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.
Iridocorneal Endothelial Syndrome

PURPOSE: To report a case of iridocorneal endothelial syndrome, which overlapped with some of the features of posterior polymorphous corneal dystrophy. CASE SUMMARY: A 61-year-old female presented with tearing pain and blurred vision in her left eye, which was aggravated in the morning. The symptom started approximately 1 year prior to her visit. At the initial visit, the visual acuities were 1.0 in both eyes and the intraocular pressures were normal. On slit-lamp examination, a single pair of horizontal parallel lines was observed at the central corneal endothelial layer in the right eye. In contrast, multiple pairs of oblique parallel lines were observed in the left eye. The lines of the lesions were more prominent and wavier in the left eye than those of the right eye. The overlying cornea was clear, and the corneal thicknesses were in the normal range in both eyes. Using a gonioscopic examination, localized peripheral anterior synechiae were observed only in the left eye. The pupil and iris were normal in both eyes. On specular microscopic examination, the corneal endothelial cell size in the right eye increased and the corneal endothelial density decreased to 668 cells/mm². In the left eye, multiple abnormal endothelial cells with dark-light reversal were observed. In conclusion, the patient was subsequently diagnosed with iridocorneal syndrome, rather than posterior polymorphous corneal dystrophy. CONCLUSIONS: Posterior polymorphous corneal dystrophy and iridocorneal endothelial syndrome may present with many similarities. Therefore, in cases of uncertain diagnosis, an understanding of the clinical features is important for proper diagnosis.
Cornea ; Diagnosis ; Endothelial Cells ; Female ; Humans ; Intraocular Pressure ; Iridocorneal Endothelial Syndrome ; Iris ; Middle Aged ; Pupil ; Reference Values ; Tears ; Visual Acuity

PURPOSE: To compare surgical outcomes and corneal endothelial cell densities in patients with glaucoma secondary to iridocorneal endothelial (ICE) syndrome who underwent trabeculectomy with mitomycin-C (MMC) versus those who underwent Ahmed Glaucoma Valve (AGV) implantation. METHODS: Twenty-one patients with ICE syndrome who underwent either trabeculectomy with MMC or AGV implantation were included in this study. All patients had more than 12 months of follow-up after glaucoma surgery. Intraocular pressure (IOP), visual acuity (VA) and corneal endothelial cell density were compared according to the method of surgery. RESULTS: Ten eyes had undergone a trabeculectomy with MMC, and 11 eyes had an AGV implantation. In eyes that underwent a trabeculectomy with a MMC, there were no statistically significant differences between preoperative and postoperative month 12 in IOP, VA or corneal endothelial cell density. In eyes that underwent AGV implantation, postoperative IOP, VA and corneal endothelial cell density were statistically significantly lower than the preoperative values. CONCLUSIONS: When determining the method of glaucoma surgery in patients with ICE syndrome, the additional procedures needed to maintain the surgical success of trabeculectomy with MMC and the corneal decompensation which is caused by a decrease in corneal endothelial cell density after AGV implantation should be considered.
Endothelial Cells ; Eye ; Follow-Up Studies ; Glaucoma ; Glaucoma Drainage Implants ; Humans ; Ice ; Intraocular Pressure ; Iridocorneal Endothelial Syndrome ; Mitomycin ; Trabeculectomy ; Visual Acuity

PURPOSE: To report a case of iridocorneal endothelial syndrome with pigmentary glaucoma. METHODS: We encountered a 44-year-old unilateral glaucoma patient who complained of intermittently decreased visual acuity in the right eye. For differential diagnosis, we carried out ophthalmic and systemic examination. RESULTS: Binocular corrected visual acuity was 1.0. His intraocular pressure, measured using a Goldmann applanation tonometer, was 50 mm Hg in the right eye and 18mm Hg in the left. Upon examination of the right eye, we found pigments with a Krukenberg's spindle appearance on the corneal endothelium, peripheral anterior synechia extending beyond Schwalbe's line, trabecular hyperpigmentation, endothelial pleomorphism, polymegathism, cell loss, dark area within the cells, a light central spot and light peripheral zone, retinal nerve fiber layer defects, and visual field defects, which together led to the diagnosis of iridocorneal endothelial syndrome with pigmentary glaucoma. We observed progressed glaucomatous injury upon examination of the disc stereo photograph, retinal nerve fiber layer photograph, and visual field test, even though we used topical IOP reducers. Eventually, we performed a trabeculectomy in the right eye. CONCLUSIONS: We experienced a case of iridocorneal endothelial syndrome with unilateral pigmentary glaucoma. The glaucoma was not well-controlled with topical IOP reducers. The glaucoma was then treated by a trabeculectomy. We report this case with a review of the literature.
Adult ; Diagnosis ; Diagnosis, Differential ; Endothelium, Corneal ; Glaucoma ; Glaucoma, Open-Angle* ; Humans ; Hyperpigmentation ; Intraocular Pressure ; Iridocorneal Endothelial Syndrome* ; Nerve Fibers ; Retinaldehyde ; Telescopes ; Trabeculectomy ; Visual Acuity ; Visual Field Tests ; Visual Fields

