Pigment Epithelial Cysts Research Articles

Pseudo-plateau iris secondary to multiple peripheral pigment epithelial cysts: A case report

Iris epithelial cysts are rare, spontaneous, epithelium-lined spaces that arise from the iris-pigmented epithelial layer and ciliary body and can cause a narrowing of the anterior chamber angle leading to a pseudo-plateau iris configuration (PPI). We present the case of an 80-year-old female patient with a history of decreased visual acuity, diagnosed with bilateral cataracts and pseudo-plateau iris secondary to multiple peripheral pigment cysts. This report describes the detection process, management strategy and course of treatment of a patient with bilateral cataracts who underwent cataract surgery by manual incision, and clinically presented with Pseudo-plateau iris secondary to Iris epithelial cyst. It highlights the importance of early detection and correct differentiation from malignant conditions, and anterior segment pathologies to prevent associated permanent complications.

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

ABSTRACT 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.

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

BackgroundCiliary 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.MethodsFour 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.ResultsFour 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.ConclusionsLocal 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.

Kyste de l’épithélium pigmentaire irien luxé dans le vitré

Kyste de l’épithélium pigmentaire irien luxé dans le vitré

Partial lamellar sclerouvectomy surgery for anteriorly located uveal tumour resection: a 20-year experience.

To investigate the results of partial lamellar sclerouvectomy (PLSU) for anteriorly located uveal tumours. We reviewed the tumour features, histopathologic findings, complications, visual acuity outcomes, eye preservation, metastasis, and mortality data of 56 cases with uveal tumours who underwent PLSU between February 1999 and February 2019. The mean largest tumour base diameters were 5.8 × 3.4 mm and the mean tumour thickness was 3.3 mm. Histopathologically, 30 (53.6%) eyes had malignant melanoma, 13 (23.2%) had nevus, 5 (8.9%) had iris stromal cyst, 4 (7.1%) had melanocytoma, 2 (3.6%) had Fuchs' adenoma, 1 (1.8%) had iris pigment epithelial cyst, and 1 (1.8%) had invasive breast cancer metastasis. The most common postoperative complications included cataract in 21 (37.5%) eyes, vitreous haemorrhage in 15 (26.8%), scleral thinning in 10 (17.9%), and hyphema in 6 (10.7%). At a mean follow-up of 40.4 (range: 10-201) months, tumour recurrence was observed in 2/30 (6.7%) eyes with melanoma and 1/5 (20.0%) eye with iris stromal cyst. Eyes with recurrent melanoma were treated with enucleation. Liver metastasis developed in only 1 (3.3%) melanoma case. All patients were alive at the end of follow-up. PLSU is a successful treatment method for many anteriorly located uveal tumours. In our series, the overall tumour recurrence and globe salvage rates were 5.4% and 96.4% respectively. Among melanoma cases, the metastasis rate was 3.3% and survival rate was 100%.

Laser puncture of symptomatic primary iris pigment epithelial cyst causing hemeralopia: a rare case

Primary iris pigment epithelium cysts involve posterior surface of the iris and can occur at pupillary margin or anterior ciliary body. They may be stationary or progressive and sometimes regress spontaneously. These cyst can occasionally lead to angle-closure glaucoma, plateau iris syndrome and secondary pigment dispersion syndrome. A steadily growing cyst may disturb the vision by covering the visual axis and provoke an increase in intraocular pressure (IOP) or even inflammation if it touches the corneal endothelium. There are multiple management options in complicated situations. We report a case of a 17-year-old girl with unusual complaints of hemeralopia and dark-brown discolouration of pupils in both eyes. On examination, she had multiple pigment epithelial iris cysts at the pupillary margins, which were punctured with Nd:Yag laser, and the response was improvement in vision and hemeralopia without any pigment dispersion or IOP changes.

