Angioid streaks and obstructive sleep apnea syndrome: are they related?

BACKGROUND: Sleep apnea syndrome (OSAS) has been associated with different ocular manifestations including glaucoma, floppy eye syndrome, punctate keratitis, keratoconus, and optic neuropathy. Angioid streaks are mainly associated with pseudoxanthoma elasticum (PXE) although they can appear in other systemic conditions affecting the elastic fibers. METHODS: This is a prospective, cross-sectional study. A complete ophthalmic examination was performed in 92 patients undergoing overnight polysomnography for suspicion of OSAS. Diagnosis and classification of OSAS were made based on apnea-hypopnea index (AHI). Stereoscopic optic disc photographs were taken in all patients and independently evaluated by two ophthalmologists. Patients with angioid streaks were referred to a dermatologist for axillary skin biopsy in order to rule out pseudoxanthoma elasticum or other skin abnormalities. RESULTS: Bilateral angioid streaks were observed in three patients who had been diagnosed with severe OSAS (AHI > 30/h). No clinical features characteristic of pseudoxanthoma elasticum or other pathological skin signs were observed. Skin biopsies were normal for all three patients, supporting the diagnosis of idiopathic angioid streaks. One of the patients developed bilateral choroidal neovascularization secondary to the angioid streaks over subsequent years. CONCLUSIONS: In view of the low prevalence of idiopathic angioid streaks in the general population, the finding of angioid streaks in patients with severe OSAS suggests OSAS as a possible risk factor for its development. The hypothesis of a connective tissue abnormality that could explain an association between both entities deserves further elucidation.

Ex-Press P50 device filtering failure due to non-visible intraluminal blockages.

PURPOSE: To describe Ex-Press P50 failures due to an internal blockage. METHODS: A retrospective series of 248 eyes of 219 patients with Ex-Press P50 surgery was conducted. Of these cases, 18 (7.2%) required surgical revision due to filtration failure with no visible blockages in the gonioscopy and no filtering bleb in the slit lamp examination. In all of these cases, we found fibrosis on the edges of the scleral flap. After dissecting the scleral flap, filtration through the implant was restored spontaneously in 10 (4.0%) cases. In two cases fibrous tissue was observed on the tip of the implant and was necessary to remove it. In 6 (2.4%) cases, there was no filtration despite no visible obstruction and attempts to restore the flow were unsuccessful, requiring extraction of the implant and reconverting to trabeculectomy. Two of the removed devices were sanded until the lumen was visible to ascertain the nature of the obstruction. RESULTS: In our series, the most common cause of failure was episcleral fibrosis. In 2.4% of the cases, the blockage was intraluminal and aqueous flow could not be restored. CONCLUSION: In the Ex-Press P50 implant an internal blockage, that is not visible through the proximal or distal orifices, may be the cause of failure. Unblocking by surgical maneuvers is not feasible due to the intraluminal design. These cases can be solved by extraction of the implant and conversion to trabeculectomy.

Anterior iris-claw intraocular lens implantation for the management of nontraumatic ectopia lentis: long-term outcomes in a paediatric cohort.

PURPOSE: To report the feasibility and long-term safety of lensectomy and iris-claw intraocular lens (IOL) implantation to treat children with severe ectopia lentis in a paediatric tertiary hospital. METHODS: Prospective cohort study of 21 eyes from 12 patients with severe ectopia lentis and visual acuity <20/63. All eyes underwent 23-gauge pars plana vitrectomy, lensectomy, iridectomy and Artisan IOL implantation in the anterior chamber with iris-claw enclavation via pars plana. Mean age at surgery was 8.0 ± 5.3 yo (range 3-17 years). A full ophthalmologic examination including best-corrected visual acuity (BCVA), biomicroscopy, intraocular pressure (IOP) measurement, fundus evaluation and central endothelial cell count (cECC) was performed pretreatment, at 3 months' postsurgery, and every 6 months thereafter. Ultrasound biomicroscopy (UBM) was performed 12 months after surgery. RESULTS: Mean follow-up was 39.3 ± 13.0 months. Best-corrected visual acuity (BCVA) (mean ± SD) improved from 0.91 ± 0.29 logMar preoperatively to 0.18 ± 0.23 logMar at final follow-up (p < 0.0001). Mean distance from the endothelium to the anterior IOL surface after surgery was 3.11 ± 0.61 mm. Postsurgically, cECC loss was 5.04% ± 9.58% with an annual cECC loss rate of 3.16% ± 4.46%. One patient developed IOL dislocation and retinal detachment after severe ocular contusion requiring vitrectomy, IOL refixation and gas tamponade. Another patient developed cystoid macular oedema, managed with intravitreal dexamethasone. CONCLUSION: This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.