APR 02, 2020
AAO 2019 Video Program
We present a case of an 8-year-old child with Ehlers-Danlos syndrome and a chronic inferior retinal detachment that was repaired with vitrectomy. The patient had a history of prior surgery in that eye related to trauma at the age of 3 and was aphakic. There was severe nasal scleromalacia, likely related to his underlying connective tissue disorder and the prior trauma/surgery. A 23-gauge vitrectomy setup was initiated. There was continuous leakage around the nasal cannula, and it failed to remain in position. The sclerotomy then enlarged significantly and caused transient hypotony with additional complications, including choroidal effusions, hyphema and a large subretinal hemorrhage, which were managed. The surgery was also challenging because of the difficulty in inducing a posterior hyaloid detachment and the need for removal of subretinal membranes. A large subretinal hemorrhage was removed with 23-gauge forceps through a retinotomy. The retina was successfully reattached, and a silicone oil tamponade was used.