Cutting-edge issues in sympathetic ophthalmia approach and six case reports: a T cell-mediated autoimmune response / As questões de ponta na abordagem da oftalmia simpática e 6 relatos de caso: uma resposta autoimune mediada por células T

ABSTRACT Sympathetic ophthalmia is a rare and potentially devastating bilateral diffuse granulomatous panuveitis. It is caused by surgical or non-surgical eye injuries and is an uncommon and serious complication of trauma. It is diagnosed clinically and supported by imaging examinations such as ocular ultrasonography and optical coherence tomography. Its treatment consists of immunosuppressive therapy with steroids and sometimes steroid-sparing drugs, such as cyclosporine, azathioprine, cyclophosphamide, and mycophenolate mofetil. Fast and effective management with systemic immunosuppressive agents allows for disease control and achievement of good visual acuity in the sympathizing eye. By contrast, enucleation should be considered only in situations where the injured eye has no light perception or in the presence of severe trauma. In addition to a bibliographic review of this topic, we report six cases involving different immunosuppressive and surgical treatment modalities.

RESUMO A oftalmia simpática consiste em uma panuveíte granulomatosa bilateral rara e potencialmente devastadora, ocorrendo geralmente após trauma ocular cirúrgico ou não cirúrgico. O diagnóstico é baseado em aspectos clínicos e apoiado por exames de imagem, como ultrassonografia ocular e tomografia de coerência óptica. O tratamento consiste em terapia imunossupressora com esteróides e, eventualmente, drogas poupadoras de esteróides, como ciclosporina, azatioprina, ciclofosfamida e micofonato de mofetila. O manejo rápido e eficaz com agentes imunossupressores sistêmicos permite o controle da doença e a obtenção de boa acuidade visual no olho simpatizante. A enucleação, por outro lado, poderia ser considerada apenas em situações em que o olho lesado não tem percepção luminosa ou há trauma grave. Além de uma revisão bibliográfica sobre o tema, foi relatada uma série de 6 casos com diferentes modalidades de tratamento imunossupressor e cirúrgico.

Melanoma uveal

Introducción: El melanoma uveal, es el cáncer intraocular más común en adultos, representa cerca del 3 % al 5 % de todos los melanomas; con un pico de incidencia en hombres en la sexta década de la vida. Objetivo: Describir la presentación clínica, diagnóstico y tratamiento de una paciente con melanoma uveal. Caso clínico: Paciente femenina de 39 años de edad, con antecedentes de salud, quien acudió a consulta por presentar dolor intenso de doce días de evolución localizado en el ojo derecho, asociado a pérdida de la visión, dolor a los movimientos oculares, astenia y anorexia. Al examen físico se encontró edema palpebral severo, abundantes secreciones mucopurulentas, quémosis conjuntival en 360 grados marcado e hiperemia cilio-conjuntival en el ojo derecho. Se realizaron estudios analíticos e imagenológicos. Al analizar el resultado de la exploración física y los complementarios se decidió la intervención quirúrgica consistente en la exenteración orbitaria derecha, esfenoidectomía y etmoidectomía transorbitaria derecha. Con una evolución satisfactoria fue egresada del centro hospitalario. La biopsia informó un melanoma coroideo. Conclusiones: El melanoma coroideo es una enfermedad ocular poco frecuente en mujeres jóvenes de piel negra. La sintomatología es extremadamente variable con las masas coroideas. Es importante conocer sus características, pues estos efectos clínicos sirven como un recordatorio para los oftalmólogos para incluirla en el diagnóstico diferencial de otras patologías oculares.

Introduction: Uveal melanoma is the most common intraocular cancer in adults, represents approximately 3 % to 5 % of all melanomas; with a peak incidence in men in the sixth decade of life. Objective : To describe the clinical presentation, diagnosis and treatment of a patient with uveal melanoma. Clinical case: A 39-year-old female patient, with a health history, who came to the consultation due to intense pain of twelve days of evolution located in the right eye, associated with loss of vision, pain with eye movements, asthenia and anorexy. Physical examination revealed severe palpebral edema, abundant mucopurulent secretions, marked 360-degree conjunctival chemosis, and cilio-conjunctival hyperemia in the right eye. Analytical and imaging studies were performed. When analyzing the results of the physical and complementary examination, the surgical intervention consisting of right orbital exenteration, sphenoidectomy and right transorbital ethmoidectomy was decided. With a satisfactory evolution, she was discharged from the hospital. Biopsy reported choroidal melanoma. Conclusions: Choroidal melanoma is a rare ocular entity in young black women. Symptomatology is extremely variable with choroidal masses. It is important to know its characteristics, since these clinical effects serve as a reminder for ophthalmologists to include it in the differential diagnosis of other ocular pathologies.

Evisceration and enucleation cases in the ophthalmologic emergency department of a tertiary Brazilian hospital / Casos de evisceração e enucleação em departamento de urgência oftalmológica de um hospital terciário Brasileiro

ABSTRACT Purpose: To analyze the epidemiological profiles of evisceration and enucleation cases in the ophthalmologic emergency department of a Brazilian tertiary hospital. Methods: Patients treated in the ophthalmologic emergency department of Hospital São Paulo (Universidade Federal de São Paulo) during the period 2013 to 2018 were retrospectively evaluated. Urgent cases of evisceration or enucleation surgery were included, and elective cases were excluded. The following information was extracted from the patients' medical records: demographic data, immediate and associated reasons for the surgical procedure, informed visual acuity, symptom duration until ophthalmologic care, complications, distance from the residence to the tertiary hospital, and time of hospitalization. Results: In total, 61 enucleations and 121 eviscerations were included in this study. The patients had a mean age of 63.27 ± 18.68 years. Of the patients, 99 were male (54.40%), and 83 were female (45.60%). The indications for evisceration or enucleation were corneal perforation with (44.50%) and without (23.63%) signs of infection, endophthalmitis (15.38%), ocular trauma (14.29%), neoplasia (0.55%), burn accident (1.10%), and phthisis bulbi (0.55%). The self-reported visual acuity was no light perception (87.36%) or light perception (1.10%). However, 3.30% of the patients did not cooperate with the examination, and no information on visual acuity was available for the remaining 8.24%. The mean symptom duration before ophthalmologic care was sought was 18.32 days. Two patients had sympathetic ophthalmia after evisceration. Conclusions: More eviscerations than enucleations were performed throughout the study period. The most common demographic characteristics were age >60 years and male sex. The main indications for urgent evisceration and enucleation procedures were corneal perforation with and without infection, endophthalmitis, and ocular trauma. The findings from this study could guide clinicians in performing preventive measures to avoid destructive eye procedures.

