Long-term outcomes of "open iridectomy" for secondary anterior chamber epithelial iris cysts.

Epithelial cysts run a high risk of recurrence and conversion to sheet-like ingrowth after surgical intervention. In this retrospective study, we introduced a modified iridectomy for treatment of secondary epithelial iris cysts (EICs) in the anterior chamber. Twenty-nine patients (29 eyes) aged 2-61 years received "open iridectomy" for EICs between April 1995 and July 2019. After viscodissection, most of the cyst wall was cut using a 20-gauge aspiration cutter via a 2.5-mm clear corneal incision. The residue closely adhering to the iris stroma was remained to avoid photophobia and diplopia. At 3 months, best corrected visual acuity was ≥ 20/100 in 55.5% (15/27, except two pediatric patients with poor cooperation) of patients. Among the eight patients suffering partial corneal edema preoperatively, six patients received surgery treatment at 3-6.5 months, and the cornea in the other two patients became transparent after medication. In a mean follow-up of 47.4 months, recurrence occurred in 3 patients at 7, 37, and 118 months, respectively. The percentage of treatment success was 96%, 87%, and 65% at 1, 5, and 10 years, respectively. "Open iridectomy" was effective for EICs, with a minimal invasion, less damage to the corneal endothelium, and a low recurrence rate.

Heterocromia de íris: uma revisão das condições que podem afetar a pigmentação iridiana / Heterochromia: a review of conditions that may affect iris pigmentation

RESUMO A íris é responsável pela cor dos olhos. Ela ainda realiza o controle da quantidade de luz que penetra no olho pela pupila. Variações nos genes de cada indivíduo, além da quantidade e da qualidade de melanina na íris, determinam a cor dos olhos. A heterocromia é caracterizada por diferenças na coloração da íris de um mesmo indivíduo, sendo, na maioria das vezes, benigna. Existem basicamente três tipos de heterocromia de íris: central, setorial e completa. A heterocromia de íris pode ter como causa alterações genéticas e congênitas, relacionadas ou não a síndromes específicas, como a de Sturge-Weber, a de Waardenburg, a de Parry-Romberg e a de Horner congênita. Há também causas adquiridas, como doenças ou lesões, trauma ocular e corpos estranhos intraoculares, uso de certas medicações tópicas, siderose ocular, irites ou uveítes como a síndrome uveítica de Fuchs, dentre outras. Diante de um paciente com heterocromia de íris, deve-se entender o contexto e o curso clínico desse sinal, pois pode se tratar de uma alteração de pigmentação benigna ou existir uma doença base em curso, que requer terapêutica específica. Este artigo de revisão de literatura visa abordar as principais etiologias relacionadas à heterocromia de íris, além de discorrer sobre a anatomia e a fisiologia da coloração iridiana e sobre a fisiopatologia de suas possíveis alterações.

ABSTRACT The iris is responsible for eye color and controls the amount of light that enters the eye through the pupil. Variation in each individual's genes, besides the quantity and quality of melanin in the iris, determine eye color. Heterochromia is characterized by different colors of irises in the same individual, and it is benign in most cases. There are basically three types of heterochromia: central, partial and complete. Heterochromia can be caused by genetic and congenital alterations, which may or may not be related to specific conditions, such as Sturge-Weber syndrome, Waardenburg syndrome, Parry-Romberg syndrome and congenital Horner syndrome. It may be associated to acquired causes like diseases or injuries, such as eye trauma and intraocular foreign bodies, use of some topical medications, ocular siderosis, iritis or uveitis, such as Fuchs´ uveitis, among others. When assessing a patient with heterochromia, one must understand the context and clinical course of this signal, since it may be a benign pigmentation disorder or there may be an underlying disease, which requires specific therapy. This literature review article was set out to address the main etiologies related to heterochromia, in addition to describing the anatomy and physiology of the iris color and the pathophysiology of possible alterations.

Congenital Grouped Albinotic Spots: New Insights on the Pathophysiology Based on Multimodal Retinal Imaging.