Human ; Adult ; GLAUCOMA-OPEN-ANGLE ; GLAUCOMA,SIMPLEX ; GLAUCOMA, COMPENSATED ; IRIDOCORNEAL ENDOTHELIAL SYNDROME ; FILTERING SURGERY ; GLAUCOMA ; SCLEROSTOMY ; TRABECULECTOMY

PURPOSE: To report a case of Chandler's syndrome associated with anterior uveitis. METHODS: We encountered a 39-year-old woman who complained of decreased visual acuity in the left eye with anterior uveitis and increased intraocular pressure. On examination, we found corectopia, endothelial pleomorphism and peripheral anterior synechiae, leading to the diagnosis of Chandler's syndrome with anterior uveitis. RESULTS: The anterior uveitis and increased intraocular pressure in Chandler's syndrome were well controlled with the use of Beta-blocker, Latanoprost and topical corticosteroids. We report this case with a review of the literatures.
Adrenal Cortex Hormones ; Adult ; Diagnosis ; Female ; Humans ; Intraocular Pressure ; Iridocorneal Endothelial Syndrome* ; Uveitis, Anterior* ; Visual Acuity

PURPOSE: To report a case of Chandler's syndrome associated with anterior uveitis. METHODS: We encountered a 39-year-old woman who complained of decreased visual acuity in the left eye with anterior uveitis and increased intraocular pressure. On examination, we found corectopia, endothelial pleomorphism and peripheral anterior synechiae, leading to the diagnosis of Chandler's syndrome with anterior uveitis. RESULTS: The anterior uveitis and increased intraocular pressure in Chandler's syndrome were well controlled with the use of Beta-blocker, Latanoprost and topical corticosteroids. We report this case with a review of the literatures.
Adrenal Cortex Hormones ; Adult ; Diagnosis ; Female ; Humans ; Intraocular Pressure ; Iridocorneal Endothelial Syndrome* ; Uveitis, Anterior* ; Visual Acuity

Iridocorneal endothelial(ICE) syndrome is charaterized by unilateral glaucoma, abnormal corneal endothelium with proliferation, and iris stromal abnormalities. Uncontrolled glaucoma in ICE syndrome has been treated with filtering surgery, but the late failures occurred frequently. The medical records of nine patients with ICE syndrome that underwent glaucoma surgery were retrospectively reviewed. 6 eyes were progressive iris atrophy, and 3 eyes were Cogan-Reese syndrome. The success rates of primary trabeculectomy at 1 and 2 years were 57.1% and 33.3%, respectively. Ahmed glaucoma valve implant oeprations were performed on 6 eyes with failed previous filtering operation. 4 of 6 eyes resulted in controlled IOP until last follow up visit(mean ; 18.8 months). Overall, 1.9 times operations were needed to control IOP. Operations were performed only one time in 4 patients and two times in 3 patients to control IOP.
Atrophy ; Endothelium, Corneal ; Filtering Surgery ; Follow-Up Studies ; Glaucoma* ; Humans ; Ice ; Iridocorneal Endothelial Syndrome* ; Iris ; Medical Records ; Retrospective Studies ; Trabeculectomy

Iridocorneal endothelial syndrome (ICE syndrome) embraces a group of rare diseases with similar characteristics in the anterior segment, namely Essential iris atrophy, Chandler`s syndrome, and Iris nevus syndrom (Cogan Reese syndrome). The origin and pathogenesis of ICE syndrome remain unkown, but it appears that it is an abnormality of the corneal endothelial cells that is fundamental to all three conditions and leads to characteristic changes in the cornea, iris, and angle. Finding by specular microscopy of abnormal cells on the posterior corneal surface has led to the speculation of possible pathogenensis of this condition. We have experienced two patients with Iris nevus syndrome. Both cases presented with glaucoma as the chief clinical problem, and they illustrated difficult challenges in the glaucoma managemant in this disorder.
Atrophy ; Cornea ; Endothelial Cells ; Glaucoma ; Humans ; Ice ; Iridocorneal Endothelial Syndrome* ; Iris* ; Microscopy ; Nevus* ; Rare Diseases

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.
Adult ; Atrophy* ; Glaucoma ; Humans ; Iridocorneal Endothelial Syndrome ; Iris* ; Male