Rat Model of Type 2 Diabetes Mellitus Recapitulates Human Disease in the Anterior Segment of the Eye.

Changes in the anterior segment of the eye due to type 2 diabetes mellitus (T2DM) are not well-characterized, in part due to the lack of a reliable animal model. This study evaluated changes in the anterior segment, including crystalline lens health, corneal endothelial cell density, aqueous humor metabolites, and ciliary body vasculature, in a rat model of T2DM compared with human eyes. Male Sprague-Dawley rats were fed a high-fat diet (45% fat) or normal diet, and rats fed the high-fat diet were injected with streptozotocin intraperitoneally to generate a model of T2DM. Cataract formation and corneal endothelial cell density were assessed using microscopic analysis. Diabetes-related rat aqueous humor alterations were assessed using metabolomics screening. Transmission electron microscopy was used to assess qualitative ultrastructural changes ciliary process microvessels at the site of aqueous formation in the eyes of diabetic rats and humans. Eyes from the diabetic rats demonstrated cataracts, lower corneal endothelial cell densities, altered aqueous metabolites, and ciliary body ultrastructural changes, including vascular endothelial cell activation, pericyte degeneration, perivascular edema, and basement membrane reduplication. These findings recapitulated diabetic changes in human eyes. These results support the use of this model for studying ocular manifestations of T2DM and support a hypothesis postulating blood-aqueous barrier breakdown and vascular leakage at the ciliary body as a mechanism for diabetic anterior segment pathology.

Tuberculous ciliary body granuloma initially diagnosed as bullous retinal detachment: a case report.

BACKGROUND: Ocular tuberculosis is a relatively rare extrapulmonary manifestation of tuberculosis. This vision-threatening disease is extremely challenging to diagnose, particularly because it can mimic other diseases. We report a case of tuberculous ciliary body granuloma initially diagnosed as bullous retinal detachment. CASE REPORT: A 52-year-old female presented with bullous retinal detachment in her left eye, and ultrasound biomicroscopy (UBM) verified the presence of a lesion with ciliary body granulomatous inflammation. The T-SPOT was positive, and the purified protein derivative (PPD) test was strongly positive (diameter of 20 mm). Following the administration of oral anti-tuberculosis regimen combined with prednisone, the retina gradually became reattached, the ciliary body granuloma became significantly reduced in size, and the visual acuity of the patient noticeably improved. CONCLUSIONS: Tuberculous ciliary body granulomas can cause bullous exudative retinal detachment and can be diagnosed with UBM. Early and full-course anti-tuberculosis treatment (ATT) combined with corticosteroid therapy can improve the patient prognosis.

Local resection via partial lamellar sclerouvectomy for ciliary body tumors - a case series.

BACKGROUND: Ciliary body tumor is extremely rare and treatment is challenging. The aim of this study is to present our experience in treating this rare entity, especially large tumors with more than 5 clock hours of involvement, and to evaluate the surgical outcomes and complications of local resection via partial lamellar sclerouvectomy in four cases of ciliary body tumors in China. METHODS: Four patients with ciliary body tumors underwent partial lamellar sclerouvectomy between October 2019 and April 2023 in Shanghai General Hospital, China. Tumor features, histopathologic findings, complications, visual acuity, and surgical outcomes were reviewed at a mean follow-up of 20.8 months. RESULTS: Four patients with a mean age of 31.8 years were included in this study. The histopathological diagnosis was adenoma of non-pigmented ciliary epithelium (ANPCE), schwannoma, and multiple ciliary body pigment epithelial cysts. The mean largest tumor base diameter was 6.00 mm (range: 2.00-10.00) and the mean tumor thickness was 3.50 mm (range: 2.00-5.00). Preoperative complications included cataract in 3 (75%) eyes, lens dislocation in 2 (50%), and secondary glaucoma in 1 (25%). Temporary ocular hypotonia was observed in one case and no other postoperative complications were observed. At a mean follow-up of 20.8 months, the best corrected visual acuity increased in 3 eyes and was stable in 1 eye. Tumor recurrence was absent in all eyes. All patients were alive at the end of follow-up. CONCLUSIONS: Local tumor resection via PLSU is useful in the treatment of ciliary body tumors, including large tumors occupying more than five clock hours of pars plicata. Surgery-related complications were manageable with adequate preoperative assessment and careful operation during surgery.