Iris Cysts: Clinical Features, Imaging Findings, and Treatment Results

Objectives:To report the clinical and demographic characteristics, imaging findings, treatment results, and follow-up data of patients with iris cysts.Materials and Methods:The medical records of 37 patients with iris cysts were retrospectively analyzed. Ultrasound biomicroscopy (UBM), swept-source optical coherence tomography (SS-OCT), and SS-OCT angiography (SS-OCTA) were performed to examine the iris cysts.Results:The mean age of the patients was 34.4 years, ranging from 5 to 85 years. Twenty-four patients (65%) were female and 13 (35%) were male. Mean follow-up period was 21.3 months, ranging from 4 months to 8 years. Thirty-five (94.5%) of the cysts were classified as primary and 2 (4.5%) were classified as secondary. Thirty-one (83.7%) of the primary cysts were pigment epithelial and 4 were stromal. Primary iris pigment epithelial cysts were classified as peripheral in 26 patients (72.2%), midzonal in 4 (11.1%), and dislodged in 1 (2.7%). Stromal cysts were classified as acquired in 3 patients (8.1%) and congenital in 1 patient (2.7%). Secondary iris cysts were caused by perforating eye injury. UBM could visualize both the anterior and posterior surfaces of the cysts (26 patients). Anterior segment SSOCT could visualize the anterior but not the posterior surface of the cysts (4 patients). Iris cysts did not display intrinsic vascularity on SS-OCTA (4 patients). All pigment epithelial cysts were managed by observation. Of the 4 primary stromal cysts, 3 were managed by surgical excision and 1 by observation. Two secondary cysts required surgical removal.Conclusion:Pigment epithelial cysts generally remain stable without need for treatment. However, iris stromal cysts frequently require surgical intervention. UBM and SS-OCT were valuable in the diagnosis of iris cysts. On UBM, iris cysts appear with a thin, hyperechoic wall with hypoechoic internal content. Iris cysts did not have intrinsic vascularity on anterior segment SS-OCTA.

Pediatric Free-floating Anterior Chamber Cyst of the Iris Pigment Epithelium

Iris pigment epithelial (IPE) cysts are a subset of iris cysts that arise from the IPE. They are spontaneously erupting epithelial-lined cavities that are found in various anatomic locations of the iris, including the iris pupillary margin, midzone, periphery, and free floating in the vitreous or anterior chamber. We report the case of an asymptomatic 13-year-old boy with an incidental finding of a dislodged anterior chamber cyst diagnosed on routine examination. Modern multimodal image analysis of the cyst including anterior segment optical coherence tomography and ultrasound biomicroscopy (UBM) was utilized to characterize the microstructural anatomy of the lesion. The patient was managed conservatively without complications. Cysts of the IPE typically do not affect vision or ocular health and can be monitored and observed after ascertaining no associated malignancy. Initial diagnostic investigation can include UBM and anterior segment optical coherence tomography. Intervention should be reserved only for cases where the cyst growth leads to obstruction of the visual axis and/or other secondary complications.

Optical Coherence Tomography Angiography Characteristics of Iris Melanocytic Tumors

To evaluate tumor vasculature with optical coherence tomography angiography (OCTA) in malignant iris melanomas and benign iris lesions. Cross-sectional observational clinical study. Patients with iris lesions and healthy volunteers. Eyes were imaged using OCTA systems operating at 1050- and 840-nm wavelengths. Three-dimensional OCTA scans were acquired. Iris melanoma patients treated with radiation therapy were imaged again after I-125 plaque brachytherapy at 6 and 18 months. OCT and OCTA images, qualitative evaluation of iris and tumor vasculature, and quantitative vessel density. One eye each of 8 normal volunteers and 9 patients with iris melanomas or benign iris lesions, including freckles, nevi, and an iris pigment epithelial (IPE) cyst, were imaged. The normal iris has radially oriented vessels within the stroma on OCTA. Penetration of flow signal in normal iris depended on iris color, with best penetration seen in light to moderately pigmented irides. Iris melanomas demonstrated tortuous and disorganized intratumoral vasculature. In 2 eyes with nevi there was no increased vascularity; in another, fine vascular loops were noted near an area of ectropion uveae. Iris freckles and the IPE cyst did not have intrinsic vascularity. The vessel density was significantly higher within iris melanomas (34.5%±9.8%, P < 0.05) than in benign iris nevi (8.0%±1.4%) or normal irides (8.0%±1.2%). Tumor regression after radiation therapy for melanomas was associated with decreased vessel density. OCTA at 1050 nm provided better visualization of tumor vasculature and penetration through thicker tumors than at 840 nm. But in very thick tumors and highly pigmented lesions even 1050-nm OCTA could not visualize their full thickness. Interpretable OCTA images were obtained in 82% of participants in whom imaging was attempted. This is the first demonstration of OCTA in iris tumors. OCTA may provide a dye-free, no-injection, cost-effective method for monitoring a variety of tumors, including iris melanocytic lesions, for growth and vascularity. This could be helpful in evaluating tumors for malignant transformation and response to treatment. Penetration of the OCT beam remains a limitation for highly pigmented tumors, as does the inability to image the entire iris in a single field.

