Outcomes of vitrectomy for retinal detachment in a patient with Ehlers-Danlos syndrome type IV: a case report.

BACKGROUND: The Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders characterized by fragile blood vessels and an increased tendency for bleeding and scarring. Here, we report the outcome of a pars plana vitrectomy for the treatment of rhegmatogenous retinal detachment in a patient with EDS type IV (vascular type). CASE PRESENTATION: A 40-year-old Slovenian man with high myopia, unilateral bullous retinal detachment, and vitreous hemorrhage was referred for surgery. The patient had a history of colon perforation, muscle and arterial rupture in both lower limbs, and recurrent shoulder joint luxations. Genetic testing revealed a pathogenic mutation in the COL3A1 gene. The patient underwent a 25-gauge three-port pars plana vitrectomy. The tendency for bleeding during surgery was prevented by endodiathermy applied to the edges of the retinal breaks. Endolaser photocoagulation was performed under air. The surgical procedure was completed with the injection of gas tamponade, followed by the patient remaining for a few days in a face-down position. Mild postoperative vitreous hemorrhage was resorbed in first week after the surgery. Postoperative extensive pigment dispersion on the posterior lens face persisted for several weeks. After the gas tamponade had resorbed, the retina was flat and remained attached during the follow-up period. Eight months after the surgery, visual acuity continued to improve from a preoperative 6/38 to 6/6.6 (Snellen chart) at the last checkup. CONCLUSION: A small-gauge pars plana vitrectomy with gas tamponade and laser photocoagulation under air may successfully achieve reattachment of the retina in patients with high myopia with EDS type IV and restore visual acuity.

Iris cerclage pupilloplasty and IOL implantation for traumatic mydriasis and aphakia after the blunt trauma of the eye.

Four patients with traumatic mydriasis and aphakia following blunt injury of the eye globe were evaluated. Patients with severe glare and photophobia due to wide pupil diameter from 6.0 to 9.0 mm were managed by combined iris cerclage pupilloplasty and retropupillar iris-claw lens implantation. The postoperative anatomic results, visual acuity, subjective degree of glare, photophobia, as well as intraoperative and postoperative complications were evaluated. The mean follow-up time was 32.6 months. Best-corrected visual acuity improved in all patients from preoperative 20/60, 20/30, 20/25, 20/22 to postoperative 20/20, 20/22, 20/20, and 20/20 (Snellen charts). All eyes achieved satisfactory anatomic result with round pupil diameter 3.5-4.5 mm. Glare and photophobia disappeared in all patients. There were no intraoperative or postoperative complications. During the follow-up period, pupils remained round and iris-claw lenses well positioned, without tilting. Combined iris cerclage pupilloplasty with retropupillar iris claw lens implantation appears to be a safe and effective surgical technique in reducing pupil size and improving visual outcomes. It is also a cost- and time-effective procedure, providing great results with a single surgery.

Prognostic Factor Analysis of Visual Outcome after Vitrectomy for Rhegmatogenous Retinal Detachment.

Pars plana vitrectomy (PPV) is a surgical approach mainly chosen for complex rhegmatogenous retinal detachment (RRD) repair with highly variable functional results. The aim of this analysis was to evaluate the impact of preoperative factors and postoperative optical coherence tomography (OCT) macular findings on the functional outcome of patients undergoing primary PPV for RRD. A retrospective analysis was performed on 88 eyes of 88 patients with complex RRD managed by PPV. A swept source OCT was used to obtain images at the postoperative visit at least 6 months after PPV. Hierarchical linear regression model was used to evaluate the influence of preoperative factors related to patient, ocular clinical and postoperative OCT macular findings on functional outcomes of PPV for RRD. Duration of symptoms (p = 0.031) and discontinuity of the ellipsoid zone (EZ) on OCT (p = 0.024) showed statistically significant negative correlation, while preoperative best-corrected visual acuity (BCVA; p < 0.001) showed statistically significant positive correlation to postoperative BCVA. Preoperative BCVA and duration of symptoms can be used as prognostic factors for visual outcome in patients undergoing PPV for RRD. Discontinuity of the EZ was the only postoperative OCT variable related to worse postoperative visual outcome.