariate model, age (p=0.001), mutation profile (p=0.006), and presence of ocular surface malignant tumor (OSMT) (p=0.014) remained significant for abnormal meiboscores. For a 1-year increase in age, the likelihood of abnormal meiboscores increased by 12%. Eyes with OSMT were 58.8 times more likely to have abnormal meiboscores than eyes without ocular surface malignant tumor. Conclusion: In the final model, age, xeroderma pigmentosum profile, previous cancer, and clinical alterations on the eyelid correlated with a meiboscore of ≥2. Meibomian gland dysfunction was common in patients with xeroderma pigmentosum, mainly in the lower eyelid. The severity of Meibomian gland dysfunction increases with age and is associated with severe eyelid changes.

Impression cytology of ocular surface in xeroderma pigmentosum

ABSTRACT Purpose: To describe cellular alterations detected by impression cytology of the ocular surface in patients with xeroderma pigmentosum. The secondary objective was to assess the reliability of impression cytology in diagnosing ocular surface squamous neoplasia. Methods: Patients with xeroderma pigmentosum underwent a single-day complete ophthalmological examination and impression cytology for ocular surface evaluation using 13 mm diameter mixed cellulose esters membrane filters and combined staining with Periodic Acid Schiff, Hematoxylin and Eosin, and Papanicolaou stains followed by microscopic analysis. The cytological findings were correlated with the clinical diagnosis. The impression cytology findings at baseline and one-year follow-up were correlated with the clinical course (no tumor, treated tumor, residual tumor recurrent tumor, new tumor). Results: Of the 42 patients examined, impression cytology was performed in 62 eyes of 34 participants (65% females). The mean age of patients was 29.6 ± 17 years (range 7-62). Fifteen eyes had a clinical diagnosis of ocular surface squamous neoplasia. Impression cytology showed goblet cells (47, 75%), inflammatory cells (12, 19%), keratinization (5, 8%), and squamous metaplasia (30, 48%). Impression cytology was positive for atypical cells in 18 patients (12 with and 6 without ocular surface squamous neoplasia). The sensitivity, specificity, positive predictive value, and negative predictive value of impression cytology (at baseline) for diagnosis of ocular surface squamous neoplasia were 80%, 87%, 67%, and 93%, respectively, using clinical diagnosis of ocular surface squamous neoplasia as the reference standard. Conclusion: Impression cytology has a moderate positive predictive value for the diagnosis of ocular surface squamous neoplasia in patients with xeroderma pigmentosum. However, the lack of detection of atypical cells on impression cytology has a high negative predictive value for ocular surface squamous neoplasia. Integration of impression cytology in the long-term management of high-risk patients, such as patients with xeroderma pigmentosum, can avoid unnecessary diagnostic biopsies.

Toluidine blue 1% eye drop versus optical coherence tomography for margin delimitation of ocular surface squamous neoplasia.

PURPOSE: To compare the use of toluidine blue 1% eye drops with anterior segment optical coherence tomography (OCT) for the determination of tumour margins in patients with ocular surface squamous neoplasia (OSSN). METHODS: The study was conducted from July 2020 to June 2021 at the Ocular Oncology department at the Federal University of São Paulo, Brazil. Slit-lamp photographs after toluidine blue staining and OCT of the anterior segment were taken on the same day from patients with OSSN. Photographs and OCT images were analyzed quantitatively using the software ImageJ and IMAGEnet®, respectively. The agreement between techniques was evaluated qualitatively through the Bland-Altman graph and quantitatively through intraclass correlation (ICC). RESULTS: A total of 21 participants (71.43% males) with a clinical diagnosis of OSSN were included in the study. The average + SD diameter along the chosen axes was 4.43 ± 2.08 mm with OCT of 4.37 ± 2.03 mm with toluidine blue, a difference not statistically significant (p = 0.2891). The Bland-Altman analysis indicated a good qualitative agreement between the methods, with all cases inserted within the limits of agreement from -0.3217 to 0.4268. The ICC quantitative analysis showed an almost perfect agreement of 99.57% (95%CI: 98.96-99.83%; p < 0.001). CONCLUSIONS: Our findings showed that OCT and toluidine eye drops are equivalent in determining margins for tumour measurements, which is particularly relevant in low-income settings where anterior segment OCT is not available. The use of toluidine blue 1% could be an useful alternative to quantify the size of the tumour, help to monitor tumour growth, and outline margins for surgical planning.

