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

Abstract

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.

Keywords: Ehlers danlos syndrome; Scleral patch graft; Scleral rupture.

Fig. 1

External color photographs of the right eye. A. At presentation revealing hyphema, extensive subconjunctival hemorrhage, and a nasal area of scleral perforation with uveal exposure. B. Prior to surgery, revealing the extent of corectopia and residual hyphema. Postoperative days 1 and 12, C and D, respectively, showing resolution of the hyphema, as well as a well-positioned scleral patch that is well covered with the conjunctiva. On the later, a small corneal dellen is noted in association with the graft. (For interpretation of the references to color in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

Physical examination findings included hyperextensibility of the elbow and wrist joints, as depicted in A and B, respectively. Chest X-Ray posteroanterior and lateral, C and D, respectively, revealed evidence of prior scoliosis repair surgery.