RESUMO Objetivo: Analisar o perfil epidemiológico dos casos de evisceração e enucleação no pronto-socorro oftalmológico de um hospital terciário brasileiro. Métodos: Análise retrospectiva dos casos tratados no pronto-socorro oftalmológico do Hospital São Paulo (Universidade Federal de São Paulo) entre os anos de 2013 a 2018. Os casos urgentes de evisceração e enucleação foram incluídos e os casos eletivos foram excluídos. A análise dos prontuários médicos foi baseada em: dados demográficos, causas imediatas e associadas ao procedimento, acuidade visual informada, duração dos sintomas antes do atendimento oftalmológico, complicações, distância da residência até o hospital e tempo de hospitalização. Resultados: 61 enucleações e 121 eviscerações foram incluídas no estudo. Os pacientes tinham uma média de idade de 63,27 ± 18,68 anos; 99 eram do sexo masculino (54,50%) e 83 do sexo feminino (45,60%). As indicações de evisceração e enucleação foram: perfuração corneana com (44,50%) e sem (23,63%) sinais infecciosos, endoftalmite (15,38%), trauma ocular (14,29%), neoplasia (0,55%), queimadura (1,10%) e phthisis bulbi (0,55%). A acuidade visual informada foi de ausência de percepção luminosa (87,36%), percepção luminosa (1.10%), ausência de colaboração (3,30%) e sem dados informados (8,24%). A média de tempo até a busca pelo serviço oftalmológico foi de 18,32 dias. Houve 2 casos de oftalmia simpática após evisceração. Conclusões: Eviscerações foram predominantemente realizadas em comparação a enucleações em todo o período de estudo. As características demográficas mais comuns foram idade >60 anos e sexo masculino. As principais indicações para procedimentos urgentes de evisceração e enucleação foram perfuração corneana com e sem infecção, endoftalmite e trauma ocular. Este estudo poderia guiar medidas preventivas para evitar procedimentos oculares destrutivos.

Analysis of the association between different treatments and high-risk histopathologic features of bilateral retinoblastomas / 中华眼底病杂志

Objective:To evaluate the pathological features of bilateral retinoblastoma (RB) and the relationship between different treatments and high-risk histopathologic features (HHF).Methods:Retrospective series of case studies. From 1999 to 2018, 73 patients with binocular RB diagnosed by pathological examination in Department of Ophthalmology, Peking University People's Hospital were included in the study. Among them, 50 patients were male (68.5%, 50/73), 23 patients were females (31.5%, 23/73); 11 patitents had a family history of RB. The mean age at the first diagnosis was 14.8±15.6 months. The average time between first diagnosis and first intervention was 3.97±4.74 months. According to the international classification standard of intraocular RB staging, among the 73 eyes, C, D and E stages were 2 (2.7%, 2/73), 15 (20.5%, 15/73), and 56 (76.7%, 56/73) eyes, respectively. Ocular images for each patient were obtained using a wide-angle contact fundus camera during examination under general anaesthesia. The treatment protocol (globe salvaging or enucleation) depended on the result of several clinical features. Globe salvaging treatment included chemotherapy combined with local therapy such as intra-arterial chemotherapy (IAC), intravitreal chemotherapeutics injection, cryotherapy, laser, transpupillary thermotherapy and radiotherapy. If globe salvaging failed, enucleation was offered and histopathologic analysis was conducted of the enucleated eye, the ophthalmic pathologist read and evaluated the presence of HHF. Independent samples t-test was performed to compare the continuous variables. The pathological features and the relationship between different treatments and HHF were analyzed. Group difference was calculated with chi-square. Results:Among the 73 eyes, the first treatment was enucleation in 21 eyes (28.8%, 21/73); 52 eyes (71.2%, 52/73) were treated with eye protection. After enucleation, 9 cases (12.3%, 9/73) had recurrence and metastasis, and 7 cases (9.6%, 7/73) died. The intervention time of patients with recurrence and metastasis and those without recurrence and metastasis were 7.4±7.3 and 3.5±4.1 months respectively; the first intervention time of patients with recurrence and metastasis was significantly later than that of patients without recurrence and metastasis, but the difference was not statistically significant ( t=-1.561, P=0.154). The pathological examination results showed that there were 26 eyes (35.6%, 26/73) with HHF, 4 (26.7%, 15/26) and 22 (39.3%, 22/56) eyes were in stage D and E, respectively. Those who received other treatments before enucleation had lower HHF percentages after enucleation than those who did not receive corresponding treatments, but the difference was not statistically significant ( χ2=1.852, 0.074, 0.000, 1.007, 0.007, 2.729; P>0.05). Among the 26 eyes, 5 (83.3%, 5/6) and 21 (31.3%, 21/67) eyes were treated with systemic chemotherapy combined with and without IAC, respectively, and there was a significant difference in the percentage of HHF ( χ2=4.422, P=0.035). Conclusions:IAC eye-preserving therapy before enucleation has a significant effect on HHF.