Lens height paraments comparison according to ciliary sulcus width (CSW): a pilot study of the predictive role of CLR and STSL for vault after ICL implantation.

BACKGROUND: To assess the relationship between postoperative implantable collamer lens (ICL) vault and lens height obtained from two different measurements. METHODS: A retrospective case series study enrolled eyes with horizontally implanted ICL. Crystal lens rise (CLR) and the distance between STS plane and anterior crystalline lens surface (STSL) were measured in the horizontal and vertical directions using ultrasound biomicroscopy (UBM). We compared the differences in the parameters measured in both horizontal and vertical directions. The participants were categorized into three groups according to ciliary sulcus width (CSW) which is defined as the distance between the posterior angle of the iris and the anterior angle of the ciliary process: narrow CSW group (NSG); medium CSW group (MSG); and wide CSW group (WSG). The correlations between CLR/STSL and vault were examined in each of the three groups. Biased correlation analysis was used further to contrast the correlation between CLR/STSL and vault. RESULTS: This retrospective study included 223 myopic eyes. Vertical STSL (VSTSL) and vertical CLR (VCLR) exhibited significantly greater values compared to their horizontal counterparts (both P < 0.05). None of the indicators were statistically different between the three groups. In both NSG and MSG, STSL/CLR correlated with vault, while in WSG, only STSL correlated with vault (r=-0.316, P = 0.013). In contrast to HCLR, the correlation between HSTSL and vault remained after controlling for HCLR (r=-0.162, P = 0.015). CONCLUSIONS: STSL should deserve more attention in the preoperative evaluation of ICL compared to CLR especially when CSW is large.

Pigmented medulloepithelioma of the optic nerve: A challenging diagnostic entity.

Because of its rarity, the diagnosis of optic nerve medulloepithelioma poses a real diagnostic challenge. Medulloepithelioma is a congenital tumor that derives from the primitive medullary epithelium present in the neural tube and the optic vesicle. Its classical location is the ciliary body. Cases of retinal or optic nerve locations have been rarely reported in the literature. Only 11 cases have been published in the English literature. Herein, we report the case of a 2-year-old boy who underwent enucleation of the right eye for a presumed diagnosis of right-eye retinoblastoma, based on the presence of leukocoria on ophthalmological examination. Pathological examination showed an optic nerve medulloepithelioma. A review of the literature is also discussed in our work.

CRISPR-Cas9-mediated deletion of carbonic anhydrase 2 in the ciliary body to treat glaucoma.

The carbonic anhydrase 2 (Car2) gene encodes the primary isoenzyme responsible for aqueous humor (AH) production and plays a major role in the regulation of intraocular pressure (IOP). The CRISPR-Cas9 system, based on the ShH10 adenovirus-associated virus, can efficiently disrupt the Car2 gene in the ciliary body. With a single intravitreal injection, Car2 knockout can significantly and sustainably reduce IOP in both normal mice and glaucoma models by inhibiting AH production. Furthermore, it effectively delays and even halts glaucomatous damage induced by prolonged high IOP in a chronic ocular hypertension model, surpassing the efficacy of clinically available carbonic anhydrase inhibitors such as brinzolamide. The clinical application of CRISPR-Cas9 based disruption of Car2 is an attractive therapeutic strategy that could bring additional benefits to patients with glaucoma.

Leukemic infiltration of the ciliary body in a child with T-cell acute lymphoblastic leukemia.