The terms "congenital grouped albinotic spots" (CGAS) and "polar bear tracks" refer to a rare, benign retinal disorder of unknown etiology characterized by multiple, predominantly unilateral, variably sized, well-circumscribed, flat white retinal spots organized in groups. To date, very few cases of CGAS have been reported. The authors describe a case of CGAS thoroughly characterized by multimodal imaging over an 8-year follow-up, aiming to provide new insights on the pathophysiology of this entity. This is the first report where a long follow-up combined with up-to-date imaging technology is used to characterize CGAS. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:236-238.].

[Congenital Simple Hamartoma of the Retinal Pigment Epithelium]. / Kongenitales einfaches Hamartom des retinalen Pigmentepithels.

BACKGROUND: Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is an uncommon benign lesion with characteristic clinical features. Ophthalmoscopically it appears as a small localized, well circumscribed, pigmented tumor in the foveal region. In contrast to the more common flat congenital hypertrophy of the RPE the CSHRPE has an elevated nodular appearance. PATIENTS AND METHODS: Retrospective case series of three patients with CSHRPE. Clinical morphological features using different imaging techniques are presented. RESULTS: A typical dark lesion was incidentally noted in the macula of two patients. Optical coherence tomography (OCT) demonstrated a nodular preretinal hyperreflectivity with shadowing of deeper structures. In one patient the CSHRPE was hypofluorescent throughout the angiogram. The third patient presented with a reduced visual acuity of 0.3. A characteristic lesion was found at the foveal center. OCT revealed a hyperreflective preretinal lesion with associated moderate disruption of the foveal architecture. Amblyopia treatment slightly improved visual acuity in this case. The lesions remained stationary in two patients (follow-up 8 - 14 months). CONCLUSIONS: CSHRPE are usually detected as an incidental finding. Given its benign character and typically asymptomatic presentation an observational treatment approach is generally recommended. The lesions generally remain stationary and are not known to grow. In cases with visual impairment due to foveal involvement amblyopia treatment should be initiated.

Congenital ectropion uveae with glaucoma: a case report.

Congenital ectropion uveae (CEU) is a rare anomaly characterized by ectropion uveae, iris hypoplasia, iridotrabecular dysgenesis and glaucoma. The apparent ectropion uveae results from the spread of iris pigment epithelium beyond the iris ruff and onto the anterior surface of the iris. Conclusion Open-angle glaucoma results due to angle dysgenesis, so patients should be carefully examined periodically for its early detection.

[Diagnosis and Therapy of Iris Lesions]. / Diagnostik und Therapie von Irisläsionen.

The most common iris lesions are iris nevi, iris melanomas and iris pigment epithelium cysts. However, there is an abundance of rare differential diagnoses that have to be considered, including other melanocytic and non-melanocytic lesions. Diagnostic tools include the slit lamp examination, gonioscopy, tonometry, transillumination, ultrasound biomicroscopy (UBM), optical coherence tomography, fluorescein angiography and standardized photography-assisted documentation. The timely identification of malignant lesions (i.e. iris melanoma) is paramount. To assess malignancy criteria of iris nevi, the ABCDEF rule (age young, blood, clock hour inferior, diffuse growth, ektropion uveae, feathery margins) can be applied. Statistically, up to 11% of iris nevi may develop into iris melanomas within 20 years. TNM Staging follows the 2010 AJCC cancer staging manual and helps determine the optimal treatment strategy. Treatment options include radiotherapy, such as plaque brachytherapy and proton beam radiation therapy, as well as surgical excision. Both the surgical and the radiotherapeutic approaches show comparable local tumor control rates. However, the spectrum of therapy-related side effects and complications may differ amongst treatment modalities. After initial treatment, patients should be followed up every 3 - 6 months. Tumor-related mortality ranges between 0 - 11% and is significantly lower than in other uveal melanomas. A prognostic value of common genetic alterations, which have been identified as significant prognostic factors in posterior uveal melanoma, could not be shown for iris melanoma.

A young Botswana patient with congenital iris ectropion uvea.

Congenital iris ectropion is a rare condition; non-progressive anomaly characterised by the presence of iris pigment epithelium on the anterior surface of the iris stroma and is frequently associated with anterior iris insertion, dysgenesis of the drainage angle and glaucoma. This paper describes unusual case of bilateral case of congenital iris ectropion in adult patient with pupillary abnormality, normal anterior chamber angle structure and with no evidence of glaucoma.

