Analysis of clinical and pathological features of ciliary body medulloepithelioma

AIM:To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma.METHODS:The clinical and pathological data of 11 patients(11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital,Capital Medical University,from 2007 to 2021 were retrospectively analyzed.RESULTS:The initial symptoms of 11 patients included vision loss(6 eyes),atrophia bulbi(1 eye),proptosis(2 eyes),and leukocoria(2 eyes).Most patients suffered with corneal opacity,anterior chamber flare and hyphema.Iris neovascularization and synechia,complicated cataract,and secondar y glaucoma occurred in several cases.Three patients even had lens subluxation and retinal detachment.B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball.Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces.All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence.In this study,6 patients had enucleation(2 patients had systemic chemotherapy after surgery),and the other 5 patients had local tumor resection(1 patient had plaque radiotherapy after surgery).CONCLUSION:Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma.Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma,and can make an accurate diagnosis and better treatment.

Ultrasound biomicroscopic imaging demonstrate thinner ciliary body thickness in eyes with angle closure

·AIM:To compare the ciliary body thickness between eyes with primary angle closure(PAC)and primary angle-closure glaucoma(PACG)with the normal eyes,and to investigate the association between ciliary body thickness and ciliary processes situation.·METHODS:In this cross-sectional study,57 patients with PAC/PACG were matched to 57 normal subjects after propensity score matching(PSM)adjusting for age and gender.All subjects underwent conventional ocular examinations and ultrasound biomicroscopy(UBM)examination,among which the patients with PAC/PACG performed the examinations one month after laser peripheral iridotomy(LPI).Quantitative parameters were measured,which included ciliary body thickness at the position of 1 mm posterior to the scleral spur(CBT1),trabecular-ciliary process distance(TCPD)and trabecularciliary process angle(TCA).·RESULTS:Eyes with PAC/PACG presented significantly thinner CBT1,shorter TCPD and smaller TCA(P<0.001)than the normal eyes,both in comparison of the means of four quadrants and in comparisons of each quadrant.After removing images with peripheral anterior synechia(PAS),the same results were also found in comparisons between the two groups.Significant correlations were found between TCPD(R~2=0.537,P<0.001)and TCA(R~2=0.517,P<0.001)with CBT1.·CONCLUSION:Eyes with PAC/PACG have thinner ciliary body thickness and more anteriorly situated ciliary processes.Thinner ciliary body thickness is associated with anterior situation of the ciliary processes.

Extracting intraocular foreign body at or near the ciliary body with scleral indentation in direct visualization

Dear Editor,I＇m Dr. Qing-Huai Liu from Department of Ophthalmology, the First Affiliated Hospital of Nanjing Medical University,Nanjing, China. I write to present four cases diagnosed with the intraocular foreign body （IOFB） at or near the ciliary body and to evaluate the effect of extracting IOFB with scleral indentation in direct visualization.

Ciliary body detachment after secondary intraocular lens implantation in childhood

Dear Sir,Iam Dr.Li-Na Meng,from the Qingdao Eye Hospital,Shandong Eye Institute,Shandong Academy of Medical Sciences,Qingdao,China.I want to present a rare case of secondary intraocular lens(IOL)implantation in an11-year-old boy who developed the complication of ciliary body detachment.When he was 2 years old,the boy underwent bilateral cataract extraction for congenital cataract in the other

Choroidal and ciliary body tubercle: a case report

Dear Editor,I am Bing Li from the Department of Ophthalmology,Peking Union Medical College Hospital in Beijing, China,and I write to present a case report of choroidal and ciliary body tubercles.Ocular tuberculosis (TB) is an extrapulmonary tuberculous condition with variable manifestations[1].

Local excision of ciliary body tumors： a long-term prospective cohort study in China

