Abstract
Abstract: :
Purpose: To describe clinical features and surgical management of patients with microphthalmic eyes having the co–association of persistent fetal vasculature (PFV) and chorio–retinal coloboma. Methods: Retrospective chart review of patients managed at a major children’s retina referral center with clinical diagnosis of PFV and chorioretinal coloboma. Results:Of 110 patients with PFV seen over a five–year interval, five patients were identified to have co–existing chorio–retinal coloboma. In two of these patients, the process was bilateral. All had significant micropthalmia in the range of 13 to 19 mm. One child had evidence of Trisomy 13 and cardiac abnormalities. All of the children had media opacity caused by pupillary occlusion, lens opacities, or retrolental and cyclitic membranes. All seven eyes of the five children underwent pars plicata vitrectomy between the ages of one and nine months, with removal of membranes. Two eyes developed retinal detachment post–operatively and were successfully repaired with a second vitrectomy and scleral buckle. One had an entry–site dialysis detected two weeks after primary surgery, and the other developed tractional detachment associated with significant vaso–proliferation. Although deprivational amblyopia was present in all eyes, this improved with therapy in all eyes, except the vaso–proliferative case, to the point that some fixation was achieved. Conclusions:Some microphthalmic eyes have a co–existence of PFV and chorio–retinal coloboma. Developmental aberrations around four weeks of gestation are presumably responsible for both abnormalities. Most of these eyes have severe anterior segment disorganization and media opacity. Modern vitreo–retinal surgery can result in some improvement of anatomy and visual function, although re–proliferation remains a major cause of anatomic failure. Surgical intervention is most likely to benefit the bilateral cases, especially when offered in the first months of life.
Keywords: vitreoretinal surgery • clinical (human) or epidemiologic studies: outcomes/complications • infant vision