Iridocorneal endothelial syndrome in a patient with keratoconus - a case report.

BACKGROUND: To describe a case of a rare association of bilateral keratoconus and unilateral essential iris atrophy and to conduct a literature review of the current strategies of treatment of the corneal disease and glaucoma in patients with Iridocorneal Endothelial Syndrome (ICE). CASE PRESENTATION: We report a rare association of bilateral keratoconus and unilateral essential iris atrophy in a 38-year-old man. Diagnosis of bilateral keratoconus was confirmed by corneal topography. Slit-lamp examination showed extensive iris atrophy with corectopia and policoria in one eye. Corneal specular microscopy revealed an abnormal endothelium morphology in the same eye with extensive peripheral anterior synechiae and closure of the drainage angle at gonioscopy. Intraocular pressure was 26 mmHg, despite maximal topical therapy. Optic disc examination showed severe glaucomatous cupping. Surgery by glaucoma drainage device implantation was performed. CONCLUSION: Essential iris atrophy is a rare clinical variant of ICE syndrome characterized by profound anatomical alterations of the anterior segment associated with corneal edema and secondary glaucoma. In these patients, selective keratoplasties have replaced penetrating keratoplasty to treat corneal decompensation and glaucoma drainage devices are preferred to conventional trabeculectomy for the treatment of secondary glaucoma.

Clinical outcome of Descemet stripping automated endothelial keratoplasty in 18 cases with iridocorneal endothelial syndrome.

PurposeTo evaluate the clinical outcome of Descemet stripping automated endothelial keratoplasty (DSAEK) in eyes with iridocorneal endothelial (ICE) syndrome.Patients and methodsA retrospective case series study was conducted. Eighteen consecutive Chinese patients with 20 DSAEK grafts were enrolled. Participants were evaluated by anterior segment optical coherence tomography and confocal microscopy. Postoperative complications, graft survival, endothelial cell counts, corneal thickness, and anterior chamber depth were analysed. A Log-rank test in a Kaplan-Meier analysis and a Cox proportional hazard regression were used to analyse potential risk factors of graft failure.ResultsThe mean follow-up duration was 19.0±8.6 months. The donors' endothelial cell density (ECD) (cells/mm2) values at 1, 3, 6, 12, 18, and 24 months were 3342.2±287.0, 1897.6±745.4, 1793.6±755.7, 1618.1±604.3, 1421.9±650.8, 1265.1±844.1, and 1148.2±1217.8, respectively. Eleven of the 20 grafts exhibited secondary graft failure, with a mean estimated graft survival of 23.4 months. Immediate postoperative complications (air bubble ventilation for elevated intraocular pressure or rebubbling for graft detachment) were more common in eyes exhibiting graft failure (P=0.040). Postkeratoplasty glaucoma surgery emerged as a risk factor of graft failure, with a hazard ratio of 5.174. Eyes with a poor prognosis showed statistically greater central corneal thickness at 1 month, greater graft thickness at 3 months, and a shallower anterior chamber at 6 and 12 months.ConclusionsThe long-term outcome of DSAEK in eyes with ICE syndrome is relatively poor. Immediate postoperative complications, postkeratoplasty glaucoma surgery, thicker corneal parameters, and a shallow anterior chamber were all associated with graft failure.

A singular case of congenital self-healing histiocytosis with skin, liver and atypical eye involvement.

PURPOSE: To describe a rare case of congenital self-healing Langerhans cell histiocytosis (CSHLCH) presenting with atypical eye involvement. DESIGN: Case report. METHODS: A female newborn presented with purpuric lesions over the trunk, limbs, and face. Liver ultrasonography revealed hypoechogenic lesions with blurred borders. Biomicroscopy showed right posterior synechiae with fibrinoid deposits on the lens. At 7 months she presented with right acute glaucoma. RESULTS: Biomicroscopy showed the presence of inflammatory pseudo-membrane covering the anterior surface of the lens, iris, and iridocorneal angle. Ab externo trabeculotomy was performed; access to the anterior chamber with capsulorrhexis forceps permitted a peeling of the pseudo-membrane with normalization of the intraocular pressure. Histologic examination of the membrane revealed an inflammatory tissue with CD1a and S-100 positive histiocytic cells. CONCLUSIONS: This is the first case of CSHLCH describing acute glaucoma secondary to a pseudo-inflammatory membrane with typical histiocytic cells, occluding the iridocorneal angle.

Ocular gene transfer of active TGF-beta induces changes in anterior segment morphology and elevated IOP in rats.

Purpose. Transforming growth factor beta (TGF-beta) is known to play a crucial role in wound healing and fibrotic tissue remodeling. A large body of evidence suggests a role for this cytokine in the pathogenesis of glaucoma; however, the mechanisms by which it affects anterior segment morphology are not well understood. Therefore, the purpose of this study was to examine the effects of TGF-beta overexpression on anterior segment morphology and subsequent effects on intraocular pressure. Methods. Adenoviral gene transfer was used to deliver active TGF-beta1 to the rat eye. Measurements of intraocular pressure were taken with a tonometer on days 0, 14, 21, and 29. Histologic analysis was undertaken to examine anterior segment morphology, and markers of matrix deposition and fibrosis were used. Results. Gene transfer of TGF-beta in the anterior segment resulted in the formation of peripheral anterior synechiae (PAS), which consisted of a fibroproliferative region of corneal endothelial cells, matrix accumulation, and decrease in trabecular meshwork expression of alpha-smooth muscle actin. These features were accompanied by ocular hypertension. Conclusions. Gene transfer of TGF-beta into the anterior segment induces aberrant PAS associated with the transition of corneal endothelial cells and subsequent matrix deposition. These features are highly reminiscent of human iridocorneal endothelial (ICE) syndrome. Gene transfer of TGF-beta can, therefore, be used to induce anatomic changes in the anterior segment in a rodent model that result in ocular hypertension.