Pay attention to the research on the mechanism of retinoblastoma and the methods of diagnosis and treatment / 中华眼底病杂志

Retinoblastoma (RB) is the most serious eye disease that causes blindness, disability and death in infant. Loss of tumor suppressor gene RB1 leads to tumorigenesis of RB. With continuous innovation and development of new methods, the management of retinoblastoma has experienced a dramatic change from enucleation, external radiotherapy, intravenous chemotherapy, to intra-arterial chemotherapy combined with local treatment, enabling a more favorable outcome for survival, salvage and vision. However, the pathogenesis of RB is unclear in many aspects, the rate of eyeball removal in children with advanced RB remains high, and the innovation of new treatment are needed to be further promoted. Therefore, the key to improve the overall treatment level of RB is to pay attention to the study on pathogenesis, early diagnosis and treatment, as well as strengthen multi-center clinical trials and translational medicine research.

Cause of death of children with intraocular retinoblastoma in the Beijing Children's Hospital 2009-2017 / 中华眼底病杂志

Objective:To observe and analyze the clinical characteristics of children who died of intraocular retinoblastoma (RB).Methods:A retrospective clinical study. Fourteen children (23 eyes) with intraocular RB who died after receiving treatment in Beijing Children's Hospital from 2009 to 2017 were included in the study. Among the children, there were 7 males (10 eyes) and 7 females (13 eyes); 5 had unilateral and 9 had bilateral tumor. Age were 17.2±15.5 months. All children underwent RetCam examination. RB was staged according to the international intraocular RB classify. Among the 23 eyes, 1 eye was in stage B, 2 eyes were in stage C, 12 eyes in stage D, and 8 eyes in stage E. Treatment methods included a systemic (vincristine, etoposide and carboplatin) chemotherapy (VEC chemotherapy), enucleation surgery, and vitrectomy. The basic conditions including age, time of diagnosis, pathological diagnosis, treatment and main causes of death were retrospectively analyzed.Results:Among the 14 cases, the first symptom was leukemia in 12 cases, red eye in 1 case, and squintin in 1 case. Systemic VEC chemotherapy was used for 1-6 courses of treatment; 5 cases were enucleated, 3 cases underwent histopathological examination; 3 cases were treated with vitrectomy. Among the 3 cases who underwent histopathological examination, the sclera and optic nerve, optic nerve and optic disc were invasted respectively. Seven patients died of tumor metastasis and/or intracranial lesions (50.0%, 7/14); the median survival time was 19 months. Four patients died of treatment (28.6%, 4/14), including 3 patients died of chemotherapy-related side effects, and 1 died of organ failure after enucleation surgery (7.1%); the median survival time was 3.5 months. Early abandonment of treatment died in 3 cases (21.4%, 3/14); the median survival time was 15 months.Conclusion:Intracranial metastasis is the main cause of death in children with intraocular RB.

El retinoblastoma: un tumor de ojo frecuente en la infancia / Retinoblastoma: a frequent eye tumor in childhood

Resumen El retinoblastoma es uno de los tumores intraoculares malignos más frecuentes en la edad pediátrica; así mismo, es posible que exista afectación en otros sitios como podría ser el ojo contralateral, por lo cual es importante mantener controles periódicos, aún después de ser diagnosticados y tratados. Actualmente se sabe que esta patología puede ser hereditaria o espontánea y tiene un importante componente genético, específicamente una mutación del gen supresor tumoral llamado RB1, el cual además está asociado a otras enfermedades.

Abstract Retinoblastoma is one of the most frequent malignant intraocular tumors in the pediatric age group, and it is possible that there is affectation to other sites such as the contralateral eye, hence, it is important to maintain periodic controls even after being diagnosed and treated. Currently it is known that this pathology can be either hereditary or spontaneous and has an important genetic component, specifically a mutation of the tumor-suppressor gene called RB1, which is also associated with other diseases.

Stimulating effects of conjunctival sac implants after eye enucleation on orbital bone tissue in rabbits / 中华实验眼科杂志

Objective To investigate the stimulating effects of conjunctival sac implants following eye enucleation on orbital bone tissue in rabbits model.Methods Eighty 1-month-old New Zealand rabbits were randomly divided into normal control group,only eyeball enucleation group,2-week interval group and 4-week interval group.The right eyeballs were enucleated in the rabbits in the only eyeball enucleation group,2-week interval group and 4-week interval group,and conjunctival sac implants were placed intraoperatively.Then a larger implant was replaced in a 2-week interval or 4-week interval in different groups,respectively.Spiral CT scan was used to evaluate the orbital bone development with aging.The peripheral blood of 3 ml was collected in the rabbits for the detection of osteocalcin and alkaline phosphatase using osteocalcin test kit (N-MID Osteocalcin) and alkaline phosphatase assay kit (NPP substrate-AMP buffer method),respectively.The animals were sacrificed at 3 months after operation for the histopathological examination of orbital bone tissue.Results The operation was successful in all the rabbits and no infection occurred after operation.The variance of orbital bone CT value was obvious in all the rabbits.The intercalated osteocalcin concentration was (48.55 ± 7.99),(59.80 ± 2.96),(57.94 ± 5.20) and (55.96 ± 3.22) μg/L and the alkaline phosphatase concentration was (284.66± 132.69),(232.96±54.39),(232.40± 118.23),(284.20± 130.41) μg/L in the normal control group,only eyeball enucleation group,2-week interval group and 4-week interval group,respectively,showing insignificant differences among the four groups (F =2.710,0.281,both at P ＞ 0.05).Similar pathological findings were in the 2-week interval group and 4-week interval group,such as no obvious orbital bone malformations,weak bone absorption and few bone osteoclasts under the optical microscope.Varying degrees of orbital deformities and bone tissue absorption were found in the only eyeball enucleation group.No orbital bone developing abnormality was seen in the normal control group and the left eyes in various groups under the optical microscope.Conclusions Compared with the only eyeball enucleated eyes,the orbital bone tissue has a well developed process in conjunctiva sac implant eyes following eyeball enucleation.Conjunctiva sac implant is an effective method to stimulate orbital growth and orbital volume increasing.