Management of iris cysts in children

There are two main categories of iris cysts in children including iris pigment epithelial (IPE) custs and iris stromal cysts. Most pigment epithelial cysts are small and stable, rarely requiring treatment. Iris stromal cysts tend to enlarge and produce photophobia and secondary glaucoma, requiring treatment.

Evaluation of iris and iridociliary body lesions with anterior segment optical coherence tomography versus ultrasound B-scan

AimsTo compare anterior segment optical coherence tomography (AS-OCT) with ultrasound B-scan (USB) in evaluating iris and iridociliary body lesions.MethodsImage features and resolution comparison between AS-OCT and USB in 126 patients...

Kyste vitréen partiellement pigmenté

Free-floating iris pigment epithelial cysts in the vitreous are very rare. We report the case of a partially pigmented cyst found on routine examination in the vitreous of a 51-year-old asymptomatic patient. Usually, non-pigmented cysts are derived from the hyaloid system. Pigmented cysts, however, are derived from the iris pigment epithelium. When they are not dislodged, these cysts can be confused with iris melanoma or primary stromal cysts. Treatment is usually unnecessary, unless they become symptomatic.

Multicavitary ciliary body melanoma presenting as a cyst

Cyst-like cavities in uveal melanoma occur rarely and can simulate a benign intraocular cystic lesion resulting in delayed diagnosis and inappropriate management. Herein, we describe a 66-year-old Caucasian female who presented with a “cystic” ciliary body mass in the right eye oculus dexter (OD). Slit lamp examination OD showed anterior bulging of the iris temporally from an underlying pigmented ciliary body mass and transillumination disclosed slight shadow from the tumor. Ultrasound biomicroscopy (UBM) revealed multiple cyst-like cavities within a tumor, lined by “thick walls” of at least 200 μm and occupying 80% of the tumor volume. A clinical diagnosis of multi-cavitary ciliary body melanoma was suspected and partial lamellar sclero iridocyclectomy was performed. Histopathology confirmed the diagnosis of low-grade spindle melanoma of the ciliary body with multiple empty and fluid filled cyst-like cavities without epithelial lining. UBM is an important diagnostic tool in the differentiation of “thick walled” cavitary melanoma from “thin walled” benign pigment epithelial cyst.

Bilateral primary iris pigment epithelial cysts

AbstractPurpose To report a patient who presented with bilateral primary iris pigment epithelial cysts.Methods Interventional case report.Results A 30‐year‐old lady with the diagnosis of bilateral primary cysts of the iris pigment epithelium was referred. Her father had been diagnosed with a uveal melanoma 13 years earlier. Two smaller circumferential midzonal iris pigment epithelial cysts and a larger inferonasal cyst located in the temporal area were observed in her right eye, and a similar but much larger cyst was observed in her left eye temporally between 1 o'clock and 6 o'clock meridians. Otherwise, the anterior and posterior segment as well as the intraocular pressure (16 mmHg) were normal. The visual acuity was 20/20 without correction, right eye, and 20/20 with a +0.75 sphere, left eye, with a shadow in the field. For the decrease of vision in her left eye the cyst was treated with neodymium‐doped yttrium aluminum garnet (Nd:YAG) laser cystotomy without any ocular tissue damage using the Abraham iridectomy lens and a 1.0 mJ single pulse. Cystotomy resulted in immediate collapse of the cyst with no change in intraocular pressure.Conclusion Laser cystotomy yielded a favorable result. Generally, these cysts which most frequently occur in young females do not need any treatment, but transpupillary laser cystotomy is an alternative to a conservative regimen if iris the cyst is symptomatic.

Combined laser treatment in a patient with pigment dispersion secondary to a large iris pigment epithelial cyst.

We reported a case of bilateral extensive iris pigment epithelial cysts masquerading as pigment dispersion. A-30-year-old male patient presented with a dull pain in both eyes and a decreased visual acuity OD. He underwent a complete ophthalmic examination. OD was injected and the cells were graded as +3 and pigmented a +2, in the OS. Intraocular pressures (IOP) were measured as 42 (OD) and 22 (OS) mmHg. Gonioscopy revealed a confluent accumulation of dense pigment in both eyes. Visual fields, peripapillary retinal nerve fiber layer thickness (Spectral OCT/SLO OTI-OPKO Health. Inc, Miami, FL) and optic nerve head tomography (HRT-II Heidelberg Engineering, Heidelberg, Germany) results were within normal limits. On ultrasound biomicroscopy (UBM), bilateral extensive cysts were identified in the midzonal portion of the iris and in the ciliary body. An, antiglaucomatous treatment was started. Then, we decided to perform both Nd:YAG laser iridocystotomyc and selective laser trabeculoplasty. Fourteen months after the combined therapy, the cysts had not recurred, and still apposed and the IOPs were under control without medication.