A 14-year-old boy presented after 2 months of vision loss, redness, and pain in the right eye, initially treated as anterior uveitis with topical corticosteroids. He had a 1-year history of T-cell acute lymphoblastic leukemia, which had been in remission for 6 months. On examination, visual acuity in the right eye was light perception, with 4+ anterior chamber cells, pupillary membrane, and an intumescent cataract. Ultrasound biomicroscopy (UBM) revealed a ciliary body mass and capsular bag rupture. After consultation with his oncologist, he received 10 radiotherapy sessions. At 1 month, UBM showed resolution of the mass. After 1 year of remission, the patient underwent pars plana vitrectomy, pupillary membranectomy, and placement of a scleral-fixated intraocular lens. Thirty months after surgery, visual acuity was 20/25. Leukemic infiltration of the ciliary body is a rare manifestation of the disease that is often misdiagnosed as uveitis.

Leiomyoma - Ciliary Body Tumor. A Case Report.

AIM: To demonstrate a rare case of ciliary body leiomyoma in our patient Case report: A 72-year-old female reported to our clinic for a preventive examination, upon which we found a dome-shaped grey-brownish mass on the retinal periphery. After completing gonioscopic and ultrasound examinations, we referred the patient to a specialist facility. Due to a finding of suspicious malignant melanoma, we completed the MRI scan and recommended enucleation of the eyeball. A histopathological examination showed a leiomyoma of the ciliary body. CONCLUSION: The aim of this case report is to demonstrate the difficulty of intraocular leiomyoma diagnosis. Only immunohistochemical examination differentiated the tumor from malignant melanoma and determined the diagnosis of ciliary body leiomyoma. Perhaps because of the extreme rarity of this type of tumor, we often neglect to consider a diagnosis of leiomyoma.

Anterior segment swept-source optical coherence tomography and ultrasound biomicroscopy in iris and ciliary body lesions.

OBJECTIVES: Differentiation of iris and ciliary body lesions as benign or malignant and cystic or solid is important. The aim of this study was to compare anterior segment swept-source optical coherence tomography (AS SS-OCT) and ultrasound biomicroscopy (UBM) findings in iris and ciliary body tumors. RESEARCH DESIGN AND METHODS: Forty-two eyes of 38 cases with iris and ciliary body tumors imaged with UBM and AS SS-OCT between September 2018 and September 2023 were evaluated retrospectively. RESULTS: Of 42 eyes, 14 had melanoma, 14 iris pigment epithelial (IPE) cysts, 7 nevi, 3 Lisch nodules, 2 iris stromal cysts, 1 pars plana cysts, and 1 iris mammillations. An equivalent (100%) visualization of the anterior tumor margin was obtained with both techniques. Compared to AS SS-OCT, UBM was superior for posterior margin visualization in melanocytic tumors and IPE cysts. Bland-Altman plots demonstrated good agreement between UBM and AS SS-OCT for melanocytic tumors < 2.5 mm in base diameter and < 2 mm in thickness. CONCLUSIONS: Although, UBM is the gold standard for ciliary body and iridociliary tumors. AS SS-OCT should be considered as an excellent alternative to UBM, especially in minimally elevated iris lesions.

Intraocular schwannoma: case series of 28 patients and literature review.

PURPOSE: Intraocular schwannoma is a rare tumour, which is often misdiagnosed. We presented the demographics and clinical characteristics of patients with intraocular schwannoma. METHODS: Retrospective case series were collected between May 2005 and July 2021 in Beijing Tongren Hospital. RESULTS: A total of 28 patients were diagnosed with intraocular schwannoma on histopathological examination of surgical specimen. The median age was 39 years (range: 12-64). Fourteen patients were female and 14 were male. Among the all subjects, 21/28 patients (75.0%) presented as visual loss, and 3/28 patients (10.7%) had visual field loss. Intraocular schwannoma presented as nonpigmented mass in the ciliary body in 12/28 cases (42.9%), in the choroid in 9/28 cases (32.1%), and in ciliochoroid in 7/28 cases (25.0%). Intraocular schwannoma was often clinically misdiagnosed as uveal melanoma, which occurred in 16/28 patients (57.1%). Tumour excision with pars plana vitrectomy was performed for all included patients. Endoresection with lens removal was performed for tumours in the choroid, while transscleral resection was performed for tumours located in ciliary body or ciliochoroid. Increased light transmission was detected in 12/28 cases (42.9%). In the consecutive follow-up (median: 73 months, range: 7-193 months), no cases of recurrence or metastatic disease were detected. CONCLUSIONS: Intraocular schwannoma is a rare benign tumour. It usually presents as nonpigmented mass, which can easily be misdiagnosed as nonpigmented uveal melanoma.