Indications for eye removal over a 13-year period at an ophthalmology referral center in São Paulo, Brazil / Indicações para remoção ocular ao longo de um período de 13 anos no centro de referência oftalmológica em São Paulo, Brazil

ABSTRACT Purpose: To analyze the indications and types of eye removal at Latin America's highest-volume ophthalmic reference center in São Paulo, Brazil. Methods: A retrospective analysis was conducted of surgical pathological reports in the electronic database of the Ophthalmology Department of the Universidade Federal de São Paulo Hospital of patients who underwent eye-removal procedures between January 2000 and December 2012. Results: A total of 412 cases met the inclusion criteria for this study. The most common indications for eye removal were ocular melanoma and retinoblastoma, representing 35.4% and 31.1% of the total cases, respectively. Other frequent indications included endophthalmitis (6.3%), nonspecific inflammation (4.1%), squamous cell carcinoma (3.6%), panophthalmitis (3.4%), and phthisis bulbi (1.2%). The remaining indications accounted for 14.8% of all cases, with each accounting for <1% of the total cases. Enucleation was the most common eye-removal procedure seen in our study. Conclusions: The two most common indications for eye removal were ocular melanoma and retinoblastoma. Alternative treatment options are effective in limiting the need for eye removal but are constrained by our public center's so cioeconomic context.

RESUMO Objetivo: Analisar os tipos e indicações para a remoção dos olhos no maior centro de referência oftalmológica da América Latina em São Paulo, Brasil. Métodos: Revisão retrospectiva de laudos cirúrgico patológico em uma base de dados eletrônica do Departamento de Oftalmologia do Hospital da Universidade Federal de São Paulo. Foram analisados pacientes submetidos ao procedimento de remoção oftálmica no período de janeiro de 2000 a dezembro de 2012. Resultados: Um total de 412 casos estiveram de acordo com os critérios de inclusão desse estudo. A indicação mais comum para remoção oftálmica foi Melanoma ocular e Retinoblastoma, representando, respectivamente, 35,4% e 31,1% do total de casos. Outras indicações frequentes incluíam Endoftalmite 6,3%, Inflamação inespecífica 4,1%, Carcinoma escamocelular 3,6%, Panoftalmite 3,4% e Phthisis Bulbi 1,2%. O restante das indicações totalizaram 14,8% de todos os casos, sendo que cada um correspondem com menos de 1% do total. Enucleação foi o procedimento de remoção oftálmica mais comum observado em nosso estudo. Conclusão: As duas indicações mais frequentes para remoção oftálmica no centro oftalmológico de referência do Brasil foi melanoma e retinoblastoma. Opções terapêuticas alternativas são efetivas para limitar a necessidade da remoção ocular, porém são restritos devido ao contexto socioeconômico de nossa população.

Nanoskin: uso para reposição de volume na cavidade anoftálmica / Use of Nanoskin for volume replacement of the eye socket

RESUMO Objetivo: Avaliar a biocompatibilidade da Nanoskin para reposição de volume em cavidades enucleadas ou evisceradas de coelhos. Métodos: Estudo experimental, utilizando implantes de Nanoskin (Innovatecs®, São Carlos, Brasil), celulose bacteriana produzida pela bactéria Acetobacter xylinum tendo como substrato o chá-verde. Implantes de 10mm de diâmetro/5mm de espessura foram colocados em cavidades enucleadas (G1) ou evisceradas (G2) de 21 coelhos, avaliados clinicamente todos os dias, sacrificados aos 7, 30 e 90 dias após a cirurgia. O material foi removido e preparado para exame de microscopia óptica. Resultados: Sinais flogísticos discretos no pósoperatório imediato, não tendo sido evidenciados sinais infecciosos ou extrusão de nenhum implante. Houve aparente redução do volume ao longo do período experimental. Histologicamente ambos os grupos foram muito semelhantes, apresentando aos 7 dias células inflamatórias (predominantemente monócitos e neutrófilos), rede de fibrina e hemácias. A Nanoskin apresentava-se como pequenas esferas, de cor rósea, com pequenos espaços entre elas, permeados por escassas células inflamatórias. As células inflamatórias se modificaram ao longo de período experimental, sendo possível observar aos 30 dias células gigantes multinucleadas e fibroblastos maduros permeando o implante. Aos 90 dias, a estrutura do implante apresentava-se desorganizada, amorfa, com restos necróticos e com áreas ovoides, revestidas por fina membrana rósea, que pareciam se agrupar, vazias ou preenchidas por material acelular, róseo ou acinzentado. Conclusão: A Nanoskin provocou reação inflamatória que levou à reabsorção e redução do volume do implante. Novas formulações devem ser estudadas a fim de ter um produto que seja permanente para reparo da cavidade anoftálmica.

ABSTRACT Objective: The aim of this study was to evaluate the biocompatibility of Nanoskin for replacing volume in enucleated or eviscerated anophthalmic sockets of rabbits. Methods: An experimental study was carried out using enucleated or eviscerated rabbits, which received Nanoskin implants (Innovatecs®, São Carlos, Brazil), a cellulose produced by a bacteria (Acetobacter xylinum) using green tea as substrate. Implants of 10mm diameter/5mm of thickness were used placed in enucleated (G1) or eviscerated (G2) anophthalmic sockets of 21 rabbits.They were clinically examined daily, sacrificed at 7, 30 and 90 days after surgery and the material was removed and prepared for histological examination. Results: There were discrete signs of inflammation in the immediate postoperative period, with no evidence of infection or extrusion in any animal. However apparent reduction of volume during the trial period occurred. Histologically both groups were similar, with inflammatory cells (mainly monocytes and neutrophils), fibrin and hemaceas at 7 days postoperatively.The Nanoskin was presented as small pink spheres, with small gaps between them and permeated by few inflammatory cells. These cells have changed over the study, at 30 days multinucleated giant cells and mature fibroblasts that permeate the implant were observed. At 90 days, the structure of the implant was disorganized, amorphous, with necrotic debris and ovoid areas covered with thin pink membrane that seemed to cluster, empty or filled with no cellular pink or gray material. Conclusion: Nanoskin caused an inflammatory reaction leading to reabsorption and reduction of implant volume. New formulations should be studied in order to have a permanent product to repair the anophthalmic socket.