Transpupillary Nd:YAG Laser Cystotomy for Iris Pigment Epithelial Cysts with Secondary Progressive Angle Closure

Two patients were diagnosed as having unilateral iris pigment epithelial (IPE) cysts that were documented to enlarge and induce angle closure. Transpupillary IPE cystotomies were performed using the Nd:YAG laser as a prophylactic measure to prevent angle-closure glaucoma. Anterior chamber anatomy and cyst dimensions were assessed before, during, and after long-term follow-up using slit-lamp biomicroscopy, gonioscopy, and 20- or 35-MHz high-frequency ultrasound imaging. Cystotomy resulted in immediate, visible deflation of both IPE cysts with subsequent resolution of angle closure. The cyst contents appeared clear and no secondary inflammation or glaucoma occurred. No evidence of cyst recurrence has been noted during 3 and 8 years of follow-up, respectively. Transpupillary Nd:YAG laser cystotomy offered a minimally invasive and effective treatment for angle closure induced by progressive IPE cysts.

Surgical treatment and histopathology of a symptomatic free-floating primary pigment epithelial iris cyst in the anterior vitreous

We report the surgical removal of an iris pigment epithelial cyst that was floating freely in the posterior chamber of an 18-month-old child. The reason for surgical removal was disturbance in near vision secondary to the movement of the cyst across the visual axis. Visual disturbance secondary to a unilateral anterior vitreous iris cyst at this age is a rare presentation and, to the best of our knowledge, has not been reported previously as an indication for surgery. We performed histopathological study of the cyst aspirate to determine its contents and its possible origin.

Spontaneous Deflation of an Iris Pigment Epithelial Cyst Documented with AS-OCT

A 3-year-old girl presented with a black pupillary margin iris mass in the right eye diagnosed as an iris pigment epithelial (IPE) cyst, central (pupillary) type. The diagnosis was verified by anterior-segment optical coherence tomography (AS-OCT), which initially showed a circumscribed, round thin-walled cyst with clear lumen attached by a strand to the IPE. On follow-up 3 months later, the IPE cyst had deflated and AS-OCT showed an irregular collapsed surface with no lumen and optical shadowing. The authors report a case in which AS-OCT was used to provide information regarding the diagnosis and behavior of an IPE cyst.

Primäre stromale Iriszysten

Cysts of the iris are uncommon and most of them occur secondarily after surgery or penetrating injury. The minority of the iris cysts is primary without a reasonable cause. They are classified into the more common pigment epithelial cysts and the rare cysts of iris stroma ("intrastromal cysts"). These intrastromal iris cysts are generally diagnosed in children and often cause symptoms such as a decrease of visual acuity because of ingrowth into the optical axis. A diagnosis of stromal cysts in adults is very rare. Most of these patients remain without any symptoms and do not need treatment. The cellular origin is so far unknown. Mesoderm, neuroectoderm and surface ectoderm have been discussed in this context. Two patients with primary intrastromal iris cysts are presented, a 5-year-old boy and a 65-year-old woman. In both cases, the cyst affected the optical axis and was removed by sector iridectomy. In histological and electron-microscopic examinations both cysts presented a typical epithelial structure. Immunohistochemical examination revealed positivity for epithelial markers and negativity for mesenchymal and neuroectodermal markers. Primary intrastromal iris cysts can occur in advanced age and may cause symptoms due to progressive growth. The cellular origin of primary intrastromal iris cysts is controversially discussed in the literature. On electron microscopy and immunohistochemistry, the iris cysts presented here showed characteristic features of surface ectodermal origin.

Ipsilateral ciliary body schwannoma and ciliary body melanoma in a child

An 11-year-old boy presented with a mid-zonal pigment epithelial iris cyst. The cyst progressed relentlessly, ultimately leading to enucleation of the eye 4 years after presentation. Histopathology revealed a ciliary body schwannoma filling the anterior chamber and a small, incidental noncontiguous ciliary body melanoma. Vision loss, pupil distortion, and relentless growth despite complete cyst deflation following aspiration were clues for the tumor-induced nature of the visible cyst.