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

Abstract

Purpose: To report a case of a woman who had Ehlers Danlos syndrome who developed a bullous retinal detachment.

Observations: A 33-year-old Caucasian woman presented with 1-day history of floaters and photopsia. Patient had extensive scleral ectasia. Scleral buckle could not be performed due the severity of the ectasia. Patient had a vitrectomy and subsequently had multiple re-detachments with sclera ruptures during the retinal detachment repairs. Patient required a 360-degree scleral patch graft to prevent scleral ruptures during the vitrectomy. Patient has a stable flat report 7 months post-op with vision of 1/60.

Conclusions and importance: This is the first case report of a patient requiring a 360-degree scleral patch graft. This option should be considered to assist in preventing scleral ruptures, intraoperatively and post operatively in patient who have an increased risk of scleral rupture, such as patients with connective tissue disorders.

Keywords: EDS; Ehlers Danlos syndrome; Retinal detachment; Scleral ectasia; Scleral patch graft; Scleral rupture.

Fig. 1

This post-operative picture shows the 360 scleral patch grafts supporting the sclera between the recti muscles. The graft was attached posteriorly to cover the equator with tissue glue.

Fig. 2

This intraoperative picture shows the extent of sclera ectasia.

Fig. 3

This is an image of the right fundus. The poor quality was due to the posterior capsule opacification. This patient was reluctant for laser to correct this.

Fig. 4

This is image of the left fundus. .