Meduloepitelioma de cuerpo ciliar. Reporte de un caso y revisión de la literatura / Ciliary body medulloepithelioma. Case report and literature review

Introducción: Se presenta el caso de un paciente de 10 años de edad a quien se le realizó el diagnóstico de meduloepitelioma de cuerpo ciliar con posterior enucleación. Objetivo: Presentar las principales características clínicas, abordaje diagnostico y terapéutico de un paciente con meduloepitelioma de cuerpo cilar, realizando una revisión de la literatura existente de esta patología. Diseño del estudio: Reporte de caso. Resumen del caso: Paciente masculino de 10 años de edad que presentó perdida de agudeza visual asociada a dolor ocular, al examen oftalmológico se encontró masa en cámara anterior. Se realizó biopsia de lesión iridiana diagnosticándose meduloepitelioma de cuerpo ciliar con posterior enucleación. Conclusión: Se presentó el caso de un niño con meduloepitelioma de cuerpo ciliar, exponiéndose las manifestaciones clínicas, su diagnóstico y tratamiento, así como la revisión de la literatura existente.

Background: We present the case of a 10-year-old patient who underwent a diagnosis of ciliary body medulloepithelioma with subsequent enucleation. Objective: To present the main clinical characteristics, diagnostic and therapeutic aproach of a patient with a ciliary body medulloepithelioma, carrying out a review of the existing literature of this pathology. Study design: Case report. Case summary: A 10-year-old male patient presented with loss of visual acuity associated with ocular pain. Ophthalmological examination revealed a mass in the anterior chamber. A biopsy of an iris lesion was made, diagnosing a ciliary body medulloepithelioma with subsequent enucleation. Conclusion: The case of a child with ciliary body medulloepithelioma was presented, exposing the clinical manifestations, their diagnosis and treatment, as well as the review of the existing literature.

Ciliary body malignant melanoma: A dilemma on staging / Melanoma maligno do corpo ciliar: um dilema no faseamento

Abstract Choroidal melanomas are the most common primary intraocular malignant tumor in adults. They tend to be more malignant; because of their location hidden behind the iris they can not be detected until they become larger. Therapeutic strategy is related by size, extension, number and location of tumor and growth patterns. High frequency ultrasound biomicroscopy (UBM) gives high resolution, cross-sectional images of the anterior segment lesions. Postequatorial lesions and intracranial extension of the melanomas are scanned by magnetic resonance imaging (MRI). We report a case of bilobed tumor with confusing appereance in preoperative imaging studies and macroscopy following enucleation. MRI is the perfect imaging method to reveal extension and size of the tumor in the posterior chamber. Combined use of UBM and MRI provides appropriate staging of ocular melanomas.

Resumo Melanomas coroidais são os tumores malignos intra-oculares primários mais comuns em adultos. Eles tendem a ser mais malignos; devido à sua localização ser escondida por detrás da íris eles não podem ser detectados até se tornarem maiores. A estratégia terapêutica está relacionada com tamanho, extensão, número e localização dos padrões tumorais e de crescimento. O biomicroscopio ultra-sónico de alta frequência (BMU) fornece imagens transversais de alta resolução das lesões do segmento anterior. Lesões pós-equatoriais e de extensão intracraniana dos melanomas são digitalizadas em ressonância magnética (RM). Relatamos um caso de tumor com dois lóbulos, com aparência confusa em exames de imagem pré-operatórios e macroscopia após enucleação. A RM é o método de imagem perfeito para revelar a extensão e o tamanho do tumor na câmara posterior. O uso combinado de BMU e MRI fornece o faseamento apropriado dos melanomas oculares.

