Axenfeld-Rieger Syndrome: Case Report

Aims:To describe a Axenfeld-Rieger Syndrome.Presentation of Case: MCL, 7 years old, female, brown, was taken to the ophthalmology outpatient clinic of the Hospital Universit醨io Ant鬾io Pedro, Brazil by her parents, complaining of low visual acuity and malformation of the pupil perceived since birth.Discussion: Axenfeld-Rieger Syndrome is a rare and hereditary disease. Clinically, Axenfeld's anomaly is characterized by the presence of posterior embryotoxon, and there may be adherence of iridian tissue in its periphery. In addition to Rieger's anomaly, posterior embryotoxon is added to iris hypoplasia and iris thickness defects, uveal ectropion and pupillary alterations, such as corectopia. Rieger's syndrome is associated with extraocular changes, of which hypodontia, myicrodontia, maxillary hypoplasia, telecanthus, hypertelorism and hypospadias stand out.Conclusions: Therefore, the importance of early diagnosis, follow-up and adequate treatment becomes evident in order to preserve the visual function of patients and thus avoid an unfavorable evolution.

Bilateral Iridocorneal Endothelial Syndrome

Objectives@#We presented a case of bilateral iridocorneal endothelial (ICE) syndrome with secondary glaucoma and discussed its clinical presentation and management.@*Study design@#This is a case report.@*Results@#A 12-year old female consulted for a one-year history of progressive blurring of vision in both eyes associated with abnormal pupils and occasional eye pain. Polycoria, shallow anterior chambers, increased intraocular pressures (IOP), areas of closed angles on gonioscopy, and increased cup-to-disc ratio in both eyes indicative of ICE syndrome were present. Management included anti-glaucoma medications and implantation of glaucoma drainage devices (GDD).@*Conclusion@#Early detection with regular follow-ups leading to prompt management of the ICE syndrome are necessary. IOP control may be challenging due to the nature of the disease and may require multiple surgeries including GDD implantation to achieve successful outcomes. Early use of GDD may be beneficial for pediatric patients with ICE and glaucoma, and may require additional surgeries to adequately manage the IOP.

Clinical research status of iridocorneal endothelial syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

@#Iridocorneal endothelial(ICE)syndrome presents a group of ocular pathologies mostly characterized by corneal endothelial abnormalities and iris atrophy. Iridocorneal endothelial syndrome is typically a unilateral and generally not involving genetic condition, frequently affecting women in early to middle adulthood. There is no treatment for absence of symptoms in early stages. When complicated with corneal edema and secondary glaucoma, treatment is mainly symptomatic. In this group of diseases, there are many similarities and some differences in their pathogenesis, clinical manifestations and treatment. So far no universal information on these features has been published. Recent clinical studies on iridocorneal endothelial syndrome are reviewed in this paper.

Progress of iridocorneal endothelial syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

@#Iridocorneal endothelial syndrome(ICES)is a rare ocular disease characterized by abnormal structure and proliferation of the corneal endothelium, the anterior chamber angle, and the iris. Common clinical features include corneal edema, secondary glaucoma and iris atrophy. ICES often occurs in young women, and most of them are monocular. Its pathogenesis is still unclear, the symptoms are various, and the blindness rate is high. The disease is difficult to diagnose, and there is no ideal treatment. The purpose of this article is to review the literature on the characteristics, diagnosis and treatment of ICES in order to help the diagnosis and treatment of the disease.

Progressive hemifacial atrophy with ciliary body atrophy and ocular hypotony.

Progressive hemifacial atrophy (PHA) is a disease of unknown etiology affecting one‑half of the face. Ocular involvement is uncommon. Atrophy of iris is rare, with only a few cases of partial atrophy being reported in the literature. We report a case of total atrophy of iris and ciliary body with associated ocular hypotony in a 16‑year‑old girl with PHA. We believe this is the Access this article online Quick Response Code: Website: www.ijo.in DOI: 10.4103/0301-4738.151474 PMID: *** first reported case of complete atrophy of iris and ciliary body in PHA. Ocular hypotony in PHA was thought to be due to intra‑ocular inflammation. However in our case it appears to be secondary to severe atrophy of the ciliary body.

