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 medulloepithelioma clinical features and management of 11 cases.

AIMS: To describe the clinical features, imaging characteristics, histopathology, treatment and outcomes of intraocular medulloepithelioma. METHODS: Medical records of 11 patients with clinically or histopathologically confirmed medulloepithelioma were retrieved and reviewed. Clinical features, diagnostic challenges, imaging characteristics, management, histopathology and prognosis were assessed. RESULTS: The median age of the patients at initial diagnosis was 4 years, with the most common manifestations being leukocoria (five eyes), loss of vision (four eyes), ocular pain (one eye) and ophthalmic screening (one eye). The clinical signs include a grey-white ciliary body lesion, cataract or lens subluxation, secondary glaucoma and evident cysts. The ultrasound biomicroscopy (UBM) imaging most commonly displays ciliary body mass with intratumoural cysts (nine eyes). Three patients underwent surgery for cataract or glaucoma while the tumours were incidentally found. Two of the three patients managed by eye preserve treatments eventually required enucleation because of local tumour recurrence or phthisis. One patient treated with intra-arterial chemotherapy and cryotherapy had successful tumour regression and globe salvage. CONCLUSIONS: Initial misdiagnosis, delay in diagnosis and subsequent misdirected management is not uncommon in medulloepithelioma. The presence of multiple cysts in the tumour and retrolental neoplastic cyclitic membrane detected by UBM can offer certain information. Selective intra-arterial melphalan may prevent further tumour growth, but longer follow-up is necessary until treatment efficacy is fully evaluated.

Peering into the eye: A comprehensive look at ultrasound biomicroscopy (UBM) and its diagnostic value in anterior segment disorders.

Background: Ultrasound biomicroscopy (UBM) is a noninvasive imaging modality that enables in-vivo visualization of the structures of the anterior segment of the eye. Unlike routine ophthalmic diagnostic ultrasound which uses frequencies of 5-10 MHz, UBM utilizes ultrasound frequencies in the range of 50-100 MHz. The high-frequency probes in UBM allows for higher resolution and better visualization of subsurface ocular structures, even in the presence of anatomic or pathological obscuration. UBM has qualitative as well as quantitative applications in various disorders affecting the anterior segment of the eye. Despite its huge importance, many clinicians lack in knowledge about the technique and its clinical usefulness. The current educational video aims to address this gap in knowledge by highlighting the technique and various clinical indications of UBM. Purpose: The purpose of this video is to demonstrate the technique of UBM and showcase its quantitative and qualitative implications and importance through various clinical cases. Synopsis: UBM is an imaging technique that assesses the depth of tissue structures by measuring the time delay of the returning ultrasound signal. This modality is capable of measuring the size of various structures within the eye, such as the cornea, iris, ciliary body, sclera, and the depth of the anterior and posterior chamber. To perform a UBM, a transducer is inserted into a specially designed eye cup filled with distilled water, creating a water bath environment. Axial and longitudinal scans can be performed in a similar fashion as in routine diagnostic B-scan ultrasound. Quantitative indications for UBM depicted in this video include measurements of corneal thickness, depth of the anterior chamber, and the width of the angle. The video also showcases how UBM can aid in the diagnosis and management of various anterior segment disorders like angle-closure glaucoma, plateau iris configuration, secondary glaucoma, and anterior uveitis with complicated cataract. Qualitative indications for UBM highlighted in this video include its role in intermediate uveitis, ocular hypotony, ocular surface tumors, cystic lesions of iris, and identifying the location and type of intraocular foreign bodies in the anterior segment based on the type of artifact seen. Additionally, the video shows the applications of UBM in scleral and episcleral pathologies. Highlights: This video will educate clinicians about the technique of UBM and showcase a bouquet of UBM findings in various case scenarios, helping one to better understand the potential of this modality in clinical practice. Video link: https://youtu.be/F626TMbJXoU.

Evaluation of ciliary body morphology and position of the implantable collamer lens in low-vault eyes using ultrasound biomicroscopy.