Blood flow velocity in monocular retinoblastoma assessed by color doppler

OBJECTIVE: To analyze the flow of retrobulbar vessels in retinoblastoma by color Doppler imaging. METHODS: A prospective study of monocular retinoblastoma treated by enucleation between 2010 and 2014. The examination comprised fundoscopy, magnetic resonance imaging, ultrasonography and color Doppler imaging. The peak blood velocities in the central retinal artery and central retinal vein of tumor-containing eyes (tuCRAv and tuCRVv, respectively) were assessed. The velocities were compared with those for normal eyes (nlCRAv and nlCRVv) and correlated with clinical and pathological findings. Tumor dimensions in the pathological sections were compared with those in magnetic resonance imaging and ultrasonography and were correlated with tuCRAv and tuCRVv. In tumor-containing eyes, the resistivity index in the central retinal artery and the pulse index in the central retinal vein were studied in relation to all variables. RESULTS: Eighteen patients were included. Comparisons between tuCRAv and nlCRAv and between tuCRVv and nlCRVv revealed higher velocities in tumor-containing eyes (p <0.001 for both), with a greater effect in the central retinal artery than in the central retinal vein (p =0.024). Magnetic resonance imaging and ultrasonography measurements were as reliable as pathology assessments (p =0.675 and p =0.375, respectively). A positive relationship was found between tuCRAv and the tumor volume (p =0.027). The pulse index in the central retinal vein was lower in male patients (p =0.017) and in eyes with optic nerve invasion (p =0.0088). CONCLUSIONS: TuCRAv and tuCRVv are higher in tumor-containing eyes than in normal eyes. Magnetic resonance imaging and ultrasonography measurements are reliable. The tumor volume is correlated with a higher tuCRAv and a reduced pulse in the central retinal vein is correlated with male sex and optic nerve invasion.

Long-term Surgical Outcomes of the Multi-purpose Conical Porous Synthetic Orbital Implant

PURPOSE: We present clinical results of the use of the multipurpose conical porous synthetic orbital implant (MCOI) in surgical procedures of evisceration, enucleation, and secondary enucleation in ophthalmology patients. METHODS: A retrospective review was performed of 59 eyes in which conical implants were used, including 36 cases of eviscerations, 11 enucleations, and 9 secondary enucleations. In all of the cases, the follow-up period was greater than six months between 2004 and 2013. The results focus on documenting surgical findings, as well as postoperative complications among patients. RESULTS: Superior sulcus deformities were found in six eyes (10.2% of conical implant patients), and two eyes received additional surgical interventions to correct the deformities (3.4%). Blepharoptosis was found in four eyes (6.8%), two of which received upper eyelid blepharoplasty (3.4%). Fornix shortening was reported in only one eye (1.7%). Forty-one eyes had a satisfactory cosmetic appearance after the final prosthetic fitting of conical implants (69.5%). The most frequent postoperative complication was orbital implant exposure, which seemed to occur when the preoperative status of the conjunctiva, Tenon's capsule, and sclera preservation were poor in the eyes of the patients. CONCLUSIONS: There was a lower incidence of blepharoptosis and fornix shortening with the MCOI in comparison to spherical implants, while the incidence of orbital implant exposure was similar with the MCOI in comparison to other types of orbital implants. In addition, the MCOI may have advantages with respect to postoperative cosmetic outcomes.

Diagnosis & Treatment of Retinoblastoma: Current Review / 임상소아혈액종양

Retinoblastoma is a rare disease, but most common tumor which arises in eye. It can affect one or both eyes, and the main pathophysiology is explained by the "Two-hit theory" - the germline mutation of the RB1 gene. Most common clinical symptoms are leuocoria, strabismus, poor visual tracking, glaucoma, and orbital cellulitis. Diagnosis is made by ophthalmologist through fundoscopic examination; Examination under General Anesthesia (EUA) is recommended until the age 3. Orbital CT and MRI can detect the tumor invasion on optic nerve, central nervous system. CSF studies, examination of bone is helpful if the distant metastasis is suspected. Biopsy is rarely done unless in the case of enucleation. Enucleated eye should be explored for the invasion to the optic nerve, choroid, anterior chamber, iris and pupil. Treatment strategies can be different according to the disease status. If the single eye is involved, the treatment goal will be the removal of tumor and prevention of relapse. Local therapies include cryotherapy, laser photocoagulation, thermotherapy can be the choice, and if the tumor is too large for the local therapy, enucleation should be concerned. Nowadays, chemo-reduction combined with local therapy, intra-arterial and intravitreous chemotherapeutic agent injections are studied to avoid enucleation. In bilateral retinoblastoma, multidisciplinary treatments include chemoreduction, external beam radiotherapy, local therapy and other experimental therapies are needed: like intra-arterial injection, intra-vitreal injection, and high-dose chemotherapy with autologous stem cell transplantation. Early detection of retinoblastoma is important to save the vision and eyeball.