Background Ciliary body tumors are usually difficult to diagnose and treat in early stages. However, treatment of such tumors has trended toward ocular conservation instead of enucleation. Local excision of ciliary body tumors has become effective with the development of the modern vitrectomy, but long-term outcomes are still not clear. Therefore, we reported the outcome of locally excised ciliary body tumors after long-term follow-up. Methods Twenty-two patients （22 eyes）, who had been diagnosed with ciliary body tumors in Beijing Tongren Hospital from January 1996 to June 2001, were enrolled in this prospective cohort study. Localized lamellar sclera-ciliary excisions were performed. In some cases, vitrectomies, scleral graft transplantations, and further excisions of the anterior choroid were performed. Diagnoses were confirmed by histopathologic examination. Patients were followed from five to eleven years after surgery. Their visual acuity, intraocular pressure and local recurrence were recorded with descriptive percentages. Results After surgery, all patients maintained normal eyeball appearances. Six patients maintained circular pupils. The final best corrected visual acuities （BCVA） varied from 0.02-1.00, including 18 patients （81.82%） who had BCVA equal to or better than that before surgery. Fifteen patients （68.18%） had BCVA better than 0.3. Only two patients had intraocular pressure （lOP） of less than 10 mmHg and the other patients maintained normal lOP. Nine cases （40.91%） were given confirmed diagnosis of malignant melanoma, four （18.18%） of melanocytoma, six （27.27%） of nonpigmented ciliary epithelial adenoma, two （9.09%） of neurofibroma, and one （4.55%） of neurinoma. Twenty patients （90.91%） had no recurrence during the follow-up period. In one case melanocytoma recurred seven years after surgery and enucleation was performed. One patient, whose operation removed a malignant melanoma with a diameter of 16 mm, died of hepatic metastasis five years after the operation. Conclusion For some ciliary body tumors, especially in the early stages, local excision is an effective and safe method to save the vision and the eyeball.

Roles of paroxetine and corticosterone on adult mammalian ciliary body cell proliferation

Background The neurogenesis in retina of adult mammals is generally abolished, and this renders the retina lack of regenerative capacity. Despite this, there is a small population of nestin-positive cells in the ciliary epithelium which retains neurogenic potential. The present study aimed at investigating the effect of two drugs, corticosterone and paroxetine, on the cell proliferation of the ciliary body. Methods Adult Sprague-Dawley rats were given vehicle, corticosterone, paroxetine, or both corticosterone and paroxetine treatment for 14 days. Cell proliferation in the ciliary body was quantified using 5-bromo-2-deoxyuridine （BrdU） immunohistochemistry. Co-labelling of BrdU and stem cell marker was used to phenotype the BrdU immunoreactive cells. Results Corticosterone treatment suppressed while paroxetine treatment increased the cell proliferation of the ciliary body. Co-labelling with cell markers revealed that the BrdU positive cells also showed nestin expression but not glial fJbrillary acidic protein （GFAP）. Conclusions The results illustrate that proliferation of retinal progenitor cells situated in ciliary body are subjected to regulation by selective serotonin reuptake Jnhibitors （SSRI） and corticosteroJd, which is similar to our previous findings in neurogenic regions in central nervous system （CNS）. Paroxetine treatment could reverse the suppressive effect of corticosterone on ciliary body cell proliferation. This provides information for future investigation of retinal stem cell biology and potential treatment of retinal degenerative diseases.

Expression of nitric oxide synthase and guanylate cyclase in the human ciliary body and trabecular meshwork

Background The role played by the nitric oxide （NO） signaling pathway in the aqueous humor dynamics is still unclear.This study was designed to investigate the expression and distribution of NO synthase （NOS） isoforms and guanylate cyclase （GC） in human ciliary body,trabecular meshwork and the Schlemm＇s canal.Methods Twelve eyes after corneal transplantation were used.Expression of three NOS isoforms （i.e.neuronal NOS （nNOS）,inducible NOS （iNOS） and endothelial NOS （eNOS）） and GC were assessed in 10 eyes by immunohistochemical staining using monoclonal or polyclonal antibody of NOS and GC.Ciliary bodies were dissected free and the total proteins were extracted.Western blotting was performed to confirm the protein expression of 3 NOS isoforms and GC.Results Expression of 3 NOS isoforms and GC were observed in the ciliary epithelium,ciliary muscle,trabecular meshwork and the endothelium of the Schlemm＇s canal.Immunoreactivity of nNOS was detected mainly along the apical cytoplasmic junction of the non-pigmented epithelium （NPE） and pigmented epithelial （PE） cells.Protein expressions of 3 NOS isoforms and GC were confirmed in isolated human ciliary body by Western blotting.Conclusions The expression of NOS isoforms and GC in human ciliary body suggest the possible involvement of NO and cyclic guanosine monophosphate （cyclic GMP,cGMP） signaling pathway in the ciliary body,and may play a role in both processes of aqueous humor formation and drainage.