PURPOSE: To investigate the ciliary body anatomy and position of the implantable collamer lens (ICL) in low-vault eyes and analyze factors related to insufficient vault. SETTING: Zhongshan Ophthalmic Center, Guangzhou, China. DESIGN: Retrospective case-control observational study. METHODS: In this study, 73 eyes of 73 patients with an insufficient vault (<250 µm) were matched with 73 eyes with an ideal vault (250 to 750 µm). Ultrasound biomicroscopy was used to determine the ciliary body morphology and ICL position. The biometric parameters acquired by Scheimpflug tomography were compared. The correlation between the vault and these factors was analyzed, and the least absolute shrinkage and selection operator method was used to screen the risk factors for low vault. RESULTS: The low-vault group had a steeper corneal curvature, thicker lens thickness (LT), higher crystalline lens rise, and shorter axial length (AL) (all P < .005). The ciliary process length (CPL) and maximum ciliary body thickness (CBTmax) were significantly smaller, and the trabecular-ciliary angle (TCA), iris-ciliary angle (ICA), and ciliary sulcus width (CSW) were significantly greater in the low-vault eyes (all P < .005). The low-vault group had more ICL haptics below the ciliary process, and TCA, ICA, CPL, CBTmax, CSW, and haptic position were related to the postoperative vault (all P < .05). CPL, AL, and LT were identified as predictors of a low vault. CONCLUSIONS: Malposition of ICL haptics behind the ciliary process is a risk factor for low vault. A shorter CPL, thicker LT, and shorter AL are significant risk factors for the postoperative low vault.

Qualitative ultrasound biomicroscopy in glaucoma.

Background: UBM is a high-resolution ultrasound technique which allows non-invasive, in vivo imaging of the anterior segment and iridocorneal angle. Purpose: This video is compilation of short video clips and images which gives description on identification of angle closure due to pupillary block, peripheral anterior synechiae, iris bombe, plateau iris, supra ciliary effusion, and malignant glaucoma. It also shows video demonstrating partially and fully patent iridotomy and features of trabeculectomy bleb. Synopsis: This video summarizes importance of UBM application in angle closure glaucoma to understand its pathophysiology by showing the relationship between the peripheral iris, trabecular meshwork and ciliary processes. Highlights: UBM provides two-dimensional, grayscale images of the angle structures and allows identification of non-pupillary block mechanism in angle closure glaucoma, which can be recorded for qualitative and quantitative analyses. Video Link: https://youtu.be/prsmGnR8jYc.

Evaluation of the characteristics of primary iridociliary cysts using ultrasound biomicroscopy at a tertiary care centre.

OBJECTIVE: To evaluate the ultrasound biomicroscopic characteristics of primary iridociliary cysts presenting to a Canadian tertiary care centre. DESIGN: Retrospective study. PARTICIPANTS: A total of 189 patients (212 eyes) referred to the Sinai Health System (Toronto) for suspected iris abnormalities. METHODS: Clinical records of patients referred between March 2016 and October 2019 were reviewed. All patients were evaluated and received a diagnosis of an iridociliary cyst using ultrasound biomicroscopy (UBM). Data were collected for age, sex, involvement (iris vs ciliary body), laterality, size, and location on initial examination and subsequent follow-up. RESULTS: Of the 189 patients (212 eyes) with iridociliary cysts, more were female (65.1%) versus male (34.9%). The highest incidence occurred in females aged 21-30 years (13.2%). The iris pigment epithelium was involved in 84.4%, and only the ciliary body was involved in 3.8%. Both the iris pigment epithelium and the ciliary body were involved in 10.8%. The size of the cysts ranged between 0.5 and 4.41 mm in diameter. Cysts greater than 1 mm in diameter occurred in 78.7%, and 86.8% of cysts occurred unilaterally. Twelve percent were multicystic, and 8.5% were multiloculated, with 1% exhibiting both features. Twenty-three eyes (12.2%) were reviewed at 1 year of follow-up with UBM. Stable iridociliary cysts with no appreciable change in size were seen in 73.9% (n = 17). Iridociliary cyst growth was noted at 4 months in 1 patient. CONCLUSION: Information regarding iridociliary cysts is not easily available in the literature. UBM is a helpful clinical tool in the evaluation of iris abnormalities. Iridociliary cysts tend to be stable and compatible with a low rate of complications.

Imaging of the Ciliary Body: A Major Review.

PURPOSE: We conducted a systematic search of literature to understand the various methods of imaging of the ciliary body. METHODS: PubMed, Science Direct, Cochrane Library and Google Scholar were searched comprehensively and systematically to find studies related to the various modalities of ciliary body imaging. RESULTS: The various ciliary body parameters that have been described are Ciliary body thickness, Ciliary body length, ciliary muscle thickness, ciliary process length, ciliary muscle length, ciliary muscle anterior length, trabecular ciliary process distance and Iris ciliary process distance. The various angles which have been measured, which mostly have a significance in Primary angle closure glaucoma (PACG) are Iris ciliary angle, Trabecular ciliary angle, scleral ciliary process angle. Various authors have defined them in various ways with subtle differences. Plateau iris and PACG mechanisms, not forgetting malignant glaucoma are better understood with imaging of the ciliary body using the ultrasound biomicroscopy (UBM). The anterior segment optical coherence tomography (ASOCT) imaging of the ciliary body has been described albeit with its own disadvantages. A few other fields dependant on the importance of ciliary body imaging are intravitreal injections, pars plana vitrectomy, measurements for implantable collamer lens (ICL) and of utmost importance, the differentiating features of ciliary body masses. CONCLUSION: The UBM is still preferred over the ASOCT for imaging of the ciliary body. A lot of lacunae of knowledge still exists and consensus has to be reached on defining all the parameters universally. Future studies will be able to shed more light on the role of the ciliary body in the many ocular disorders mentioned in this review.