Diagnosis & Treatment of Retinoblastoma: Current Review / 임상소아혈액종양

Retinoblastoma is a rare disease, but most common tumor which arises in eye. It can affect one or both eyes, and the main pathophysiology is explained by the "Two-hit theory" - the germline mutation of the RB1 gene. Most common clinical symptoms are leuocoria, strabismus, poor visual tracking, glaucoma, and orbital cellulitis. Diagnosis is made by ophthalmologist through fundoscopic examination; Examination under General Anesthesia (EUA) is recommended until the age 3. Orbital CT and MRI can detect the tumor invasion on optic nerve, central nervous system. CSF studies, examination of bone is helpful if the distant metastasis is suspected. Biopsy is rarely done unless in the case of enucleation. Enucleated eye should be explored for the invasion to the optic nerve, choroid, anterior chamber, iris and pupil. Treatment strategies can be different according to the disease status. If the single eye is involved, the treatment goal will be the removal of tumor and prevention of relapse. Local therapies include cryotherapy, laser photocoagulation, thermotherapy can be the choice, and if the tumor is too large for the local therapy, enucleation should be concerned. Nowadays, chemo-reduction combined with local therapy, intra-arterial and intravitreous chemotherapeutic agent injections are studied to avoid enucleation. In bilateral retinoblastoma, multidisciplinary treatments include chemoreduction, external beam radiotherapy, local therapy and other experimental therapies are needed: like intra-arterial injection, intra-vitreal injection, and high-dose chemotherapy with autologous stem cell transplantation. Early detection of retinoblastoma is important to save the vision and eyeball.

Managing the consequences of aggressive conservative treatment for refractory retinoblastoma with vitreous seeding / Lidando com as consequências do tratamento agressivo para retinoblastoma refratário com semeadura vítrea

A 4 year-old girl with bilateral, non-familial retinoblastoma (RB) was referred to our care after primary enucleation OS and active tumor OD refractory to multiple therapies (intravenous chemotherapy, laser/cryotherapy, and I-125 plaque radiotherapy). Vitreous seeding OD, initially controlled by several sessions of Ophthalmic Artery Infusion Chemotherapy (OAIC) and periocular chemotherapy, recurred shortly thereafter. The patient underwent intravitreal (IVit) Melphalan injections achieving tumor control despite the concurrent development of keratopathy, pupillary synechiae, cataract, and necrosis of the inferior fornix and the adjacent orbital fat, all secondary to the treatments administered. Repeated amniotic membrane implants and tarsorrhaphy were performed to alleviate the symptoms. Despite being tumor free for 6 months, a poor fundus view and treatment-related complications prompted us to consider enucleation, but parents declined. Following recent negative magnetic resonance imaging (MRI), her cataract was removed. She was then found to have tumor recurrence. Her eye was enucleated 12 months ago and she recovered well from the surgery. As ocular oncology embarks in eye-preserving treatments for retinoblastoma, it is important to address the cumulative effects and associated impact of such treatments and the possibility of failure.

Uma menina de 4 anos com retinoblastoma (RB) bilateral, não-familiar foi encaminhada após enucleação OE e tumor ativo OD refratário a múltiplas terapias (quimioterapia endovenosa, laser/crioterapia e braquiterapia com I-125). Semeadura vitrea OD, inicialmente controlada por inúmeras sessões de Quimioterapia Intra-Arterial Oftálmica (QIAO) e quimioterapia periocular, recorreu em seguida. Paciente recebeu injeções intravítreas de Melphalan obtendo controle tumoral apesar do desenvolvimento concomitante de ceratopatia, sinéquias pupilares, catarata, necrose do fórnice inferior e gordura periorbitária adjacente, todos secundários aos tratamentos usados. Implantes repetidos de membrana amniótica e tarsorrafias foram realizadas para melhora sintomatológica. Apesar de estar livre de tumor por 6 meses, a baixa visibilidade do fundo e complicações terapêuticas nos levaram a considerar enucleação que foi descartada pelos pais. Após recente ressonância magnética nuclear (RMN) negativa, a catarata foi removida. Foi então detectada recorrência tumoral. O olho foi enucleado há 12 meses e ela se recuperou bem da cirurgia. Enquanto a oncologia ocular embarca em tratamentos para preservar em retinoblastoma, é importante considerar os efeitos cumulativos e impacto associado desses tratamentos, e a possibilidade de fracasso.

Combined chemotherapy and intra-arterial chemotherapy of retinoblastoma / 소아과

PURPOSE: Retinoblastoma (RB) is the most common primary malignant intraocular tumor in children. Although systemic chemotherapy has been the primary treatment, intra-arterial chemotherapy (IAC) represents a new treatment option. Here, we performed alternate systemic chemotherapy and IAC and retrospectively reviewed the efficacy and safety of this approach. METHODS: Patients diagnosed with intraocular RB between January 2000 and December 2011 at Severance Children's Hospital, Yonsei University, were reviewed. Before February 2010, the primary treatment for RB was chemotherapy (non-IAC/CTX). Since February 2010, the primary treatment for RB has been IAC (IAC/CTX). External beam radiotherapy or high-dose chemotherapy were used as "last resort" treatments just prior to enucleation at the time of progression or recurrence during primary treatment. Enucleation-free survival (EFS) and progression-free survival were assessed. RESULTS: We examined 19 patients (median age, 11.9 months; range, 1.4 to 75.6 months) with a sum of 25 eyes, of which, 60.0% were at advanced Reese Ellsworth (RE) stages. The enucleation rate was 33.3% at early RE stages and 81.8% at advanced RE stages (P=0.028). At 36 months, EFS was significantly higher in the IAC/CTX group than in the non-IAC/CTX group (100% vs. 40.0%, P=0.016). All 5 patients treated with IAC achieved eye preservation, although most patients were at advanced RE stages (IV-V). CONCLUSION: Despite the limitation of a small sample size, our work shows that an alternative combined approach using IAC and CTX may be safe and effective for eye preservation in advanced RB.