A Case of Primary Central Nervous System Lymphoma with Ciliary Body Involvement

INTRODUCTION Primary central nervous system lymphoma （PCNSL） is defined as lymphoma confined to the brain, eye, spinal cord, or leptomeninges. It constitutes approximately 3% of all brain tumors, and 2-3% of all cases of non-Hodgkin lymphoma. Ocular involvement can be detected during clinical staging in about 20% of patients with PCNSL, with primary vitreous retinal lymphoma being the most common subtype. Uveal involvement of PCNSL is uncommon, and PCNSL with ciliary body involvement （CBL）, to the best of our knowledge, has not been reported.

Clinical prediction of insufficient vaults after implantable collamer lens implantation

AIM:To determine the factors related to preoperative ocular characters that are predictive of insufficient vault(<250μm)after implantable collamer lens(ICL V4c;STAAR Surgical)implantation.METHODS:The participants underwent ICL surgery and were divided into the low(<250μm)and normal(250-1000μm)vault groups based on the postoperative vault at 3mo.The preoperative biometric parameters and clinical outcomes were compared between the two groups.The relationship between the 3-month vault values and preoperative ocular parameters were evaluated by Generalized estimating equations.RESULTS:Sixteen(23 eyes)and 36 patients(63 eyes)were in the low and normal vault groups,respectively.All implantation procedures were uneventful with no cataract formation in the early postoperative period.The sulcusto-sulcus lens rise(STSL)and iris ciliary angle(ICA)were correlated with vault at 3mo after surgery.Every 0.1 mm increase in STSL was associated with 38.9μm decrease in the postoperative 3-month vault.A rise of 1 degree in ICA is associated with a reduction of 4μm in vault.CONCLUSION:Eyes with a narrow ciliary sulcus are associated with a higher rate of low vault after ICL implantation,suggesting a need for adjustments to the ICL size in these patients.Evaluating the characteristics of the ciliary sulcus contributes valuable information to predict low vault after surgery.

Ocular manifestations of central insulin resistance

Central insulin resistance, the diminished cellular sensitivity to insulin in the brain, has been implicated in diabetes mellitus, Alzheimer’s disease and other neurological disorders. However, whether and how central insulin resistance plays a role in the eye remains unclear. Here, we performed intracerebroventricular injection of S961, a potent and specific blocker of insulin receptor in adult Wistar rats to test if central insulin resistance leads to pathological changes in ocular structures. 80 mg of S961 was stereotaxically injected into the lateral ventricle of the experimental group twice at 7 days apart, whereas buffer solution was injected to the sham control group. Blood samples, intraocular pressure, trabecular meshwork morphology, ciliary body markers, retinal and optic nerve integrity, and whole genome expression patterns were then evaluated. While neither blood glucose nor serum insulin level was significantly altered in the experimental or control group, we found that injection of S961 but not buffer solution significantly increased intraocular pressure at 14 and 24 days after first injection, along with reduced porosity and aquaporin 4 expression in the trabecular meshwork, and increased tumor necrosis factor α and aquaporin 4 expression in the ciliary body. In the retina, cell density and insulin receptor expression decreased in the retinal ganglion cell layer upon S961 injection. Fundus photography revealed peripapillary atrophy with vascular dysregulation in the experimental group. These retinal changes were accompanied by upregulation of pro-inflammatory and pro-apoptotic genes, downregulation of anti-inflammatory, anti-apoptotic, and neurotrophic genes, as well as dysregulation of genes involved in insulin signaling. Optic nerve histology indicated microglial activation and changes in the expression of glial fibrillary acidic protein, tumor necrosis factor α, and aquaporin 4. Molecular pathway architecture of the retina revealed the three most significant pathways involved being inflammation/cell stress, insulin signaling, and extracellular matrix regulation relevant to neurodegeneration. There was also a multimodal crosstalk between insulin signaling derangement and inflammation-related genes. Taken together, our results indicate that blocking insulin receptor signaling in the central nervous system can lead to trabecular meshwork and ciliary body dysfunction, intraocular pressure elevation, as well as inflammation, glial activation, and apoptosis in the retina and optic nerve. Given that central insulin resistance my lead to neurodegenerative phenotype in the visual system, targeting insulin signaling may hold promise for vision disorders involving the retina and optic nerve.