Scleral communication between Glaucoma drainage device capsule and the suprachoroidal space simulating amelanotic choroidal melanoma / Comunicação escleral entre a cápsula de dispositivo de drenagem para glaucoma e o espaço supracoroidal simulando melanoma de coroide amelanótico

ABSTRACT This is a case report involving a 56-year-old male patient with a history of pars plana vitrectomy due to a rhegmatogenous retinal detachment in the right eye that resulted in the implantation of a drainage device after the patient developed secondary glaucoma. Two years after the device's implantation, the patient was referred to our care as his visual acuity had decreased to 20/200 (1.00 LogMAR). At the fundus evaluation, a choroidal amelanotic elevation was observed at the upper temporal equator, and a potential diagnosis was made of amelanotic choroidal melanoma. The ultrasound exam visualized the patient's implanted superotemporal justabulbar drainage device, which revealed a transscleral communication from the plate fibrocapsular's draining space to the suprachoroidal space (fistula). The ultrasound also revealed a focal pocket of choroidal detachment in the patient's superotemporal region, simulating an amelanotic choroidal melanoma. A new pars plana vitrectomy was performed to remove the internal limiting membrane without repercussions at the fistula site. The patient's recovery progressed well, and he regained a visual acuity of 20/70 (0.55 LogMAR). To the best of our knowledge, this is the first case report of this condition.

RESUMO Relato de caso de paciente 56 anos, sexo masculino, com histórico de vitrectomia via pars plana por descolamento de retina em olho direito e posterior implante de dispositivo de drenagem por glaucoma secundário. Dois anos após o procedimento foi encaminhado ao serviço por baixa de acuidade visual (AV) de 20/200 (1.00 LogMAR). À fundoscopia, observou-se uma elevação amelanótica temporal no equador com hipótese diagnóstica de melanoma de coroide amelanótico. O exame de ultrassom mostrou implante de dispositivo de drenagem justabulbar temporal superior com comunicação transescleral para espaço subcoroidal (fístula), sugerindo bolsão focal de descolamento de coroide em equador temporal superior simulando melanoma de coroide amelanótico. O paciente foi abordado cirurgicamente devido membrana epirretiniana com nova vitrectomia via pars plana para peeling de membrana limitante interna, sem repercussões no local da fístula, evoluindo bem com acuidade visual de 20/70 (0.55 LogMAR). Ao nosso conhecimento, este é o primeiro caso relatado nessa condição.

Scleral communication between Glaucoma drainage device capsule and the suprachoroidal space simulating amelanotic choroidal melanoma.

This is a case report involving a 56-year-old male patient with a history of pars plana vitrectomy due to a rhegmatogenous retinal detachment in the right eye that resulted in the implantation of a drainage device after the patient developed secondary glaucoma. Two years after the device's implantation, the patient was referred to our care as his visual acuity had decreased to 20/200 (1.00 LogMAR). At the fundus evaluation, a choroidal amelanotic elevation was observed at the upper temporal equator, and a potential diagnosis was made of amelanotic choroidal melanoma. The ultrasound exam visualized the patient's implanted superotemporal justabulbar drainage device, which revealed a transscleral communication from the plate fibrocapsular's draining space to the suprachoroidal space (fistula). The ultrasound also revealed a focal pocket of choroidal detachment in the patient's superotemporal region, simulating an amelanotic choroidal melanoma. A new pars plana vitrectomy was performed to remove the internal limiting membrane without repercussions at the fistula site. The patient's recovery progressed well, and he regained a visual acuity of 20/70 (0.55 LogMAR). To the best of our knowledge, this is the first case report of this condition.

