RD repair using 360-degree scleral graft for extensive scleral ectasia in a patient with Ehlers Danlos syndrome

Whitlow, Shane; Idrees, Zubair

doi:10.1016/j.ajoc.2019.100554

Cited by 4 publications

(8 citation statements)

References 10 publications

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“…A similar course of the disease was reported by Whitlow et al [ 7 ]. Their patient with EDS type VIa and bullous retinal detachment required several surgeries with scleral patch grafts and 20-gauge PPVs because of retinal detachment with proliferative vitreoretinopathy.…”

Section: Discussionsupporting

confidence: 87%

“…Very few reports have been published on the occurrence of retinal detachment in patients with EDS [ 4 – 7 ], and only two of these describe a clinical case of RRD that was managed by a pars plana vitrectomy (PPV) [ 6 , 7 ]. In both of the cases described, the patients underwent several surgeries due to redetachment with proliferative vitreoretinopathy, ultimately ending up with silicone oil tamponade and worse visual acuity [ 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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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.

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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“…A recent case report described the use of a 360-degree scleral patch graft in a patient with Type VI EDS who developed re-detachments and scleral rupture during vitrectomy for retinal detachment repairs. 4 In 1986, Nakazawa and colleagues reported the use of preserved scleral graft for the management of a post-traumatic staphyloma in a patient with Type VI EDS. 10 Trabeculectomy with a scleral patch graft has also been reported for the management of a patient with Type VI EDS and advanced glaucoma.…”

Section: Discussionmentioning

confidence: 99%

“… 1 , 2 The EDS comprises a heterogeneous spectrum of monogenetic conditions with multisystemic and variable clinical manifestations affecting the skin; ligaments and joints, blood vessels; and internal organs. 3 , 4 …”

Section: Introductionmentioning

confidence: 99%

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Successful repair of a spontaneous scleral rupture in a patient with type VI Ehlers-Danlos syndrome

Lozada

Amaral

Alvarez-Falcón

et al. 2020

American Journal of Ophthalmology Case Reports

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Purpose To describe ocular findings in a patient with Type VI Ehlers-Danlos syndrome (EDS) and make ophthalmologists aware of the potential ophthalmic complications of this particular type of EDS. To briefly report the surgical technique utilized for the repair of spontaneous scleral rupture that may be associated with Type VI Ehlers-Danlos syndrome. Observations A 36-year-old female visited the Emergency Room due to sudden vision loss, edema, and redness of the right eye consistent with spontaneous scleral rupture secondary to scleral thinning due to Type VI EDS. Repair with scleral patch graft resulted in improvement in visual acuity, a decrease in hyphema, and discomfort resolution. Conclusions and Importance Spontaneous scleral perforation may occur in patients with Type VI EDS. A scleral patch graft may serve as a viable surgical repair alternative for such patients.

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Scleral rupture during retinal detachment repair with primary scleral buckle and cryoretinopexy in a patient with microspherophakia

Johnson

Tausif

Tanna

et al. 2022

American Journal of Ophthalmology Case Reports

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RD repair using 360-degree scleral graft for extensive scleral ectasia in a patient with Ehlers Danlos syndrome

Cited by 4 publications

References 10 publications

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

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

Successful repair of a spontaneous scleral rupture in a patient with type VI Ehlers-Danlos syndrome

Scleral rupture during retinal detachment repair with primary scleral buckle and cryoretinopexy in a patient with microspherophakia

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