Osteopontin is a biomarker for early autoimmune uveoretinitis

Osteopontin(OPN)is an extracellular matrix protein with a diverse range of functions,including roles in cell adhesion,migration,and immunomodulation,which are associated with the modulation of neuroinflammation in the central nervous system.The present study was performed to evaluate the involvement of OPN in the eyes of an experimental autoimmune uveoretinitis(EAU)model.The EAU model was developed by immunization of Lewis rats with interphotoreceptor retinoid-binding protein.The results showed the OPN level was remarkably upregulated in the eye of EAU rats on day 9 post-immunization.The level of CD44,a ligand of OPN,was increased in the ciliary body of EAU rats.Furthermore,OPN was also detected in the ciliary body and activated microglia/macrophages in the EAU retina.The results suggest that OPN was significantly upregulated in the eyes of EAU rats,and that it may be useful as an early biomarker of ocular autoimmune diseases.All animal experiments were approved by the Institutional Animal Care and Use Committee of Jeju National University(approval No.2020-0012)on March 11,2020.

Evaluation of the Changes in Anterior Segment Morphology with Ultrasound Biomicroscopy after Vitrectomy without Use of Tamponade in Pseudophakic Eyes

Aim: In this study we investigated the changes in anterior segment morphology in pseudophakia patients that underwent ultrasound biomicroscopy (UBM) after pars plana vitrectomy (PPV) operation without use of tamponade. Method: Pseudophakic patients who undergo PPV were enrolled in this prospective study between October 2012 and April 2015. Study included patients in whom intraocular tamponade was not used during PPV operation. UBM measurements were performed both before and 10 days after the operation. Anterior chamber depth (ACD) was measured using axial images of anterior segment. Trabecular meshwork-iris angle (TIA), ciliary body thickness (CBT), sclera thickness (ST), trabecular meshwork-ciliary process distance (T-CPD), iris-ciliary processes distance (I-CPD), and iris thickness (IT) were measured at temporal quadrant based on radial section images of the angle. Values measured before and after the operation were statistically compared with each other. Results: This study included 30 patients (18 females, 12 males) that underwent an operation ultrasound biomicroscopy (UBM) after pars plana vitrectomy (PPV) operation without use of tamponade. Mean age was 69.6 ± 9.1 (55 - 85) years. Eighteen operations occurred on the left eye whereas twelve operations occurred on the right eye. CBT1, CBT2, CBT Max, T-CPD, and I-CPD were significantly decreased after operation when compared with the values of baseline (before) (p = 0.018, p = 0.012, p = 0.001, p = 0.033, p = 0.015, respectively). Other evaluated parameters did not show statistically significant changes after the operation (p > 0.05). Discussion: PPV results in significant changes in ciliary body morphology together with changes in anterior segment parameters in pseudophakic cases.

Dilemma in management of ocular medulloepithelioma in a child

Medulloepithelioma,a rare tumor,arises from the epithelium of the medullary tube.In this article,we present a 3-year-old.boy who suffered from secondary glaucoma,initially presumed the primary disease was endophthalmitis.Subconjunctival mass was later found,pathologically proved to be medulloepithelioma.We discuss the patient management with emphasis on the early signs of examination and the role of ultrabiomicroscopy （UBM） in evaluating pediatric secondary glaucoma and in influencing the management of patients with medulloepithelioma.

Intraocular tumours imaging with transpalpebral near-infrared LED transillumination:Pilot study

Purpose:To determine the possible use of near-infrared(NIR)light-emitting-diode(LED)radiation for imaging intraocular tumours by transpalpebral transillumination.Methods:This study was a pilot,open-label,prospective and non-interventional.Thirty patients(30 eyes;age 30–72 years)with uveal melanomas located in the iris,ciliary body,or choroid were under our observation.A biomicroscopy,ophthalmoscopy,transpalpebral NIR transillumination,and ultrasound examination were performed in all cases.Results:In all cases,NIR transillumination with transpalpebral approach enables visualization of the ciliary body and accurately estimates the projection of the pars plicata and ora serrata onto the sclera.In all patients,transpalpebral NIR transillumination made it possible to image the shadow of intraocular melanoma,estimate its dimensions and location concerning the ciliary body structures.Conclusions:We recommend the non-invasive transpalpebral NIR transillumination technique,together with traditional ultrasound imaging,to improve the accuracy of assessing the size and location of intraocular tumours.