Conjunctival leiomyosarcoma.

Purpose: Leiomyosarcoma (LMS) is a mesenchymal neoplasm with smooth muscle differentiation, being considered one of the most common soft tissue sarcomas. However, it rarely affects the eye, and when it does, it is usually located in the orbit, being extremely rare in the conjunctiva. Observations: We report a case of a 45 years old male patient, with a recurrent rapid growing conjunctival mass on the temporal limbus of his left eye, which was excised, and the anatomopathological report was suggestive of a grade 1 leiomyosarcoma. Since the lesion was recurrent, we decided to perform an extended enucleation for treating this condition. Nevertheless, the patient is being followed up to 30 months, with systemic metastasis screening, showing no other lesions or recurrences. Conclusions and importance: Conjunctival leiomyosarcoma is an extremely rare ocular tumor, which can be clinically indistinguishable from other conditions such as squamous cell carcinoma, so, biopsy is essential. Albeit there is no standard treatment, complete surgical removal with safety margins is mandatory.

Sebaceous adenoma of the conjunctiva and caruncle: a clinicopathological report of three cases and literature review.

Sebaceous tumors of the conjunctiva and caruncle are rare conditions, accounting for 1% of caruncle lesions and even lower among conjunctival lesions. Almost 50% of cases are associated with Muir-Torre syndrome, a rare autosomal-dominant condition characterized by at least one sebaceous skin tumor and one visceral malignancy. We report 3 cases of sebaceous adenoma with different presentations that were submitted to excisional biopsy and immunohistochemical study. Diagnosis of these tumors should increase the level of suspicion and lead to clinical investigation to rule out neoplasms, particularly because in up to 41% of cases, these can be the first sign of the disease.

Upper Eyelid Necrosis Secondary to Hordeolum: A Case Report.

We reported a case of upper eyelid necrosis initially misdiagnosed as a preseptal cellulitis following a hordeolum externum resulting in great damage to the upper eyelid (anterior lamella). The infection was successfully treated with surgical cleansing, drainage, and endovenous antibiotics. Early treatment may avoid severe complications such as eyelid deformity, systemic involvement, and blindness.

Acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM) associated with choroidal melanoma.

BACKGROUND: To report a case of acute exudative polymorphous paraneoplastic vitelliform maculopathy in a patient with a history of choroidal melanoma, with metastases to the pancreas, liver, and central nervous system. CASE PRESENTATION: A 63-year-old patient, with a history of enucleation of the right eye due to choroidal melanoma, complained of progressive visual loss during a follow-up visit. Fundoscopic examination revealed multiple small areas of serous retinal detachment scattered throughout the posterior pole and ancillary tests confirmed the diagnosis of acute exudative polymorphous paraneoplastic vitelliform maculopathy (AEPPVM). Screening for systemic metastases showed pancreatic, hepatic, and central nervous system involvement. CONCLUSIONS: We describe a rare case of acute exudative polymorphous paraneoplastic vitelliform maculopathy, which should be considered in patients with or without a history of melanoma, who have vitelliform retinal detachments. Nevertheless, no previous reviews of literature have shown a correlation between AEPPVM and pancreatic metastasis.

Effects of oral propranolol for circumscribed choroidal hemangioma.

PURPOSE: We aimed to evaluate the effects of oral propranolol for circumscribed choroidal hemangioma. METHODS: In this prospective, longitudinal interventional study, we administered oral propranolol at a dosage of 1.5 mg/kg/day to five patients with circumscribed choroidal hemangioma. We then evaluated visual acuity, binocular indirect ophthalmoscopy, optical coherence tomography, optical coherence tomography angiography, fluorescein and indocyanine green angiography, and ocular ultrasonography at regular intervals and compared changes from the baseline assessments. RESULTS: No clinical or diagnostic changes were observed in the sizes of the circumscribed choroidal hemangiomas during treatment. Complications due to the hemangioma were reduced in the first four months of treatment, followed by maintenance, before worsening in the subsequent three months. CONCLUSIONS: The study showed that oral propranolol at a dose of 1.5 mg/kg/day did not offer effective monotherapy in the treatment of circumscribed choroidal hemangioma.

