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.

Outcomes of Vitrectomy for Long-Duration Macular Hole.

: The present study investigated the functional and anatomical outcomes of idiopathic chronic macular hole (MH) surgery with different surgical approaches related to the chronicity and diameter of the MH. A comparative retrospective study between three groups of patients who underwent vitrectomy for long-duration MH (mean: 13.5 months) was conducted. In the first group of patients (G1 or IP), the internal limiting membrane (ILM) was systematically peeled; in the second group (G2 or IPEP), the ILM and epiretinal membrane (ERM) were peeled; and in the third group (G3 or IF), patients underwent inverted ILM flap technique surgery. Pre- and post-operative best corrected visual acuities (pre- and post-op BCVA) were studied. Macular optical coherence tomography (OCT) scans were performed to measure the MH minimum and maximum diameter pre-operatively, as well as to confirm its post-op closure and evaluate the integrity of the ellipsoid zone (EZ). Fifty eyes of 48 patients (33 female and 15 male) were retrospectively evaluated. MH closure rate was 100% in IP group, 66.7% in IPEP, and 95.2% in IF group. All three groups had a statistically significant improvement of BCVA. EZ post-op was restored in 88.2% of the cases from G1, 41.6% from G2, and 23.8% from G3. No statistically significant relationship between the smaller or larger MH diameter and the visual acuity improvement was found. Patients with chronic MH and ERM have worse functional and anatomical outcomes after surgery. Treatment of chronic MHs without ERM results in a better closure rate with either an inverted ILM flap approach or systematic ILM peel.