Evaluating Ciliary Body Damage Induced by Blunt Low Speed Impact Using Finite Element Simulation.

PURPOSE: To evaluate the ciliary body mechanical load during low speed impact using finite element method to explain the mechanism of the cause of angle recession and cyclodialysis cleft. METHODS: Optical coherence tomography images were obtained to assess the patient's ciliary body angle recession. A finite element eye model was established based on Virginia Tech eye model with the consideration of dynamic impact of a projectile striking an eye. The mechanical properties of the ocular tissues were obtained from literatures. The stress and strain were evaluated. RESULTS: The stress distribution of the eye was calculated. The stress concentration at zonules was observed after 0.75 ms of the impact. The maximum stress at the cornea reached 3.8 MPa. The maximum stress at ciliary body was 57 KPa, which has high probability to cause ciliary body injury. The maximum stress at zonules was 0.98 MPa. The lateral expansion also reduces the forces transmitted along the sclera to the rear part of the eye. CONCLUSIONS: The eye under frontal impact will result in lateral expansion, which increase the stretch force of the lens, zonules and ciliary body. This mechanism can be seen as the protection for retina. The boundary of ciliary body is the most vulnerable position, where angle recession and cyclodialyses cleft will occur before retina damage occurrence. TRANSLATIONAL RELEVANCE: The finite element model explains the blunt low speed impact induced ciliary body related injuries, which enables us to assess the ocular injury for low energy impact and better diagnosis and treatment in clinics.

Diagnostic imaging of the ciliary body: Technologies, outcomes, and future perspectives.

The ciliary body (CB) is part of the uvea and is a complex, highly specialized structure with multiple functions and significant relationships with nearby structures. Its functions include the aqueous humor (AH) production in the ciliary processes, the regulation of the AH output through the uveoscleral pathway, and accommodation, which depends on the ciliary muscle. Also, the CB is an important determinant of angle width as it forms part of the ciliary sulcus. Until recently, knowledge of the CB was based on histological studies. However, this structure can currently be assessed in vivo using imaging techniques such as ultrasound biomicroscopy (UBM) and optical coherence tomography (OCT). Both techniques have shown good reproducibility of their measurements allowing for quantification of CB dimensions and their localization. In effect, studies have shown a larger CB in myopia and its diminishing size with age. Swept-source OCT devices offer fast, non-invasive high-resolution imaging allowing the identification of multiple structures. UBM requires contact and is uncomfortable for the patient. However, this technique offers deeper imaging and therefore remains the gold standard for assessing the posterior chamber, ciliary processes, or zonula. The clinical utility of CB imaging includes its assessment in different types of glaucoma such as angle-closure, malignant or plateau iris. Diagnostic CB imaging is also invaluable for the assessment of ciliochoroidal detachment when suspected, the position after the implantation of a pre-crystalline or sulcus-sutured lenses, diagnosis or monitoring of cysts or tumors, sclerotomies after retinal surgery, intermediate uveitis, or accommodation.

Ultrasound biomicroscopy of the anterior segment in patients with primary congenital glaucoma: a review of the literature.

PURPOSE: Primary congenital glaucoma (PCG) is a form of childhood glaucoma caused by maldevelopment of the anterior chamber. Disease severity differs greatly amongst patients. Ultrasound biomicroscopy (UBM) is a non-invasive technique that can visualize the anterior segment in infants in vivo. The purpose of this narrative review is to make an overview of the UBM data in PCG and study the applicability of UBM in characterizing the disease. METHODS: An online search was performed on PubMed in December 2020. After a critical appraisal of the included articles, study and patient characteristics were summarized. The UBM measurements of the anterior segment in PCG of the different studies were analysed. RESULTS: Six studies were included in this review. All were cross-sectional prospective studies. A total of 221 PCG eyes were examined. PCG eyes showed a larger trabecular iris angle, decreased iris thickness, narrower or absent Schlemm's canal and an increased zonular length compared to controls. Abnormal tissue membrane covering the trabecular meshwork and abnormal insertion of the iris and ciliary process were frequently found. The success rate of glaucoma surgery depended on the severity of anterior segment malformations found with UBM. CONCLUSION: Malformations of the anterior segment in PCG can be demonstrated by UBM in vivo. This imaging can help to characterize disease severity and might support surgical treatment decisions.