Ambliopía secundaria a quiste de iris / Amblyopia secondary to iris cyst

CASO CLÍNICO: Varón de 5 años de edad con ambliopía anisometrópica meridional secundaria a quiste de epitelio pigmentario de iris. Es evaluado mediante biomicroscopia ultrasónica (BMU) y tomografía de coherencia óptica de polo anterior (OCT Visante). DISCUSIÓN: La OCT de polo anterior, aunque con limitaciones, es una herramienta útil en la evaluación de lesiones de polo anterior. Puede ser preferible, en la infancia, a la BMU

CLINICAL CASE: A 5 year-old child diagnosed with moderate anisometropic amblyopia secondary to primary cyst of iris pigment epithelium. He was evaluated with ultrasound biomicroscopy (BMU) and optical coherence tomography (OCT) of anterior segment. DISCUSSION: The OCT, although with some limitations, is a useful tool to study the anterior segment. It is probably more recommendable than BMU in the childhood (AU)

Amblyopia secondary to iris cyst.

CLINICAL CASE: A 5 year-old child diagnosed with moderate anisometropic amblyopia secondary to primary cyst of iris pigment epithelium. He was evaluated with ultrasound biomicroscopy (BMU) and optical coherence tomography (OCT) of anterior segment. DISCUSSION: The OCT, although with some limitations, is a useful tool to study the anterior segment. It is probably more recommendable than BMU in the childhood.

Congenital ectropion uveae presenting as acute glaucoma in a 3-year-old child.

Congenital ectropion uveae is a rare anomaly commonly associated with neurofibromatosis and occasionally with other ocular abnormalities. Glaucoma related with this condition may be present in infancy, or may develop later in life, and is thought to be due to an associated angle dysgenesis. Diagnosis is frequently delayed due to the subtle signs and the absence of symptoms and management is primarily surgical. We report an unusual case of unilateral congenital ectropion uveae in a 3-year-old child, with no evidence of neurofibromatosis, presenting as acute glaucoma, which was successfully managed by topical treatment only, avoiding surgical intervention.

[Partially pigmented vitreous cyst]. / 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.

Effect of primary iris and ciliary body cyst on anterior chamber angle in patients with shallow anterior chamber.

OBJECTIVE: To evaluate the prevalence of primary iris and/or ciliary body cysts in eyes with shallow anterior chamber and their effect on the narrowing of the anterior chamber angle. METHODS: Among the general physical check-up population, subjects with shallow anterior chambers, as judged by van Herick technique, were recruited for further investigation. Ultrasound biomicroscope (UBM) was used to detect and measure the cysts located in the iris and/or ciliary body, the anterior chamber depth (ACD), the angle opening distance at 500 µm (AOD500), and the trabecular-iris angle (TIA). A-scan ultrasonography was used to measure the ocular biometry, including lens thickness, axial length, lens/axial length factor (LAF), and relative lens position (RLP). The effect of the cyst on narrowing the corresponding anterior chamber angle and the entire angle was evaluated by the UBM images, ocular biometry, and gonioscopic grading. The eye with unilateral cyst was compared with the eye without the cyst for further analysis. RESULTS: Among the 727 subjects with shallow anterior chamber, primary iris and ciliary body cysts were detected in 250 (34.4%) patients; among them 96 (38.4%) patients showed unilateral single cyst, 21 (8.4%) patients had unilateral double cysts, and 42 (16.8%) patients manifested unilateral multiple and multi-quadrants cysts. Plateau iris configuration was found in 140 of 361 (38.8%) eyes with cysts. The mean size of total cysts was (0.6547 ± 0.2319) mm. In evaluation of the effect of the cyst size and location on narrowing the corresponding angle to their position, the proportion of the cysts causing corresponding angle narrowing or closure among the cysts larger than 0.8 mm (113/121, 93.4%) was found to be significantly higher than that of the cysts smaller than 0.8 mm (373/801, 46.6%), and a significant higher proportion was also found in the cysts located at iridociliary sulcus (354/437, 81.0%) than in that at the pars plicata (131/484, 27.1%). In evaluating the effect of the cyst on the entire anterior chamber angle, the eyes with multiple and multi-quadrants cysts manifested significant narrowing of the entire anterior chamber angle as compared with the eyes without cysts, based on the data analysis in comparison of TIA, AOD500, and gonioscopic grading evaluation. The unilateral single or double cysts in the eyes had no significant effect on narrowing of anterior chamber angle as compared with eyes without cysts. The iris and/or ciliary body cysts did not seem to affect the axial length, ACD, lens thickness, RLP, LAF. CONCLUSIONS: The prevalence of primary iris and ciliary body cyst was 34.4% in the subjects with shallow anterior chamber. The cysts larger than 0.8 mm, locating at iridociliary sulcus, or multiple and extensive cysts were inclined to cause the angle narrowing or closure.