Internal eye wall resection of uveal tumors: long-term evaluation / Ressecção em bloco de tumores uveais: avaliação de longo prazo

The authors report five cases of uveal tumors managed with internal eye wall resection and evaluate the long-term results. Patient age varied from 31 to 61 years. Four patients were female and one was male. Uveal tumors involved ciliary body and choroid in three patients and choroid alone in the other two. Histopathological examination demonstrated three uveal melanomas, one leiomyoma, and one meduloepithelioma. The largest tumor diameter ranged from 8 to 15 mm and tumor thickness from 5 to 11 mm. Follow-up of the patients was over a period of 6 to 23 years. Final visual acuity was 20/20 in 3 patients and 20/200 in one patient, and in the fifth patient, the affected eye was enucleated. No cases of metastatic disease or local recurrence of the tumor were observed, suggesting that internal eye wall resection is a valid alternative for the treatment of selected cases of uveal tumors.

Os autores relatam 5 casos que apresentavam tumores uveais e foram submetidos à remoção cirúrgica em bloco do tumor, avaliando resultados de acuidade visual, índice de retenção do globo ocular e de doença metastática. Quatro mulheres e um homem, com idade entre 31 e 61anos foram submetidos ao procedimento. O tumor envolvia corpo ciliar e coroide em três olhos e somente a coroide em dois. A histopatologia demonstrou que 3 tumores eram melanomas, um leiomioma e um meduloepitelioma. O maior diâmetro dos tumores foi entre 8 e 15 mm e a espessura entre 5 e 11 mm. O tempo de seguimento dos pacientes foi de 6 a 23 anos. A acuidade visual final foi de 20/20 em 3 olhos, 20/200 em outro e um olho foi enucleado. Não foram observados casos de doença metastática ou recorrência tumoral, sugerindo que a ressecção em bloco de tumores uveais é uma alternativa válida para o tratamento destes tumores em casos selecionados.

¿Melanoma o glaucoma? A propósito de un caso / Melanoma or glaucoma? A case report

CASO CLÍNICO: Presentamos a un paciente remitido con sospecha de melanoma de cuerpo ciliar debido a una lesión pigmentada en la raíz del iris del ojo izquierdo, asociada con presión intraocular alta a pesar de tratamiento máximo con medicación tópica y sistémica. El estudio sistemático reveló cambios unilaterales en el endotelio corneal, compatibles con el síndrome iridocorneal. Se implantó una válvula de Ahmed(R), logrando un control sostenido de la presión intraocular y de los defectos del campo visual. Discusión: Los síndromes iridocorneales son un amplio y heterogéneo grupo de enfermedades donde las células endoteliales crecen sobre el trabéculo, lo que lleva a un aumento en la presión intraocular. Debido a la naturaleza de la enfermedad, la cirugía de implante valvular puede ser la mejor opción terapéutica de entrada. El estudio clínico sistemático es crucial, ya que el diagnóstico diferencial puede incluir entidades potencialmente cegadoras e incluso mortales

CASE REPORT: The case is presented of a patient referred to us with suspicion of a ciliary body melanoma due to a pigmented lesion in iris root of left eye, associated with high intraocular pressure, despite maximal topical and systemic medication. The systematic workup revealed unilateral changes in the corneal endothelium, compatible with an iridocorneal syndrome. An Ahmed(R) valve was inserted, achieving sustained control of intraocular pressure and visual field defects. DISCUSSION: Iridocorneal syndromes are a wide and heterogeneous group of diseases, in which endothelial cells grow over the trabeculum, leading to an increase in intraocular pressure. Due to the nature of the disease, tube shunt surgery may be the best option in its treatment. Systematic workup is crucial, since the differential diagnosis may include potentially blinding and even life threatening conditions

Melanoma de corpo ciliar e coróide: relato de caso / Choroidal and ciliary body melanoma: case report