Phacoemulsification and foldable acrylic IOL implantation in children with treated retinoblastoma / Facoemulsificação e implante de lente intraocular acrílica dobrável em crianças com retinoblastoma

PURPOSE: To study the results of cataract surgery in children with radiation-induced cataract after treatment for retinoblastoma. METHODS: Retrospective interventional case series. Six consecutive patients diagnosed with secondary cataracts due to radiation therapy for retinoblastoma. Intervention: Phacoemulsification and foldable acrylic intraocular lens implantation. Outcomes measu- red: Visual acuity, binocular indirect ophthalmoscopy and slit-lamp biomicroscopy. Aspirated lens material and aqueous humor samples were collected during surgery. RESULTS: Six uniocular children between 3 to 5 years of age at time of surgery were studied. The mean time interval between radiotherapy and cataract diagnosis was 22.3 months. The mean follow-up after surgery was 17.2 months (range: 12 to 23 months). All eyes achieved a clear visual axis after surgery allowing monitoring of the tumor status. None developed recurrence or retinoblastoma dissemination. Histopathological analysis of the aspired material showed no tumoral cells in all samples. All patients improved vision after cataract surgery. CONCLUSIONS: Phacoemulsification with acrylic intraocular lens implantation seems to be a safe, feasible, and effective method for the removal of radiation-induced cataracts in patients with treated retinoblastoma.

OBJETIVOS: Estudar os resultados da cirurgia da catarata induzida pela radioterapia para o tratamento do retinoblastoma em crianças. MÉTODOS: Estudo retrospectivo intervencional em série de casos onde seis pacientes consecutivos apresentaram catarata secundária à terapia por radiação para o retinoblastoma. Intervenção: Facoemulsificação e implante de lente intraocular acrílica dobrável. Foram avaliadas: acuidade visual, oftalmoscopia binocular indireta e biomicroscopia. Material para análise histológica do cristalino e do humor aquoso foi coletado durante as cirurgias. RESULTADOS: Seis crianças, entre 3 e 5 anos de idade, com catarata secundária à radiação para tratamento de retinoblastoma foram submetidas à cirurgia de facoemulsificação com implante de lente intraocular. A média do intervalo de tempo decorrido entre a radioterapia e o diagnóstico da catarata foi 22,3 meses. O período médio de seguimento após a cirurgia foi de 17,2 meses (intervalo: 12 a 23 meses). Todos os olhos melhoraram a visão e mantiveram eixo visual livre permitindo a fundoscopia para monitorar o tumor. Nenhum paciente evoluiu com recorrência ou disseminação do retinoblastoma. A análise histopatológica do material colhido resultou em ausência de células tumorais nas amostras. CONCLUSÕES: A cirurgia de facoemulsificação com implante de lente intraocular acrílica dobrável mostrou ser um procedimento seguro e efetivo para o tratamento da catarata induzida pela radioterapia em pacientes portadores de retinoblastoma.

Perfil dos pacientes submetidos à reconstrução primária da cavidade orbitária com implante de Mules após enucleação e evisceração / Primary reconstruction of the orbital cavity with Mules implant after enucleation and evisceration patients' profile

OBJETIVO: Determinar o perfil clínico dos pacientes submetidos à reconstrução da cavidade orbitária com implante de Mules. MÉTODOS: Foi realizado um estudo epidemiológico com delineamento transversal, baseado na análise dos prontuários de pacientes submetidos à cirurgia de enucleação ou evisceração do globo ocular assistidos no Departamento de Plástica Ocular e Órbita do Hospital Regional de São José (HRSJ) entre dezembro de 1999 a dezembro de 2009. As variáveis utilizadas foram: idade, sexo, cirurgia realizada, indicação, tamanho do implante e complicações pós-operatórias associadas. RESULTADOS: A análise incidiu sob registros de 68 pacientes, com predomínio do gênero masculino (60,3%) cuja média de idade foi de 38,4 anos. Adultos jovens destacaram-se com a maior frequência de perda do globo ocular. As principais etiologias que cursaram com a enucleação ou evisceração do globo ocular foram trauma ocular, glaucoma, melanoma de coróide e phthisis bulbi. O trauma ocular apresentou maior prevalência no sexo masculino (p<0,01). As complicações pós-cirúrgicas com maior frequência foram expulsão ou extrusão do implante (20,6%) e retração de cavidade ocular (5,9%). CONCLUSÃO: As principais causas de perda do globo ocular foram trauma ocular, glaucoma, melanoma de coroide e phthisis bulbi. As complicações pós-cirúrgicas após implante de Mules foram expulsão ou extrusão do implante e retração de cavidade ocular. Os implantes de 15 e 16 mm apresentaram maior assiduidade e o tamanho da esfera não interferiu no risco de extrusão.

OBJECTIVE: To access the patient's profile after enucleation or evisceration of ocular globe using mules implant. METHODS: An epidemiological study with a cross-sectional design was conducted, based on analysis of medical records of patients who underwent enucleation or evisceration of the eyeball assisted in the Department of Ocular Plastic and Orbit of the HRSJ from December 1999 to December 2009. The variables used were age, sex, surgery, indication, size of implant and postoperative complications associated. RESULTS: A total of 68 patients were analyzed. The mean age was 38,4 years and the most prevalent genre was male (60.3%). Young adults stood out with greater frequency of loss of the eyeball. The main etiologies presenting with enucleation or evisceration of the eyeball were ocular trauma, glaucoma, choroidal melanoma and phthisis bulbi. The ocular trauma was more prevalent in males (p<0.01). The postoperative complications more frequently were expulsion or extrusion of the implant (20.6%) and retraction of the orbital cavity (5.9%). CONCLUSION: The main causes of loss of the eyeball were ocular trauma, glaucoma, choroidal melanoma and phthisis bulbi. The postoperative complications after Mules´ implant were expulsion or extrusion of the implant and retraction of the orbital cavity. The implants 15 and 16 mm showed greater frequency and the size of the ball did not affect the risk of extrusion.