Don't it make my blue eyes brown: heterochromia and other abnormalities of the iris.

Eye colour is one of the most important characteristics in determining facial appearance. In this paper I shall discuss the anatomy and genetics of normal eye colour, together with a wide and diverse range of conditions that may produce an alteration in normal iris pigmentation or form.

Congenital ectropion uveae with iris coloboma and telecanthus.

Congenital ectropion uveae (CEU) is a rare, non progressive anomaly characterised by the presence of iris pigment epithelium on the anterior surface and is frequently associated with anterior iris insertion, dysgenesis of the drainage angle and glaucoma. This paper describes an unusual case of bilateral congenital ectropion uveae with iris coloboma and telecanthus. The anterior chamber angle was normal and there was no evidence of glaucoma. To the best of our knowledge, this association has not been reported previously.

Congenital iris ectropion associated with juvenile glaucoma.

Congenital iris ectropion is an uncommon malformation and no reports exist about the use of modern technologies in this pathology. The authors describe a case of unilateral and isolated congenital iris ectropion associated with juvenile glaucoma in a healthy and completely asymptomatic 6-year-old girl with an unusual form of anisocoria. Optical coherence tomography and confocal scanning laser tomography showed a progressive glaucomatous neuropathy. A trabeculectomy without antimetabolites was performed and intraocular pressure normalized without other medications during a follow-up of 2 years. The authors assert the utility of various diagnostic technologies to recognize congenital iris ectropion early to prevent blindness in young patients and improve their prognosis.

Acute multifocal placoid pigment epitheliopathy associated with erythema nodosum and a flu-like illness.

Acute multifocal placoid pigment epitheliopathy (AMPPE) is a rare inflammatory vasculitis of the choroid. Despite primarily being a disorder of the eye, a number of extra-ophthalmological features have been described in AMPPE and may accompany the visual disturbances. Such patients may be admitted under the care of physicians for evaluation of a systemic illness. We report AMPPE occurring in a 37-year-old man in his thirties admitted with a flu-like illness, erythema nodosum, visual disturbances and raised inflammatory markers. Conditions such as bacterial endocarditis and vasculitis, were considered before a diagnosis of AMPPE was made. The case illustrates how AMPPE can be a diagnostic dilemma, particularly when associated with extra-ophthalmological features. The epidemiology, clinical features, diagnosis and therapy of AMPPE are also discussed.

Indian hedgehog signaling from endothelial cells is required for sclera and retinal pigment epithelium development in the mouse eye.

The development of extraocular orbital structures, in particular the choroid and sclera, is regulated by a complex series of interactions between neuroectoderm, neural crest and mesoderm derivatives, although in many instances the signals that mediate these interactions are not known. In this study we have investigated the function of Indian hedgehog (Ihh) in the developing mammalian eye. We show that Ihh is expressed in a population of non-pigmented cells located in the developing choroid adjacent to the RPE. The analysis of Hh mutant mice demonstrates that the RPE and developing scleral mesenchyme are direct targets of Ihh signaling and that Ihh is required for the normal pigmentation pattern of the RPE and the condensation of mesenchymal cells to form the sclera. Our findings also indicate that Ihh signals indirectly to promote proliferation and photoreceptor specification in the neural retina. This study identifies Ihh as a novel choroid-derived signal that regulates RPE, sclera and neural retina development.