Endoscopy-guided in vivo evaluation of ciliary sulcus location in children with ectopia lentis.

OBJECTIVE: To assess a new method to measure the distance of the needle passage from the ciliary sulcus to the corneal limbus anterior border (CTC) in eyes with ectopia lentis directly in vivo via endoscopy and to further evaluate the correlations among the CTC, age, automated horizontal white-to-white distance (WTW), and ocular axial length (AL). METHODS: The WTW and AL were measured using an optical biometer. An intraocular endoscope was used during transscleral suture fixation of posterior chamber intraocular lenses to identify the true location of the ciliary sulcus. Linear regression analysis was used to assess the correlation between the CTC and other ocular biological parameters, including age, WTW, and AL. RESULTS: Thirty eyes of 30 children with ectopia lentis were evaluated. A statistically significant correlation was found between age and the CTC. The CTC could be predicted by the equation CTC = 0.1313 × Age + 0.9666. No statistically significant correlations were found between CTC and WTW, CTC and AL, WTW and AL, or WTW and age. CONCLUSION: Endoscopy is useful for precisely suturing intraocular lens haptics in the real ciliary sulcus. Age can be used as an equivalent parameter for prediction of the true ciliary sulcus location.

Subconjunctival Mass as Rare Presentation of Even Rarer Intraocular Medulloepithelioma: A Case Report.

Medulloepithelioma is a rare childhood embryonal tumor arising from the non-pigmented ciliary epithelium of the pars plicata. We report a case of an 11-year-old male who presented with painless loss of vision of the right eye for the last three years and progressively increasing mass on the superior aspect of the globe for the last three months. On ocular examination, a firm, non-tender mass of 4cm x 3cm was noted in the superior aspect of the globe. CT-Scan of the orbit was suggestive of a foreign body with a haemorrhage or infection. The patient underwent enucleation with minimal manipulation. Histopathological examination of the enucleated globe revealed medulloepithelioma. The intraocular medulloepithelioma presentation is often late and masquerading, which may lead to extraocular extension and metastasis and ultimately unfavorable prognosis.

Clinical 3D Imaging of the Anterior Segment With Ultrasound Biomicroscopy.

Purpose: Ultrasound biomicroscopy (UBM) is an important ophthalmic imaging modality due to its ability to see behind pigmented iris and to visualize anterior chamber when the eye's transparency is compromised. We created a three-dimensional UBM (3D-UBM) system and acquired example images to illustrate its potential. Methods: A commercial 50-MHz two-dimensional UBM (2D-UBM) system was attached to a precision translation stage and translated across the eye to acquire an image volume. The stage was mounted on a surgical microscope, which enabled safe, stable positioning. Image processing steps included image alignment, noise reduction, and calibration. 3D visualization included alignment of the optic axis, multiplanar reformatting at arbitrary orientations, and volume rendering with optimized transfer functions. Scans were performed on cadaver and rabbit eyes. Results: 3D-UBM allowed visualization of the anterior segment tissues within a 3D anatomical context, unlike 2D-UBM. En face views and interactive slicer operations suggested an ability to plan and assess treatments, including lens placement and microcatheter cannulation of Schlemm's canal. Interactive software allowed us to make accurate measurements of tissue structures (e.g., iridocorneal angles, cyst volumes). In addition, unique measurements of ciliary tissues included single ciliary process volumes of 0.234 ± 0.093 mm3 with surface areas of 3.02 ± 1.07 mm2 and ciliary muscle volume of 67.87 mm3. Conclusions: 3D-UBM imaging of the anterior segment can be used to enable unique visualization and quantification of anterior segment structures. Translational Relevance: 3D-UBM provides informative 3D imaging of tissues in the eye that are invisible to light to potentially provide physicians with improved diagnosis, treatment planning, and treatment assessment as compared to conventional 2D-UBM.

Ring lymphoma: highly indicative ultrasound biomicroscopy findings of ciliary body lymphoma.

BACKGROUND: To describe the ultrasound biomicroscopy (UBM) findings of extremely rare ciliary body lymphoma. METHODS: This was a retrospective noncomparative case series study. Five patients (8 affected eyes) diagnosed with ciliary body lymphoma at Peking Union Medical College Hospital from 2008 to 2019 were included. The UBM findings, including the location, height, and acoustic features, were documented to assess the tumour characteristics. RESULTS: UBM in all 8 (100%) affected eyes revealed 360° ring-like, solid infiltration of the ciliary body with low and homogeneous internal reflectivity. The continuity of the tumours could be most vividly demonstrated by transverse sections. CONCLUSIONS: Ciliary body lymphoma tends to grow in a characteristic circumferential 360° pattern, and the name "ring lymphoma of the ciliary body" is proposed.