Melanomas oculares correspondem a 5 por cento de todos os melanomas e 85 por cento deles têm origem no trato uveal. Melanoma uveal é o tumor maligno intraocular primário mais comum no adulto. Relatamos neste artigo um caso de melanoma uveal em paciente, sexo feminino, 31 anos, com quadro de fotopsia, hiperemia e baixa da acuidade visual no olho esquerdo com evolução de quatro meses. Apresentava ao exame oftalmológico acuidade visual menor que 20/400, grande massa tumoral na região nasal retroiriana, com deslocamento anterior do cristalino, estreitamento da câmara anterior e descolamento seroso da retina. A ecografia sugeriu tratar-se de grande massa tumoral suspeita de melanoma de coróide com invasão do corpo ciliar. A confirmação diagnóstica foi possível por meio do exame anatomopatológico.

Ocular melanomas correspond to 5 percent of all melanomas and 85 percent of them have its origin in the uveal tract. Uveal melanoma is the most commom primary intraocular malignant tumor in the adult. In this article, a case of uveal melanoma in a 31 year-old female patient, with photopsia, hyperemia and low visual acuity in the left eye with evolution of 4 months is presented. In the ophthalmologic examination, visual acuity was lower than 20/400, a large tumoral mass was noted at the nasal region behind the iris with anterior lens displacement, anterior chamber narrowing and serous retinal detachment. The ocular echography suggested a large tumoral mass as a choroidal melanoma extending to the ciliary body. The confirmation diagnosis was possible through the histopathologic examination.

Macroadenoma del epitelio no pigmentado del cuerpo ciliar / Macroadenoma of the non-pigmented ciliary epithelium

CASO CLÍNICO: Se describen los hallazgos clínicos y la cirugía conservadora de un paciente con un adenoma no pigmentado del cuerpo ciliar. El adenoma presentaba un tamaño de 5 × 7 mm. El paciente fue intervenido con una cirugía conservadora mediante iridociclectomía parcial asociada a esclerouvectomía lamelar. DISCUSIÓN: Los adenomas del cuerpo ciliar clínicamente pueden imitar a los melanomas amelanóticos. La cirugía conservadora del globo ocular permite realizar un diagnóstico y un tratamiento del paciente manteniendo la función visual

CASE REPORT: We report the clinical features and surgery of a patient with an adenoma of the non-pigmented ciliary epithelium. The adenoma measured 5 × 7 mm. The patient underwent radical ocular surgery consisting of partial iridocyclectomy associated to lamellar sclerouvectomy. DISCUSSION: Adenomas of ciliary body can mimic clinically amelanotic melanomas. We present details of the patient's medical records and review the literature. Clinically, adenoma in ciliary body can mimic amelanotic melanomas. Conservative surgery of the eye allows diagnosis and treatment, maintaining visual function

Tumor pigmentado del cuerpo ciliar: ¿benigno o maligno? / Pigmented ciliary body tumours: benign or malignant?

Casos clínicos: Se presentan dos casos de mujeres con una tumoración pigmentada en cuerpo ciliar, un melanoma y un melanocitoma, con diferentes manifestaciones clínicas. La primera refiere disminución de agudeza visual asociada a la tumoración de reciente aparición, así como subluxación y opacificación sectorial cristaliniana. La segunda está asintomática y ha permanecido en observación más de 30 años. Discusión: Aunque al diagnóstico definitivo de un tumor pigmentado del cuerpo ciliar solo se llega a través del estudio anatomopatológico, el conjunto de características clínicas es determinante cuando se plantea un tratamiento conservador (AU)

Cases report: We report the cases of 2 women with a pigmented tumour in the ciliary body, one a melanocytoma and the other a melanoma, with different clinical manifestations. The first one presented with decreased visual acuity associated with recent growth of the tumour, as well as sectorial opacities of the lens and subluxation. The second one is asymptomatic and has been kept under observation for more than 30 years. Discussion: Although the definitive diagnosis of a pigmented tumour of the ciliary body is only achieved by the histopathology study, the group of clinical features is a determining factor when a conservative treatment is indicated (AU)