Effects of oral propranolol for circumscribed choroidal hemangioma / Efeitos do propranolol oral para de hemangioma circunscrito de coroide

ABSTRACT Purpose: We aimed to evaluate the effects of oral propranolol for circumscribed choroidal hemangioma. Methods: In this prospective, longitudinal interventional study, we administered oral propranolol at a dosage of 1.5 mg/kg/day to five patients with circumscribed choroidal hemangioma. We then evaluated visual acuity, binocular indirect ophthalmoscopy, optical coherence tomography, optical coherence tomography angiography, fluorescein and indocyanine green angiography, and ocular ultrasonography at regular intervals and compared changes from the baseline assessments. Results: No clinical or diagnostic changes were observed in the sizes of the circumscribed choroidal hemangiomas during treatment. Complications due to the hemangioma were reduced in the first four months of treatment, followed by maintenance, before worsening in the subsequent three months. Conclusions: The study showed that oral propranolol at a dose of 1.5 mg/kg/day did not offer effective monotherapy in the treatment of circumscribed choroidal hemangioma.

RESUMO Objetivo: Avaliar o efeito do propranolol oral para hemangioma circunscrito da coroide. Métodos: O estudo é do tipo prospectivo, quantitativo e descritivo. Propranolol oral na dose de 1.5 mg/kg/dia foi administrada em cinco pacientes com hemangioma circunscrito da coroide. Todos os pacientes foram avaliados com acuidade visual, oftalmoscopia binocular indireta, tomografia de coerência óptica, angiografia com tomografia de coerência óptica, angiografia com fluoresceína e indocianina verde e ultrassonografia ocular. Resultados: Nenhuma mudança clínica ou no tamanho do hemangioma circunscrito da coroide foi vista através de métodos diagnósticos em qualquer momento do tratamento. Uma atenuação das complicações foi observada nos primeiros quatro meses de tratamento, com manutenção da condição e piora nos meses seguintes. Conclusão: O estudo mostrou que o propranolol oral na dose de 1.5 mg/kg/dia não se mostrou efetivo como monoterapia no tratamento do hemangioma circunscrito da coroide.

Iris metastases from systemic cancer: a report of three cases / Metástase de íris de câncer sistêmico: relato de três casos

ABSTRACT Ocular metastasis is relatively uncommon, with a reported incidence of approximately 8%, according to the results of autopsy evaluation. The majority of ocular metastases are located within the choroid, while metastatic tumors affecting the iris are rare. Metastatic tumors may manifest as stromal nodules or ill-defined iris thickening, or they may present with nonspecific features such as pain, iridocyclitis, and hyphema. Here, we describe three patients with iris metastasis and discuss the diagnostic challenges and unusual findings associated with these cases.

RESUMO A maioria das metástases oculares do câncer sistêmico são encontrados na coroide. As metástases para a íris são incomuns, podendo se manifestar como nódulo estromal, espessamento de íris de limites mal definidos ou como uma iridociclite ou hifema. Relatamos 3 pacientes com lesão de íris e história pregressa de câncer sistêmico. Enfatizamos a dificuldade no diagnóstico e raridade dessas lesões comparando com relatos anteriores.

Iris metastases from systemic cancer: a report of three cases.

Ocular metastasis is relatively uncommon, with a reported incidence of approximately 8%, according to the results of autopsy evaluation. The majority of ocular metastases are located within the choroid, while metastatic tumors affecting the iris are rare. Metastatic tumors may manifest as stromal nodules or ill-defined iris thickening, or they may present with nonspecific features such as pain, iridocyclitis, and hyphema. Here, we describe three patients with iris metastasis and discuss the diagnostic challenges and unusual findings associated with these cases.