Three Cases of Pupil Abnormality in Herpes Zoster Ophthalmicus

PURPOSE: We report the occurrence of pupil abnormality in 3 patients with herpes zoster ophthalmicus. CASE SUMMARY: Three patients diagnosed with herpes zoster ophthalmicus developed pupil abnormality. (Case 1) A 37-year-old male diagnosed 1 month prior with anterior uveitis secondary to herpes zoster ophthalmicus presented with peripheral corneal erosions, inflammatory cells in the anterior chamber, diffuse iris atrophy, almost fully-dilated pupils, and loss of pupil light reflex in the right eye. (Case 2) A 72-year-old male presented with vesicles on the right side of the face, and dendritic corneal ulcer, and inflammatory cells in the anterior chamber on initial examinations. After 5 days without treatment by his choice, decreased vision, decreased pupil light reflex, and ovoid-shaped pupils developed. (Case 3) A 63-year-old female presented with left ocular pain, vesicles around the left eye, dendritic corneal ulcer, inflammatory cells in the anterior chamber, and isocoric pupils with normal pupil light reflexes. However, in her left eye, the pupillary ruff was partially lost and the pupil was larger than the right pupil after the start of a 3-week treatment regimen. The pupil in Case 2 returned to normal after 1 month, but in cases 1 and 3, no improvements of pupil abnormalities were observed during the follow-up period. CONCLUSIONS: Herein we presented 3 patients that were diagnosed with herpes zoster ophthalmicus and subsequently developed pupil abnormalities. In the cases of pupil abnormalities, checking for a history of herpes zoster ophthalmicus is necessary to make a differential diagnosis.

Síndrome iridocórneo-endotelial: presentación de un caso / Iridocorneal-endothelial syndrome: presentation of a case

El síndrome iridocórneo-endotelial agrupa tres síndromes que cuando se presentan en las formas puras son relativamente fáciles de distinguir; sin embargo, es más frecuente que aparezca con un solapamiento considerable dificultando así el establecer una clara definición. Es una entidad nosológica muy poco común en la consulta de Oftalmología del Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez Hernández, de Matanzas, lo cual motivó la presentación de este caso. Se trata de una paciente de 47 años, que acudió a consulta en el mencionado hospital, que acudió a consulta en el mes de enero de 2012, portadora de un síndrome iridocórneo-endotelial unilateral en ojo derecho, con agudeza visual de 20/20, atrofia del iris, corectopia, pseudopolicoria y glaucoma secundario con hipertensión ocular marcada, a la que se le realizó trabeculectomía como proceder quirúrgico, con evolución satisfactoria.

The iridocorneal-endothelial syndrome comprehends three syndromes that when they appear in their pure forms are relatively easy to distinguish; but more frequently it appears considerably sneaked, making it difficult to stablish a clear definition. It is a nosological entity very uncommon in the consultation of Oftalmology of the Clinica-surgical Teaching Hospital Comandante Faustino Perez Hernandez, of Matanzas. That motivated the presentation of this case. It deals with a female patient, aged 47 years, assisting the consultation of the before mentioned hospital in January 2012, carrying a unilateral iridocorneal-endothelial syndrome in the right eye, with a visual acuity of 20/20, iris atrophy, corectopia, pseudopolychoria and secondary glaucoma with remarked ocular hypertension. She was subject of a trabeculectomy as a surgical procedure, with a satisfactory evolution.

Progressive Iris Atrophy

Progressive iris atrophy is a disease characterized by marked iris atrophy, iris hole, corneal endotherial abnormality, perripheral anterior synechia, and glaucoma. It is considered as a variant of iridocorneal endotherial syndrome with Chandler's syndrome and Cogan-Reese syndrome. We have experianced a 34 year old male patient complaning visual blurring in his left eye. At examination, we found characteristics of progressive iris atrophy. After filerring surgery, associated glaucoma was well controlled. Then we report a case with review of the literatures.

A Case of Cogan-Reese Syndrome

Cogan-Reese syndrome is a disease characterized by glaucoma in an eye with peripheral anterior synechia, multiple pigmented iris nodules, and ectopic Descemet's membranes. Cogan-Reese syndrome is a variant of iridocorneal endothelial syndrome and shares common properties whcih are corneal endothelial degeneration, iris atrophy and secondary glaucoma, with progressive iris atrophy and Chandler's syndrome. We have experienced a 43-year-old female patient complaining of visual dimness in the left eye. On examination, we found characteristics of Cogan-Reese syndrome which were corneal endothelial pleomorphism and cell loss, corneal edema, iris atrophy and multiple pigmented nodules on the surface of the iris, peripheral anterior synechia, and increased intraocular pressure in the left eye. We report a case of Cogan-Reese syndrome which has not been reported in Korea.