Use of a gel biopolymer for the treatment of eviscerated eyes: experimental model in rabbits / Uso de um biopolímero na forma gel para o tratamento de olhos eviscerados: modelo experimental em coelhos

PURPOSE: To evaluate histologically the integration process of cellulose gel produced by Zoogloea sp when implanted into rabbits' eviscerated eyes. METHODS: This experimental study employed 36 eyes of 18 rabbits subjected to evisceration of their right eyes. The sclerocorneal bag was sutured and filled with biopolymer from sugar cane in the gel state. All animals were clinically examined by biomicroscopy until the day of their sacrifice which occurred on the 7th, 30th, 60th, 90th, 120th, or 240th day. The eyeballs obtained, including the left eyes considered controls were sent for histopathological study by optical macroscopy and microscopy. Tissue staining techniques used included hematoxylin-eosin, Masson trichrome (with aniline), Gomori trichrome, Van Gienson, Picrosirius red, and periodic acid-Schiff (PAS). RESULTS: No clinical signs of infection, allergy, toxicity, or extrusion were observed throughout the experiment. The corneas were relatively preserved. Macroscopic examination revealed a decrease of ~ 8% in the volume of the bulbs implanted with the biopolymer. After cutting, the sclerocorneal bag was solid, compact, elastic, and resistant to traction, with a smooth and whitish surface, and showed no signs of necrosis or liquefaction. The episcleral tissues were somewhat hypertrophied. The histological preparations studied in different colors revealed an initial lymphoplasmacytic infiltration, replaced by a fibroblastic response and proliferation of histiocytes, along with formation of giant cells. Few polymorphonuclearneutrophils and eosinophils were also found. Neovascularization and collagen deposition were present in all animals starting from day 30; although on the 240th day of the experiment the chronic inflammatory response, neovascularization and collagen deposition had not yet reached the center of the implant. CONCLUSION: In this model, the cellulose gel produced by Zoogloea sp proved to be biocompatible and integrated into the orbits. Morphometric, immunohistochemical and biodegradability studies should be performed in the future.

OBJETIVOS: Avaliar histologicamente o processo de integração do gel da celulose produzida pela Zoogloe asp implantado em olhos eviscerados de coelhos. MÉTODOS: Estudo experimental utilizando 36 olhos de 18 coelhos. Todos eles tiveram seus olhos direitos eviscerados. A bolsa escleral foi suturada e preenchida com biopolimero da cana de açúcar, no estado gel. Todos os animais foram examinados clinicamente, sob biomicroscopia, até o dia de seus sacrifícios e enucleações que aconteceram no 7º, 30º, 60º,90º,120º e 240º dia. Os bulbos obtidos, inclusive os esquerdos, considerados controles, foram encaminhados para estudo histopatológicos de macroscopia e microscopia óptica. As colorações pela hematoxilina-eosina, tricômio de Masson (com anilina), tricômio de Gomori, Van Gienson e Picrosirius red e ácido periódico de Schiff (PAS) foram usadas. RESULTADOS: Durante o experimento não foram observados sinais clínicos de infecção, alergias, intoxicação ou extrusão. As córneas apresentavam-se relativamente preservadas. Exame macroscópico revelou uma diminuição de aproximadamente 8% no volume dos bulbos nos quais o biopolímero foi implantado. O corte do saco escleral mostrou um conteúdo sólido, compacto, elástico, resistente à tração, com superfície lisa e brancacenta. Não foram observados sinais de necrose, ou liquefação. O tecido epiescleral estava algo hipertrofiado. As preparações histológicas estudadas, nas diversas colorações, revelaram uma infiltração linfomonomorfonuclear inicial, substituída posteriormente por uma resposta fibroblástica e proliferação de histiócitos com formação de células multinucleadas gigantes. Foram também encontrados poucos polimorfonucleares neutrófilos e eosinófilos. A partir do 30º dia houve proliferação vascular e deposição de calágeno em todos os espécimes estudados, embora, no 240º dia do experimento, a resposta inflamatória crônica, a neovascularização e a deposição do colágeno não tinham ainda atingido o centro do implante. CONCLUSÃO: Neste modelo, o gel da celulose produzido pela Zoogloea sp, mostrou-se biocompatível e integrado às órbitas. Estudos morfométrico, imuno-histoquímico e de biodegradabilidade devem ser realizados futuramente.

Estudo comparativo entre as esferas de silicone gelatinosas e rígidas em cavidade eviscerada de coelho / Comparative study between gelatinous and hard spheres of silicone in rabbit eviscerated scleral cavity

OBJETIVO: Avaliar e comparar a biocompatibilidade de esferas de silicone gelatinosas e rígidas em cavidades evisceradas de coelhos. MÉTODOS: Trinta coelhos tiveram o olho direito eviscerado com implantação de esferas de silicone gelatinosas (Grupo I) ou rígidas (Grupo II). Foi realizada avaliação clínica diária, ultrassonografia da cavidade orbitária, análise histológica e morfométrica da pseudocápsula que se formou ao redor dos implantes aos 7, 30 e 90 dias após a cirurgia, com avaliação estatística dos resultados. RESULTADOS: Houve boa integração das esferas com os tecidos orbitários e semelhança de resposta tecidual com ambas as esferas. Duas esferas de silicone gelatinosas e uma rígida extruíram. A pseudocápsula que se formou ao redor das esferas gelatinosas foi mais organizada, com espessura e reação inflamatória menores que a observada nas esferas rígidas. CONCLUSÕES: Esferas de silicone gelatinosas e rígidas tiveram boa integração tecidual em cavidades evisceradas de coelhos.

PURPOSE: To evaluate and to compare the biocompatibility of gelatinous and hard silicone spheres placed into eviscerated scleral cavities of rabbits. METHOD: Thirty rabbits underwent right eye evisceration surgery and replacement of orbital volume using gelatinous (Group I) or hard silicone (Group II) spheres. Seven, 30 and 90 days after the surgical procedure, clinical assessment, ultrasound of the orbit, histological and morphometric evaluation of the pseudocapsule were performed. Data was submitted to statistical analysis. RESULTS: Similarity of tissue response was observed with both materials. Two gelatinous and one hard silicone spheres had extrusion. The pseudocapsule around the gelatinous spheres was better organized, thinner and with less inflammatory reaction. CONCLUSIONS: Both spheres had good integration to the orbital tissue in rabbit eviscerated cavities.